Normal pressure hydrocephalus

MedlinePlus

Ferri FF. Normal pressure hydrocephalus. In: Ferri FF, ed. Ferri's Clinical Advisor 2016 . Philadelphia, PA: Elsevier; 2016:chap 648. Rosenberg GA. Brain edema and disorders of cerebrospinal fluid circulation. ...

CSF Flow in the Brain in the Context of Normal Pressure Hydrocephalus.

PubMed

Bradley, W G

2015-05-01

CSF normally flows back and forth through the aqueduct during the cardiac cycle. During systole, the brain and intracranial vasculature expand and compress the lateral and third ventricles, forcing CSF craniocaudad. During diastole, they contract and flow through the aqueduct reverses. Hyperdynamic CSF flow through the aqueduct is seen when there is ventricular enlargement without cerebral atrophy. Therefore, patients presenting with clinical normal pressure hydrocephalus who have hyperdynamic CSF flow have been found to respond better to ventriculoperitoneal shunting than those with normal or decreased CSF flow. Patients with normal pressure hydrocephalus have also been found to have larger intracranial volumes than sex-matched controls, suggesting that they may have had benign external hydrocephalus as infants. While their arachnoidal granulations clearly have decreased CSF resorptive capacity, it now appears that this is fixed and that the arachnoidal granulations are not merely immature. Such patients appear to develop a parallel pathway for CSF to exit the ventricles through the extracellular space of the brain and the venous side of the glymphatic system. This pathway remains functional until late adulthood when the patient develops deep white matter ischemia, which is characterized histologically by myelin pallor (ie, loss of lipid). The attraction between the bare myelin protein and the CSF increases resistance to the extracellular outflow of CSF, causing it to back up, resulting in hydrocephalus. Thus idiopathic normal pressure hydrocephalus appears to be a "2 hit" disease: benign external hydrocephalus in infancy followed by deep white matter ischemia in late adulthood. © 2015 by American Journal of Neuroradiology.

Impaired pulsation absorber mechanism in idiopathic normal pressure hydrocephalus: laboratory investigation.

PubMed

Park, Eun-Hyoung; Eide, Per Kristian; Zurakowski, David; Madsen, Joseph R

2012-12-01

The pathophysiology of normal pressure hydrocephalus (NPH), and the related problem of patient selection for treatment of this condition, have been of great interest since the description of this seemingly paradoxical condition nearly 50 years ago. Recently, Eide has reported that measurements of the amplitude of the intracranial pressure (ICP) can both positively and negatively predict response to CSF shunting. Specifically, the fraction of time spent in a "high amplitude" (> 4 mm Hg) state predicted response to shunting, which may represent a marker for hydrocephalic pathophysiology. Increased ICP amplitude might suggest decreased brain compliance, meaning a static measure of a pressure-volume ratio. Recent studies of canine data have shown that the brain compliance can be described as a frequency-dependent function. The normal canine brain seems to show enhanced ability to absorb the pulsations around the heart rate, quantified as a cardiac pulsation absorbance (CPA), with properties like a notch filter in engineering. This frequency dependence of the function is diminished with development of hydrocephalus in dogs. In this pilot study, the authors sought to determine whether frequency dependence could be observed in humans, and whether the frequency dependence would be any different in epochs with high ICP amplitude compared with epochs of low ICP amplitude. Systems analysis was applied to arterial blood pressure (ABP) and ICP waveforms recorded from 10 patients undergoing evaluations of idiopathic NPH to calculate a time-varying transfer function that reveals frequency dependence and CPA, the measure of frequency-dependent compliance previously used in animal experiments. The ICP amplitude was also calculated in the same samples, so that epochs with high (> 4 mm Hg) versus low (≤ 4 mm Hg) amplitude could be compared in CPA and transfer functions. Transfer function analysis for the more "normal" epochs with low amplitude exhibits a dip or notch in the

Correlation between lumbo-ventricular perfusion and MRI-CSF flow studies in idiopathic normal pressure hydrocephalus.

PubMed

Hakim, R; Black, P M

1998-01-01

After the initial description of normal pressure hydrocephalus (NPH) and its clinical triad, there has been a continuous interest from clinicians and researchers to set different diagnostic criteria that would make the selection of candidates for shunt surgery easier and more precise. A preliminary group of 12 patients was given a diagnosis of idiopathic normal pressure hydrocephalus by clinical and radiologic criteria. Each patient underwent two different tests: a magnetic resonance imaging-cerebrospinal fluid (MRI-CSF) flow study and a lumbo-ventricular perfusion test. The purpose was to compare the correlation of the results obtained with these tests and the clinical results obtained after CSF diversion. Eleven patients were given shunts and one was managed with lumbar punctures. One year after treatment, 10 of the 12 patients had improved with good results. The MRI-CSF flow studies were reliable in six patients; there were five false negatives and one false positive. The lumbo-ventricular perfusion test showed reliability in nine patients; there were two false negatives and one false positive. In only three patients were the results of both of these tests in accordance with the outcome. Even though there are few patients in this study so far, the data suggests that at the present time the most predictive guides for the diagnosis of NPH and its outcome after shunting are the clinical criteria and the radiological findings in computed tomography (CT) and/or MRI rather than lumbo-ventricular perfusion and CSF flow studies.

Idiopathic normal pressure hydrocephalus: the CSF tap-test may predict the clinical response to shunting.

PubMed

Sand, T; Bovim, G; Grimse, R; Myhr, G; Helde, G; Cappelen, J

1994-05-01

A follow-up study was performed in nine patients with idiopathic normal pressure hydrocephalus (NPH) 37 months (mean) after shunting and 10 non-operated controls with comparable degrees of ventricular enlargement, gait disorder, and dementia. Five operated patients vs. no controls reported sustained general improvement (p < 0.02). Objectively improved gait at follow-up (compared with preoperative status) was found in five of the six tested NPH-patients vs. none of the controls (p < 0.005). Improved gait and/or psychometric function was found in four of six NPH vs. none of eight control patients (p < 0.02) after drainage of 40 ml cerebrospinal fluid (CSF tap-test). Improved gait during the CSF tap-test predicted continued improvement at follow-up. Temporal horn size was the only radiological variable which showed a (moderate) positive correlation with resistance to CSF absorption and rate of pressure increase. The size of the third ventricle diminished in parallel with clinical improvement.

Oxygen metabolism changes in patients with idiopathic normal pressure hydrocephalus before and after shunting operation.

PubMed

Miyamoto, J; Tatsuzawa, K; Inoue, Y; Imahori, Y; Mineura, K

2007-09-01

The present study revealed the changes in cerebral oxygen metabolism before and after ventriculo-peritoneal shunt (VPS) using (15)O positron emission tomography ((15)O-PET). Eight patients with idiopathic normal pressure hydrocephalus (i-NPH) underwent VPS. A (15)O-PET study was undertaken before and approximately 3 months after VPS. In five patients, the symptoms improved based on the classification by Krauss et al. [Neurosurgery 1996;39:292] (good responders) after VPS. In three patients, the symptoms improved subjectively following VPS (poor responders). The changes in oxygen metabolism before and after VPS were analyzed. The postoperative regional cerebral metabolic rate of oxygen (rCMRO(2)) of the good responders increased significantly. The postoperative regional oxygen extraction fraction (rOEF) is reduced in the poor responders. The improvement of rCMRO(2) correlated with the response to VPS. Significant changes in rOEF might predict poor response to VPS.

Fluid Distribution Pattern in Adult-Onset Congenital, Idiopathic, and Secondary Normal-Pressure Hydrocephalus: Implications for Clinical Care.

PubMed

Yamada, Shigeki; Ishikawa, Masatsune; Yamamoto, Kazuo

2017-01-01

In spite of growing evidence of idiopathic normal-pressure hydrocephalus (NPH), a viewpoint about clinical care for idiopathic NPH is still controversial. A continuous divergence of viewpoints might be due to confusing classifications of idiopathic and adult-onset congenital NPH. To elucidate the classification of NPH, we propose that adult-onset congenital NPH should be explicitly distinguished from idiopathic and secondary NPH. On the basis of conventional CT scan or MRI, idiopathic NPH was defined as narrow sulci at the high convexity in concurrent with enlargement of the ventricles, basal cistern and Sylvian fissure, whereas adult-onset congenital NPH was defined as huge ventricles without high-convexity tightness. We compared clinical characteristics and cerebrospinal fluid distribution among 85 patients diagnosed with idiopathic NPH, 17 patients with secondary NPH, and 7 patients with adult-onset congenital NPH. All patients underwent 3-T MRI examinations and tap-tests. The volumes of ventricles and subarachnoid spaces were measured using a 3D workstation based on T2-weighted 3D sequences. The mean intracranial volume for the patients with adult-onset congenital NPH was almost 100 mL larger than the volumes for patients with idiopathic and secondary NPH. Compared with the patients with idiopathic or secondary NPH, patients with adult-onset congenital NPH exhibited larger ventricles but normal sized subarachnoid spaces. The mean volume ratio of the high-convexity subarachnoid space was significantly less in idiopathic NPH than in adult-onset congenital NPH, whereas the mean volume ratio of the basal cistern and Sylvian fissure in idiopathic NPH was >2 times larger than that in adult-onset congenital NPH. The symptoms of gait disturbance, cognitive impairment, and urinary incontinence in patients with adult-onset congenital NPH tended to progress more slowly compared to their progress in patients with idiopathic NPH. Cerebrospinal fluid distributions and

18F-FDG PET-CT pattern in idiopathic normal pressure hydrocephalus.

PubMed

Townley, Ryan A; Botha, Hugo; Graff-Radford, Jonathan; Boeve, Bradley F; Petersen, Ronald C; Senjem, Matthew L; Knopman, David S; Lowe, Val; Jack, Clifford R; Jones, David T

2018-01-01

Idiopathic normal pressure hydrocephalus (iNPH) is an important and treatable cause of neurologic impairment. Diagnosis is complicated due to symptoms overlapping with other age related disorders. The pathophysiology underlying iNPH is not well understood. We explored FDG-PET abnormalities in iNPH patients in order to determine if FDG-PET may serve as a biomarker to differentiate iNPH from common neurodegenerative disorders. We retrospectively compared 18 F-FDG PET-CT imaging patterns from seven iNPH patients (mean age 74 ± 6 years) to age and sex matched controls, as well as patients diagnosed with clinical Alzheimer's disease dementia (AD), Dementia with Lewy Bodies (DLB) and Parkinson's Disease Dementia (PDD), and behavioral variant frontotemporal dementia (bvFTD). Partial volume corrected and uncorrected images were reviewed separately. Patients with iNPH, when compared to controls, AD, DLB/PDD, and bvFTD, had significant regional hypometabolism in the dorsal striatum, involving the caudate and putamen bilaterally. These results remained highly significant after partial volume correction. In this study, we report a FDG-PET pattern of hypometabolism in iNPH involving the caudate and putamen with preserved cortical metabolism. This pattern may differentiate iNPH from degenerative diseases and has the potential to serve as a biomarker for iNPH in future studies. These findings also further our understanding of the pathophysiology underlying the iNPH clinical presentation.

Choroidal fissure acts as an overflow device in cerebrospinal fluid drainage: morphological comparison between idiopathic and secondary normal-pressure hydrocephalus

PubMed Central

Yamada, Shigeki; Ishikawa, Masatsune; Iwamuro, Yasushi; Yamamoto, Kazuo

2016-01-01

To clarify the pathogenesis of two different types of adult-onset normal-pressure hydrocephalus (NPH), we investigated cerebrospinal fluid distribution on the high-field three-dimensional MRI. The subarachnoid spaces in secondary NPH were smaller than those in the controls, whereas those in idiopathic NPH were of similar size to the controls. In idiopathic NPH, however, the basal cistern and Sylvian fissure were enlarged in concurrence with ventricular enlargement towards the z-direction, but the convexity subarachnoid space was severely diminished. In this article, we provide evidence that the key cause of the disproportionate cerebrospinal fluid distribution in idiopathic NPH is the compensatory direct CSF communication between the inferior horn of the lateral ventricles and the ambient cistern at the choroidal fissure. In contrast, all parts of the subarachnoid spaces were equally and severely decreased in secondary NPH. Blockage of CSF drainage from the subarachnoid spaces could cause the omnidirectional ventricular enlargement in secondary NPH. PMID:27941913

Poor cognitive outcome in shunt-responsive idiopathic normal pressure hydrocephalus.

PubMed

Koivisto, Anne M; Alafuzoff, Irina; Savolainen, Sakari; Sutela, Anna; Rummukainen, Jaana; Kurki, Mitja; Jääskeläinen, Juha E; Soininen, Hilkka; Rinne, Jaakko; Leinonen, Ville

2013-01-01

Idiopathic normal pressure hydrocephalus (iNPH) causes cognitive decline that can be alleviated by shunting, but long-term outcome studies are scarce. To elucidate the long-term cognitive condition of shunt-responsive iNPH patients. The follow-up data (Kuopio University Hospital NPH Registry) of 146 patients diagnosed with iNPH by clinical and radiological examination, 24-hour intraventricular pressure monitoring, frontal cortical biopsy, and response to the shunt were analyzed for signs of dementia. The Diagnostic and Statistical Manual for Mental Disorders, Fourth Edition, and specified memory disorder criteria were used. Median follow-up was 4.8 years. At the end of follow-up, 117 (80%) of the 146 iNPH patients had cognitive decline and 67 (46%) had clinical dementia. The most common clinical diagnoses were Alzheimer disease and vascular dementia. In multivariate analysis of the 146 iNPH patients, memory deficit as a first symptom before shunt (odds ratio [OR] 18.3; 95% confidence interval [CI] 1.9-175), male sex (OR 3.29; 95% CI 1.11-9.73), age (OR 1.17 year; 95% CI 1.07-1.28), and follow-up time (OR 1.20 year; 95% CI 1.02-1.40) predicted dementia. Interestingly, 8 (5%) iNPH patients had dementia without any signs of other neurodegenerative diseases in clinical, neuroradiological, or brain biopsy evaluation. These patients initially presented a full triad of symptoms, with gait disturbance being the most frequent initial symptom followed by deterioration in cognition. The novel findings were (a) a significant risk of dementia in iNPH initially responsive to cerebrospinal fluid shunt, (b) cognitive impairment most commonly due to iNPH-related dementia followed by concurrent degenerative brain disease, and (c) a subgroup with dementia related to iNPH without comorbidities.

Idiopathic normal pressure hydrocephalus: analysis of factors related to cerebrospinal fluid dynamics determining functional prognosis.

PubMed

Bárcena, A; Mestre, C; Cañizal, J M; Rivero, B; Lobato, R D

1997-01-01

This investigation has been undertaken to analyze the findings with both the cerebrospinal fluid (CSF) pressure (Pcsf) and CSF pulse pressure (PP) in order to predict the outcome of patients with the syndrome of idiopathic normal pressure hydrocephalus (NPH). Accordingly, a prospective clinical study was planned in which two groups of patients with NPH, having analogous prevalence of several matched clinical and radiological parameters, were separated on the basis of their positive or negative response to shunting. Both the resting Pcsf and CSF PP profiles were compared in these two groups, and between them and normal controls. CSF PP amplitude and CSF PP latency correlated directly in conditions associated with either normal or high compliance (controls and patients with Alzheimer-like disorders), whereas this correlation was inverse in states of low compliance (NPH). On the other hand, shunt-responders showed a resting Pcsf significantly higher than both non-responders and controls. The following conclusions were obtained: 1) CSF PP is a high-amplitude and relative low-latency wave in NPH when compared with controls: 2) CSF PP amplitude and latency correlate directly in normal subjects and in those with primary cerebral atrophy; 3) a non-reversible stage of NPH could be conceived in contradistinction to the reversible one, in both of which an inverse correlation between the amplitude and the latency takes place, the main difference between them being the resting Pcsf, which is significantly lower in the former than in the latter, depending on the degree of atrophic changes developed.

Reversed aqueductal cerebrospinal fluid net flow in idiopathic normal pressure hydrocephalus.

PubMed

Yin, L K; Zheng, J J; Zhao, L; Hao, X Z; Zhang, X X; Tian, J Q; Zheng, K; Yang, Y M

2017-11-01

The changes of CSF flow dynamics in idiopathic normal pressure hydrocephalus (iNPH) are not fully elucidated. Most previous studies took the whole cardiac cycle as a unit. In this work, it is divided into systole and diastole phase and compared between iNPH patients and normal elderly and paid special attention to the change of netflow direction. Twenty iNPH patients according to international guideline and twenty healthy volunteers were included in this study and examined by MRI. Three categories of CSF flow parameters were measured: peak velocity (V peak ), stroke volume (SV), and minute flow volume (MinV) covering the whole cycle; peak velocity (V peak-s , V peak-d ) and flow volume (Vol s , Vol d ) of the systole and diastole, respectively; net flow. Evans index (EI) was also measured and compared statistically between the two groups. EI, V peak , SV, MinV, Vol s , Vol d , and V peak-d significantly increased in iNPH group (P<0.05). V peak-s of the two groups were not significantly different (P>0.05). The net flow of 16 iNPH patients (16/20) was in the caudo-cranial direction, while 15 volunteers (15/20) were in the opposite direction, which showed statistically significant differences (P=.001). INPH patients present hyperdynamic flow with increased velocity and volume both in systole and diastole phase. Degree of rising in diastole phase exceeds that of systole phase. The resulting reversal of netflow direction may play a key role in the occurrence of ventriculomegaly in iNPH patients. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Amyloid deposits and response to shunt surgery in idiopathic normal-pressure hydrocephalus.

PubMed

Hiraoka, Kotaro; Narita, Wataru; Kikuchi, Hirokazu; Baba, Toru; Kanno, Shigenori; Iizuka, Osamu; Tashiro, Manabu; Furumoto, Shozo; Okamura, Nobuyuki; Furukawa, Katsutoshi; Arai, Hiroyuki; Iwata, Ren; Mori, Etsuro; Yanai, Kazuhiko

2015-09-15

In previous studies, patients with idiopathic normal-pressure hydrocephalus (iNPH) occasionally showed Alzheimer's pathology in frontal lobe cortical biopsy during cerebrospinal fluid shunt surgery or intracranial pressure monitoring. In clinical practice, the differential diagnosis of iNPH from Alzheimer's disease (AD) can be problematic, particularly because some iNPH cases exhibit AD comorbidity. In this study, we evaluated amyloid deposition in the brains of patients with iNPH before shunt surgery, and investigated the association between brain amyloid deposits and clinical improvement following the surgery. Amyloid imaging was performed in patients with iNPH or AD and also in healthy control subjects by using positron emission tomography (PET) and a radiolabeled pharmaceutical compound, (11)C-BF227. Using the cerebellar hemispheres as reference regions, the standard uptake value ratio (SUVR) of the neocortex was estimated and used as an index for amyloid deposition. In patients with iNPH, clinical symptoms were assessed before shunt surgery and 3 months after surgery. Five of the 10 patients with iNPH had neocortical SUVRs that were as high as those of AD subjects, whereas the SUVRs of the 5 patients were as low as those of healthy controls. A significant inverse correlation between neocortical SUVRs and cognitive improvements after shunt surgery was observed in iNPH. The amount of amyloid deposits ranges widely in the brains of patients with iNPH and is associated with the degree of cognitive improvement after shunt surgery. Copyright © 2015 Elsevier B.V. All rights reserved.

Reversible grasp reflexes in normal pressure hydrocephalus.

PubMed

Thomas, Rhys H; Bennetto, Luke; Silva, Mark T

2009-05-01

We present two cases of normal pressure hydrocephalus in combination with grasp reflexes. In both cases the grasp reflexes disappeared following high volume cerebrospinal fluid removal. In one of the cases the grasp reflexes returned over a period of weeks but again resolved following definitive cerebrospinal fluid shunting surgery, and remained absent until final follow up at 9 months. We hypothesise that resolving grasp reflexes following high volume CSF removal has both diagnostic and prognostic value in normal pressure hydrocephalus, encouraging larger studies on the relevance of primitive reflexes in NPH.

Endoscopic third ventriculostomy (ETV) for idiopathic normal pressure hydrocephalus (iNPH).

PubMed

Tudor, Katarina Ivana; Tudor, Mario; McCleery, Jenny; Car, Josip

2015-07-29

Idiopathic normal pressure hydrocephalus (iNPH) is a type of communicating hydrocephalus also known as non-obstructive hydrocephalus. This type of hydrocephalus is caused by impaired cerebrospinal fluid reabsorption without any obstruction in the ventricular system and is associated with normal cerebrospinal fluid pressure. It is characterised clinically by gait disturbance, cognitive dysfunction, and urinary incontinence (known as the Hakim-Adams triad). The exact cause of iNPH is unknown. It may be managed conservatively or treated surgically by inserting a ventriculoperitoneal (VP) or ventriculoatrial (VA) shunt. However, a substantial number of patients do not respond well to surgical treatment, complication rates are high and there is often a need for further surgery. Endoscopic third ventriculostomy (ETV) is an alternative surgical intervention. It has been suggested that ETV may lead to better outcomes, including fewer complications. To determine the effectiveness of ETV for treatment of patients with iNPH compared to conservative therapy, or shunting of CSF using VP or VA shunts.To assess the perioperative and postoperative complication rates in patients with iNPH after ETV compared to conservative therapy, VP or VA shunting. We searched for eligible studies using ALOIS: a comprehensive register of dementia studies, The Cochrane Central Register of Controlled Trials (CENTRAL) and several bibliographic databases such as MEDLINE (Ovid SP), EMBASE (Ovid SP), PsycINFO (Ovid SP), CINAHL (EBSCOhost) and LILACS (BIREME).We also searched the Database of Abstracts of Reviews of Effects (DARE) to identify potentially relevant reviews. The search strategy was adapted for other databases, using the most appropriate controlled vocabulary for each. We did not apply any language or time restrictions. The searches were performed in August 2014. We included randomised controlled trials (RCTs) of ETV treatment of iNPH. Patients had to have at least two symptoms of the Hakim

Outcome of shunting in idiopathic normal-pressure hydrocephalus and the value of outcome assessment in shunted patients.

PubMed

Klinge, Petra; Marmarou, Anthony; Bergsneider, Marvin; Relkin, Norman; Black, Peter McL

2005-09-01

To develop guidelines for assessing shunt outcome in patients with idiopathic normal-pressure hydrocephalus (INPH). To date, the literature available on this topic has been marked by disparate definitions of clinical improvement, varying postoperative follow-up protocols and periods, and substantial differences in the postoperative management. Because specific criteria for defining clinical improvement are seldom reported, conclusions drawn about shunt outcome may be subjective. A MEDLINE search back to 1966 was undertaken using the query NPH, normal-pressure hydrocephalus, shunting, shunt treatment, shunt response, outcome, and clinical outcome. The criteria for selection were studies that included INPH from 1966 to the present in which the outcome of INPH was reported in patient groups of 20 or more. To date, there is no standard for outcome assessment of shunt treatment in INPH. The variable improvement rates reported are not only because of different criteria for selection of patients but also because of different postoperative assessment procedures and follow-up intervals. Studies that have established fixed protocols for follow-up have shown that short- and long-term periods after shunting are determined by many factors. Whereas short-term results were more likely to be influenced by shunt-associated risks, long-term results were independent of factors inherent to the shunt procedure and shunt complications, i.e., death and morbidity related to concomitant cerebrovascular and vascular diseases. Studies have shown that beyond 1 year after surgery, these factors definitely influence the clinical effect of shunting, making the 1-year postshunt period a potential determinant of the shunt outcome. Guidelines for outcome assessment were developed on the basis of the available evidence and consensus of expert opinion.

Counting-backward test for executive function in idiopathic normal pressure hydrocephalus.

PubMed

Kanno, S; Saito, M; Hayashi, A; Uchiyama, M; Hiraoka, K; Nishio, Y; Hisanaga, K; Mori, E

2012-10-01

The aim of this study was to develop and validate a bedside test for executive function in patients with idiopathic normal pressure hydrocephalus (INPH). Twenty consecutive patients with INPH and 20 patients with Alzheimer's disease (AD) were enrolled in this study. We developed the counting-backward test for evaluating executive function in patients with INPH. Two indices that are considered to be reflective of the attention deficits and response suppression underlying executive dysfunction in INPH were calculated: the first-error score and the reverse-effect index. Performance on both the counting-backward test and standard neuropsychological tests for executive function was assessed in INPH and AD patients. The first-error score, reverse-effect index and the scores from the standard neuropsychological tests for executive function were significantly lower for individuals in the INPH group than in the AD group. The two indices for the counting-backward test in the INPH group were strongly correlated with the total scores for Frontal Assessment Battery and Phonemic Verbal Fluency. The first-error score was also significantly correlated with the error rate of the Stroop colour-word test and the score of the go/no-go test. In addition, we found that the first-error score highly distinguished patients with INPH from those with AD using these tests. The counting-backward test is useful for evaluating executive dysfunction in INPH and for differentiating between INPH and AD patients. In particular, the first-error score may reflect deficits in the response suppression related to executive dysfunction in INPH. © 2012 John Wiley & Sons A/S.

CSF tapping also improves mental imagery of gait in normal pressure hydrocephalus.

PubMed

Marques, Bruno; Laidet, Magali; Armand, Stéphane; Assal, Frédéric; Allali, Gilles

2017-11-01

This study aims to compare the changes of Timed Up and Go (TUG) and its imagined version (iTUG) after CSF tapping between patients with idiopathic normal pressure hydrocephalus (iNPH) and its mimics. TUG and iTUG were performed before and 24 h after CSF tapping in 117 patients (75.8 ± 6.9 years; 35% female) with suspicion of iNPH (68 iNPH and 49 mimics). Mental imagery of locomotion was modified after CSF tapping in iNPH patients, but not in the mimics.

Comorbid Normal Pressure Hydrocephalus with Parkinsonism: A Clinical Challenge and Call for Awareness.

PubMed

Cucca, A; Biagioni, M C; Sharma, K; Golomb, J; Gilbert, R M; Di Rocco, A; Fleisher, J E

2018-01-01

Idiopathic normal pressure hydrocephalus (iNPH) is the most common cause of hydrocephalus in adults. The diagnosis may be challenging, requiring collaborative efforts between different specialists. According to the International Society for Hydrocephalus and Cerebrospinal Fluid Disorders, iNPH should be considered in the differential of any unexplained gait failure with insidious onset. Recognizing iNPH can be even more difficult in the presence of comorbid neurologic disorders. Among these, idiopathic Parkinson's disease (PD) is one of the major neurologic causes of gait dysfunction in the elderly. Both conditions have their peak prevalence between the 6th and the 7th decade. Importantly, postural instability and gait dysfunction are core clinical features in both iNPH and PD. Therefore, diagnosing iNPH where diagnostic criteria of PD have been met represents an additional clinical challenge. Here, we report a patient with parkinsonism initially consistent with PD who subsequently displayed rapidly progressive postural instability and gait dysfunction leading to the diagnosis of concomitant iNPH. In the following sections, we will review the clinical features of iNPH, as well as the overlapping and discriminating features when degenerative parkinsonism is in the differential diagnosis. Understanding and recognizing the potential for concomitant disease are critical when treating both conditions.

Shunting for normal pressure hydrocephalus (NPH).

PubMed

Esmonde, T; Cooke, S

2002-01-01

Since the condition was first described in 1965, the syndrome of normal pressure hydrocephalus (NPH) has conventionally been managed by placement of a cerebrospinal fluid (CSF) shunt. To determine the effectiveness of shunting procedures in promoting stability or improvement in the neurological symptoms and signs of NPH. The trials were identified from a search of the Specialized Register of the Cochrane Dementia and Cognitive Improvement Group on 26 June 2001 using the terms 'shunt*' and 'normal pressure hydrocephalus'. Studies included for analysis were those involving the placement of a CSF shunt for the treatment of NPH as part of a randomized controlled trial. No data matching the selection criteria were found. No randomized controlled trials of shunt placement versus no shunt were found. There is no evidence to indicate whether placement of a shunt is effective in the management of NPH.

["Normal pressure" hydrocephalus].

PubMed

Philippon, Jacques

2005-03-01

Normal pressure hydrocephalus (NPH) or, more precisely, chronic adult hydrocephalus, is a complex condition. Even if the basic mechanism is found in an impediment to CSF absorption, the underlying pathology is heterogeneous. In secondary NPH, the disruption of normal CSF pathways, following meningitis or sub-arachnoid haemorrhage, is responsible for ventricular dilatation. However, in about half of the cases, the etiology remains obscure. NPH is more frequently found in elderly people, probably in relation with the increased incidence of cerebrovascular disease. The diagnosis of NPH is based upon a triad of clinical symptoms. The main symptom is gait disturbances, followed by urinary incontinence and various degree of cognitive changes. The latter two symptoms are not prerequisites for the diagnosis. Radiological ventricular dilatation without cortical sulcal enlargement is a key factor, as well as substantial clinical improvement after CSF withdrawal (CSF tap test). Other CSF dynamic studies and various imaging investigations have been proposed to improve diagnostic accuracy, but no simple test can predict the results of CSF drainage. The current treatment is ventriculo-peritonial shunting, ideally using an adjustable valve. Results are directly dependent upon the accuracy of the preoperative diagnosis. Post-surgical complications may be observed in about 10% of cases.

[Lower urinary tract dysfunction in normal pressure hydrocephalus: Review of the literature].

PubMed

Bey, E; Nicot, B; Casez, O; Le Normand, L

2016-12-01

Lower urinary tract dysfunction in normal pressure hydrocephalus has received little attention from the scientific community. The aim of this review article was to discuss diagnostic and therapeutic options for these patients. A literature review of MedLine publications on urinary incontinence in normal pressure hydrocephalus was conducted. The following keywords were used: "hydrocephalus, normal pressure" and "bladder dysfunction" or "urinary incontinence" or "overactive bladder" or "urinary bladder, neurogenic". Prospective and retrospective studies as well as previous reviews were analyzed. Urinary symptoms in normal pressure hydrocephalus are mainly represented by overactive bladder, which is a significant burden for the concerned patients. Isolated overactive bladder is more frequent (64%) than urinary incontinence (57%). Detrusor overactivity is seen in 95.2% of the cases. Neuro-surgery is efficient on urinary symptoms for 61.5% of the patients. Bladder recovery after surgery relates with increased mid-cingulate perfusion, probably linked with a functional restoration of the mid-cingulate that normally inhibits the micturition reflex. Medical options, added or not to surgery, include anticholinergic drugs unable to pass through the blood-brain barrier, Transcutaneous Electrical Nerve Stimulation and sacral neuromodulation. There is actually an insufficient concern about urinary symptoms in normal pressure hydrocephalus. This article highlights the importance of a harmonization of neuro-urological practices in the pre-therapeutic evaluation of patients suffering from normal pressure hydrocephalus. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

Idiopathic normal-pressure hydrocephalus: temporal changes in ADC during cardiac cycle.

PubMed

Ohno, Naoki; Miyati, Tosiaki; Mase, Mitsuhito; Osawa, Tomoshi; Kan, Hirohito; Kasai, Harumasa; Hara, Masaki; Shibamoto, Yuta; Hayashi, Norio; Gabata, Toshifumi; Matsui, Osamu

2011-11-01

To determine whether temporal changes in apparent diffusion coefficient (ADC) over the cardiac cycle are different in patients with idiopathic normal-pressure hydrocephalus (INPH) as compared with patients with ex vacuo ventricular dilatation and healthy control subjects. This prospective study was approved by the institutional review board and was performed only after informed consent was obtained from each patient. At 1.5 T, electrocardiographically triggered single-shot diffusion echo-planar magnetic resonance imaging (b = 0 and 1000 sec/mm(2)) was performed with sensitivity encoding and half-scan techniques to minimize bulk motion. ΔADC was defined as the difference between maximum and minimum ADC on a pixel-by-pixel basis over 20 phases of the cardiac cycle. Mean ADC during the diastolic phase and ΔADC in the frontal white matter were determined in patients with INPH (n = 13), patients with ex vacuo ventricular dilatation (n = 8), and healthy volunteers (n = 10). Kruskal-Wallis tests were used to determine significance between groups. Mean ΔADC in the INPH group was significantly higher than that in the ex vacuo ventricular dilatation and control groups (P < .01 for both). There was no significant difference in ΔADC between the ex vacuo ventricular dilatation and control groups (P = .86). There was no significant difference in mean ADC during the diastolic phase among groups (P > .05 for all). There was no significant correlation between ΔADC and mean ADC during the diastolic phase in any group. Determination of fluctuation of ADC over the cardiac cycle may render it possible to noninvasively obtain new and more detailed information than that provided by standard ADC measurement in suspected INPH, potentially facilitating the diagnosis of this disease. RSNA, 2011

[Surgical treatment of normal pressure hydrocephalus].

PubMed

Svendsen, F; Hugdahl, K; Wester, K

2001-05-30

Normal pressure hydrocephalus (NPH) is an important diagnosis to keep in mind, i.e. the possibility that NPH and not a neurodegenerative disease causes the patient's symptoms with ataxia, urinary incontinence and dementia. Clinical improvement, sometimes a complete reversal of the symptoms, may be seen after a simple surgical procedure. This prospective study was performed by testing eight consecutively shunted patients with a walking test and a cognitive test battery pre- and postoperatively. Improvement 3-4 months after the shunting procedure suggests that NPH was present in six of eight patients. Walking ability was improved after surgery, also in patients with severe dementia. Severe dementia caused by NPH is hardly reversible, though cognitive tests may indicate some improvement. However, early surgical treatment of NPH in patients not suffering from a dementia according to the Mini Mental Status Test may bring improvement in some cognitive functions. Patients with both clinical and radiological signs of normal pressure hydrocephalus should be offered a shunting procedure.

Flow void of cerebrospinal fluid in idiopathic normal pressure hydrocephalus of the elderly: can it predict outcome after shunting?

PubMed

Krauss, J K; Regel, J P; Vach, W; Jüngling, F D; Droste, D W; Wakhloo, A K

1997-01-01

We investigate the predictive value of cerebrospinal fluid (CSF) flow void on outcome after shunting in a prospective series of patients with idiopathic normal pressure hydrocephalus (NPH). The degree and extension of CSF flow void were examined on T2-weighted magnetic resonance imaging scans of 37 elderly patients with idiopathic NPH who underwent subsequent shunting. The degree of flow void was assessed in comparison with the signal of large cerebral arteries. The extension was evaluated via the calculation of sum scores for the occurrence of flow void in different locations of the ventricular system. Those parameters were not considered in the decision to perform shunting. CSF flow void in the aqueduct and the adjacent third and fourth ventricles of the 37 patients with idiopathic NPH was compared with that of 37 age-matched control patients. CSF flow void scores in patients with idiopathic NPH were investigated for correlations between postoperative outcome scores and ventricular width indices. No difference was found between the occurrence of aqueductal CSF flow void in patients with idiopathic NPH and the control group. A significant difference, however, was noted for the extension of the CSF flow void, which was greater in the NPH group. Postoperative improvement was found in 33 of 37 patients with idiopathic NPH at a mean follow-up of 15.6 months. Only small, statistically not significant correlations were found between CSF flow void and postoperative outcome. Flow void sum scores, however, correlated significantly with ventricular width indices. The degree and extension of CSF flow void on T2-weighted magnetic resonance imaging scans have little predictive value for outcome after shunting in patients with idiopathic NPH. The greater extension of the CSF flow void in patients with NPH is most likely related to increased ventricular width. It is not useful to consider CSF flow void findings on conventional magnetic resonance imaging scans in making the

Improvement in social function and health-related quality of life after shunt surgery for idiopathic normal-pressure hydrocephalus.

PubMed

Petersen, Jakob; Hellström, Per; Wikkelsø, Carsten; Lundgren-Nilsson, Asa

2014-10-01

To investigate the impact of shunt surgery on the activity, participation, autonomy, and health-related quality of life (HRQOL) of patients with idiopathic normal-pressure hydrocephalus (iNPH) as well as the effect on caregiver burden. Thirty-seven patients (median age 70 years, range 50-89 years) with iNPH were evaluated before and 6 months after surgery. Symptoms and signs were assessed by the iNPH scale, activities of daily living (ADL) with the Functional Independence Measure (FIM) and Assessment of Motor and Process Skills (AMPS), autonomy and participation with Impact on Participation and Autonomy (IPA), and caregiver burden with the Caregiver Burden Scale (CBS). HRQOL was evaluated with the EQ-5D (EuroQol Group-5 Dimension health survey). Twenty-four patients (65%) improved clinically (iNPH scale score) and 31 (86%) improved their HRQOL after surgery, almost to the same level as found in the normal population. The patients became more independent in physical and cognitive activities, and participation and autonomy improved. The caregiver burden was decreased among caregivers to male patients but remained unchanged on the overall group level. After shunt surgery, patients with iNPH showed improvement in most aspects of social life, they became more independent, and their quality of life returned to nearly normal.

Does aqueductal stenosis influence the lumbar infusion test in normal-pressure hydrocephalus?

PubMed

González-Martínez, Emilio Luis; Santamarta, David

2016-12-01

Late-onset idiopathic aqueductal stenosis may present with clinical features indistinct from idiopathic normal-pressure hydrocephalus (NPH). Moreover, aqueductal stenosis (AS) is not always detected by conventional magnetic resonance imaging (MRI). The aim of this study was to compare the hydrodynamic pattern among NPH patients according to the patency of the aqueduct. Fifty-six consecutive lumbar infusion tests were performed in patients with NPH syndrome. Precipitating causes of hydrocephalus were excluded, and aqueductal patency was examined through high-resolution, T2-weighted 3D MRI. Patients were classified into two groups: non-patent aqueduct and patent aqueduct. Mean values of pressure and pulse amplitude were obtained from basal and plateau stages of infusion studies. Twelve of 56 patients with NPH-like symptoms presented with morphological AS (21.4 %). Patent aqueduct and non-patent aqueduct groups had similar values of mean opening lumbar pressure (8.2 vs. 8.1 mmHg), and mean opening pulse amplitude (3.1 vs. 2.9 mmHg). Mean pressure in the plateau stage (28.6 vs. 23.2 mmHg), and mean pulse amplitude in the plateau stage (12.5 vs. 10.6 mmHg) were higher in the patent aqueduct group. These differences were not statistically significant. Only Rout was significantly higher in the patent aqueduct group (13.6 vs. 10.1 mmHg/ml/min). One-third of NPH patients with AS presented Rout >12 mmHg/ml/min. No differences in mean pressure or pulse amplitude during basal and plateau epochs of the lumbar infusion test in NPH patients were detected, regardless of aqueductal patency. However, Rout was significantly higher in patients with patent aqueduct.

Practice guideline: Idiopathic normal pressure hydrocephalus: Response to shunting and predictors of response

PubMed Central

Halperin, John J.; Kurlan, Roger; Schwalb, Jason M.; Cusimano, Michael D.; Gronseth, Gary; Gloss, David

2015-01-01

Objective: We evaluated evidence for utility of shunting in idiopathic normal pressure hydrocephalus (iNPH) and for predictors of shunting effectiveness. Methods: We identified and classified relevant published studies according to 2004 and 2011 American Academy of Neurology methodology. Results: Of 21 articles, we identified 3 Class I articles. Conclusions: Shunting is possibly effective in iNPH (96% chance subjective improvement, 83% chance improvement on timed walk test at 6 months) (3 Class III). Serious adverse event risk was 11% (1 Class III). Predictors of success included elevated Ro (1 Class I, multiple Class II), impaired cerebral blood flow reactivity to acetazolamide (by SPECT) (1 Class I), and positive response to either external lumbar drainage (1 Class III) or repeated lumbar punctures. Age may not be a prognostic factor (1 Class II). Data are insufficient to judge efficacy of radionuclide cisternography or aqueductal flow measurement by MRI. Recommendations: Clinicians may choose to offer shunting for subjective iNPH symptoms and gait (Level C). Because of significant adverse event risk, risks and benefits should be carefully weighed (Level B). Clinicians should inform patients with iNPH with elevated Ro and their families that they have an increased chance of responding to shunting compared with those without such elevation (Level B). Clinicians may counsel patients with iNPH and their families that (1) positive response to external lumbar drainage or to repeated lumbar punctures increases the chance of response to shunting, and (2) increasing age does not decrease the chance of shunting being successful (both Level C). PMID:26644048

Normal-pressure hydrocephalus and the saga of the treatable dementias

DOE Office of Scientific and Technical Information (OSTI.GOV)

Friedland, R.P.

1989-11-10

A case study of a 74-year-old woman is presented which illustrates the difficulty of understanding dementing illnesses. A diagnosis of normal-pressure hydrocephalus (NPH) was made because of the development of abnormal gait, with urinary incontinence and severe, diffuse, white matter lesions on the MRI scan. Computed tomographic, MRI scans and positron emission tomographic images of glucose use are presented. The treatable dementias are a large, multifaceted group of illnesses, of which NPH is one. The author proposes a new term for this disorder commonly known as NPH because the problem with the term normal-pressure hydrocephalus is that the cerebrospinal fluidmore » pressure is not always normal in the disease.« less

Selection of patients with idiopathic normal-pressure hydrocephalus for shunt placement: a single-institution experience.

PubMed

Anile, Carmelo; De Bonis, Pasquale; Albanese, Alessio; Di Chirico, Alessandro; Mangiola, Annunziato; Petrella, Gianpaolo; Santini, Pietro

2010-07-01

The ability to predict outcome after shunt placement in patients with idiopathic normal-pressure hydrocephalus (NPH) represents a challenge. To date, no single diagnostic tool or combination of tools has proved capable of reliably predicting whether the condition of a patient with suspected NPH will improve after a shunting procedure. In this paper, the authors report their experience with 120 patients with the goal of identifying CSF hydrodynamics criteria capable of selecting patients with idiopathic NPH. Specifically, they focused on the comparison between CSF-outflow resistance (R-out) and intracranial elastance (IE). Between January 1977 and December 2005, 120 patients in whom idiopathic NPH had been diagnosed (on the basis of clinical findings and imaging) underwent CSF hydrodynamics evaluation based on an intraventricular infusion test. Ninety-six patients underwent CSF shunt placement: 32 between 1977 and 1989 (Group I) on the basis of purely clinical and radiological criteria; 44 between 1990 and 2002 (Group II) on the basis of the same criteria as Group I and because they had an IE slope > 0.25; and 20 between 2003 and 2005 (Group III) on the basis of the same criteria as Group II but with an IE slope > or = 0.30. Outcomes were evaluated by means of both Stein-Langfitt and Larsson scores. Patients' conditions were considered improved when there was a stable decrease (at 6- and 12-month follow-up) of at least 1 point in the Stein-Langfitt score and 2 points in the Larsson score. Group I: while no statistically significant difference in mean R-out value between improved and unimproved cases was observed, a clear-cut IE slope value of 0.25 differentiated very sharply between unimproved and improved cases. Group II: R-out values in the 2 unimproved cases were 20 and 47 mm Hg/ml/min, respectively. The mean IE slope in the improved cases was 0.56 (range 0.30-1.4), while the IE slopes in the 2 unimproved cases were 0.26 and 0.27. Group III: the mean IE slope was

Baseline neuropsychological profile and cognitive response to cerebrospinal fluid shunting for idiopathic normal pressure hydrocephalus.

PubMed

Thomas, George; McGirt, Matthew J; Woodworth, Graeme; Heidler, Jennifer; Rigamonti, Daniele; Hillis, Argye E; Williams, Michael A

2005-01-01

To evaluate neurocognitive changes and predict neurocognitive outcome after ventriculoperitoneal shunting for idiopathic normal pressure hydrocephalus (INPH). Reports of neurocognitive response to shunting have been variable and studies that predict cognitive outcomes after shunting are limited. We reviewed our experience with cognitive outcomes for INPH patients who were selected for shunting based on abnormal cerebrospinal fluid (CSF) pressure monitoring and positive response in any of the NPH symptoms following large volume CSF drainage. Forty-two INPH patients underwent neurocognitive testing and Folstein Mini-Mental State Examination (MMSE) prior to shunting. Neurocognitive testing or MMSEwere performed at least 3 months after shunt insertion. Significant improvement in a neurocognitive subtest was defined as improvement by one standard deviation (1 SD) for the patient's age, sex and education level. Significant improvement in overall neurocognitive outcome was defined as a 4-point improvement in MMSE or improvement by 1 SD in 50% of the administered neurocognitive subtests. Nonparametric tests were used to assess changes. Predictors of outcome were assessed via logistic regression analysis. Twenty-two patients (52.3%) showed overall neurocognitive improvement, and significant improvement was seen in tests of verbal memory and psychomotor speed. Predictive analysis showed that patients scoring more than 1 SD below mean at baseline on verbal memory immediate recall were fourfold less likely to show overall cognitive improvement, and sixfold less likely if also associated with visuoconstructional deficit or executive dysfunction. Verbal memory scores at baseline were higher in patients who showed overall cognitive improvement. Shunting INPH patients on the basis of CSF pressure monitoring and drainage response shows a significant rate of cognitive improvement, and baseline neurocognitive test scores may distinguish patients likely to respond to shunt surgery

Normal pressure hydrocephalus in patients with myelomeningocele.

PubMed

Hammock, M K; Milhorat, T H; Baron, I S

1976-01-01

Although the syndrome of normal pressure hydrocephalus (NPH) was described in the adult as early as 1964, it has only recently been recognized in the child. In this preliminary report, eight myelomeningocele patients with presumed NPH were evaluated before and after ventricular shunting procedures. Cranial computed tomography and serial psychological testing have proved to be particularly valuable both in the pre-operative and post-operative assessment of these patients and have the distinct advantage of being simple, non-invasive diagnostic measures. Continuous intra-ventricular pressure monitoring has shown what promises to be characteristic elevated pressure plateaux imposed on normal baseline cerebrospinal fluid (CSF) pressures in so-called NPH but is a more difficult clinical procedure, necessarily associated with potential complications. Although decreasing response to growth-stimulating hormone can be demonstrated in patients with long-standing hydrocephalus, this endocrine malfunction cannot be considered an early indicator of intracranial pathology. Single IQ scores are inadequate measures of intellectural function in children with NPH and serial examinations should be carried out. Detailed neuropsychological testing will document performance IQ scores well below verbal IQ scores and will generally show failure of psychomotor development to keep pace with chronological ageing. Initial studies indicate that improved performance scores can be expected within 1 1/2 to 3 months following successful ventricular shunting operations, and that any downward trend in pre-operative test scoring can at least be reversed. Statistically significant improvements in full-scale IQ scores have not been seen, however, before the end of the first post-operative year. Clinically, improved attentiveness and sociability, and decreased spasticity (if present prior to surgery) can be expected following shunting. Over-all, ventriculomegaly, normal CSF pressure, stable head size

The prognostic value of clinical characteristics and parameters of cerebrospinal fluid hydrodynamics in shunting for idiopathic normal pressure hydrocephalus.

PubMed

Delwel, E J; de Jong, D A; Avezaat, C J J

2005-10-01

It is difficult to predict which patients with symptoms and radiological signs of normal pressure hydrocephalus (NPH) will benefit from a shunting procedure and which patients will not. Risk of this procedure is also higher in patients with NPH than in the overall population of hydrocephalic patients. The aim of this study is to investigate which clinical characteristics, CT parameters and parameters of cerebrospinal fluid dynamics could predict improvement after shunting. Eighty-three consecutive patients with symptoms and radiological signs of NPH were included in a prospective study. Parameters of the cerebrospinal fluid dynamics were measured by calculation of computerised data obtained by a constant-flow lumbar infusion test. Sixty-six patients considered candidates for surgery were treated with a medium-pressure Spitz-Holter valve; in seventeen patients a shunting procedure was not considered indicated. Clinical and radiological follow-up was performed for at least one year postoperatively. The odds ratio, the sensitivity and specificity as well as the positive and negative predictive value of individual and combinations of measured parameters did not show a statistically significant relation to clinical improvement after shunting. We conclude that neither individual parameters nor combinations of measured parameters show any statistically significant relation to clinical improvement following shunting procedures in patients suspected of NPH. We suggest restricting the term normal pressure hydrocephalus to cases that improve after shunting and using the term normal pressure hydrocephalus syndrome for patients suspected of NPH and for patients not improving after implantation of a proven well-functioning shunt.

Impact of Cerebrospinal Fluid Shunting for Idiopathic Normal Pressure Hydrocephalus on the Amyloid Cascade

PubMed Central

Moriya, Masao; Miyajima, Masakazu; Nakajima, Madoka; Ogino, Ikuko; Arai, Hajime

2015-01-01

The aim of this study was to determine whether the improvement of cerebrospinal fluid (CSF) flow dynamics by CSF shunting, can suppress the oligomerization of amyloid β-peptide (Aβ), by measuring the levels of Alzheimer’s disease (AD)-related proteins in the CSF before and after lumboperitoneal shunting. Lumbar CSF from 32 patients with idiopathic normal pressure hydrocephalus (iNPH) (samples were obtained before and 1 year after shunting), 15 patients with AD, and 12 normal controls was analyzed for AD-related proteins and APLP1-derived Aβ-like peptides (APL1β) (a surrogate marker for Aβ). We found that before shunting, individuals with iNPH had significantly lower levels of soluble amyloid precursor proteins (sAPP) and Aβ38 compared to patients with AD and normal controls. We divided the patients with iNPH into patients with favorable (improvement ≥ 1 on the modified Rankin Scale) and unfavorable (no improvement on the modified Rankin Scale) outcomes. Compared to the unfavorable outcome group, the favorable outcome group showed significant increases in Aβ38, 40, 42, and phosphorylated-tau levels after shunting. In contrast, there were no significant changes in the levels of APL1β25, 27, and 28 after shunting. After shunting, we observed positive correlations between sAPPα and sAPPβ, Aβ38 and 42, and APL1β25 and 28, with shifts from sAPPβ to sAPPα, from APL1β28 to 25, and from Aβ42 to 38 in all patients with iNPH. Our results suggest that Aβ production remained unchanged by the shunt procedure because the levels of sAPP and APL1β were unchanged. Moreover, the shift of Aβ from oligomer to monomer due to the shift of Aβ42 (easy to aggregate) to Aβ38 (difficult to aggregate), and the improvement of interstitial-fluid flow, could lead to increased Aβ levels in the CSF. Our findings suggest that the shunting procedure can delay intracerebral deposition of Aβ in patients with iNPH. PMID:25821958

[Long-term prognosis of idiopathic chronic adult hydrocephalus: I. The University Hospital Marqués de Valdecilla diagnostic and therapeutic protocol].

PubMed

Martín-Láez, Rubén; Vázquez-Barquero, Alfonso

Despite the existence of published guidelines for more than a decade, there is still a substantial variation in the management of idiopathic normal pressure hydrocephalus due to its diagnostic and therapeutic complexity. The diagnostic and therapeutic protocol for the management of idiopathic normal pressure hydrocephalus in use at the Department of Neurosurgery of the University Hospital Marqués de Valdecilla is presented. The diagnostic process includes neuropsychological testing, phase contrast cine MRI, urodynamic evaluation, continuous intracranial pressure monitoring, cerebrospinal fluid hydrodynamics by means of lumbar infusion testing, and intra-abdominal pressure measurement. A patient is considered a surgical candidate if any of the following criteria is met: mean intracranial pressure >15mmHg, or B-waves present in >10% of overnight recording; pressure-volume index <15ml, or resistance to cerebrospinal fluid outflow (R OUT ) >4.5mmHg/ml/min in bolus infusion test; R OUT >12mmHg/ml/min, intracranial pressure >22mmHg, or high amplitude B-waves in the steady-state of the continuous rate infusion test; or a clinical response to high-volume cerebrospinal fluid withdrawal. The implementation of a diagnostic and therapeutic protocol for idiopathic normal pressure hydrocephalus management could improve various aspects of patient care. It could reduce variability in clinical practice, optimise the use of health resources, and help in identifying scientific uncertainty areas, in order to direct research efforts in a more appropriate way. Copyright © 2016 Sociedad Española de Neurocirugía. Publicado por Elsevier España, S.L.U. All rights reserved.

[The Current Status of Medical Care for Idiopathic Normal-pressure Hydrocephalus in Medical Centers for Dementia in Japan].

PubMed

Yoshiyama, Kenji; Kazui, Hiroaki; Takeda, Masatoshi

2015-09-01

We used a questionnaire to evaluate how patients with idiopathic normal-pressure hydrocephalus (iNPH) are examined in Medical Centers for Dementia (MCDs), which are qualified medical organizations for dementia patients in Japan. Ninety-eight of 205 MCDs responded to our questionnaire. Accordingly, more than 590 patients with iNPH underwent an MCD examination during the period of 1 year. In regards to the Guidelines for Management of iNPH, 40 of the 98 MCDs used neither the first nor second edition of the Guidelines, even though up-to-date information is available in the second edition of the Guidelines. At most MCDs, consultation with iNPH specialists was performed without a cerebrospinal fluid tap test. At more than half of the responding MCDs, doctors felt that the selection of appropriate candidates for shunt surgery differed from those selected by neurosurgeons. Moreover, 73 out of 87 MCDs argued that patients with iNPH should be examined by both dementia specialists and neurosurgeons after shunt surgery. These results suggest that doctors at MCDs should take advantage of up-to-date information and cooperate with iNPH specialists and neurosurgeons.

Characterization of lower urinary tract symptoms in patients with idiopathic normal pressure hydrocephalus.

PubMed

Krzastek, Sarah C; Bruch, William M; Robinson, Samuel P; Young, Harold F; Klausner, Adam P

2017-04-01

The purpose of this study was to evaluate lower urinary tract symptoms (LUTS) in idiopathic normal pressure hydrocephalus (iNPH). Patients with new-onset iNPH were prospectively evaluated for LUTS via detailed history and physical, and administration of questionnaires from the International Consultation on Incontinence to assess incontinence (ICIq-UI), overactive bladder (ICIq-OAB), and quality of life (ICIq-LUTqol), as well as the American Urological Association Symptom Score bother scale. All patients with moderate-to-severe LUTS were offered urodynamic testing. Sub-analysis was performed based on gender, medical comorbidities, and age. Fifty-five consecutive patients with iNPH completed the initial evaluation and surveys. Total urinary incontinence score was mild to moderate (8.71 ± 0.64: 0-21 scale) with 90.9% experiencing leakage and 74.5% reporting urge incontinence. The most common OAB symptom was nocturia (2.2 ± 0.14: 0-4 scale) with urge incontinence the most bothersome (3.71 ± 0.44: 0-10 scale). Quality-of-life impact was moderate (4.47 ± 0.4: 0-10 scale) and American Urological Association Symptom Score bother scale was 2.89 ± 0.22 (0-6 scale). Urodynamics testing revealed 100% detrusor overactivity and mean bladder capacity of 200 mL. Several differences were identified based on gender, medical comorbidities, and age. Patients with iNPH present with mild-moderate incontinence of which nocturia is the most common symptom, urge incontinence the most bothersome, with 100% of patients having detrusor overactivity. Younger patients experienced greater bother related to LUTS. To our knowledge, this is the only prospective evaluation of urinary symptoms in patients with new-onset iNPH. © 2016 Wiley Periodicals, Inc.

Relationship between intracranial pressure and phase contrast cine MRI derived measures of intracranial pulsations in idiopathic normal pressure hydrocephalus.

PubMed

Jaeger, Matthias; Khoo, Angela K; Conforti, David A; Cuganesan, Ramesh

2016-11-01

Phase contrast cine MRI with determination of pulsatile aqueductal cerebrospinal fluid (CSF) stroke volume and flow velocity has been suggested to assess intracranial pulsations in idiopathic normal pressure hydrocephalus (iNPH). We aimed to compare this non-invasive measure of pulsations to intracranial pressure (ICP) pulse wave amplitude from continuous ICP monitoring. We hypothesised that a significant correlation between these two markers of intracranial pulsations exists. Fifteen patients with suspected iNPH had continuous computerised ICP monitoring with calculation of mean ICP pulse wave amplitude (MWA) from time-domain analysis. MRI measured CSF aqueductal stroke volume and peak flow velocity. Mean MWA was 5.4mmHg (range 2.3-12.4mmHg). Mean CSF stroke volume and peak flow velocity were 65μl (range 3-195μl) and 9.31cm/s (range 1.68-15.0cm/s), respectively. No significant correlation between the invasive and non-invasive measures of pulsations existed (Spearman r=-0.30 and r=-0.27, respectively; p>0.05). We observed marked intra-individual fluctuation of MWA during continuous ICP monitoring of an average of 6.0mmHg (range 2.8-12.2mmHg). The results suggest a complex interplay between measures of pulsations derived from snapshot MRI measurements and continuous computerised ICP measurements, as no significant relationship existed in our data. Further study is needed to better understand the temporal profile of CSF MRI flow studies, as substantial variation in MWA over the course of several hours of ICP monitoring is common, suggesting that these physiologic fluctuations might obscure MRI snapshot measures of intracranial pulsations. Copyright © 2016 Elsevier Ltd. All rights reserved.

Delayed clearance of cerebrospinal fluid tracer from entorhinal cortex in idiopathic normal pressure hydrocephalus: A glymphatic magnetic resonance imaging study.

PubMed

Eide, Per K; Ringstad, Geir

2018-01-01

The glymphatic system plays a key role for clearance of waste solutes from the rodent brain. We recently found evidence of glymphatic circulation in the human brain when using magnetic resonance imaging (MRI) contrast agent as cerebrospinal fluid (CSF) tracer in conjunction with multiple MRI acquisitions (gMRI). The present study explored the hypothesis that reduced glymphatic clearance in entorhinal cortex (ERC) may be instrumental in idiopathic normal pressure hydrocephalus (iNPH) dementia. gMRI acquisitions were obtained over a 24-48 h time span in cognitively affected iNPH patients and non-cognitively affected patients with suspected CSF leaks. The CSF tracer enrichment was determined as changes in normalized MRI T1 signal units. The study included 30 patients with iNPH and 8 individuals with suspected CSF leaks (i.e. reference individuals). Compared to reference individuals, iNPH patients presented with higher medial temporal lobe atrophy score and Evan's index and inferior ERC thickness. We found delayed clearance of the intrathecal CSF tracer gadobutrol from CSF, the ERC and adjacent white matter, suggesting impaired glymphatic circulation. Reduced clearance and accumulation of toxic waste product such as amyloid-β may be a mechanism behind dementia in iNPH. Glymphatic MRI (gMRI) may become a tool for assessment of early dementia.

The reversibility of reduced cortical vein compliance in normal-pressure hydrocephalus following shunt insertion.

PubMed

Bateman, G A

2003-02-01

Superficial cortical venous compression secondary to alterations in craniospinal compliance is implicated in the pathogenesis of normal pressure hydrocephalus (NPH). A reduction in the pulsation in the outflow of the cortical veins would be expected to occur following compression of these veins and this has been shown in NPH. If cortical vein compression is a causative factor in NPH, it would be expected that cortical vein compliance as measured by pulsatility would be significantly altered by a curative procedure i.e. shunt tube insertion. My purpose is to compare the blood flow pulsatility characteristics in a group of patients with NPH before and after shunt tube insertion. I initially studied 18 subjects without pathology with MRI flow quantification studies of the cerebral arteries and veins to define the range of normality. The main study involved 18 patients with idiopathic dementia and mild leukoaraiosis who served as controls and seven patients with NPH studied before and after shunt insertion. Arterial, superior sagittal and straight sinus pulsatility was not significantly different between the patients with idiopathic dementia and those NPH patients before or after shunting. Cortical vein pulsatility before shunting in the patients with NPH was 43% lower than in those with idiopathic dementia ( P=0.006). Following shunting, cortical vein pulsatility increased by 186% ( P=0.007). There is thus reduced compliance in cortical veins in NPH which is significantly increased in patients who respond to insertion of a shunt tube. These findings suggest that reversible elevation in cortical vein pressure and reversal of the normal absorption pathway for cerebrospinal fluid may be behind the pathophysiology of NPH.

Alteration of cerebral perfusion in patients with idiopathic normal pressure hydrocephalus measured by 3D perfusion weighted magnetic resonance imaging.

PubMed

Walter, Christof; Hertel, F; Naumann, E; Mörsdorf, M

2005-12-01

It is controversial whether alteration of cerebral perfusion plays an important role in the pathophysiology of patients with idiopathic normal pressure hydrocephalus (NPH) and can help to predict the outcome after shunt surgery. 28 patients with suspected NPH were examined clinically (Homburg Hydrocephalus Scale, walking test, incontinence protocol) and by 3D dynamic susceptibility based perfusion weighted magnetic resonance imaging (PWI-MRI) before and after cerebrospinal fluid release (spinal tap test, STT). The perfusion parameters (negative integral (NI), time of arrival (T0), time to peak (TTP), mean transit time, and the difference TTP-T0 were analysed. Three different groups of patients were identified preoperatively: In group 1 seven patients showed an increase in the cerebral perfusion and a clinical improvement after STT. The second group (9 patients) also revealed an increase of the cerebral perfusion, but no significant alteration of the clinical assessment could be found. In the third group neither the cerebral perfusion nor the clinical assessment changed. 14 of the 16 patients (group 1 and 2) were examined three months after shunt placement. 11 patients showed a good or excellent result, 2 patients revealed a fair assessment, and only 1 patient had transiently improved. No patient was downgraded after shunting. In the patient group 1 and 2 the NI increased significantly (effect size: 34%), whereas in group 3 no significant alteration of NI was observed. PWI-MRI improves the prediction of outcome after shunt placement in patients with NPH and can offer new insights into the pathophysiology.

Cerebrospinal Fluid Biomarker and Brain Biopsy Findings in Idiopathic Normal Pressure Hydrocephalus

PubMed Central

Pyykkö, Okko T.; Lumela, Miikka; Rummukainen, Jaana; Nerg, Ossi; Seppälä, Toni T.; Herukka, Sanna-Kaisa; Koivisto, Anne M.; Alafuzoff, Irina; Puli, Lakshman; Savolainen, Sakari; Soininen, Hilkka; Jääskeläinen, Juha E.; Hiltunen, Mikko; Zetterberg, Henrik; Leinonen, Ville

2014-01-01

Background The significance of amyloid precursor protein (APP) and neuroinflammation in idiopathic normal pressure hydrocephalus (iNPH) and Alzheimer's disease (AD) is unknown. Objective To investigate the role of soluble APP (sAPP) and amyloid beta (Aβ) isoforms, proinflammatory cytokines, and biomarkers of neuronal damage in the cerebrospinal fluid (CSF) in relation to brain biopsy Aβ and hyperphosphorylated tau (HPτ) findings. Methods The study population comprised 102 patients with possible NPH with cortical brain biopsies, ventricular and lumbar CSF samples, and DNA available. The final clinical diagnoses were: 53 iNPH (91% shunt-responders), 26 AD (10 mixed iNPH+AD), and 23 others. Biopsy samples were immunostained against Aβ and HPτ. CSF levels of AD-related biomarkers (Aβ42, p-tau, total tau), non-AD-related Aβ isoforms (Aβ38, Aβ40), sAPP isoforms (sAPPα, sAPPβ), proinflammatory cytokines (several interleukins (IL), interferon-gamma, monocyte chemoattractant protein-1, tumor necrosis factor-alpha) and biomarkers of neuronal damage (neurofilament light and myelin basic protein) were measured. All patients were genotyped for APOE. Results Lumbar CSF levels of sAPPα were lower (p<0.05) in patients with shunt-responsive iNPH compared to non-iNPH patients. sAPPβ showed a similar trend (p = 0.06). CSF sAPP isoform levels showed no association to Aβ or HPτ in the brain biopsy. Quantified Aβ load in the brain biopsy showed a negative correlation with CSF levels of Aβ42 in ventricular (r = −0.295, p = 0.003) and lumbar (r = −0.356, p = 0.01) samples, while the levels of Aβ38 and Aβ40 showed no correlation. CSF levels of proinflammatory cytokines and biomarkers of neuronal damage did not associate to the brain biopsy findings, diagnosis, or shunt response. Higher lumbar/ventricular CSF IL-8 ratios (p<0.001) were seen in lumbar samples collected after ventriculostomy compared to the samples collected before the procedure

Post‐surgical changes in brain metabolism detected by magnetic resonance spectroscopy in normal pressure hydrocephalus: results of a pilot study

PubMed Central

del Mar Matarín, María; Pueyo, Roser; Poca, María Antonia; Falcón, Carles; Mataró, María; Bargalló, Núria; Sahuquillo, Juan; Junqué, Carme

2007-01-01

Background Adult normal pressure hydrocephalus (NPH) is one of the few potentially treatable causes of dementia. Some morphological and functional abnormalities attributed to hydrocephalus improve following treatment. Objectives We focused on analysis of changes in cerebral metabolites using proton magnetic resonance spectroscopy (1H‐MRS) after NPH treatment, and its clinical and cognitive correlation. Methods 1H‐MRS, neuropsychological and clinical status examinations were performed before and 6 months after shunting in 12 adults with idiopathic NPH. We obtained N‐acetyl‐aspartate (NAA), choline (Cho), myoinositol (MI) and creatine (Cr) values. Results After surgery, NAA/Cr was significantly increased. Moreover, NAA/Cr values were related to cognitive deterioration. Conclusion MRS could be a marker of neuronal dysfunction in NPH. PMID:17299021

Shunting normal-pressure hydrocephalus: do the benefits outweigh the risks? A multicenter study and literature review.

PubMed

Vanneste, J; Augustijn, P; Dirven, C; Tan, W F; Goedhart, Z D

1992-01-01

We performed a multicenter retrospective study in 166 consecutive patients shunted for presumed normal-pressure hydrocephalus (NPH) in the four neurosurgical departments of Amsterdam. Overall improvement occurred in 36%, substantial improvement in 21%. In the subgroup of idiopathic NPH (N = 127), marked improvement was only 15%. The incidence of shunt-responsive NPH in our area was 2.2/million/year. The rate of severe and moderate shunt-related complications was 28%, leading to death or severe residual morbidity in 7%. The substantial benefit/serious harm ration in the whole group was only three (21%/7%), decreasing to 1.7 in idiopathic NPH. By excluding patients at high surgical risk, this ratio might have risen to 10 in the whole group and to six in idiopathic NPH. Our experience is much less favorable than that encountered in the literature, reporting overall improvement in 74% and marked improvement in 55% of the shunted patients. We conclude that NPH is probably a very rare and still overdiagnosed syndrome and that the overall morbidity rate for each patient demonstrating meaningful improvement is high.

Cerebral hemodynamics before and after shunting in normal pressure hydrocephalus.

PubMed

Bakker, S L M; Boon, A J W; Wijnhoud, A D; Dippel, D W J; Delwel, E J; Koudstaal, P J

2002-09-01

To study the relationship between cerebral hemodynamics and clinical performance in normal pressure hydrocephalus (NPH), before and after surgery. Ten patients were studied prospectively before and 3 months after shunt surgery by means of transcranial Doppler (TCD). Clinical performance was scored by means of an NPH scale and the modified Rankin scale. Peak systolic and mean cerebral blood flow velocity (MCV) were lower and cerebrovascular CO2 reactivity was higher after shunt surgery. The three patients with clinical improvement had higher preoperative end diastolic cerebral blood flow velocity and MCV. All postoperative cerebral blood flow velocities were higher in patients with clinical improvement. Our data suggest that higher cerebral blood flow velocity before surgery in patients with NPH is related to clinical improvement after shunt surgery. Cerebral hemodynamic parameters may develop into predictors of successful shunt surgery in patients with normal pressure hydrocephalus.

Shunting effects in patients with idiopathic normal pressure hydrocephalus; correlation with cerebral and leptomeningeal biopsy findings.

PubMed

Bech, R A; Waldemar, G; Gjerris, F; Klinken, L; Juhler, M

1999-01-01

Normal Pressure Hydrocephalus (NPH) is a potentially treatable syndrome with abnormal cerebrospinal fluid dynamics. Meningeal fibrosis and/or obliteration of the subarachnoid space have been suggested as one of the patho-anatomical substrates. However, other types of adult onset dementia, predominantly Alzheimer's disease and Vascular Dementia, may mimic the clinical NPH characteristics. The purpose of the present study was to correlate cerebral parenchymal and leptomeningeal biopsy findings to the clinical outcome after CSF shunting in a prospective group of idiopathic NPH (INPH) patients. The study comprises 27 patients with INPH, diagnosed and shunted according to generally accepted clinical, imaging and hydrodynamic criteria. In all patients a frontal leptomeningeal and brain biopsy was obtained prior to the shunt insertion. Degenerative cerebral changes, most often Alzheimer (6 cases) or vascular changes (7 cases) were described in 14 out of 27 biopsies. Arachnoid fibrosis was found in 9 of the 18 biopsies containing arachnoid tissue. Overall, nine patients (33%) improved, of whom 6 presented Alzheimer or vascular changes in their biopsies. No correlation was found between clinical outcome and the presence or absence of degenerative cerebral changes and/or arachnoid fibrosis. However, a tendency towards higher improvement rates was noted in the subgroups presenting degenerative cerebral changes or arachnoid fibrosis. The results suggest that no constant morphological element exists in the syndrome of INPH. Various aetiologies may be involved in the pathogenesis and possibly in some cases co-existing: Patients may also improve by shunting despite the presence of degenerative cerebral parenchymal changes.

Improvement in lower urinary tract symptoms across multiple domains following ventriculoperitoneal shunting for idiopathic normal pressure hydrocephalus.

PubMed

Krzastek, Sarah C; Robinson, Samuel P; Young, Harold F; Klausner, Adam P

2017-11-01

The purpose of this study was to evaluate the change in lower urinary tract symptoms following ventriculoperitoneal shunting in patients with idiopathic normal pressure hydrocephalus (iNPH). Lower urinary tract symptoms in patients with new-onset iNPH were prospectively evaluated using validated questionnaires from the International Consultation on Incontinence to assess overactive bladder (ICIq-OAB), incontinence (ICIq-UI), and quality of life (ICIq-LUTqol), as well as the American Urological Association Symptom Score bother scale, prior to and following ventriculoperitoneal shunting for iNPH. Sub-analysis was performed based on gender, age, and medical comorbidities. Twenty-three consecutive patients with new-onset iNPH were evaluated prior to, and following, surgical intervention for iNPH via ventriculoperitoneal shunting. Shunting resulted in a significant improvement in urinary urgency, urge incontinence, ability to perform physical activities, and overall quality of life. Women had improvement across more domains than men following shunting, particularly in terms of urinary urgency and overall quality of life. Younger patients experienced significant improvement in scores following shunting as compared to older patients. Patients with two or more medical comorbidities, as well as those with fewer than two comorbidities, reported a significant improvement in overall quality of life. Surgical intervention for iNPH results in significant improvement in urinary symptoms, specifically in terms of urinary urgency and urge incontinence as well as overall quality of life, particularly in women and younger patients. © 2017 Wiley Periodicals, Inc.

Prospective estimation of mean axon diameter and extra-axonal space of the posterior limb of the internal capsule in patients with idiopathic normal pressure hydrocephalus before and after a lumboperitoneal shunt by using q-space diffusion MRI.

PubMed

Hori, Masaaki; Kamiya, Kouhei; Nakanishi, Atsushi; Fukunaga, Issei; Miyajima, Masakazu; Nakajima, Madoka; Suzuki, Michimasa; Suzuki, Yuriko; Irie, Ryusuke; Kamagata, Koji; Arai, Hajime; Aoki, Shigeki

2016-09-01

To prospectively estimate the mean axon diameter (MAD) and extracellular space of the posterior limb of the internal capsule (PLIC) in patients with idiopathic normal pressure hydrocephalus (iNPH) before and after a lumboperitoneal (LP) shunting operation using q-space diffusion MRI analysis. We studied 12 consecutive patients with iNPH and 12 controls at our institution. After conventional magnetic resonance imaging (MRI), q-space image (QSI) data were acquired with a 3-T MRI scanner. The MAD and extra-axonal space of the PLIC before and after LP shunting were calculated using two-component q-space imaging analyses; the before and after values were compared. After LP shunt surgery, the extracellular space of the PLIC was significantly higher than that of the same patients before the operation (one-way analysis of variance (ANOVA) with Scheffé's post-hoc test, P = 0.024). No significant differences were observed in the PLIC axon diameters among normal controls or in patients before and after surgery. Increases in the root mean square displacement in the extra-axonal space of the PLIC in patients with iNPH after an LP shunt procedure are associated with the microstructural changes of white matter and subsequent abatement of patient symptoms. • Q-space diffusion MRI provides information on microstructural changes in the corticospinal tract • Lumboperitoneal (LP) shunting operation is useful for idiopathic normal pressure hydrocephalus • Q-space measurement may be a biomarker for the effect of the LP shunt procedure.

Normal Pressure Hydrocephalus: Diagnosis and Treatment

PubMed Central

Shprecher, David; Schwalb, Jason; Kurlan, Roger

2009-01-01

Normal pressure hydrocephalus (NPH) is a syndrome of gait dysfunction and enlarged cerebral ventricles in the absence of another cause. It is frequently accompanied by frontal and subcortical cognitive deficits and bladder detrusor overactivity. NPH is rare relative to other potential causes of these symptoms in the elderly, but timely diagnosis can lead to reversal of symptoms through ventricular shunting. There are many tests used to predict possible response to surgery, such as MRI of the brain, formalized neuropsychological and gait testing, large-volume lumbar puncture, and prolonged lumbar drainage, but no one test has been validated to rule out potential response to surgery. PMID:18713572

Cerebrospinal fluid pulse pressure amplitude during lumbar infusion in idiopathic normal pressure hydrocephalus can predict response to shunting

PubMed Central

2010-01-01

Background We have previously seen that idiopathic normal pressure hydrocephalus (iNPH) patients having elevated intracranial pressure (ICP) pulse amplitude consistently respond to shunt surgery. In this study we explored how the cerebrospinal fluid pressure (CSFP) pulse amplitude determined during lumbar infusion testing, correlates with ICP pulse amplitude determined during over-night ICP monitoring and with response to shunt surgery. Our goal was to establish a more reliable screening procedure for selecting iNPH patients for shunt surgery using lumbar intrathecal infusion. Methods The study population consisted of all iNPH patients undergoing both diagnostic lumbar infusion testing and continuous over-night ICP monitoring during the period 2002-2007. The severity of iNPH was assessed using our NPH grading scale before surgery and 12 months after shunting. The CSFP pulse was characterized from the amplitude of single pressure waves. Results Totally 62 iNPH patients were included, 45 of them underwent shunt surgery, in whom 78% were shunt responders. Among the 45 shunted patients, resistance to CSF outflow (Rout) was elevated (≥ 12 mmHg/ml/min) in 44. The ICP pulse amplitude recorded over-night was elevated (i.e. mean ICP wave amplitude ≥ 4 mmHg) in 68% of patients; 92% of these were shunt responders. In those with elevated overnight ICP pulse amplitude, we found also elevated CSFP pulse amplitude recorded during lumbar infusion testing, both during the opening phase following lumbar puncture and during a standardized period of lumbar infusion (15 ml Ringer over 10 min). The clinical response to shunting after 1 year strongly associated with the over-night ICP pulse amplitude, and also with the pulsatile CSFP during the period of lumbar infusion. Elevated CSFP pulse amplitude during lumbar infusion thus predicted shunt response with sensitivity of 88 and specificity of 60 (positive and negative predictive values of 89 and 60, respectively). Conclusions In i

Dynamic state of water molecular displacement of the brain during the cardiac cycle in idiopathic normal pressure hydrocephalus.

PubMed

Kan, Hirohito; Miyati, Tosiaki; Mase, Mitsuhito; Osawa, Tomoshi; Ohno, Naoki; Kasai, Harumasa; Arai, Nobuyuki; Kawano, Makoto; Shibamoto, Yuta

2015-03-01

The predictive accuracy of iNPH diagnoses could be increased using a combination of supplemental tests for iNPH. To evaluate the dynamic state of water displacement during the cardiac cycle in idiopathic normal pressure hydrocephalus (iNPH), we determined the change in water displacement using q-space analysis of diffusion magnetic resonance image. ECG-triggered single-shot diffusion echo planar imaging was used. Water displacement was obtained from the displacement probability profile calculated by Fourier transform of the signal decay fitted as a function of the reciprocal spatial vector q. Then maximum minus minimum displacement (delta-displacement), of all cardiac phase images was calculated. We assessed the delta-displacement in white matter in patients with iNPH and atrophic ventricular dilation (atrophic VD), and in healthy volunteers (control group). Delta-displacement in iNPH was significantly higher than those in the atrophic VD and control. This shows that water molecules of the white matter in iNPH are easily fluctuated by volume loading of the cranium during the cardiac cycle, due to the decrease in intracranial compliance. There was no significant correlation between delta-displacement and displacement. The delta-displacement and the displacement do not necessarily yield the same kind of information. Delta-displacement demonstrated to obtain biophysical information about fluctuation. This analysis may be helpful in the understanding physiology and pathological condition in iNPH and the assisting in the diagnosis. Copyright © 2015 Elsevier Ltd. All rights reserved.

Influence of comorbidities in idiopathic normal pressure hydrocephalus — research and clinical care. A report of the ISHCSF task force on comorbidities in INPH

PubMed Central

2013-01-01

Idiopathic normal pressure hydrocephalus (INPH) is a syndrome of ventriculomegaly, gait impairment, cognitive decline and incontinence that occurs in an elderly population prone to many types of comorbidities. Identification of the comorbidities is thus an important part of the clinical management of INPH patients. In 2011, a task force was appointed by the International Society for Hydrocephalus and Cerebrospinal Fluid Disorders (ISHCSF) with the objective to compile an evidence-based expert analysis of what we know and what we need to know regarding comorbidities in INPH. This article is the final report of the task force. The expert panel conducted a comprehensive review of the literature. After weighing the evidence, the various proposals were discussed and the final document was approved by all the task force members and represents a consensus of expert opinions. Recommendations regarding the following topics are given: I. Musculoskeletal conditions; II. Urinary problems; III. Vascular disease including risk factors, Binswanger disease, and white matter hyperintensities; IV. Mild cognitive impairment and Alzheimer disease including biopsies; V. Other dementias (frontotemporal dementia, Lewy body, Parkinson); VI. Psychiatric and behavioral disorders; VII. Brain imaging; VIII. How to investigate and quantify. The task force concluded that comorbidity can be an important predictor of prognosis and post-operative outcome in INPH. Reported differences in outcomes among various INPH cohorts may be partly explained by variation in the rate and types of comorbidities at different hydrocephalus centers. Identification of comorbidities should thus be a central part of the clinical management of INPH where a detailed history, physical examination, and targeted investigations are the basis for diagnosis and grading. Future INPH research should focus on the contribution of comorbidity to overall morbidity, mortality and long-term outcomes. PMID:23758953

[Contribution of phase-contrast MRI to the management of patients with normal pressure hydrocephalus: Can it predict response to shunting?].

PubMed

Mouton Paradot, Gaëlle; Baledent, Olivier; Sallioux, Guillaume; Lehmann, Pierre; Gondry-Jouet, Catherine; Le Gars, Daniel

2010-02-01

The diagnosis and management of patients with idiopathic normal-pressure hydrocephalus (NPH) remain somewhat controversial and there is no clear guideline for assessing the post-shunt outcome. The objective of this study was to investigate whether cerebrospinal fluid (CSF) flow dynamics is linked to post-shunt improvement. Fourteen NPH patients (nine males and five females; mean age, 68 years) investigated by magnetic resonance imaging (MRI) before surgical diversion of CSF were retrospectively reviewed. Phase-contrast sequences were added to the morphological clinical protocol for quantification of CSF oscillations, which were recorded at the level of the cerebral aqueduct and the C2 and C3 subarachnoid spaces (SAS). The phase-contrast images were analysed with custom-designed dedicated flow segmentation software. The oscillations measured in this hydrocephalus population were compared to a previously studied healthy population. A difference of at least two standard deviations was used to define a hyperdynamic or hypodynamic state of CSF flow. The cervical CSF flow of the hydrocephalus patients was not significantly different from those of the volunteer population. Of the 14 hydrocephalus patients, 12 had a good response to the shunt. Of these, 10 presented an increased ventricular CSF flow, one a low ventricular CSF flow, and the last one had a normal ventricular CSF flow. Phase-contrast MRI can help develop guidelines for surgical management of NPH. The shunt responders appear to be the patients with hyperdynamic ventricular CSF flow and normal cervical CSF flow. Copyright 2009 Elsevier Masson SAS. All rights reserved.

Automated method to compute Evans index for diagnosis of idiopathic normal pressure hydrocephalus on brain CT images

NASA Astrophysics Data System (ADS)

Takahashi, Noriyuki; Kinoshita, Toshibumi; Ohmura, Tomomi; Matsuyama, Eri; Toyoshima, Hideto

2017-03-01

The early diagnosis of idiopathic normal pressure hydrocephalus (iNPH) considered as a treatable dementia is important. The iNPH causes enlargement of lateral ventricles (LVs). The degree of the enlargement of the LVs on CT or MR images is evaluated by using a diagnostic imaging criterion, Evans index. Evans index is defined as the ratio of the maximal width of frontal horns (FH) of the LVs to the maximal width of the inner skull (IS). Evans index is the most commonly used parameter for the evaluation of ventricular enlargement. However, manual measurement of Evans index is a time-consuming process. In this study, we present an automated method to compute Evans index on brain CT images. The algorithm of the method consisted of five major steps: standardization of CT data to an atlas, extraction of FH and IS regions, the search for the outmost points of bilateral FH regions, determination of the maximal widths of both the FH and the IS, and calculation of Evans index. The standardization to the atlas was performed by using linear affine transformation and non-linear wrapping techniques. The FH regions were segmented by using a three dimensional region growing technique. This scheme was applied to CT scans from 44 subjects, including 13 iNPH patients. The average difference in Evans index between the proposed method and manual measurement was 0.01 (1.6%), and the correlation coefficient of these data for the Evans index was 0.98. Therefore, this computerized method may have the potential to accurately compute Evans index for the diagnosis of iNPH on CT images.

The role of diffusion tensor imaging and fractional anisotropy in the evaluation of patients with idiopathic normal pressure hydrocephalus: a literature review.

PubMed

Siasios, Ioannis; Kapsalaki, Eftychia Z; Fountas, Kostas N; Fotiadou, Aggeliki; Dorsch, Alexander; Vakharia, Kunal; Pollina, John; Dimopoulos, Vassilios

2016-09-01

OBJECTIVE Diffusion tensor imaging (DTI) for the assessment of fractional anisotropy (FA) and involving measurements of mean diffusivity (MD) and apparent diffusion coefficient (ADC) represents a novel, MRI-based, noninvasive technique that may delineate microstructural changes in cerebral white matter (WM). For example, DTI may be used for the diagnosis and differentiation of idiopathic normal pressure hydrocephalus (iNPH) from other neurodegenerative diseases with similar imaging findings and clinical symptoms and signs. The goal of the current study was to identify and analyze recently published series on the use of DTI as a diagnostic tool. Moreover, the authors also explored the utility of DTI in identifying patients with iNPH who could be managed by surgical intervention. METHODS The authors performed a literature search of the PubMed database by using any possible combinations of the following terms: "Alzheimer's disease," "brain," "cerebrospinal fluid," "CSF," "diffusion tensor imaging," "DTI," "hydrocephalus," "idiopathic," "magnetic resonance imaging," "normal pressure," "Parkinson's disease," and "shunting." Moreover, all reference lists from the retrieved articles were reviewed to identify any additional pertinent articles. RESULTS The literature search retrieved 19 studies in which DTI was used for the identification and differentiation of iNPH from other neurodegenerative diseases. The DTI protocols involved different approaches, such as region of interest (ROI) methods, tract-based spatial statistics, voxel-based analysis, and delta-ADC analysis. The most studied anatomical regions were the periventricular WM areas, such as the internal capsule (IC), the corticospinal tract (CST), and the corpus callosum (CC). Patients with iNPH had significantly higher MD in the periventricular WM areas of the CST and the CC than had healthy controls. In addition, FA and ADCs were significantly higher in the CST of iNPH patients than in any other patients with other

Neuroimaging in normal pressure hydrocephalus

PubMed Central

Damasceno, Benito Pereira

2015-01-01

Normal pressure hydrocephalus (NPH) is a syndrome characterized by the triad of gait disturbance, mental deterioration and urinary incontinence, associated with ventriculomegaly and normal cerebrospinal fluid (CSF) pressure. The clinical presentation (triad) may be atypical or incomplete, or mimicked by other diseases, hence the need for supplementary tests, particularly to predict postsurgical outcome, such as CSF tap-tests and computed tomography (CT) or magnetic resonance imaging (MRI). The CSF tap-test, especially the 3 to 5 days continuous external lumbar drainage of at least 150 ml/day, is the only procedure that simulates the effect of definitive shunt surgery, with high sensitivity (50-100%) and high positive predictive value (80-100%). According to international guidelines, the following are CT or MRI signs decisive for NPH diagnosis and selection of shunt-responsive patients: ventricular enlargement disproportionate to cerebral atrophy (Evans index >0.3), and associated ballooning of frontal horns; periventricular hyperintensities; corpus callosum thinning and elevation, with callosal angle between 40º and 90º; widening of temporal horns not fully explained by hippocampal atrophy; and aqueductal or fourth ventricular flow void; enlarged Sylvian fissures and basal cistern, and narrowing of sulci and subarachnoid spaces over the high convexity and midline surface of the brain. On the other hand, other imaging methods such as radionuclide cisternography, SPECT, PET, and also DTI or resting-state functional MRI, although suitable for NPH diagnosis, do not yet provide improved accuracy for identifying shunt-responsive cases. PMID:29213984

Relationship of new-onset systemic hypertension and normal pressure hydrocephalus.

PubMed

Mysiw, W J; Jackson, R D

1990-01-01

Communicating normal pressure hydrocephalus (NPH) is an important remote complication of traumatic brain injury (TBI). The diagnosis of this hydrocephalus depends largely on clinical signs and symptoms, including cognitive deterioration, gait changes and incontinence. However, many of these signs are also seen during post-traumatic amnesia, making early recognition of this syndrome difficult. A case study of one man post-TBI, who presented with new-onset hypertension as a sign of NPH, prompted a retrospective chart review of all patients admitted over a 2-year period with a diagnosis of NPH. Ninety per cent of patients had one or more of the classic triad of NPH and 25% of patients had symptoms suggestive of raised intracranial pressure (unexplained nausea, headache and visual disturbance). Mean systolic and diastolic blood pressures among the 20 subjects for six consecutive days pre-operatively compared with those for days 8-14 and 15-21 post-operatively showed no significant differences; a subgroup of five patients (25%), however, demonstrated a significant change in blood pressure temporally related to shunting. We suggest that demonstration of new-onset systemic hypertension may also be a clinical sign suggestive of NPH useful in the evaluation of the TBI patient.

Use of cerebrospinal fluid flow rates measured by phase-contrast MR to predict outcome of ventriculoperitoneal shunting for idiopathic normal-pressure hydrocephalus.

PubMed

Dixon, Geoffrey R; Friedman, Jonathan A; Luetmer, Patrick H; Quast, Lynn M; McClelland, Robyn L; Petersen, Ronald C; Maher, Cormac O; Ebersold, Michael J

2002-06-01

To determine whether favorable clinical response and magnitude of improvement are associated with increased aqueductal cerebrospinal fluid (CSF) flow rates in patients who undergo ventriculoperitoneal shunting (VPS) for idiopathic normal-pressure hydrocephalus (NPH). Between January 1995 and June 2000, 49 patients (14 men and 35 women; mean age, 72.9 years; range, 54-88 years) underwent magnetic resonance quantification of aqueductal CSF flow followed by VPS for presumed idiopathic NPH at the Mayo Clinic, Rochester, Minn. Logistic regression models for the odds of any improvement in score as a function of aqueductal CSF flow and separate models for any improvement in gait, incontinence, cognition, and total score were constructed. Forty-two patients (86%) had improvement in gait at postoperative follow-up (mean, 10 months). Of the 32 patients with incontinence, 27 (69%) improved. Of the 36 patients with cognitive impairment, 16 (44%) improved. In univariate and fully adjusted models, increased CSF flow through the aqueduct was not significantly associated with improvement or the magnitude of improvement in gait, cognition, or incontinence. Thirty-six patients underwent high-volume lumbar puncture preoperatively, of whom 5 (14%) had no response. The aqueductal CSF flow rates of these 5 patients were significantly higher than those of the patients who improved after lumbar puncture. Postoperative complications occurred in 15 patients. The aqueductal CSF flow rates in these 15 patients were not significantly different from those of patients who experienced no complications. Among patients who underwent VPS for the treatment of NPH, measurement of CSF flow through the cerebral aqueduct did not reliably predict which patients would improve after shunting or the magnitude of improvement.

A randomised trial of high and low pressure level settings on an adjustable ventriculoperitoneal shunt valve for idiopathic normal pressure hydrocephalus: results of the Dutch evaluation programme Strata shunt (DEPSS) trial.

PubMed

Delwel, Ernst J; de Jong, Dirk A; Dammers, Ruben; Kurt, Erkan; van den Brink, Wimar; Dirven, Clemens M F

2013-07-01

In treating idiopathic normal pressure hydrocephalus (INPH) with a shunt there is always a risk of underdrainage or overdrainage. The hypothesis is tested whether patients treated using an adjustable valve preset at the highest opening pressure leads to comparable good clinical results with less subdural effusions than in a control group with an opening pressure preset at a low pressure level. A multicentre prospective randomised trial was performed on a total of 58 patients suspected of INPH. Thirty patients were assigned to (control) group 1 and received a Strata shunt (Medtronic, Goleta, USA) with the valve preset at a performance level (PL) of 1.0, while 28 patients were assigned to group 2 and received a Strata shunt with the valve preset at PL 2.5. In this group the PL was allowed to be lowered until improvement or radiological signs of overdrainage were met. Significantly more subdural effusions were observed in the improved patients of group 1. There was no statistically significant difference in improvement between both groups overall. On the basis of this multicentre prospective randomised trial it is to be recommended to treat patients with INPH with a shunt with an adjustable valve, preset at the highest opening pressure and lowered until clinical improvement or radiological signs of overdrainage occur although slower improvement and more shunt adjustments might be the consequence.

Axon diameter and intra-axonal volume fraction of the corticospinal tract in idiopathic normal pressure hydrocephalus measured by q-space imaging.

PubMed

Kamiya, Kouhei; Hori, Masaaki; Miyajima, Masakazu; Nakajima, Madoka; Suzuki, Yuriko; Kamagata, Koji; Suzuki, Michimasa; Arai, Hajime; Ohtomo, Kuni; Aoki, Shigeki

2014-01-01

Previous studies suggest that compression and stretching of the corticospinal tract (CST) potentially cause treatable gait disturbance in patients with idiopathic normal pressure hydrocephalus (iNPH). Measurement of axon diameter with diffusion MRI has recently been used to investigate microstructural alterations in neurological diseases. In this study, we investigated alterations in the axon diameter and intra-axonal fraction of the CST in iNPH by q-space imaging (QSI) analysis. Nineteen patients with iNPH and 10 age-matched controls were recruited. QSI data were obtained with a 3-T system by using a single-shot echo planar imaging sequence with the diffusion gradient applied parallel to the antero-posterior axis. By using a two-component low-q fit model, the root mean square displacements of intra-axonal space ( =  axon diameter) and intra-axonal volume fraction of the CST were calculated at the levels of the internal capsule and body of the lateral ventricle, respectively. Wilcoxon's rank-sum test revealed a significant increase in CST intra-axonal volume fraction at the paraventricular level in patients (p<0.001), whereas no significant difference was observed in the axon diameter. At the level of the internal capsule, neither axon diameter nor intra-axonal volume fraction differed significantly between the two groups. Our results suggest that in patients with iNPH, the CST does not undergo irreversible axonal damage but is rather compressed and/or stretched owing to pressure from the enlarged ventricle. These analyses of axon diameter and intra-axonal fraction yield insights into microstructural alterations of the CST in iNPH.

Vascular factors in suspected normal pressure hydrocephalus

PubMed Central

Agerskov, Simon; Rabiei, Katrin; Marlow, Thomas; Jensen, Christer; Guo, Xinxin; Kern, Silke; Wikkelsø, Carsten; Skoog, Ingmar

2016-01-01

Objective: We examined clinical and imaging findings of suspected idiopathic normal pressure hydrocephalus (iNPH) in relation to vascular risk factors and white matter lesions (WMLs), using a nested case-control design in a representative, population-based sample. Methods: From a population-based sample, 1,235 persons aged 70 years or older were examined with CT of the brain between 1986 and 2000. We identified 55 persons with hydrocephalic ventricular enlargement, i.e., radiologic findings consistent with iNPH. Among these, 26 had clinical signs that fulfilled international guideline criteria for probable iNPH. These cases were labeled suspected iNPH. Each case was matched to 5 controls from the same sample, based on age, sex, and study cohort. Data on risk factors were obtained from clinical examinations and the Swedish Hospital Discharge Register. History of hypertension, diabetes mellitus (DM), smoking, overweight, history of coronary artery disease, stroke/TIA, and WMLs on CT were examined. Risk factors associated with iNPH with a p value <0.1 in χ2 tests were included in conditional logistic regression models. Results: In the regression analyses, suspected iNPH was related to moderate to severe WMLs (odds ratio [OR] 5.2; 95% confidence interval [CI]: 1.5–17.6), while hydrocephalic ventricular enlargement was related to hypertension (OR 2.7; 95% CI: 1.1–6.8), moderate to severe WMLs (OR 6.5; 95% CI: 2.1–20.3), and DM (OR 4.3; 95% CI: 1.1–16.3). Conclusions: Hypertension, WMLs, and DM were related to clinical and imaging features of iNPH, suggesting that vascular mechanisms are involved in the pathophysiology. These findings might have implications for understanding disease mechanisms in iNPH and possibly prevention. PMID:26773072

Comparison between the lumbar infusion and CSF tap tests to predict outcome after shunt surgery in suspected normal pressure hydrocephalus.

PubMed

Kahlon, B; Sundbärg, G; Rehncrona, S

2002-12-01

To compare the lumbar infusion test and the cerebrospinal fluid (CSF) tap test for predicting the outcome of shunt surgery in patients with suspected normal pressure hydrocephalus. 68 patients with suspected normal pressure hydrocephalus were studied. The absence of preceding history indicated idiopathic disease in 75% of these. All patients were assessed twice with walking and psychometric tests before lumbar infusion test and tap test assessments. The lumbar infusion test was done using a constant infusion rate (0.80 ml/min) and regarded as positive if the steady state CSF plateau pressure reached levels of > 22 mm Hg (resistance to outflow > 14 mm Hg/ml/min). The tap test was regarded as positive if two or more of four different test items improved after CSF removal. As the variability in baseline test results was large, the better of two evaluations was used in comparisons with the results after CSF removal, as well as to evaluate the outcome after shunt surgery. Only patients with a positive lumbar infusion test or a positive tap test had surgery. The results of the CSF tap test and the lumbar infusion test agreed in only 45% of the patients. Of the total cohort, 47 (69%) had positive test results and were operated on; 45 (96%) of these reported subjective improvement, and postoperative assessments verified the improvements in 38 (81%). Improvements were highly significant in walking, memory, and reaction time tests (p < 0.001). Most of the patients improved by surgery (84%) were selected by a positive lumbar infusion test, and only 42% by a positive tap test. Positive predictive values were 80% for lumbar infusion test and 94% for tap test. The false negative predictions in the operated group were much higher (58%) with the tap test than with the lumbar infusion test (16%). Both the lumbar infusion test and the tap test can predict a positive outcome of shunt operations in unselected patients with suspected normal pressure hydrocephalus. The two tests are

Cardiac output in idiopathic normal pressure hydrocephalus: association with arterial blood pressure and intracranial pressure wave amplitudes and outcome of shunt surgery

PubMed Central

2011-01-01

Background In patients with idiopathic normal pressure hydrocephalus (iNPH) responding to shunt surgery, we have consistently found elevated intracranial pressure (ICP) wave amplitudes during diagnostic ICP monitoring prior to surgery. It remains unknown why ICP wave amplitudes are increased in these patients. Since iNPH is accompanied by a high incidence of vascular co-morbidity, a possible explanation is that there is reduced vascular compliance accompanied by elevated arterial blood pressure (ABP) wave amplitudes and even altered cardiac output (CO). To investigate this possibility, the present study was undertaken to continuously monitor CO to determine if it is correlated to ABP and ICP wave amplitudes and the outcome of shunting in iNPH patients. It was specifically addressed whether the increased ICP wave amplitudes seen in iNPH shunt responders were accompanied by elevated CO and/or ABP wave amplitude levels. Methods Prospective iNPH patients (29) were clinically graded using an NPH grading scale. Continuous overnight minimally-invasive monitoring of CO and ABP was done simultaneously with ICP monitoring; the CO, ABP, and ICP parameters were parsed into 6-second time windows. Patients were assessed for shunt surgery on clinical grade, Evan's index, and ICP wave amplitude. Follow-up clinical grading was performed 12 months after surgery. Results ICP wave amplitudes but not CO or ABP wave amplitude, showed good correlation with the response to shunt treatment. The patients with high ICP wave amplitude did not have accompanying high levels of CO or ABP wave amplitude. Correlation analysis between CO and ICP wave amplitudes in individual patients showed different profiles [significantly positive in 10 (35%) and significantly negative in 16 (55%) of 29 recordings]. This depended on whether there was also a correlation between ABP and ICP wave amplitudes and on the average level of ICP wave amplitude. Conclusions These results gave no evidence that the increased

Rectification of pulsatile stress on soft tissues: a mechanism for normal-pressure hydrocephalus

NASA Astrophysics Data System (ADS)

Jalikop, Shreyas; Hilgenfeldt, Sascha

2011-11-01

Hydrocephalus is a pathological condition of the brain that occurs when cerebrospinal fluid (CSF) accumulates excessively in the brain cavities, resulting in compression of the brain parenchyma. Counter-intuitively, normal-pressure hydrocephalus (NPH) does not show elevated pressure differences across the compressed parenchyma. We investigate the effects of nonlinear tissue mechanics and periodic driving in this system. The latter is due to the cardiac cycle, which provides significant intracranial pressure and volume flow rate fluctuations. Nonlinear rectification of the periodic driving within a model of fluid flow in poroelastic material can lead to compression or expansion of the parenchyma, and this effect does not rely on changes in the mean intracranial pressure. The rectification effects can occur gradually over several days, in agreement with clinical studies of NPH.

Cervical spine disease may result in a negative lumbar spinal drainage trial in normal pressure hydrocephalus: case report.

PubMed

Komotar, Ricardo J; Zacharia, Brad E; Mocco, J; Kaiser, Michael G; Frucht, Stephen J; McKhann, Guy M

2008-10-01

In this case report, we present a patient with normal pressure hydrocephalus in whom a lumbar drainage trial yielded a false-negative result secondary to cervical spondylosis. An 80-year-old woman presented with classic symptoms of normal pressure hydrocephalus as well as evidence of cervical myelopathy. Magnetic resonance imaging of the brain and spine showed enlarged ventricles and single-level cervical canal narrowing. An initial lumbar drainage trial was performed, which revealed negative results. The patient then underwent cervical decompression and fusion. Despite this procedure, the patient's symptoms continued to worsen. A repeat lumbar drainage trial was performed with positive results. Subsequently, a ventriculoperitoneal shunt was placed, resulting in significant improvement of her symptoms. This case report illustrates how altered cerebrospinal fluid flow dynamics may impact the accuracy of the lumbar spinal drainage trial in patients with normal pressure hydrocephalus.

Gait and Balance Measures Can Identify Change From a Cerebrospinal Fluid Tap Test in Idiopathic Normal Pressure Hydrocephalus.

PubMed

Gallagher, Ryan; Marquez, Jodie; Osmotherly, Peter

2018-04-25

To identify in patients with idiopathic normal pressure hydrocephalus (iNPH) undergoing a cerebrospinal fluid (CSF) tap test (TT) for consideration of a ventricular peritoneal (VP) shunt: (1) gait and balance measures, which identify symptom change; (2) differences present between pre- and post-CSF TT scores between patients classified as responders and nonresponder; (3) ability of patients with iNPH to accurately quantify change in their gait and balance symptoms from a CSF TT. Prospective observational study. Post-CSF TT assessment was completed 2-4 hours post. Tertiary referral neurological and neurosurgical hospital. Patients (N=74) with iNPH receiving a 30 mL CSF TT for consideration of a VP shunt. Patients underwent a battery of gait and balance measures pre- and post-CSF TT and indicated their perceived change on a global rating of change (GRC). Patients deemed to improve and offered VP shunt insertion by a neurologist or neurosurgeon were labeled responders. Performance oriented mobility assessment (Tinetti), Berg Balance Scale (BBS), timed Up and Go (TUG), 10-meter walk test (10MWT), GRC. Forty patients were classified responders, 34 nonresponders. Significant differences were identified for responders: Tinetti (3.88 points), TUG (3.98 seconds), 10MWT (0.08 m/sec), and BBS (5.29 points). Significant differences were found for nonresponders for the Tinetti (0.91 points) and BBS (2.06 points). Change scores for responders and nonresponders were significantly different for all tests between responders and nonresponders. GRC scores for gait (+2 for responders, 0 for nonresponders) and balance (+2.5 for responders, 0 for nonresponders) were both significantly different. The Tinetti, BBS, and TUG can identify change in patients undergoing a CSF TT for iNPH. Patients appear to be able to accurately identify if change has occurred. Copyright © 2018 American Congress of Rehabilitation Medicine. Published by Elsevier Inc. All rights reserved.

Shunt Intervention for Possible Idiopathic Normal Pressure Hydrocephalus Improves Patient Outcomes: A Nationwide Hospital-Based Survey in Japan.

PubMed

Nakajima, Madoka; Miyajima, Masakazu; Ogino, Ikuko; Akiba, Chihiro; Kawamura, Kaito; Kurosawa, Michiko; Kuriyama, Nagato; Watanabe, Yoshiyuki; Fukushima, Wakaba; Mori, Etsuro; Kato, Takeo; Sugano, Hidenori; Karagiozov, Kostadin; Arai, Hajime

2018-01-01

Background and Purpose: This study aimed to investigate the efficacy of cerebrospinal fluid shunt intervention for idiopathic normal pressure hydrocephalus (iNPH) using data from a nationwide epidemiological survey in Japan. Methods: We conducted a cross-sectional study using data from a nationwide epidemiological survey performed in Japan. Propensity score matching was used to select 874 patients from 1,423 patients aged ≥60 years, who were diagnosed with iNPH based on clinical guidelines following a hospital visit in 2012. Patients who experienced an improvement of at least 1 modified Rankin Scale (mRS) grade after the intervention were classified as "improved," while the remaining patients were classified as "non-improved." In the shunt intervention ( n = 437) and non-shunt intervention ( n = 437) groups, the differences in mRS grade improvement were analyzed using the Mann-Whitney U -test. Finally, we examined subjects in the shunt intervention group ( n = 974) to compare the outcomes and complications of ventriculoperitoneal (VP) shunt (n = 417) with lumboperitoneal (LP) shunt ( n = 540). Results: We examined subjects with iNPH to compare the non-shunt intervention group to the shunt intervention group following adjustment for age and mRS grade at baseline by propensity score matching (0.31-0.901). The mRS grade (mean [SD]) was found to improve with non-shunt intervention (2.46 [0.88]) and shunt intervention (1.93 [0.93]) ( p < 0.001) in iNPH patients. The mRS outcome score and complications comparison between the VP and LP shunt groups did not show significant difference. Conclusions: In this study, analysis of the efficacy of shunts for possible iNPH conducted in Japan indicated a significant improvement in the mRS grade between baseline and outcome within 1 year, regardless of the surgical technique, and shunt intervention was found to be effective.

Psychotic symptoms in normal-pressure hydrocephalus.

PubMed

Lying-Tunell, U

1979-04-01

Two patients with a psychiatric history of about 20 years, and clinical and neuroradiological signs of normal-pressure hydrocephalus (NPH) are reported. One had a periodic psychosis subsequent to a tuberculous meningitis, and this overshadowed the slight classical symptoms of NPH. She had received at least 120 treatments with electro-convulsive therapy. The second patient suffered from a paranoid psychosis; other signs of NPH were moderate though progressive. Both patients showed definite improvement of their NPH symptoms after ventriculo-atrial shunting, and psychotic symptoms ceased totally. Follow-up was 5 years for the patient with periodic psychosis. The other patient died from septicaemia 2.5 years after shunting. A large-scale screening of patients with psychiatric symptoms or dementia, particularly when combined with gait disturbance, should be done by using computerized tomography. Patients suspected of having NPH should then be referred for further examination with the aim of selecting patients suitable for shunting. These measures seem well motivated from humanitarian as well as economic points of view.

Normal pressure hydrocephalus: long-term outcome after shunt surgery.

PubMed

Pujari, S; Kharkar, S; Metellus, P; Shuck, J; Williams, M A; Rigamonti, D

2008-11-01

Little is known about the long-term clinical course and management of patients with normal pressure hydrocephalus (NPH) treated by cerebrospinal fluid (CSF) shunting. We retrospectively reviewed records of 55 patients diagnosed with idiopathic NPH (INPH) and treated with CSF shunts, all of whom were followed for more than 3 years after the original shunt surgery. At each annual follow-up visit, the patient was assessed by Folstein Mini Mental State Examination, detailed clinical evaluation of gait and assessment of headache, cognition, gait or urination, as assessed by the patient and relatives. The mean duration of follow-up was 5.9+/-2.5 years. There was an overall sustained improvement among all symptoms. Gait showed the highest maintenance of improvement over baseline (83% at 3 years and 87% at the last analysed follow-up of 7 years), cognition showed intermediary improvement (84% and 86%, respectively), and urinary incontinence showed the least improvement (84% and 80%, respectively). Fifty-three percent of patients required shunt revisions. Indications for revision included shunt malfunction (87%), infection (10%) and change of shunt configuration (3%). Overall, 74% revisions resulted in clinical improvement. Clinical improvement of patients with NPH can be sustained for 5-7 years in some patients with NPH, even if shunt revision surgery is needed multiple times. With earlier diagnosis and treatment of NPH and the increasing lifespan of the ageing population, the need for long-term follow-up after shunt surgery for NPH may be greater than it was in the past. Monitoring, identification and treatment of shunt obstruction is a key management principle.

Optimized surgical treatment for normal pressure hydrocephalus: comparison between gravitational and differential pressure valves.

PubMed

Suchorska, B; Kunz, M; Schniepp, R; Jahn, K; Goetz, C; Tonn, J C; Peraud, A

2015-04-01

In idiopathic normal pressure hydrocephalus (NPH) ventriculoperitoneal (VP) shunt insertion is the method of choice to improve cardinal symptoms such as gait disturbance, urge incontinence and/or dementia. With reduced compliance, the brain of the elderly is prone for overdrainage complications. This was especially true with the use of differential pressure valve implantation. The present study compares clinical outcome and complication rates after VP shunt insertion with differential pressure valves in the early years and gravitational valves since 2005. The authors reviewed patients treated at our institution for NPH since 1995. Differential pressure valves were solely used in the initial years, while the treatment regimen changed to gravitational valves in 2005. Clinical improvement/surgical success rates as well as complications were compared between the two groups. Eighty-nine patients were enrolled for the present study. Mean age at the time of surgery was 73.5 ± 6.3 years. Male patients predominated with 73, compared with 16 female patients. Median follow-up time was 28 ± 26 months. Date of last follow-up was 1st October 2013. Forty-nine patients received a gravitational valve, while 40 were treated with differential pressure valves. In the gravitational group a significant improvement was observed after shunt insertion for gait disorder, cognitive impairment and urge incontinence (p < 0.0001, resp. p = 0.004), while a significant change in the differential pressure group was only seen for gait disorder (p = 0.03) but not for cognition or urinary incontinency (p > 0.05). The risk of hygroma as a sign of shunt overdrainage requiring surgical intervention was significantly higher in the differential pressure group (5 versus 0 in the gravitational group). Patients with NPH treated with gravitational valves in the present cohort showed a more profound improvement in their initial symptoms, including gait disorder, cognitive impairment

Comparison between the lumbar infusion and CSF tap tests to predict outcome after shunt surgery in suspected normal pressure hydrocephalus

PubMed Central

Kahlon, B; Sundbarg, G; Rehncrona, S

2002-01-01

Objective: To compare the lumbar infusion test and the cerebrospinal fluid (CSF) tap test for predicting the outcome of shunt surgery in patients with suspected normal pressure hydrocephalus. Methods: 68 patients with suspected normal pressure hydrocephalus were studied. The absence of preceding history indicated idiopathic disease in 75% of these. All patients were assessed twice with walking and psychometric tests before lumbar infusion test and tap test assessments. The lumbar infusion test was done using a constant infusion rate (0.80 ml/min) and regarded as positive if the steady state CSF plateau pressure reached levels of > 22 mm Hg (resistance to outflow > 14 mm Hg/ml/min). The tap test was regarded as positive if two or more of four different test items improved after CSF removal. As the variability in baseline test results was large, the better of two evaluations was used in comparisons with the results after CSF removal, as well as to evaluate the outcome after shunt surgery. Only patients with a positive lumbar infusion test or a positive tap test had surgery. Results: The results of the CSF tap test and the lumbar infusion test agreed in only 45% of the patients. Of the total cohort, 47 (69%) had positive test results and were operated on; 45 (96%) of these reported subjective improvement, and postoperative assessments verified the improvements in 38 (81%). Improvements were highly significant in walking, memory, and reaction time tests (p < 0.001). Most of the patients improved by surgery (84%) were selected by a positive lumbar infusion test, and only 42% by a positive tap test. Positive predictive values were 80% for lumbar infusion test and 94% for tap test. The false negative predictions in the operated group were much higher (58%) with the tap test than with the lumbar infusion test (16%). Conclusions: Both the lumbar infusion test and the tap test can predict a positive outcome of shunt operations in unselected patients with suspected normal

Normal pressure hydrocephalus: Diagnostic and predictive evaluationon

PubMed Central

Damasceno, Benito Pereira

2009-01-01

In typical cases, normal pressure hydrocephalus (NPH) manifests itself with the triad of gait disturbance, which begins first, followed by mental deterioration and urinary incontinence associated with ventriculomegaly (on CT or MRI) and normal cerebrospinal fluid (CSF) pressure. These cases present minor diagnostic difficulties and are the most likely to improve after shunting. Problems arise when NPH shows atypical or incomplete clinical manifestations (25–50% of cases) or is mimicked by other diseases. In this scenario, other complementary tests have to be used, preferentially those that can best predict surgical outcome. Radionuclide cisternography, intracranial pressure monitoring (ICP) and lumbar infusion tests can show CSF dynamics malfunction, but none are able to confirm whether the patient will benefit from surgery. The CSF tap test (CSF-TT) is the only procedure that can temporarily simulate the effect of definitive shunt. Since the one tap CSF-TT has low sensitivity, it cannot be used to exclude patients from surgery. In such cases, we have to resort to a repeated CSF-TT (RTT) or continuous lumbar external drainage (LED). The most reliable prediction would be achieved if RTT or LED proved positive, in addition to the occurrence of B-waves during more than 50% of ICP recording time. This review was based on a PubMed literature search from 1966 to date. It focuses on clinical presentation, neuroimaging, complementary prognostic tests, and differential diagnosis of NPH, particularly on the problem of selecting appropriate candidates for shunt. PMID:29213603

Normal pressure hydrocephalus

PubMed Central

Ahlskog, J. Eric

2011-01-01

Objective: To determine our community's incidence of clinically suspected normal pressure hydrocephalus (NPH), the rate of shunting for NPH, and short- and long-term outcomes of shunting. Methods: A retrospective query of the Mayo Clinic medical records linkage system was conducted to identify residents of Olmsted County, Minnesota, undergoing an invasive diagnostic procedure for evaluation of suspected NPH from 1995 through 2003. Among patients with shunts, early- and long-term outcomes were determined via a review of available medical records. Results: Forty-one patients underwent an invasive diagnostic procedure for evaluation of suspected NPH; 13 ultimately received shunts, representing an incidence of 1.19/100,000/year. The incidence of sustained definite improvements at 3 years after shunting was only 0.36/100,000/year. Definite gait improvement was documented in 75% at 3–6 months after shunt placement, although it dropped to 50% at 1 year and to 33% at 3 years. Only 1 of 8 patients with cognitive impairment and 1 of 6 patients with urinary incontinence had definite improvement in these symptoms at 3 years. No patient with moderate to severe postural instability experienced sustained definite improvement in any symptom. Complications occurred in 33% of patients including one perioperative death. Additional or alternative neurologic diagnoses later surfaced in 5 of 12 patients. Conclusions: In this community, NPH is relatively rare with an incidence approximating that of progressive supranuclear palsy in this population. Whereas early gait improvement was common, only one-third of patients enjoyed continued improvement by 3 years; cognition or urinary incontinence was even less responsive long term. Baseline postural instability predicted poor outcome. Clinicians should balance potential benefits of shunting against the known risks. PMID:21849644

Hydrostatic and hydrodynamic considerations in shunted normal pressure hydrocephalus.

PubMed

de Jong, D A; Delwel, E J; Avezaat, C J

2000-01-01

Symptoms of overdrainage, a frequent complication following shunting procedures especially in patients with normal-pressure hydrocephalus (NPH), are presumed to be caused by siphoning: gravity-enhanced flow of cerebro spinal fluid (CSF) through shunts in upright position. A critical reappraisal of siphoning and overdrainage (OD) is presented considering the occurrence of subdural hygromas (SDH) encountered in a group of patients shunted for NPH. The patients participated in the multicentre Dutch NPH study: 101 NPH patients were randomised for treatment with shunts with low or medium-high working pressure. Treatment with shunts with low working pressure resulted in an increased incidence of SDH (iSDH). Current physiological concepts of overdrainage are discussed and criticised. A new theory is presented on the basis of general physiological considerations. The theoretical model proposed yields a revised explanation for the origin of extremely low ventricular pressure in the upright position found with overdrainage. The efficacy of siphoning-preventing shunts and subtemporal decompression is explored in the light of the proposed theory.

Alzheimer CSF biomarkers may be misleading in normal-pressure hydrocephalus

PubMed Central

2014-01-01

Objective: This article discusses why CSF biomarkers found in normal-pressure hydrocephalus (NPH) can be misleading when distinguishing NPH from comorbid NPH with Alzheimer disease (AD). Methods: We describe NPH CSF biomarkers and how shunt surgery can change them. We hypothesize the effects that hydrocephalus may play on interstitial fluid space and amyloid precursor protein (APP) fragment drainage into the CSF based on a recent report and how this may explain the misleading CSF NPH biomarker findings. Results: In NPH, β-amyloid protein 42 (Aβ42) is low (as in AD), but total tau (t-tau) and phospho-tau (p-tau) levels are normal, providing conflicting biomarker findings. Low Aβ42 supports an AD diagnosis but tau findings do not. Importantly, not only Aβ42, but all APP fragments and tau proteins are low in NPH CSF. Further, these proteins increase after shunting. An increase in interstitial space and APP fragment drainage into the CSF during sleep was reported recently. Conclusions: In the setting of hydrocephalus when the brain is compressed, a decrease in interstitial space and APP protein fragment drainage into the CSF may be impeded, resulting in low levels of all APP fragments and tau proteins, which has been reported. Shunting, which decompresses the brain, would create more room for the interstitial space to increase and protein waste fragments to drain into the CSF. In fact, CSF proteins increase after shunting. CSF biomarkers in pre-shunt NPH have low Aβ42 and tau protein levels, providing misleading information to distinguish NPH from comorbid NPH plus AD. PMID:25332445

[Normal pressure hydrocephalus: prognostic value of height in patients treated with an identical shunt system].

PubMed

Aguas, Jesús; Rodrigo, Victor; Estupiñan, Francisco; Nogues, Pere; Villalba, Gloria; Villagrasa, Javier; Caral, Luis

2013-01-01

Normal pressure hydrocephalus (NPH) is a clinical entity frequently managed by means of a cerebrospinal fluid shunt. Hydrodynamic hypotheses consider hydrostatic pressure (as well as height) a very important variable for shunt system function. However, we did not find empirical studies supporting the influence of height on clinical response in the literature. Our objective was to study the prognostic value of height, as a variable related to hydrostatic pressure, when an identical shunt system is used. A prospective series of 61 idiopathic NPH cases was analyzed. All cases were shunted by means of a ventricle-peritoneal system with a 100mmH2O opening pressure valve. Anthropometric, clinical, radiological and pressure variables were registered, as well as delay for treatment, improvement and complications. 78.7% of cases improved after shunting. This group of patients was significantly taller (P=.005) than the group without response (median value 165cm versus 152cm). There was also a significant correlation between height and ventricular size decrease after the shunt. In our series opening valve pressure was a constant (100mmHg) and we could consequently focus on the effect of hydrostatic pressure (height). Moreover, we found a positive predictive value for taller patients, probably because we had selected an opening pressure especially suitable for them. Current gravitational valve shunt systems also recommend considering patient height when customising the system. Our study empirically supports this idea. Copyright © 2012 Sociedad Española de Neurocirugía. Published by Elsevier España. All rights reserved.

Hyperdynamic CSF motion profiles found in idiopathic normal pressure hydrocephalus and Alzheimer's disease assessed by fluid mechanics derived from magnetic resonance images.

PubMed

Takizawa, Ken; Matsumae, Mitsunori; Hayashi, Naokazu; Hirayama, Akihiro; Yatsushiro, Satoshi; Kuroda, Kagayaki

2017-10-18

Magnetic resonance imaging (MRI) does not only ascertain morphological features, but also measures physiological properties such as fluid velocity or pressure gradient. The purpose of this study was to investigate cerebrospinal fluid (CSF) dynamics in patients with morphological abnormalities such as enlarged brain ventricles and subarachnoid spaces. We used a time-resolved three dimensional phase contrast (3D-PC) MRI technique to quantitatively evaluate CSF dynamics in the Sylvian aqueduct of healthy elderly individuals and patients with either idiopathic normal pressure hydrocephalus (iNPH) or Alzheimer's disease (AD) presenting with ventricular enlargement. Nineteen healthy elderly individuals, ten iNPH patients, and seven AD patients (all subjects ≥ 60 years old) were retrospectively evaluated 3D-PC MRI. The CSF velocity, pressure gradient, and rotation in the Sylvian aqueduct were quantified and compared between the three groups using Kolmogorov-Smirnov and Mann-Whitney U tests. There was no statistically significant difference in velocity among the three groups. The pressure gradient was not significantly different between the iNPH and AD groups, but was significantly different between the iNPH group and the healthy controls (p < 0.001), and similarly, between the AD group and the healthy controls (p < 0.001). Rotation was not significantly different between the iNPH and AD groups, but was significantly different between the iNPH group and healthy controls (p < 0.001), and similarly, between the AD group and the healthy controls (p < 0.001). Quantitative analysis of CSF dynamics with time resolved 3D-PC MRI revealed differences and similarities in the Sylvian aqueduct between healthy elderly individuals, iNPH patients, and AD patients. The results showed that CSF motion is in a hyperdynamic state in both iNPH and AD patient groups compared to healthy elderly individuals, and that iNPH patients and AD patients display similar CSF motion profiles.

Functional and magnetic resonance imaging correlates of corpus callosum in normal pressure hydrocephalus before and after shunting

PubMed Central

Mataró, Maria; Matarín, Mar; Poca, Maria Antonia; Pueyo, Roser; Sahuquillo, Juan; Barrios, Maite; Junqué, Carme

2007-01-01

Background Normal pressure hydrocephalus (NPH) is associated with corpus callosum abnormalities. Objectives To study the clinical and neuropsychological effect of callosal thinning in 18 patients with idiopathic NPH and to investigate the postsurgical callosal changes in 14 patients. Methods Global corpus callosum size and seven callosal subdivisions were measured. Neuropsychological assessment included an extensive battery assessing memory, psychomotor speed, visuospatial and frontal lobe functioning. Results After surgery, patients showed improvements in memory, visuospatial and frontal lobe functions, and psychomotor speed. Two frontal corpus callosum areas, the genu and the rostral body, were the regions most related to the clinical and neuropsychological dysfunction. After surgery, total corpus callosum and four of the seven subdivisions presented a significant increase in size, which was related to poorer neuropsychological and clinical outcome. Conclusion The postsurgical corpus callosum increase might be the result of decompression, re‐expansion and increase of interstitial fluid, although it may also be caused by differences in shape due to cerebral reorganisation. PMID:17056634

Patients with a Normal Pressure Hydrocephalus Shunt Have Fewer Complications than Do Patients with Other Shunts.

PubMed

Schenker, Pascale; Stieglitz, Lennart H; Sick, Beate; Stienen, Martin N; Regli, Luca; Sarnthein, Johannes

2018-02-01

Ventriculoperitoneal (VP) shunting is a well-established therapy for hydrocephalus. However, complications are frequent. The incidence of idiopathic normal pressure hydrocephalus (NPH) increases with the aging of the population. We evaluated the functional status of patients and the classification of complications associated with VP shunt procedures in our center. We recorded all VP shunt procedures in our prospective patient registry from January 2013 to December 2015. Functional outcome (Karnofsky Performance Status [KPS] and modified Rankin Scale) and complications were compiled from patient records. Any deviation from the normal postoperative course within 3 months after surgery was considered a complication. Complications were classified with the therapy-oriented Clavien-Dindo grading system. We evaluated potential risk factors with a logistic regression model. From 285 procedures in the reporting period, 90 were excluded, resulting in 195 patients. Among those patients, 174 (90%) were shunt implantations and 21 (11%) were shunt revisions. Forty-four shunts (23%) were implanted for NPH. Median KPS improved over the first year after surgery. Although some type of complication was observed in 114 patients (58%), 60 of those complications (31%) did not require surgical treatment (Clavien-Dindo grade <3). In 50 patients (26%), the complication concerned the shunt itself. A high KPS at admission and NPH as underlying indication significantly reduced the odds ratio for a complication. Although shunt surgery has a high general rate of complications, this rate is significantly lower for patients with NPH. The decision for shunting in patients with NPH should consider the low complication rate specific for the group of patients with NPH. Copyright © 2017 Elsevier Inc. All rights reserved.

Phosphorylated tau/amyloid beta 1-42 ratio in ventricular cerebrospinal fluid reflects outcome in idiopathic normal pressure hydrocephalus

PubMed Central

2012-01-01

Background Idiopathic normal pressure hydrocephalus (iNPH) is a potentially reversible cause of dementia and gait disturbance that is typically treated by operative placement of a ventriculoperitoneal shunt. The outcome from shunting is variable, and some evidence suggests that the presence of comorbid Alzheimer's disease (AD) may impact shunt outcome. Evidence also suggests that AD biomarkers in cerebrospinal fluid (CSF) may predict the presence of AD. The aim of this study was to investigate the relationship between the phosphorylated tau/amyloid beta 1-42 (ptau/Aβ1-42) ratio in ventricular CSF and shunt outcome in patients with iNPH. Methods We conducted a prospective trial with a cohort of 39 patients with suspected iNPH. Patients were clinically and psychometrically assessed prior to and approximately 4 months after ventriculoperitoneal shunting. Lumbar and ventricular CSF obtained intraoperatively, and tissue from intraoperative cortical biopsies were analyzed for AD biomarkers. Outcome measures included performance on clinical symptom scales, supplementary gait measures, and standard psychometric tests. We investigated relationships between the ptau/Aβ1-42 ratio in ventricular CSF and cortical AD pathology, initial clinical features, shunt outcome, and lumbar CSF ptau/Aβ1-42 ratios in the patients in our cohort. Results We found that high ptau/Aβ1-42 ratios in ventricular CSF correlated with the presence of cortical AD pathology. At baseline, iNPH patients with ratio values most suggestive of AD presented with better gait performance but poorer cognitive performance. Patients with high ptau/Aβ1-42 ratios also showed a less robust response to shunting on both gait and cognitive measures. Finally, in a subset of 18 patients who also underwent lumbar puncture, ventricular CSF ratios were significantly correlated with lumbar CSF ratios. Conclusions Levels of AD biomarkers in CSF correlate with the presence of cortical AD pathology and predict aspects of

Glaucoma in patients with shunt-treated normal pressure hydrocephalus.

PubMed

Gallina, Pasquale; Savastano, Alfonso; Becattini, Eleonora; Orlandini, Simone; Scollato, Antonio; Rizzo, Stanislao; Carreras, Giulia; Di Lorenzo, Nicola; Porfirio, Berardino

2017-11-17

OBJECTIVE Changes in the pressure gradient between intraocular and intracranial compartments at the lamina cribrosa level are a possible explanation of normal tension glaucoma (NTG). Shunt-treated normal pressure hydrocephalus (NPH) is a model for testing whether the increase (time from disease onset to CSF shunt placement, i.e., "protection period") and decrease (time from shunt placement to observation, i.e., "exposure period") in intracranial pressure (ICP) are glaucoma protective or risk factors, respectively. The authors estimated the prevalence of NTG in patients with shunt-treated NPH and calculated the extent of optic nerve exposure to changes in the trans-lamina cribrosa gradient. METHODS Data obtained in patients with NPH who had undergone ventriculoperitoneal (VP) shunt placement were analyzed. Patients with more than 6 months' follow-up, no pathologies associated with ICP changes or CSF dynamics disturbances, and no surgical or valve-related complications were scheduled for ophthalmic evaluation. RESULTS Nine of 22 patients had NTG, which is about a 40-fold increase in rate compared with the rate in the general elderly population without hydrocephalus (p < 0.001). The median protection period was 12.0 months in patients with NTG and 18.0 months in those without NTG (p = 0.033). The median ICP decrease multiplied by duration of exposure in months was 76.0 mm Hg × months in the NTG group and 24.1 mm Hg × months in the no-NTG group (p = 0.048). The patients' median adjusted age (adjusted for "protection" and "exposure" times) was 85.1 years in the NTG group and 78.8 years in the no-NTG group (p = 0.001). CONCLUSIONS A crucial risk factor for development of NTG in patients with shunt-treated NPH is the duration of optic nerve exposure to the lowering of ICP. Patients with NPH who are candidates for CSF shunting should be informed of the risk of incurring glaucoma. Longitudinal studies could provide estimates of tolerated times for a given ICP decrease.

Pulse pressure waveform in hydrocephalus: what it is and what it isn't.

PubMed

Czosnyka, Marek; Czosnyka, Zofia; Keong, Nicole; Lavinio, Andreas; Smielewski, Piotr; Momjian, Shahan; Schmidt, Eric A; Petrella, Gianpaolo; Owler, Brian; Pickard, John D

2007-04-15

Apart from its mean value, the pulse waveform of intracranial pressure (ICP) is an essential element of pressure recording. The authors reviewed their experience with the measurement and interpretation of ICP pulse amplitude by referring to a database of recordings in hydrocephalic patients. The database contained computerized pressure recordings from 2100 infusion studies (either lumbar or intraventricular) or overnight ICP monitoring sessions in patients suffering from hydrocephalus of various types (both communicating and noncommunicating), origins, and stages of management (shunt or no shunt). Amplitude was calculated from ICP waveforms by using a spectral analysis methodology. The appearance of a pulse waveform amplitude is positive evidence of a technically correct recording of ICP and helps to distinguish between postural and vasogenic variations in ICP. Pulse amplitude is significantly correlated with the amplitude of cerebral blood flow velocity (R = 0.4, p = 0.012) as assessed using Doppler ultrasonography. Amplitude is positively correlated with a mean ICP (R = 0.21 in idiopathic normal-pressure hydrocephalus [NPH]; number of cases 131; p < 0.01) and resistance to cerebrospinal fluid outflow (R = 0.22) but does not seem to be correlated with cerebrospinal elasticity, dilation of ventricles, or severity of hydrocephalus (NPH score). Amplitude increases slightly with age (R = 0.39, p < 0.01; number of cases 46). A positive association between pulse amplitude and increased ICP during an infusion study is helpful in distinguishing between hydrocephalus and predominant brain atrophy. A large amplitude is associated with a good outcome after shunting (positive predictive power 0.9), whereas a low amplitude has no predictive power in outcome prognostication (0.5). Pulse amplitude is reduced by a properly functioning shunt. Proper recording, detection, and interpretation of ICP pulse waveforms provide clinically useful information about patients suffering from

Colour vision abnormality as the only manifestation of normal pressure hydrocephalus.

PubMed

Asensio-Sánchez, V M; Martín-Prieto, A

2018-01-01

The case is presented of a 73-year-old male patient who referred to having black and white vision. Computed tomography showed normal pressure hydrocephalus (NPH). Magnetic resonance imaging was not performed because the patient refused to undergo further examinations. Achromatopsia may be the first or only NPH symptom. It may be prudent to ask patients with NPH regarding colour vision. Copyright © 2017 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.

Corpus callosum functioning in patients with normal pressure hydrocephalus before and after surgery.

PubMed

Mataró, Maria; Poca, Maria Antonia; Matarín, Mar; Sahuquillo, Juan; Sebastián, Nuria; Junqué, Carme

2006-05-01

Our aim was to evaluate corpus callosum functioning in a group of patients with normal pressure hydrocephalus (NPH) before and after shunting. Left ear-extinction under a dichotic listening task was evaluated in twenty-three patients with NPH, 30 patients with Alzheimer's disease and 30 aged controls. Patients with NPH had higher levels of left ear extinction than the control and Alzheimer's groups. Sixty-one percent of NPH patients exhibited left ear suppression, compared with 13% of Alzheimer's patients and 17% of controls. Following surgery, NPH patients showed a significant change in the degree of asymmetry in the dichotic listening task. Hydrocephalus was associated with left-ear extinction,which diminished after surgery. Our results may indicate reversible functional damage in the corpus callosum.

Adjustable valves in normal-pressure hydrocephalus: a retrospective study of 218 patients.

PubMed

Zemack, Göran; Romner, Bertil

2002-12-01

We sought to assess the value of adjusting shunt valve opening pressure, complications, and outcomes with the use of an adjustable shunt valve in the treatment of patients with normal-pressure hydrocephalus (NPH). In a single-center retrospective study, 231 adjustable valves (range, 30-200 mm H(2)O) were the first shunt implantations in 147 patients with idiopathic NPH (INPH) and 71 patients with secondary NPH (SNPH). The effect of adjustment on gait disturbance, cognitive impairment, urinary incontinence and other symptoms were evaluated, and an improvement index was created. In the INPH group, 138 adjustments were performed in 49.0% of the patients (average, 0.94 adjustments/patient). For the SNPH group, 49 adjustments were performed in 32.4% of the patients (average, 0.69 adjustments/patient). The reasons for adjustment were overdrainage in 48 patients (25.7%), underdrainage in 98 patients (52.4%), subdural hematoma in 37 patients (19.8%), and other reasons in 2 patients (2.1%). Clinical status improved after 56 (49.1%) of all 114 adjustments, whereas 23 (42.6%) of 54 minor (< or =20 mm H2O) and 33 (66.0%) of 50 larger adjustments improved the patient's clinical status. The correlation of the improvement index with the size of the individual adjustments was not significant. Complications occurred in 43 (19.7%) of 218 patients, valve malfunction occurred in 3 patients (1.3%), infection occurred in 14 patients (6.4%), and nontraumatic subdural effusion occurred in 15 patients (6.9%; 8 were treated by adjustment alone). The 5-year shunt survival rate was 80.2%. Outcomes were excellent or good in 71 (78.9%) of 90 patients with INPH and in 30 (69.8%) of 43 patients with SNPH. Noninvasive, particularly consecutive, minor or single larger adjustments to the valve opening pressure can further improve outcome in patients with NPH who undergo shunting.

Pulse amplitude of intracranial pressure waveform in hydrocephalus.

PubMed

Czosnyka, Z; Keong, N; Kim, D J; Radolovich, D; Smielewski, P; Lavinio, A; Schmidt, E A; Momjian, S; Owler, B; Pickard, J D; Czosnyka, M

2008-01-01

There is increasing interest in evaluation of the pulse amplitude of intracranial pressure (AMP) in explaining dynamic aspects of hydrocephalus. We reviewed a large number of ICP recordings in a group of hydrocephalic patients to assess utility of AMP. From a database including approximately 2,100 cases of infusion studies (either lumbar or intraventricular) and overnight ICP monitoring in patients suffering from hydrocephalus of various types (both communicating and non-communicating), etiology and stage of management (non-shunted or shunted) pressure recordings were evaluated. For subgroup analysis we selected 60 patients with idiopathic NPH with full follow-up after shunting. In 29 patients we compared pulse amplitude during an infusion study performed before and after shunting with a properly functioning shunt. Amplitude was calculated from ICP waveforms using spectral analysis methodology. A large amplitude was associated with good outcome after shunting (positive predictive value of clinical improvement for AMP above 2.5 mmHg was 95%). However, low amplitude did not predict poor outcome (for AMP below 2.5 mmHg 52% of patients improved). Correlations of AMP with ICP and Rcsf were positive and statistically significant (N = 131 with idiopathic NPH; R = 0.21 for correlation with mean ICP and 0.22 with Rcsf; p< 0.01). Correlation with the brain elastance coefficient (or PVI) was not significant. There was also no significant correlation between pulse amplitude and width of the ventricles. The pulse amplitude decreased (p < 0.005) after shunting. Interpretation of the ICP pulse waveform may be clinically useful in patients suffering from hydrocephalus. Elevated amplitude seems to be a positive predictor for clinical improvement after shunting. A properly functioning shunt reduces the pulse amplitude.

Index of cerebrospinal compensatory reserve in hydrocephalus.

PubMed

Kim, Dong-Joo; Czosnyka, Zofia; Keong, Nicole; Radolovich, Danila K; Smielewski, Peter; Sutcliffe, Michael P F; Pickard, John D; Czosnyka, Marek

2009-03-01

An index of cerebrospinal compensatory reserve (RAP) has been introduced as a potential descriptor of neurological deterioration after head trauma. It is numerically computed as a linear correlation coefficient between the mean intracranial pressure and the pulse amplitude of the pressure waveform. We explore how RAP varies with different forms of physiological or nonphysiological intracranial volume loads in adult hydrocephalus, with and without a functioning cerebrospinal fluid (CSF) shunt. A database of intracranial pressure recordings during CSF infusion studies and overnight monitoring in hydrocephalic patients was reviewed for clinical comparison of homogeneous subgroups of patients with hypothetical differences of pressure-volume compensatory reserve. The database includes 980 patients of mixed etiology: idiopathic normal pressure hydrocephalus (NPH), 47%; postsubarachnoid hemorrhage NPH, 12%; noncommunicating hydrocephalus, 22%; others, 19%. All CSF compensatory parameters were calculated by using intracranial pressure waveforms. In NPH, RAP correlated strongly with the resistance to CSF outflow (r(s) = 0.35; P = 0.045), but weakly correlated with ventriculomegaly (r(s) = 0.13; P = 0.41). In idiopathic nonshunted NPH patients, RAP did not correlate significantly with elasticity calculated from the CSF infusion test (r(s) = 0.11; P = 0.21). During infusion studies, RAP increased in comparison to values recorded at baseline (from a median of 0.45-0.86, P = 0.14 * 10(-8)), indicating a narrowing of the volume-pressure compensatory reserve. During B-waves associated with the REM (rapid eye movement) phase of sleep, RAP increased from a median of 0.53 to 0.89; P = 1.2 * 10(-5). After shunting, RAP decreased (median before shunting, 0.59; median after shunting, 0.34; P = 0.0001). RAP also showed the ability to reflect the functional state of the shunt (patent shunt median, 0.36; blocked shunt median, 0.84; P = 0.0002). RAP appears to characterize pressure

Cerebrospinal fluid and blood flow in mild cognitive impairment and Alzheimer's disease: a differential diagnosis from idiopathic normal pressure hydrocephalus

PubMed Central

2011-01-01

Background Phase-contrast magnetic resonance imaging (PC-MRI) enables quantification of cerebrospinal fluid (CSF) flow and total cerebral blood (tCBF) flow and may be of value for the etiological diagnosis of neurodegenerative diseases. This investigation aimed to study CSF flow and intracerebral vascular flow in patients with Alzheimer's disease (AD) and patients with amnesic mild cognitive impairment (a-MCI) and to compare the results with patients with idiopathic normal pressure hydrocephalus (NPH) and with healthy elderly volunteers (HEV). Methods Ten a-MCI and 9 mild AD patients were identified in a comprehensive neurological and neuropsychological assessment. They underwent brain MRI; PC-MRI pulse sequence was performed with the following parameters: two views per segment; flip angle: 25° for vascular flow and 20° for CSF flow; field-of-view (FOV): 14 × 14 mm²; matrix: 256 × 128; slice thickness: 5 mm; with one excitation for exams on the 3 T machine, and 2 excitations for the 1.5 T machine exams. Velocity (encoding) sensitization was set to 80 cm/s for the vessels at the cervical level, 10 or 20 cm/s for the aqueduct and 5 cm/s for the cervical subarachnoid space (SAS). Dynamic flow images were analyzed with in-house processing software. The patients' results were compared with those obtained for HEVs (n = 12), and for NPH patients (n = 13), using multivariate analysis. Results Arterial tCBF and the calculated pulsatility index were significantly greater in a-MCI patients than in HEVs. In contrast, vascular parameters were lower in NPH patients. Cervical CSF flow analysis yielded similar values for all four populations. Aqueductal CSF stroke volumes (in μl per cardiac cycle) were similar in HEVs (34 ± 17) and AD patients (39 ± 18). In contrast, the aqueductal CSF was hyperdynamic in a-MCI patients (73 ± 33) and even more so in NPH patients (167 ± 89). Conclusion Our preliminary data show that a-MCI patients present with high systolic arterial peak

Changes in sensorimotor-related thalamic diffusion properties and cerebrospinal fluid hydrodynamics predict gait responses to tap test in idiopathic normal-pressure hydrocephalus.

PubMed

Tsai, Ping-Huei; Chen, Yung-Chieh; Chiang, Shih-Wei; Huang, Teng-Yi; Chou, Ming-Chung; Liu, Hua-Shan; Chung, Hsiao-Wen; Peng, Giia-Sheun; Ma, Hsin-I; Kao, Hung-Wen; Chen, Cheng-Yu

2018-05-07

To compare diffusion tensor (DT)-derived indices from the thalamic nuclei and cerebrospinal fluid (CSF) hydrodynamic parameters for the prediction of gait responsiveness to the CSF tap test in early iNPH patients. In this study, 22 patients with iNPH and 16 normal controls were enrolled with the approval of an institutional review board. DT imaging and phase-contrast magnetic resonance imaging were performed in patients and controls to determine DT-related indices of the sensorimotor-related thalamic nuclei and CSF hydrodynamics. Gait performance was assessed in patients using gait scale before and after the tap test. The Mann-Whitney U test and receiver operating characteristic (ROC) curve analysis were applied to compare group differences between patients and controls and assess the predictive performance of gait responsiveness to the tap test in the patients. Fractional anisotropy (FA) and axial diffusivity showed significant increases in the ventrolateral (VL) and ventroposterolateral (VPL) nuclei of the iNPH group compared with those of the control group (p < 0.05). The predictions of gait responsiveness of ventral thalamic FA alone (area under the ROC curve [AUC] < 0.8) significantly outperformed those of CSF hydrodynamics alone (AUC < 0.6). The AUC curve was elevated to 0.812 when the CSF peak systolic velocity and FA value were combined for the VPL nucleus, yielding the highest sensitivity (0.769) and specificity (0.778) to predict gait responses. Combined measurements of sensorimotor-related thalamic FA and CSF hydrodynamics can provide potential biomarkers for gait response to the CSF tap test in patients with iNPH. • Ventrolateral and ventroposterolateral thalamic FA may predict gait responsiveness to tap test. • Thalamic neuroplasticity can be assessed through DTI in idiopathic normal-pressure hydrocephalus. • Changes in the CST associated with gait control could trigger thalamic neuroplasticity. • Activities of sensorimotor-related circuits

Validation of a noninvasive test routinely used in otology for the diagnosis of cerebrospinal fluid shunt malfunction in patients with normal pressure hydrocephalus.

PubMed

Sakka, Laurent; Chomicki, Alexandre; Gabrillargues, Jean; Khalil, Toufic; Chazal, Jean; Avan, Paul

2016-02-01

Ventriculoperitoneal shunting is the first-line treatment for normal pressure hydrocephalus. Noninvasive auditory tests based on recorded otoacoustic emissions were assessed, as currently used for universal neonatal hearing screenings, for the diagnosis of cerebrospinal fluid shunt malfunction. The test was designed based on previous works, which demonstrated that an intracranial pressure change induces a proportional, characteristic, otoacoustic-emission phase shift. Forty-four patients with normal pressure hydrocephalus (23 idiopathic and 21 secondary cases) were included in this prospective observational study. The male:female sex ratio was 1.44, the age range was 21-87 years (mean age 64.3 years), and the range of the follow-up period was 1-3 years (mean 20 months). Patients were implanted with a Sophy SU8 adjustable-pressure valve as the ventriculoperitoneal shunt. The phase shifts of otoacoustic emissions in response to body tilt were measured preoperatively, immediately postoperatively, and at 3-6 months, 7-15 months, 16-24 months, and more than 24 months postoperatively. Three groups were enrolled: Group 1, 19 patients who required no valve opening-pressure adjustment; Group 2, 18 patients who required valve opening-pressure adjustments; and Group 3, 7 patients who required valve replacement. In Group 1, phase shift, which was positive before surgery, became steadily negative after surgery and during the follow-up. In Group 2, phase shift, which was positive before surgery, became negative immediately after surgery and increasingly negative after a decrease in the valve-opening pressure. In Group 3, phase shift was positive in 6 cases and slightly negative in 1 case before revision, but after revision phase shift became significantly negative in all cases. Otoacoustic emissions noninvasively reflect cerebrospinal fluid shunt function and are impacted by valve-opening pressure adjustments. Otoacoustic emissions consistently diagnosed shunt malfunction and

Does CSF outflow resistance predict the response to shunting in patients with normal pressure hydrocephalus?

PubMed

Boon, A J; Tans, J T; Delwel, E J; Egeler-Peerdeman, S M; Hanlo, P W; Wurzer, J A; Avezaat, C J; de Jong, D A; Gooskens, R H; Hermans, J

1998-01-01

The value of the measurements of CSF outflow resistance (Rcsf) relative to predicting outcome after shunting was studied. In a group of 101 patients with mainly idiopathic normal pressure hydrocephalus (NPH) Rcsf was obtained by lumbar constant flow infusion. Gait disturbance and dementia were quantified using an NPH scale (NPHS) and disability by the Modified Rankin scale (MRS). Patients were assessed before and at 1, 3, 6, 9 and 12 months after surgery. Outcome measures were differences between the preoperative and last NPHS and MRS scores. Improvement was defined as a change of > or = 15% in NPHS and > or = 1 grade in MRS. Intention-to-treat analysis of all patients at one year yielded improvement of 57% in NPHS and 59% in MRS. Efficacy analysis, excluding comorbidity unrelated to NPH, revealed positive predictive values of around 80% at Rcsf < 18, and between 90% and 100% at Rcsf > or = 18 mm Hg/ml/min. For Rcsf > or = 18, the likelihood ratios were also higher. We conclude that the best predictor of the response to shunting is an Rcsf > or = 18 mm Hg/ml/min. Since two-thirds of the patients with Rcsf < 18 showed improvement as well, these patients should not be denied shunting.

Cine phase-contrast MR imaging in normal pressure hydrocephalus patients: relation to surgical outcome.

PubMed

Egeler-Peerdeman, S M; Barkhof, F; Walchenbach, R; Valk, J

1998-01-01

Phase-contrast cine MR flow imaging through the aqueduct was used to establish the diagnosis of normal pressure hydrocephalus (NPH), and to predict outcome after shunting. From 1990-1994 16 patients, who were participants in the Dutch Normal Pressure Hydrocephalus Study [3], were studied. The patients included in this study met clinical and CT-scan criteria as described in this study, underwent cine phase-contrast MR imaging prior to placement of a CSF shunt, and had a follow-up 12 months after the operation. Claustrophobic patients, patients with a pacemaker or extremely agitated patients were excluded. Normal Flux was calculated in each patients, as the average difference in caudal and rostral flux (Fdiff) +2 times standard deviation (0.97 cc/sec) [2]. The clinical outcome was measured with a modified scale of activities of daily living (ADL) as described by Rankin. Of the 16 patients, 8 could not be evaluated due to restlessness during MR measurements, disabling cerebral vascular accidents or death before the end of the follow-up period. Of the remaining 8 patients, 5 had a normal flux, of which only one improved. Two patients had a Fdiff twice the normal range, which improved in both patients. One patient had no measurable flux, consistent with an aqueduct stenosis; he too improved. Overall, there was a concordance of MR findings with final outcome after shunting in 7 out of 8 patients. This pilot study, therefore, support the need to further evaluate flow with MR imaging techniques to select patients with shunt responsive NPH.

[An improved case of bedridden mental impairment with normal pressure hydrocephalus associated with acoustic neurinoma after tumor resection].

PubMed

Sugimoto, Seiichiro; Sugimoto, Akiko; Saita, Kazuko; Kishi, Masahiko; Shioya, Keiichi; Higa, Toshinobu

2008-08-01

A 67-year-old woman developed gait disturbance, dysarthria, cognitive impairment and incontinence at age 65, and became bedridden. She showed mutism, stupor and lower limb spasticity. Cranial CT and MRI revealed marked ventricular enlargement and a cerebellopontine angle tumor. CSF study showed normal pressure (125 mmH2O) and elevated protein (143 mg/dl). Radionuclide cisternography showed redistribution of radionuclide to the ventricles and intraventricular residual radionuclide after 72 hours, which allowed a diagnosis of normal pressure hydrocephalus. After removal of the tumor, ventricle size and CSF protein decreased, and the symptoms of cognitive impairment and motor dysfunction resolved. Histological examination showed acoustic neurinoma. Over the half of hydrocephalus following acoustic neurinoma shows a tendency to improve by surgical resection of the tumor. Neurologists who see cognitively impaired spastic bedridden patients should not overlook this pathology.

Lumboperitoneal Shunts for the Treatment of Idiopathic Normal Pressure Hydrocephalus: A Comparison of Small-Lumen Abdominal Catheters to Gravitational Add-On Valves in a Single Center.

PubMed

Nakajima, Madoka; Miyajima, Masakazu; Akiba, Chihiro; Ogino, Ikuko; Kawamura, Kaito; Sugano, Hidenori; Hara, Takeshi; Tange, Yuichi; Fusegi, Keiko; Karagiozov, Kostadin; Arai, Hajime

2018-04-23

Treating idiopathic normal pressure hydrocephalus (iNPH) with lumboperitoneal shunts (LPSs) may cause cerebrospinal fluid (CSF) overdrainage. To investigate whether LPSs, including gravitational "add-on" and programmable pressure valves (PPVs/+GVs), reduce complications and improve outcomes. We compared PPVs/+small lumen abdominal catheters (SLs) to PPVs/+GVs using different opening pressures for supine and standing positions. We analyzed 115 patients with iNPH in 2 consequent cohorts: 48 patients receiving LPSs with PPVs/+SLs and 67 patients receiving LPSs with PPVs/+GVs. The modified Rankin Scale (mRS), Japan iNPH grading scale, Mini Mental State Examination, Frontal Assessment Battery, and CSF biomarkers were evaluated. Comparisons of postoperative clinical factors in 64 patients in the PPV/+SL and PPV/+GV groups using 1:1 propensity score matching revealed differences in the mean (±standard deviation) postoperative mRS (2.65 ± 1.07 vs 2.16 ± 1.02, P = .049) and gait disturbance scores (1.97 ± 1.03 vs 1.39 ± 0.92, P = .011). Thus, outcomes improved in the LPS group with the GV. Serious and nonserious adverse event rates for the PPV/+SL and PPV/+GV groups were 22.9% and 19.4% (P = .647) and 38% and 17.9% (P = .018), respectively, indicating higher rates of subdural collections for the PPV/+SL group. This is the first study to examine LPS treatment for iNPH using a GV in tandem with a PPV. Our results suggest that the CSF shunt flow volume is restricted in the standing position and maintained in the supine position, thus improving iNPH symptoms. This may reduce intracranial CSF hypotension-related complications.

Pulse wave myelopathy: An update of an hypothesis highlighting the similarities between syringomyelia and normal pressure hydrocephalus.

PubMed

Bateman, Grant A

2015-12-01

Most hypotheses trying to explain the pathophysiology of idiopathic syringomyelia involve mechanisms whereby CSF is pumped against a pressure gradient, from the subarachnoid space into the cord parenchyma. On review, these theories have universally failed to explain the disease process. A few papers have suggested that the syrinx fluid may originate from the cord capillary bed itself. However, in these papers, the fluid is said to accumulate due to impaired fluid drainage out of the cord. Again, there is little evidence to substantiate this. This proffered hypothesis looks at the problem from the perspective that syringomyelia and normal pressure hydrocephalus are almost identical in their manifestations but only differ in their site of effect within the neuraxis. It is suggested that the primary trigger for syringomyelia is a reduction in the compliance of the veins draining the spinal cord. This reduces the efficiency of the pulse wave dampening, occurring within the cord parenchyma, increasing arteriolar and capillary pulse pressure. The increased capillary pulse pressure opens the blood-spinal cord barrier due to a direct effect upon the wall integrity and interstitial fluid accumulates due to an increased secretion rate. An increase in arteriolar pulse pressure increases the kinetic energy within the cord parenchyma and this disrupts the cytoarchitecture allowing the fluid to accumulate into small cystic regions in the cord. With time the cystic regions coalesce to form one large cavity which continues to increase in size due to the ongoing interstitial fluid secretion and the hyperdynamic cord vasculature. Crown Copyright © 2015. Published by Elsevier Ltd. All rights reserved.

Focal dilation and paradoxical collapse of cortical fissures and sulci in patients with normal-pressure hydrocephalus.

PubMed

Holodny, A I; George, A E; de Leon, M J; Golomb, J; Kalnin, A J; Cooper, P R

1998-11-01

The authors describe a subgroup of patients with shunt-proven normal-pressure hydrocephalus (NPH) who presented with focal fissural and sulcal dilation on imaging studies. The specific radiological features and methods of differentiating this condition from cortical atrophy are delineated. Normal-pressure hydrocephalus has been described as dilation of the ventricles that is out of proportion to the sulci. Sulcal dilation has been taken as evidence of cortical atrophy and has even been used as a criterion to exclude patients from undergoing a shunting procedure. The authors describe five cases of patients with shunt-proven NPH who presented with focal dilation of cortical fissures and sulci. In three of the cases, there was a paradoxical decrease in the size of the dilated fissures and sulci that paralleled the decrease in the size of the lateral ventricles following successful shunting. This study demonstrates that focal fissural and sulcal dilation may represent reservoirs of cerebrospinal fluid analogous to the ventricular system. Patients should not be denied a shunting procedure solely on the basis of focally dilated fissures of sulci.

The relationship between CSF circulation and cerebrovascular pressure-reactivity in normal pressure hydrocephalus.

PubMed

Czosnyka, Z; van den Boogaard, F; Czosnyka, M; Momjian, S; Gelling, L; Pickard, J D

2005-01-01

Previously, we documented association between CSF circulation and transcranial-Doppler derived autoregulation in non-shunted patients suffering from hydrocephalus. In the present study we sought to investigate the relationship between the resistance to CSF outflow and pressure-reactivity both in shunted and non-shunted NPH patients. Sixty-eight patients (47 non-shunted and 21 shunted) with NPH have been examined as a part of routine diagnostic procedure. Resistance to CSF outflow (Rcsf) was measured using a ventricular constant rate infusion test. Cerebrovascular pressure-reactivity was assessed as a moving correlation coefficient (PRx) between coherent 'slow waves' of ICP and arterial blood pressure (ABP). This variable has previously been demonstrated to correlate with the autoregulation of CBF in patients following head injury. Results. In non-shunted patients cerebrovascular pressure-reactivity (PRx) was negatively correlated with Rcsf (R = -0.5; p < 0.0005). This relationship was inverted in shunted patients: a positive correlation between PRx and Rcsf was found (R = 0.51; p < 0.03). Cerebrovascular pressure-reactivity is disturbed in patients with normal resistance to CSF outflow, suggesting underlying cerebrovascular disease. This result confirms our previous finding where transcranial Doppler autoregulation was investigated. After shunting the pressure-reactivity strongly depends on shunt functioning and deteriorates when the shunt is blocked.

Theoretical considerations on the pathophysiology of normal pressure hydrocephalus (NPH) and NPH-related dementia.

PubMed

Hamlat, Abderrahmane; Adn, Mahmoudreza; Sid-ahmed, Seddik; Askar, Brahim; Pasqualini, Edouardo

2006-01-01

Normal pressure hydrocephalus (NPH) is considered to be an example of reversible dementia although clinical improvement after shunting varies from subject to subject, and recent studies have pointed to a possible link with other dementia. The authors consider that the craniospinal compartment is a partially closed sphere with control device systems represented by the spinal axis and the sagittal sinus-arachnoid villi complex which interact with each other in the clinical patient setting. We hypothesise that changing spinal compliance by altering the flow process and CSF dynamics lead to hydrocephalus. Therefore four NPH types have been distinguished according to the alterations in spinal compliance, decrease in CSF absorption at the sagittal sinus or both occurrences. The authors consider that NPH and NPH-related diseases (NPH-RD) are initiated by the same common final pathway and demonstrate that NPH could represent an initial stage of NPH-RD. Progression of clinical signs can be explained as damage to the cerebral tissue by both intermittent increased intracranial pressure and pulse pressure waves leading to periventricular ischaemia. In addition, they believe that both volume equilibrium and spinal compliance are restored in patients who improve after CSF shunt, whereas in patients whose condition does not improve, only volume equilibrium is restored and not spinal compliance, which was the underlying cause of hydrocephalus in such cases. They therefore wonder whether cervical decompression should not be indicated in patients who show no improvement. Although attractive, this analysis warrants confirmation from clinical, radiological, and hydrodynamic studies.

Is it possible to minimize overdrainage complications with gravitational units in patients with idiopathic normal pressure hydrocephalus? Protocol of the randomized controlled SVASONA Trial (ISRCTN51046698).

PubMed

Lemcke, J; Meier, U; Müller, C; Fritsch, M; Eymann, R; Kiefer, M; Kehler, U; Langer, N; Rohde, V; Ludwig, H-Ch; Weber, F; Remenez, V; Schuhmann, M; Stengel, D

2010-01-01

Overdrainage is a common complication observed after shunting patients with idiopathic normal-pressure hydrocephalus (iNPH), with an estimated incidence up to 25%. Gravitational units that counterbalance intracranial pressure changes were developed to overcome this problem. We will set out to investigate whether the combination of a programmable valve and a gravitational unit (proGAV, Aesculap/Miethke, Germany) is capable of reducing the incidence of overdrainage and improving patient-centered outcomes compared to a conventional programmable valve (Medos-Codman, Johnson & Johnson, Germany). SVASONA is a pragmatic randomized controlled trial conducted at seven centers in Germany. Patients with a high probability of iNPH (based on clinical signs and symptoms, lumbar infusion and/or tap test, cranial computed tomography [CCT]) and no contraindications for surgical drainage will randomly be assigned to receive (1) a shunt assistant valve (proGAV) or (2) a conventional, programmable shunt valve (programmable Medos-Codman).We will test the primary hypothesis that the experimental device reduces the rate of overdrainage from 25% to 10%. As secondary analyses, we will measure iNPH-specific outcomes (i.e., the Black grading scale and the NPH Recovery Rate), generic quality of life (Short Form 36), and complications and serious adverse events (SAE). One planned interim analysis for safety and efficacy will be performed halfway through the study. To detect the hypothesized difference in the incidence of overdrainage with a type I error of 5% and a type II error of 20%, correcting for multiple testing and an anticipated dropout rate of 10%, 200 patients will be enrolled.The presented trial is currently recruiting patients, with the first results predicted to be available in late 2008.

DTI-MRI biomarkers in the search for normal pressure hydrocephalus aetiology: a review.

PubMed

Hoza, David; Vlasák, Aleš; Hořínek, Daniel; Sameš, Martin; Alfieri, Alex

2015-04-01

Normal pressure hydrocephalus (NPH) is a clinical syndrome characterized by gait disturbances, urinary incontinence and dementia. Clinical presentation overlaps with Alzheimer disease (AD). Early recognition thus early intervention (shunting) is important for successful treatment, but lack of a diagnostic test with sufficient sensitivity and specificity complicates the diagnosis. We performed literature search and composed a structured review of imaging biomarkers of NPH. Morphometric studies are not sufficient to diagnose NPH. Hydrocephalus is a common finding in elderly people due to the symmetric brain atrophy and is even more pronounced in patients with AD. The key MRI biomarker seems to be diffusion tensor imaging (DTI). According to recent studies, the DTI analysis of the splenium corporis callosi, posterior limb of internal capsule, hippocampus and fornix combined with measurement of Evans index is a promising MRI biomarker of NPH and could be used for NPH diagnostics and in the differential diagnosis from AD and other dementias.

Non-invasive assessment of pulsatile intracranial pressure with phase-contrast magnetic resonance imaging

PubMed Central

Lindstrøm, Erika Kristina; Vatnehol, Svein Are Sirirud; Mardal, Kent-André; Emblem, Kyrre Eeg; Eide, Per Kristian

2017-01-01

Invasive monitoring of pulsatile intracranial pressure can accurately predict shunt response in patients with idiopathic normal pressure hydrocephalus, but may potentially cause complications such as bleeding and infection. We tested how a proposed surrogate parameter for pulsatile intracranial pressure, the phase-contrast magnetic resonance imaging derived pulse pressure gradient, compared with its invasive counterpart. In 22 patients with suspected idiopathic normal pressure hydrocephalus, preceding invasive intracranial pressure monitoring, and any surgical shunt procedure, we calculated the pulse pressure gradient from phase-contrast magnetic resonance imaging derived cerebrospinal fluid flow velocities obtained at the upper cervical spinal canal using a simplified Navier-Stokes equation. Repeated measurements of the pulse pressure gradient were also undertaken in four healthy controls. Of 17 shunted patients, 16 responded, indicating high proportion of “true” normal pressure hydrocephalus in the patient cohort. However, there was no correlation between the magnetic resonance imaging derived pulse pressure gradient and pulsatile intracranial pressure (R = -.18, P = .43). Pulse pressure gradients were also similar in patients and healthy controls (P = .26), and did not differ between individuals with pulsatile intracranial pressure above or below established thresholds for shunt treatment (P = .97). Assessment of pulse pressure gradient at level C2 was therefore not found feasible to replace invasive monitoring of pulsatile intracranial pressure in selection of patients with idiopathic normal pressure hydrocephalus for surgical shunting. Unlike invasive, overnight monitoring, the pulse pressure gradient from magnetic resonance imaging comprises short-term pressure fluctuations only. Moreover, complexity of cervical cerebrospinal fluid flow and -pulsatility at the upper cervical spinal canal may render the pulse pressure gradient a poor surrogate marker for

Non-invasive assessment of pulsatile intracranial pressure with phase-contrast magnetic resonance imaging.

PubMed

Ringstad, Geir; Lindstrøm, Erika Kristina; Vatnehol, Svein Are Sirirud; Mardal, Kent-André; Emblem, Kyrre Eeg; Eide, Per Kristian

2017-01-01

Invasive monitoring of pulsatile intracranial pressure can accurately predict shunt response in patients with idiopathic normal pressure hydrocephalus, but may potentially cause complications such as bleeding and infection. We tested how a proposed surrogate parameter for pulsatile intracranial pressure, the phase-contrast magnetic resonance imaging derived pulse pressure gradient, compared with its invasive counterpart. In 22 patients with suspected idiopathic normal pressure hydrocephalus, preceding invasive intracranial pressure monitoring, and any surgical shunt procedure, we calculated the pulse pressure gradient from phase-contrast magnetic resonance imaging derived cerebrospinal fluid flow velocities obtained at the upper cervical spinal canal using a simplified Navier-Stokes equation. Repeated measurements of the pulse pressure gradient were also undertaken in four healthy controls. Of 17 shunted patients, 16 responded, indicating high proportion of "true" normal pressure hydrocephalus in the patient cohort. However, there was no correlation between the magnetic resonance imaging derived pulse pressure gradient and pulsatile intracranial pressure (R = -.18, P = .43). Pulse pressure gradients were also similar in patients and healthy controls (P = .26), and did not differ between individuals with pulsatile intracranial pressure above or below established thresholds for shunt treatment (P = .97). Assessment of pulse pressure gradient at level C2 was therefore not found feasible to replace invasive monitoring of pulsatile intracranial pressure in selection of patients with idiopathic normal pressure hydrocephalus for surgical shunting. Unlike invasive, overnight monitoring, the pulse pressure gradient from magnetic resonance imaging comprises short-term pressure fluctuations only. Moreover, complexity of cervical cerebrospinal fluid flow and -pulsatility at the upper cervical spinal canal may render the pulse pressure gradient a poor surrogate marker for

Usefulness of the convexity apparent hyperperfusion sign in 123I-iodoamphetamine brain perfusion SPECT for the diagnosis of idiopathic normal pressure hydrocephalus.

PubMed

Ohmichi, Takuma; Kondo, Masaki; Itsukage, Masahiro; Koizumi, Hidetaka; Matsushima, Shigenori; Kuriyama, Nagato; Ishii, Kazunari; Mori, Etsuro; Yamada, Kei; Mizuno, Toshiki; Tokuda, Takahiko

2018-03-16

OBJECTIVE The gold standard for the diagnosis of idiopathic normal pressure hydrocephalus (iNPH) is the CSF removal test. For elderly patients, however, a less invasive diagnostic method is required. On MRI, high-convexity tightness was reported to be an important finding for the diagnosis of iNPH. On SPECT, patients with iNPH often show hyperperfusion of the high-convexity area. The authors tested 2 hypotheses regarding the SPECT finding: 1) it is relative hyperperfusion reflecting the increased gray matter density of the convexity, and 2) it is useful for the diagnosis of iNPH. The authors termed the SPECT finding the convexity apparent hyperperfusion (CAPPAH) sign. METHODS Two clinical studies were conducted. In study 1, SPECT was performed for 20 patients suspected of having iNPH, and regional cerebral blood flow (rCBF) of the high-convexity area was examined using quantitative analysis. Clinical differences between patients with the CAPPAH sign (CAP) and those without it (NCAP) were also compared. In study 2, the CAPPAH sign was retrospectively assessed in 30 patients with iNPH and 19 healthy controls using SPECT images and 3D stereotactic surface projection. RESULTS In study 1, rCBF of the high-convexity area of the CAP group was calculated as 35.2-43.7 ml/min/100 g, which is not higher than normal values of rCBF determined by SPECT. The NCAP group showed lower cognitive function and weaker responses to the removal of CSF than the CAP group. In study 2, the CAPPAH sign was positive only in patients with iNPH (24/30) and not in controls (sensitivity 80%, specificity 100%). The coincidence rate between tight high convexity on MRI and the CAPPAH sign was very high (28/30). CONCLUSIONS Patients with iNPH showed hyperperfusion of the high-convexity area on SPECT; however, the presence of the CAPPAH sign did not indicate real hyperperfusion of rCBF in the high-convexity area. The authors speculated that patients with iNPH without the CAPPAH sign, despite showing

The Effect of Spinal Tap Test on Different Sensory Modalities of Postural Stability in Idiopathic Normal Pressure Hydrocephalus.

PubMed

Abram, Katrin; Bohne, Silvia; Bublak, Peter; Karvouniari, Panagiota; Klingner, Carsten M; Witte, Otto W; Guntinas-Lichius, Orlando; Axer, Hubertus

2016-01-01

Postural instability in patients with normal pressure hydrocephalus (NPH) is a most crucial symptom leading to falls with secondary complications. The aim of the current study was to evaluate the therapeutic effect of spinal tap on postural stability in these patients. Seventeen patients with clinical symptoms of NPH were examined using gait scale, computerized dynamic posturography (CDP), and neuropsychological assessment. Examinations were done before and after spinal tap test. The gait score showed a significant improvement 24 h after spinal tap test in all subtests and in the sum score (p < 0.003), while neuropsychological assessment did not reveal significant differences 72 h after spinal tap test. CDP showed significant improvements after spinal tap test in the Sensory Organization Tests 2 (p = 0.017), 4 (p = 0.001), and 5 (p = 0.009) and the composite score (p = 0.01). Patients showed best performance in somatosensory and worst performance in vestibular dominated tests. Vestibular dominated tests did not improve significantly after spinal tap test, while somatosensory and visual dominated tests did. Postural stability in NPH is predominantly affected by deficient vestibular functions, which did not improve after spinal tap test. Conditions which improved best were mainly independent from visual control and are based on proprioceptive functions.

EEG and Neuronal Activity Topography analysis can predict effectiveness of shunt operation in idiopathic normal pressure hydrocephalus patients.

PubMed

Aoki, Yasunori; Kazui, Hiroaki; Tanaka, Toshihisa; Ishii, Ryouhei; Wada, Tamiki; Ikeda, Shunichiro; Hata, Masahiro; Canuet, Leonides; Musha, Toshimitsu; Matsuzaki, Haruyasu; Imajo, Kaoru; Yoshiyama, Kenji; Yoshida, Tetsuhiko; Shimizu, Yoshiro; Nomura, Keiko; Iwase, Masao; Takeda, Masatoshi

2013-01-01

Idiopathic normal pressure hydrocephalus (iNPH) is a neuropsychiatric syndrome characterized by gait disturbance, cognitive impairment and urinary incontinence that affect elderly individuals. These symptoms can potentially be reversed by cerebrospinal fluid (CSF) drainage or shunt operation. Prior to shunt operation, drainage of a small amount of CSF or "CSF tapping" is usually performed to ascertain the effect of the operation. Unfortunately, conventional neuroimaging methods such as single photon emission computed tomography (SPECT) and functional magnetic resonance imaging (fMRI), as well as electroencephalogram (EEG) power analysis seem to have failed to detect the effect of CSF tapping on brain function. In this work, we propose the use of Neuronal Activity Topography (NAT) analysis, which calculates normalized power variance (NPV) of EEG waves, to detect cortical functional changes induced by CSF tapping in iNPH. Based on clinical improvement by CSF tapping and shunt operation, we classified 24 iNPH patients into responders (N = 11) and nonresponders (N = 13), and performed both EEG power analysis and NAT analysis. We also assessed correlations between changes in NPV and changes in functional scores on gait and cognition scales before and after CSF tapping. NAT analysis showed that after CSF tapping there was a significant decrease in alpha NPV at the medial frontal cortex (FC) (Fz) in responders, while nonresponders exhibited an increase in alpha NPV at the right dorsolateral prefrontal cortex (DLPFC) (F8). Furthermore, we found correlations between cortical functional changes and clinical symptoms. In particular, delta and alpha NPV changes in the left-dorsal FC (F3) correlated with changes in gait status, while alpha and beta NPV changes in the right anterior prefrontal cortex (PFC) (Fp2) and left DLPFC (F7) as well as alpha NPV changes in the medial FC (Fz) correlated with changes in gait velocity. In addition, alpha NPV changes in the right DLPFC (F

Magnetoencephalography and normal pressure hydrocephalus: A case report.

PubMed

Kotini, A; Birbilis, T; Anninos, P; Seimenis, I

2018-04-18

A 82-year-old male experiencing headaches, dementia, urinary incontinence and gait instability was diagnosed with normal pressure hydrocephalus (NPH) and underwent a resting state magnetoencephalography (MEG) examination. MEG data were recorded in a magnetically shielded room with a whole-head 122 channel biomagnetometer. Following MEG, a ventriculoperitoneal (VP) shunt was placed in his head and greatly improved his symptomatology. Spontaneous MEG recordings revealed lower magnetic fields at frontal and frontotemporal regions compared to central and posterior regions. This finding correlated well with the significant ventricular distention, and specifically the enlargement of the frontal horns of the lateral ventricles, observed in presurgical CT. The regional pattern of MEG signal decrease in NPH seems to be quite different from that encountered in brain atrophy. In the latter case, a more generalized distribution of low magnetic fields is observed, possibly reflecting the high sensitivity of MEG to activity originating in sulci. Acquired data suggest that MEG may be able to differentiate between NPH and brain atrophy. Furthermore, MEG could potentially constitute a non-invasive, non-imaging tool, useful in the selection of patients with NPH to undergo shunt surgery. The findings of this study warrant further research in patient groups before firm conclusions can be drawn.

Indications for endoscopic third ventriculostomy in normal pressure hydrocephalus.

PubMed

Paidakakos, Nikolaos; Borgarello, S; Naddeo, M

2012-01-01

Controversies remain regarding the proper diagnostic studies and prediction of outcome in patients with normal pressure hydrocephalus (NPH), and their management remains controversial. We propose a preoperative assessment routine the aim of which is to correctly select NPH patients, and to differentiate between them in terms of surgical treatment, identifying probable endoscopic third ventriculostomy (ETV) responders. We prospectively considered a group of 44 patients with suspected NPH on the basis of clinical symptoms and neuroradiological evidence, who have undergone supplemental diagnostic testing (tap test, external lumbar drainage, cerebrospinal fluid outflow resistance [Rout] determination through lumbar and ventricular infusion test). All 44 of these patients were treated with either shunt procedures or ETV. To choose the kind of treatment (shunt or ETV), we evaluated the individual response during infusion tests. The efficacy of both surgical techniques was approximately 70%, with a significantly lower complication rate for ETV. We evaluated the correlation between the various tests and the postoperative outcomes both for shunting and for ETV. Rout proved useful for preoperative assessment and choice of treatment. In carefully selected patients, ETV had qualitative results similar to shunting, presenting significantly fewer complications.

EEG and Neuronal Activity Topography analysis can predict effectiveness of shunt operation in idiopathic normal pressure hydrocephalus patients☆

PubMed Central

Aoki, Yasunori; Kazui, Hiroaki; Tanaka, Toshihisa; Ishii, Ryouhei; Wada, Tamiki; Ikeda, Shunichiro; Hata, Masahiro; Canuet, Leonides; Musha, Toshimitsu; Matsuzaki, Haruyasu; Imajo, Kaoru; Yoshiyama, Kenji; Yoshida, Tetsuhiko; Shimizu, Yoshiro; Nomura, Keiko; Iwase, Masao; Takeda, Masatoshi

2013-01-01

Idiopathic normal pressure hydrocephalus (iNPH) is a neuropsychiatric syndrome characterized by gait disturbance, cognitive impairment and urinary incontinence that affect elderly individuals. These symptoms can potentially be reversed by cerebrospinal fluid (CSF) drainage or shunt operation. Prior to shunt operation, drainage of a small amount of CSF or “CSF tapping” is usually performed to ascertain the effect of the operation. Unfortunately, conventional neuroimaging methods such as single photon emission computed tomography (SPECT) and functional magnetic resonance imaging (fMRI), as well as electroencephalogram (EEG) power analysis seem to have failed to detect the effect of CSF tapping on brain function. In this work, we propose the use of Neuronal Activity Topography (NAT) analysis, which calculates normalized power variance (NPV) of EEG waves, to detect cortical functional changes induced by CSF tapping in iNPH. Based on clinical improvement by CSF tapping and shunt operation, we classified 24 iNPH patients into responders (N = 11) and nonresponders (N = 13), and performed both EEG power analysis and NAT analysis. We also assessed correlations between changes in NPV and changes in functional scores on gait and cognition scales before and after CSF tapping. NAT analysis showed that after CSF tapping there was a significant decrease in alpha NPV at the medial frontal cortex (FC) (Fz) in responders, while nonresponders exhibited an increase in alpha NPV at the right dorsolateral prefrontal cortex (DLPFC) (F8). Furthermore, we found correlations between cortical functional changes and clinical symptoms. In particular, delta and alpha NPV changes in the left-dorsal FC (F3) correlated with changes in gait status, while alpha and beta NPV changes in the right anterior prefrontal cortex (PFC) (Fp2) and left DLPFC (F7) as well as alpha NPV changes in the medial FC (Fz) correlated with changes in gait velocity. In addition, alpha NPV changes in the right

Practice guideline: Idiopathic normal pressure hydrocephalus: Response to shunting and predictors of response: Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology.

PubMed

Halperin, John J; Kurlan, Roger; Schwalb, Jason M; Cusimano, Michael D; Gronseth, Gary; Gloss, David

2015-12-08

We evaluated evidence for utility of shunting in idiopathic normal pressure hydrocephalus (iNPH) and for predictors of shunting effectiveness. We identified and classified relevant published studies according to 2004 and 2011 American Academy of Neurology methodology. Of 21 articles, we identified 3 Class I articles. Shunting is possibly effective in iNPH (96% chance subjective improvement, 83% chance improvement on timed walk test at 6 months) (3 Class III). Serious adverse event risk was 11% (1 Class III). Predictors of success included elevated Ro (1 Class I, multiple Class II), impaired cerebral blood flow reactivity to acetazolamide (by SPECT) (1 Class I), and positive response to either external lumbar drainage (1 Class III) or repeated lumbar punctures. Age may not be a prognostic factor (1 Class II). Data are insufficient to judge efficacy of radionuclide cisternography or aqueductal flow measurement by MRI. Clinicians may choose to offer shunting for subjective iNPH symptoms and gait (Level C). Because of significant adverse event risk, risks and benefits should be carefully weighed (Level B). Clinicians should inform patients with iNPH with elevated Ro and their families that they have an increased chance of responding to shunting compared with those without such elevation (Level B). Clinicians may counsel patients with iNPH and their families that (1) positive response to external lumbar drainage or to repeated lumbar punctures increases the chance of response to shunting, and (2) increasing age does not decrease the chance of shunting being successful (both Level C). © 2015 American Academy of Neurology.

Chronic ("normal pressure") hydrocephalus in childhood and adolescence. A review of 16 cases and reappraisal of the syndrome.

PubMed

Bret, P; Chazal, J

1995-12-01

"Normal pressure" hydrocephalus (NPH) is generally considered to be a disorder of the adult and geriatric population. Only a few reports have described the possible occurrence of this condition in children. A series of 16 patients aged less than 20 years forms the basis of the present report. Among these 16 patients, 11 had a clearly identified etiologic factor and 7 had had a shunt previously implanted. The majority of patients exhibited at least two elements of the adult's triad of psychomotor retardation (14 cases) and/or psychotic-like symptoms (4 cases), gait anomalies (8 cases), and sphincter disturbances (3 cases). Six patients had their intracranial pressure (ICP) monitored. ICP values were estimated to be within the normal limits for age. All the 16 patients underwent shunting or shunt revision. Surgical results were as follows (mean follow-up 20 +/- 17.2 months): a good response to shunting was obtained in 12 cases ("cured": 5, improved: 7), while the other 4 patients failed to improve. It seems likely that associated parenchymal disorders have played a major role in therapeutic failures. In children showing ventricular dilation on computed tomographic (CT) analysis and a clinical picture of subtle psychomotor deterioration, it may be difficult to distinguish an active disorder of the CSF dynamics from "arrested hydrocephalus." Since intracranial manometry cannot be undertaken as a routine procedure, less invasive methods such as cerebrospinal fluid (CSF) tap test, psychometric, or urodynamic tests deserve special attention as reliable predictors of outcome after shunting. Because most patients undergo shunting without prior assessment of their CSF pressure, the term "chronic hydrocephalus" is proposed as an alternative designation to "NPH," since there is little argument for maintaining an instrumentally based definition of the syndrome.

An update on research priorities in hydrocephalus: overview of the third National Institutes of Health-sponsored symposium "Opportunities for Hydrocephalus Research: Pathways to Better Outcomes".

PubMed

McAllister, James P; Williams, Michael A; Walker, Marion L; Kestle, John R W; Relkin, Norman R; Anderson, Amy M; Gross, Paul H; Browd, Samuel R

2015-12-01

longitudinal metrics to validate or refute implemented practices, procedures, or tests. 4) Outcome in Hydrocephalus-development of specific, reliable batteries with metrics focused on the hydrocephalic patient; measurements of neurocognitive outcome and quality-of-life measures that are adaptable, trackable across the growth spectrum, and applicable cross-culturally; development of comparison metrics against normal aging and sensitive screening tools to diagnose idiopathic normal pressure hydrocephalus against appropriate normative age-based data; better understanding of the incidence and prevalence of hydrocephalus within both pediatric and adult populations; and comparisons of aging patterns in adults with hydrocephalus against normal aging patterns.

Reduced thalamic N-acetylaspartate in idiopathic normal pressure hydrocephalus: a controlled 1H-magnetic resonance spectroscopy study of frontal deep white matter and the thalamus using absolute quantification.

PubMed

Lundin, F; Tisell, A; Dahlqvist Leinhard, O; Tullberg, M; Wikkelsö, C; Lundberg, P; Leijon, G

2011-07-01

Patients with idiopathic normal pressure hydrocephalus (INPH) frequently have a reduction in cerebral blood flow in the subcortical frontal lobe/basal ganglia/thalamic areas. With magnetic resonance spectroscopy, the metabolism in the brain can be examined. The aim of this study was to investigate if there was a compromised metabolism in the thalamus and in the subcortical frontal areas in INPH patients. This was done by measuring total creatine, myo-inositol, total choline, N-acetylaspartate (NAA), total N-acetylaspartate (tNA), glutamate and lactate levels. A comparison was made with healthy individuals (HI). 16 patients (nine males, seven females, mean age 74 years, range 49-83) diagnosed as INPH and 15 HI (nine males, six females, mean age 74 years, range 62-89) were examined. (1)H magnetic resonance spectroscopy (1.5 T, point-resolved spectroscopy, echo time/relaxation time 30/3000 ms, volume of interest 2.5-3 ml) was performed in frontal deep white matter and in the thalamus. Absolute quantification with internal water as a reference was used. INPH patients had lower NAA (p=0.02) and lower tNA (p=0.05) concentrations in the thalamus compared with HI. NAA and tNA in the frontal deep white matter did not differ between patients and HI. The absolute metabolic concentrations of total creatine, myo-inositol total choline, tNA, lactate and Cr ratios in frontal deep white matter and in the thalamus were similar in INPH patients and HI. Reduced thalamic NAA and tNA in INPH patients suggest a compromised metabolic neuronal function in these regions. Thus, the thalamus might have an important role in the pathogenesis of INPH.

Measurement of peak CSF flow velocity at cerebral aqueduct, before and after lumbar CSF drainage, by use of phase-contrast MRI: utility in the management of idiopathic normal pressure hydrocephalus.

PubMed

Sharma, Ashwani Kumar; Gaikwad, Shailesh; Gupta, Vipul; Garg, Ajay; Mishra, Nalini K

2008-04-01

Since it was first described, normal pressure hydrocephalus (NPH) and its treatment by means of cerebrospinal fluid (CSF) shunting have been the focus of much investigation. Whatever be the cause of NPH, it has been hypothesized that in this disease there occurs decreased arterial expansion and an increased brain expansion leading to increased transmantle pressure. We cannot measure the latter, but fortunately the effect of these changes (increased peak flow velocity through the aqueduct) can be quantified with cine phase-contrast magnetic resonance imaging (MRI). This investigation was thus undertaken to characterize and measure CSF peak flow velocity at the level of the aqueduct, before and after lumbar CSF drainage, by means of a phase-contrast cine MRI and determine its role in selecting cases for shunt surgery. 37 patients with clinically suspected NPH were included in the study. Changes in the hyperdynamic peak CSF flow velocity with 50 ml lumbar CSF drainage (mimicking shunt) were evaluated in them for considering shunt surgery. 14 out of 15 patients who were recommended for shunt surgery, based on changes peak flow velocity after lumbar CSF drainage, improved after shunt surgery. None of the cases which were not recommended for shunt surgery, based on changes in CSF peak flow velocity after lumbar CSF drainage, improved after shunt surgery (2 out of 22 cases). The study concluded that the phase-contrast MR imaging, done before and after CSF drainage, is a sensitive method to support the clinical diagnosis of normal pressure hydrocephalus, selecting patients of NPH who are likely to benefit from shunt surgery, and to select patients of NPH who are not likely to benefit from shunt surgery.

Reduction of ventricular size after shunting for normal pressure hydrocephalus related to CSF dynamics before shunting.

PubMed Central

Tans, J T; Poortvliet, D C

1988-01-01

Reduction of ventricular size was determined by repeated computed tomography in 30 adult patients shunted for normal pressure hydrocephalus (NPH) and related to the pressure-volume index (PVI) and resistance to outflow of cerebrospinal fluid (Rcsf) measured before shunting. Rapid and marked reduction of ventricular size (n = 10) was associated with a significantly lower PVI than slow and moderate to marked (n = 13) or minimal to mild reduction (n = 7). Otherwise no relationship could be found between the reduction of ventricular size and PVI or Rcsf. It is concluded that both rate and magnitude of reduction of ventricular size after shunting for NPH are extremely variable. High brain elasticity seems to be the best predictor of rapid and marked reduction. PMID:3379425

The predictive value of cerebrospinal fluid tap-test in normal pressure hydrocephalus.

PubMed

Damasceno, B P; Carelli, E F; Honorato, D C; Facure, J J

1997-06-01

Eighteen patients (mean age of 66.5 years) with normal pressure hydrocephalus (NPH) underwent a ventriculo-peritoneal shunt surgery. Prior to operation a cerebrospinal fluid tap-test (CSF-TT) was performed with measurements of gait pattern and psychometric functions (memory, visuo-motor speed and visuo-constructive skills) before and after the removal of 50 ml CSF by lumbar puncture (LP). Fifteen patients improved and 3 were unchanged after surgery. Short duration of disease, gait disturbance preceding mental deterioration, wide temporal horns and small sulci on CT-scan were associated with good outcome after shunting. There was a good correlation between the results of CSF-TT and shunt surgery (chi 2 = 4.11, phi = 0.48, p < 0.05), with gait test showing highest correlation (r = 0.99, p = 0.01). In conclusion, this version of CSF-TT proved to be an effective test to predict improvement after shunting in patients with NPH.

Use of the proGAV shunt valve in normal-pressure hydrocephalus.

PubMed

Toma, Ahmed K; Tarnaris, Andrew; Kitchen, Neil D; Watkins, Laurence D

2011-06-01

Overdrainage is a common complication associated with shunt insertion in normal-pressure hydrocephalus (NPH) patients. Using adjustable valves with antigravity devices has been shown to reduce its incidence. The optimal starting setting of an adjustable shunt valve in NPH is debatable. To audit our single-center practice of setting adjustable valves. We performed a retrospective review of clinical records of all NPH patients treated in our unit between 2006 and 2009 by the insertion of shunts with a proGAV valve, recording demographic and clinical data, shunt complications, and revision rates. Radiological reports of postoperative follow-up computed tomography scans of the brain were reviewed for detected subdural hematomas. A proGAV adjustable valve was inserted in 50 probable NPH patients between July 2006 and November 2009. Mean ± SD age was 76 ± 7 years. Mean follow-up was 15 months. The initial shunt setting was 6 ± 3 cm H2O, and the final setting was 4.9 ± 1.9 cm H2O. Nineteen patients required 24 readjustment procedures (readjustment rate, 38%; readjustment number, 0.48 times per patient). One patient (2%) developed delayed bilateral subdural hematoma after readjustment of his shunt valve setting as an outpatient. Starting with a low opening pressure setting on a proGAV adjustable shunt valve does not increase the chances of overdrainage complications and reduces the need for repeated readjustments.

An overview of normal pressure hydrocephalus and its importance: how much do we really know?

PubMed

Siraj, Seema

2011-01-01

Normal pressure hydrocephalus (NPH) is a clinical triad of gait disturbance, dementia, and urinary incontinence combined with radiographic findings of ventriculomegaly and laboratory findings of normal cerebrospinal fluid pressures. Although it was first described by Hakim and Adams in 1965, there is no formal definition of NPH, causing discrepancy in its incidence in various studies. This ranges from 2 to 20 per million per year. It is estimated to be the cause of about 5% of cases of dementia and is one of the few reversible causes of dementia. Early diagnosis increases the rate of success to treatment. This makes accurate diagnosis and identification of responders to treatment important. There have been various studies on NPH in the general population, but not much has been said about it in long-term care facilities and the question arises on how many cases are missed. If a screening tool is in place to identify possible cases then further workup could be done to confirm the diagnosis and determine the need for shunting. Copyright © 2011 American Medical Directors Association. Published by Elsevier Inc. All rights reserved.

Magnetic Resonance Elastography Demonstrates Increased Brain Stiffness in Normal Pressure Hydrocephalus

PubMed Central

N, Fattahi; A, Arani; A, Perry; F, Meyer; A, Manduca; K, Glaser; ML, Senjem; RL, Ehman; J, Huston

2015-01-01

Introduction Normal pressure hydrocephalus (NPH) is a reversible neurologic disorder characterized by a triad of cognitive impairment, gait abnormality and urinary incontinence that is commonly treated with ventriculoperitoneal shunt placement. However, there are multiple overlapping symptoms which often make it difficult to differentiate NPH from other types of dementia and improved diagnostic techniques would help patient management. MR elastography (MRE) is a novel diagnostic tool that could potentially identify patients with NPH. The purpose of this study was to assess brain stiffness changes in NPH patients compared with age- and sex-matched cognitively normal individuals. Methods MRE was performed on 10 NPH patients and 21 age- and sex-matched volunteers with no known neurologic disorders. Image acquisition was conducted on a 3T MRI scanner. Shear waves with 60Hz vibration frequency were transmitted into the brain by a pillow-like passive driver. A novel postprocessing technique resistant to noise and edge artifacts was implemented to determine regional brain stiffness. The Wilcoxon rank sum test and linear regression were used for statistical analysis. Results A significant increase in stiffness was observed in the cerebrum (p = 0.001), occipital lobe (p = 0.0002), parietal lobe (p= 0.001), and the temporal lobe (p = 0.02) in the NPH group compared with normal controls. However, no significant difference was noted in other regions of the brain including the frontal lobe (p = 0.07), deep gray and white matter (p = 0.43), or the cerebellum (p = 0.20). Conclusion This study demonstrates increased brain stiffness in NPH patients compared to age- and sex-matched normal controls which motivates future studies investigating the use of MRE for NPH diagnosis and efficacy of shunt therapy. PMID:26542235

Hydrocephalus

MedlinePlus

... buildup of too much cerebrospinal fluid in the brain. Normally, this fluid cushions your brain. When you have too much, though, it puts harmful pressure on your brain. Hydrocephalus can be congenital, or present at birth. ...

Changes of cerebrospinal fluid protein concentrations and gait patterns in geriatric normal pressure hydrocephalus patients after ventriculoperitoneal shunting surgery.

PubMed

Chen, Carl P C; Huang, Yin-Cheng; Chang, Chen-Nen; Chen, Jean-Lon; Hsu, Chih-Chin; Lin, Wan-Ying

2018-06-01

Normal pressure hydrocephalus (NPH) was the first type of dementia ever described that can be treated using ventriculoperitoneal shunting surgery. Three typical clinical symptoms of NPH include gait disturbance, progressive cognitive dysfunction, and urinary incontinence. Although there are articles that have discovered several cerebrospinal fluid (CSF) protein biomarkers associated with NPH; however, studies examining individual and total protein concentrations from the ventricular CSF before and after shunting surgery are lacking. This study used proteomics to calculate the CSF individual and total protein concentrations before, and one week, one month and three months after the shunting surgery. Parameters of cadence, step length, walking speed, and percentages of single- and double-limb support in a gait cycle were measured. Protein concentrations associated with anti-oxidation, aging, and in the prevention of neurotoxic agent production increased by at least 2-folds after the surgery, indicating that the brain may become less susceptible to neurodegeneration. These proteins were alpha-1B-glycoprotein, apolipoproteins A-1 & A-IV, prostaglandin-H2 D-isomerase, alpha-1-antitrypsin, and serotransferrin. In gait analysis, lower cadence, decreased double-limb support, longer step length, and increased single-limb support were observed after the surgery, indicating a more stable walking balance. These changes lasted for a period of at least 3 months. As a result, shunting surgery may be recommended for geriatric patients with confirmed diagnosis of normal pressure hydrocephalus. Copyright © 2018 Elsevier Inc. All rights reserved.

Can MR measurement of intracranial hydrodynamics and compliance differentiate which patient with idiopathic normal pressure hydrocephalus will improve following shunt insertion?

PubMed

Bateman, G A; Loiselle, A M

2007-01-01

Between 10 and 90% of patients with normal pressure hydrocephalus (NPH) treated with a shunt will improve but they risk significant morbidity/mortality from this procedure. NPH is treated hydrodynamically and it has been assumed that a hydrodynamic difference must exist to differentiate which patient will respond. The purpose of this study is to see whether MRI hydrodynamics can differentiate which patients will improve post shunting. Thirty-two patients with NPH underwent MRI with flow quantification measuring the degree of ventricular enlargement, sulcal compression, white matter disease, total blood inflow, sagittal sinus outflow, aqueduct stroke volume, relative compliance ratio and arteriovenous delay. Patients were followed up after shunt insertion to gauge the degree of improvement and were compared with 12 age-matched controls and 12 patients with Alzheimer's disease. 63% of patients improved with insertion. The responders were identical to the non-responders in all variables. The NPH patients were significantly different to the controls (e.g. Total blood inflow reduced 20%, sagittal sinus outflow reduced 35%, aqueduct stroke volume increased 210%, relative compliance ratio reduced 60% and arteriovenous delay reduced 57% with p = 0.007, 0.03, 0.04, 0.0002 and 0.0003 respectively. The patient's with Alzheimer's disease values were midway between the NPH and control patients. Significant hydrodynamic differences were noted between NPH and controls but these were unable to differentiate the responders from non-responders. The hydrodynamics of Alzheimer's disease makes exclusion of comorbidity from this disease difficult.

Is a combination of Tc-SPECT or perfusion weighted magnetic resonance imaging with spinal tap test helpful in the diagnosis of normal pressure hydrocephalus?

PubMed

Hertel, F; Walter, C; Schmitt, M; Mörsdorf, M; Jammers, W; Busch, H P; Bettag, M

2003-04-01

The aim of this study was to evaluate the combination of spinal tap test (STT) with cerebral perfusion measurement assessed either by Tc-bicisate-SPECT (Tc-SPECT) or perfusion weighted MRI (pwMRI), or both, for a better preoperative selection of promising candidates for shunt operations in suspected idiopathic normal pressure hydrocephalus. 27 consecutive patients were examined with a standard clinical protocol (assessed by the Homburg Hydrocephalus Scale (HHS)) as well as with 99m Tc-bicisate-SPECT (n=27) or additionally by pwMRI (n=12) before and after STT. The results of these examinations were compared preoperatively for each patient and correlated with postoperative clinical outcome after shunt surgery. Nine patients showed both, a clinical improvement, and increased cerebral perfusion after STT. They underwent shunt surgery with good to excellent results. In another nine patients increasing cerebral perfusion was detected although they did not show a clear clinical improvement after STT. Six of them also received a shunt operation with good to excellent outcome. Three patients of the last group could have an operation. Nine patients did not show any clinical improvement or any kind of increasing cerebral perfusion after STT. Therefore, they did not undergo surgery. The results of SPECT and pwMRI correlated in 92 % of the patients (11 of 12). It is concluded that a combination of clinical assessment with SPECT or pwMRI is helpful in the preoperative selection of patients for shunting procedures with suspected NPH syndrome. This combination is a minimal invasive and objective test modality that is superior to STT alone. Further studies are necessary for a comparison of the described imaging techniques with different diagnostic tests in this difficult field of cerebral disease.

Current treatment of normal-pressure hydrocephalus: comparison of flow-regulated and differential-pressure shunt valves.

PubMed

Weiner, H L; Constantini, S; Cohen, H; Wisoff, J H

1995-11-01

FROM THE RECORDS of approximately 1500 shunt operations performed between 1987 and 1992, we identified 37 adults between ages 38 and 86 years (mean, 70 yr) with the normal-pressure hydrocephalus (NPH) syndrome who underwent surgery by a single surgeon. Since 1990, we have routinely used a flow-regulated shunt system (Orbis-Sigma valve [OSV]; Cordis Corporation, Miami, FL) in these patients. In this study, we compared the OSV system with conventional differential-pressure (DP) shunt systems uniformly used before 1990. This series (n = 37) consisted of 62% men (n = 23) and 38% women (n = 14). We excluded all patients with hydrocephalus associated with central nervous system neoplasms, intracerebral hemorrhage, or trauma as well those with radiographically documented late-onset aqueductal stenosis. All patients presented with the NPH clinical syndrome, chiefly with magnetic gait. In addition, 75% of patients experienced cognitive loss and 59% experienced urinary incontinence. The mean duration of preoperative symptoms was 35 months (range, 7-120 mo). Eight patients (22%) had undergone previous shunting procedures before referral to our service. A total of 89 shunt operations were performed in the 37 patients. Using actuarial methods and controlling for a history of prior shunt surgery, we found no significant difference in the time to initial malfunction (shunt survival) between the OSV and the DP shunts. There were three subdural hematomas and one infection in the OSV group compared with no complications in the DP valve group (P = 0.11). Thirty-six patients were available for follow-up, at a mean of 14 months after surgery. Nearly 90% of all patients experienced improvement in gait after shunting, regardless of the valve system that was used. There was one unrelated death. Realizing the limitations of a retrospective analysis and on the basis of the limited number of patients in this study, we conclude that using actuarial methods, we found no significant difference

The predictive value of ventricular CSF removal in normal pressure hydrocephalus.

PubMed

Krauss, J K; Regel, J P

1997-08-01

We report the impact of diagnostic ventricular CSF removal (VCR) on the gait disturbance in 24 patients with suspected normal pressure hydrocephalus (NPH) who had an intraventricular catheter for studies of CSF dynamics in order to select patients for shunt surgery. There were no complications related to removal of 24 ml CSF over a period of 6 hours in four instances or to the removal of 15-18 ml at one time in twenty instances. Seventeen out of 24 patients were diagnosed as having NPH. Two of those patients who had relatively mild symptoms had a prolonged amelioration of their gait disturbance after diagnostic VCR. Thirteen patients underwent subsequent shunt operations. Eleven of them had good functional and symptomatic improvement on follow-up. In those six patients who had a marked amelioration of their gait disturbance after diagnostic VCR, not only improvement of the gait disturbance was observed after shunting, but a consistent overall improvement as well. The predictive value of VCR was more variable in those six patients who had only a moderate response to VCR. No change of the gait disturbance after VCR was noted in seven patients in whom the diagnosis of NPH was not confirmed. Diagnostic VCR might be a valuable ancillary test in specialized centers which routinely use intraventricular CSF monitoring in the selection of patients for shunt surgery.

Experimental hydrocephalus following mechanical increment of intraventricular pulse pressure.

PubMed

Di Rocco, C; Pettorossi, V E; Caldarelli, M; Mancinelli, R; Velardi, F

1977-11-15

Experimental hydrocephalus has been induced in lambs by artificial increase of the amplitude of intraventricular cerebrospinal fluid (CSF) oscillations related to arterial pulsations, without concomitant changes of the mean CSF-pressure. The characteristics of this hydrocephalus demonstrate that the intraventricular CSF-pulsations can play a role in the genesis of ventricular dilation. Such a method may be used to produce an original model of hydrocephalus independent of changes of CSF-circulation or absorption.

Cerebrospinal fluid circulation and hydrocephalus.

PubMed

Leinonen, Ville; Vanninen, Ritva; Rauramaa, Tuomas

2017-01-01

Hydrocephalus (HC) is classically defined as dynamic imbalance between the production and absorption of cerebrospinal fluid (CSF) leading to enlarged ventricles. Potential causative factors include various brain disorders like tumors causing obstruction of CSF flow within the ventricular system or the subarachnoid space. Classification of HC is based on the site of CSF flow obstruction guiding optimal treatment, with endoscopic third ventriculostomy in intraventricular obstruction and CSF shunt in communicating HC. Another clinically relevant classification is acute and chronic; the most frequent chronic form is idiopathic normal-pressure hydrocephalus (iNPH). The reported incidence of HC varies according to the study population and classification used. The incidence of congenital HC is approximately 0.4-0.6/1,000 newborns and the annual incidence of iNPH varies from 0.5/100,000 to 5.5/100,000. Radiologically, ventricular dilatation may be nonspecific, and differentiation of iNPH from other neurodegenerative diseases may be ambiguous. There are no known specific microscopic findings of HC but a systematic neuropathologic examination is needed to detect comorbid diseases and possible etiologic factors of HC. Depending on the etiology of HC, there are several nonspecific signs potentially to be seen. Copyright © 2017 Elsevier B.V. All rights reserved.

Intracranial cerebrospinal fluid distribution and its postoperative changes in normal pressure hydrocephalus.

PubMed

Tsunoda, A; Mitsuoka, H; Bandai, H; Arai, H; Sato, K; Makita, J

2001-01-01

This study was conducted to investigate the usefulness of intracranial cerebrospinal fluid (CSF) volume measurement using MR-based methods in the management of patients with normal pressure hydrocephalus (NPH). The study group comprised 19 patients with NPH who showed a favorable outcome after ventricular shunting, 15 normal volunteers (NV), and 15 patients with cerebrovascular disease (CVD). A 3D-fast asymmetric spin echo MR imaging sequence and the region-growing method were used to extract the CSF space from MR images. Ventricular volume (VV) and intracranial CSF volume (ICV) were measured and the VV/ICV ratio was calculated in each case. In NPH patients, the CSF volume was measured again after shunting. The mean VV and VV/ICV ratio in the NPH group (91.1 mL and 45.2%, respectively) were significantly (p < 0.01) higher than those in the NV group (26.5 mL and 13.7%) and in the CVD group (44.5 mL and 17.8%). On the other hand, mean ICV values were not significantly different among the three groups. The VV was markedly decreased postoperatively (mean -40.7%), whereas the ICV was unchanged, resulting in a marked reduction in the VV/ICV ratio (mean -39.3%). These results suggest that patients with NPH have a unique intracranial CSF distribution, with an enlarged VV and a slightly increased ICV, resulting in a high VV/ICV ratio. Shunting led to dramatic improvement in our patients. It is likely that CSF measurement can provide valuable information in the management of patients with NPH.

Diagnostic effectiveness of quantitative [18F]flutemetamol PET imaging for detection of fibrillar amyloid β using cortical biopsy histopathology as the standard of truth in subjects with idiopathic normal pressure hydrocephalus

PubMed Central

2014-01-01

Introduction PET imaging of amyloid-β (Aβ) in vivo holds promise for aiding in earlier diagnosis and intervention in Alzheimer’s disease (AD) and mild cognitive impairment. AD-like Aβ pathology is a common comorbidity in patients with idiopathic normal pressure hydrocephalus (iNPH). Fifty patients with iNPH needing ventriculo-peritoneal shunting or intracranial pressure monitoring underwent [18F]flutemetamol PET before (N = 28) or after (N = 22) surgery. Cortical uptake of [18F]flutemetamol was assessed visually by blinded reviewers, and also quantitatively via standard uptake value ratio (SUVR) in specific neocortical regions in relation to either cerebellum or pons reference region: the cerebral cortex of (prospective studies) or surrounding (retrospective studies) the biopsy site, the contralateral homolog, and a calculated composite brain measure. Aβ pathology in the biopsy specimen (standard of truth [SoT]) was measured using Bielschowsky silver and thioflavin S plaque scores, percentage area of grey matter positive for monoclonal antibody to Aβ (4G8), and overall pathology impression. We set out to find (1) which pair(s) of PET SUVR and pathology SoT endpoints matched best, (2) whether quantitative measures of [18F]flutemetamol PET were better for predicting the pathology outcome than blinded image examination (BIE), and (3) whether there was a better match between PET image findings in retrospective vs. prospective studies. Results Of the 24 possible endpoint/SoT combinations, the one with composite-cerebellum SUVR and SoT based on overall pathology had the highest Youden index (1.000), receiver operating characteristic area under the curve (1.000), sensitivity (1.000), specificity (1.000), and sum of sensitivity and specificity for the pooled data as well as for the retrospective and prospective studies separately (2.00, for all 3). The BIE sum of sensitivity and specificity, comparable to that for quantitation, was highest using Bielschowsky

Normal pressure hydrocephalus: how often does the diagnosis hold water?

PubMed

Klassen, Bryan T; Ahlskog, J Eric

2011-09-20

To determine our community's incidence of clinically suspected normal pressure hydrocephalus (NPH), the rate of shunting for NPH, and short- and long-term outcomes of shunting. A retrospective query of the Mayo Clinic medical records linkage system was conducted to identify residents of Olmsted County, Minnesota, undergoing an invasive diagnostic procedure for evaluation of suspected NPH from 1995 through 2003. Among patients with shunts, early- and long-term outcomes were determined via a review of available medical records. Forty-one patients underwent an invasive diagnostic procedure for evaluation of suspected NPH; 13 ultimately received shunts, representing an incidence of 1.19/100,000/year. The incidence of sustained definite improvements at 3 years after shunting was only 0.36/100,000/year. Definite gait improvement was documented in 75% at 3-6 months after shunt placement, although it dropped to 50% at 1 year and to 33% at 3 years. Only 1 of 8 patients with cognitive impairment and 1 of 6 patients with urinary incontinence had definite improvement in these symptoms at 3 years. No patient with moderate to severe postural instability experienced sustained definite improvement in any symptom. Complications occurred in 33% of patients including one perioperative death. Additional or alternative neurologic diagnoses later surfaced in 5 of 12 patients. In this community, NPH is relatively rare with an incidence approximating that of progressive supranuclear palsy in this population. Whereas early gait improvement was common, only one-third of patients enjoyed continued improvement by 3 years; cognition or urinary incontinence was even less responsive long term. Baseline postural instability predicted poor outcome. Clinicians should balance potential benefits of shunting against the known risks.

Comparison of the sagittal sinus cross-sectional area between patients with multiple sclerosis, hydrocephalus, intracranial hypertension and spontaneous intracranial hypotension: a surrogate marker of venous transmural pressure?

PubMed

Bateman, Grant A; Lechner-Scott, Jeannette; Copping, Ross; Moeskops, Christopher; Yap, Swee Leong

2017-07-06

There is evidence that patients with multiple sclerosis (MS) and hydrocephalus share some common pathophysiological mechanisms. Alterations in CSF pressure are known to affect cerebral venous sinus geometry. To further explore these mechanisms, we measured the superior sagittal sinus (SSS) cross-sectional area 3 cm above the torcular using T2 images in 20 MS, 10 spontaneous intracranial hypotension (SIH), 21 hydrocephalus and 20 idiopathic intracranial hypertension (IIH) patients and compared with 20 matched controls. The SSS area was reduced by 25% in hydrocephalus (p = 0.0008), increased by 22% (p = 0.037) in SIH and unchanged in IIH compared to matched controls. In MS there was a 16% increase in SSS area (p = 0.01).The findings suggest that changes in SSS cross-sectional are common between MS and SIH patients, while in hydrocephalus and IIH these are different.

Differential diagnosis of normal pressure hydrocephalus by MRI mean diffusivity histogram analysis.

PubMed

Ivkovic, M; Liu, B; Ahmed, F; Moore, D; Huang, C; Raj, A; Kovanlikaya, I; Heier, L; Relkin, N

2013-01-01

Accurate diagnosis of normal pressure hydrocephalus is challenging because the clinical symptoms and radiographic appearance of NPH often overlap those of other conditions, including age-related neurodegenerative disorders such as Alzheimer and Parkinson diseases. We hypothesized that radiologic differences between NPH and AD/PD can be characterized by a robust and objective MR imaging DTI technique that does not require intersubject image registration or operator-defined regions of interest, thus avoiding many pitfalls common in DTI methods. We collected 3T DTI data from 15 patients with probable NPH and 25 controls with AD, PD, or dementia with Lewy bodies. We developed a parametric model for the shape of intracranial mean diffusivity histograms that separates brain and ventricular components from a third component composed mostly of partial volume voxels. To accurately fit the shape of the third component, we constructed a parametric function named the generalized Voss-Dyke function. We then examined the use of the fitting parameters for the differential diagnosis of NPH from AD, PD, and DLB. Using parameters for the MD histogram shape, we distinguished clinically probable NPH from the 3 other disorders with 86% sensitivity and 96% specificity. The technique yielded 86% sensitivity and 88% specificity when differentiating NPH from AD only. An adequate parametric model for the shape of intracranial MD histograms can distinguish NPH from AD, PD, or DLB with high sensitivity and specificity.

Extrapyramidal signs in normal pressure hydrocephalus: an objective assessment

PubMed Central

Mandir, Allen S; Hilfiker, Jennifer; Thomas, George; Minahan, Robert E; Crawford, Thomas O; Williams, Michael A; Rigamonti, Daniele

2007-01-01

Background Beyond the classic Normal Pressure Hydrocephalus (NPH) triad of gait disturbance, incontinence, and dementia are characteristic signs of motor dysfunction in NPH patients. We used highly sensitive and objective methods to characterize upper limb extrapyramidal signs in a series of NPH subjects compared with controls. Concentrated evaluation of these profound, yet underappreciated movement disorders of NPH before and after techniques of therapeutic intervention may lead to improved diagnosis, insight into pathophysiology, and targeted treatment. Methods Twenty-two (22) consecutive NPH patients and 17 controls performed an upper limb motor task battery where highly sensitive and objective measures of akinesia/bradykinesia, tone, and tremor were conducted. NPH subjects performed this test battery before and more than 36 h after continuous CSF drainage via a spinal catheter over 72 h and, in those subjects undergoing permanent ventriculo-peritoneal shunt placement, at least 12 weeks later. Control subjects performed the task battery at the same dates as the NPH subjects. Statistical analyses were applied to group populations of NPH and control subjects and repeated measures for within subject performance. Results Twenty (20) NPH subjects remained in the study following CSF drainage as did 14 controls. NPH subjects demonstrated akinesia/bradykinesia (prolonged reaction and movement times) and increased resting tone compared with controls. Furthermore, the NPH group demonstrated increased difficulty with self-initiated tasks compared with stimulus-initiated tasks. Following CSF drainage, some NPH subjects demonstrated reduced movement times with greater improvement in self- versus stimulus-initiated tasks. Group reaction time was unchanged. Resting tremor present in one NPH subject resolved following shunt placement. Tone measures were consistent for all subjects throughout the study. Conclusion Clinical motor signs of NPH subjects extend beyond gait deficits

Decreased Expression of hsa-miR-4274 in Cerebrospinal Fluid of Normal Pressure Hydrocephalus Mimics with Parkinsonian Syndromes.

PubMed

Jurjević, Ivana; Miyajima, Masakazu; Ogino, Ikuko; Akiba, Chihiro; Nakajima, Madoka; Kondo, Akihide; Kikkawa, Mika; Kanai, Mitsuyasu; Hattori, Nobutaka; Arai, Hajime

2017-01-01

Patients presenting with the classical idiopathic normal pressure hydrocephalus (iNPH) triad often show additional parkinsonian spectrum signs. Accurate differential diagnosis strongly influences the long-term outcome of cerebrospinal fluid (CSF) shunting. The aim of this study was to find potential CSF microRNA (miRNA) biomarkers for NPH mimics with parkinsonian syndromes that can reliably distinguish them from iNPH patients. Two cohorts of 81 patients (cohort 1, n = 55; cohort 2, n = 26) with possible iNPH who were treated in two centers between January 2011 and May 2014 were studied. In both cohorts, CSF samples were obtained from patients clinically diagnosed with iNPH (n = 21 and n = 10, respectively), possible iNPH with parkinsonian spectrum (PS) (n = 18, n = 10, respectively), possible iNPH with Alzheimer's disease (AD) (n = 16), and non-affected elderly individuals (NC) (n = 6). A three-step qRT-PCR analysis of the CSF samples was performed to detect miRNAs that were differentially expressed in the groups. The expression of hsa-miR-4274 in CSF was decreased in both cohorts of PS group patients (cohort 1: p < 0.0001, cohort 2: p < 0.0001), and was able to distinguish PS from iNPH with high accuracy (area under the curve = 0.908). The CSF concentration of hsa-miR-4274 also correlated with the specific binding ratio of ioflupane (123I) dopamine transporter scan (r = -0.494, p = 0.044). By contrast, the level of hsa-miR-4274 was significantly increased in the PS group after CSF diversion. Levels of CSF hsa-miR-4274 can differentiate PS from patients with iNPH, AD, and NC. This may be clinically useful for diagnostic purposes and predicting shunt treatment responses.

High Risk of Dementia in Ventricular Enlargement with Normal Pressure Hydrocephalus Related Symptoms1.

PubMed

Koivisto, Anne M; Kurki, Mitja I; Alafuzoff, Irina; Sutela, Anna; Rummukainen, Jaana; Savolainen, Sakari; Vanninen, Ritva; Jääskeläinen, Juha E; Soininen, Hilkka; Leinonen, Ville

2016-03-22

Differential diagnosis of ventricular enlargement with normal pressure hydrocephalus (NPH) related symptoms is challenging. Patients with enlarged ventricles often manifest cognitive deterioration but their long-term outcome is not well known. We aim to evaluate long-term cognitive outcome in patients with enlarged ventricles and clinically suspected NPH. A neurologist and a neurosurgeon clinically evaluated 468 patients with enlarged ventricles and suspected NPH using radiological methods, intraventricular pressure monitoring, and frontal cortical brain biopsy. The neurologist confirmed final diagnoses after a median follow-up interval of 4.8 years. Altogether, 232 patients (50%) with enlarged ventricles did not fulfill the criteria for shunt surgery. The incidence of dementia among patients with enlarged ventricles, and at least one NPH-related symptom with adequate follow-up data (n = 446) was high, varying from 77 (iNPH, shunt responders) to 141/1000 person-years (non-shunted patients with enlarged ventricles). At the end of the follow-up, 59% of all these patients were demented. The demented population comprised 73% of non-shunted patients with enlarged ventricles, 63% of shunted iNPH patients that did not respond to treatment, and 46% of iNPH patients that were initially responsive to shunting. The most common cause of dementia was Alzheimer's disease (n = 94, 36%), followed by vascular dementia (n = 68, 26%). One-half of patients with enlarged ventricles and clinically suspected NPH were not shunted after intraventricular pressure monitoring. Dementia caused by various neurodegenerative diseases was frequently seen in patients with ventricular enlargement. Thus, careful diagnostic evaluation in collaboration with neurologists and neurosurgeons is emphasized.

Dutch normal-pressure hydrocephalus study: prediction of outcome after shunting by resistance to outflow of cerebrospinal fluid.

PubMed

Boon, A J; Tans, J T; Delwel, E J; Egeler-Peerdeman, S M; Hanlo, P W; Wurzer, H A; Avezaat, C J; de Jong, D A; Gooskens, R H; Hermans, J

1997-11-01

The authors examined whether measurement of resistance to outflow of cerebrospinal fluid (Rcsf) predicts outcome after shunting for patients with normal-pressure hydrocephalus (NPH). In four centers 101 patients (most of whom had idiopathic NPH) who fulfilled strict entry criteria underwent shunt placement irrespective of their level of Rcsf obtained by lumbar constant flow infusion. Gait disturbance and dementia were quantified by using an NPH scale and the patient's level of disability was assessed by using the modified Rankin scale (mRS). In addition the Modified Mini-Mental State Examination was performed. Patients were assessed prior to and 1, 3, 6, 9, and 12 months after surgery. Primary outcome measures were based on differences between the preoperative and last NPH scale scores and mRS grades. Improvement was defined as a change measuring at least 15% in the NPH scale score and at least one mRS grade. Intention-to-treat analysis of all patients at 1 year yielded improvement for 57% in NPH scale score and 59% in mRS grade. Efficacy analysis, excluding serious events and deaths that were unrelated to NPH, was performed for 95 patients. Improvement rose to 76% in NPH scale score and 69% in mRS grade. Six cut-off levels of Rcsf were related to improvement in NPH scale score using two-by-two tables. Positive predictive values were approximately 80% for an Rcsf of 10, 12, or 15 mm Hg/ml/minute, 92% for an Rcsf of 18 mm Hg/ml/minute, and 100% for an Rcsf of 24 mm Hg/ml/minute. Negative predictive values were low. More important was the highest likelihood ratio of 3.5 for an Rcsf of 18 mm Hg/ml/minute. Extensive comorbidity was a major prognostic factor. Measurement of Rcsf reliably predicts outcome if the limit for shunting is raised to 18 mm Hg/ml/minute. At lower Rcsf values the decision depends mainly on the extent to which clinical and computerized tomography findings are typical of NPH.

Shunting normal pressure hydrocephalus: the predictive value of combined clinical and CT data.

PubMed

Vanneste, J; Augustijn, P; Tan, W F; Dirven, C

1993-03-01

The value of an ordinal global scale derived from combined clinical and CT data (clin/CT scale) to predict the clinical outcome in 112 patients shunted for presumed normal pressure hydrocephalus (NPH) was analysed. The clinical data were retrospectively collected, all CT scans were re-evaluated, and the clin/CT scale was determined blind to the results of further ancillary tests and to the post-surgical outcome. The scale ranked three classes of prediction: on the basis of clinical and CT characteristics, improvement after shunting was probable, possible, or improbable. The predictive value of the clin/CT scale for the subgroup of communicating NPH was established for two different strategies, depending on the strictness of selection criteria for shunting. In the subgroup of patients with presumed communicating NPH, the prevalence of shunt responsiveness was 29%; the best strategy was to shunt only patients with probable shunt-responsive NPH: the sensitivity was 0.54, the specificity 0.84, and the predictive accuracy 0.75, with a limited number of ineffective shunts (11%) and missed improvements (13%). The study illustrates its need to assess the pre-test probability of NPH based on combined clinical and CT data, before establishing the clinical usefulness of an ancillary test.

Shunting normal pressure hydrocephalus: the predictive value of combined clinical and CT data.

PubMed Central

Vanneste, J; Augustijn, P; Tan, W F; Dirven, C

1993-01-01

The value of an ordinal global scale derived from combined clinical and CT data (clin/CT scale) to predict the clinical outcome in 112 patients shunted for presumed normal pressure hydrocephalus (NPH) was analysed. The clinical data were retrospectively collected, all CT scans were re-evaluated, and the clin/CT scale was determined blind to the results of further ancillary tests and to the post-surgical outcome. The scale ranked three classes of prediction: on the basis of clinical and CT characteristics, improvement after shunting was probable, possible, or improbable. The predictive value of the clin/CT scale for the subgroup of communicating NPH was established for two different strategies, depending on the strictness of selection criteria for shunting. In the subgroup of patients with presumed communicating NPH, the prevalence of shunt responsiveness was 29%; the best strategy was to shunt only patients with probable shunt-responsive NPH: the sensitivity was 0.54, the specificity 0.84, and the predictive accuracy 0.75, with a limited number of ineffective shunts (11%) and missed improvements (13%). The study illustrates its need to assess the pre-test probability of NPH based on combined clinical and CT data, before establishing the clinical usefulness of an ancillary test. PMID:8459240

Diffusion tensor imaging profiles reveal specific neural tract distortion in normal pressure hydrocephalus.

PubMed

Keong, Nicole C; Pena, Alonso; Price, Stephen J; Czosnyka, Marek; Czosnyka, Zofia; DeVito, Elise E; Housden, Charlotte R; Sahakian, Barbara J; Pickard, John D

2017-01-01

The pathogenesis of normal pressure hydrocephalus (NPH) remains unclear which limits both early diagnosis and prognostication. The responsiveness to intervention of differing, complex and concurrent injury patterns on imaging have not been well-characterized. We used diffusion tensor imaging (DTI) to explore the topography and reversibility of white matter injury in NPH pre- and early after shunting. Twenty-five participants (sixteen NPH patients and nine healthy controls) underwent DTI, pre-operatively and at two weeks post-intervention in patients. We interrogated 40 datasets to generate a full panel of DTI measures and corroborated findings with plots of isotropy (p) vs. anisotropy (q). Concurrent examination of DTI measures revealed distinct profiles for NPH patients vs. controls. PQ plots demonstrated that patterns of injury occupied discrete white matter districts. DTI profiles for different white matter tracts showed changes consistent with i) predominant transependymal diffusion with stretch/ compression, ii) oedema with or without stretch/ compression and iii) predominant stretch/ compression. Findings were specific to individual tracts and dependent upon their proximity to the ventricles. At two weeks post-intervention, there was a 6·7% drop in axial diffusivity (p = 0·022) in the posterior limb of the internal capsule, compatible with improvement in stretch/ compression, that preceded any discernible changes in clinical outcome. On PQ plots, the trajectories of the posterior limb of the internal capsule and inferior longitudinal fasciculus suggested attempted 'round trips'. i.e. return to normality. DTI profiling with p:q correlation may offer a non-invasive biomarker of the characteristics of potentially reversible white matter injury.

Long-term Response of Cerebrospinal Fluid Pressure in Patients with Idiopathic Intracranial Hypertension - A Prospective Observational Study.

PubMed

Gafoor, V Abdul; Smita, B; Jose, James

2017-01-01

Idiopathic intracranial hypertension (IIH) is increased intracranial pressure (ICP) with normal cerebrospinal fluid (CSF) contents, in the absence of an intracranial mass, hydrocephalus, or other identifiable causes. The current knowledge of the treatment outcome of IIH is limited, and the data on the natural history of this entity are scant. The objective of the study is to study the treatment response of IIH by serially measuring the CSF opening pressure and to delineate the factors influencing the same. A prospective observational study in a cohort of fifty patients with IIH in whom CSF opening pressure was serially measured at pre-specified intervals. The mean CSF opening pressure at baseline was 302.4 ± 51.69 mm of H 2 O (range: 220-410). Even though a higher body mass index (BMI) showed a trend toward a higher CSF opening pressure, the association was not significant ( P = 0.168). However, the age of the patient had a significant negative correlation with the CSF pressure ( P = 0.006). The maximum reduction in CSF pressure occurred in the first 3 months of treatment, and thereafter it plateaued. Remission was attained in 12 (24%) patients. BMI had the strongest association with remission ( P = 0.001). In patients with IIH, treatment response is strongly related to BMI. However, patients with normal BMI are also shown to relapse and hence should have continuous, long-term follow-up. The reduction in CSF pressure attained in the first 3 months could reflect the long-term response to treatment.

Prediction of intracranial hypertension through noninvasive intracranial pressure waveform analysis in pediatric hydrocephalus.

PubMed

Ballestero, Matheus Fernando Manzolli; Frigieri, Gustavo; Cabella, Brenno Caetano Troca; de Oliveira, Sergio Mascarenhas; de Oliveira, Ricardo Santos

2017-09-01

The purpose of this study is to evaluate a noninvasive device to assess intracranial pressure wave form in children with hydrocephalus. A prospective and non-experimental descriptive-analytic study was performed. Fifty-six patients were enrolled in this study. They were divided in four groups: group A, children with clinically compensated hydrocephalus; B, surgically treated hydrocephalus; C, patients with acute intracranial hypertension due to hydrocephalus; and D, children without neurological disease (control). Data were collected through the installation of an extracranial deformation sensor, coupled to the children's scalp, which allowed registration of noninvasive intracranial pressure curves. Parameters obtained were analyzed: P2/P1 ratio, "classification P1 and P2 and P1 slope. P2/P1 index and "classification of P1 and P2" had a sensitivity of 80% and specificity of 100% for predicting intracranial hypertension. "P1 slope" presented no statistical difference. This study showed a useful and noninvasive method for monitoring intracranial pressure, which was able to indicate the intracranial hypertension in children with hydrocephalus and, thus, should be further investigated for clinical applications.

The value of temporary external lumbar CSF drainage in predicting the outcome of shunting on normal pressure hydrocephalus

PubMed Central

Walchenbach, R; Geiger, E; Thomeer, R; Vanneste, J

2002-01-01

Objective: It has been reported that temporary external lumbar CSF drainage (ELD) is a very accurate test for predicting the outcome after ventricular shunting in patients with normal pressure hydrocephalus (NPH). However, only a limited number of patients have been studied for assessing the predictive accuracy of ELD. Therefore, the value of ELD in predicting the outcome after a ventriculoperitoneal shunt in patients with presumed NPH was assessed. Methods: All patients with presumed NPH were invited to participate in this study. Clinical assessment, MRI, and neuropsychological evaluation were followed by a lumbar CSF tap test consisting of removing 40 ml CSF. When this test resulted in marked clinical improvement of gait impairment, mental disturbances, or both, the patient was shunted without further tests. In patients with either questionable or no improvement after the CSF tap test, ELD was carried out. The value of ELD for predicting the outcome after shunting was calculated by correlating the results of ELD with that of ventriculoperitoneal shunting. Results: Between January 1994 and December 2000, 49 presumed NPH patients from three institutes were included. Forty three had idiopathic, and the remaining six had secondary NPH. Forty eight patients were shunted; 39 had an ELD of whom 38 completed the test. After 2 months 35 of the 48 (73%) shunted patients had improved. The predictive value of a positive ELD was 87% (95% confidence interval (95% CI) 62–98) and that of a negative ELD 36% (95% CI 17–59). In two patients serious test related complications (meningitis) occurred without residual deficit. Conclusion: The study suggests that although the predictive value of a positive ELD is high, that of a negative ELD is deceptively low because of the high rate of false negative results. The costs and invasiveness of the test and the possibility of serious test related complications further limits its usefulness in managing patients with presumed NPH. PMID

Increased CSF aquaporin-4, and interleukin-6 levels in dogs with idiopathic communicating internal hydrocephalus and a decrease after ventriculo-peritoneal shunting.

PubMed

Schmidt, Martin J; Rummel, Christoph; Hauer, Jessica; Kolecka, Malgorzata; Ondreka, Nele; McClure, Vanessa; Roth, Joachim

2016-06-29

Studies in animal models, in which internal hydrocephalus has been induced by obstructing the cerebrospinal fluid pathways, have documented an up-regulation of the concentrations of aquaporin-4 (AQP4) in the brain. In this study, the concentrations of aquaporin-1 (AQP1), AQP1, AQP4 and interleukin-6 (IL-6) were determined in the CSF of dogs with idiopathic communicating hydrocephalus before and after the reduction of intraventricular volume following ventriculo-peritoneal shunt (VP-shunt) treatment. The concentrations of AQP4 and IL-6 were increased in the cerebrospinal fluid of dogs with hydrocephalus compared to controls. Both parameters significantly decreased after surgical treatment, accompanied by decrease of ventricular size and the clinical recovery of the dogs. AQP1 was not detectable in CSF. Brain AQP4 up-regulation might be a compensatory response in dogs with hydrocephalus. Future determination of AQP4 at the mRNA and protein level in brain tissue is warranted to substantiate this hypothesis.

Cerebrospinal fluid dynamics study in communicating hydrocephalus.

PubMed

Ramesh, Vengalathur Ganesan; Narasimhan, Vidhya; Balasubramanian, Chandramouli

2017-01-01

Communicating hydrocephalus often poses a challenge in diagnosis and management decisions. The objective of this study is to measure the opening pressure (P o ), pressure volume index (PVI), and cerebrospinal fluid outflow resistance (R out ), in patients with communicating hydrocephalus using bolus lumbar injection method and to evaluate its diagnostic and prognostic value. The study was conducted in 50 patients with communicating hydrocephalus, including normal pressure hydrocephalus (NPH) (19), post-meningitic hydrocephalus (23) and post-traumatic hydrocephalus (8). An improvised bolus lumbar injection method [the Madras Institute of Neurology (MIN) method] was used. In the NPH Group, the CSF dynamics studies correlated well with the clinico-radiological classification. The prediction of shunt responsiveness by CSF dynamics studies correlated with good outcome in 87.5%. In the post-meningitic hydrocephalus group, the value of CSF dynamics studies in predicting patients needing shunt was 89.5%. The CSF dynamics studies detected patients who needed shunt earlier than clinical or radiological indications. In the post-traumatic hydrocephalus group, 62.5% of patients improved with the treatment based on CSF dynamics studies. The improvised bolus lumbar injection method (MIN method) is a very simple test with fairly reliable and reproducible results. Study of CSF dynamics is a valuable tool in communicating hydrocephalus for confirmation of diagnosis and predicting shunt responsiveness. This is the first time that the value of CSF dynamics has been studied in patients with post-meningitic hydrocephalus. It was also useful for early selection of cases for shunting and for identifying patients with atrophic ventriculomegaly, thereby avoiding unnecessary shunt.

Diffusion tensor imaging profiles reveal specific neural tract distortion in normal pressure hydrocephalus

PubMed Central

Pena, Alonso; Price, Stephen J.; Czosnyka, Marek; Czosnyka, Zofia; DeVito, Elise E.; Housden, Charlotte R.; Sahakian, Barbara J.; Pickard, John D.

2017-01-01

Background The pathogenesis of normal pressure hydrocephalus (NPH) remains unclear which limits both early diagnosis and prognostication. The responsiveness to intervention of differing, complex and concurrent injury patterns on imaging have not been well-characterized. We used diffusion tensor imaging (DTI) to explore the topography and reversibility of white matter injury in NPH pre- and early after shunting. Methods Twenty-five participants (sixteen NPH patients and nine healthy controls) underwent DTI, pre-operatively and at two weeks post-intervention in patients. We interrogated 40 datasets to generate a full panel of DTI measures and corroborated findings with plots of isotropy (p) vs. anisotropy (q). Results Concurrent examination of DTI measures revealed distinct profiles for NPH patients vs. controls. PQ plots demonstrated that patterns of injury occupied discrete white matter districts. DTI profiles for different white matter tracts showed changes consistent with i) predominant transependymal diffusion with stretch/ compression, ii) oedema with or without stretch/ compression and iii) predominant stretch/ compression. Findings were specific to individual tracts and dependent upon their proximity to the ventricles. At two weeks post-intervention, there was a 6·7% drop in axial diffusivity (p = 0·022) in the posterior limb of the internal capsule, compatible with improvement in stretch/ compression, that preceded any discernible changes in clinical outcome. On PQ plots, the trajectories of the posterior limb of the internal capsule and inferior longitudinal fasciculus suggested attempted ‘round trips’. i.e. return to normality. Conclusion DTI profiling with p:q correlation may offer a non-invasive biomarker of the characteristics of potentially reversible white matter injury. PMID:28817574

Relative Position of the Third Characteristic Peak of the Intracranial Pressure Pulse Waveform Morphology Differentiates Normal-Pressure Hydrocephalus Shunt Responders and Nonresponders.

PubMed

Hamilton, Robert; Fuller, Jennifer; Baldwin, Kevin; Vespa, Paul; Hu, Xiao; Bergsneider, Marvin

2016-01-01

The diversion of cerebrospinal fluid (CSF) remains the principal treatment option for patients with normal-pressure hydrocephalus (NPH). External lumbar drain (ELD) and overnight intracranial pressure (ICP) monitoring are popular prognostic tests for differentiating which patients will benefit from shunting. Using the morphological clustering and analysis of continuous intracranial pulse (MOCAIP) algorithm to extract morphological metrics from the overnight ICP signal, we hypothesize that changes in the third peak of the ICP pulse pressure waveform can be used to differentiate ELD responders and nonresponders. Our study involved 66 patients (72.2 ± 9.8 years) undergoing evaluation for possible NPH, which included overnight ICP monitoring and ELD. ELD outcome was based on clinical notes and divided into nonresponders and responders. MOCAIP was used to extract mean ICP, ICP wave amplitude (waveAmp), and a metric derived to study P3 elevation (P3ratio). Of the 66 patients, 7 were classified as nonresponders and 25 as significant responders. The mean ICP and waveAmp did not vary significantly (p = 0.19 and p = 0.41) between the outcome groups; however, the P3ratio did show a significant difference (p = 0.04). Initial results suggest that the P3ratio might be used as a prognostic indicator for ELD outcome.

Hydrocephalus: an underrated long-term complication of microvascular decompression for trigeminal neuralgia. A single institute experience.

PubMed

Muratorio, Francesco; Tringali, G; Levi, V; Ligarotti, G K I; Nazzi, V; Franzini, A A

2016-11-01

Hydrocephalus is a common complication of posterior fossa surgery, but its real incidence after microvascular decompression (MVD) for idiopathic trigeminal neuralgia (TN) still remains unclear. The aim of this study was to focus on the potential association between MVD and hydrocephalus as a surgery-related complication. All patients who underwent MVD procedure for idiopathic TN at our institute between 2009 and 2014 were reviewed to search for early or late postoperative hydrocephalus. There were 259 consecutive patients affected by idiopathic TN who underwent MVD procedure at our institution between 2009 and 2014 (113 men, 146 women; mean age 59 years, range 30-87 years; mean follow-up 40.92 months, range 8-48 months). Nine patients (3.47 %) developed communicating hydrocephalus after hospital discharge and underwent standard ventriculo-peritoneal shunt. No cases of acute hydrocephalus were noticed. Our study suggests that late communicating hydrocephalus may be an underrated potential long-term complication of MVD surgery.

Prospective flutemetamol positron emission tomography and histopathology in normal pressure hydrocephalus.

PubMed

Rinne, Juha O; Frantzen, Janek; Leinonen, Ville; Lonnrot, Kimmo; Laakso, Aki; Virtanen, Kirsi A; Solin, Olof; Kotkansalo, Anna; Koivisto, Anne; Sajanti, Juha; Karppinen, Atte; Lehto, Hanna; Rummukainen, Jaana; Buckley, Chris; Smith, Adrian; Jones, Paul A; Sherwin, Paul; Farrar, Gill; McLain, Richard; Kailajarvi, Marita; Grachev, Igor D

2014-01-01

BACKGOUND/OBJECTIVE: To determine the level of association between uptake of the amyloid positron emission tomography (PET) imaging agent [(18)F]flutemetamol and the level of amyloid-β measured by immunohistochemical and histochemical staining in a frontal cortical region biopsy site. Seventeen patients with probable normal pressure hydrocephalus (NPH) underwent prospective [(18)F]flutemetamol PET and subsequent frontal cortical brain biopsy during ventriculoperitoneal shunting. Tissue amyloid-β was evaluated using the monoclonal antibody 4G8, thioflavin S and Bielschowsky silver stain. Four of the 17 patients (23.5%) had amyloid-β pathology based on the overall pathology read and also showed increased [(18)F]flutemetamol uptake. [(18)F]Flutemetamol standardized uptake values from the biopsy site were significantly associated with biopsy specimen amyloid-β levels (Pearson's r = 0.67; p = 0.006). There was also good correlation between the biopsy specimen amyloid-β level and uptake of [(18)F]flutemetamol in the region contralateral to the biopsy site (r = 0.67; p = 0.006), as well as with composite cortical [(18)F]flutemetamol uptake (r = 0.65; p = 0.008). The blinded visual read showed a high level of agreement between all readers (κ = 0.88). Two of 3 readers were in full agreement on all images; 1 reader disagreed on 1 of the 17 NPH cases. Blinded visual assessments of PET images by 1 reader were associated with 100% sensitivity to the overall pathology read, and assessments by the 2 others were associated with 75% sensitivity (overall sensitivity by majority read was 75%); specificity of all readers was 100%. [(18)F]Flutemetamol detects brain amyloid-β in vivo and shows promise as a valuable tool to study and possibly facilitate diagnosis of Alzheimer's disease both in patients with suspected NPH and among the wider population.

Cerebrospinal fluid dynamics study in communicating hydrocephalus

PubMed Central

Ramesh, Vengalathur Ganesan; Narasimhan, Vidhya; Balasubramanian, Chandramouli

2017-01-01

Context: Communicating hydrocephalus often poses a challenge in diagnosis and management decisions. Aims: The objective of this study is to measure the opening pressure (Po), pressure volume index (PVI), and cerebrospinal fluid outflow resistance (Rout), in patients with communicating hydrocephalus using bolus lumbar injection method and to evaluate its diagnostic and prognostic value. Materials and Methods: The study was conducted in 50 patients with communicating hydrocephalus, including normal pressure hydrocephalus (NPH) (19), post-meningitic hydrocephalus (23) and post-traumatic hydrocephalus (8). An improvised bolus lumbar injection method [the Madras Institute of Neurology (MIN) method] was used. Results: In the NPH Group, the CSF dynamics studies correlated well with the clinico-radiological classification. The prediction of shunt responsiveness by CSF dynamics studies correlated with good outcome in 87.5%. In the post-meningitic hydrocephalus group, the value of CSF dynamics studies in predicting patients needing shunt was 89.5%. The CSF dynamics studies detected patients who needed shunt earlier than clinical or radiological indications. In the post-traumatic hydrocephalus group, 62.5% of patients improved with the treatment based on CSF dynamics studies. Conclusions: The improvised bolus lumbar injection method (MIN method) is a very simple test with fairly reliable and reproducible results. Study of CSF dynamics is a valuable tool in communicating hydrocephalus for confirmation of diagnosis and predicting shunt responsiveness. This is the first time that the value of CSF dynamics has been studied in patients with post-meningitic hydrocephalus. It was also useful for early selection of cases for shunting and for identifying patients with atrophic ventriculomegaly, thereby avoiding unnecessary shunt. PMID:28484522

MR imaging of the hippocampus in normal pressure hydrocephalus: correlations with cortical Alzheimer's disease confirmed by pathologic analysis.

PubMed

Savolainen, S; Laakso, M P; Paljärvi, L; Alafuzoff, I; Hurskainen, H; Partanen, K; Soininen, H; Vapalahti, M

2000-02-01

MR studies have shown hippocampal atrophy to be a sensitive diagnostic feature of Alzheimer's disease (AD). In this study, we measured the hippocampal volumes of patients with a clinical diagnosis of normal pressure hydrocephalus (NPH), a potentially reversible cause of dementia when shunted. Further, we examined the relationship between the hippocampal volumes and cortical AD pathologic findings, intracranial pressure, and clinical outcomes in cases of NPH. We measured hippocampal volumes from 37 patients with a clinical diagnosis of NPH (27 control volunteers and 24 patients with AD). The patients with NPH underwent biopsy, and their clinical outcomes were followed for a year. Compared with those for control volunteers, the findings for patients with NPH included a minor left-side decrease in the hippocampal volumes (P < .05). Compared with those for patients with AD, the findings for patients with NPH included significantly larger hippocampi on both sides. Although not statistically significant, trends toward larger volumes were observed in patients with NPH who had elevated intracranial pressure, who benefited from shunting, and who did not display cortical AD pathologic findings. Measurements of hippocampal volumes among patients with a clinical diagnosis of NPH have clear clinical implications, providing diagnostic discrimination from AD and possibly prediction of clinical outcome after shunting.

Decreased Expression of hsa-miR-4274 in Cerebrospinal Fluid of Normal Pressure Hydrocephalus Mimics with Parkinsonian Syndromes

PubMed Central

Jurjević, Ivana; Miyajima, Masakazu; Ogino, Ikuko; Akiba, Chihiro; Nakajima, Madoka; Kondo, Akihide; Kikkawa, Mika; Kanai, Mitsuyasu; Hattori, Nobutaka; Arai, Hajime

2016-01-01

Background: Patients presenting with the classical idiopathic normal pressure hydrocephalus (iNPH) triad often show additional parkinsonian spectrum signs. Accurate differential diagnosis strongly influences the long-term outcome of cerebrospinal fluid (CSF) shunting. Objective: The aim of this study was to find potential CSF microRNA (miRNA) biomarkers for NPH mimics with parkinsonian syndromes that can reliably distinguish them from iNPH patients. Methods: Two cohorts of 81 patients (cohort 1, n = 55; cohort 2, n = 26) with possible iNPH who were treated in two centers between January 2011 and May 2014 were studied. In both cohorts, CSF samples were obtained from patients clinically diagnosed with iNPH (n = 21 and n = 10, respectively), possible iNPH with parkinsonian spectrum (PS) (n = 18, n = 10, respectively), possible iNPH with Alzheimer’s disease (AD) (n = 16), and non-affected elderly individuals (NC) (n = 6). A three-step qRT-PCR analysis of the CSF samples was performed to detect miRNAs that were differentially expressed in the groups. Results: The expression of hsa-miR-4274 in CSF was decreased in both cohorts of PS group patients (cohort 1: p < 0.0001, cohort 2: p < 0.0001), and was able to distinguish PS from iNPH with high accuracy (area under the curve = 0.908). The CSF concentration of hsa-miR-4274 also correlated with the specific binding ratio of ioflupane (123I) dopamine transporter scan (r = –0.494, p = 0.044). By contrast, the level of hsa-miR-4274 was significantly increased in the PS group after CSF diversion. Conclusion: Levels of CSF hsa-miR-4274 can differentiate PS from patients with iNPH, AD, and NC. This may be clinically useful for diagnostic purposes and predicting shunt treatment responses. PMID:27911315

Cortical brain biopsy in long-term prognostication of 468 patients with possible normal pressure hydrocephalus.

PubMed

Leinonen, Ville; Koivisto, Anne M; Alafuzoff, Irina; Pyykkö, Okko T; Rummukainen, Jaana; von Und Zu Fraunberg, Mikael; Jääskeläinen, Juha E; Soininen, Hilkka; Rinne, Jaakko; Savolainen, Sakari

2012-01-01

Normal pressure hydrocephalus (NPH) can be alleviated by cerebrospinal fluid shunting but the differential diagnosis and patient selection are challenging. Intraventricular intracranial pressure monitoring as part of the diagnostic workup as well as shunting enable to obtain cortical brain biopsies to detect amyloid-β (Aβ) and hyperphosphorylated tau (HPτ), the hallmark lesions of Alzheimer's disease (AD). In possible NPH, Aβ alone indicates an increased risk of AD and when present with HPτ probable AD, but the effect of those brain lesions on survival is not known. The aim of this study was to evaluate the predictive value of brain biopsy for the long-term outcome of possible NPH. Between 1991 and 2006, the Neurosurgery Department of the Kuopio University Hospital evaluated 468 patients for possible NPH by intraventricular intracranial pressure monitoring and frontal cortical brain biopsy immunostained against Aβ and HPτ. All patients were followed up until the end of 2008 (n = 201) or death (n = 267) with a median follow-up of 4.6 years (range 0-17). Logistic regression analysis with Cox models was applied. Out of the 468 cases, Aβ was detected in 197 (42%) cortical biopsies, and together with HPτ in 44 (9%). Aβ alone indicated increased risk of AD and with HPτ probable AD, but it did not affect survival. Vascular aetiology was the most frequent cause of death. Cortical biopsy findings indicate that NPH is at present a heterogeneous syndrome and has notable overlapping with AD. Brain biopsy did not predict survival but may open a novel research window to study the pathobiology of neurodegeneration. Copyright © 2012 S. Karger AG, Basel.

A quantitative index of intracranial cerebrospinal fluid distribution in normal pressure hydrocephalus using an MRI-based processing technique.

PubMed

Tsunoda, A; Mitsuoka, H; Sato, K; Kanayama, S

2000-06-01

Our purpose was to quantify the intracranial cerebrospinal fluid (CSF) volume components using an original MRI-based segmentation technique and to investigate whether a CSF volume index is useful for diagnosis of normal pressure hydrocephalus (NPH). We studied 59 subjects: 16 patients with NPH, 14 young and 13 elderly normal volunteers, and 16 patients with cerebrovascular disease. Images were acquired on a 1.5-T system, using a 3D-fast asymmetrical spin-echo (FASE) method. A region-growing method (RGM) was used to extract the CSF spaces from the FASE images. Ventricular volume (VV) and intracranial CSF volume (ICV) were measured, and a VV/ICV ratio was calculated. Mean VV and VV/ICV ratio were higher in the NPH group than in the other groups, and the differences were statistically significant, whereas the mean ICV value in the NPH group was not significantly increased. Of the 16 patients in the NPH group, 13 had VV/ICV ratios above 30%. In contrast, no subject in the other groups had a VV/ICV ratios higher than 30%. We conclude that these CSF volume parameters, especially the VV/ICV ratio, are useful for the diagnosis of NPH.

What Do Physicians Know About Normal Pressure Hydrocephalus and When Did They Know It? A Survey of 284 Physicians

PubMed Central

Conn, Harold O.; Lobo, Francis M.

2008-01-01

Normal pressure hydrocephalus (NPH) is a relatively new neurologic disorder first described by Salamon Hakim of Bogotá, Colombia, in 1965. NPH is characterized by three symptoms — impaired gait, incontinence and dementia — and an anatomic abnormality, i.e., enlargement of the cerebral ventricles, which can be seen on computerized tomographic or magnetic resonance imaging. Surprisingly, the intracranial pressure is normal. The first author of this article, a Yale Medical School faculty member, developed NPH over the decade from 1992 to 2002, during which it was erroneously diagnosed as cerebral atrophy and/or Parkinson’s disease. On recognizing the lack of awareness of NPH by physicians, he initiated a survey to explore this problem. He interviewed 166 practicing physicians who graduated from 50 American and 33 foreign medical schools, using a one-page, 10-point questionnaire (Part I). Almost one-third of the physicians had never heard of NPH. One-fifth had learned of NPH in medical school, and about half learned of it after medical school. Because there were insufficient physicians surveyed from 1986 to 2005, we recruited 118 additional physicians from the 20 Yale Medical School graduating classes from 1986 through 2005 (Part II). Two-thirds of them had learned of NPH in medical school, and one-fourth during residency and fellowship. Seven percent had never heard of NPH. The significance of these studies is discussed. PMID:18604308

A Pilot Study of Quantitative MRI Measurements of Ventricular Volume and Cortical Atrophy for the Differential Diagnosis of Normal Pressure Hydrocephalus

PubMed Central

Moore, Dana W.; Kovanlikaya, Ilhami; Heier, Linda A.; Raj, Ashish; Huang, Chaorui; Chu, King-Wai; Relkin, Norman R.

2012-01-01

Current radiologic diagnosis of normal pressure hydrocephalus (NPH) requires a subjective judgment of whether lateral ventricular enlargement is disproportionate to cerebral atrophy based on visual inspection of brain images. We investigated whether quantitative measurements of lateral ventricular volume and total cortical thickness (a correlate of cerebral atrophy) could be used to more objectively distinguish NPH from normal controls (NC), Alzheimer's (AD), and Parkinson's disease (PD). Volumetric MRIs were obtained prospectively from patients with NPH (n = 5), PD (n = 5), and NC (5). Additional NC (n = 5) and AD patients (n = 10) from the ADNI cohort were examined. Although mean ventricular volume was significantly greater in the NPH group than all others, the range of values overlapped those of the AD group. Individuals with NPH could be better distinguished when ventricular volume and total cortical thickness were considered in combination. This pilot study suggests that volumetric MRI measurements hold promise for improving NPH differential diagnosis. PMID:21860791

Diamox((R)) challenge test to decide indications for cerebrospinal fluid shunting in normal pressure hydrocephalus.

PubMed

Miyake, H; Ohta, T; Kajimoto, Y; Deguchi, J

1999-01-01

The indications for cerebrospinal fluid (CSF) shunting in patients with normal pressure hydrocephalus (NPH) have not been established. Establishment of clear-cut indications for this procedure is essential to ensure cost-effective, and safe treatment. We report the usefulness of the Diamox((R)) challenge test in evaluating indications for CSF shunting in patients with NPH. Pre- and post-operative responses in cerebral blood flow (CBF) and intracranial pressure (ICP) to intravenous administration of Diamox((R)) 1000mg (Diamox((R)) administration) were analysed in 41 patients with NPH who were treated by ventriculoperitoneal (VP) shunt with a programmable valve and an on-off valve. The preoperative response of ICP to Diamox((R)) administration was more than 10 mmHg in most patients in whom the shunt was effective (shunt effective group), however, it was less than 10 mmHg in most patients in whom the shunt was ineffective (shunt non-effective group). Furthermore, the postoperative response of ICP to Diamox((R)) administration decreased to less than 10 mmHg in most patients in the shunt effective group. The increases in CBF in response to Diamox((R)) administration were similar in the two groups both before and after placement of the VP shunt. Patients in whom ICP increased by more than 10 mmHg in response to Diamox((R)) administration were regarded to have poor CSF circulation and to thus be candidates for CSF shunting. The Diamox((R)) challenge test is a simple, safe procedure, useful in evaluating the response to treatment.

Negative-pressure and low-pressure hydrocephalus: the role of cerebrospinal fluid leaks resulting from surgical approaches to the cranial base.

PubMed

Filippidis, Aristotelis S; Kalani, M Yashar S; Nakaji, Peter; Rekate, Harold L

2011-11-01

Negative-pressure and low-pressure hydrocephalus are rare clinical entities that are frequently misdiagnosed. They are characterized by recurrent episodes of shunt failure because the intracranial pressure is lower than the opening pressure of the valve. In this report the authors discuss iatrogenic CSF leaks as a cause of low- or negative-pressure hydrocephalus after approaches to the cranial base. The authors retrospectively reviewed cases of low-pressure or negative-pressure hydrocephalus presenting after cranial approaches complicated with a CSF leak at their institution. Three patients were identified. Symptoms of high intracranial pressure and ventriculomegaly were present, although the measured pressures were low or negative. A blocked communication between the ventricles and the subarachnoid space was documented in 2 of the cases and presumed in the third. Shunt revisions failed repeatedly. In all cases, temporary clinical and radiographic improvement resulted from external ventricular drainage at subatmospheric pressures. The CSF leaks were sealed and CSF communication was reestablished operatively. In 1 case, neck wrapping was used with temporary success. Negative-pressure or low-pressure hydrocephalus associated with CSF leaks, especially after cranial base approaches, is difficult to treat. The solution often requires the utilization of subatmospheric external ventricular drains to establish a lower ventricular drainage pressure than the drainage pressure created in the subarachnoid space, where the pressure is artificially lowered by the CSF leak. Treatment involves correction of the CSF leak, neck wrapping to increase brain turgor and allow the pressure in the ventricles to rise to the level of the opening pressure of the valve, and reestablishing the CSF route.

Negative-Pressure Hydrocephalus: A Case Report on Successful Treatment Under Intracranial Pressure Monitoring with Bilateral Ventriculoperitoneal Shunts.

PubMed

Pandey, Sajan; Jin, Yi; Gao, Liang; Zhou, Cheng Cheng; Cui, Da Ming

2017-03-01

Negative-pressure hydrocephalus (NegPH), a very rare condition of unknown etiology and optimal treatment, usually presents postneurosurgery with clinical and imaging features of hydrocephalus, but with negative cerebrospinal fluid pressure. We describe a NegPH case of -3 mm Hg intracranial pressure that was successfully treated to achieve 5 mm Hg under continuous intracranial pressure monitoring with horizontal positioning, head down and legs elevated to 10°-15°, neck wrapping for controlled venous drainage, chest and abdomen bandages, infusion of 5% dextrose fluid to lower plasma osmolarity (Na + , 130-135 mmol/L), daily cerebrospinal fluid drainage >200 mL, and arterial blood gas partial pressure of carbon dioxide >40 mm Hg. Copyright © 2016 Elsevier Inc. All rights reserved.

[The role of computerized rheoencephalography in the assessment of normal pressure hydrocephalus. Preliminary report].

PubMed

Traczewski, Wojciech; Moskała, Marek; Szwabowska, Dorota; Gościński, Igor; Krupa, Mariusz; Polak, Jarosław

2005-01-01

It is generally agreed that the positive result of lumbar cerebrospinal fluid (CSF) withdrawal offers a reliable means for selection of patients likely to respond to shunting in normal pressure hydrocephalus (NPH). However the studies of cerebral hemodynamics in NPH are performed Routinely only in few neurosurgical centers. We therefore studied the effect of CSF withdrawal on cerebrovascular autoregulation (CVA) in this condition by means of computerized rheoencephalography [REG]. The study group consisted of 27 patients with presumed posttraumatic NPH. In each patient both the tap test and infusion test were performed. Psychometric tests and rheoencephalographic examinations were made twice: before and after CSF withdrawal. The obvious restoration of the functional state of CVA after CSF withdrawal was considered as a positive result of the tap test. Fourteen patients with a positive tap test and/or with resistance to CSF outflow (Rout) of more than 11 mmHg/ml/min were shunted. The improvement was obtained in 10 of them. Only one patient with a positive tap test did not improve. Our study suggests that restoration of CVA after CSF withdrawal is associated with high likelihood of shunt success, but not vice versa. Evaluation of CVA using REG seems to offer a new diagnostic tool in selecting patients likely to respond to shunting. Further studies are necessary to optimize the amount of CSF withdrawal, the delay between CSF withdrawal and control examinations and methodology of neuropsychological examinations.

Cytokine and growth factor concentration in cerebrospinal fluid from patients with hydrocephalus following endovascular embolization of unruptured aneurysms in comparison with other types of hydrocephalus.

PubMed

Killer, Monika; Arthur, Adam; Al-Schameri, Abdul Rahman; Barr, John; Elbert, Donald; Ladurner, Gunther; Shum, Julie; Cruise, Gregory

2010-10-01

To better understand the development of hydrocephalus of different origins, we evaluated cytokine and growth factor concentration in cerebrospinal fluid from patients with hydrocephalus. CSF was collected from patients developing hydrocephalus following hemorrhage (n = 15), patients with normal pressure hydrocephalus (n = 10), and following the embolization of unruptured intracranial aneurysms (n = 9). Myelography patients (n = 15) served as controls. Quantification of 11 molecules relating angiogenesis, inflammation, and wound healing in the CSF was performed using ELISA. All three hydrocephalus groups had decreased concentration of TIMP-4 compared to the normal group. The hemorrhage group showed increased concentration of IL-6, IL-8, MCP-1, MMP-9, and TIMP-1 compared to the control group. The unruptured aneurysm group had increased concentration of IL-6 and decreased concentration of TIMP-2 compared to the control group. Compared to the normal patients, increased concentrations of wound healing molecules were evident in all three groups. Increased inflammation was evident in the hemorrhage and unruptured aneurysm groups.

The Dynamic Gait Index in Evaluating Patients with Normal Pressure Hydrocephalus for Cerebrospinal Fluid Diversion.

PubMed

Chivukula, Srinivas; Tempel, Zachary J; Zwagerman, Nathan T; Newman, W Christopher; Shin, Samuel S; Chen, Ching-Jen; Gardner, Paul A; McDade, Eric M; Ducruet, Andrew F

2015-12-01

Diagnosing normal pressure hydrocephalus (NPH) remains challenging. Most clinical tests currently used to evaluate suspected NPH patients for shunt surgery are invasive, require inpatient admission, and are not without complications. An objective, noninvasive, and low-cost alternative would be ideal. A retrospective review was performed of prospectively collected dynamic gait index (DGI) scores, obtained at baseline and on every day of a 3- to 5-day lumbar cerebrospinal fluid (CSF) drainage trial on patients with suspected NPH at our institution. Between 2003 and 2014, 170 patients were suspected to have primary NPH (166, 97.6%) or secondary NPH (4, 2.4%). Using responsiveness to lumbar CSF drainage and subsequent shunting as the reference standard, we found that a baseline DGI ≥ 7 was found to have significant ability in selecting patients for permanent CSF diverting shunt surgery: sensitivity of 84.2% (95% confidence interval [95% CI]: 75.6%-90.2%), specificity of 80.6% (95% CI 70.0%-88.0%), and diagnostic odds ratio of 22.1 (95% CI 9.9-49.3). A baseline DGI ≥ 7 appears to provide an objective, low-cost, noninvasive measure to select patients with suspected NPH for a positive response to CSF diversion with high sensitivity, specificity and diagnostic odds ratio. Copyright © 2015 Elsevier Inc. All rights reserved.

Correlates of local cerebral blood flow (CBF) in normal pressure hydrocephalus patients before and after shunting--A retrospective analysis of [(15)O]H(2)O PET-CBF studies in 65 patients.

PubMed

Klinge, Petra M; Brooks, David J; Samii, Amir; Weckesser, Eva; van den Hoff, Jörg; Fricke, Harald; Brinker, Thomas; Knapp, Wolfram H; Berding, Georg

2008-04-01

Findings in local cerebral blood flow (rCBF) in Normal pressure hydrocephalus (NPH) have always been challenged by the variable and inconsistent relation to clinical symptoms before and after shunt treatment. [(15)O]H(2)O PET data from a consecutive cohort of 65 idiopathic NPH patients were retrospectively analyzed questioning whether the functional status before and after shunt treatment might correlate with local blood flow. Using statistical parametric mapping (SPM99, Wellcome Department of Cognitive Neurology, London), the [(15)O]H(2)O uptake was correlated with the preoperative clinical scores, graded according to a modified Stein and Langfitt score. Furthermore, differences in the uptake in the pre-and post-shunt treatment study after seven to 10 days in patients with and without clinical improvement were studied. A higher clinical score significantly correlated with a reduced tracer uptake in mesial frontal (k=1,239 voxel, Z=4.41) and anterior temporal (k=469, Z=4.07) areas. In the mesial frontal areas, tracer uptake showed significant reciprocal changes in the clinically improved vs. the unimproved patients. Matched with the existing literature, the regional blood flow alterations are suggested relevant to the NPH syndrome and to post-treatment functional changes. The present rCBF findings warrant prospective studies on the accuracy of neuroimaging studies as they may provide a more specific insight into disease mechanisms.

MR elastography of hydrocephalus

NASA Astrophysics Data System (ADS)

Pattison, Adam J.; Lollis, S. Scott; Perrinez, Phillip R.; Weaver, John B.; Paulsen, Keith D.

2009-02-01

Hydrocephalus occurs due to a blockage in the transmission of cerebrospinal fluid (CSF) in either the ventricles or subarachnoid space. Characteristics of this condition include increased intracranial pressure, which can result in neurologic deterioration [1]. Magnetic resonance elastography (MRE) is an imaging technique that estimates the mechanical properties of tissue in vivo. While some investigations of brain tissue have been performed using MRE [2,3,4,5], the effects due to changes in interstitial pressure and fluid content on the mechanical properties of the brain remain unknown. The purpose of this work is to assess the potential of MRE to differentiate between the reconstructed properties of normal and hydrocephalic brains. MRE data was acquired in 18 female feline subjects, 12 of which received kaolin injections resulting in an acute form of hydrocephalus. In each animal, four MRE scans were performed during the process including one pre-injection and three post-injection scans. The elastic parameters were obtained using a subzone-based reconstruction algorithm that solves Navier's equations for linearly elastic materials [6]. The remaining cats were used as controls, injected with saline instead of kaolin. To determine the state of hydrocephalus, ventricular volume was estimated from segmenting anatomical images. The mean ventricular volume of hydrocephalic cats significantly increased (P <~ 0.0001) between the first and second scans. The mean volume was not observed to increase (P >~ 0.5) for the control cats. Also, there was an observable increase in the recorded elastic shear modulus of brain tissue in the normal and hydrocephalic acquisitions. Results suggest that MRE is able to detect changes in the mechanical properties of brain tissue resulting from kaolin-induced hydrocephalus, indicating the need for further study.

Normal pressure hydrocephalus: survey on contemporary diagnostic algorithms and therapeutic decision-making in clinical practice.

PubMed

Krauss, J K; Halve, B

2004-04-01

There is no agreement on the best diagnostic criteria for selecting patients with normal pressure hydrocephalus (NPH) for CSF shunting. The primary objective of the present study was to provide a contemporary survey on diagnostic algorithms and therapeutic decision-making in clinical practice. The secondary objective was to estimate the incidence of NPH. Standardized questionnaires with sections on the incidence of NPH and the frequency of shunting, evaluation of clinical symptoms, and signs, diagnostic studies, therapeutic decision-making and operative techniques, postoperative outcome and complications, and the profiles of different centers, were sent to 82 neurosurgical centers in Germany known to participate in the care of patients with NPH. Data were analyzed from 49 of 53 centers which responded to the survey (65%). The estimated annual incidence of NPH was 1.8 cases/100.000 inhabitants. Gait disturbance was defined as the most important sign of NPH (61%). There was a wide variety in the choice of diagnostic tests. Cisternography was performed routinely only in single centers. Diagnostic CSF removal was used with varying frequency by all centers except one, but the amount of CSF removed by lumbar puncture differed markedly between centers. There was poor agreement on criteria for evaluation of continuous intracranial pressure recordings regarding both the amplitude and the relative frequency of B-waves. Both periventricular and deep white matter lesions were present in about 50% of patients being shunted, indicating that vascular comorbidity in NPH patients has gained more acceptance. Programmable shunts were used by more than half of the centers, and newer valve types such as gravitational valves have become more popular. According to the present survey, new diagnostic and therapeutic concepts on NPH have penetrated daily routine to a certain extent. Wide variability, however, still exists among different neurosurgical centers.

Should ventriculoatrial shunting be the procedure of choice for normal-pressure hydrocephalus?

PubMed

McGovern, Robert A; Kelly, Kathleen M; Chan, Andrew K; Morrissey, Nicholas J; McKhann, Guy M

2014-06-01

Ventriculoatrial (VA) shunting is rarely used for patients with normal-pressure hydrocephalus (NPH), likely due to surgeon technical preference and case reports indicating cardiopulmonary complications. However, these complications have typically been limited to adults in whom VA shunts had been placed when they were children. Few studies have directly compared VA shunting to ventriculoperitoneal (VP) shunting in cases of NPH. The authors retrospectively analyzed all NPH patients treated by a single surgeon at their center from January 2002 through December 2011. Thirty patients were treated with VA shunts (14 male) and 157 with VP shunts (86 male). The patients' mean age (± SD) at surgery was 73.7 ± 9.4 years for VA shunting and 76.0 ± 8.2 years for VP shunting; the median durations of follow-up were 42.0 months (IQR 19.2-63.6 months) and 34.2 months (IQR 15.8-67.5), respectively. Statistical analysis was performed using chi-square tests and Wilcoxon rank-sum tests. Perioperative and postoperative complications for VA and VP shunting cohorts, respectively, included distal revision (2.7% vs 6.6%, p = 0.45), proximal revision (2.7% vs 2.5%, p = 0.97), and postoperative seizure (2.7% vs 1.5%, p = 0.62). Shunt drainage-related subdural hematomas/hygromas developed in 8.1%/27.0% of VA shunt-treated patients versus 6.6%/26.4% of VP shunt-treated patients (p = 0.76/0.98) and were nearly always successfully managed with programmable-valve adjustment. Symptomatic intracerebral hemorrhage (1.5%) and shunt infection (2.0%) were only observed in those who underwent VP shunting. Of note, no cardiovascular complications were observed in any patient, and there were no cases of distal occlusion of the VA shunt. The authors found no significant differences in complication rates between VA and VP shunting, and VA shunting was not associated with any cardiopulmonary complications. Thus, in the authors' experience, VA shunting is at least as safe as VP shunting for treating NPH.

Five-year outcome of normal pressure hydrocephalus with or without a shunt: predictive value of the clinical signs, neuropsychological evaluation and infusion test.

PubMed

Savolainen, S; Hurskainen, H; Paljärvi, L; Alafuzoff, I; Vapalahti, M

2002-06-01

Between 1993-1995, 51 patients under 75 years of age with clinical symptoms and CT-based diagnosis of normal pressure hydrocephalus were investigated prospectively in order to clarify the value of neuropsychological tests, clinical symptoms and signs and infusion test in the differential diagnosis and prediction of outcome in normal pressure hydrocephalus. Patients had a thorough neurological examination, and neuropsychological evaluation. A 24-hour intraventricular ICP-measurement, infusion test, neurophysiological investigations and MRI study were performed, and a cortical biopsy was obtained. The ICP measurement defined the need for a shunt. All 51 patients were re-examined three and twelve months later. The final follow-up was accomplished five years postoperatively. 25 of the patients needed a shunt operation. One year after a shunt placement 72% of these patients had a good recovery concerning activities of daily living, 58% benefited in their urinary incontinence and 57% walked better. During the 5 years of follow-up 8 patients with shunt and 9 without shunt had died. Positive effect of shunting remained. Only one neuropsychological test, recognition of words test, distinguishes the patients with the need for a shunt. Simple mini mental examination test was not different in those who improved. In the postoperative follow-up patients with shunt showed no change in neuropsychological tests even if they were subjectively better. The infusion test was of no value in diagnosing NPH. The 16 patients with Alzheimer's disease did worse after one year than those without pathological changes, but the mortality was not increased. Specific neuropsychological tests are of little value in diagnosing NPH. Mini-Mental status examination was neither of value in diagnosing NPH nor in prediction of the outcome. In this study the infusion test did not improve diagnostic accuracy of NPH, but shunt placement relieves urinary incontinence and walking disability in patients with

Development of involuntary movements after ventriculoperitoneal shunting for normal pressure hydrocephalus in a patient with chronic-phase thalamic haemorrhage.

PubMed

Shindo, Keiichiro; Kondo, Takeo; Sugiyama, Ken; Nishijima, Kazunori; Furusawa, Yoshihito; Mori, Takayuki; Izumi, Shin-Ichi

2007-10-01

Delayed-onset involuntary movements have been described after thalamic stroke. We treated a patient with involuntary movements that increased after ventriculoperitoneal shunting (VPS) for normal pressure hydrocephalus (NPH) following thalamic haemorrage. One and one-half years after right thalamic and intraventricular haemorrhage, NPH suggested clinical evaluation and neuroimaging studies in a 56-year-old man. Hemidystonia and pseudochoreoathetosis were evident in the left arm, leg and trunk. Proprioceptive impairment and mild cerebellar dysfunction affected the left upper and lower extremity. Yet the patient could walk unassisted and carry out activities of daily living (ADL) rated as 90 points according to the Barthel Index (BI). Lumbar puncture lessened both gait disturbance and cognitive impairment. After VPS, cognition and urinary continence improved, but involuntary movements worsened, precluding unaided ambulation and decreasing the BI score to 65 points. Computed tomography after VPS showed resolution of NPH, while single-photon emission computed tomography showed increased cerebral blood flow after VPS. Increased cerebral blood flow after VPS is suspected to have promoted development of abnormal neuronal circuitry.

Evaluating the effect of hydrocephalus cause on the manner of changes in the effective parameters and clinical symptoms of the disease.

PubMed

Gholampour, Seifollah; Fatouraee, Nasser; Seddighi, Amir Saeed; Seddighi, Afsoun

2017-01-01

In the present study, the heads of 11 normal subjects and 21 patients affected by hydrocephalus due to three different causes were simulated using fluid-structure interaction (FSI). To validate the results, the calculated diagram of CSF velocity in aqueduct of Sylvius (AS) was compared with the similar velocity diagram measured using Cine PC-MRI for the same subject. After ensuring the agreement of results, other outputs such as CSF pressure were calculated non-invasively using FSI. The intracranial pressure and CSF pressure in AS and behind the optic nerve sheath were in patients 5-5.3 times the value in normal subjects and the ventricular system volume in patients was 10.2-11.1 times the value in normal subjects. However, the difference between the coefficient of variation and the maximum value of pressure and volume in different types of hydrocephalus was small. Furthermore, the difference between CSF stroke volumes in various types of hydrocephalus patients was less than 4.4%. Results showed that the intensity of clinical symptoms was similar in patients with similar CSF pressure and the cause of the hydrocephalus disease didn't have any significant effect on the intensity of patients' clinical symptoms and the manner of changes in effective parameters on disease. It was also found that the relation of CSF pressure and volume was 16.7% greater in patients with non-communicating hydrocephalus than in patients with communicating hydrocephalus. These results enhance the insight into hydrocephalus bio-mechanism and can help to choose the proper treatment method for hydrocephalus patients. Copyright © 2016 Elsevier Ltd. All rights reserved.

Cerebrospinal Fluid Amyloid-β 42, Total Tau and Phosphorylated Tau are Low in Patients with Normal Pressure Hydrocephalus: Analogies and Differences with Alzheimer's Disease.

PubMed

Santangelo, Roberto; Cecchetti, Giordano; Bernasconi, Maria Paola; Cardamone, Rosalinda; Barbieri, Alessandra; Pinto, Patrizia; Passerini, Gabriella; Scomazzoni, Francesco; Comi, Giancarlo; Magnani, Giuseppe

2017-01-01

Co-existence of Alzheimer's disease (AD) in normal pressure hydrocephalus (NPH) is a frequent finding, thus a common pathophysiological basis between AD and NPH has been postulated. We measured CSF amyloid-β 42 (Aβ42), total tau (t-tau), and phosphorylated tau (p-tau) concentrations in a sample of 294 patients with different types of dementia and 32 subjects without dementia. We then compared scores on neuropsychological tests of NPH patients with pathological and normal CSF Aβ42 values. Aβ42 levels were significantly lower in NPH than in control patients, with no significant differences between AD and NPH. On the contrary, t-tau and p-tau levels were significantly lower in NPH than in AD, with no differences between NPH and controls. NPH patients with pathological Aβ42 levels did not perform worse than NPH patients with normal Aβ42 levels in any cognitive domains. Our data seem to support the hypothesis of amyloid accumulation in brains of NPH patients. Nevertheless, amyloid does not seem to play a pathogenetic role in the development of cognitive deficits in NPH.

The predictive value of external continuous lumbar drainage, with cerebrospinal fluid outflow controlled by medium pressure valve, in normal pressure hydrocephalus.

PubMed

Panagiotopoulos, V; Konstantinou, D; Kalogeropoulos, A; Maraziotis, T

2005-09-01

Although sporadic studies have described temporary external cerebrospinal fluid (CSF) lumbar drainage as a highly accurate test for predicting the outcome after ventricular shunting in normal pressure hydrocephalus (NPH) patients, a more recent study reports that the positive predictive value of external lumbar drainage (ELD) is high but the negative predictive value is deceptively low. Therefore, we conducted a prospective study in order to evaluate the predictive value of a continuous ELD, with CSF outflow controlled by medium pressure valve, in NPH patients. Twenty-seven patients with presumed NPH were admitted to our department and CSF drainage was carried out by a temporary (ELD) with CSF outflow controlled by a medium pressure valve for five days. All patients received a ventriculoperitoneal shunt using a medium pressure valve based upon preoperative clinical and radiographic criteria of NPH, regardless of ELD outcome. Clinical evaluation of gait disturbances, urinary incontinence and mental status, and radiological evaluation with brain CT was performed prior to and after ELD test, as well as three months after shunting. Twenty-two patients were finally shunted and included in this study. In a three-month follow-up, using a previously validated score system, overall improvement after permanent shunting correlated well to improvement after ELD test (Spearman's rho = 0.462, p = 0.03). When considering any degree of improvement as a positive response, ELD test yielded high positive predictive values for all individual parameters (gait disturbances 94%, 95% CI 71%-100%, urinary incontinence 100%, 95% CI 66%-100%, and mental status 100%, 95% CI 66%-100%) but negative predictive values were low (< 50%) except for cognitive impairment (85%, 95% CI 55%-98%). This study suggests that a positive ELD-valve system test should be considered a reliable criterion for preoperative selection of shunt-responsive NPH patients. In case of a negative ELD-valve system test

The effect of shunt surgery on neuropsychological performance in normal pressure hydrocephalus: a systematic review and meta-analysis.

PubMed

Peterson, Katie A; Savulich, George; Jackson, Dan; Killikelly, Clare; Pickard, John D; Sahakian, Barbara J

2016-08-01

We conducted a systematic review of the literature and used meta-analytic techniques to evaluate the impact of shunt surgery on neuropsychological performance in patients with normal pressure hydrocephalus (NPH). Twenty-three studies with 1059 patients were identified for review using PubMed, Web of Science, Google scholar and manual searching. Inclusion criteria were prospective, within-subject investigations of cognitive outcome using neuropsychological assessment before and after shunt surgery in patients with NPH. There were statistically significant effects of shunt surgery on cognition (Mini-Mental State Examination; MMSE), learning and memory (Rey Auditory Verbal Learning Test; RAVLT, total and delayed subtests), executive function (backwards digit span, phonemic verbal fluency, trail making test B) and psychomotor speed (trail making test A) all in the direction of improvement following shunt surgery, but with considerable heterogeneity across all measures. A more detailed examination of the data suggested robust evidence for improved MMSE, RAVLT total, RAVLT delayed, phonemic verbal fluency and trail making test A only. Meta-regressions revealed no statistically significant effect of age, sex or follow-up interval on improvement in the MMSE. Our results suggest that shunt surgery is most sensitive for improving global cognition, learning and memory and psychomotor speed in patients with NPH.

Cerebrospinal fluid markers before and after shunting in patients with secondary and idiopathic normal pressure hydrocephalus

PubMed Central

Tullberg, Mats; Blennow, Kaj; Månsson, Jan-Eric; Fredman, Pam; Tisell, Magnus; Wikkelsö, Carsten

2008-01-01

Background The aim of this study was to explore biochemical changes in the cerebrospinal fluid (CSF) induced by shunt surgery and the relationship between these changes and clinical improvement. Methods We measured clinical symptoms and analysed lumbar CSF for protein content, neurodegeneration and neurotransmission markers in patients with secondary (SNPH, n = 17) and idiopathic NPH (INPH, n = 18) before and 3 months after shunt surgery. Patients were divided into groups according to whether or not there was improvement in clinical symptoms after surgery. Results Preoperatively, the only pathological findings were elevated neurofilament protein (NFL), significantly more so in the SNPH patients than in the INPH patients, and elevated albumin content. Higher levels of NFL correlated with worse gait, balance, wakefulness and neuropsychological performance. Preoperatively, no differences were seen in any of the CSF biomarkers between patients that improved after surgery and those that did not improve. Postoperatively, a greater improvement in gait and balance performance correlated with a more pronounced reduction in NFL. Levels of albumin, albumin ratio, neuropeptide Y, vasoactive intestinal peptide and ganglioside GD3 increased significantly after shunting in both groups. In addition, Gamma amino butyric acid increased significantly in SNPH and tau in INPH. Conclusion We conclude that a number of biochemical changes occur after shunt surgery, but there are no marked differences between the SNPH and INPH patients. The results indicate that NFL may be a marker that can predict a surgically reversible state in NPH. PMID:18439296

Numerical simulation of cerebrospinal fluid hydrodynamics in the healing process of hydrocephalus patients

NASA Astrophysics Data System (ADS)

Gholampour, S.; Fatouraee, N.; Seddighi, A. S.; Seddighi, A.

2017-05-01

Three-dimensional computational models of the cerebrospinal fluid (CSF) flow and brain tissue are presented for evaluation of their hydrodynamic conditions before and after shunting for seven patients with non-communicating hydrocephalus. One healthy subject is also modeled to compare deviated patients data to normal conditions. The fluid-solid interaction simulation shows the CSF mean pressure and pressure amplitude (the superior index for evaluation of non-communicating hydrocephalus) in patients at a greater point than those in the healthy subject by 5.3 and 2 times, respectively.

A proposed role for efflux transporters in the pathogenesis of hydrocephalus

PubMed Central

Krishnamurthy, Satish; Tichenor, Michael D.; Satish, Akhila G.; Lehmann, David B.

2014-01-01

Hydrocephalus is a common brain disorder that is treated only with surgery. The basis for surgical treatment rests on the circulation theory. However, clinical and experimental data to substantiate circulation theory have remained inconclusive. In brain tissue and in the ventricles, we see that osmotic gradients drive water diffusion in water-permeable tissue. As the osmolarity of ventricular CSF increases within the cerebral ventricles, water movement into the ventricles increases and causes hydrocephalus. Macromolecular clearance from the ventricles is a mechanism to establish the normal CSF osmolarity, and therefore ventricular volume. Efflux transporters, (p-glycoprotein), are located along the blood brain barrier and play an important role in the clearance of macromolecules (endobiotics and xenobiotics) from the brain to the blood. There is clinical and experimental data to show that macromolecules are cleared out of the brain in normal and hydrocephalic brains. This article summarizes the existing evidence to support the role of efflux transporters in the pathogenesis of hydrocephalus. The location of p-gp along the pathways of macromolecular clearance and the broad substrate specificity of this abundant transporter to a variety of different macromolecules are reviewed. Involvement of p-gp in the transport of amyloid beta in Alzheimer disease and its relation to normal pressure hydrocephalus is reviewed. Finally, individual variability of p-gp expression might explain the variability in the development of hydrocephalus following intraventricular hemorrhage. PMID:25165050

[Cognitive event-related brain potentials (P300) in patients with normal pressure hydrocephalus. Results of a prospective study].

PubMed

de la Calzada, M D; Poca, M A; Sahuquillo, J; Matarín, M; Mataró, M; Solana, E

2010-01-01

To determine the response to cognitive event-related potentials (P300) in patients with normal-pressure hydrocephalus (NPH) and their relationship with clinical and cognitive status before and after shunt surgery. We performed a prospective study in a series of 26 patients with NPH who underwent clinical and cognitive assessment before surgery and 6 months afterwards. Visual P300 potentials obtained before and after treatment were also compared with those obtained in 18 healthy volunteers. Before shunting, the P300 wave was detected in 11 (42.3%) NPH patients, compared with the 18 (100%) volunteers. Six months after shunting, the P300 wave was found in 20 (76.9%) NPH patients. P300 latency was significantly longer in NPH patients than in the control group before surgery, but not at 6 months after surgery. No significant differences in neuropsychological studies or in the level of dependence for daily life activities were found between the subgroups of NPH patients with and without pre-surgical P300 waves, or between changes in P300 parameters and clinical and cognitive changes. The P300 wave was delayed or undetectable in a substantial percentage of patients with NPH before surgery. These alterations can be reversed by shunting. P300 analysis and neuropsychological tests could be complementary measures to evaluate functional status in patients with NPH.

Frequency of Alzheimer's disease pathology at autopsy in patients with clinical normal pressure hydrocephalus.

PubMed

Cabral, Danielle; Beach, Thomas G; Vedders, Linda; Sue, Lucia I; Jacobson, Sandra; Myers, Kent; Sabbagh, Marwan N

2011-09-01

Normal pressure hydrocephalus (NPH) is considered to be potentially treatable with the placement of a cerebrospinal fluid (CSF) shunt. However, the procedure has been reported to have variable success, particularly with respect to improving the cognitive impairment in NPH. The presence of neurologic comorbidities, particularly Alzheimer's disease (AD), may contribute to shunt responsiveness. Uncovering the extent to which AD and NPH co-occur has implications for diagnosis and treatment of NPH. Autopsy studies of patients with NPH during their lifetime would elucidate the frequency of such comorbidities. A search of the Sun Health Research Institute Brain Donation Program database was conducted between January 1, 1997 and April 1, 2009 to identify all cases with neuropathologic evidence of dementia as well as those of clinically diagnosed NPH. We reviewed the medical records and brain findings of each NPH case. Of the 761 cases autopsied over the study interval, 563 were found to have neuropathologic evidence meeting criteria for a dementing illness. Of 563 cases, AD was found exclusively in 313 (56%), and 94 suffered from secondary diagnosis of dementia. Nine of 761 cases were identified with a clinical diagnosis of NPH, which were among the 563 cases with neuropathology of dementing illness at autopsy, representing 1.6% (9/563) of the cases. On review of brain autopsy reports of these nine patients, eight (89%) were found to have AD and one (11%) had progressive supranuclear palsy. Review of the medical records of the nine NPH cases revealed the following clinical comorbidities: five suffered from AD, one from Parkinson's Disease, one from mild cognitive impairment, and one from seizure disorder. Given the findings of the present study, we support the AD-NPH theory and posit that AD is a common pathologic comorbidity in the setting of NPH and may preclude cognitive improvement postshunt placement. This may influence the selection of cases for shunting in the

Frequency of Alzheimer's Disease Pathology at Autopsy in Patients with Clinical Normal Pressure Hydrocephalus

PubMed Central

Cabral, Danielle; Beach, Thomas G; Vedders, Linda; Sue, Lucia I; Jacobson, Sandra; Myers, Kent; Sabbagh, Marwan N

2011-01-01

Background Normal pressure hydrocephalus (NPH) is considered potentially treatable with the placement of a cerebrospinal fluid (CSF) shunt. Yet, the procedure has had variable success, particularly with respect to improving the cognitive impairment in NPH. The presence of neurologic co-morbidities, particularly Alzheimer's Disease (AD), may contribute to shunt responsiveness. Uncovering the extent to which AD and NPH co-occur has implications for diagnosis and treatment of NPH. Autopsy studies of patients with NPH during life would elucidate the frequency of such co-morbidities. Methods We conducted a search of the Sun Health Research Institute Brain Donation Program database between 1/1/1997 and 4/1/09 to identify all cases with neuropathologic evidence of dementia as well as those cases of clinically diagnosed NPH. We reviewed the medical records and brain findings of each NPH case. Results Of the 761 cases autopsied over the study interval, 563 cases were found to have neuropathological evidence meeting criteria for a dementing illness. AD was found exclusively in 313/563 (56%) cases with 94/563 cases having a secondary diagnosis of dementia. We identified 9/761 cases with a clinical diagnosis of NPH, all nine cases were among the 563 cases with neuropathology of dementing illness at autopsy, representing 1.6% (9/563). Upon review of brain autopsy reports, 8/9 (89%) cases were found to have AD and 1/9 (11%) had progressive supranuclear palsy. Review of the medical records of the nine NPH cases revealed the following clinical co-morbidities: 5/9 with AD; 1/9 with Parkinson's Disease (PD); 1/9 with Mild Cognitive Impairment (MCI); 1/9 with seizure disorder. Conclusions Given the findings of our study, we support the AD-NPH theory and posit that AD is a common pathological co-morbidity in the setting of NPH and may preclude cognitive improvement post-shunt placement. This may have influence on selection of cases for shunting in the future. PMID:21723206

Clinical assessment of cerebrospinal fluid dynamics in hydrocephalus. Guide to interpretation based on observational study.

PubMed

Weerakkody, R A; Czosnyka, M; Schuhmann, M U; Schmidt, E; Keong, N; Santarius, T; Pickard, J D; Czosnyka, Z

2011-08-01

The term hydrocephalus encompasses a range of disorders characterised by clinical symptoms, abnormal brain imaging and derangement of cerebrospinal fluid (CSF) dynamics. The ability to elucidate which patients would benefit from CSF diversion (a shunt or third ventriculostomy) is often unclear. Similar difficulties are often encountered in shunted patients to predict the scope for improvement by shunt re-adjustment or revision. In this study we aimed to update our knowledge of how key quantitative parameters describing CSF dynamics may be used in diagnosis of shunt-responsive hydrocephalus and in the assessment of shunt function. A number of quantitative parameters [including resistance to CSF outflow (Rcsf), pulse amplitude of intracranial pressure waveform (AMP), RAP index and slow vasogenic waves] were studies in 1423 patients with 2665 CSF infusion tests and 305 overnight intracranial pressure (ICP)-monitoring sessions over a 17 year period. We demonstrate our observations for typical values of Pb, Rcsf, AMP, slow vasogenic waves derived from infusion studies or overnight ICP monitoring in differentiating atrophy from shunt-responsive normal pressure hydrocephalus or acute hydrocephalus. From the same variables tested on shunted patients we demonstrate a standardised approach to help differentiate a properly-functioning shunt from underdrainage or overdrainage. Quantitative variables derived from CSF dynamics allow differentiation between clinically overlapping entities such as shunt-responsive normal pressure hydrocephalus and brain atrophy (not shunt responsive) as well as allowing the detection of shunt malfunction (partial or complete blockage) or overdrainage. This observational study is intended to serve as an update for our understanding of quantitative testing of CSF dynamics. © 2011 John Wiley & Sons A/S.

[Hydrocephalus in childhood : causes and imaging patterns].

PubMed

Pomschar, A; Koerte, I; Peraud, A; Heinen, F; Herber-Jonat, S; Reiser, M; Ertl-Wagner, B

2012-09-01

Causes and imaging patterns of hydrocephalus differ depending on the age of the patient. Traditionally, hydrocephalus was classified into non-communicating and communicating hydrocephalus but more recent classifications also take the site of occlusion and the etiology into account. For the diagnostic work-up computed tomography (CT), sonography and magnetic resonance imaging (MRI) are available and MRI is the method of choice for children and adolescents as it allows determination of the cause and location of a possible obstruction. In the first 12-18 months sonography allows evaluation of the lateral ventricles and the third ventricle and CT is usually only chosen in children in emergency situations and/or if no other modality is available. We retrospectively evaluated a population of 785 children and adolescents (426 males aged 0-17 years) referred for MRI between April 2009 and March 2012 due to headaches, somnolence, concentration difficulties or developmental delay. Among these 80 (49 male) met the MRI criteria for hydrocephalus, 75 (46 male) had non-communicating hydrocephalus and 5 (3 male) communicating hydrocephalus. Of the patients 24 (15 male) had posthemorrhagic aqueductal stenosis, 16 (8 male) intracranial tumors, 9 (6 male) Chiari II malformations, 5 (4 male) other congenital malformations including malformations of the Dandy Walker spectrum, 9 (3 male) idiopathic aqueductal stenosis, 7 (5 male) arachnoidal cysts and 10 (8 male) other disorders, such as post-infections, macrocephaly cutis marmorata telangiectatica congenita (M-CMTC) syndrome, mesencephalic arteriovenous malformation (AVM), Langerhans cell histiocystosis. It is important to take the age of the patient and the imaging pattern into account and to exclude tumors when reporting MR images of children with hydrocephalus.

Segmentation and labeling of the ventricular system in normal pressure hydrocephalus using patch-based tissue classification and multi-atlas labeling

NASA Astrophysics Data System (ADS)

Ellingsen, Lotta M.; Roy, Snehashis; Carass, Aaron; Blitz, Ari M.; Pham, Dzung L.; Prince, Jerry L.

2016-03-01

Normal pressure hydrocephalus (NPH) affects older adults and is thought to be caused by obstruction of the normal flow of cerebrospinal fluid (CSF). NPH typically presents with cognitive impairment, gait dysfunction, and urinary incontinence, and may account for more than five percent of all cases of dementia. Unlike most other causes of dementia, NPH can potentially be treated and the neurological dysfunction reversed by shunt surgery or endoscopic third ventriculostomy (ETV), which drain excess CSF. However, a major diagnostic challenge remains to robustly identify shunt-responsive NPH patients from patients with enlarged ventricles due to other neurodegenerative diseases. Currently, radiologists grade the severity of NPH by detailed examination and measurement of the ventricles based on stacks of 2D magnetic resonance images (MRIs). Here we propose a new method to automatically segment and label different compartments of the ventricles in NPH patients from MRIs. While this task has been achieved in healthy subjects, the ventricles in NPH are both enlarged and deformed, causing current algorithms to fail. Here we combine a patch-based tissue classification method with a registration-based multi-atlas labeling method to generate a novel algorithm that labels the lateral, third, and fourth ventricles in subjects with ventriculomegaly. The method is also applicable to other neurodegenerative diseases such as Alzheimer's disease; a condition considered in the differential diagnosis of NPH. Comparison with state of the art segmentation techniques demonstrate substantial improvements in labeling the enlarged ventricles, indicating that this strategy may be a viable option for the diagnosis and characterization of NPH.

[Predictive value of postural and dynamic walking parameters after high-volume lumbar puncture in normal pressure hydrocephalus].

PubMed

Mary, P; Gallisa, J-M; Laroque, S; Bedou, G; Maillard, A; Bousquet, C; Negre, C; Gaillard, N; Dutray, A; Fadat, B; Jurici, S; Olivier, N; Cisse, B; Sablot, D

2013-04-01

Normal pressure hydrocephalus (NPH) was described by Adams et al. (1965). The common clinical presentation is the triad: gait disturbance, cognitive decline and urinary incontinence. Although these symptoms are suggestive, they are not specific to diagnosis. The improvement of symptoms after high-volume lumbar puncture (hVLP) could be a strong criterion for diagnosis. We tried to determine a specific pattern of dynamic walking and posture parameters in NPH. Additionally, we tried to specify the evolution of these criteria after hVLP and to determine predictive values of ventriculoperitoneal shunting (VPS) efficiency. Sixty-four patients were followed during seven years from January 2002 to June 2009. We identified three periods: before (S1), after hVLP (S2) and after VPS (S3). The following criteria concerned walking and posture parameters: walking parameters were speed, step length and step rhythm; posture parameters were statokinesigram total length and surface, length according to the surface (LFS), average value of equilibration for lateral movements (Xmoyen), anteroposterior movements (Ymoyen), total movement length in lateral axis (longX) and anteroposterior axis (longY). Among the 64 patients included, 22 had VPS and 16 were investigated in S3. All kinematic criteria are decreased in S1 compared with normal values. hVLP improved these criteria significantly (S2). Among posture parameters, only total length and surface of statokinesigram showed improvement in S1, but no improvement in S2. A gain in speed greater or equal to 0.15m/s between S1 and S2 predicted the efficacy of VPS with a positive predictive value (PPV) of 87.1% and a negative predictive value (NPV) of 69.7% (area under the ROC curve [AUC]: 0.86). Kinematic walking parameters are the most disruptive and are partially improved after hVLP. These parameters could be an interesting test for selecting candidates for VPS. These data have to be confirmed in a larger cohort. Copyright © 2013 Elsevier

Alterations of pulsation absorber characteristics in experimental hydrocephalus.

PubMed

Park, Eun-Hyoung; Dombrowski, Stephen; Luciano, Mark; Zurakowski, David; Madsen, Joseph R

2010-08-01

Analysis of waveform data in previous studies suggests that the pulsatile movement of CSF may play a role in attenuating strong arterial pulsations entering the cranium, and its effectiveness in attenuating these pulsations may be altered by changes in intracranial pressure (ICP). These findings were obtained in studies performed in canines with normal anatomy of the CSF spaces. How then would pulsation absorbance respond to changes in CSF movement under obstructive conditions such as the development of hydrocephalus? In the present study, chronic obstructive hydrocephalus was induced by the injection of cyanoacrylate gel into the fourth ventricle of canines, and pulsation absorbance was compared before and after hydrocephalus induction. Five animals were evaluated with simultaneous recordings of ICP and arterial blood pressure (ABP) before and at 4 and 12 weeks after fourth ventricle obstruction by cyanoacrylate. To assess how the intracranial system responds to the arterial pulsatile component, ABP and ICP waveforms recorded in a time domain had to be analyzed in a frequency domain. In an earlier study the authors introduced a particular technique that allows characterization of the intracranial system in the frequency domain with sufficient accuracy and efficiency. This same method was used to analyze the relationship between ABP and ICP waveforms recorded during several acute states including hyperventilation as well as CSF withdrawal and infusion under conditions before and after inducing chronic obstructive hydrocephalus. Such a relationship is reflected in terms of a gain, which is a function of frequency. The cardiac pulsation absorbance (CPA) index, which is simply derived from a gain evaluated at the cardiac frequency, was used to quantitatively evaluate the changes in pulsation absorber function associated with the development of hydrocephalus within each of the animals, which did become hydrocephalic. To account for normal and hydrocephalic conditions

Three-year outcome of shunted idiopathic NPH patients.

PubMed

Aygok, G; Marmarou, A; Young, H F

2005-01-01

The incidence of idiopathic normal pressure hydrocephalus (iNPH) has increased as a result of improved longevity. This report describes the 3-year outcome of shunted iNPH patients compared to three-month outcome after shunting. Patients (n = 50) (Age 70.4 +/- 8.9) admitted to our service were diagnosed and treated according to a fixed protocol for management of iNPH and after shunting were followed at least three times per year in clinic. The outcome of 50 patients was graded according to the level of improvement in symptoms as Excellent/Good, Partial or None in each category of Gait, Incontinence and Dementia. If we lump favorable (excellent, good, partial recovery) vs poor recovery (none), we found from 3 months to 3 years, a moderate decline in gait performance (91% to 75%), a retention of memory improvement (80%-80%) and an improvement in incontinence occurred over time (70%-82.5%). With proper diagnosis and management of iNPH, shunting of patients is associated with a favorable risk/benefit ratio that is reasonably long lasting.

Quantifying response to intracranial pressure normalization in idiopathic intracranial hypertension via dynamic neuroimaging.

PubMed

Lublinsky, Svetlana; Kesler, Anat; Friedman, Alon; Horev, Anat; Shelef, Ilan

2018-04-01

Idiopathic intracranial hypertension (IIH) is characterized by elevated intracranial pressure without a clear cause. To investigate dynamic imaging findings in IIH and their relation to mechanisms underlying intracranial pressure normalization. Prospective. Eighteen IIH patients and 30 healthy controls. T 1 -weighted, venography, fluid attenuation inversion recovery, and apparent diffusion coefficients were acquired on 1.5T scanner. The dural sinus was measured before and after lumbar puncture (LP). The degree of sinus occlusion was evaluated, based on 95% confidence intervals of controls. We studied a number of neuroimaging biomarkers associated with IIH (sinus occlusion; optic nerve; distribution of cerebrospinal fluid into the subarachnoid space, sulci and lateral ventricles (LVs); Meckel's caves; arachnoid granulation; pituitary and choroid plexus), before and after LP, using a set of specially developed quantification techniques. Relationships among various biomarkers were investigated (Pearson correlation coefficient) and linked to long-term disease outcomes (logistic regression). The t-test and the Wilcoxon rank test were used to compare between controls and before and after LP data. As a result of LP, the following were found to be in good accordance with the opening pressure: relative compression of cerebrospinal fluid (R = -0.857, P < 0.001) and brain volumes (R = -0.576, P = 0.012), LV expansion (R = 0.772, P < 0.001) and venous volume (R = 0.696, P = 0.001), enlargement of the pituitary (R = 0.640, P = 0.023), and shrinkage of subarachnoid space (R = -0.887, P < 0.001). The only parameter that had an impact on long-term prognosis was cross-sectional size of supplemental drainage veins after LP (sensitivity of 92%, specificity of 20%, and area under the curve of 0.845, P < 0.001). We present an approach for quantitative characterization of the intracranial venous system and its implementation as a diagnostic assistance

Single pulse analysis of intracranial pressure for a hydrocephalus implant.

PubMed

Elixmann, I M; Hansinger, J; Goffin, C; Antes, S; Radermacher, K; Leonhardt, S

2012-01-01

The intracranial pressure (ICP) waveform contains important diagnostic information. Changes in ICP are associated with changes of the pulse waveform. This change has explicitly been observed in 13 infusion tests by analyzing 100 Hz ICP data. An algorithm is proposed which automatically extracts the pulse waves and categorizes them into predefined patterns. A developed algorithm determined 88 %±8 % (mean ±SD) of all classified pulse waves correctly on predefined patterns. This algorithm has low computational cost and is independent of a pressure drift in the sensor by using only the relationship between special waveform characteristics. Hence, it could be implemented on a microcontroller of a future electromechanic hydrocephalus shunt system to control the drainage of cerebrospinal fluid (CSF).

Epidemiology of adult-onset hydrocephalus: institutional experience with 2001 patients.

PubMed

Bir, Shyamal C; Patra, Devi Prasad; Maiti, Tanmoy K; Sun, Hai; Guthikonda, Bharat; Notarianni, Christina; Nanda, Anil

2016-09-01

OBJECTIVE Adult-onset hydrocephalus is not commonly discussed in the literature, especially regarding its demographic distribution. In contrast to pediatric hydrocephalus, which is related to a primary CSF pathway defect, its development in adults is often secondary to other pathologies. In this study, the authors investigated the epidemiology of adult-onset hydrocephalus as it pertains to different etiologies and in reference to age, sex, and race distributions. METHODS The authors retrospectively reviewed the clinical notes of 2001 patients with adult-onset hydrocephalus who presented to Louisiana State University Health Sciences Center within a 25-year span. Significant differences between the groups were analyzed by a chi-square test; p < 0.05 was considered significant. RESULTS The overall mean (± SEM) incidence of adult hydrocephalus in this population was 77 ± 30 per year, with a significant increase in incidence in the past decade (55 ± 3 [1990-2003] vs 102 ± 6 [2004-2015]; p < 0.0001). Hydrocephalus in a majority of the patients had a vascular etiology (45.5%) or was a result of a tumor (30.2%). The incidence of hydrocephalus in different age groups varied according to various pathologies. The incidence was significantly higher in males with normal-pressure hydrocephalus (p = 0.03) or head injury (p = 0.01) and higher in females with pseudotumor cerebri (p < 0.0001). In addition, the overall incidence of hydrocephalus was significantly higher in Caucasian patients (p = 0.0002) than in those of any other race. CONCLUSIONS Knowledge of the demographic variations in adult-onset hydrocephalus is helpful in achieving better risk stratification and better managing the disease in patients. For general applicability, these results should be validated in a large-scale meta-analysis based on a national population database.

Hydrocephalus as a rare compilation of vertebrobasilar dolichoectasia: A case report and review of the literature.

PubMed

Ebrahimzadeh, Keveh; Bakhtevari, Mehrdad H; Shafizad, Misagh; Rezaei, Omidvar

2017-01-01

Vertebrobasilar dolichoectasia (VBD) is a rare disease characterized by significant expansion, elongation, and tortuosity of the vertebrobasilar arteries. Hydrocephalus is a rare compilation of VBD. In this study, we report a 68-year-old male presenting with headache, progressive decreased visual acuity, memory loss, imbalance while walking, and episodes of urinary incontinency. The patient was diagnosed with dolichoectasia of basilar artery causing compression of the third ventricular outflow and thus, presenting with hydrocephalus documented with brain computed tomography scan and brain magnetic resonance imaging. The patient underwent surgical operation and ventriculoperitoneal shunt placement. In the case of hydrocephalus or normal pressure hydrocephalous, VBD should be considered as a differential diagnosis.

Control volume based hydrocephalus research; a phantom study

NASA Astrophysics Data System (ADS)

Cohen, Benjamin; Voorhees, Abram; Madsen, Joseph; Wei, Timothy

2009-11-01

Hydrocephalus is a complex spectrum of neurophysiological disorders involving perturbation of the intracranial contents; primarily increased intraventricular cerebrospinal fluid (CSF) volume and intracranial pressure are observed. CSF dynamics are highly coupled to the cerebral blood flows and pressures as well as the mechanical properties of the brain. Hydrocephalus, as such, is a very complex biological problem. We propose integral control volume analysis as a method of tracking these important interactions using mass and momentum conservation principles. As a first step in applying this methodology in humans, an in vitro phantom is used as a simplified model of the intracranial space. The phantom's design consists of a rigid container filled with a compressible gel. Within the gel a hollow spherical cavity represents the ventricular system and a cylindrical passage represents the spinal canal. A computer controlled piston pump supplies sinusoidal volume fluctuations into and out of the flow phantom. MRI is used to measure fluid velocity and volume change as functions of time. Independent pressure measurements and momentum flow rate measurements are used to calibrate the MRI data. These data are used as a framework for future work with live patients and normal individuals. Flow and pressure measurements on the flow phantom will be presented through the control volume framework.

In vitro flow measurements in ion sputtered hydrocephalus shunts

NASA Technical Reports Server (NTRS)

Cho, Y. I.; Back, L. H.

1989-01-01

This paper describes an experimental procedure for accurate measurements of the pressure-drop/flow rate relationship in hydrocephalus shunts. Using a fish-hook arrangement, small flow rates in a perforated ion-sputtered Teflon microtubule were measured in vitro in a pressured system and were correlated with pressure in the system. Results indicate that appropriate drainage rates could be obtained in the physiological range for hydrocephalus shunts.

Neuropsychological Assessment of Adult Patients with Shunted Hydrocephalus

PubMed Central

Bakar, Emel Erdogan

2010-01-01

Objective This study is planned to determine the neurocognitive difficulties of hydrocephalic adults. Methods The research group contained healthy adults (control group, n : 15), and hydrocephalic adults (n : 15). Hydrocephalic group consisted of patients with idiopathic aquaduct stenosis and post-meningitis hydrocephalus. All patients were followed with shunted hydrocephalus and not gone to shunt revision during last two years. They were chosen from either asymptomatic or had only minor symptoms without motor and sensorineural deficit. A neuropsychological test battery (Raven Standart Progressive Matrices, Bender-Gestalt Test, Cancellation Test, Clock Drawing Test, Facial Recognition Test, Line Orientation Test, Serial Digit Learning Test, Stroop Color Word Interference Test-TBAG Form, Verbal Fluency Test, Verbal Fluency Test, Visual-Aural Digit Span Test-B) was applied to all groups. Results Neuropsychological assessment of hydrocephalic patients demonstrated that they had poor performance on visual, semantic and working memory, visuoconstructive and frontal functions, reading, attention, motor coordination and executive function of parietal lobe which related with complex and perseverative behaviour. Eventually, these patients had significant impairment on the neurocognitive functions of their frontal, parietal and temporal lobes. On the other hand, the statistical analyses performed on demographic data showed that the aetiology of the hydrocephalus, age, sex and localization of the shunt (frontal or posterior parietal) did not affect the test results. Conclusion This prospective study showed that adult patients with hydrocephalus have serious neuropsychological problems which might be directly caused by the hydrocephalus; and these problems may cause serious adaptive difficulties in their social, cultural, behavioral and academic life. PMID:20379471

Hydrocephalus as a rare compilation of vertebrobasilar dolichoectasia: A case report and review of the literature

PubMed Central

Ebrahimzadeh, Keveh; Bakhtevari, Mehrdad H.; Shafizad, Misagh; Rezaei, Omidvar

2017-01-01

Background: Vertebrobasilar dolichoectasia (VBD) is a rare disease characterized by significant expansion, elongation, and tortuosity of the vertebrobasilar arteries. Hydrocephalus is a rare compilation of VBD. Case Description: In this study, we report a 68-year-old male presenting with headache, progressive decreased visual acuity, memory loss, imbalance while walking, and episodes of urinary incontinency. The patient was diagnosed with dolichoectasia of basilar artery causing compression of the third ventricular outflow and thus, presenting with hydrocephalus documented with brain computed tomography scan and brain magnetic resonance imaging. The patient underwent surgical operation and ventriculoperitoneal shunt placement. Conclusion: In the case of hydrocephalus or normal pressure hydrocephalous, VBD should be considered as a differential diagnosis. PMID:29184707

Cerebral blood flow velocity and vasomotor reactivity before and after shunting surgery in patients with normal pressure hydrocephalus.

PubMed

Lee, E J; Hung, Y C; Chang, C H; Pai, M C; Chen, H H

1998-01-01

The purpose of this study was to evaluate pre- and post-shunting haemodynamic changes and their correlation with the clinical results in normal pressure hydrocephalus (NPH). Accordingly, eleven demented patients with clinical signs suggestive of NPH received examinations of cerebral blood flow velocity (BFV) and vasomotor reactivity (VMR) by transcranial Doppler sonography with carbogen testing before and after shunt treatment. Computerized tomography (CT), clinical assessment and neuropsychological grading were performed prior to and at 3 months following surgery. A control group consisting of 10 patients was included to establish baseline data. The pre-operative CBF studies in the anterior cerebral artery (ACA) and the middle cerebral artery (MCA) revealed the NPH patients did not have significant decreases of BFVs, but had significant decreases of carbogen VMR (P < 0.05). After shunting, there were no significant changes of the BFVs as compared with the pre-shunting data. The post-shunting VMR of the ACA was significantly higher than the pre-shunting one (p < 0.05), but there was no variation in that of the MCA. Both the values of post-shunting VMR in ACA and the post-shunting increase in VMR in MCA of the 7 shunt-responsive patients who improved mentally and in other symptoms were significantly higher than those of patients without improvement (p < 0.05). In addition, the five patients with gait improvement showed significantly higher values of post-shunting VMR of ACA and the post-shunting increase of VMR for both ACA and MCA when compared with those patients without gait improvement (p < 0.05, respectively). Our study supports the view that patients with NPH had various degrees of impaired VMR in both the ACA and the MCA, but showed insignificant reduction in BFVs, indicating a compensatory mechanism of CBF over time to accommodate the subnormal state of cerebral perfusion pressure. Shunt placement would improve the VMR in responsive patients. Postoperatively

Magnetic Resonance Elastography Demonstrating Low Brain Stiffness in a Patient with Low-Pressure Hydrocephalus: Case Report.

PubMed

Olivero, William C; Wszalek, Tracey; Wang, Huan; Farahvar, Arash; Rieth, Sandra M; Johnson, Curtis L

2016-01-01

The authors describe the case of a 19-year-old female with shunted aqueductal stenosis who presented with low-pressure hydrocephalus that responded to negative pressure drainage. A magnetic resonance elastography scan performed 3 weeks later demonstrated very low brain tissue stiffness (high brain tissue compliance). An analysis of the importance of this finding in understanding this rare condition is discussed. © 2016 S. Karger AG, Basel.

A Mechanics-Based Framework Leading to Improved Diagnosis and Treatment of Hydrocephalus

NASA Astrophysics Data System (ADS)

Cohen, Benjamin; Soren, Vedels; Wagshul, Mark; Egnor, Michael; Voorhees, Abram; Wei, Timothy

2007-11-01

Hydrocephalus is defined as an accumulation of cerebrospinal fluid (CSF) in the cranium, at the expense of brain tissue. The result is a disruption of the normal pressure and/or flow dynamics of the intracranial blood and CSF. We seek to introduce integral control volume analysis to the study of hydrocephalus. The goal is to provide a first principles framework to integrate a broad spectrum of sometimes disparate investigations into a highly complex, multidisciplinary problem. The general technique for the implementation of control volumes to hydrocephalus will be presented. This includes factors faced in choosing control volumes and making the required measurements to evaluate mass and momentum conservation. In addition, the use of our Digital Particle Image Velocimetry (DPIV) processing program has been extended to measure the displacement of the ventricles' walls from Magnetic Resonance (MR) images. This is done to determine the volume change of the intracranial fluid spaces.

A comparison between the pathophysiology of multiple sclerosis and normal pressure hydrocephalus: is pulse wave encephalopathy a component of MS?

PubMed

Bateman, Grant A; Lechner-Scott, Jeannette; Lea, Rodney A

2016-09-22

It has been suggested there is a chronic neurodegenerative disorder, underlying the pathophysiology of multiple sclerosis (MS), which is distinct from the more obvious immune-mediated attack on the white matter. Limited data exists indicating there is an alteration in pulse wave propagation within the craniospinal cavity in MS, similar to the findings in normal pressure hydrocephalus (NPH). It is hypothesized MS may harbor pulse wave encephalopathy. The purpose of this study is to compare blood flow and pulse wave measurements in MS patients with a cohort of NPH patients and control subjects, to test this hypothesis. Twenty patients with MS underwent magnetic resonance (MR) flow quantification techniques. Mean blood flow and stroke volume were measured in the arterial inflow and venous out flow from the sagittal (SSS) and straight sinus (ST). The arteriovenous delay (AVD) was defined. The results were compared with both age-matched controls and NPH patients. In MS there was a 35 % reduction in arteriovenous delay and a 5 % reduction in the percentage of the arterial inflow returning via the sagittal sinus compared to age matched controls. There was an alteration in pulse wave propagation, with a 26 % increase in arterial stroke volume but 30 % reduction in SSS and ST stroke volume. The AVD and blood flow changes were in the same direction to those of NPH patients. There are blood flow and pulsation propagation changes in MS patients which are similar to those of NPH patients. The findings would be consistent with an underlying pulse wave encephalopathy component in MS.

Repeatable Battery for the Assessment of Neuropsychological Status (RBANS) as a Cognitive Evaluation Tool for Patients with Normal Pressure Hydrocephalus.

PubMed

Nakatsu, Daisuke; Fukuhara, Toru; Chaytor, Naomi S; Phatak, Vaishali S; Avellino, Anthony M

2016-01-01

External lumbar drainage (ELD) is recognized as a screening method for ventriculo-peritoneal shunting (VPS) candidacy for possible normal pressure hydrocephalus (NPH). This study focused on the ELD predictability of the cognitive outcome after VPS for NPH. In addition, Repeatable Battery for the Assessment of Neuropsychological Status (RBANS) was examined in ELD cognition screening. ELD results were considered positive with any improvement in gait and/or cognition. Among 36 patients examined for possible NPH, 26 underwent VPS because of positive ELD. Cognitive outcome after VPS was assessed at 6-month follow-up. The RBANS scores, examined pre- and post-ELD, were evaluated statistically to identify consistency with the neuropsychologist judgment and the predictability of cognitive outcome after VPS. Among 26 shunted patients, gait was improved in 24. Cognitive improvement was rated in 19, and there were 9 false negative and 5 false positive in ELD cognition screening. The neuropsychologist judgment in ELD cognition screening is most consistent with the RBANS score in delayed memory. The patients rated as improved in cognition after VPS had significantly lower RBANS scores pre-ELD in immediate memory and delayed memory. If both scores at pre-ELD were ≤ 80 (13 patients), all were rated as improved in cognition after VPS. ELD screening was highly predictive of clinical gait improvement but not of cognitive improvement after VPS for possible NPH. Particularly among patients with a positive ELD gait response, pre-ELD low RBANS scores in memory predicted cognitive improvement after VPS. RBANS seems effective in evaluating cognition for NPH.

Repeatable Battery for the Assessment of Neuropsychological Status (RBANS) as a Cognitive Evaluation Tool for Patients with Normal Pressure Hydrocephalus

PubMed Central

NAKATSU, Daisuke; FUKUHARA, Toru; CHAYTOR, Naomi S.; PHATAK, Vaishali S.; AVELLINO, Anthony M.

2016-01-01

External lumbar drainage (ELD) is recognized as a screening method for ventriculo-peritoneal shunting (VPS) candidacy for possible normal pressure hydrocephalus (NPH). This study focused on the ELD predictability of the cognitive outcome after VPS for NPH. In addition, Repeatable Battery for the Assessment of Neuropsychological Status (RBANS) was examined in ELD cognition screening. ELD results were considered positive with any improvement in gait and/or cognition. Among 36 patients examined for possible NPH, 26 underwent VPS because of positive ELD. Cognitive outcome after VPS was assessed at 6-month follow-up. The RBANS scores, examined pre- and post-ELD, were evaluated statistically to identify consistency with the neuropsychologist judgment and the predictability of cognitive outcome after VPS. Among 26 shunted patients, gait was improved in 24. Cognitive improvement was rated in 19, and there were 9 false negative and 5 false positive in ELD cognition screening. The neuropsychologist judgment in ELD cognition screening is most consistent with the RBANS score in delayed memory. The patients rated as improved in cognition after VPS had significantly lower RBANS scores pre-ELD in immediate memory and delayed memory. If both scores at pre-ELD were ≤ 80 (13 patients), all were rated as improved in cognition after VPS. ELD screening was highly predictive of clinical gait improvement but not of cognitive improvement after VPS for possible NPH. Particularly among patients with a positive ELD gait response, pre-ELD low RBANS scores in memory predicted cognitive improvement after VPS. RBANS seems effective in evaluating cognition for NPH. PMID:26369720

A Girl with Idiopathic Epilepsy Showing Forced Normalization after Levetiracetam Administration.

PubMed

Kawakami, Yasuhiko; Okazaki, Tetsuya; Takase, Masato; Fujino, Osamu; Itoh, Yasuhiko

2015-01-01

Forced normalization has been reported in association with almost all anti-epileptic drugs. We report on a 9-year-old girl with idiopathic epilepsy who showed forced normalization after administration of levetiracetam (LEV). She initially presented with generalized tonic-clonic seizures when she was 4 years old. Diffuse sharp and slow wave complexes (SWCs) were observed on electroencephalography (EEG). We prescribed sodium valproate (VPA) and benzodiazepines, but the seizures and EEG findings worsened gradually. Although subsequent administration of LEV stopped the seizures, the patient became subject to episodes of rage and violent behavior. Forced normalization was confirmed by the disappearance of SWCs on EEG. We reduced the dose of LEV and tried in various ways to resolve the situation, but finally we had to abandon LEV. To the best of our knowledge, this is the first report of a patient with idiopathic epilepsy but without disabilities in everyday life showing forced normalization associated with LEV administration.

Acute infarction of corpus callosum due to transient obstructive hydrocephalus.

PubMed

Kaymakamzade, Bahar; Eker, Amber

2016-01-01

Acute ischemia of the corpus callosum (CC) is not a well-known feature in patients with acute hydrocephalus. Herein, we describe a case with acute CC infarction due to another rare entity; transient obstructive hydrocephalus. A 66-year-old male was admitted with sudden onset right-sided hemiparesia. CT demonstrated a hematoma on the left basal ganglia with extension to all ventricles. The following day, the patient's neurological status progressed to coma and developed bilateral pyramidal signs. MRI demonstrated obstructive hydrocephalus and acute diffuse infarction accompanied by elevation of the CC. On the same day there was improvement in his neurological status with significant decrease in ventricular size and complete resolution of the clot in the third ventricle. The mechanism of signal abnormalities is probably related with the neural compression of the CC against the falx. Presumably, the clot causing obstruction in the third ventricle dissolved or decayed by the help of fibrinolytic activity of CSF, which was raised after IVH and caused spontaneous improvement of hydrocephalus. Bilateral neurological symptoms suggest diffuse axonal damage and normalization of the intracranial pressure should be performed on the early onset of clinical detorioration in order to prevent axonal injury. Copyright © 2016 Polish Neurological Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.

[Hydrocephalus Associated with Small Clinoidal Meningioma that Resolved after Tumor Removal:A Case Report].

PubMed

Fujiwara, Hidemoto; Aiba, Toyotaka; Watanabe, Toru; Hiraishi, Tetsuya; Fujii, Yukihiko

2016-12-01

Small meningiomas causing hydrocephalus without obstruction of the ventricular system are rare. Herein, we report a case of small clinoidal meningioma with communicating hydrocephalus, which resolved after tumor removal. A 70-year-old woman presented with a 1-month history of memory disturbance followed by gait disturbance. MR images revealed a right clinoidal meningioma, 2 cm in diameter, and dilatation of the ventricles suggesting communicating hydrocephalus. The cerebrospinal fluid(CSF)pressure was 130 mmH₂O, as determined via a lumbar puncture. High concentrations of protein(65mg/dL)were detected in the lumbar CSF. The tumor was completely removed via a frontotemporal craniotomy. Higher protein concentrations(94mg/dL)were detected in the CSF obtained intraoperatively from the sylvian cistern. The histopathological diagnosis was meningothelial meningioma. The patient's symptoms improved markedly after surgery. Postoperative MR images revealed resolution of the hydrocephalus. The lumbar CSF protein concentration returned to normal(43mg/dL). Neither tumor recurrence nor progression of hydrocephalus has been observed for 4 years. Communicating hydrocephalus, associated with a small meningioma at the supratentorial region, has not been described. Previous studies have shown that patients with meningioma may develop communicating hydrocephalus after tumor removal or stereotactic radiosurgery. Thus, it is interesting that the small supratentorial meningioma in our case developed communicating hydrocephalus without any therapeutic intervention. Considering the CSF protein concentration, we speculate that the hydrocephalus was the result of CSF malabsorption associated with high CSF protein concentration and CSF pathway obstruction at the suprasellar cistern caused by the tumor.

Quantitative assessment of cerebrospinal fluid hydrodynamics using a phase-contrast cine MR image in hydrocephalus.

PubMed

Kim, D S; Choi, J U; Huh, R; Yun, P H; Kim, D I

1999-09-01

This investigation was undertaken to characterize CSF flow at the level of the aqueduct of Sylvius with a phase-contrast cine MR pulse sequence in 28 healthy volunteers. Sixteen patients with obstructive hydrocephalus and 11 patients with normal pressure hydrocephalus (NPH) were investigated with the same sequence before and after CSF diversion. The peak CSF flow velocity and stroke volume in the aqueduct increased significantly in the NPH group and decreased significantly in the obstructive hydrocephalus group. After lumboperitoneal shunting in the NPH group, the retrograde flow of CSF was anterogradely converted and the peak flow velocities decreased somewhat. The clinical diagnosis of NPH was well correlated with the results of cine MRI. After endoscopic III ventriculostomy in the obstructive hydrocephalus group we noted increased CSF flow velocity with markedly increased stroke volume at the prepontine cistern. Phase-contrast cine MR is useful in evaluating CSF dynamics in patients with hyperdynamic aqueductal CSF or aqueductal obstruction.

Normal Pressure Hydrocephalus

MedlinePlus

... NINDS Focus on Disorders Alzheimer's & Related Dementias Epilepsy Parkinson's Disease Spinal Cord Injury Traumatic Brain Injury Focus On ... those of other disorders such as Alzheimer's disease, Parkinson's disease, and Creutzfeldt-Jakob disease, the disorder is often ...

Low-Pressure Hydrocephalus: A Case Report and Review of the Literature.

PubMed

Strand, Adam; Balise, Stephen; Leung, Lawrence Jun; Durham, Susan

2018-01-01

The entity of low-pressure hydrocephalus remains poorly understood and thoroughly debated. Symptomatic improvement accompanied by decrease in ventricular size after prolonged subatmospheric drainage has been well documented, and this method has been considered the criterion standard of management. Few studies have examined alternative treatment options, either to avoid the risks associated with prolonged external ventricular drainage or because of the failure of traditional methods. This study compiled and examined reported cases of low-pressure hydrocephalus in an attempt to provide an up-to-date summary of the condition. A literature search was conducted by use of Ovid Medline and PubMed filtered for the past 25 years with specific key terms, inclusion criteria, and exclusion criteria. Selected case studies and case series were then compared, and statistical analysis was performed where appropriate. Over 25 years, 17 articles met our criteria. In addition to our case, 90 cases of LPH were reported. Magnetic resonance elastography (MRE) has proved to be an effective means of studying the viscoelastic properties of the brain. Endoscopic third ventriculostomy (ETV) appears to be a strong alternative, or additional, treatment. MRE may prove to be effective in studying LPH because of its ability to quantify viscoelastic properties in response to therapy. Additionally, ETV should be considered in cases of LPH, although there is little evidence in the current literature to support its use. There are suggestions that it may lead to fewer shunt-dependent patients. Future studies are needed because there are few documented examples. Copyright © 2017 Elsevier Inc. All rights reserved.

[Normal lung volumes in patients with idiopathic pulmonary fibrosis and emphysema].

PubMed

Casas, Juan Pablo; Abbona, Horacio; Robles, Adriana; López, Ana María

2008-01-01

Pulmonary function tests in idiopathic pulmonary fibrosis characteristically show a restrictive pattern, resulting from reduction of pulmonary compliance due to diffuse fibrosis. Conversely, an obstructive pattern with hyperinflation results in emphysema by loss of elastic recoil, expiratory collapse of the peripheral airways and air trapping. Previous reports suggest that when both diseases coexist, pulmonary volumes are compensated and a smaller than expected reduction or even normal lung volumes can be found. We report 4 male patients of 64, 60, 73 and 70 years, all with heavy cigarette smoking history and progressive breathlessness. Three of them had severe limitation in their quality of life. All four showed advanced lung interstitial involvement, at high resolution CT scan, fibrotic changes predominantly in the subpleural areas of lower lung fields and concomitant emphysema in the upper lobes. Emphysema and pulmonary fibrosis was confirmed by open lung biopsy in one patient. The four patients showed normal spirometry and lung volumes with severe compromise of gas exchange and poor exercise tolerance evaluated by 6 minute walk test. Severe pulmonary arterial hypertension was also confirmed in three patients. Normal lung volumes does not exclude diagnosis of idiopathic pulmonary fibrosis in patients with concomitant emphysema. The relatively preserved lung volumes may underestimate the severity of idiopathic pulmonary fibrosis and attenuate its effects on lung function parameters.

Magnetic resonance spectroscopic determination of a neuronal and axonal marker in white matter predicts reversibility of deficits in secondary normal pressure hydrocephalus

PubMed Central

Shiino, A; Nishida, Y; Yasuda, H; Suzuki, M; Matsuda, M; Inubushi, T

2004-01-01

Background: Normal pressure hydrocephalus (NPH) is considered to be a treatable form of dementia, because cerebrospinal fluid (CSF) shunting can lessen symptoms. However, neuroimaging has failed to predict when shunting will be effective. Objective: To investigate whether 1H (proton) magnetic resonance (MR) spectroscopy could predict functional outcome in patients after shunting. Methods: Neurological state including Hasegawa's dementia scale, gait, continence, and the modified Rankin scale were evaluated in 21 patients with secondary NPH who underwent ventriculo-peritoneal shunting. Outcomes were measured postoperatively at one and 12 months and were classified as excellent, fair, or poor. MR spectra were obtained from left hemispheric white matter. Results: Significant preoperative differences in N-acetyl aspartate (NAA)/creatine (Cr) and NAA/choline (Cho) were noted between patients with excellent and poor outcome at one month (p = 0.0014 and 0.0036, respectively). Multiple regression analysis linked higher preoperative NAA/Cr ratio, gait score, and modified Rankin scale to better one month outcome. Predictive value, sensitivity, and specificity for excellent outcome following shunting were 95.2%, 100%, and 87.5%. Multiple regression analysis indicated that NAA/Cho had the best predictive value for one year outcome (p = 0.0032); predictive value, sensitivity, and specificity were 89.5%, 90.0%, and 88.9%. Conclusions: MR spectroscopy predicted long term post-shunting outcomes in patients with secondary NPH, and it would be a useful assessment tool before lumbar drainage. PMID:15258216

Diffusion tensor imaging detects early brain microstructure changes before and after ventriculoperitoneal shunt in children with high intracranial pressure hydrocephalus

PubMed Central

Zhao, Cailei; Li, Yongxin; Cao, Weiguo; Xiang, Kui; Zhang, Heye; Yang, Jian; Gan, Yungen

2016-01-01

Abstract To explore the use of diffusion tensor imaging (DTI) parameters in the quantitative assessment of early brain microstructure changes before and after ventriculoperitoneal shunt in children with high intracranial pressure hydrocephalus. Ten patients with communicating hydrocephalus (age: 2–36 months) and 14 age-/gender-matched controls (age: 2–36 months) were enrolled in this study. All patients underwent the ventriculoperitoneal shunt procedure. The imaging data were collected before and 3 months after the operation. Regions of interests (ROIs) included the white matter near the frontal horn of the lateral ventricles (FHLV), the occipital horn of the lateral ventricles (OHLV), occipital subcortical (OS) area, frontal subcortical (FS) area, and thalamus. Fractional anisotropies (FA) and apparent diffusion coefficients (ADC) of the ROIs before and after ventriculoperitoneal shunt were compared between the patients and the controls. Three months after surgery, the patients recovered from the surgery with ameliorated intracranial pressure and slight improvement of clinical intelligence scale and motor scale. Before ventriculoperitoneal shunt, the FA values (except the right FHLV) were significantly decreased and the ADC values were significantly increased in the patients with hydrocephalus, compared with the controls. After the ventriculoperitoneal shunt, the FA values in the FHLV and OHLV of the patients were similar to the controls, but the FA values in other ROIs were still significantly lower than controls. The ADC values in the FS and OS white matter areas of the patients were similar to the controls; however, the ADC values in other ROIs were still significantly higher in patients. The increase of FA and the reduction in ADC in the ROIs preceded the clinical function improvement in patients with high intracranial pressure hydrocephalus and reflected the early changes in brain tissue microstructure, such as the compression of the white matter areas in

Classification of hydrocephalus: critical analysis of classification categories and advantages of "Multi-categorical Hydrocephalus Classification" (Mc HC).

PubMed

Oi, Shizuo

2011-10-01

Hydrocephalus is a complex pathophysiology with disturbed cerebrospinal fluid (CSF) circulation. There are numerous numbers of classification trials published focusing on various criteria, such as associated anomalies/underlying lesions, CSF circulation/intracranial pressure patterns, clinical features, and other categories. However, no definitive classification exists comprehensively to cover the variety of these aspects. The new classification of hydrocephalus, "Multi-categorical Hydrocephalus Classification" (Mc HC), was invented and developed to cover the entire aspects of hydrocephalus with all considerable classification items and categories. Ten categories include "Mc HC" category I: onset (age, phase), II: cause, III: underlying lesion, IV: symptomatology, V: pathophysiology 1-CSF circulation, VI: pathophysiology 2-ICP dynamics, VII: chronology, VII: post-shunt, VIII: post-endoscopic third ventriculostomy, and X: others. From a 100-year search of publication related to the classification of hydrocephalus, 14 representative publications were reviewed and divided into the 10 categories. The Baumkuchen classification graph made from the round o'clock classification demonstrated the historical tendency of deviation to the categories in pathophysiology, either CSF or ICP dynamics. In the preliminary clinical application, it was concluded that "Mc HC" is extremely effective in expressing the individual state with various categories in the past and present condition or among the compatible cases of hydrocephalus along with the possible chronological change in the future.

[Idiopathic intracranial hypertension: a caesarean with epidural anaesthesia after bringing the cerebrospinal fluid pressure back to normal].

PubMed

Pérez Rodríguez, M; de Carlos Errea, J; Dorronsoro Auzmendi, M; Batllori Gastón, M

2013-12-01

Idiopathic intracranial hypertension is diagnosed by exclusion. Because of its uncertain physiopathology and infrequent occurrence, its anaesthetic management is not well defined. The patient in this case is a pregnant woman with this disease with no lumbar-peritoneal shunt who was referred for non-urgent caesarean section, consisting of CSF drainage and pressure normalisation before the administration of epidural anaesthesia. We believe this technique can de effective to achieve adequate blockage and increased patient comfort, as well as improving postoperative recovery. Copyright © 2012 Sociedad Española de Anestesiología, Reanimación y Terapéutica del Dolor. Published by Elsevier España. All rights reserved.

Brain CT scan indexes in the normal pressure hydrocephalus: predictive value in the outcome of patients and correlation to the clinical symptoms.

PubMed

Chatzidakis, Emmanuel M; Barlas, George; Condilis, Nicolas; Bouramas, Dimos; Anagnostopoulos, Demetrios; Volikas, Zacharias; Simopoulos, Konstantinos

2008-01-01

The aim of this study is to find out the correlation of the ventricular size of the brain, as it is estimated using brain computed tomography (CT) scan indexes in patients with normal pressure hydrocephalus (NPH), to: a) the clinical symptoms, and b) the results of cerebrospinal fluid (CSF) shunting procedures. We looked for any predictive value in the estimation of brain CT scan indexes, in patients as above, in whom a shunt is going to be placed. It is well known that it is very difficult to decide who is going to improve after shunting. We studied 40 cases of patients with the diagnosis "NPH" in whom the ventricular shunts were placed. Every symptom (motor disturbance, deficit of memory, incontinence) was separately evaluated preoperatively. The outcome of shunting was also evaluated and the patients were graded. The following CT scan indexes were estimated from the preoperative CT scans of the brain in every case: the ventricle-brain ratio (VBR), the bi-caudate and bi-frontal ratios, the third ventricle-Sylvian fissure (3V-SF) ratio, and the four largest cortical gyri. The method we have used for statistics is "one way analysis of variance", correlating the CT scan indexes to the symptoms of the patients preoperatively, and the outcome of them postoperatively. The main conclusion is that the size of the lateral ventricles of the brain preoperatively is not correlated to the outcome after CSF shunting surgery, but it is correlated to the symptoms of NPH preoperatively.

Diagnosis and Differential Diagnosis of Hydrocephalus in Adults.

PubMed

Langner, Sönke; Fleck, Steffen; Baldauf, Jörg; Mensel, Birger; Kühn, Jens Peter; Kirsch, Michael

2017-08-01

Purpose  Hydrocephalus is caused by an imbalance of production and absorption of cerebrospinal fluid (CSF) or obstruction of its pathways, resulting in ventricular dilatation and increased intracranial pressure. Imaging plays a crucial role in the diagnosis, differential diagnosis and planning of treatment. Methods  This review article presents the different types of hydrocephalus und their typical imaging appearance, describes imaging techniques, and discusses differential diagnoses of the different forms of hydrocephalus. Results and Conclusion  Imaging plays a central role in the diagnosis of hydrocephalus. While magnetic resonance (MR) imaging is the first-line imaging modality, computed tomography (CT) is often the first-line imaging test in emergency patients. Key points   · Occlusive hydrocephalus is caused by obstruction of CSF pathways.. · Malabsorptive hydrocephalus is caused by impaired CSF absorption.. · The MR imaging protocol should always include sagittal high-resolution T2-weighted images.. · When an inflammatory etiology is suspected, imaging with contrast agent administration is necessary.. Citation Format · Langner S, Fleck S, Baldauf J et al. Diagnosis and Differential Diagnosis of Hydrocephalus in Adults. Fortschr Röntgenstr 2017; 189: 728 - 739. © Georg Thieme Verlag KG Stuttgart · New York.

Biodriven microsystem for treatment of hydrocephalus

NASA Astrophysics Data System (ADS)

Joswig, Jurgen; Oswald, Jens; Seifert, Steffen

1995-09-01

A microvalve system made of silicon for use in hydrocephalus therapy is presented, which will provide an excellent intracranial pressure stabilization. Design and processing are described. Testing results (in vitro) are presented.

Our initial experience with ventriculo-epiplooic shunt in treatment of hydrocephalus in two centers.

PubMed

Grigorean, Valentin Titus; Sandu, Aurelia Mihaela; Popescu, Mihai; Florian, Ioan Stefan; Lupascu, Cristian Dumitru; Ursulescu, Corina Lupascu

Hydrocephalus represents impairment in cerebrospinal fluid (CSF) dynamics. If the treatment of hydrocephalus is considered difficult, the repeated revisions of ventriculo-peritoneal (VP) shunts are even more challenging. The aim of this article is to evaluate the efficiency of ventriculo-epiplooic (VEp) shunt as a feasible alternative in hydrocephalic patients. A technical modification regarding the insertion of peritoneal catheter was imagined: midline laparotomy 8-10cm long was performed in order to open the peritoneal cavity; the great omentum was dissected between its two layers; we placed the distal end of the catheter between the two epiplooic layers; a fenestration of 4cm in diameter into the visceral layer was also performed. A retrospective study of medical records of 15 consecutive patients with hydrocephalus treated with VEp shunt is also presented. Between 2008 and 2014 we performed VEp shunt in 15 patients: 5 with congenital hydrocephalus, 8 with secondary hydrocephalus and 2 with normal pressure hydrocephalus. There were 7 men and 8 women. VEp shunt was performed in 13 patients with multiple distal shunt failures and in 2 patients, with history of abdominal surgery, as de novo extracranial drainage procedure. The outcome was favorable in all cases, with no significant postoperative complications. VEp shunt is a new, safe and efficient surgical technique for the treatment of hydrocephalus. VEp shunt is indicated in patients with history of recurrent distal shunt failures, and in patients with history of open abdominal surgery and high risk for developing abdominal complications. Copyright © 2017 Polish Neurological Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.

Treatment of posthemorrhagic hydrocephalus in premature infants with subcutaneous reservoir drainage.

PubMed

Yu, Bo; Li, Shasha; Lin, Zhenlang; Zhang, Nu

2009-01-01

To investigate the effectiveness of subcutaneous reservoir drainage as a treatment for the different types of posthemorrhagic hydrocephalus in premature infants. 11 premature infants with posthemorrhagic hydrocephalus underwent subcutaneous reservoir embedment surgery, and cerebrospinal fluid (CSF) was drained via the reservoir intermittently for 8 weeks. During the period of drainage, ultrasound and computerized tomography were used to measure ventricle size. CSF examinations were performed routinely to assess the presence of intraventricular hemorrhage (IVH) and/or infection. (1) Five infants were diagnosed as having obstructive hydrocephalus; 2 had nearly normal ventricle sizes with treatment and drainage was stopped after 8 weeks, 2 had nearly normal ventricle sizes after 4 more weeks of drainage, and 1 underwent ventriculoperitoneal shunt (V-P shunt) surgery due to failure of ventricle size reduction. (2) Six infants were diagnosed as having communicating hydrocephalus; 4 had further enlarged ventricle size after 8 weeks and underwent V-P shunt surgery, 1 had treatment aborted at week 8 of drainage, and only 1 had moderate reduction of ventricle size after 8 weeks. (3) None of the 11 infants had progressive IVH and/or intracranial infection during drainage. Subcutaneous reservoir drainage is a suitable and safe treatment for posthemorrhagic hydrocephalus in premature infants. It is more effective for obstructive hydrocephalus than for communicating hydrocephalus. Copyright (c) 2009 S. Karger AG, Basel.

MRI, volumetry, 1H spectroscopy, and cerebropetal blood flowmetry in childhood idiopathic anatomic megalencephaly.

PubMed

Koudijs, Suzanne M; van der Grond, Jeroen; Hoogendoorn, Mechteld L C; Hulshoff Pol, Hilleke E; Schnack, Hugo G; Witkamp, Theo D; Gooskens, Rob H J M; van Nieuwenhuizen, Onno; Braun, Kees P J

2006-08-01

To evaluate cerebral abnormalities in childhood idiopathic anatomic megalencephaly (MC) by means of different magnetic resonance (MR) modalities. MRI, volumetry, spectroscopy, and cerebropetal blood flowmetry were performed in six children with idiopathic anatomic MC, and seven volunteers. MRI revealed an increased ventricular system in five of six patients. A thalamic hamartoma was found in one patient and a Chiari I malformation was found in two. Volumetric analysis showed a disproportional increase of ventricular volume but normal subarachnoid cerebrospinal fluid (CSF) volume. Supratentorial volume was disproportionally increased compared to cerebellar volume. Intracranial volume correlated significantly with skull circumference. MR spectroscopy (MRS) N-acetyl aspartate/choline (NAA/Cho) peak ratios in WM were significantly higher in patients than in controls. Choline/creatine (Cho/Cr) peak ratios in WM were significantly lower in patients. Cortical gray matter (GM) MRS ratios were unaltered. Cerebropetal flow was increased in MC, possibly related to increased brain volume. This study reveals associated developmental anomalies for idiopathic anatomic MC. A relative ventriculomegaly was found, which should not be misinterpreted as true hydrocephalus. In contrast to metabolic MC, MRS showed no severe disturbances. Total intracranial volume is correlated to skull circumference and cerebropetal blood flow.

Long-term outcomes of adult chronic idiopathic hydrocephalus treated with a ventriculo-peritoneal shunt.

PubMed

Illán-Gala, I; Pérez-Lucas, J; Martín-Montes, A; Máñez-Miró, J; Arpa, J; Ruiz-Ares, G

2017-05-01

Adult chronic idiopathic hydrocephalus (ACIH) is a cause of dementia that can be treated by implanting a ventriculo-peritoneal shunt (VPS). We aim to study clinical and functional outcomes in patients with ACIH corrected with a VPS. Observational cohort study of patients diagnosed with probable ACIH (Japan Neurosurgical Society guidelines) and undergoing shunt placement between 2008 and 2013 in a centre of reference for neurosurgery in Spain. Clinical improvement was classified in 4 categories (resolution, partial improvement, equivocal improvement, and no improvement); functional outcome was assessed on the modified Rankin scale (mRS). The study included 29 patients with a mean age of 73.9 years; 62.1% were male and 65.5% had hypertension. Clinical improvement (complete or partial) was observed in 58% after one year and in 48% by the end of the follow-up period (mean follow-up time was 37.8 months). Older age, presence of hypertension, and surgery-related complications were more prevalent in the group responding poorly to treatment. One patient died, 20.7% experienced severe complications, and 69% were dependent (mRS ≥ 3) by the end of the follow-up period. Age at diagnosis was independently associated with poorer clinical response at one year and a higher degree of dependency by the end of follow-up. Symptomatic benefits offered by VPS were partial and transient; treatment was associated with a high complication rate and poor functional outcomes in the long term, especially in the oldest patients. Copyright © 2015 Sociedad Española de Neurología. Publicado por Elsevier España, S.L.U. All rights reserved.

Management of hydrocephalus associated with autoimmune diseases: a series of 19 cases.

PubMed

Ma, Baitao; Wu, Hao; Yin, Hexiang; Chang, Jianbo; Wang, Li; Wang, Renzhi; Ma, Wenbin; Li, Yongning; Guan, Jian; Liu, Jinjing; Wei, Junji

2017-11-01

To analyze the diagnosis and treatment of hydrocephalus associated with autoimmune diseases and to explore the possible mechanism of hydrocephalus in these patients. A retrospective case series study was conducted at Peking Union Medical College Hospital, Beijing, China. Files were retrieved from the hospital archives by screening records from Jan 1990 to Jan 2016. Medical records were screened for data regarding (1) the number of patients diagnosed with hydrocephalus associated with autoimmune diseases, (2) the clinical manifestation of hydrocephalus associated with autoimmune disease, and (3) the outcomes of these patients treated with medication or ventriculoperitoneal shunt (VPS). A total of 19 of 19,643 hospitalized autoimmune diseases patients were found to have hydrocephalus. Seven of the 19 patients had systemic lupus erythematosus (SLE), 3 patients had Sjögren's syndrome, 2 patients had rheumatoid arthritis (RA), 1 patient had connective tissue disease, 1 patient had juvenile idiopathic arthritis (JIA), 1 patient had Guillain-Barre syndrome (GBS), 1 patient had systemic sclerosis, 1 patient had Crohn's disease, 1 patient had relapsing polychondritis (RPC), and 1 patient had autoinflammatory disease (AID). Of the 19 patients, 13 received medication treatment, and the most commonly used drugs were corticosteroids and mannitol. A total of 6 patients received both medication therapy and VPS treatment with a programable valve. After average follow-up lengths of 11 months for patients who received VPS and 8.2 for patients who received medical treatment, the clinical symptoms of patients treated by VPS or medication were improved (83% (5/6) vs. 15.4% (2/13), respectively), patients were in stable condition (17% (1/6) vs. 30.8% (4/13), respectively), and mortality decreased (0% vs. 53.8% (7/13), respectively). VPS along with corticosteroids and immunosuppressants represents an effective treatment approach for patients who suffer from hydrocephalus associated

Comparative observational study on the clinical presentation, intracranial volume measurements, and intracranial pressure scores in patients with either Chiari malformation Type I or idiopathic intracranial hypertension.

PubMed

Frič, Radek; Eide, Per Kristian

2017-04-01

OBJECTIVE Several lines of evidence suggest common pathophysiological mechanisms in Chiari malformation Type I (CMI) and idiopathic intracranial hypertension (IIH). It has been hypothesized that tonsillar ectopy, a typical finding in CMI, is the result of elevated intracranial pressure (ICP) combined with a developmentally small posterior cranial fossa (PCF). To explore this hypothesis, the authors specifically investigated whether ICP is comparable in CMI and IIH and whether intracranial volumes (ICVs) are different in patients with CMI and IIH, which could explain the tonsillar ectopy in CMI. The authors also examined whether the symptom profile is comparable in these 2 patient groups. METHODS The authors identified all CMI and IIH patients who had undergone overnight diagnostic ICP monitoring during the period from 2002 to 2014 and reviewed their clinical records and radiological examinations. Ventricular CSF volume (VV), PCF volume (PCFV), and total ICV were calculated from initial MRI studies by using volumetric software. The static and pulsatile ICP scores during overnight monitoring were analyzed. Furthermore, the authors included a reference (REF) group consisting of patients who had undergone ICP monitoring due to suspected idiopathic normal-pressure hydrocephalus or chronic daily headache and showed normal pressure values. RESULTS Sixty-six patients with CMI and 41 with IIH were identified, with comparable demographics noted in both groups. The occurrence of some symptoms (headache, nausea, and/or vomiting) was comparable between the cohorts. Dizziness and gait ataxia were significantly more common in patients with CMI, whereas visual symptoms, diplopia, and tinnitus were significantly more frequent in patients with IIH. The cranial volume measurements (VV, PCFV, and ICV) of the CMI and IIH patients were similar. Notably, 7.3% of the IIH patients had tonsillar descent qualifying for diagnosis of CMI (that is, > 5 mm). The extent of tonsillar ectopy was

Normal Pressure Hydrocephalus (NPH)

MedlinePlus

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Craniectomy-associated Progressive Extra-Axial Collections with Treated Hydrocephalus (CAPECTH): redefining a common complication of decompressive craniectomy.

PubMed

Nalbach, Stephen V; Ropper, Alexander E; Dunn, Ian F; Gormley, William B

2012-09-01

Extra-axial fluid collections following decompressive craniectomy have been observed in a variety of patient populations. These collections have traditionally been thought to represent extra-axial signs of hydrocephalus, but they often occur even in settings where hydrocephalus has been optimally treated. This study aims to elucidate the phenomenon of extra-axial fluid collections after decompressive craniectomy in patients with treated hydrocephalus, in order to improve identification, classification, prevention and treatment. We retrospectively reviewed all patients at a single institution undergoing decompressive craniectomy for refractory intracranial pressure elevations from June 2007 through December 2009. We identified 39 patients by reviewing clinical reports and imaging. Any patient who died on or prior to the third post-operative day (POD) was excluded. The analysis focused on patients with extra-axial collections and treated hydrocephalus. Twenty-one of 34 (62%) patients developed extra-axial collections and 18 of these developed collections despite ventricular drainage. Subgroup analysis revealed that seven of seven patients (100%) with subarachnoid hemorrhage, and 11 of 14 (79%) with traumatic brain injury developed collections. Extra-axial collections may develop after decompressive craniectomy despite aggressive treatment of communicating hydrocephalus. In these patients, the term "external hydrocephalus" does not appropriately capture the relevant pathophysiology. Instead, we define a new phenomenon, "Craniectomy-associated Progressive Extra-Axial Collections with Treated Hydrocephalus" (CAPECTH), as progressive collections despite aggressive cerebral spinal fluid (CSF) drainage. Our data indicate that early cranioplasty can help prevent the formation and worsening of this condition, presumably by returning normal CSF dynamics. Copyright © 2012 Elsevier Ltd. All rights reserved.

Quantification of abnormal intracranial pressure waves and isotope cisternography for diagnosis of occult communicating hydrocephalus

DOE Office of Scientific and Technical Information (OSTI.GOV)

Cardoso, E.R.; Piatek, D.; Del Bigio, M.R.

1989-01-01

Nineteen consecutive patients with suspected occult communicating hydrocephalus were investigated by means of clinical evaluation, neuropsychological testing, isotope cisternography, computed tomography scanning, and continuous intracranial pressure monitoring. Semi-quantitative grading systems were used in the evaluation of the clinical, neuropsychological, and cisternographic assessments. Clinical examination, neuropsychological testing, and computed tomography scanning were repeated 3 months after ventriculoperitoneal shunting. All patients showed abnormal intracranial pressure waves and all improved after shunting. There was close correlation between number, peak, and pulse pressures of B waves and the mean intracranial pressure. However, quantification of B waves by means of number, frequency, and amplitude didmore » not help in predicting the degree of clinical improvement postshunting. The most sensitive predictor of favorable response to shunting was enlargement of the temporal horns on computed tomography scan. Furthermore, the size of temporal horns correlated with mean intracranial pressure. There was no correlation between abnormalities on isotope cisternography and clinical improvement.« less

Hydrocephalus treatment in children: long-term outcome in 975 consecutive patients.

PubMed

Beuriat, Pierre-Aurelien; Puget, Stephanie; Cinalli, Giuseppe; Blauwblomme, Thomas; Beccaria, Kevin; Zerah, Michel; Sainte-Rose, Christian

2017-07-01

OBJECTIVE Hydrocephalus remains one of the more common pathologies managed in pediatric neurosurgical units. Endoscopic third ventriculostomy (ETV) has an advantage over ventriculoperitoneal shunting as it enables patients to remain device free. Multiple shunt devices with various valve designs exist, with no one valve proven to be superior to another. The aim of this study was to describe the management of hydrocephalus and its long-term outcome. METHODS The authors retrospectively reviewed the medical records of all patients who had been treated for hydrocephalus at the Hôpital Necker-Enfants Malades in the period from 1985 to 1995. RESULTS Nine hundred seventy-five children had been treated for hydrocephalus. The mean follow-up was 11 ± 7.4 years (mean ± standard deviation). The most common cause of hydrocephalus was tumor related (32.3%), followed by malformative (24.5%) and inflammatory (20.9%) causes. Two hundred eighty patients underwent ETV as the first-line treatment. The procedure was effective in controlling hydrocephalus due to posterior fossa tumors and aqueductal stenosis. Six hundred ninety-five children had initial shunt insertion, with the majority receiving an Orbis-Sigma valve (OSV). The overall OSV shunt survival was 70% at 1 year, 58% at 10 years, and 49% at 20 years. The most common cause for mechanical shunt failure was obstruction (50.7%). Overall shunt survival was statistically different between the OSV and the differential-pressure valve (p = 0.009). CONCLUSIONS Endoscopic third ventriculostomy is effective in the management of childhood hydrocephalus. Its success is directly related to the underlying pathology. In the long term, the OSV has significantly higher event-free shunt survival than the classic differential-pressure valve systems.

Are in-shoe pressure characteristics in symptomatic idiopathic pes cavus related to the location of foot pain?

PubMed

Crosbie, Jack; Burns, Joshua

2008-01-01

People who have extremely high arched feet may be subject to substantial levels of foot pain, despite the lack of obvious pathology. This study sought to investigate the effect of pes cavus on pain intensity and location and on the magnitude and distribution of foot pressure. Measurements were derived from the more symptomatic foot of 130 participants with painful, idiopathic pes cavus. Data were collected using Pedar in-shoe pressure sensors and averaged over nine randomly selected steps. Participant information, including location and intensity of pain, Foot Posture Index values and anthropometric and "quality of life" variables, were also recorded. Painful idiopathic pes cavus seems to provoke a more cautious gait pattern than normal, with reduced peak and mean pressure values, particularly in the fore- and rear-foot regions. In particular, participants with pain confined to the rear-foot exhibit an antalgic gait pattern, with lower pressure values and a longer period of foot-ground contact in the heel region than those with pain only in the fore-foot. We determined no clear predictors of pain in terms of foot posture or demographics, although people with high body mass index values are more likely to have pain in several regions. The relationship between the posture of the foot and the presentation of pain remains unclear, however we believe that the presence of heel pain in pes cavus may be more restricting than fore-foot pain.

Arrested Hydrocephalus in Childhood: Case Series and Review of the Literature.

PubMed

Hurni, Yannick; Poretti, Andrea; Schneider, Jacques; Guzman, Raphael; Ramelli, Gian Paolo

2018-06-22

 Hydrocephalus can be progressive or spontaneously arrested. In arrested hydrocephalus, the balance between production and absorption of the cerebrospinal fluid is restored. Patients are mostly asymptomatic, and no surgical treatment is necessary for them.  We performed a two-center consecutive case series study, aimed at investigating the safety of nonsurgical management of hydrocephalus in selected pediatric patients. We retrospectively selected all consecutive patients, suspected to suffer from arrested hydrocephalus and referred to our two institutions between January 2011 and December 2013. Data on clinical and radiological follow-up were collected until June 2017.  Five children diagnosed with arrested hydrocephalus were included in the study. All patients presented macrocephaly as the main presenting sign. Associated mild-to-moderate stable motor disorders were assessed in four out of five cases. Typical symptoms and signs associated with acute raised intracranial pressure were absent in all patients. Magnetic resonance imaging studies showed ventriculomegaly in all patients. A diagnosis of arrested hydrocephalus was made in all five cases based on stable clinical and radiological findings during the initial observation. Conservative management based on active surveillance was, therefore, proposed. During the follow-up period, we observed stable or improved conditions in four out of five patients, while the remaining patient presented progressive hydrocephalus.  Making a distinction between arrested and progressive hydrocephalus is fundamental, because of the opposed appropriate management. Any newly discovered case of hydrocephalus, not characterized by clear signs of progressive hydrocephalus, should benefit from active surveillance before any definitive decision is taken. Georg Thieme Verlag KG Stuttgart · New York.

Outcome in pediatric hydrocephalus: a comparison between previously used outcome measures and the hydrocephalus outcome questionnaire.

PubMed

Platenkamp, Marc; Hanlo, Patrick W; Fischer, Kathelijn; Gooskens, Rob H J M

2007-07-01

The objectives of this study were to assess, in a cohort of children with recently treated hydrocephalus, the correlation between scores on the Hydrocephalus Outcome Questionnaire (HOQ) and the children's type of schooling and motor functioning, and to assess the overall outcome of the children. The health status of 142 pediatric patients (85 boys) with previous hydrocephalus, born between 1995 and 1999, was assessed. Outcomes were determined using the HOQ, type of schooling, and motor functioning. Data were obtained from parental interviews and patient medical records. RESULTS. Twelve patients died (8.5%). Responses to the HOQ were obtained from 107 patients (65 boys). The mean age of the patients was 7 years and 9 months +/- 1.42 years (range 6-10 years). The Physical Health score of the HOQ correlated well with the motor functioning score (r = 0.652) as did the Cognitive Health score with the type of schooling (r = 0.672). Fifty-nine percent of the patients were able to attend a school for students with normal intelligence. Disabling motor functioning was found in only 30% of patients. Epilepsy was present in 14%. The results show a good correlation between the type of schooling and the Cognitive HOQ score and between the Physical HOQ score and the motor functioning score. The HOQ is a simple and very useful measurement for determining outcome in pediatric hydrocephalus.

Genetics of human hydrocephalus

PubMed Central

Williams, Michael A.; Rigamonti, Daniele

2006-01-01

Human hydrocephalus is a common medical condition that is characterized by abnormalities in the flow or resorption of cerebrospinal fluid (CSF), resulting in ventricular dilatation. Human hydrocephalus can be classified into two clinical forms, congenital and acquired. Hydrocephalus is one of the complex and multifactorial neurological disorders. A growing body of evidence indicates that genetic factors play a major role in the pathogenesis of hydrocephalus. An understanding of the genetic components and mechanism of this complex disorder may offer us significant insights into the molecular etiology of impaired brain development and an accumulation of the cerebrospinal fluid in cerebral compartments during the pathogenesis of hydrocephalus. Genetic studies in animal models have started to open the way for understanding the underlying pathology of hydrocephalus. At least 43 mutants/loci linked to hereditary hydrocephalus have been identified in animal models and humans. Up to date, 9 genes associated with hydrocephalus have been identified in animal models. In contrast, only one such gene has been identified in humans. Most of known hydrocephalus gene products are the important cytokines, growth factors or related molecules in the cellular signal pathways during early brain development. The current molecular genetic evidence from animal models indicate that in the early development stage, impaired and abnormal brain development caused by abnormal cellular signaling and functioning, all these cellular and developmental events would eventually lead to the congenital hydrocephalus. Owing to our very primitive knowledge of the genetics and molecular pathogenesis of human hydrocephalus, it is difficult to evaluate whether data gained from animal models can be extrapolated to humans. Initiation of a large population genetics study in humans will certainly provide invaluable information about the molecular and cellular etiology and the developmental mechanisms of human

Bedside Optic Nerve Sheath Diameter Assessment in the Identification of Increased Intracranial Pressure in Suspected Idiopathic Intracranial Hypertension.

PubMed

Irazuzta, Jose E; Brown, Martha E; Akhtar, Javed

2016-01-01

We determined whether the bedside assessment of the optic nerve sheath diameter could identify elevated intracranial pressure in individuals with suspected idiopathic intracranial hypertension. This was a single-center, prospective, rater-blinded study performed in a freestanding pediatric teaching hospital. Patients aged 12 to 18 years scheduled for an elective lumbar puncture with the suspicion of idiopathic intracranial hypertension were eligible to participate. Optic nerve sheath diameter was measured via ultrasonography before performing a sedated lumbar puncture for measuring cerebrospinal fluid opening pressure. Abnormal measurements were predefined as optic nerve sheath diameter ≥4.5 mm and a cerebrospinal fluid opening pressure greater than 20 cmH2O. Thirteen patients participated in the study, 10 of whom had elevated intracranial pressure. Optic nerve sheath diameter was able to predict or rule out elevated intracranial pressure in all patients. Noninvasive assessment of the optic nerve sheath diameter could help to identify patients with elevated intracranial pressure when idiopathic intracranial hypertension is suspected. Copyright © 2016 Elsevier Inc. All rights reserved.

Biomarkers in adult posthemorrhagic hydrocephalus.

PubMed

Hua, Cong; Zhao, Gang

2017-08-01

Posthemorrhagic hydrocephalus is a severe complication following intracranial hemorrhage. Posthemorrhagic hydrocephalus is often associated with high morbidity and mortality and serves as an important clinical predictor of adverse outcomes after intracranial hemorrhage. Currently, no effective medical intervention exists to improve functional outcomes in posthemorrhagic hydrocephalus patients because little is still known about the mechanisms of posthemorrhagic hydrocephalus pathogenesis. Because a better understanding of the posthemorrhagic hydrocephalus pathogenesis would facilitate development of clinical treatments, this is an active research area. The purpose of this review is to describe recent progress in elucidation of molecular mechanisms that cause posthemorrhagic hydrocephalus. What we are certain of is that the entry of blood into the ventricular system and subarachnoid space results in release of lytic blood products which cause a series of physiological and pathological changes in the brain. Blood components that can be linked to pathology would serve as disease biomarkers. From studies of posthemorrhagic hydrocephalus, such biomarkers are known to mutually synergize to initiate and promote posthemorrhagic hydrocephalus progression. These findings suggest that modulation of biomarker expression or function may benefit posthemorrhagic hydrocephalus patients.

Shunting for hydrocephalus: analysis of techniques and failure patterns.

PubMed

Nigim, Fares; Critchlow, Jonathan F; Schneider, Benjamin E; Chen, Clark; Kasper, Ekkehard M

2014-09-01

Hydrocephalus is characterized by ventricular dilatation because of progressive accumulation of cerebrospinal fluid. Normal pressure hydrocephalus (NPH) affects a subset of patients representing a reversible clinical triad of gait disturbance, urinary incontinence, and dementia with normal cerebrospinal fluid pressure and composition. Various shunting procedures have been used for treatment, but techniques and outcomes remain under debate. The objective of this study was to evaluate the clinical outcomes of 232 patients with and without NPH after the first-time Ventriculoperitoneal shunt placement and assessed patterns of failure between December 2004 and December 2012. Mean age was 54.7 y in non-NPH and 71.9 y in NPH patients. We used open technique in 34.3% and laparoscopic technique in 65.7% of NPH patients and 32.7% and 67.3% of the non-NPH patients, respectively. A total of 36 of 232 patients displayed shunt failure, 16.4% in NPH and 15.2% in non-NPH patients. Twenty-three of 155 patients failed after laparoscopic and 13 of 77 failed after open placement. Proximal shunt failure was more frequent in the non-NPH cohort. Distal failures accounted for 13 of 232 cases, and the difference between laparoscopic (six of 155) and open failures (seven of 77) was profound, but not between NPH- and non-NPH patients. Shunt failures are related to the placement method. Non-NPH patients showed more proximal failures. NPH patients showed fewer proximal failures. Less distal failures were observed after laparoscopic ventriculoperitoneal shunt placement without significant differences between NPH and non-NPH patients. Beyond this, laparoscopic surgery carries distinct advantages such as shorter operating room times and hospital stays, which should translate into less use of pain medications, earlier mobilization, and a lower incidence of ileus. Copyright © 2014 Elsevier Inc. All rights reserved.

Comparison of segmental spinal movement control in adolescents with and without idiopathic scoliosis using modified pressure biofeedback unit.

PubMed

Luo, Hong-Ji; Lin, Shi-Xiang; Wu, Shyi-Kuen; Tsai, Mei-Wun; Lee, Shwn-Jen

2017-01-01

Postural rehabilitation emphasizing on motor control training of segmental spinal movements has been proposed to effectively reduce the scoliotic spinal deformities in adolescent idiopathic scoliosis (AIS). However, information regarding the impairments of segmental spinal movement control involving segmental spinal stabilizers in adolescent idiopathic scoliosis remains limited. Examination of segmental spinal movement control may provide a window for investigating the features of impaired movement control specific to spinal segments that may assist in the development of physiotherapeutic management of AIS. To compare segmental spinal movement control in adolescents with and without idiopathic scoliosis using modified pressure biofeedback unit. Segmental spinal movement control was assessed in twenty adolescents with idiopathic scoliosis (AISG) and twenty healthy adolescents (CG) using a modified pressure biofeedback unit. Participants performed segmental spinal movements that primarily involved segmental spinal stabilizing muscles with graded and sustained muscle contraction against/off a pressure cuff from baseline to target pressures and then maintained for 1 min. Pressure data during the 1-minute maintenance phase were collected for further analysis. Pressure deviation were calculated and compared between groups. The AISG had significantly greater pressure deviations for all segmental spinal movements of cervical, thoracic, and lumbar spine than the CG. Pressure biofeedback unit was feasible for assessing segmental spinal movement control in AIS. AISG exhibited poorer ability to grade and sustain muscle activities for local movements of cervical, thoracic, and lumbar spine, suggesting motor control training of segmental spinal movements involving segmental spinal stabilizing muscles on frontal, sagittal, and transverse planes were required.

Cerebrospinal fluid biomarkers of infantile congenital hydrocephalus

PubMed Central

Limbrick, David D.; Baksh, Brandon; Morgan, Clinton D.; Habiyaremye, Gakwaya; McAllister, James P.; Inder, Terrie E.; Mercer, Deanna; Holtzman, David M.; Strahle, Jennifer; Wallendorf, Michael J.; Morales, Diego M.

2017-01-01

Introduction Hydrocephalus is a complex neurological disorder with a pervasive impact on the central nervous system. Previous work has demonstrated derangements in the biochemical profile of cerebrospinal fluid (CSF) in hydrocephalus, particularly in infants and children, in whom neurodevelopment is progressing in parallel with concomitant neurological injury. The objective of this study was to examine the CSF of children with congenital hydrocephalus (CHC) to gain insight into the pathophysiology of hydrocephalus and identify candidate biomarkers of CHC with potential diagnostic and therapeutic value. Methods CSF levels of amyloid precursor protein (APP) and derivative isoforms (sAPPα, sAPPβ, Aβ42), tau, phosphorylated tau (pTau), L1CAM, NCAM-1, aquaporin 4 (AQP4), and total protein (TP) were measured by ELISA in 20 children with CHC. Two comparative groups were included: age-matched controls and children with other neurological diseases. Demographic parameters, ventricular frontal-occipital horn ratio, associated brain malformations, genetic alterations, and surgical treatments were recorded. Logistic regression analysis and receiver operating characteristic curves were used to examine the association of each CSF protein with CHC. Results CSF levels of APP, sAPPα, sAPPβ, Aβ42, tau, pTau, L1CAM, and NCAM-1 but not AQP4 or TP were increased in untreated CHC. CSF TP and normalized L1CAM levels were associated with FOR in CHC subjects, while normalized CSF tau levels were associated with FOR in control subjects. Predictive ability for CHC was strongest for sAPPα, especially in subjects ≤12 months of age (p<0.0001 and AUC = 0.99), followed by normalized sAPPβ (p = 0.0001, AUC = 0.95), tau, APP, and L1CAM. Among subjects ≤12 months, a normalized CSF sAPPα cut-point of 0.41 provided the best prediction of CHC (odds ratio = 528, sensitivity = 0.94, specificity = 0.97); these infants were 32 times more likely to have CHC. Conclusions CSF proteins such as s

Papilledema in children with hydrocephalus: incidence and associated factors.

PubMed

Lee, Haeng Jin; Phi, Ji Hoon; Kim, Seung-Ki; Wang, Kyu-Chang; Kim, Seong-Joon

2017-06-01

OBJECTIVE The aim of this study was to report the incidence of and the factors associated with papilledema in children with hydrocephalus. METHODS Patients younger than 15 years of age who had been diagnosed with hydrocephalus and treated by extra-ventricular drainage or ventriculoperitoneal shunt surgery between 2005 and 2015 were retrospectively reviewed. Factors including patient age and sex, etiology of hydrocephalus, duration of signs or symptoms, intracranial pressure (ICP), and presence of papilledema were evaluated. RESULTS Forty-six patients, whose mean age was 6.3 ± 4.7 years, were included in the study. The 19 patients without papilledema had a mean age of 2.7 ± 2.7 years, and the 27 patients with papilledema had a mean age of 8.8 ± 4.2 years (p < 0.001). The mean ICP was 19.9 ± 10.0 cm H 2 O among those without papilledema and 33.3 ± 9.1 cm H 2 O among those with papilledema (p < 0.001). The mean duration of signs or symptoms was 3.0 ± 4.6 months in the patients without papilledema and 3.4 ± 3.9 months in those with papilledema (p = 0.704). The patients with papilledema were older and presented with higher ICP than those without. The causes of hydrocephalus were tumor (59%), congenital anomaly (19%), hemorrhage (13%), and infection (9%). CONCLUSIONS Papilledema was more common in patients who were older, who had higher ICP, and whose hydrocephalus had been induced by brain tumor. However, since papilledema was absent in 41% of the children with hydrocephalus, papilledema's absence does not ensure the absence of hydrocephalus, especially in younger patients.

Effect of venous stenting on intracranial pressure in idiopathic intracranial hypertension.

PubMed

Matloob, Samir A; Toma, Ahmed K; Thompson, Simon D; Gan, Chee L; Robertson, Fergus; Thorne, Lewis; Watkins, Laurence D

2017-08-01

Idiopathic intracranial hypertension (IIH) is characterised by an increased intracranial pressure (ICP) in the absence of any central nervous system disease or structural abnormality and by normal CSF composition. Management becomes complicated once surgical intervention is required. Venous sinus stenosis has been suggested as a possible aetiology for IIH. Venous sinus stenting has emerged as a possible interventional option. Evidence for venous sinus stenting is based on elimination of the venous pressure gradient and clinical response. There have been no studies demonstrating the immediate effect of venous stenting on ICP. Patients with a potential or already known diagnosis of IIH were investigated according to departmental protocol. ICP monitoring was performed for 24 h. When high pressures were confirmed, CT venogram and catheter venography were performed to look for venous stenosis to demonstrate a pressure gradient. If positive, venous stenting would be performed and ICP monitoring would continue for a further 24 h after deployment of the venous stent. Ten patients underwent venous sinus stenting with concomitant ICP monitoring. Nine out of ten patients displayed an immediate reduction in their ICP that was maintained at 24 h. The average reduction in mean ICP and pulsatility was significant (p = 0.003). Six out of ten patients reported a symptomatic improvement within the first 2 weeks. Venous sinus stenting results in an immediate reduction in ICP. This physiological response to venous stenting has not previously been reported. Venous stenting could offer an alternative treatment option in correctly selected patients with IIH.

Hydrocephalus (For Parents)

MedlinePlus

... any of the ventricles, or something disturbs the recycling, CSF backs up and causes hydrocephalus. Hydrocephalus can ... Note: All information on KidsHealth® is for educational purposes only. For specific medical advice, diagnoses, and treatment, ...

[Clinical experience with the Sp[hy adjustable valve in the treatment of adult hydrocephalus. A series of 147 cases].

PubMed

Bret, P; Guyotat, J; Ricci, A C; Mottolese, C; Jouanneau, E

1999-05-01

Evaluate the advantages, results and drawbacks of the Sophy adjustable valve (SV) in the treatment of hydrocephalus in the adult. One-hundred and forty-seven adult hydrocephalic patients (mean age = 64.5 years) shunted with a SV between 1990 and 1997 were retrospectively reviewed. Etiologies of hydrocephalus were: "normal pressure" chronic hydrocephalus (NPH) in 124 and tumoral hydrocephalus with intracranial hypertension in 23 patients. One-hundred fifty shunts were established in 147 patients: 14 ventriculoatrial and 136 ventriculoperitoneal shunts. The initial pressure settings of the SV were: high pressure: 12 times (8%), medium pressure: 136 times (90.6%) and low pressure: 2 times (1.3%). Sixteen patients were lost to follow-up. Two patients died within hours following the procedure. So, the results were analyzed in the 129 remaining patients, with a follow-up of 16.7 months (+/- 17.8). Three patients died from shunted-related causes (2.3%). Eighty-four patients had good results (65%), 25 had fair results (19.5%) and 20 failed to improve or deteriorated (15.5%) after shunting. Fifty-three reoperations were performed in 44 patients (34%), including 37 procedures for mechanical obstruction and 6 for septic complications. Subdural effusions (SDE) occurred in 16 patients (12.5%): 10 surgical evacuations were required in 7 patients; 8 patients were successfully treated using uprating of the SV pressure alone; one was managed conservatively. Fifty-eight pressure readjustments were performed in 47 patients. The pressure settings were uprated 28 times in patients showing clinical or computerized tomographic (CT) features suggesting overdrainage; 20 of those proved to be successful (71.5%). The pressure settings were lowered 30 times in patients showing a lack of improvement after shunting, attributed to underdrainage; only 12 of those proved to be successful (40%). Spontaneous changes of the pressure settings were observed in 14 patients (10.8%). After a MRI exposure

Evidence of an application of a variable MEMS capacitive sensor for detecting shunt occlusions

NASA Astrophysics Data System (ADS)

Apigo, David J.; Bartholomew, Philip L.; Russell, Thomas; Kanwal, Alokik; Farrow, Reginald C.; Thomas, Gordon A.

2017-04-01

A sensor was tested subdural and in vitro, simulating a supine infant with a ventricular-peritoneal shunt and controlled occlusions. The variable MEMS capacitive device is able to detect and forecast blockages, similar to early detection procedures in cancer patients. For example, with gradual occlusion development over a year, the method forecasts a danger over one month ahead of blockage. The method also distinguishes between ventricular and peritoneal occlusions. Because the sensor provides quantitative data on the dynamics of the cerebrospinal fluid, it can help test new therapies and work toward understanding hydrocephalus as well as idiopathic normal pressure hydrocephalus. The sensor appears to be a substantial advance in treating brain injuries treated with shunts and has the potential to bring significant impact in a clinical setting.

Results of Isotope Cisternography in 175 Patients with a Suspected Hydrocephalus

PubMed Central

Lee, Sang-Mi; Shim, Jae-Joon; Yoon, Seok-Mann; Bae, Hack-Gun; Doh, Jae-Won

2015-01-01

Objective Normal pressure hydrocephalus (NPH) is a syndrome characterized by gait disturbance, memory impairment and urinary incontinence. The isotope cisternography (ICG) became less useful because of low accuracy and complications. We tried to evaluate the safety and value of the ICG. Methods We retrospectively collected data on ICG of 175 consecutive patients with a suspected hydrocephalus. We classified the ICG into four types by the ventricular reflux and circulation time. The ventricular size was measured by Evans index and the width of the third ventricle. Results There were three complications including one case of paraplegia. Type 4 was the most common type, observed in 53%. Type 3 (33%), type 2 (7%), and type 1 (7%) were observed less often. Type 4 was more common in patients with large ventricles. Types of the ICG were not related to the causes of hydrocephalus, gender, or age of the patients. Shunting was more frequently performed in type 4 (71%), compared to type 1 (17%), type 2 (33%), and type 3 (46%). Surgery was more common when the cause was vascular. After the shunt surgery, 33.0% were graded as the improved. Although there were some improvements even in the not-improved patients, they still needed many helps. The improvement was related to the preoperative state. Conclusion ICG may bring a serious complication, however the incidence is very low. Although the predictability of response rate on the shunting is doubtful, ICG is a cheap and useful tool to select surgical candidates in NPH. PMID:27169059

Risk factors for hydrocephalus and neurological deficit in children born with an encephalocele.

PubMed

Da Silva, Stephanie L; Jeelani, Yasser; Dang, Ha; Krieger, Mark D; McComb, J Gordon

2015-04-01

There is a known association of hydrocephalus with encephaloceles. Risk factors for hydrocephalus and neurological deficit were ascertained in a series of patients born with an encephalocele. A retrospective analysis was undertaken of patients treated for encephaloceles at Children's Hospital Los Angeles between 1994 and 2012. The following factors were evaluated for their prognostic value: age at presentation, sex, location of encephalocele, size, contents, microcephaly, presence of hydrocephalus, CSF leak, associated cranial anomalies, and neurological outcome. Seventy children were identified, including 38 girls and 32 boys. The median age at presentation was 2 months. The mean follow-up duration was 3.7 years. Encephalocele location was classified as anterior (n = 14) or posterior (n = 56) to the coronal suture. The average maximum encephalocele diameter was 4 cm (range 0.5-23 cm). Forty-seven encephaloceles contained neural tissue. Eight infants presented at birth with CSF leaking from the encephalocele, with 1 being infected. Six patients presented with hydrocephalus, while 11 developed progressive hydrocephalus postoperatively. On univariate analysis, the presence of neural tissue, cranial anomalies, encephalocele size of at least 2 cm, seizure disorder, and microcephaly were each positively associated with hydrocephalus. On multivariate logistic regression modeling, the single prognostic factor for hydrocephalus of borderline statistical significance was the presence of neural tissue (odds ratio [OR] = 5.8, 95% confidence interval [CI] = 0.8-74.0). Fourteen patients had severe developmental delay, 28 had mild/moderate delay, and 28 were neurologically normal. On univariate analysis, the presence of cranial anomalies, larger size of encephalocele, hydrocephalus, and microcephaly were positively associated with neurological deficit. In the multivariable model, the only statistically significant prognostic factor for neurological deficit was presence of

SNX27 Deletion Causes Hydrocephalus by Impairing Ependymal Cell Differentiation and Ciliogenesis

PubMed Central

Zhou, Ying; Wang, Jian; Tseng, I-Chu; Huang, Timothy; Zhao, Yingjun; Zheng, Qiuyang; Gao, Yue; Luo, Hong; Zhang, Xian; Bu, Guojun; Hong, Wanjin

2016-01-01

Hydrocephalus is a brain disorder derived from CSF accumulation due to defects in CSF clearance. Although dysfunctional apical cilia in the ependymal cell layer are causal to the onset of hydrocephalus, mechanisms underlying proper ependymal cell differentiation are largely unclear. SNX27 is a trafficking component required for normal brain function and was shown previously to suppress γ-secretase-dependent amyloid precursor protein and Notch cleavage. However, it was unclear how SNX27-dependent γ-secretase inhibition could contribute to brain development and pathophysiology. Here, we describe and characterize an Snx27-deleted mouse model for the ependymal layer defects of deciliation and hydrocephalus. SNX27 deficiency results in reductions in ependymal cells and cilia density, as well as severe postnatal hydrocephalus. Inhibition of Notch intracellular domain signaling with γ-secretase inhibitors reversed ependymal cells/cilia loss and dilation of lateral ventricles in Snx27-deficient mice, giving strong indication that Snx27 deletion triggers defects in ependymal layer formation and ciliogenesis through Notch hyperactivation. Together, these results suggest that SNX27 is essential for ependymal cell differentiation and ciliogenesis, and its deletion can promote hydrocephalus pathogenesis. SIGNIFICANCE STATEMENT Down's syndrome (DS) in humans and mouse models has been shown previously to confer a high risk for the development of pathological hydrocephalus. Because we have previously described SNX27 as a component that is consistently downregulated in DS, we present here a robust Snx27-deleted mouse model that produces hydrocephalus and associated ciliary defects with complete penetrance. In addition, we find that γ-secretase/Notch modulation may be a candidate drug target in SNX27-associated hydrocephalus such as that observed in DS. Based on these findings, we anticipate that future study will determine whether modulation of a SNX27/Notch

Congenital Hydrocephalus.

PubMed

Estey, Chelsie M

2016-03-01

There are several types of hydrocephalus, which are characterized based on the location of the cerebrospinal fluid (CSF) accumulation. Physical features of animals with congenital hydrocephalus may include a dome-shaped skull, persistent fontanelle, and bilateral ventrolateral strabismus. Medical therapy involves decreasing the production of CSF. The most common surgical treatment is placement of a ventriculoperitoneal shunt. Postoperative complications may include infection, blockage, drainage abnormalities, and mechanical failure. Copyright © 2016 Elsevier Inc. All rights reserved.

Cognitive disorders in children's hydrocephalus.

PubMed

Zielińska, Dorota; Rajtar-Zembaty, Anna; Starowicz-Filip, Anna

Hydrocephalus is defined as an increase of volume of cerebrospinal fluid in the ventricular system of the brain. It develops as a result of cerebrospinal fluid flow disorder due to dysfunctions of absorption or, less frequently, as a result of the increase of its production. Hydrocephalus may lead to various cognitive dysfunctions in children. In order to determine cognitive functioning in children with hydrocephalus, the authors reviewed available literature while investigating this subject. The profile of cognitive disorders in children with hydrocephalus may include a wide spectrum of dysfunctions and the process of neuropsychological assessment may be very demanding. The most frequently described cognitive disorders within children's hydrocephalus include attention, executive, memory, visual, spatial or linguistic dysfunctions, as well as behavioral problems. Copyright © 2017 Polish Neurological Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.

Comparison of hydrocephalus appearance at spinaldysraphia.

PubMed

Elshani, Besnik; Lenjani, Basri

2013-01-01

Congenital malformation of spinal dysraphism followed by hydrocephalus are phenomenon reveals during intrauterine child growth. Prime objective of this work was to present Comparison of hydrocephalus appearance at spinal dysraphism respectively at its meningocele and myelomeningocele forms in Neurosurgery Clinic in UCC in Prishtina. It is perfected with retrospective and prospective method precisely of its epidemiologic part summarizing notices from patients' histories which in 2000-2006 are hospitalized in Neurosurgery Clinic from (QFLPK)--Pediatric Clinic and Children Box (Department)--Gynecology Clinic and from Sanitary Regional Center throughout Kosova. Our study objects were two groups, as the first group 90 patients with spinal dysraphism where neurosurgery operations were done and classified types of dysraphism. At myelomeningocele hydrocephalus has dominated and in a percent of appearance and as acute of its active form was 97% of hydrocephalus form where subjected to cerebrospinal liquid derivation with ventriculo -peritoneal shunt in comparison with meningocele we do not have involvation of spinal nerve element, hydrocephalus takes active form with intervention indication in 60% of cases. Reflection in shown deficit aspect is totally different at myelomeningoceles where lower paraplegia dominate more than paraparesis. The second patient operative technique developed by hydrocephalus with neurosurgical intervention indication has to do with placing of (VP) ventriculo- peritoneal system (shant) at myelomeningoceles with hydrocephalus 58 cases and 12 cases meningoceles with hydrocephalus. Post operative meningitis (shant meningitis): from 70 operated cases of hydrocephalus with spinal dysraphism shunts complications from all types are just cases. Finally that appearance of hydrocephalus compared at spinal dysraphism dominate at myellomeningoceles as in notice time aspect, it is persisting and further acute, with vital motivation for neurosurgical

MR venography in idiopathic intracranial hypertension: unappreciated and misunderstood

PubMed Central

Higgins, J; Gillard, J; Owler, B; Harkness, K; Pickard, J

2004-01-01

Background: Venous sinus disease must be excluded before diagnosing idiopathic intracranial hypertension but is found only rarely in typical cases. Magnetic resonance venography (MRV) is the technique of choice for investigating this, and provides images that are diagnostic and easy to interpret. However, recent work using more invasive techniques has documented pressure gradients and stenoses in the lateral venous sinuses in many cases of idiopathic intracranial hypertension. Objective: To examine the reason for this discrepancy and to establish whether there are characteristic appearances on MRV in idiopathic intracranial hypertension that are routinely overlooked in clinical practice. Methods: MRVs from 20 patients with idiopathic intracranial hypertension were reviewed, unblinded, by two neuroradiologists, and their appearances rated for focal narrowings and signal gaps. A control group of 40 asymptomatic volunteers, matched for age and sex with the patient group, was recruited prospectively for MRV, and their scans rated in the same way. Results: The lateral sinuses presented a range of appearances with quite different distributions in the two groups (p<0.001). Bilateral lateral sinus flow gaps were seen in 13 of 20 patients with idiopathic intracranial hypertension and in none of 40 controls. Conclusions: A historical failure to use normal healthy controls to establish the boundaries between imaging artefact, normal anatomical variant, and disease means that the pathological significance of the different appearances of the lateral sinuses on MRV has not so far been appreciated. PMID:15026510

Outcome prediction of third ventriculostomy: a proposed hydrocephalus grading system.

PubMed

Kehler, U; Regelsberger, J; Gliemroth, J; Westphal, M

2006-08-01

An important factor in making a recommendation for different treatment modalities in hydrocephalus patients (VP shunt versus endoscopic third ventriculostomy) is the definition of the underlying pathology which determines the prognosis/outcome of the surgical procedure. Third ventriculostomies (3rd VS) are successful mainly in obstructive hydrocephalus but also in some subtypes of communicating hydrocephalus. A simple, easily applicable grading system that is designed to predict the outcome of 3rd VS is proposed. The hydrocephalus is graded on the basis of the extent of downward bulging of the floor of the third ventricle, which reflects the pressure gradient between the 3rd ventricle and the basal cisterns, presence of directly visualised CSF pathway obstruction in MRI, and the progression of the clinical symptoms resulting in five different grades. In this proposed grading system, grade 1 hydrocephalus subtype shows no downward bulged floor of the 3rd ventricle, no obstruction of the CSF pathway, and no progressive symptoms of hydrocephalus. There is no indication for 3rd VS. Grades 2 to 4 show different combinations of the described parameters. Grade 5 subtype shows a markedly downward bulged floor of the 3rd ventricle and direct detection of the CSF pathway obstruction (i.e., aqueductal stenosis) with progressive clinical deterioration. Retrospective application of this grading scheme to a series of 72 3rd VS has demonstrated a high correlation with the outcome: The success rate in grade 3 reached 40%, in grade 4: 58%, and in grade 5: 95%. This standardised grading system predicts the outcome of 3rd VS and helps in decision making for 3rd VS versus VP shunting.

Cerebrospinal fluid hypersecretion in pediatric hydrocephalus.

PubMed

Karimy, Jason K; Duran, Daniel; Hu, Jamie K; Gavankar, Charuta; Gaillard, Jonathan R; Bayri, Yasar; Rice, Hunter; DiLuna, Michael L; Gerzanich, Volodymyr; Marc Simard, J; Kahle, Kristopher T

2016-11-01

Hydrocephalus, despite its heterogeneous causes, is ultimately a disease of disordered CSF homeostasis that results in pathological expansion of the cerebral ventricles. Our current understanding of the pathophysiology of hydrocephalus is inadequate but evolving. Over this past century, the majority of hydrocephalus cases has been explained by functional or anatomical obstructions to bulk CSF flow. More recently, hydrodynamic models of hydrocephalus have emphasized the role of abnormal intracranial pulsations in disease pathogenesis. Here, the authors review the molecular mechanisms of CSF secretion by the choroid plexus epithelium, the most efficient and actively secreting epithelium in the human body, and provide experimental and clinical evidence for the role of increased CSF production in hydrocephalus. Although the choroid plexus epithelium might have only an indirect influence on the pathogenesis of many types of pediatric hydrocephalus, the ability to modify CSF secretion with drugs newer than acetazolamide or furosemide would be an invaluable component of future therapies to alleviate permanent shunt dependence. Investigation into the human genetics of developmental hydrocephalus and choroid plexus hyperplasia, and the molecular physiology of the ion channels and transporters responsible for CSF secretion, might yield novel targets that could be exploited for pharmacotherapeutic intervention.

Epidemiology of Benign External Hydrocephalus in Norway-A Population-Based Study.

PubMed

Wiig, Ulrikke S; Zahl, Sverre M; Egge, Arild; Helseth, Eirik; Wester, Knut

2017-08-01

Benign external hydrocephalus is defined as a rapidly increasing head circumference (occipitofrontal circumference) with characteristic radiological findings of increased subarachnoid cerebrospinal fluid spaces on neuroimaging. The incidence of benign external hydrocephalus has not been previously reported, and there is no available information on the ratio of benign external hydrocephalus in the population of hydrocephalic children. This study is retrospective and population-based study, geographically covering two health regions in the southern half of Norway with a total mean population of 3.34 million in the ten-year study period, constituting approximately 75% of the Norwegian population. Children with a head circumference crossing two percentiles, or greater than the 97.5th percentile, and with typical imaging findings of enlarged frontal subarachnoid spaces with or without enlarged ventricles were included. Children were excluded if they had a history of head trauma, intracranial hemorrhage, central nervous system infection, other known causes of hydrocephalus, or were born preterm defined as birth before 37 weeks of gestation. A total of 176 children fitting the criteria were identified, giving an incidence of 0.4 per 1000 live births. One hundred fifty-two (86.4%) of the patients were male, and mean age at referral was 7.3 months. Increasing head circumference was the main reason for referral in 158 (89.8%) patients and the only finding in 60 (34.1%) patients. Thirty-seven (21%) children had normal ventricles on imaging; the remainder had increased ventricular size. The incidence of pediatric hydrocephalus in Norway is reported to be 0.75 per 1000 live births, thus benign external hydrocephalus accounts for approximately 50% of hydrocephalic conditions in this population. The incidence of benign external hydrocephalus was found to be 0.4 per 1000 live births in this population. Copyright © 2017 The Authors. Published by Elsevier Inc. All rights

SNX27 Deletion Causes Hydrocephalus by Impairing Ependymal Cell Differentiation and Ciliogenesis.

PubMed

Wang, Xin; Zhou, Ying; Wang, Jian; Tseng, I-Chu; Huang, Timothy; Zhao, Yingjun; Zheng, Qiuyang; Gao, Yue; Luo, Hong; Zhang, Xian; Bu, Guojun; Hong, Wanjin; Xu, Huaxi

2016-12-14

Hydrocephalus is a brain disorder derived from CSF accumulation due to defects in CSF clearance. Although dysfunctional apical cilia in the ependymal cell layer are causal to the onset of hydrocephalus, mechanisms underlying proper ependymal cell differentiation are largely unclear. SNX27 is a trafficking component required for normal brain function and was shown previously to suppress γ-secretase-dependent amyloid precursor protein and Notch cleavage. However, it was unclear how SNX27-dependent γ-secretase inhibition could contribute to brain development and pathophysiology. Here, we describe and characterize an Snx27-deleted mouse model for the ependymal layer defects of deciliation and hydrocephalus. SNX27 deficiency results in reductions in ependymal cells and cilia density, as well as severe postnatal hydrocephalus. Inhibition of Notch intracellular domain signaling with γ-secretase inhibitors reversed ependymal cells/cilia loss and dilation of lateral ventricles in Snx27-deficient mice, giving strong indication that Snx27 deletion triggers defects in ependymal layer formation and ciliogenesis through Notch hyperactivation. Together, these results suggest that SNX27 is essential for ependymal cell differentiation and ciliogenesis, and its deletion can promote hydrocephalus pathogenesis. Down's syndrome (DS) in humans and mouse models has been shown previously to confer a high risk for the development of pathological hydrocephalus. Because we have previously described SNX27 as a component that is consistently downregulated in DS, we present here a robust Snx27-deleted mouse model that produces hydrocephalus and associated ciliary defects with complete penetrance. In addition, we find that γ-secretase/Notch modulation may be a candidate drug target in SNX27-associated hydrocephalus such as that observed in DS. Based on these findings, we anticipate that future study will determine whether modulation of a SNX27/Notch/γ-secretase pathway can also be of

Concurrent Alzheimer's pathology in patients with clinical normal pressure hydrocephalus: correlation of high-volume lumbar puncture results, cortical brain biopsies, and outcomes.

PubMed

Pomeraniec, I Jonathan; Bond, Aaron E; Lopes, M Beatriz; Jane, John A

2016-02-01

Normal pressure hydrocephalus (NPH) remains most often a clinical diagnosis and has been widely considered responsive to the placement of a cerebrospinal fluid (CSF) shunt. The high incidence of patients with Alzheimer's disease (AD) with NPH symptoms leads to poorer outcomes than would be expected in patients with NPH alone. This article reviews a series of patients operated on for presumed NPH in whom preoperative high-volume lumbar puncture (HVLP) and intraoperative cortical brain biopsies were performed. The data derived from these procedures were then used to understand the incidence of AD in patients presenting with NPH symptoms and to analyze the efficacy of HVLP in patients with NPH and patients with concurrent AD (NPH+AD). A review of the outcomes of shunt surgery is provided. The cases of all patients who underwent placement of a CSF shunt for NPH from 1998 to 2013 at the University of Virginia by the senior author were retrospectively reviewed. Patients who underwent HVLP and patients who underwent cortical brain biopsies were stratified based on the biopsy results into an NPH-only group and an NPH+AD group. The HVLP results and outcomes were then compared in these 2 groups. From 1998 to 2013, 142 patients underwent shunt operations because of a preoperative clinical diagnosis of NPH. Of the patients with a shunt who had a diagnosis of NPH, 105 (74%) received HVLPs. Of 142 shunt-treated patients with NPH, 27 (19%) were determined to have concomitant Alzheimer's pathology based on histopathological findings at the time of shunting. Patients who underwent repeat biopsies had an initial positive outcome. After they clinically deteriorated, they underwent repeat biopsies during shunt interrogation, and 13% of the repeat biopsies demonstrated Alzheimer's pathology. Improvements in gait and cognition did not reach significance between the NPH and NPH+AD groups. In total, 105 patients underwent HVLP before shunt placement. In the NPH cohort, 44.6% of patients

Application of MR virtual endoscopy in children with hydrocephalus.

PubMed

Zhao, Cailei; Yang, Jian; Gan, Yungen; Liu, Jiangang; Tan, Zhen; Liang, Guohua; Meng, Xianlei; Sun, Longwei; Cao, Weiguo

2015-12-01

To evaluate the performance of MR virtual endoscopy (MRVE) in children with hydrocephalus. Clinical and imaging data were collected from 15 pediatric patients with hydrocephalus and 15 normal control children. All hydrocephalus patients were confirmed by ventriculoscopy or CT imaging. The cranial 3D-T1 weighted imaging data from fast spoiled gradient echo scan (FSPGR) were transported to working station. VE images of cerebral ventricular cavity were constructed with Navigator software. Cerebral ventricular MRVE can achieve similar results as ventriculoscopy in demonstrating the morphology of ventricular wall or intracavity lesion. In addition, MRVE can observe the lesion from distal end of obstruction, as well as other areas that are inaccessible to ventriculoscopy. MRVE can also reveal the pathological change of ventricular inner wall surface, and help determine patency of the cerebral aqueduct and fourth ventricle outlet. MR virtual endoscopy provides a non-invasive diagnostic modality that can be used as a supplemental approach to ventriculoscopy. However, its sensitivity and specificity need to be determined in the large study. Copyright © 2015 Elsevier Inc. All rights reserved.

Hydrocephalus: the role of cerebral aquaporin-4 channels and computational modeling considerations of cerebrospinal fluid.

PubMed

Desai, Bhargav; Hsu, Ying; Schneller, Benjamin; Hobbs, Jonathan G; Mehta, Ankit I; Linninger, Andreas

2016-09-01

Aquaporin-4 (AQP4) channels play an important role in brain water homeostasis. Water transport across plasma membranes has a critical role in brain water exchange of the normal and the diseased brain. AQP4 channels are implicated in the pathophysiology of hydrocephalus, a disease of water imbalance that leads to CSF accumulation in the ventricular system. Many molecular aspects of fluid exchange during hydrocephalus have yet to be firmly elucidated, but review of the literature suggests that modulation of AQP4 channel activity is a potentially attractive future pharmaceutical therapy. Drug therapy targeting AQP channels may enable control over water exchange to remove excess CSF through a molecular intervention instead of by mechanical shunting. This article is a review of a vast body of literature on the current understanding of AQP4 channels in relation to hydrocephalus, details regarding molecular aspects of AQP4 channels, possible drug development strategies, and limitations. Advances in medical imaging and computational modeling of CSF dynamics in the setting of hydrocephalus are summarized. Algorithmic developments in computational modeling continue to deepen the understanding of the hydrocephalus disease process and display promising potential benefit as a tool for physicians to evaluate patients with hydrocephalus.

Hydrocephalus

MedlinePlus

... support and help with the care of a child with hydrocephalus who has serious brain damage. ... such as meningitis or encephalitis Intellectual impairment Nerve damage (decrease in movement, sensation, function) Physical disabilities

Demonstration of uneven distribution of intracranial pulsatility in hydrocephalus patients.

PubMed

Eide, Per K

2008-11-01

Data from intracranial pressure (ICP) recordings in patients with hydrocephalus were reviewed to determine whether intracranial pulsatility within the cerebrospinal fluid (CSF) of cerebral ventricles (ICP(LV)) may differ from that within the brain parenchyma (ICP(PAR)), and whether pulsatility may differ between noncommunicating ventricles. The authors retrieved data from recordings previously obtained in 7 patients with hydrocephalus (noncommunicating in 4 and communicating in 3) and shunt failure who received both an external ventricular drainage (EVD) and an ICP sensor as part of surveillance during intensive care. Simultaneous ICP(LV) and ICP(PAR) signals were available in 6 cases, and simultaneous signals from the lateral and fourth ventricles (ICP(LV) and ICP4V, respectively) were recorded in 1 case. The recordings with both signals were parsed into 6-second time windows. Pulsatility was characterized by the wave amplitude and rise time coefficient, and differences in pulsatility between the ICP(LV) and ICP(PAR) signals (6 cases) or ICP(LV) and ICP4V signals (1 case) were determined. There was uneven distribution of intracranial pulsatility in all 7 patients, shown as significantly elevated pulsatility (that is, higher wave amplitudes and rise time coefficients) within the ventricles (ICP(LV)) than within brain parenchyma (ICP(PAR)) in 6 patients, and significantly higher pulsatility in the fourth (ICP4V) than in the lateral (ICP(LV)) ventricles in 1 patient. Differences > or = 1 mm Hg in ICP wave amplitude were found in 0.5-100% (median 9.4%) of observations in the 7 patients (total number of 6-second time windows, 68,242). The present observations demonstrate uneven distribution of intracranial pulsatility in patients with hydrocephalus, higher pulse pressure amplitudes within the ventricular CSF (ICP(LV)) than within the brain parenchyma (ICP(PAR)). This may be one mechanism behind ventricular enlargement in hydrocephalus.

Radiologically occult medulloblastoma with hydrocephalus: case report.

PubMed

Honma, Hirokuni; Ogiwara, Hideki

2017-09-01

There have been no reports of occult medulloblastoma nor noncommunicating hydrocephalus due to radiologically occult brain tumors. Herein, we report radiologically occult medulloblastoma with noncommunicating hydrocephalus. A 3-year-old boy presented with macrocephaly, visual field constriction, and papilledema. Neuroimagings showed enlargement of the ventricles without any mass lesions. The CT cisternography did not show influx of the contrast into the ventricles, which suggested local cerebrospinal fluid (CSF) circulatory disturbance at the outlet of the fourth ventricle. Due to possible obstructive nature of hydrocephalus, endoscopic third ventriculostomy (ETV) was performed. Three months after the ETV, he presented with repeated vomiting. Neuroimagings showed a 3-cm fourth ventricular mass with progressive hydrocephalus. Surgical resection was performed, which revealed the pathology was medulloblastoma. We report the case of radiologically occult medulloblastoma which was demonstrated radiologically in the follow-up period of ETV for noncommunicating hydrocephalus of uncertain etiology. This is the first description of a radiologically occult medulloblastoma and also the first description of an occult brain tumor with noncommunicating hydrocephalus. The occult brain tumor may be included in the etiology of hydrocephalus.

The combination of a programmable valve and a subclavicular anti-gravity device in hydrocephalus patients at high risk for hygromas.

PubMed

Zachenhofer, Iris; Donat, Markus; Roessler, Karl

2012-04-01

In order to avoid occurrence of post-operative hygromas in specific hydrocephalus patients being at high risk of overdrainage, a combination of programmable valve and anti-gravity device is widely recommended. We analyzed our series of hydrocephalus patients implanted with such a shunt configuration focusing on complications in relation to over-/underdrainage and neurological outcome. In 28 hydrocephalic patients (14 women and 14 men; mean age 65 years, range from 14 to 82 years; 11 normal pressure, 7 post-traumatic, and 4 post-hemorrhagic hydrocephalus), a Codman Medos programmable valve combined with a Miethke shunt assistant (SA) was implanted at the Department of Neurosurgery of the Academic Teaching Hospital Feldkirch. Implantation was performed simultaneously in 20 patients during the primary procedure: in five patients, SA was placed during revision surgery, and in three patients, the patent system was completed by additional implantation of an SA. Subdural hematoma occurred in one out of 20 patients with SA implantation during primary procedure and in two out of eight patients with SA implantation as secondary procedure, respectively. Shunt occlusion occurred in one patient out of the patients with SA implantation during primary procedure, but was seen in three patients with pre-existing shunt without SA. Shunt infection occurred in one case. Our results suggest the combination of an adjustable valve and SA as an effective treatment for a specific group of hydrocephalus patients being at high risk for overdrainage.

Control volume based hydrocephalus research

NASA Astrophysics Data System (ADS)

Cohen, Benjamin; Voorhees, Abram; Wei, Timothy

2008-11-01

Hydrocephalus is a disease involving excess amounts of cerebral spinal fluid (CSF) in the brain. Recent research has shown correlations to pulsatility of blood flow through the brain. However, the problem to date has presented as too complex for much more than statistical analysis and understanding. This talk will highlight progress on developing a fundamental control volume approach to studying hydrocephalus. The specific goals are to select physiologically control volume(s), develop conservation equations along with the experimental capabilities to accurately quantify terms in those equations. To this end, an in vitro phantom is used as a simplified model of the human brain. The phantom's design consists of a rigid container filled with a compressible gel. The gel has a hollow spherical cavity representing a ventricle and a cylindrical passage representing the aquaducts. A computer controlled piston pump supplies pulsatile volume fluctuations into and out of the flow phantom. MRI is used to measure fluid velocity, and volume change as functions of time. Independent pressure measurements and flow rate measurements are used to calibrate the MRI data. These data are used as a framework for future work with live patients.

Shortfalls in pediatric hydrocephalus clinical outcome analysis.

PubMed

Walid, Mohammad Sami; Robinson, Joe Sam

2012-06-01

In this paper, we used search engine technology to study outcome analysis and cost awareness of child hydrocephalus in the literature. The aggregate hospital charges of hydrocephalus treatment procedures for patients <18 years old was extracted from the Nationwide Inpatient Sample (NIS) data. Hydrocephalus literature was probed through the PubMed biomedical search engine. Aggregate hospital charges associated with ventriculo-peritoneal shunting as the principle procedure for patients <18 years old have increased 1.7-fold over a 13-year period to 235.6 million in 2009. Hospital discharges, however, decreased from 3,390 in 1997 to 2,525 in 2009 (25.5% decrease over 13 years). The number of papers in English language indexed by PubMed in relation to child hydrocephalus in humans increased from 81 papers in 1996 to 133 in 2010 (1.6-fold increase), totaling 1,694 over 15 years. Randomized controlled trials published in relation to child hydrocephalus totaled 16 over the same period (0.94% of child hydrocephalus papers). Papers related to child hydrocephalus with "costs and cost analysis" as medical subject heading totaled 13 papers (0.77%). Over the past 15 years, disappointingly the number of printed child hydrocephalus papers appeared to have only plateaued. Strikingly, only a very small number of these papers were directed toward randomized control studies, the sine qua non of high-grade clinical evidence. Moreover, very few papers make reference to cost analysis or economics in the treatment of hydrocephalus - an issue coming increasingly before the nation at this point.

Mathematics skills in good readers with hydrocephalus.

PubMed

Barnes, Marcia A; Pengelly, Sarah; Dennis, Maureen; Wilkinson, Margaret; Rogers, Tracey; Faulkner, Heather

2002-01-01

Children with hydrocephalus have poor math skills. We investigated the nature of their arithmetic computation errors by comparing written subtraction errors in good readers with hydrocephalus, typically developing good readers of the same age, and younger children matched for math level to the children with hydrocephalus. Children with hydrocephalus made more procedural errors (although not more fact retrieval or visual-spatial errors) than age-matched controls; they made the same number of procedural errors as younger, math-level matched children. We also investigated a broad range of math abilities, and found that children with hydrocephalus performed more poorly than age-matched controls on tests of geometry and applied math skills such as estimation and problem solving. Computation deficits in children with hydrocephalus reflect delayed development of procedural knowledge. Problems in specific math domains such as geometry and applied math, were associated with deficits in constituent cognitive skills such as visual spatial competence, memory, and general knowledge.

Parvovirus associated cerebellar hypoplasia and hydrocephalus in day-old broiler chickens

USDA-ARS?s Scientific Manuscript database

Cerebellar hypoplasia and hydrocephalus were detected in day-old broiler chickens. Brains of chickens evaluated at necropsy appeared to be abnormal; some were disfigured and cerebellae appeared to be smaller than normal. Histopathologic examination of brains revealed cerebellar folia that were sho...

Constitutive Cyclin O deficiency results in penetrant hydrocephalus, impaired growth and infertility

PubMed Central

Núnez-Ollé, Marc; Jung, Carole; Terré, Berta; Balsiger, Norman A.; Plata, Cristina; Roset, Ramon; Pardo-Pastor, Carlos; Garrido, Marta; Rojas, Santiago; Alameda, Francesc; Lloreta, Josep; Martín-Caballero, Juan; Flores, Juana M.; Stracker, Travis H.; Valverde, Miguel A.; Muñoz, Francisco J.; Gil-Gómez, Gabriel

2017-01-01

Cyclin O (encoded by CCNO) is a member of the cyclin family with regulatory functions in ciliogenesis and apoptosis. Homozygous CCNO mutations have been identified in human patients with Reduced Generation of Multiple Motile Cilia (RGMC) and conditional inactivation of Ccno in the mouse recapitulates some of the pathologies associated with the human disease. These include defects in the development of motile cilia and hydrocephalus. To further investigate the functions of Ccno in vivo, we have generated a new mouse model characterized by the constitutive loss of Ccno in all tissues and followed a cohort during ageing. Ccno-/- mice were growth impaired and developed hydrocephalus with high penetrance. In addition, some Ccno+/- mice also developed hydrocephalus and affected Ccno-/- and Ccno+/- mice exhibited additional CNS defects including cortical thinning and hippocampal abnormalities. In addition to the CNS defects, both male and female Ccno-/- mice were infertile and female mice exhibited few motile cilia in the oviduct. Our results further establish CCNO as an important gene for normal development and suggest that heterozygous CCNO mutations could underlie hydrocephalus or diminished fertility in some human patients. PMID:29245899

Constitutive Cyclin O deficiency results in penetrant hydrocephalus, impaired growth and infertility.

PubMed

Núnez-Ollé, Marc; Jung, Carole; Terré, Berta; Balsiger, Norman A; Plata, Cristina; Roset, Ramon; Pardo-Pastor, Carlos; Garrido, Marta; Rojas, Santiago; Alameda, Francesc; Lloreta, Josep; Martín-Caballero, Juan; Flores, Juana M; Stracker, Travis H; Valverde, Miguel A; Muñoz, Francisco J; Gil-Gómez, Gabriel

2017-11-21

Cyclin O (encoded by CCNO ) is a member of the cyclin family with regulatory functions in ciliogenesis and apoptosis. Homozygous CCNO mutations have been identified in human patients with Reduced Generation of Multiple Motile Cilia (RGMC) and conditional inactivation of Ccno in the mouse recapitulates some of the pathologies associated with the human disease. These include defects in the development of motile cilia and hydrocephalus. To further investigate the functions of Ccno in vivo , we have generated a new mouse model characterized by the constitutive loss of Ccno in all tissues and followed a cohort during ageing. Ccno -/- mice were growth impaired and developed hydrocephalus with high penetrance. In addition, some Ccno +/- mice also developed hydrocephalus and affected Ccno -/- and Ccno +/- mice exhibited additional CNS defects including cortical thinning and hippocampal abnormalities. In addition to the CNS defects, both male and female Ccno -/- mice were infertile and female mice exhibited few motile cilia in the oviduct. Our results further establish CCNO as an important gene for normal development and suggest that heterozygous CCNO mutations could underlie hydrocephalus or diminished fertility in some human patients.

The genetic landscape of familial congenital hydrocephalus.

PubMed

Shaheen, Ranad; Sebai, Mohammed Adeeb; Patel, Nisha; Ewida, Nour; Kurdi, Wesam; Altweijri, Ikhlass; Sogaty, Sameera; Almardawi, Elham; Seidahmed, Mohammed Zain; Alnemri, Abdulrahman; Madirevula, Sateesh; Ibrahim, Niema; Abdulwahab, Firdous; Hashem, Mais; Al-Sheddi, Tarfa; Alomar, Rana; Alobeid, Eman; Sallout, Bahauddin; AlBaqawi, Badi; AlAali, Wajeih; Ajaji, Nouf; Lesmana, Harry; Hopkin, Robert J; Dupuis, Lucie; Mendoza-Londono, Roberto; Al Rukban, Hadeel; Yoon, Grace; Faqeih, Eissa; Alkuraya, Fowzan S

2017-06-01

Congenital hydrocephalus is an important birth defect, the genetics of which remains incompletely understood. To date, only 4 genes are known to cause Mendelian diseases in which congenital hydrocephalus is the main or sole clinical feature, 2 X-linked (L1CAM and AP1S2) and 2 autosomal recessive (CCDC88C and MPDZ). In this study, we aimed to determine the genetic etiology of familial congenital hydrocephalus with the assumption that these cases represent Mendelian forms of the disease. Exome sequencing combined, where applicable, with positional mapping. We identified a likely causal mutation in the majority of these families (21 of 27, 78%), spanning 16 genes, none of which is X-linked. Ciliopathies and dystroglycanopathies were the most common etiologies of congenital hydrocephalus in our cohort (19% and 26%, respectively). In 1 family with 4 affected members, we identified a homozygous truncating variant in EML1, which we propose as a novel cause of congenital hydrocephalus in addition to its suggested role in cortical malformation. Similarly, we show that recessive mutations in WDR81, previously linked to cerebellar ataxia, mental retardation, and disequilibrium syndrome 2, cause severe congenital hydrocephalus. Furthermore, we confirm the previously reported candidacy of MPDZ by presenting a phenotypic spectrum of congenital hydrocephalus associated with 5 recessive alleles. Our study highlights the importance of recessive mutations in familial congenital hydrocephalus and expands the locus heterogeneity of this condition. Ann Neurol 2017;81:890-897. © 2017 American Neurological Association.

LONG-TERM INTRAOCULAR PRESSURE AFTER UNCOMPLICATED PARS PLANA VITRECTOMY FOR IDIOPATHIC EPIRETINAL MEMBRANE.

PubMed

Tognetto, Daniele; Pastore, Marco R; Cirigliano, Gabriella; DʼAloisio, Rossella; Borelli, Massimo; De Giacinto, Chiara

2017-11-16

To investigate long-term intraocular pressure trends after uncomplicated pars plana vitrectomy for idiopathic epiretinal membrane. Three hundred and sixty-eight eyes of 368 consecutive patients were enrolled. Changes in intraocular pressure 1, 3, 6, and 12 months after surgery and during the final follow-up visit were evaluated in vitrectomized eyes and nonvitrectomized fellow eyes. The median follow-up period was 36 months (range 12-92 months). Longitudinal data analysis evidenced a 2.5-mmHg (2.2 mmHg; 2.7 mmHg, 95% confidence interval) statistically significant difference in intraocular pressure 30 days after surgery between treated and fellow untreated eyes, gradually recovering to a not significant 0.2-mmHg (-0.1 mmHg; 0.4 mmHg, 95% confidence interval) difference within 26 months. The incidence of late-onset ocular hypertension was 5.7% (21 over 347, 2%; 12%, 95% confidence interval) without difference between the treated eyes and the group control. No significant difference in the incidence of late-onset ocular hypertension and sex, lens status, or gauge of vitrectomy instruments was detected. Only patient's age was significantly higher (mean difference 4.2 years; 0.1-8.0 years, Monte Carlo, 95% confidence interval) in those who developed late-onset ocular hypertension in the vitrectomized eye. Uncomplicated pars plana vitrectomy for idiopathic epiretinal membrane seems not to increase the risk of late-onset ocular hypertension or open-angle glaucoma development.

Posthemispherectomy hydrocephalus: results of a comprehensive, multiinstitutional review.

PubMed

Lew, Sean M; Matthews, Anne E; Hartman, Adam L; Haranhalli, Neil

2013-02-01

Hemispherectomy surgery for medically intractable epilepsy is known to cause hydrocephalus in a subset of patients. Existing data regarding the incidence of, and risk factors for, developing posthemispherectomy hydrocephalus have been limited by the relatively small number of cases performed by any single center. Our goal was to better understand this phenomenon and to identify risk factors that may predispose patients to developing hydrocephalus after hemispherectomy surgery. Fifteen pediatric epilepsy centers participated in this study. A retrospective chart review was performed on all available patients who had hemispherectomy surgery. Data collected included surgical techniques, etiology of seizures, prior brain surgery, symptoms and signs of hydrocephalus, timing of shunt placement, and basic demographics. Data were collected from 736 patients who underwent hemispherectomy surgery between 1986 and 2011. Forty-six patients had preexisting shunted hydrocephalus and were excluded from analysis, yielding 690 patients for this study. One hundred sixty-two patients (23%) required hydrocephalus treatment. The timing of hydrocephalus ranged from the immediate postoperative period to 8.5 years after surgery, with 43 patients (27%) receiving shunts >90 days after surgery. Multivariate regression analysis revealed anatomic hemispherectomies (odds ratio [OR] 4.1, p < 0.0001) and previous brain surgery (OR 1.7, p = 0.04) as independent significant risk factors for developing hydrocephalus. There was a trend toward significance for the use of hemostatic agents (OR 2.2, p = 0.07) and the involvement of basal ganglia or thalamus in the resection (OR 2.2, p = 0.08) as risk factors. Hydrocephalus is a common sequela of hemispherectomy surgery. Surgical technique and prior brain surgery influence the occurrence of posthemispherectomy hydrocephalus. A significant portion of patients develop hydrocephalus on a delayed basis, indicating the need for long-term surveillance. Wiley

Concept and treatment of hydrocephalus in the Greco-Roman and early Arabic medicine.

PubMed

Grunert, P; Charalampaki, P; Ayyad, A

2007-10-01

In the ancient medical literature hydrocephalus was not often described although its existence and symptomatology were well known. Most detailed descriptions of hydrocephalus including the surgical treatment are extant in the encyclopaedic works on medicine of the physicians Oreibasios and Aetios from Amida from the 4th and 6th centuries AD, respectively. Because of their broad scientific interests, this type of physicians, typical for the late Roman empire, were known as philosophy-physicians (iota alpha tau rho o sigma o phi iota sigma tau alpha iota). They defined hydrocephalus in contrast to our present understanding as a fluid collection excluding abscesses visible as a bulging tumour localised either outside or inside the skull of an infant. They classified the hydrocephalus similar as stated first by Galen in the 2nd century AD in four types corresponding to the assumed anatomic localisation of the fluid collection: 1st Type between the skin and the pericranium corresponding to the subgaleal haematoma or caput succedaneum of the newborn in our terminology, 2nd Type between the pericranium and the skull corresponding to the cephal haematoma after delivery, 3rd Type between skull and the meninges with increased head circumference, bone sutures being increasingly driven apart corresponding most likely to the hydrocephalus in our understanding, and 4th Type between the menings and the brain characterised by severe neurological deficit with lethal prognosis corresponding probably to all pathologies which were accompanied by an excessive increase of the intracranial pressure with a bulging fontanel. Due to the lack of autopsies in ancient times, the hydrocephalus was never linked to the pathology of the ventricles. All forms of hydrocephalus were believed to be caused by improper handling of the head by the midwife during delivery. Only the extracranial fluid collections, but not hydrocephalus in our sense, were considered to be suitable for surgical treatment

Utility of Intracranial Pressure Monitoring for Diagnosis of Idiopathic Intracranial Hypertension in the Absence of Papilledema.

PubMed

Bridges, Kelly J; Raslan, Ahmed M

2018-03-01

Idiopathic intracranial hypertension (IIH) is characterized by headaches, visual obscurations, and papilledema, and the diagnosis involves lumbar puncture (LP) with an elevated opening pressure (OP) ≥20 cm H 2 0. When papilledema is absent, the diagnosis becomes less clear. Some physicians have argued that the absence of papilledema rules out IIH, whereas others maintain that elevated OP is sufficient for diagnosis. The authors performed a single-institution 4-year retrospective analysis of patients who underwent invasive intracranial pressure (ICP) monitoring for presumed IIH. A total of 22 patients were reviewed, and 13 had classic symptoms of IIH, documented elevated OP, and absence of papilledema; 5/13 (38%) patients had proven intracranial hypertension as shown by invasive ICP monitoring, whereas 8/13 (62%) had normal ICP. With the use of current diagnostic algorithms of clinical presentation and elevated OP, over half of patients without papilledema in our series would be falsely diagnosed with IIH, which could result in unnecessary medical and surgical intervention. Thus, elevated OP as determined by LP is insufficient to diagnose IIH. On the other hand, the absence of papilledema does not rule out intracranial hypertension. Copyright © 2017 Elsevier Inc. All rights reserved.

Hydrocephalus: a rare initial manifestation of sporadic intramedullary hemangioblastoma : Intramedullary hemangioblastoma presenting as hydrocephalus.

PubMed

Morais, Barbara Albuquerque; Cardeal, Daniel Dante; Ribeiro E Ribeiro, Renan; Frassetto, Fernando Pereira; Andrade, Fernanda Goncalves; Matushita, Hamilton; Teixeira, Manoel Jacobsen

2017-08-01

Intramedullary hemangioblastomas are rare benign vascular tumors, infrequent in pediatric patients. Clinical symptoms vary according to the age of presentation, tumor size, location, and concomitant syringomyelia. This is the second reported case of hemangioblastoma presenting with acute hydrocephalus. A 3-month-old infant with acute hydrocephalus was asymptomatic after a ventriculoperitoneal shunt was placed. She returned 3 months later with irritability, acute paraplegia, and respiratory distress. Magnetic resonance imaging (MRI) showed an intramedullary T8-T9 tumor with syringomyelia. She underwent surgical resection with good results during the 6-month follow-up. Intramedullary tumors may present as hydrocephalus and other nonspecific symptoms, with invariably delayed diagnosis in children, but must be considered in suspicious cases.

Prevalence of Alzheimer's disease in patients investigated for presumed normal pressure hydrocephalus: a clinical and neuropathological study.

PubMed

Savolainen, S; Paljärvi, L; Vapalahti, M

1999-01-01

During 1991-1995, 223 patients were investigated in the Department of Neurosurgery, Kuopio University Hospital because of a clinical and CT diagnosis of NPH. All patients underwent intracranial pressure measurements and were formed into 3 biopsy groups. Group A included incidentally biopsied patients (104 patients, 34 biopsies) seen during 1991-1992; Group B was a prospective study group from 1993-1995 (all 51 patients biopsied); and Group C patients excluded from Group B (68 patients, 34 biopsies) by age and concomitant diseases. A cortical biopsy was taken before intracranial pressure recording altogether in 118 of the 223 patients. The biopsy revealed normal brain tissue in 66 patients. Prevalence of Alzheimer's disease (AD) in biopsied patients was 42% in Group A, 31.3% in Group B and 50% in Group C. A shunt was placed according to pressure measurement in 110 patients; of these, 8 had both AD and raised ICP. Two patients with both AD and raised ICP improved after shunt placement during the first follow-up year, 4 patients deteriorated and the condition of 2 was similar to that before shunting. The frequency of haematomas after biopsy was 2.9% in groups A and C; in Group B patients had no postoperative haematomas. There was no difference in the incidence of complications in patients who had or did not have a biopsy. The relatively high prevalence of AD in patients with NPH may explain the unsuccessful recovery of many patients after shunt placement. Cortical biopsy is an effective and safe method for finding the co-existence of AD and thus improving the diagnosis of NPH and may prevent unnecessary shunt surgery.

Infantile hydrocephalus: a review of epidemiology, classification and causes

PubMed Central

Tully, Hannah M; Dobyns, William B

2015-01-01

Hydrocephalus is a common but complex condition caused by physical or functional obstruction of CSF flow that leads to progressive ventricular dilatation. Though hydrocephalus was recently estimated to affect 1.1 in 1,000 infants, there have been few systematic assessments of the causes of hydrocephalus in this age group, which makes it a challenging condition to approach as a scientist or as a clinician. Here, we review contemporary literature on the epidemiology, classification and pathogenesis of infantile hydrocephalus. We describe the major environmental and genetic causes of hydrocephalus, with the goal of providing a framework to assess infants with hydrocephalus and guide future research. PMID:24932902

The Lhx9 homeobox gene controls pineal gland development and prevents postnatal hydrocephalus.

PubMed

Yamazaki, Fumiyoshi; Møller, Morten; Fu, Cong; Clokie, Samuel J; Zykovich, Artem; Coon, Steven L; Klein, David C; Rath, Martin F

2015-01-01

Lhx9 is a member of the LIM homeobox gene family. It is expressed during mammalian embryogenesis in the brain including the pineal gland. Deletion of Lhx9 results in sterility due to failure of gonadal development. The current study was initiated to investigate Lhx9 biology in the pineal gland. Lhx9 is highly expressed in the developing pineal gland of the rat with transcript abundance peaking early in development; transcript levels decrease postnatally to nearly undetectable levels in the adult, a temporal pattern that is generally similar to that reported for Lhx9 expression in other brain regions. Studies with C57BL/6J Lhx9(-/-) mutant mice revealed marked alterations in brain and pineal development. Specifically, the superficial pineal gland is hypoplastic, being reduced to a small cluster of pinealocytes surrounded by meningeal and vascular tissue. The deep pineal gland and the pineal stalk are also reduced in size. Although the brains of neonatal Lhx9(-/-) mutant mice appear normal, severe hydrocephalus develops in about 70% of the Lhx9(-/-) mice at 5-8 weeks of age; these observations are the first to document that deletion of Lhx9 results in hydrocephalus and as such indicate that Lhx9 contributes to the maintenance of normal brain structure. Whereas hydrocephalus is absent in neonatal Lhx9(-/-)mutant mice, the neonatal pineal gland in these animals is hypoplastic. Accordingly, it appears that Lhx9 is essential for early development of the mammalian pineal gland and that this effect is not secondary to hydrocephalus.

Mechanisms of Hydrocephalus after Neonatal and Adult Intraventricular Hemorrhage

PubMed Central

Strahle, Jennifer; Garton, Hugh J.L.; Maher, Cormac O.; Muraszko, Karin M.; Keep, Richard F.; Xi, Guohua

2013-01-01

Intraventricular hemorrhage (IVH) is a cause of significant morbidity and mortality and is an independent predictor of a worse outcome in intracerebral hemorrhage (ICH) and germinal matrix hemorrhage (GMH). IVH may result in both injuries to the brain as well as hydrocephalus. This paper reviews evidence on the mechanisms and potential treatments for IVH-induced hydrocephalus. One frequently cited theory to explain hydrocephalus after IVH involves obliteration of the arachnoid villi by microthrombi with subsequent inflammation and fibrosis causing CSF outflow obstruction. Although there is some evidence to support this theory, there may be other mechanisms involved, which contribute to the development of hydrocephalus. It is also unclear whether the causes of acute and chronic hydrocephalus after hemorrhage occur via different mechanisms; mechanical obstruction by blood in the former, and inflammation and fibrosis in the latter. Management of IVH and strategies for prevention of brain injury and hydrocephalus are areas requiring further study. A better understanding of the pathogenesis of hydrocephalus after IVH, may lead to improved strategies to prevent and treat post-hemorrhagic hydrocephalus. PMID:23976902

Visual outcome after endoscopic third ventriculostomy for hydrocephalus.

PubMed

Jung, Ji-Ho; Chai, Yong-Hyun; Jung, Shin; Kim, In-Young; Jang, Woo-Youl; Moon, Kyung-Sub; Kim, Seul-Kee; Chong, Sangjoon; Kim, Seung-Ki; Jung, Tae-Young

2018-02-01

Hydrocephalus-related symptoms are mostly improved after successful endoscopic third ventriculostomy (ETV). However, visual symptoms can be different. This study was focused on visual symptoms. We analyzed the magnetic resonance images (MRI) of the orbit and visual outcomes. From August 2006 to November 2016, 50 patients with hydrocephalus underwent ETV. The male-to-female ratio was 33:17, and the median age was 61 years (range, 5-74 years). There were 18 pediatric and 32 adult patients. Abnormal orbital MRI findings included prominent subarachnoid space around the optic nerves and vertical tortuosity of the optic nerves. We retrospectively analyzed clinical symptoms, causes of hydrocephalus, ETV success score (ETVSS), ETV success rate, ETV complications, orbital MRI findings, and visual impairment score (VIS). The median duration of follow-up was 59 months (range, 3-113 months). The most common symptoms were headache, vomiting, and gait disturbance. Visual symptoms were found in 6 patients (12%). The most common causes of hydrocephalus were posterior fossa tumor in 13 patients, pineal tumor in 12, aqueductal stenosis in 8, thalamic malignant glioma in 7, and tectal glioma in 4. ETVSS was 70 in 3 patients, 80 in 34 patients, and 90 in 13 patients. ETV success rate was 80%. ETVSS 70 showed the trend in short-term survival compared to ETVSS 90 and 80. ETV complications included epidural hematoma requiring operation in one patient, transient hemiparesis in two patients, and infection in two patients. Preoperative abnormal orbital MRI findings were found in 18 patients and postoperative findings in 7 patients. Four of six patients with visual symptoms had abnormal MR findings. Three patients did not show VIS improvement, including two with severe visual symptoms. Patients with severe visual impairment were found to have bad outcomes. The visual symptoms related with increased intracranial pressure should be carefully monitored and controlled to improve outcomes.

Relation between adolescent idiopathic scoliosis and morphologic somatotypes.

PubMed

LeBlanc, R; Labelle, H; Rivard, C H; Poitras, B

1997-11-01

A prospective and controlled comparative study. To verify the difference in morphologic appearance between a group of adolescents with progressive adolescent idiopathic scoliosis and a control group of normal adolescents. In a previous retrospective study, the possibility of a relation between progressive adolescent idiopathic scoliosis and specific morphotypes was demonstrated. Fifty-two adolescent girls with progressive adolescent idiopathic scoliosis were compared with an age-matched control group of 62 unaffected girls using a classification technique based on morphologic somatotypes. Morphotypes were evaluated with standardized pre-established criteria based on Sheldon's technique. Patients with progressive adolescent idiopathic scoliosis showed significantly less mesomorphism (mean value of 0.88 +/- 0.51) than control girls (mean value of 1.72 +/- 0.52). Adolescent girls with progressive adolescent idiopathic scoliosis have a morphologic somatotype that is different from the normal adolescent population. Subjects with progressive adolescent idiopathic scoliosis are significantly less mesomorphic than control girls. This observation may be of value as a predictive factor for early identification of subjects with adolescent idiopathic scoliosis at greater risk of progression.

Subdural fluid collection and hydrocephalus following cervical schwannoma resection: hydrocephalus resolution after spinal pseudomeningocele repair: case report.

PubMed

Benedetto, Nicola; Cagnazzo, Federico; Gambacciani, Carlo; Perrini, Paolo

2016-12-01

The authors report the case of a 31-year-old man who developed neck pain and headache 2 months after the uncomplicated resection of a cervical schwannoma. MR imaging revealed infratentorial subdural fluid collections and obstructive hydrocephalus associated with cervical pseudomeningocele. The clinical symptoms, subdural fluid collections, and ventricular dilation resolved after surgical correction of the pseudomeningocele. This report emphasizes that hydrocephalus may be related to disorders of cerebrospinal fluid flow dynamics induced by cervical pseudomeningocele. In these rare cases, both the hydrocephalus and the symptoms are resolved by the simple correction of the pseudomeningocele.

The pathophysiology of chronic noncommunicating hydrocephalus: lessons from continuous intracranial pressure monitoring and ventricular infusion testing.

PubMed

Eide, Per Kristian

2017-08-11

OBJECTIVE The pathophysiology of chronic noncommunicating hydrocephalus (ncHC) is poorly understood. This present study explored whether lessons about the pathophysiology of this clinical entity might be retrieved from results of overnight monitoring of pulsatile and static intracranial pressure (ICP) and ventricular infusion testing. METHODS The study cohort included adult patients (> 20 years of age) with chronic ncHC due to aqueductal stenosis in whom symptoms had lasted a minimum of 6 months. A reference cohort consisted of age- and sex-matched patients managed for communicating HC (cHC). Information about symptoms and clinical improvement following surgery was retrieved from a quality register, and results of overnight ICP recordings and ventricular infusion testing were retrieved from the hospital ICP database. RESULTS The cohort with ncHC consisted of 61 patients of whom 6 (10%) were managed conservatively, 34 (56%) by endoscopic third ventriculostomy (ETV), and 21 (34%) using ETV and subsequent shunt surgery. In patients responding to surgery, pulsatile ICP (mean ICP wave amplitude) was significantly increased to a similar magnitude in patients with ncHC and the reference cohort (cHC). Furthermore, intracranial compliance (ICC) was reduced in clinical responders. The results of ventricular infusion testing provided evidence that patients responding to ETV have impaired ventricular CSF absorption, while those requiring shunt placement after ETV present with impaired CSF absorption both in the intraventricular and extraventricular compartments. CONCLUSIONS The study may provide some lessons about the pathophysiology of chronic ncHC. First, increased pulsatile ICP and impaired ICC characterize patients with chronic ncHC who respond clinically to CSF diversion surgery, even though static ICP is not increased. Second, in patients responding clinically to ETV, impaired ventricular CSF absorption may be a key factor. Patients requiring shunt placement for clinical

Cognitive Decline in Patients with Chronic Hydrocephalus and Normal Aging: ‘Growing into Deficits’

PubMed Central

de Beer, Marlijn H.; Scheltens, Philip

2016-01-01

Background/Aim To explore the theory of ‘growing into deficits’, a concept known from developmental neurology, in a series of cases with chronic hydrocephalus (CH). Methods Patients were selected from the Amsterdam Dementia Cohort and underwent extensive dementia screening. Results Twelve patients with CH were selected, in whom Alzheimer's disease was considered unlikely, based on biomarker information and follow-up. Mean Mini-Mental State Examination score was 24 (range 7-30). Most patients were functioning on a level of mild dementia [Clinical Dementia Rating score of 0.5 in 8/11 (66.7%) patients]. On neuropsychological examination, memory and executive functions, as well as processing speed were most frequently impaired. Conclusion In our opinion, the theory of ‘growing into deficits’ shows a parallel with the clinical course of CH and normal aging when Alzheimer's disease was considered very unlikely, because most of these patients were functioning well for a very large part of their lives. The altered cerebrospinal fluid dynamics might make the brain more vulnerable to aging-related changes, leading to a faster cognitive decline in CH patients compared to healthy subjects, especially in case of concomitant brain damage such as traumatic brain injury or meningitis. PMID:27920793

The Lhx9 homeobox gene controls pineal gland development and prevents postnatal hydrocephalus

PubMed Central

Yamazaki, Fumiyoshi; Møller, Morten; Fu, Cong; Clokie, Samuel J.; Zykovich, Artem; Coon, Steven L.; Klein, David C.; Rath, Martin F.

2014-01-01

Lhx9 is a member of the LIM homeobox gene family. It is expressed during mammalian embryogenesis in the brain including the pineal gland. Deletion of Lhx9 results in sterility due to failure of gonadal development. The current study was initiated to investigate Lhx9 biology in the pineal gland. Lhx9 is highly expressed in the developing pineal gland of the rat with transcript abundance peaking early in development; transcript levels decrease postnatally to nearly undetectable levels in the adult, a temporal pattern that is generally similar to that reported for Lhx9 expression in other brain regions. Studies with C57BL/6J Lhx9−/− mutant mice revealed marked alterations in brain and pineal development. Specifically, the superficial pineal gland is hypoplastic, being reduced to a small cluster of pinealocytes surrounded by meningeal and vascular tissue. The deep pineal gland and the pineal stalk are also reduced in size. Although the brains of neonatal Lhx9−/− mutant mice appear normal, severe hydrocephalus develops in about 70 % of the Lhx9−/− mice at 5–8 weeks of age; these observations are the first to document that deletion of Lhx9 results in hydrocephalus and as such indicate that Lhx9 contributes to the maintenance of normal brain structure. Whereas hydrocephalus is absent in neonatal Lhx9−/−mutant mice, the neonatal pineal gland in these animals is hypoplastic. Accordingly, it appears that Lhx9 is essential for early development of the mammalian pineal gland and that this effect is not secondary to hydrocephalus. PMID:24647753

Hydrodynamics of the Certas™ programmable valve for the treatment of hydrocephalus

PubMed Central

2012-01-01

Background The new Certas™ shunt for the treatment of hydrocephalus has seven standard pressure settings that according to the manufacturer range from 36 to 238 mmH2O, and an additional “Virtual Off” setting with an opening pressure >400 mmH2O. Information on actual pressure response and reliability of shunt performance is important in clinical application, especially the “Virtual Off” setting as a non-surgical replacement for shunt ligation. The objective of this study was to evaluate the in-vitro hydrodynamic performance of the Certas™ shunt. Methods Six new Certas™ shunts with proximal and distal catheters were tested with an automated, computerized test system that raised the pressure from zero to a maximum pressure and back to zero at each valve setting. Opening pressure and flow resistance were determined. Results For settings 1–7 the measured opening pressure range was 26 to 247 mmH2O, and the mean change in opening pressure for a one-step adjustment was between 33 and 38 mmH2O. For setting 8 (“Virtual Off”) the measured mean opening pressure was 494 ± 34 mmH2O (range 451 to 556 mmH2O). The mean outflow resistance was 7.0 mmHg/ml/min (outflow conductance 17.9 μl/s/kPa). Conclusions The six shunts had similar characteristics and closely matched the manufacturer’s specifications for opening pressure at settings 1–7. The opening pressure for the “Virtual Off” setting was nearly 500 mmH2O, which is 100 mmH2O higher than the manufacturer’s specification of “>400” and should be functionally off for most patients with communicating hydrocephalus. Clinical studies are needed to evaluate if the CSF dynamic profile persists after implantation in patients. PMID:22643114

Unrecognised ventriculitis/meningitis presenting as hydrocephalus in infancy.

PubMed

Udani, Vrajesh; Udani, Soonu; Merani, Rohan; Bavdekar, Manisha

2003-09-01

Infantile hydrocephalus due to unrecognized neonatal-onset meningitis/ventriculitis, was studied retrospectively using 1991-1998 chart review. Seventy two patients with hydrocephalus were reviewed. Thirteen infants had hydrocephalus associated with active meningitis/ventriculitis which had remained unrecognized. Active meningitis/ventriculitis was confirmed by the finding of an abnormal lumbar and ventricular CSF with or without positive culture. All had perinatal risk factors and 10/13 had been given antibiotics in the postnatal period. 6/13 infants appeared to be well. The most common presentation was increasing head size. All lumbar and ventricular CSFs were abnormal and 10/13 had positive cultures as well. Imaging revealed hydrocephalus in all. The infants were treated with antibiotics for a mean of 32.8 days before VP shunting. 7/11 were severely disabled. Unrecognized active meningitis/ventriculitis is an important cause of infantile hydrocephalus.

Blood-brain barrier and foetal-onset hydrocephalus, with a view on potential novel treatments beyond managing CSF flow.

PubMed

Guerra, M; Blázquez, J L; Rodríguez, E M

2017-07-13

Despite decades of research, no compelling non-surgical therapies have been developed for foetal hydrocephalus. So far, most efforts have pointed to repairing disturbances in the cerebrospinal fluid (CSF) flow and to avoid further brain damage. There are no reports trying to prevent or diminish abnormalities in brain development which are inseparably associated with hydrocephalus. A key problem in the treatment of hydrocephalus is the blood-brain barrier that restricts the access to the brain for therapeutic compounds or systemically grafted cells. Recent investigations have started to open an avenue for the development of a cell therapy for foetal-onset hydrocephalus. Potential cells to be used for brain grafting include: (1) pluripotential neural stem cells; (2) mesenchymal stem cells; (3) genetically-engineered stem cells; (4) choroid plexus cells and (5) subcommissural organ cells. Expected outcomes are a proper microenvironment for the embryonic neurogenic niche and, consequent normal brain development.

236 children with developmental hydrocephalus: causes and clinical consequences

PubMed Central

Tully, Hannah M; Ishak, Gisele E; Rue, Tessa C; Dempsey, Jennifer C; Browd, Samuel R; Millen, Kathleen J; Doherty, Dan; Dobyns, William B

2016-01-01

Few systematic assessments of developmental forms of hydrocephalus exist. We reviewed MRIs and clinical records of patients with infancy-onset hydrocephalus. Among 411 infants, 236 had hydrocephalus with no recognizable extrinsic cause. These children were assigned to one of five subtypes and compared on the basis of clinical characteristics, developmental and surgical outcomes. At an average age of 5.3 years, 72% of children were walking independently and 87% could eat by mouth. 18% had epilepsy. Distinct patterns of associated malformations and syndromes were observed within each subtype. On average, children with aqueductal obstruction, cysts and encephaloceles had worse clinical outcomes than those with other forms of developmental hydrocephalus. 53% of surgically-treated patients experienced at least one shunt failure, but hydrocephalus associated with posterior fossa crowding required fewer shunt revisions. We conclude that each subtype of developmental hydrocephalus is associated with distinct clinical characteristics, syndromology, and outcomes, suggesting differences in underlying mechanisms. PMID:26184484

Post-hemispherectomy hydrocephalus: results of a comprehensive, multi-institutional review

PubMed Central

Lew, Sean M.; Matthews, Anne E.; Hartman, Adam L.; Haranhalli, Neil

2012-01-01

SUMMARY Purpose Hemispherectomy surgery for medically intractable epilepsy is known to cause hydrocephalus in a subset of patients. Existing data regarding the incidence of, and risk factors for developing, post-hemispherectomy hydrocephalus has been limited by the relatively small number of cases performed by any single center. Our goal was to better understand this phenomenon and to identify risk factors that may predispose patients to developing hydrocephalus after hemispherectomy surgery. Methods Fifteen pediatric epilepsy centers participated in this study. A retrospective chart review was performed on all available patients who had hemispherectomy surgery. Data collected included surgical techniques, etiology of seizures, prior brain surgery, symptoms and signs of hydrocephalus, timing of shunt placement and basic demographics. Key findings Data were collected from 736 patients who underwent hemispherectomy surgery between 1986 and 2011. Forty-six patients had pre-existing shunted hydrocephalus and were excluded from analysis, yielding 690 patients for this study. One hundred sixty-two patients (23%) required hydrocephalus treatment. The timing of hydrocephalus ranged from the immediate post-operative period to 8.5 years after surgery, with 43 patients (27%) receiving shunts more than 90 days after surgery. Multivariate regression analysis revealed anatomic hemispherectomies (OR 4.1, p<0.0001) and previous brain surgery (O.R. 1.7, p=0.04) as independently significant risk factors for developing hydrocephalus. There was a trend towards significance for the use of hemostatic agents (O.R. 2.2, p=0.07) and the involvement of basal ganglia or thalamus in the resection (O.R. 2.2, p=0.08) as risk factors. Significance Hydrocephalus is a common sequela of hemispherectomy surgery. Surgical technique and prior brain surgery influence the occurrence of post-hemispherectomy hydrocephalus. A significant portion of patients develop hydrocephalus on a delayed basis

Yap is required for ependymal integrity and is suppressed in LPA-induced hydrocephalus

PubMed Central

Park, Raehee; Moon, Uk Yeol; Park, Jun Young; Hughes, Lucinda J.; Johnson, Randy L.; Cho, Seo-Hee; Kim, Seonhee

2016-01-01

Timely generation and normal maturation of ependymal cells along the aqueduct are critical for preventing physical blockage between the third and fourth ventricles and the development of fetal non-communicating hydrocephalus. Our study identifies Yap, the downstream effector of the evolutionarily conserved Hippo pathway, as a central regulator for generating developmentally controlled ependymal cells along the ventricular lining of the aqueduct. Yap function is necessary for proper proliferation of progenitors and apical attachment of ependymal precursor cells. Importantly, an injury signal initiated by lysophosphatidic acid (LPA), an upstream regulator of Yap that can cause fetal haemorrhagic hydrocephalus, deregulates Yap in the developing aqueduct. LPA exposure leads to the loss of N-cadherin concentrations at the apical endfeet, which can be partially restored by forced Yap expression and more efficiently by phosphomimetic Yap. These results reveal a novel function of Yap in retaining tissue junctions during normal development and after fetal brain injury. PMID:26754915

Control volume based hydrocephalus research; analysis of human data

NASA Astrophysics Data System (ADS)

Cohen, Benjamin; Wei, Timothy; Voorhees, Abram; Madsen, Joseph; Anor, Tomer

2010-11-01

Hydrocephalus is a neuropathophysiological disorder primarily diagnosed by increased cerebrospinal fluid volume and pressure within the brain. To date, utilization of clinical measurements have been limited to understanding of the relative amplitude and timing of flow, volume and pressure waveforms; qualitative approaches without a clear framework for meaningful quantitative comparison. Pressure volume models and electric circuit analogs enforce volume conservation principles in terms of pressure. Control volume analysis, through the integral mass and momentum conservation equations, ensures that pressure and volume are accounted for using first principles fluid physics. This approach is able to directly incorporate the diverse measurements obtained by clinicians into a simple, direct and robust mechanics based framework. Clinical data obtained for analysis are discussed along with data processing techniques used to extract terms in the conservation equation. Control volume analysis provides a non-invasive, physics-based approach to extracting pressure information from magnetic resonance velocity data that cannot be measured directly by pressure instrumentation.

Ventricular-subcutaneous shunt for the treatment of experimental hydrocephalus in young rats: technical note.

PubMed

Santos, Marcelo Volpon; Garcia, Camila Araujo Bernardino; Jardini, Evelise Oliveira; Romeiro, Thais Helena; da Silva Lopes, Luiza; Machado, Hélio Rubens; de Oliveira, Ricardo Santos

2016-08-01

Hydrocephalus is a complex disease that affects cerebrospinal fluid (CSF) dynamics and is very common in children. To this date, CSF shunting is still the standard treatment for childhood hydrocephalus, but, nevertheless, the effects of such an operation on the developing brain are widely unknown. To help overcome this, experimental models of CSF shunts are surely very useful tools. The objective of this study was to describe a feasible and reliable technique of an adapted ventricular-subcutaneous shunt for the treatment of kaolin-induced hydrocephalus in young rats. We developed a ventricular-subcutaneous shunt (VSCS) technique which was used in 31 Wistar young rats with kaolin-induced hydrocephalus. Hydrocephalus was induced at 7 days of age, and shunt implantation was performed 7 days later. Our technique used a 0.7-mm gauge polypropylene catheter tunneled to a subcutaneous pocket created over the animal's back and inserted into the right lateral ventricle. All animals were sacrificed 14 days after shunt insertion. Twenty-four rats survived and remained well until the study was ended. No major complications were seen. Their weight gain went back to normal. They all underwent ambulatory behavioral testing prior and after VSCS, which showed improvement in their motor skills. We have also obtained magnetic resonance (MR) scans of 16 pups confirming reduction of ventricular size after shunting and indicating effective treatment. Histopathological analysis of brain samples before and after shunting showed reversion of ependymal and corpus callosum disruption, as well as fewer reactive astrocytes in shunted animals. An experimental CSF shunt technique was devised. Excessive CSF of hydrocephalic rats is diverted into the subcutaneous space where it can be resorbed. This technique has a low complication rate and is effective. It might be applied to various types of experimental studies involving induction and treatment of hydrocephalus.

Factors Associated with Acute and Chronic Hydrocephalus in Nonaneurysmal Subarachnoid Hemorrhage.

PubMed

Kang, Peter; Raya, Amanda; Zipfel, Gregory J; Dhar, Rajat

2016-02-01

Hydrocephalus requiring external ventricular drain (EVD) or shunt placement commonly complicates aneurysmal subarachnoid hemorrhage (SAH), but its frequency is not as well known for nonaneurysmal SAH (NA-SAH). Those with diffuse bleeding may have greater risk of hydrocephalus compared to those with a perimesencephalic pattern. We evaluated the frequency of hydrocephalus in NA-SAH and whether imaging factors could predict the need for EVD and shunting. We collected admission clinical and imaging variables for 105 NA-SAH patients, including bicaudate index (BI), Hijdra sum score (HSS), intraventricular hemorrhage (IVH) score, modified Fisher scale (mFS), and bleeding pattern. Hydrocephalus was categorized as acute (need for EVD) or chronic (shunt). We applied logistic regression to determine whether hydrocephalus risk was independently related to bleeding pattern or mediated through blood volume or ventriculomegaly. Acute hydrocephalus was seen in 26 (25%) patients but was more common with diffuse (15/28, 54%) versus perimesencephalic (10/59, 17%, p < 0.001) bleeding. Patients developing acute hydrocephalus had worse clinical grade and higher BI, HSS, and IVH scores. Adjusting the relationship between hydrocephalus and diffuse bleeding for HSS (but not BI) nullified this association. Nine (35%) patients requiring EVD eventually required shunting for chronic hydrocephalus, which was associated with greater blood burden but not poor clinical grade. Acute hydrocephalus occurs in one-quarter of NA-SAH patients. The greater risk in diffuse bleeding appears to be mediated by greater cisternal blood volume but not by greater ventriculomegaly. Imaging characteristics may aid in anticipatory management of hydrocephalus in NA-SAH.

Hydrocephalus caused by unilateral foramen of Monro obstruction: A review on terminology

PubMed Central

Nigri, Flavio; Gobbi, Gabriel Neffa; da Costa Ferreira Pinto, Pedro Henrique; Simões, Elington Lannes; Caparelli-Daquer, Egas Moniz

2016-01-01

Background: Hydrocephalus caused by unilateral foramen of Monro (FM) obstruction has been referred to in literature by many different terminologies. Precise terminology describing hydrocephalus confined to just one lateral ventricle has a very important prognostic value and determines whether or not the patient can be shunt free after an endoscopic procedure. Methods: Aiming to define the best term for unilateral FM obstruction, 19 terms were employed on PubMed database (http://www.ncbi.nlm.nih.gov/pubmed) as quoted phrases. Results: A total of 194 articles were found. Four patterns of hydrocephalus were discriminated as a result of our research term query and were divided by types for didactic purpose. Type A - partial dilation of the lateral ventricle; Type B - pure unilateral obstruction of the FM; Type C - previously shunted patients with secondary obstruction of the FM; and Type D - asymmetric lateral ventricles with patent FM. Conclusion: In unilateral FM obstruction hydrocephalus, an in-depth review on terminology application is critical to avoid mistakes that may compromise comparisons among different series. This terminology review suggests that Type B hydrocephalus, i.e., the hydrocephalus confined to just one lateral ventricle with no other sites of cerebrospinal fluid circulation blockage, are best described by the terms unilateral hydrocephalus (UH) and monoventricular hydrocephalus, the first being by far the most popular. Type A hydrocephalus is best represented in the literature by the terms uniloculated hydrocephalus and loculated ventricle; Type C hydrocephalus by the terms isolated lateral ventricle and isolated UH; and Type D hydrocephalus by the term asymmetric hydrocephalus. PMID:27274402

Obstructive hydrocephalus as a result of giant cell tumor of the thoracic spine: A case report

PubMed Central

WEI, CHENG-YU; CHEN, SHUO-TSUNG; TAI, HSU-CHIH; WANG, WEN-BING; CHANG, CHI-CHU; WANG, YAO-CHIN; WEI, LI; KUNG, WOON-MAN

2016-01-01

Giant cell tumors (GCTs) are rare bone tumors that account for ~5% of all primary bone tumors. When GCTs occur in the spine, patients usually present with localized pain and neurological symptoms, such as radiating pain or hyperesthesia. In the current report, an unusual case of a GCT of the thoracic spine associated with hydrocephalus is described. A 48-year-old male presented with urinary retention, loss of sensation in the lower limbs and inability to walk. The patient eventually developed hydrocephalus combined with altered consciousness, indicated by an inability to follow simple commands. Magnetic resonance (MR) imaging demonstrated the presence of a soft tissue mass at the T2 level, and biopsy examination of the tissue confirmed that it was a GCT. The patient experienced a sudden loss of consciousness due to an acute episode of obstructive hydrocephalus. A ventriculoperitoneal shunting procedure was performed to treat the hydrocephalus, and the patient regained normal consciousness, although the paraplegia persisted. An MR examination performed 30 months following surgery demonstrated that the tumor size was stable, consistent with the slow growth that is characteristic of GCTs. Diagnosis of GCTs may be challenging, and relies on radiographic and histopathologic findings. Although rare, acute hydrocephalus as a result of GCTs should not be excluded from a differential diagnosis. PMID:26870164

New concept of the pathogenesis and therapeutic orientation of acquired communicating hydrocephalus.

PubMed

Xu, Hao

2016-09-01

Hydrocephalus is a common medical condition characterized by abnormalities in the secretion, circulation and absorption of cerebrospinal fluid (CSF), resulting in ventricle dilatation. For the communicating hydrocephalus, without etiological treatment, its pathogenesis has been considered as a research emphasis. Many factors can damage the CSF system and trigger communicating hydrocephalus, including tumor surgery and hydrocephalus neurological diseases, such as brain trauma, infection, ICH and SAH. But according to our clinical experience, a big proportion of patients do not develop hydrocephalus. That is because the absorbing ability of CSF can compensate within a certain range. If the damage exceeds that range, hydrocephalus will occur. Once it occurs, it is not likely to be reversed, so a shunt surgery is always needed. Therefore, we believe that our orientation could transform the treatment of patient who has already showed hydrocephalus symptoms to the prevention of the occurrence in the patient with high risk of hydrocephalus. Based on the hypothesis above, we first divide the process of hydrocephalus into three stages and we believe that hydrocephalus are possible be reversed or halted in stage 1 and 2. The new concept of the pathogenesis in hydrocephalus will enrich our understanding and provide new insights to the therapeutic orientation. In conclusion, the future research direction should be the prevention of hydrocephalus, which should take a long period from the immediate occurrence of brain injury to several months or even years after the injury.

Cognitive changes after cerebrospinal fluid shunting in young adults with spina bifida and assumed arrested hydrocephalus

PubMed Central

Mataro, M.; Poca, M. A.; Sahuquillo, J.; Cuxart, A.; Iborra, J.; de la Calzada, M. D.; Junque, C.

2000-01-01

OBJECTIVES—To establish whether surgery can improve the neuropsychological functioning of young adult patients with spina bifida and apparent clinically arrested hydrocephalus showing abnormal intracranial pressure.
METHODS—Twenty three young adults with spina bifida and assumed arrested hydrocephalus (diagnosed as active or compensated by continuous intracranial pressure monitoring) underwent surgery. All patients received neuropsychological examination before surgery and 6 months later. Neuropsychological assessment included tests of verbal and visual memory, visuospatial functions, speed of mental processing, and frontal lobe functions.
RESULTS—Shunt placement in this subgroup of patients improves neuropsychological functioning, especially in verbal and visual memory and attention and cognitive flexibility.
CONCLUSIONS—Young adults with spina bifida and suspected non-functioning shunt or non-shunted ventriculomegaly should be carefully monitored to identify those who could benefit from shunting.

 PMID:10766893

Optic nerve head drusen and idiopathic intracranial hypertension in a 14-year-old girl.

PubMed

Granger, Robert H; Bonnelame, Thomas; Daubenton, John; Dreyer, Michael; McCartney, Paul

2009-01-01

A 14-year-old girl had a 3-month history of headache and blurred vision. Funduscopy showed bilateral optic disc edema. Findings on brain imaging were normal, and a diagnosis of idiopathic intracranial hypertension was confirmed after lumbar puncture showed an elevated opening pressure of 32 cm H(2)O. Optic nerve head drusen were noted on computed tomography scan and confirmed with B-scan ultrasound. After 2 years, resolution of symptoms coincided with variable compliance to treatment with acetazolamide and concomitant papilledema. In general, optic disc edema poses a clinical conundrum due to the more common occurrence of optic nerve head drusen, potentially resulting in delayed diagnosis and treatment of idiopathic intracranial hypertension. Copyright 2009, SLACK Incorporated.

Hydrocephalus and Shunts

MedlinePlus

... someone with Spina Bilda and shunted hydrocephalus. When making decisions, here is some helpful advice to families and health care providers: • Pay attention to a parent’s (and particularly mom’s) gut feeling ...

National Hydrocephalus Foundation

MedlinePlus

... Types of Seizures About the Foundation Mission, History & Philosophy of NHF Treatment of Hydrocephalus What is a Shunt? Treatment Third Ventriculostomy Shunt Malfunction Prognosis and Research Medical Dictionary Resources Success Stories Blessing in Disguise ...

The Intracranial Volume Pressure Response in Increased Intracranial Pressure Patients: Clinical Significance of the Volume Pressure Indicator.

PubMed

Lai, Hung-Yi; Lee, Ching-Hsin; Lee, Ching-Yi

2016-01-01

For patients suffering from primary brain injury, monitoring intracranial pressure alone is not enough to reflect the dynamic intracranial condition. In our previous study, a segment of the pressure-volume curve can be expressed by the parabolic regression model with single indicator "a". The aim of this study is to evaluate if the indicator "a" can reflect intracranial conditions. Patients with traumatic brain injury, spontaneous intracranial hemorrhage, and/or hydrocephalus who had external ventricular drainage from January 2009 to February 2010 were included. The successive volume pressure response values were obtained by successive drainage of cerebral spinal fluid from intracranial pressure 20-25 mm Hg to 10 mm Hg. The relationship between withdrawn cerebral spinal fluid volume and intracranial pressure was analyzed by the parabolic regression model with single parameter "a". The overall mean for indicator "a" was 0.422 ± 0.046. The mean of "a" in hydrocephalus was 0.173 ± 0.024 and in severe intracranial mass with slender ventricle, it was 0.663 ± 0.062. The two extreme intracranial conditions had a statistical significant difference (p<0.001). The indicator "a" of a pressure-volume curve can reflect the dynamic intracranial condition and is comparable in different situations. A significantly larger indicator "a" with increased intracranial pressure is always observed in severe intracranial mass lesions with cerebral edema. A significantly smaller indicator "a" with increased intracranial pressure is observed in hydrocephalus. Brain computed tomography should be performed early if a rapid elevation of indicator "a" is detected, as it can reveal some ongoing intracranial pathology prior to clinical deterioration. Increased intracranial pressure was frequently observed in patients with intracranial pathology. The progression can be differentiated using the pattern of the volume pressure indicator.

The Intracranial Volume Pressure Response in Increased Intracranial Pressure Patients: Clinical Significance of the Volume Pressure Indicator

PubMed Central

2016-01-01

Background For patients suffering from primary brain injury, monitoring intracranial pressure alone is not enough to reflect the dynamic intracranial condition. In our previous study, a segment of the pressure-volume curve can be expressed by the parabolic regression model with single indicator “a”. The aim of this study is to evaluate if the indicator “a” can reflect intracranial conditions. Methods Patients with traumatic brain injury, spontaneous intracranial hemorrhage, and/or hydrocephalus who had external ventricular drainage from January 2009 to February 2010 were included. The successive volume pressure response values were obtained by successive drainage of cerebral spinal fluid from intracranial pressure 20–25 mm Hg to 10 mm Hg. The relationship between withdrawn cerebral spinal fluid volume and intracranial pressure was analyzed by the parabolic regression model with single parameter “a”. Results The overall mean for indicator “a” was 0.422 ± 0.046. The mean of “a” in hydrocephalus was 0.173 ± 0.024 and in severe intracranial mass with slender ventricle, it was 0.663 ± 0.062. The two extreme intracranial conditions had a statistical significant difference (p<0.001). Conclusion The indicator “a” of a pressure-volume curve can reflect the dynamic intracranial condition and is comparable in different situations. A significantly larger indicator “a” with increased intracranial pressure is always observed in severe intracranial mass lesions with cerebral edema. A significantly smaller indicator “a” with increased intracranial pressure is observed in hydrocephalus. Brain computed tomography should be performed early if a rapid elevation of indicator “a” is detected, as it can reveal some ongoing intracranial pathology prior to clinical deterioration. Increased intracranial pressure was frequently observed in patients with intracranial pathology. The progression can be differentiated using the pattern of the volume

A hydroelastic model of hydrocephalus

NASA Astrophysics Data System (ADS)

Smillie, Alan; Sobey, Ian; Molnar, Zoltan

2005-09-01

We combine elements of poroelasticity and of fluid mechanics to construct a mathematical model of the human brain and ventricular system. The model is used to study hydrocephalus, a pathological condition in which the normal flow of the cerebrospinal fluid is disturbed, causing the brain to become deformed. Our model extends recent work in this area by including flow through the aqueduct, by incorporating boundary conditions that we believe accurately represent the anatomy of the brain and by including time dependence. This enables us to construct a quantitative model of the onset, development and treatment of this condition. We formulate and solve the governing equations and boundary conditions for this model and give results that are relevant to clinical observations.

Hydrocephalus in herpes simplex type 2 meningitis.

PubMed

Yap, Elaine; Ellis-Pegler, Rod

2006-08-01

A 34-year-old woman presented to hospital with symptoms of meningitis, later confirmed to be due to herpes simplex virus type 2. She developed hydrocephalus on day 2 of her admission. We describe the first case of hydrocephalus associated with herpes simplex type 2 meningitis in an adult.

Hydrocephalus after decompressive craniectomy for malignant hemispheric cerebral infarction.

PubMed

Wang, Qiang-Ping; Ma, Jun-Peng; Zhou, Zhang-Ming; Yang, Min; You, Chao

2016-08-01

Several studies have investigated the incidence and risk factors of hydrocephalus after decompressive craniectomy (DC) for malignant hemispheric cerebral infarction. However, the results are controversial. Therefore, the following is a retrospective cohort study to determine the incidence and risk factors of hydrocephalus after DC for malignant hemispheric cerebral infarction. From January 2004 to June 2014, patients at two medical centres in south-west China, who underwent DC for malignant hemispheric cerebral infarction, were included. The patients' clinical and radiologic findings were retrospectively reviewed. A chi-square test, Mann-Whitney U-test and logistic regression model were used to identify the risk factors. A total of 128 patients were included in the study. The incidence of ventriculomegaly and shunt-dependent hydrocephalus were 42.2% (54/128) and 14.8% (19/128), respectively. Lower preoperative Glasgow Coma Scale (GCS) score and presence of subarachnoid haemorrhage (SAH) were factors significantly associated with the development of post-operative hydrocephalus after DC. Cerebral infarction patients receiving DC have a moderate tendency to suffer from post-operative hydrocephalus. A poor GCS score and the presence of SAH were significantly associated with the development of hydrocephalus after DC.

Decompressive craniectomy and hydrocephalus: proposal of a therapeutic flow chart.

PubMed

Peraio, Simone; Calcagni, Maria Lucia; Mattoli, Maria Vittoria; Marziali, Giammaria; DE Bonis, Pasquale; Pompucci, Angelo; Anile, Carmelo; Mangiola, Annunziato

2017-12-01

Decompressive craniectomy (DC) may be necessary to save the lives of patients suffering from intracranial hypertension. However, this procedure is not complication-free. Its two main complications are hydrocephalus and the sinking skin-flap syndrome (SSFS). The radiological findings and the clinical evaluation may be not enough to decide when and/or how to treat hydrocephalus in a decompressed patient. SSFS and hydrocephalus may be not unrelated. In fact, a patient affected by hydrocephalus, after the ventriculo-peritoneal shunt, can develop SSFS; on the other hand, SSFS per se can cause hydrocephalus. Treating hydrocephalus in decompressed patients can be challenging. Radiological findings and clinical evaluation may not be enough to define the most appropriate therapeutic strategy. Cerebrospinal fluid (CSF) dynamics and metabolic evaluations can represent important diagnostic tools for assessing the need of a CSF shunt in patients with a poor baseline neurologic status. Based on our experience, we propose a flow chart for treating decompressed patients affected by ventriculomegaly.

Ruptured Massa Intermedia Secondary to Hydrocephalus.

PubMed

El Damaty, Ahmed; Langner, Soenke; Schroeder, Henry W S

2017-01-01

We report a case of ruptured massa intermedia (MI) as a sequela of hydrocephalus. A single case report is presented describing the sequelae of tumor bed hematoma after a posterior fossa hemangioblastoma resection in which the patient bled 3 days after surgery, resulting in secondary hydrocephalus and subsequently dilatation of the third ventricle, which resulted in rupture of the MI. The patient was managed on emergency basis with an external ventricular drain then endoscopically with a third ventriculostomy and clot extraction. Absent MI is not uncommon in hydrocephalic patients, and it is assumed to be the result of rupture from acute dilatation of the third ventricle. Our case report proves this assumption and documents the presence and absence of the MI before and after developing hydrocephalus. Copyright © 2016 Elsevier Inc. All rights reserved.

Prevalence of lateral ventricle asymmetry in brain MRI studies of neurologically normal dogs and dogs with idiopathic epilepsy.

PubMed

Pivetta, Mauro; De Risio, Luisa; Newton, Richard; Dennis, Ruth

2013-01-01

Asymmetry of the cerebral lateral ventricles is a common finding in cross-sectional imaging of otherwise normal canine brains and has been assumed to be incidental. The purpose of this retrospective study was to compare the prevalence of ventricular asymmetry in brain MRI studies of normal dogs and dogs with idiopathic epilepsy. Brain MRI archives were searched for 100 neurologically normal dogs (Group 1) and 100 dogs with idiopathic epilepsy (Group 2). For each dog, asymmetry of the lateral ventricles was subjectively classified as absent, mild, moderate, and severe based on a consensus of two observers who were unaware of group status. Ventricular areas were measured from transverse T1W images at the level of the interthalamic adhesion. An asymmetry ratio was calculated as the ratio of the larger to smaller ventricular transverse area. There was excellent agreement between subjective assessments of ventricular asymmetry and quantitative assessments using asymmetry ratios (k = 0.995). The prevalence of asymmetry was 38% in Group 1 dogs and 44% in Group 2 dogs. Assymmetry was scored as mild in the majority of Group 2 dogs. There was no significant association between presence/absence and degree of ventricular asymmetry vs. dog group, age, gender, or skull conformation. Findings from the current study supported previously published assumptions that asymmetry of the lateral cerebral ventricles is an incidental finding in MRI studies of the canine brain. © 2013 Veterinary Radiology & Ultrasound.

The importance of the cortical subarachnoid space in understanding hydrocephalus.

PubMed

Rekate, Harold L; Nadkarni, Trimurti D; Wallace, Donna

2008-07-01

In this paper the authors define the role of the cortical subarachnoid space (CSAS) in poorly understood forms of hydrocephalus to cerebrospinal fluid (CSF) dynamics to improve understanding of the importance of the CSAS and its role in selecting patients for endoscopic third ventriculostomy (ETV). The secondary purpose of this work was to define testable hypotheses to explain enigmatic disorders of CSF dynamics and to suggest how these concepts could be tested. The magnitude of the contribution of the CSAS is explored using the solid geometry of concentric spheres. With this starting point, clinical conditions in which CSF dynamics are not easily understood are explored regarding the potential role of the CSAS. Overall, problems of CSF dynamics are easily understood. Insights may be gained when the results of a pathological process or its treatment vary from what has been expected. Acute changes in ventricular volume at the time that hydrocephalus develops, the failure of shunts, and the changes in ventricular volume with shunt repair may occur very rapidly. Changes in the volume of water in the brain, especially in the brain substance itself, are unlikely to occur at this rapid rate and may be interpreted as a simple redistribution of the CSF between the ventricle and CSAS with no initial change in the actual volume of brain parenchyma. Problems such as pseudotumor cerebri, shunt failure with nonresponsive ventricles, and negative-pressure hydrocephalus can be explained by assessing the ability of ventricular CSF to flow to the CSAS and the ability of this fluid to exit this compartment. Ventricular enlargement at the time of shunt failure implies a failure of flow between the ventricles and CSAS, implying that all patients who show this phenomenon are potential candidates for ETV. The important role of the CSAS in the pathophysiology of various forms of hydrocephalus has been largely ignored. Attention to the dynamics of the CSF in this compartment will improve

Global hydrocephalus epidemiology and incidence: systematic review and meta-analysis.

PubMed

Dewan, Michael C; Rattani, Abbas; Mekary, Rania; Glancz, Laurence J; Yunusa, Ismaeel; Baticulon, Ronnie E; Fieggen, Graham; Wellons, John C; Park, Kee B; Warf, Benjamin C

2018-04-27

OBJECTIVE Hydrocephalus is one of the most common brain disorders, yet a reliable assessment of the global burden of disease is lacking. The authors sought a reliable estimate of the prevalence and annual incidence of hydrocephalus worldwide. METHODS The authors performed a systematic literature review and meta-analysis to estimate the incidence of congenital hydrocephalus by WHO region and World Bank income level using the MEDLINE/PubMed and Cochrane Database of Systematic Reviews databases. A global estimate of pediatric hydrocephalus was obtained by adding acquired forms of childhood hydrocephalus to the baseline congenital figures using neural tube defect (NTD) registry data and known proportions of posthemorrhagic and postinfectious cases. Adult forms of hydrocephalus were also examined qualitatively. RESULTS Seventy-eight articles were included from the systematic review, representative of all WHO regions and each income level. The pooled incidence of congenital hydrocephalus was highest in Africa and Latin America (145 and 316 per 100,000 births, respectively) and lowest in the United States/Canada (68 per 100,000 births) (p for interaction < 0.1). The incidence was higher in low- and middle-income countries (123 per 100,000 births; 95% CI 98-152 births) than in high-income countries (79 per 100,000 births; 95% CI 68-90 births) (p for interaction < 0.01). While likely representing an underestimate, this model predicts that each year, nearly 400,000 new cases of pediatric hydrocephalus will develop worldwide. The greatest burden of disease falls on the African, Latin American, and Southeast Asian regions, accounting for three-quarters of the total volume of new cases. The high crude birth rate, greater proportion of patients with postinfectious etiology, and higher incidence of NTDs all contribute to a case volume in low- and middle-income countries that outweighs that in high-income countries by more than 20-fold. Global estimates of adult and other forms of

Parent ratings of executive functioning in children with shunted hydrocephalus.

PubMed

Lacy, Maureen; Baldassarre, Megan; Nader, Todd; Frim, David

2012-01-01

The present study examined the executive functioning of a group of children with a history of communicating hydrocephalus and how their level of functioning was correlated with parent ratings of executive functioning. The study examined the executive functioning of 39 shunted children with a history of hydrocephalus and 20 healthy peers. Additionally, parents of both groups of children completed the Behavior Rating Inventory of Executive Function (BRIEF) to assess the parents' perceptions of their children's executive functioning. Finally, the study investigated the relationship between the shunted hydrocephalus children's executive functioning and the parent ratings of their executive functioning. Overall, the children with a history of shunted hydrocephalus displayed more executive dysfunction than their healthy peers. These children were rated by their parents as having more executive dysfunction than their healthy peers and displaying working memory, initiation, mental flexibility and self-monitoring difficulties, which appear to increase with age among the shunted hydrocephalus group. While parent ratings as measured by the BRIEF indices did not correlate with all executive tasks within the shunted hydrocephalus group, the cognitive tests assessing mental flexibility may be sensitive to the problems noted by parents at home. The children with a history of shunted hydrocephalus displayed executive functioning deficits on formal examination. The parents of children with a history of shunted hydrocephalus report ongoing executive difficulties which may increase with age. Copyright © 2012 S. Karger AG, Basel.

Technology preferences among caregivers of children with hydrocephalus.

PubMed

Naftel, Robert P; Safiano, Nicole A; Falola, Michael I; Shannon, Chevis N; Wellons, John C; Johnston, James M

2013-01-01

The Internet and social media are powerful disseminators of medical information, providing new portals for patient care. The authors of this study evaluated current technology hardware, Internet, and social media use and their socioeconomic relationships among caregivers of children with hydrocephalus. A written survey was completed in the neurosurgical clinics at the University of Alabama at Birmingham by 300 parents of children with shunted hydrocephalus between October 26, 2010, and July 26, 2011. Computer use (94.6%), Internet use (91.7%), smartphone use (56.9%), and Internet research on hydrocephalus (81.9%) were prevalent. However, for each of these four utilizations there was significantly lower access by caregivers of minority races (p = 0.04, 0.03, 0.002, and < 0.0001, respectively), lower income (p = 0.02, 0.01, < 0.0001, and < 0.0001, respectively), and lower level of education (p = 0.001, 0.002, < 0.0001, and 0.001, respectively). Personal use of social media was prevalent (95.1% of all Internet users) with use being more prevalent among less-educated than higher-educated caregivers (p = 0.017). Hydrocephalus-related social media use (59.5% of Internet users) was not associated with socioeconomic factors. For hydrocephalus education on the Internet, caregivers chose information websites such as Wikipedia or the Hydrocephalus Association as preferred platforms; these preferences were followed by use of social media websites. Facebook and YouTube were the preferred social media platforms for personal and hydrocephalus-related use. Parents indicate moderate skepticism about the trustworthiness of the Internet; only 21.7% always trust the online sources. Most parents (89.8%) say that they would visit neurosurgeon-recommended websites. Of Internet-using caregivers, 28.6% use the Internet or social media to find hydrocephalus support groups, and 34.8% have used the Internet to communicate with other caregivers who have children with similar conditions

Controversy about Management of Hydrocephalus - Shunt vs. Endoscopic Third Ventriculostomy.

PubMed

Kumar, Vikas; Bodeliwala, Shaam; Singh, Daljit

2017-08-01

The best management of hydrocephalus is still controversial in the twenty-first century. Shunt treatment for hydrocephalus is the most common procedure performed in neurosurgical practice and is associated with the highest complications rate. But during the last 2 decades, the treatment of hydrocephalus has improved with better shunt devices available today, increased facilities for investigations and newer approaches like endoscopic third ventriculostomy. The recent advances in development of better endoscopes have provided the patient and treating doctor with an option for an alternative surgery for treatment of hydrocephalus.

Increased risk of hydrocephalus in long-term dialysis patients.

PubMed

Wang, I-Kuan; Lin, Cheng-Li; Cheng, Yu-Kai; Chou, Che-Yi; Liang, Chih-Chia; Yen, Tzung-Hai; Sung, Fung-Chang

2016-05-01

The risk of hydrocephalus in end-stage renal disease (ESRD) patients on dialysis has not been studied in depth. Using Taiwan National Health Insurance claims data, we identified 29 684 incident ESRD patients from 2000 to 2010, including 10 030 peritoneal dialysis (PD) patients and 19 654 hemodialysis (HD) patients. The control cohort consisted of 118 736 people randomly selected from those without kidney disease, frequency matched with ESRD patients by age, sex and index year. We also established propensity score-matched cohorts with 10 014 PD and 10 014 HD patients. The incidence rates and hazard ratios (HRs) of hydrocephalus were calculated until the end of 2011. Incidence rates of hydrocephalus were greater in HD and PD patients than in controls (8.44 and 11.0 versus 4.11 per 10 000 person-years, respectively), with an adjusted HR of 1.86 [95% confidence interval (CI) 1.43-2.41] for all ESRD patients compared with controls. A higher proportion of hydrocephalus patients underwent surgical bypass to relieve hydrocephalus in ESRD patients than controls, 40.7% (46/113) versus 24.5% (67/273), with an adjusted odds ratio of 2.11 (95% CI 1.33-3.36). Compared with controls, the adjusted HRs of communicating hydrocephalus for HD and PD patients were 1.77 (95% CI 1.22-2.55) and 2.51 (95% CI 1.61-3.89), respectively. The propensity score-matched analysis showed an HR of 0.72 (95% CI 0.42-1.23) for hydrocephalus in HD patients compared with PD patients. Patients with ESRD are at an increased risk of hydrocephalus. The risk difference between HD and PD patients is not significant. © The Author 2016. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved.

Vertebrobasilar Dolichoectasia Induced Hydrocephalus: the Water-Hammer Effect

PubMed Central

Zisimopoulou, Vaso; Ntouniadaki, Aikaterini; Aggelidakis, Panagiotis; Siatouni, Anna; Gatzonis, Stylianos; Tavernarakis, Antonios

2015-01-01

Vertebrobasilar dolichoectasia is a clinical entity associated rarely with obstructive hydrocephalus. We present a 48-year old male with a profound dilatation of the ventricular system due to a dolichoectatic basilar artery, as appeared in imaging studies. The patient suffered from longstanding hydrocephalus and presenile dementia. The underlying mechanism for obstructive hydrocephalus due to vertebrobasilar dolichoectasia is considered to be both a water-hammer effect and a direct compression of adjacent structures. We suggest prompt surgical intervention upon diagnosis as a first choice treatment in order to avoid further complications. PMID:26236456

Vertebrobasilar Dolichoectasia Induced Hydrocephalus: the Water-Hammer Effect.

PubMed

Zisimopoulou, Vaso; Ntouniadaki, Aikaterini; Aggelidakis, Panagiotis; Siatouni, Anna; Gatzonis, Stylianos; Tavernarakis, Antonios

2015-04-24

Vertebrobasilar dolichoectasia is a clinical entity associated rarely with obstructive hydrocephalus. We present a 48-year old male with a profound dilatation of the ventricular system due to a dolichoectatic basilar artery, as appeared in imaging studies. The patient suffered from longstanding hydrocephalus and presenile dementia. The underlying mechanism for obstructive hydrocephalus due to vertebrobasilar dolichoectasia is considered to be both a water-hammer effect and a direct compression of adjacent structures. We suggest prompt surgical intervention upon diagnosis as a first choice treatment in order to avoid further complications.

Endoscopic third ventriculostomy for hydrocephalus in brainstem glioma: a case series.

PubMed

Kobayashi, Natsuki; Ogiwara, Hideki

2016-07-01

A brainstem glioma is an incurable brain tumor that can be complicated by hydrocephalus. A ventriculoperitoneal (VP) shunt is generally performed for the control of hydrocephalus. This study aimed to reveal the safety and efficacy of an endoscopic third ventriculostomy (ETV) for hydrocephalus in brainstem gliomas. Six patients who had pontine glioma with hydrocephalus underwent an ETV between May 2010 and November 2015. In all the cases, there were one or more symptoms of hydrocephalus (headache, nausea, vomiting, or lethargy). Retrospective review of these patients was performed using the medical records and neuroimagings. The ETV was performed safely and there were no intraoperative complications in all patients. The mean follow-up period was 12.3 months. An immediate symptomatic relief of hydrocephalus and an adequate control of symptoms were achieved without a VP shunt in all patients. The ETV is considered to be an effective and safe procedure for the treatment of hydrocephalus in brainstem gliomas. Determining the ventriculostomy site according to the preoperative MRI in each case is considered to be important for the safe procedure.

Study on the Automatic Detection Method and System of Multifunctional Hydrocephalus Shunt

NASA Astrophysics Data System (ADS)

Sun, Xuan; Wang, Guangzhen; Dong, Quancheng; Li, Yuzhong

2017-07-01

Aiming to the difficulty of micro pressure detection and the difficulty of micro flow control in the testing process of hydrocephalus shunt, the principle of the shunt performance detection was analyzed.In this study, the author analyzed the principle of several items of shunt performance detection,and used advanced micro pressure sensor and micro flow peristaltic pump to overcome the micro pressure detection and micro flow control technology.At the same time,This study also puted many common experimental projects integrated, and successfully developed the automatic detection system for a shunt performance detection function, to achieve a test with high precision, high efficiency and automation.

Fractionated Stereotactic Radiotherapy in the Treatment of Vestibular Schwannoma (Acoustic Neuroma): Predicting the Risk of Hydrocephalus;Vestibular schwannoma; Hydrocephalus; Fractionated; Stereotactic radiotherapy

DOE Office of Scientific and Technical Information (OSTI.GOV)

Powell, Ceri; Micallef, Caroline; Gonsalves, Adam

2011-07-15

Purpose: To determine the incidence and predictive factors for the development of hydrocephalus in patients with acoustic neuromas (AN) treated with fractionated stereotactic radiotherapy. Patients and Methods: Seventy-two patients with AN were treated with fractionated stereotactic radiotherapy between 1998 and 2007 (45-50 Gy in 25-30 fractions over 5 to 6 weeks). The pretreatment MRI scan was assessed for tumor characteristics and anatomic distortion independently of subsequent outcome and correlated with the risk of hydrocephalus. Results: At a median follow-up of 49 months (range, 1-120 months), 5-year event-free survival was 95%. Eight patients (11%) developed hydrocephalus within 19 months of radiotherapy,more » which was successfully treated. On univariate analysis, pretreatment factors predictive of hydrocephalus were maximum diameter (p = 0.005), proximity to midline (p = 0.009), displacement of the fourth ventricle (p = 0.02), partial effacement of the fourth ventricle (p < 0.001), contact with the medulla (p = 0.005), and more brainstem structures (p = 0.004). On multivariate analysis, after adjusting for fourth ventricular effacement, no other variables remained independently associated with hydrocephalus formation. Conclusions: Fractionated stereotactic radiotherapy results in excellent tumor control of AN, albeit with a risk of developing hydrocephalus. Patients at high risk, identified as those with larger tumors with partial effacement of the fourth ventricle before treatment, should be monitored more closely during follow-up. It would also be preferable to offer treatment to patients with progressive AN while the risk of hydrocephalus is low, before the development of marked distortion of fourth ventricle before tumor diameter significantly exceeds 2 cm.« less

Meaning Construction and Integration in Children with Hydrocephalus

ERIC Educational Resources Information Center

Barnes, Marcia A.; Faulkner, Heather; Wilkinson, Margaret; Dennis, Maureen

2004-01-01

Text comprehension processes were investigated in children with hydrocephalus, a neurodevelopmental disorder associated with good word decoding, but deficient reading comprehension. In Experiment 1, hydrocephalus and control groups were similar in processes related to activating word meanings and using context to enhance meaning. The hydrocephalus…

Idiopathic Intracranial Hypertension in Monozygotic Female Twins: Intracranial Pressure Dynamics and Treatment Outcome.

PubMed

Polemikos, Manolis; Heissler, Hans E; Hermann, Elvis J; Krauss, Joachim K

2017-05-01

Familial cases of idiopathic intracranial hypertension (IIH) are exceedingly rare, and its occurrence in monozygotic twins has not been reported previously. We report monozygotic female twins who developed IIH, one at age 25 years and the other at age 28 years. Continuous intracranial pressure (ICP) monitoring confirmed elevated ICP as measured initially by lumbar puncture. In both cases, successful treatment with resolution of papilledema and symptoms relief was achieved after ventriculoperitoneal shunting. This report documents the first case of IIH in monozygotic twins and the associated changes in ICP dynamics. Interestingly, almost equivalent alterations in ICP dynamics were found in the 2 patients. Copyright © 2017 Elsevier Inc. All rights reserved.

Impact of operation details on hydrocephalus after decompressive craniectomy

PubMed Central

Wang, Qiang-Ping; Ma, Jun-Peng; Zhou, Zhang-Ming; You, Chao

2016-01-01

Objective: To evaluate the correlation between the distance of craniectomy from the midline and hydrocephalus after DC. Methods: The following electronic databases were searched from their inception to June 2015: Cochrane Library, MEDLINE, Science Direct, EMBASE, Scopus, Google Scholar, the Chinese Biomedical Database (CBM), and the Chinese National Knowledge Infrastructure (CNKI). All randomized clinical trials, prospective cohort, retrospective observational cohort, and case-control studies investigating the relationship between distance of craniectomy from the midline and hydrocephalus after DC were enrolled. The Cochrane Collaboration’s software RevMan 5.3 was used for meta-analysis. Results: Six retrospective cohort studies involving 462 participants were included. Pooled analysis of 4 studies suggested that craniectomy close to the midline (<25 mm) was associated with a significantly increased risk of postoperative hydrocephalus (odds ratio [OR] = 3.61, 95% confidence interval [CI]: 1.3 - 9.97, p=0.01). However, meta-analysis of 4 studies did not find statistical differences when comparing the distance of craniectomy from the midline in the hydrocephalus group and that in the non-hydrocephalus group (OR = −0.14, 95% CI: −0.44 - 0.15, p=0.34). Conclusions: Available evidence was insufficient to support the theory that craniectomy close to the midline increases the risk of developing hydrocephalus after DC. Well-conducted randomized clinical trials are required to verify this issue. PMID:26818161

Contemporary occurrence of hydrocephalus and Chiari I malformation in sagittal craniosynostosis. Case report and review of the literature.

PubMed

Sgulò, Francesco Giovanni; Spennato, Pietro; Aliberti, Ferdinando; Di Martino, Giuliana; Cascone, Daniele; Cinalli, Giuseppe

2017-01-01

Chiari malformation type I (CM-I) and hydrocephalus are often associated with complex craniosynostosis. On the contrary, their simultaneous occurrence in monosutural synostosis is extremely rare. The pathophysiological hypothesis is that they may alter posterior fossa growth and lead to cerebellar tonsil herniation also without skull base primary involvement. Hydrocephalus is multifactorial and may be secondary to fourth ventricle outlet obstruction. The management of these cases is quite complex and not well defined. Cranial vault remodeling should be the only treatment when CM-I is asymptomatic and not related to syringomyelia. Suboccipital decompression should be reserved only in complicated CM-I, usually as a second surgical step following the correction of the supratentorial deformity. In our opinion, the associated hydrocephalus should be treated first in order to normalize intracranial hypertension before opening the cranial sutures. We report the case of a 26-month-old child that presented with sagittal craniosynostosis, hydrocephalus, and CM-I. He was managed by performing endoscopic third ventriculostomy (ETV) first and cranial vault remodeling thereafter. Clinico-radiological outcome was very satisfying. Concerning literature is reviewed; physiopathology and surgical management are discussed.

Incidence and predictors of dural venous sinus pressure gradient in idiopathic intracranial hypertension and non-idiopathic intracranial hypertension headache patients: results from 164 cerebral venograms.

PubMed

Levitt, Michael R; Hlubek, Randall J; Moon, Karam; Kalani, M Yashar S; Nakaji, Peter; Smith, Kris A; Little, Andrew S; Knievel, Kerry; Chan, Jane W; McDougall, Cameron G; Albuquerque, Felipe C

2017-02-01

OBJECTIVE Cerebral venous pressure gradient (CVPG) from dural venous sinus stenosis is implicated in headache syndromes such as idiopathic intracranial hypertension (IIH). The incidence of CVPG in headache patients has not been reported. METHODS The authors reviewed all cerebral venograms with manometry performed for headache between January 2008 and May 2015. Patient demographics, headache etiology, intracranial pressure (ICP) measurements, and radiographic and manometric results were recorded. CVPG was defined as a difference ≥ 8 mm Hg by venographic manometry. RESULTS One hundred sixty-four venograms were performed in 155 patients. There were no procedural complications. Ninety-six procedures (58.5%) were for patients with IIH. The overall incidence of CVPG was 25.6% (42 of 164 procedures): 35.4% (34 of 96 procedures) in IIH patients and 11.8% (8 of 68 procedures) in non-IIH patients. Sixty procedures (36.6%) were performed in patients with preexisting shunts. Seventy-seven patients (49.7%) had procedures preceded by an ICP measurement within 4 weeks of venography, and in 66 (85.7%) of these patients, the ICP had been found to be elevated. CVPG was seen in 8.3% (n = 5) of the procedures in the 60 patients with a preexisting shunt and in 0% (n = 0) of the 11 procedures in the 77 patients with normal ICP (p < 0.001 for both). Noninvasive imaging (MR venography, CT venography) was assessed prior to venography in 112 (68.3%) of 164 cases, and dural venous sinus abnormalities were demonstrated in 73 (65.2%) of these cases; there was a trend toward CVPG (p = 0.07). Multivariate analysis demonstrated an increased likelihood of CVPG in patients with IIH (OR 4.97, 95% CI 1.71-14.47) and a decreased likelihood in patients with a preexisting shunt (OR 0.09, 95% CI 0.02-0.44). CONCLUSIONS CVPG is uncommon in IIH patients, rare in those with preexisting shunts, and absent in those with normal ICP.

A physical framework for implementing virtual models of intracranial pressure and cerebrospinal fluid dynamics in hydrocephalus shunt testing.

PubMed

Venkataraman, Pranav; Browd, Samuel R; Lutz, Barry R

2016-09-01

OBJECTIVE The surgical placement of a shunt designed to resolve the brain's impaired ability to drain excess CSF is one of the most common treatments for hydrocephalus. The use of a dynamic testing platform is an important part of shunt testing that can faithfully reproduce the physiological environment of the implanted shunts. METHODS A simulation-based framework that serves as a proof of concept for enabling the application of virtual intracranial pressure (ICP) and CSF models to a physical shunt-testing system was engineered. This was achieved by designing hardware and software that enabled the application of dynamic model-driven inlet and outlet pressures to a shunt and the subsequent measurement of the resulting drainage rate. RESULTS A set of common physiological scenarios was simulated, including oscillations in ICP due to respiratory and cardiac cycles, changes in baseline ICP due to changes in patient posture, and transient ICP spikes caused by activities such as exercise, coughing, sneezing, and the Valsalva maneuver. The behavior of the Strata valve under a few of these physiological conditions is also demonstrated. CONCLUSIONS Testing shunts with dynamic ICP and CSF simulations can facilitate the optimization of shunts to be more failure resistant and better suited to patient physiology.

Loss of Dishevelleds disrupts planar polarity in ependymal motile cilia and results in hydrocephalus

PubMed Central

Ohata, Shinya; Nakatani, Jin; Herranz-Pérez, Vicente; Cheng, JrGang; Belinson, Haim; Inubushi, Toshiro; Snider, William D.; García-Verdugo, Jose Manuel; Wynshaw-Boris, Anthony; Álvarez-Buylla, Arturo

2014-01-01

SUMMARY Defects in ependymal (E) cells, which line the ventricle and generate cerebrospinal fluid flow through ciliary beating, can cause hydrocephalus. Dishevelled genes (Dvls) are essential for Wnt signaling and Dvl2 has been shown to localize to the rootlet of motile cilia. Using the hGFAP-Cre;Dvl1−/−;2flox/flox;3+/− mouse, we show that compound genetic ablation of Dvls causes hydrocephalus. In hGFAP-Cre;Dvl1−/−;2flox/flox;3+/− mutants, E cells differentiated normally, but the intracellular and intercellular rotational alignments of ependymal motile cilia were disrupted. As a consequence, the fluid flow generated by the hGFAP-Cre;Dvl1−/−;2flox/flox;3+/− E cells was significantly slower than that observed in control mice. Dvls were also required for the proper positioning of motile cilia on the apical surface. Tamoxifen-induced conditional removal of Dvls in adult mice also resulted in defects in intracellular rotational alignment and positioning of ependymal motile cilia. These results suggest that Dvls are continuously required for E cell planar polarity and may prevent hydrocephalus. PMID:25043421

Long-term hydrocephalus alters the cytoarchitecture of the adult subventricular zone

PubMed Central

Campos-Ordoñez, Tania; Herranz-Pérez, Vicente; Chaichana, Kaisorn L.; Rincon-Torroella, Jordina; Rigamonti, Daniele; García-Verdugo, Jose M.; Quiñones-Hinojosa, Alfredo; Gonzalez-Perez, Oscar

2014-01-01

Hydrocephalus can develop secondarily to a disturbance in production, flow and/or absorption of cerebrospinal fluid. Experimental models of hydrocephalus, especially subacute and chronic hydrocephalus, are few and limited, and the effects of hydrocephalus on the subventricular zone are unclear. The aim of this study was to analyze the effects of long-term obstructive hydrocephalus on the subventricular zone, which is the neurogenic niche lining the lateral ventricles. We developed a new method to induce hydrocephalus by obstructing the aqueduct of Sylvius in the mouse brain, thus simulating aqueductal stenosis in humans. In 120-day-old rodents (n = 18 per group), the degree of ventricular dilatation and cellular composition of the subventricular zone were studied by immunofluorescence and transmission electron microscopy. In adult patients (age > 18 years), the sizes of the subventricular zone, corpus callosum, and internal capsule were analyzed by magnetic resonance images obtained from patients with and without aqueductal stenosis (n=25 per group). Mice with 60-day hydrocephalus had a reduced number of Ki67+ and doublecortin+ cells on immunofluorescence, as well as decreased number of neural progenitors and neuroblasts in the subventricular zone on electron microscopy analysis as compared to non-hydrocephalic mice. Remarkably, a number of extracellular matrix structures (fractones) contacting the ventricular lumen and blood vessels were also observed around the subventricular zone in mice with hydrocephalus. In humans, the widths of the subventricular zone, corpus callosum, and internal capsule in patients with aqueductal stenosis were significantly smaller than age and gender-matched patients without aqueductal stenosis. In summary, supratentorial hydrocephalus reduces the proliferation rate of neural progenitors and modifies the cytoarchitecture and extracellular matrix compounds of the subventricular zone. In humans, this similar process reduces the

Long-term hydrocephalus alters the cytoarchitecture of the adult subventricular zone.

PubMed

Campos-Ordoñez, Tania; Herranz-Pérez, Vicente; Chaichana, Kaisorn L; Rincon-Torroella, Jordina; Rigamonti, Daniele; García-Verdugo, Jose M; Quiñones-Hinojosa, Alfredo; Gonzalez-Perez, Oscar

2014-11-01

Hydrocephalus can develop secondarily to a disturbance in production, flow and/or absorption of cerebrospinal fluid. Experimental models of hydrocephalus, especially subacute and chronic hydrocephalus, are few and limited, and the effects of hydrocephalus on the subventricular zone are unclear. The aim of this study was to analyze the effects of long-term obstructive hydrocephalus on the subventricular zone, which is the neurogenic niche lining the lateral ventricles. We developed a new method to induce hydrocephalus by obstructing the aqueduct of Sylvius in the mouse brain, thus simulating aqueductal stenosis in humans. In 120-day-old rodents (n=18 per group), the degree of ventricular dilatation and cellular composition of the subventricular zone were studied by immunofluorescence and transmission electron microscopy. In adult patients (age>18years), the sizes of the subventricular zone, corpus callosum, and internal capsule were analyzed by magnetic resonance images obtained from patients with and without aqueductal stenosis (n=25 per group). Mice with 60-day hydrocephalus had a reduced number of Ki67+ and doublecortin+cells on immunofluorescence, as well as decreased number of neural progenitors and neuroblasts in the subventricular zone on electron microscopy analysis as compared to non-hydrocephalic mice. Remarkably, a number of extracellular matrix structures (fractones) contacting the ventricular lumen and blood vessels were also observed around the subventricular zone in mice with hydrocephalus. In humans, the widths of the subventricular zone, corpus callosum, and internal capsule in patients with aqueductal stenosis were significantly smaller than age and gender-matched patients without aqueductal stenosis. In summary, supratentorial hydrocephalus reduces the proliferation rate of neural progenitors and modifies the cytoarchitecture and extracellular matrix compounds of the subventricular zone. In humans, this similar process reduces the subventricular

Predictors and outcomes of shunt-dependent hydrocephalus in patients with aneurysmal sub-arachnoid hemorrhage

PubMed Central

2012-01-01

Background Hydrocephalus following spontaneous aneurysmal sub-arachnoid hemorrhage (SAH) is often associated with unfavorable outcome. This study aimed to determine the potential risk factors and outcomes of shunt-dependent hydrocephalus in aneurysmal SAH patients but without hydrocephalus upon arrival at the hospital. Methods One hundred and sixty-eight aneurysmal SAH patients were evaluated. Using functional scores, those without hydrocephalus upon arrival at the hospital were compared to those already with hydrocephalus on admission, those who developed it during hospitalization, and those who did not develop it throughout their hospital stay. The Glasgow Coma Score, modified Fisher SAH grade, and World Federation of Neurosurgical Societies grade were determined at the emergency room. Therapeutic outcomes immediately after discharge and 18 months after were assessed using the Glasgow Outcome Score. Results Hydrocephalus accounted for 61.9% (104/168) of all episodes, including 82 with initial hydrocephalus on admission and 22 with subsequent hydrocephalus. Both the presence of intra-ventricular hemorrhage on admission and post-operative intra-cerebral hemorrhage were independently associated with shunt-dependent hydrocephalus in patients without hydrocephalus on admission. After a minimum 1.5 years of follow-up, the mean Glasgow outcome score was 3.33 ± 1.40 for patients with shunt-dependent hydrocephalus and 4.21 ± 1.19 for those without. Conclusions The presence of intra-ventricular hemorrhage, lower mean Glasgow Coma Scale score, and higher mean scores of the modified Fisher SAH and World Federation of Neurosurgical grading on admission imply risk of shunt-dependent hydrocephalus in patients without initial hydrocephalus. These patients have worse short- and long-term outcomes and longer hospitalization. PMID:22765765

[Disorders of higher mental functions in the early stages of hydrocephalus].

PubMed

Simernitskaia, E G; Simernitskiĭ, B P

1981-01-01

The authors have carried out neuropsychological examinations of 32 children operated for hydrocephalus on the first year of the life. A syndrome of the higher psychic function disturbances characteristics for children with early hydrocephalus is described, and the results of the neuropsychological function examinations were compared with the character, etiology, and gravity of the hydrocephalus. In the children with the early hydrocephalus a high incidence of sinistrality was revealed, the fact, that gives one grounds to explain the peculiarities of the syndrome observed in them by an anomaly of the development of the brain functional asymmetry.

Communicating Hydrocephalus Associated with Intracranial Schwannoma Treated by Gamma Knife Radiosurgery.

PubMed

Park, Chang Kyu; Lee, Sung Ho; Choi, Man Kyu; Choi, Seok Keun; Park, Bong Jin; Lim, Young Jin

2016-05-01

Gamma knife radiosurgery (GKRS) has been established as an effective and safe treatment for intracranial schwannoma. However, serious complications can occur after GKRS, including hydrocephalus. The pathophysiology and risk factors of this disorder are not yet fully understood. The objective of the study was to assess potential risk factors for hydrocephalus after GKRS. We retrospectively reviewed the medical radiosurgical records of 244 patients who underwent GKRS to treat intracranial schwannoma. The following parameters were analyzed as potential risk factors for hydrocephalus after GKRS: age, sex, target volume, irradiation dose, prior tumor resection, treatment technique, and tumor enhancement pattern. The tumor enhancement pattern was divided into 2 groups: group A (homogeneous enhancement) and group B (heterogeneous or rim enhancement). Of the 244 patients, 14 of them (5.7%) developed communicating hydrocephalus. Communicating hydrocephalus occurred within 2 years after GKRS in most patients (92.8%). No significant association was observed between any of the parameters investigated and the development of hydrocephalus, with the exception of tumor enhancement pattern. Group B exhibited a statistically significant difference by univariate analysis (P = 0.002); this difference was also significant by multivariate analysis (P = 0.006). Because hydrocephalus is curable, patients should be closely monitored for the development of this disorder after GKRS. In particular, patients with intracranial schwannomas with irregular enhancement patterns or cysts should be meticulously observed. Copyright © 2015 Elsevier Inc. All rights reserved.

Simulation of hydrocephalus condition in infant head

NASA Astrophysics Data System (ADS)

Wijayanti, Erna; Arif, Idam

2014-03-01

Hydrocephalus is a condition of an excessive of cerebrospinal fluid in brain. In this paper, we try to simulate the behavior of hydrocephalus conditions in infant head by using a hydro-elastic model which is combined with orthotropic elastic skull and with the addition of suture that divide the skull into two lobes. The model then gives predictions for the case of stenosis aqueduct by varying the cerebral aqueduct diameter, time constant and brain elastic modulus. The hydrocephalus condition which is shown by the significant value of ventricle displacement, as the result shows, is occurred when the aqueduct is as resistant as brain parenchyma for the flow of cerebrospinal fluid. The decrement of brain elastic modulus causes brain parenchyma displacement value approach ventricle displacement value. The smaller of time constant value causes the smaller value of ventricle displacement.

A new shunt for obstructive hydrocephalus: ventricle-Sylvian fissure shunt A preliminary report.

PubMed

Patkar, Sushil

2017-06-01

Cerbrospinal fluid (CSF) pathway studies have revealed that after egressing from the fourth ventricle reaches the basal supra sellar cistern and ultimately the sylvian cisterns from where the CSF travels over the cerebral convexity subarachnoid space to reach the superior saggital sinus and enters the blood stream. Diverting CSF from the lateral ventricle with a shunt catheter to the sylvian cistern can be an option to treat obstructive hydrocephalus. 2 patients underwent this procedure of diverting CSF from the lateral ventricle with a shunt catheter (Chabbra, India) to the sylvian cistern successfully and had immediate relief of symptoms of raised intracranial pressure. Additional 4 patients had relief for 3mths to 6 mths and are under follow up. Though preliminary results seem logical and promising, more cases and longer follow-up is required to consider this shunt operation as an option in treatment of obstructive hydrocephalus.

Two Hundred Thirty-Six Children With Developmental Hydrocephalus: Causes and Clinical Consequences.

PubMed

Tully, Hannah M; Ishak, Gisele E; Rue, Tessa C; Dempsey, Jennifer C; Browd, Samuel R; Millen, Kathleen J; Doherty, Dan; Dobyns, William B

2016-03-01

Few systematic assessments of developmental forms of hydrocephalus exist. We reviewed magnetic resonance images (MRIs) and clinical records of patients with infancy-onset hydrocephalus. Among 411 infants, 236 had hydrocephalus with no recognizable extrinsic cause. These children were assigned to 1 of 5 subtypes and compared on the basis of clinical characteristics and developmental and surgical outcomes. At an average age of 5.3 years, 72% of children were walking independently and 87% could eat by mouth; in addition, 18% had epilepsy. Distinct patterns of associated malformations and syndromes were observed within each subtype. On average, children with aqueductal obstruction, cysts, and encephaloceles had worse clinical outcomes than those with other forms of developmental hydrocephalus. Overall, 53% of surgically treated patients experienced at least 1 shunt failure, but hydrocephalus associated with posterior fossa crowding required fewer shunt revisions. We conclude that each subtype of developmental hydrocephalus is associated with distinct clinical characteristics, syndromology, and outcomes, suggesting differences in underlying mechanisms. © The Author(s) 2015.

Ion beam sputter-etched ventricular catheter for hydrocephalus shunt

NASA Technical Reports Server (NTRS)

Banks, B. A. (Inventor)

1983-01-01

A cerebrospinal fluid shunt in the form of a ventricular catheter for controlling the condition of hydrocephalus by relieving the excessive cerebrospinal fluid pressure is described. A method for fabrication of the catheter and shunting the cerebral fluid from the cerebral ventricles to other areas of the body is also considered. Shunt flow failure occurs if the ventricle collapse due to improper valve function causing overdrainage. The ventricular catheter comprises a multiplicity of inlet microtubules. Each microtubule has both a large openings at its inlet end and a multiplicity of microscopic openings along its lateral surfaces.

Blood pressure normalization post-jugular venous balloon angioplasty.

PubMed

Sternberg, Zohara; Grewal, Prabhjot; Cen, Steven; DeBarge-Igoe, Frances; Yu, Jinhee; Arata, Michael

2015-05-01

This study is the first in a series investigating the relationship between autonomic nervous system dysfunction and chronic cerebrospinal venous insufficiency in multiple sclerosis patients. We screened patients for the combined presence of the narrowing of the internal jugular veins and symptoms of autonomic nervous system dysfunction (fatigue, cognitive dysfunction, sleeping disorders, headache, thermal intolerance, bowel/bladder dysfunction) and determined systolic and diastolic blood pressure responses to balloon angioplasty. The criteria for eligibility for balloon angioplasty intervention included ≥ 50% narrowing in one or both internal jugular veins, as determined by the magnetic resonance venography, and ≥ 3 clinical symptoms of autonomic nervous system dysfunction. Blood pressure was measured at baseline and post-balloon angioplasty. Among patients who were screened, 91% were identified as having internal jugular veins narrowing (with obstructing lesions) combined with the presence of three or more symptoms of autonomic nervous system dysfunction. Balloon angioplasty reduced the average systolic and diastolic blood pressure. However, blood pressure categorization showed a biphasic response to balloon angioplasty. The procedure increased blood pressure in multiple sclerosis patients who presented with baseline blood pressure within lower limits of normal ranges (systolic ≤ 105 mmHg, diastolic ≤ 70 mmHg) but decreased blood pressure in patients with baseline blood pressure above normal ranges (systolic ≥ 130 mmHg, diastolic ≥ 80 mmHg). In addition, gender differences in baseline blood pressure subcategories were observed. The coexistence of internal jugular veins narrowing and symptoms of autonomic nervous system dysfunction suggests that the two phenomena may be related. Balloon angioplasty corrects blood pressure deviation in multiple sclerosis patients undergoing internal jugular vein dilation. Further studies should investigate the

Impairment of a Motor Skill in Children with Spina Bifida Cystica and Hydrocephalus: An Exploratory Study

ERIC Educational Resources Information Center

Anderson, Elizabeth M.; Plewis, Ian

1977-01-01

Twenty 7-10 year old children with spina bifida cystica and hydrocephalus and 20 normals matched for age, sex and IQ were compared on a 12-trial target task, first used by Connolly, Brown & Bassett (1968). Findings are discussed in relation to neurological abnormalities in the spina bifida group. (Editor/RK)

A mutation in Ccdc39 causes neonatal hydrocephalus with abnormal motile cilia development in mice.

PubMed

Abdelhamed, Zakia; Vuong, Shawn M; Hill, Lauren; Shula, Crystal; Timms, Andrew; Beier, David; Campbell, Kenneth; Mangano, Francesco T; Stottmann, Rolf W; Goto, June

2018-01-09

Pediatric hydrocephalus is characterized by an abnormal accumulation of cerebrospinal fluid (CSF) and is one of the most common congenital brain abnormalities. However, little is known about the molecular and cellular mechanisms regulating CSF flow in the developing brain. Through whole-genome sequencing analysis, we report that a homozygous splice site mutation in coiled-coil domain containing 39 ( Ccdc39 ) is responsible for early postnatal hydrocephalus in the progressive hydrocephal us ( prh ) mouse mutant. Ccdc39 is selectively expressed in embryonic choroid plexus and ependymal cells on the medial wall of the forebrain ventricle, and the protein is localized to the axoneme of motile cilia. The Ccdc39 prh/prh ependymal cells develop shorter cilia with disorganized microtubules lacking the axonemal inner arm dynein. Using high-speed video microscopy, we show that an orchestrated ependymal ciliary beating pattern controls unidirectional CSF flow on the ventricular surface, which generates bulk CSF flow in the developing brain. Collectively, our data provide the first evidence for involvement of Ccdc39 in hydrocephalus and suggest that the proper development of medial wall ependymal cilia is crucial for normal mouse brain development. © 2018. Published by The Company of Biologists Ltd.

The effect of backpack weight on the standing posture and balance of schoolgirls with adolescent idiopathic scoliosis and normal controls.

PubMed

Chow, Daniel H K; Kwok, Monica L Y; Cheng, Jack C Y; Lao, Miko L M; Holmes, Andrew D; Au-Yang, Alexander; Yao, Fiona Y D; Wong, M S

2006-10-01

Concerns have been raised regarding the effect of carrying a backpack on adolescent posture and balance, but the effect of backpack loading combined with other factors affecting balance, such as adolescent idiopathic scoliosis (AIS), has not been determined. This study examines the effects of backpack load on the posture and balance of schoolgirls with AIS and normal controls. The standing posture of 26 schoolgirls with mild AIS (mean age 13, Cobb angle 10-25 degrees ) and 20 age-matched normal schoolgirls were recorded without a backpack and while carrying a standard dual-strap backpack loaded at 7.5%, 10%, 12.5% and 15% of the subject's bodyweight (BW). Kinematics of the pelvis, trunk and head were recorded using a motion analysis system and centre of pressure (COP) data were recorded using a force platform. Reliable COP data could only be derived for 13 of the subjects with AIS. Increasing backpack load causes a significantly increased flexion of the trunk in relation to the pelvis and extension of the head in relation to the trunk, as well as increased antero-posterior range of COP motion. While backpack load appears to affect balance predominantly in the antero-posterior direction, differences between groups were more evident in the medio-lateral direction, with AIS subjects showing poor balance in this direction. Overall, carrying a backpack causes similar sagittal plane changes in posture and balance in both normal and AIS groups. Load size or subject group did not influence balance, but the additive effect of backpack carrying and AIS on postural control alters the risk of fall in this population. Therefore, load limit recommendations based on normal subjects should not be applicable to subjects with AIS.

Efficacy of endoscopic third ventriculostomy in non-communicating hydrocephalus.

PubMed

ul Haq, Mian Iftikhar; Khan, Shahbaz Ali; Raja, Riaz A; Ahmed, Ehtisham

2012-01-01

Hydrocephalus is common problem requiring either extra-cranial (shunts) or intracranial (ventriculostomy) diversion of cerebrospinal fluid. Endoscopic third ventriculostomy obviates all the complications of shunts and has been accepted as the procedure of choice for the treatment of obstructed hydrocephalus in adults and children because of the minimally invasive nature. This study was conducted to determine the efficacy of endoscopic third ventriculostomy in the treatment of non-communicating hydrocephalus. This cross sectional descriptive study was done in neurosurgery department of Hayatabad Medical Complex, Peshawar, from 2nd February 2011 to 1st march 2012. A total of 171 patients with non-communicating hydrocephalous, irrespective of gender discrimination and Glasgow coma scale score of 10 and above were included in this study. Patients below one year of age, with lesion in the floor of the third ventricle or near basilar artery, and hydrocephalus with infected CSF or haemorrhage were excluded. Hydrocephalous was diagnosed on CT-scan brain. All the patients were followed up till 72 hours post-operatively for the determination of effectiveness in terms of improvement in Glasgow coma scale by at least 2 points. All the above mentioned information including name, age, gender and address were recorded in a predesigned proforma. The data was analysed using SPSS-17. Frequency and percentage was calculated for categorical variables. Mean +/- SD was calculated for age. A total of 171 patients with non-communicating hydrocephalous were included in the study. Out of 171 patients, there were 104 (60.8%) males and 67 (39.2%) females. Age ranged from 1-70 years with majority of the patients was below 10 years of age. Majority of the patients had hydrocephalus due to tuberculous meningitis 39.2% of the whole. In 134 (78.4%) patients the procedure was effective. Procedure was more effective in hydrocephalus due to space occupying lesion. Endoscopic third ventriculostomy is

Imaging normal pressure hydrocephalus: theories, techniques, and challenges.

PubMed

Keong, Nicole C H; Pena, Alonso; Price, Stephen J; Czosnyka, Marek; Czosnyka, Zofia; Pickard, John D

2016-09-01

The pathophysiology of NPH continues to provoke debate. Although guidelines and best-practice recommendations are well established, there remains a lack of consensus about the role of individual imaging modalities in characterizing specific features of the condition and predicting the success of CSF shunting. Variability of clinical presentation and imperfect responsiveness to shunting are obstacles to the application of novel imaging techniques. Few studies have sought to interpret imaging findings in the context of theories of NPH pathogenesis. In this paper, the authors discuss the major streams of thought for the evolution of NPH and the relevance of key imaging studies contributing to the understanding of the pathophysiology of this complex condition.

Clinical and Surgical Factors Associated With Increased Epilepsy Risk in Children With Hydrocephalus.

PubMed

Tully, Hannah M; Kukull, Walter A; Mueller, Beth A

2016-06-01

Children with hydrocephalus are at risk for epilepsy both due to their underlying condition and as a consequence of surgical treatment; however, the relative contributions of these factors remain unknown. The authors sought to characterize epilepsy among children with infancy-onset hydrocephalus and to examine the risks of epilepsy associated with hydrocephalus subtype and with factors related to surgical treatment. We conducted a longitudinal cohort study of all children with infancy-onset hydrocephalus treated at a major regional children's hospital during 2002 to 2012, with follow-up to ascertain risk factors and epilepsy outcome through April 2015. Poisson regression was used to calculate adjusted risk ratios and 95% confidence intervals for associations. Among 379 children with hydrocephalus, 86 (23%) developed epilepsy (mean onset age = 2.7 years), almost one fifth of whom had a history of infantile spasms. Relative to spina bifida-associated hydrocephalus, children with other major hydrocephalus subtypes had fourfold higher risks of developing epilepsy. Among children who underwent surgery, surgical infection doubled the risk of epilepsy (risk ratio = 2.0, 95% confidence interval = 1.4 to 3.0). Epilepsy was associated with surgical failure for intracranial reasons but not extracranial reasons (risk ratio = 1.7, 95% confidence interval = 1.1 to 2.7; risk ratio = 1.1, 95% confidence interval = 0.7 to 1.9, respectively). Epilepsy is common among children with hydrocephalus. Compared with children with spina bifida-associated hydrocephalus, children with other major hydrocephalus subtypes have a markedly increased risk of epilepsy. Surgical infection doubles the risk of epilepsy. Copyright © 2016 Elsevier Inc. All rights reserved.

Intra-ocular pressure normalization technique and equipment

NASA Technical Reports Server (NTRS)

Baehr, E. F. (Inventor)

1979-01-01

A method and apparatus is described for safely reducing abnormally high intraocular pressure in an eye during a predetermined time interval. This allows maintenance of normal intraocular pressure during glaucoma surgery. A pressure regulator of the spring-biassed diaphragm type is provided with additional bias by a column of liquid. The hypodermic needle can be safely inserted into the anterior chamber of the eye. Liquid is then bled out of the column to reduce the bias on the diaphragm of the pressure regulator and, consequently, the output pressure of the regulator. This lowering pressure of the regulator also occurs in the eye by means of a small second bleed path provided between the pressure regulator and the hypodermic needle.

Telemetric implantable pressure sensor for short- and long-term monitoring of intracranial pressure.

PubMed

Frischholz, M; Sarmento, L; Wenzel, M; Aquilina, K; Edwards, R; Coakham, H B

2007-01-01

Patients with hydrocephalus, idiopathic intracranial hypertension and head injury frequently require monitoring of intracranial pressure (ICP) and may need repeated episodes of monitoring months or years apart. The gold standard for measurement of ICP remains the external ventricular catheter. This is a fluid-filled catheter transducer system that allows regular recalibration and correction of zero drift by its position relative to a fixed anatomical reference. It also allows drainage of cerebrospinal fluid (CSF), providing a means of lowering the ICP. Several catheter tip transducer systems are currently in clinical use, including using strain gauges or fiber-optical pressure sensing techniques. In these devices, zero drift and calibration cannot be checked in vivo. All the ICP monitoring devices in current clinical use require a physical connection between the brain and the external environment. This is a source of infection and limits the duration of monitoring. A number of telemetric monitoring devices, in which data is in some way transmitted transcutaneously, have been developed over the last twenty years, but significant technical problems have precluded their use in routine clinical practice. All current ICP monitors are temporary percutaneous implanted devices. Placement of these devices carries significant morbidity, particularly infection. Patients undergoing repeated monitoring require multiple surgical procedures. Apart from decreasing the risk of infection in patients with severe head injury, the clinical value of an accurate telemetric ICP monitoring system which maintains its reliability over a long period of implantation is high.

Early variations of laboratory parameters predicting shunt-dependent hydrocephalus after subarachnoid hemorrhage.

PubMed

Na, Min Kyun; Won, Yu Deok; Kim, Choong Hyun; Kim, Jae Min; Cheong, Jin Hwan; Ryu, Je Il; Han, Myung-Hoon

2017-01-01

Hydrocephalus is a frequent complication following subarachnoid hemorrhage. Few studies investigated the association between laboratory parameters and shunt-dependent hydrocephalus. This study aimed to investigate the variations of laboratory parameters after subarachnoid hemorrhage. We also attempted to identify predictive laboratory parameters for shunt-dependent hydrocephalus. Multiple imputation was performed to fill the missing laboratory data using Bayesian methods in SPSS. We used univariate and multivariate Cox regression analyses to calculate hazard ratios for shunt-dependent hydrocephalus based on clinical and laboratory factors. The area under the receiver operating characteristic curve was used to determine the laboratory risk values predicting shunt-dependent hydrocephalus. We included 181 participants with a mean age of 54.4 years. Higher sodium (hazard ratio, 1.53; 95% confidence interval, 1.13-2.07; p = 0.005), lower potassium, and higher glucose levels were associated with higher shunt-dependent hydrocephalus. The receiver operating characteristic curve analysis showed that the areas under the curve of sodium, potassium, and glucose were 0.649 (cutoff value, 142.75 mEq/L), 0.609 (cutoff value, 3.04 mmol/L), and 0.664 (cutoff value, 140.51 mg/dL), respectively. Despite the exploratory nature of this study, we found that higher sodium, lower potassium, and higher glucose levels were predictive values for shunt-dependent hydrocephalus from postoperative day (POD) 1 to POD 12-16 after subarachnoid hemorrhage. Strict correction of electrolyte imbalance seems necessary to reduce shunt-dependent hydrocephalus. Further large studies are warranted to confirm our findings.

Early variations of laboratory parameters predicting shunt-dependent hydrocephalus after subarachnoid hemorrhage

PubMed Central

Kim, Choong Hyun; Kim, Jae Min; Cheong, Jin Hwan; Ryu, Je il

2017-01-01

Background and purpose Hydrocephalus is a frequent complication following subarachnoid hemorrhage. Few studies investigated the association between laboratory parameters and shunt-dependent hydrocephalus. This study aimed to investigate the variations of laboratory parameters after subarachnoid hemorrhage. We also attempted to identify predictive laboratory parameters for shunt-dependent hydrocephalus. Methods Multiple imputation was performed to fill the missing laboratory data using Bayesian methods in SPSS. We used univariate and multivariate Cox regression analyses to calculate hazard ratios for shunt-dependent hydrocephalus based on clinical and laboratory factors. The area under the receiver operating characteristic curve was used to determine the laboratory risk values predicting shunt-dependent hydrocephalus. Results We included 181 participants with a mean age of 54.4 years. Higher sodium (hazard ratio, 1.53; 95% confidence interval, 1.13–2.07; p = 0.005), lower potassium, and higher glucose levels were associated with higher shunt-dependent hydrocephalus. The receiver operating characteristic curve analysis showed that the areas under the curve of sodium, potassium, and glucose were 0.649 (cutoff value, 142.75 mEq/L), 0.609 (cutoff value, 3.04 mmol/L), and 0.664 (cutoff value, 140.51 mg/dL), respectively. Conclusions Despite the exploratory nature of this study, we found that higher sodium, lower potassium, and higher glucose levels were predictive values for shunt-dependent hydrocephalus from postoperative day (POD) 1 to POD 12–16 after subarachnoid hemorrhage. Strict correction of electrolyte imbalance seems necessary to reduce shunt-dependent hydrocephalus. Further large studies are warranted to confirm our findings. PMID:29232410

Uptake of (/sup 14/C)deoxyglucose into brain of young rats with inherited hydrocephalus

DOE Office of Scientific and Technical Information (OSTI.GOV)

Richards, H.K.; Bucknall, R.M.; Jones, H.C.

1989-02-01

The effect of hydrocephalus on cerebral glucose utilization as reflected by deoxyglucose uptake has been examined in rats with inherited hydrocephalus at 10, 20, and 28 days after birth using a semiquantitative method. Injection of (14C)deoxyglucose intraperitoneally was followed by freezing the brain, sectioning, and quantitative autoradiography of 10 brain regions. Brain (14C) concentration, cortical thickness, and plasma glucose concentrations were measured. Maximal thinning of the cerebral cortex had already occurred by 10 days after birth, although obvious symptoms such as gait disturbance developed after 20 days. In control rats, the cerebral isotope concentration was lower and more homogeneous atmore » 10 days than at 20 or 28 days, which may be a reflection of the use of metabolic substrates other than glucose in younger animals. In order to make comparisons between control and hydrocephalic groups, tissue isotope concentrations were normalized to cerebellar cortex which was not affected by the hydrocephalus at any age. In hydrocephalic rats at 10 and 20 days, the concentration of (14C) was lower in all areas except the inferior colliculi and pons but the reduction was only significant in the sensory-motor cortex at 10 days and in the caudate nuclei at 20 days. By 28 days after birth, all areas except the cerebellum (six cortical regions, inferior colliculi, pons, and caudate) had significantly lower isotope concentrations in the hydrocephalic group. It is concluded that cerebral glucose metabolism is significantly reduced by 28 days after birth in H-Tx rats with congenital hydrocephalus and that less marked reductions occur prior to 28 days.« less

Dementia paralytica: deterioration from communicating hydrocephalus.

PubMed Central

Giménez-Roldán, S; Benito, C; Martin, M

1979-01-01

Five patients suffering from dementia paralytica who failed to improve or deteriorated after one or several high dosage courses of penicillin, had pneumoencephalographic patterns suggesting communicating hydrocephalus. Measurements of the ventricular index, ratio of cella media to width of the temporal horn, and the callosal angle differed from that in seven cases of dementia paralytica with associated cerebral atrophy. An isotope cisternogram in three cases with communicating hydrocephalus further confirmed a blockage of the cerebrospinal fluid (CSF) at the parasagittal subarachnoid space. Three patients exhibited the full syndrome of gait apraxia, incontinence, and pyramidal tract signs associated with a severe degree of dementia. Shunting of the CSF in three cases was followed by immediate improvement in two, one in a longlasting way. No active parenchymal inflammation was observed in any of three brain biopsy samples taken during surgery, except for leptomeningeal fibrosis in one. Chronic leptomeningitis in dementia paralytica may impair subarachnoid CSF absorption with subsequent communicating hydrocephalus. Progression or inadequate responses after therapeutic dose of penicillin in dementia paralytica should prompt investigation for this complication as an alternative, effective treatment could be offered. Images PMID:469557

Left hemisphere structural connectivity abnormality in pediatric hydrocephalus patients following surgery.

PubMed

Yuan, Weihong; Meller, Artur; Shimony, Joshua S; Nash, Tiffany; Jones, Blaise V; Holland, Scott K; Altaye, Mekibib; Barnard, Holly; Phillips, Jannel; Powell, Stephanie; McKinstry, Robert C; Limbrick, David D; Rajagopal, Akila; Mangano, Francesco T

2016-01-01

Neuroimaging research in surgically treated pediatric hydrocephalus patients remains challenging due to the artifact caused by programmable shunt. Our previous study has demonstrated significant alterations in the whole brain white matter structural connectivity based on diffusion tensor imaging (DTI) and graph theoretical analysis in children with hydrocephalus prior to surgery or in surgically treated children without programmable shunts. This study seeks to investigate the impact of brain injury on the topological features in the left hemisphere, contratelateral to the shunt placement, which will avoid the influence of shunt artifacts and makes further group comparisons feasible for children with programmable shunt valves. Three groups of children (34 in the control group, 12 in the 3-month post-surgery group, and 24 in the 12-month post-surgery group, age between 1 and 18 years) were included in the study. The structural connectivity data processing and analysis were performed based on DTI and graph theoretical analysis. Specific procedures were revised to include only left brain imaging data in normalization, parcellation, and fiber counting from DTI tractography. Our results showed that, when compared to controls, children with hydrocephalus in both the 3-month and 12-month post-surgery groups had significantly lower normalized clustering coefficient, lower small-worldness, and higher global efficiency (all p  < 0.05, corrected). At a regional level, both patient groups showed significant alteration in one or more regional connectivity measures in a series of brain regions in the left hemisphere (8 and 10 regions in the 3-month post-surgery and the 12-month post-surgery group, respectively, all p  < 0.05, corrected). No significant correlation was found between any of the global or regional measures and the contemporaneous neuropsychological outcomes [the General Adaptive Composite (GAC) from the Adaptive Behavior Assessment System, Second Edition (ABAS

Axial diffusivity of the corona radiata correlated with ventricular size in adult hydrocephalus.

PubMed

Cauley, Keith A; Cataltepe, Oguz

2014-07-01

Hydrocephalus causes changes in the diffusion-tensor properties of periventricular white matter. Understanding the nature of these changes may aid in the diagnosis and treatment planning of this relatively common neurologic condition. Because ventricular size is a common measure of the severity of hydrocephalus, we hypothesized that a quantitative correlation could be made between the ventricular size and diffusion-tensor changes in the periventricular corona radiata. In this article, we investigated this relationship in adult patients with hydrocephalus and in healthy adult subjects. Diffusion-tensor imaging metrics of the corona radiata were correlated with ventricular size in 14 adult patients with acute hydrocephalus, 16 patients with long-standing hydrocephalus, and 48 consecutive healthy adult subjects. Regression analysis was performed to investigate the relationship between ventricular size and the diffusion-tensor metrics of the corona radiata. Subject age was analyzed as a covariable. There is a linear correlation between fractional anisotropy of the corona radiata and ventricular size in acute hydrocephalus (r = 0.784, p < 0.001), with positive correlation with axial diffusivity (r = 0.636, p = 0.014) and negative correlation with radial diffusivity (r = 0.668, p = 0.009). In healthy subjects, axial diffusion in the periventricular corona radiata is more strongly correlated with ventricular size than with patient age (r = 0.466, p < 0.001, compared with r = 0.058, p = 0.269). Axial diffusivity of the corona radiata is linearly correlated with ventricular size in healthy adults and in patients with hydrocephalus. Radial diffusivity of the corona radiata decreases linearly with ventricular size in acute hydrocephalus but is not significantly correlated with ventricular size in healthy subjects or in patients with long-standing hydrocephalus.

Non-invasive detection and monitoring of experimental hydrocephalus with distortion product otoacoustic emissions.

PubMed

Ezerarslan, Hande; Beriat, Güçlü Kaan; Nurhat, Raziye Handan; Kazancı, Burak; Çelikkan, Ferda Topal; Sabuncuoğlu, Bizden; Sabuncuoğlu, Hakan

2016-08-01

We aimed to find out the effects of short term and long term hydrocephalus and intracranial ventricular volume changes on cochlear functions by using distortion product otoacoustic emission (DPOAE) in experimental hydrocephalus rat models for the first time in literature. This study was performed with 48 healthy, adult (8 weeks old), Sprague-Dawley rats which weighed between 200 and 240g. Six groups were formed in this study: short term control, short term sham, short term hydrocephalus, long term control, long term sham and long term hydrocephalus groups. Each group contained eight rats. Short term period was 4 weeks and long term period was 8 weeks after the study started. At the end of these periods, DPOAE measurements were performed and then rats were sacrificed to determine ventricular volumes. DPOAE values at all frequencies were significantly decreased in the short term hydrocephalus group when compared to the short term control and short term sham groups. DPOAE values at all frequencies were significantly decreased in the long term hydrocephalus group when compared to the long term control and long term sham groups. Besides, long term sham group which had higher ventricular volumes than long term control group also had lower DPOAE measurements. Significant associations were present between DPOAE measurements and ventricular volumes in hydrocephalus models. The functional disturbances in cochlear functions due to hydrocephalus have been demonstrated with DPOAE measurements in this study. DPOAE measurements may be thought as an easily applicable non-invasive method in detection and follow-up of patients with hydrocephalus. Our findings should be supported with clinical studies in humans. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

A wireless monitoring system for Hydrocephalus shunts.

PubMed

Narayanaswamy, A; Nourani, M; Tamil, L; Bianco, S

2015-08-01

Patients with Hydrocephalus are usually treated by diverting the excess Cerebrospinal Fluid (CSF) to other parts of the body using shunts. More than 40 percentage of shunts implanted fail within the first two years. Obstruction in the shunts is one of the major causes of failure (45 percent) and the detection of obstruction reduces the complexity of the revision surgery. This paper describes a proposed wireless monitoring system for clog detection and flow measurement in shunts. A prototype was built using multiple pressure sensors along the shunt catheters for sensing the location of clog and flow rate. Regular monitoring of flow rates can be used to adjust the valve in the shunt to prevent over drainage or under drainage of CSF. The accuracy of the flow measurement is more than 90 percent.

Cerebral spinal fluid (CSF) collection

MedlinePlus

... establish the diagnosis of normal pressure hydrocephalus. Normal Results Normal values typically range as follows: Pressure: 70 ... measurements or may test different specimens. What Abnormal Results Mean If the CSF looks cloudy, it could ...

Sensory Impairment and Head Circumference in Fragile X Syndrome, Down Syndrome and Idiopathic Intellectual Disability.

ERIC Educational Resources Information Center

Turk, Jeremy; Patton, Michael

2000-01-01

Eighteen boys with fragile X syndrome were compared with 42 with idiopathic intellectual disability, and 45 with Down syndrome. Boys with Down syndrome had more sensory problems and smaller head circumferences than normal. Head circumferences of boys with fragile X syndrome and with idiopathic intellectual disability were larger than normal.…

Amenorrhoea and reversible infertility due to obstructive hydrocephalus: literature review and case report.

PubMed

Hamilton, Kimberly; Iskandar, Bermans

2018-02-12

Endocrine abnormalities are well-recognized consequences of intracranial pathology such as pituitary tumours. Less commonly, hydrocephalus may lead to dysfunction of the endocrine system, presenting as amenorrhoea or precocious puberty. We present a case report and literature review of hydrocephalus causing endocrine abnormalities including reversible infertility. A 34 year-old female presented with amenorrhoea and infertility. MRI showed a third ventricular mass and hydrocephalus. The amenorrhoea resolved within weeks of endoscopic third ventriculostomy and tumour biopsy; pregnancy ensued within 6 months. Thirty-two cases of hydrocephalus-related amenorrhoea were reported between 1915 and 2007. All patients who underwent modern hydrocephalus treatment experienced partial or complete resolution of endocrine dysfunction. Successful pregnancy was reported in three patients, as in our case presentation. While mechanisms of dysfunction have not been completely elucidated, studies point toward loss of GnRH pulsatility due to compression of the medio-basal hypothalamic structures. Hydrocephalus can cause endocrine dysfunction, including amenorrhoea, which may reverse with CSF diversion. Therefore, cranial imaging is an important component in the evaluation of such endocrine abnormalities.

Conditional N-WASP knockout in mouse brain implicates actin cytoskeleton regulation in hydrocephalus pathology.

PubMed

Jain, Neeraj; Lim, Lee Wei; Tan, Wei Ting; George, Bhawana; Makeyev, Eugene; Thanabalu, Thirumaran

2014-04-01

Cerebrospinal fluid (CSF) is produced by the choroid plexus and moved by multi-ciliated ependymal cells through the ventricular system of the vertebrate brain. Defects in the ependymal layer functionality are a common cause of hydrocephalus. N-WASP (Neural-Wiskott Aldrich Syndrome Protein) is a brain-enriched regulator of actin cytoskeleton and N-WASP knockout caused embryonic lethality in mice with neural tube and cardiac abnormalities. To shed light on the role of N-WASP in mouse brain development, we generated N-WASP conditional knockout mouse model N-WASP(fl/fl); Nestin-Cre (NKO-Nes). NKO-Nes mice were born with Mendelian ratios but exhibited reduced growth characteristics compared to their littermates containing functional N-WASP alleles. Importantly, all NKO-Nes mice developed cranial deformities due to excessive CSF accumulation and did not survive past weaning. Coronal brain sections of these animals revealed dilated lateral ventricles, defects in ciliogenesis, loss of ependymal layer integrity, reduced thickness of cerebral cortex and aqueductal stenosis. Immunostaining for N-cadherin suggests that ependymal integrity in NKO-Nes mice is lost as compared to normal morphology in the wild-type controls. Moreover, scanning electron microscopy and immunofluorescence analyses of coronal brain sections with anti-acetylated tubulin antibodies revealed the absence of cilia in ventricular walls of NKO-Nes mice indicative of ciliogenesis defects. N-WASP deficiency does not lead to altered expression of N-WASP regulatory proteins, Fyn and Cdc42, which have been previously implicated in hydrocephalus pathology. Taken together, our results suggest that N-WASP plays a critical role in normal brain development and implicate actin cytoskeleton regulation as a vulnerable axis frequently deregulated in hydrocephalus. Copyright © 2014 Elsevier Inc. All rights reserved.

Melatonin Attenuates Histopathological Changes in the Hippocampus of Infantile Rats with Kaolin-Induced Hydrocephalus.

PubMed

Turgut, Mehmet; Baka, Meral; Uyanıkgil, Yiğit

2018-05-23

Hydrocephalus is defined as an incapacitating neurological disorder characterized by ventricular enlargement in children, but the effects of melatonin on this hydrocephalus have not yet been fully elucidated. In the present experiment, we attempted to investigate the effects of exogenous melatonin administration on hydrocephalus-induced hippocampal changes in infantile rats. In this study, we randomly divided 45 Swiss albino rats aged 2 weeks into 3 groups: group I, the control group received a sham injection with needle insertion only; groups II and III were given kaolin injections before treatment - group II, the hydrocephalus group, was treated with an isotonic NaCl solution, and group III, the hydrocephalus plus melatonin group, was treated with 0.5 mg/100 g body weight of exogenous melatonin. Both immunohistochemical and histological analyses were performed after hydrocephalus induction and melatonin administration. Immunohistochemical staining consisted anti-glial fibrillary acidic protein staining. The TUNEL technique was used for defining quantitate apoptosis. Melatonin administration significantly attenuated chronic hydrocephalus-induced histopathological changes in the hippocampal subregions of infantile rats. Compared to hydrocephalic rats treated with saline solution, melatonin significantly decreased the number of apoptotic cells and pyknotic index values of each hippocampal subregion after the kaolin-induced hydrocephalus (p < 0.001). The present results demonstrate that the chronic hydrocephalus-induced histopathological changes in the hippocampus were partially reversible with melatonin treatment, suggesting its neuroprotective effects in infantile rats. However, these findings need to be confirmed by further experimental studies and clinical trials. © 2018 S. Karger AG, Basel.

Altered Regional Cerebral Blood Flow in Idiopathic Hypersomnia.

PubMed

Boucetta, Soufiane; Montplaisir, Jacques; Zadra, Antonio; Lachapelle, Francis; Soucy, Jean-Paul; Gravel, Paul; Dang-Vu, Thien Thanh

2017-10-01

Idiopathic hypersomnia is characterized by excessive daytime sleepiness, despite normal or long sleep time. Its pathophysiological mechanisms remain unclear. This pilot study aims at characterizing the neural correlates of idiopathic hypersomnia using single photon emission computed tomography. Thirteen participants with idiopathic hypersomnia and 16 healthy controls were scanned during resting wakefulness using a high-resolution single photon emission computed tomography scanner with 99mTc-ethyl cysteinate dimer to assess cerebral blood flow. The main analysis compared regional cerebral blood flow distribution between the two groups. Exploratory correlations between regional cerebral blood flow and clinical characteristics evaluated the functional correlates of those brain perfusion patterns. Significance was set at p < .05 after correction for multiple comparisons. Participants with idiopathic hypersomnia showed regional cerebral blood flow decreases in medial prefrontal cortex and posterior cingulate cortex and putamen, as well as increases in amygdala and temporo-occipital cortices. Lower regional cerebral blood flow in the medial prefrontal cortex was associated with higher daytime sleepiness. These preliminary findings suggest that idiopathic hypersomnia is characterized by functional alterations in brain areas involved in the modulation of vigilance states, which may contribute to the daytime symptoms of this condition. The distribution of regional cerebral blood flow changes was reminiscent of the patterns associated with normal non-rapid-eye-movement sleep, suggesting the possible presence of incomplete sleep-wake transitions. These abnormalities were strikingly distinct from those induced by acute sleep deprivation, suggesting that the patterns seen here might reflect a trait associated with idiopathic hypersomnia rather than a non-specific state of sleepiness. © Sleep Research Society 2017. Published by Oxford University Press on behalf of the Sleep

An epidemiologic study of environmental and genetic factors in congenital hydrocephalus.

PubMed

Stoll, C; Alembik, Y; Dott, B; Roth, M P

1992-11-01

Risk factors were studied in 96 children with congenital hydrocephalus (CH) coming from 118,265 consecutive births of known outcome. Hydrocephalus with neural tube defects, intracranial tumors or secondary to brain atrophy were excluded. The prevalence of CH was 0.81 per thousand. Diagnosis was performed prenatally in 41 cases. Forty-three (44.8%) of the cases had hydrocephalus without other malformations (isolated hydrocephalus), 18 (18.7%) infants had recognized chromosomal or non-chromosomal syndromes and 35 children (36.4%) had multiple malformations. Each case was matched to a control. Weight and length at birth of children with hydrocephalus were less than in the controls (p < 0.001). The weight of the placenta was lower than in the controls (p < 0.05). The pregnancy with a hydrocephalic child was more often complicated by threatened abortion, polyhydramnios and oligohydramnios. The mothers of children with hydrocephalus and multiple malformations had used oral contraceptives during the first trimester of pregnancy more often than the mothers of the controls. No differences appeared between the mothers of children with CH and the controls for the other risk factors studied: parental age, parity, previous pregnancies, previous stillbirths, smoking, diabetes, epilepsy, X-rays, hypertension, fever "flu", medication and occupational exposure. There was an increase of parental consanguinity in the parents of our patients (6.2% v. 1.1%, p < 0.001) and first degree relatives had more non-cerebral malformations than the controls (7.3% v. 3.2%, p < 0.05).

Spontaneous third ventriculostomy 8 years after diagnosis of obstructive hydrocephalus.

PubMed

Öğrenci, Ahmet; Ekşi, Murat Şakir; Koban, Orkun

2016-09-01

Spontaneous ventriculostomy is spontaneous rupture of membranes separating the ventricular system from the subarachnoid space in patients with chronic obstructive hydrocephalus that ends with resolution of symptoms. We present a case of spontaneous third ventriculostomy occurred in a 19-year-old girl 8 years after the initial diagnosis of hydrocephalus. An 11-year-old girl applied to the clinic with intermittent headaches. She was neurologically stable with no visual problems. On her brain MRI, obstructive hydrocephalus was observed. Cerebrospinal fluid diversion procedures were recommended, yet the family denied any interventional procedure. She had routine follow-ups with occasional clinical admissions because of ongoing intermittent headaches. On her last clinical visit, 8 years after the first one, she was in well condition with improvement in her headache in the last 4 months. Her new brain MRI showed an active CSF flow between the basal cistern and the third ventricle. In patients with aqueductal stenosis and without any other mass lesion, wait and see protocol might be conveyed in case of mild symptoms of hydrocephalus. However, there is need for large-scaled studies to make a more comprehensive statement for benign obstructive hydrocephalus cases.

Post-hemorrhagic hydrocephalus: Recent advances and new therapeutic insights.

PubMed

Chen, Qianwei; Feng, Zhou; Tan, Qiang; Guo, Jing; Tang, Jun; Tan, Liang; Feng, Hua; Chen, Zhi

2017-04-15

Post-hemorrhagic hydrocephalus (PHH), also referred to as progressive ventricular dilatation, is caused by disturbances in cerebrospinal fluid (CSF) flow or absorption following hemorrhage in the brain. As one of the most serious complications of neonatal/adult intraventricular hemorrhage (IVH), subarachnoid hemorrhage (SAH), and traumatic brain injury (TBI), PHH is associated with increased morbidity and disability of these events. Common sequelae of PHH include neurocognitive impairment, motor dysfunction, and growth impairment. Non-surgical measures to reduce increased intracranial pressure (ICP) in PHH have shown little success and most patients will ultimately require surgical management, such as external ventricular drainage and shunting which mostly by inserting a CSF drainage shunt. Unfortunately, shunt complications are common and the optimum time for intervention is unclear. To date, there remains no comprehensive strategy for PHH management and it becomes imperative that to explore new therapeutic targets and methods for PHH. Over past decades, increasing evidence have indicated that hemorrhage-derived blood and subsequent metabolic products may play a key role in the development of IVH-, SAH- and TBI-associated PHH. Several intervention strategies have recently been evaluated and cross-referenced. In this review, we summarized and discussed the common aspects of hydrocephalus following IVH, SAH and TBI, relevant experimental animal models, clinical translation of in vivo experiments, and potential preventive and therapeutic targets for PHH. Copyright © 2017 Elsevier B.V. All rights reserved.

Prolonged hydrocephalus induced by intraventricular hemorrhage in rats is reduced by curcumin therapy.

PubMed

Qi, Zhihua; Zhang, Huiqin; Fu, Chuhua; Liu, Xiao; Chen, Bo; Dang, Yanwei; Chen, Huayun; Liu, Lijun

2017-01-10

Prolonged hydrocephalus is a major cause of severe disability and death of intraventricular hemorrhage (IVH) patients. However, the therapeutic options to minimize the detrimental effects of post-hemorrhagic hydrocephalus are limited. Curcumin has been reported to confer neuroprotective effects in numerous neurological diseases and injuries, but its role in IVH-induced hydrocephalus has not been determined. The aim of present study was to determine whether curcumin treatment ameliorates blood brain barrier (BBB) damage and reduces the incidence of post-hemorrhagic hydrocephalus in IVH rat model. Autologous blood intraventricular injection was used to establish the IVH model. Our results revealed that repeated intraperitoneal injection of curcumin ameliorated IVH-induced learning and memory deficits as determined by Morris water maze and reduced the incidence of post-hemorrhagic hydrocephalus in a dose-dependent manner at 28 d post-IVH induction. Further, the increased BBB permeability and brain edema induced by IVH were significantly reduced by curcumin administration. In summary, these findings highlighted the important role of curcumin in improving neurological function deficits and protecting against BBB disruption via promoting the neurovascular unit restoration, and thus it reduced the severity of post-hemorrhagic hydrocephalus in the long term. It is believed that curcumin might prove to be an effective therapeutic component in prevent the post-IVH hydrocephalus in the near future. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

Exercise Blood Pressure Guidelines: Time to Re-evaluate What is Normal and Exaggerated?

PubMed

Currie, Katharine D; Floras, John S; La Gerche, Andre; Goodman, Jack M

2018-03-24

Blood pressure responses to graded exercise testing can provide important diagnostic and prognostic information. While published guidelines outline what constitutes a "normal" and "abnormal" (i.e., exaggerated) blood pressure response to exercise testing, the widespread use of exaggerated blood pressure responses as a clinical tool is limited due to sparse and inconsistent data. A review of the original sources from these guidelines reveals an overall lack of empirical evidence to support both the normal blood pressure responses and their upper limits. In this current opinion, we critically evaluate the current exercise blood pressure guidelines including (1) the normal blood pressure responses to graded exercise testing; (2) the upper limits of this normal response; (3) the blood pressure criteria for test termination; and (4) the thresholds for exaggerated blood pressure responses. We provide evidence that exercise blood pressure responses vary according to subject characteristics, and subsequently a re-evaluation of what constitutes normal and abnormal responses is necessary to strengthen the clinical utility of this assessment.

Cerebral haemodynamics in patients with hydrocephalus after subarachnoid haemorrhage due to ruptured aneurysm.

PubMed

Chang, Chia-Cheng; Kuwana, Nobumasa; Ito, Susumu; Yokoyama, Takaakira; Kanno, Hiroshi; Yamamoto, Isao

2003-01-01

Cerebral blood flow (CBF) and cerebrovascular reactivity (CVR) may be reduced in patients with normal pressure hydrocephalus (NPH) after subarachnoid haemorrhage (SAH). However, little is known about brain circulation in asymptomatic patients with ventriculomegaly after SAH. This study investigated CBF and CVR in symptomatic and asymptomatic patients with ventriculomegaly to clarify the mechanism of NPH. CBF and CVR were investigated in 48 patients with ventriculomegaly after SAH due to ruptured aneurysm. Mean CBF of the whole brain was measured by first-pass radionuclide angiography using technetium-99m hexamethylpropylene amine oxime. CVR was measured as the percentage change from the baseline mean CBF value after administration of 500 mg acetazolamide. Thirty patients with NPH who responded to shunting had significantly ( P<0.01) reduced mean CBF and CVR compared with normal controls. Fourteen asymptomatic patients with ventriculomegaly showed significant ( P<0.01) reduction in CVR but no difference in mean CBF. Four symptomatic patients who did not respond to shunting showed significantly ( P<0.01) reduced mean CBF but had preserved CVR. Postoperative mean CBF and CVR increased significantly ( P<0.01) in 21 patients who responded to shunting, but showed no significant change in four symptomatic patients who did not respond to shunting. Reduction of CBF superimposed on pre-existing impairment of CVR may be an essential step in the mechanism responsible for the manifestation of symptoms of NPH.

Acute hydrocephalus caused by intraspinal neurocysticercosis: case report

PubMed Central

2014-01-01

Background Intraspinal neurocysticercosis is an uncommon manifestation that may present as an isolated lesion. Furthermore, acute hydrocephalus caused by isolated intraspinal neurocysticercosis without concomitant cerebral involvement is extremely rare. Case presentation A 64-year-old man presented with a history of severe headache, an unsteady gait, and occasional urinary incontinence. Magnetic resonance imaging of the thoraco-lumbar spine revealed multiple, cystic, contrast-enhancing intraspinal lesions. A computed tomographic scan of the brain showed marked ventricular dilatation but no intraparenchymal lesions or intraventricular cysticercal lesions. This case of acute hydrocephalus was found to be caused by isolated intraspinal neurocysticercosis and was treated by ventriculoperitoneal shunt placement and surgical removal of the intraspinal lesions (which were histologically confirmed as neurocysticercosis), followed by administration of dexamethasone and albendazole. Conclusion Isolated spinal neurocysticercosis should be considered in the differential diagnosis of acute hydrocephalus when no explanation is found in the brain, particularly in geographical regions endemic for cysticercosis. PMID:24383427

Hydrocephalus and mucopolysaccharidoses: what do we know and what do we not know?

PubMed

Dalla Corte, Amauri; de Souza, Carolina F M; Anés, Maurício; Giugliani, Roberto

2017-07-01

The precise incidence of hydrocephalus in patients with mucopolysaccharidoses (MPS) is hard to determine, because the condition lacks a formal, consensus-based definition. The diagnosis of hydrocephalus depends on symptom profile, presence of neuroimaging features, and the outcome of diagnostic tests. Although numerous techniques are used to identify MPS patients who are most likely to have hydrocephalus and respond to treatment, no definitive method exists to prove diagnosis. The authors propose an algorithm to aid in the diagnosis and management of hydrocephalus in MPS patients. The theory of venous hypertension associated with the morphological changes in the skull base and craniocervical junction indicate the need for future neuroimaging studies including cerebrospinal fluid (CSF) and venous flow measurements to monitor hydrocephalus progression and select therapeutic interventions in MPS patients. Preoperative planning should also be based on the increased risk of intraoperative and postoperative hemorrhagic complications.

Intra-ocular pressure normalization technique and equipment

NASA Technical Reports Server (NTRS)

Mcgannon, W. J. (Inventor)

1980-01-01

A method and apparatus for safely reducing abnormally high intraocular pressure in an eye during a predetermined time interval is presented. This allows maintenance of normal intraocular pressure during glaucoma surgery. According to the invention, a pressure regulator of the spring biased diaphragm type is provided with additional bias by a column of liquid. The height of the column of liquid is selected such that the pressure at a hypodermic needle connected to the output of the pressure regulator is equal to the measured pressure of the eye. The hypodermic needle can then be safely inserted into the anterior chamber of the eye. Liquid is then bled out of the column to reduce the bias on the diaphragm of the pressure regulator and, consequently, the output pressure of the regulator. This lowering pressure of the regulator also occurs in the eye by means of a small second bleed path provided between the pressure regulator and the hypodermic needle. Alternately, a second hypodermic needle may be inserted into the eye to provide a controlled leak off path for excessive pressure and clouded fluid from the anterior chamber.

Hydrocephalus in a yellow-headed Amazon parrot (Amazona ochrocephala oratrix).

PubMed

Keller, Krista A; Guzman, David Sanchez-Migallon; Muthuswamy, Anantharaman; Forrest, Lisa J; Steinberg, Howard; Sladky, Kurt; Petersen, Sophie

2011-09-01

A 37-year-old female yellow-headed Amazon parrot (Amazona ochrocephala oratrix) was presented after a 4-month-period behavior change and intermittent episodes of obtunded mentation. Clinical findings on physical examination included ataxia, a weak grasp, and reluctance to move. Results of magnetic resonance imaging were consistent with severe hydrocephalus without evidence of cerebrospinal fluid obstruction. The bird was treated with tapering dosages of prednisolone over a 4-month period, during which time the episodes did not occur. Discontinuation of treatment was attempted several times but resulted in relapse. After 3.5 years of maintenance treatment with prednisolone, the bird was presented subsequent to a 5-hour episode of obtunded mentation and worsening neurologic signs. Despite increasing the dose of prednisolone and providing additional supportive care, the bird's condition worsened, and euthanasia was elected. Necropsy findings included severe hydrocephalus with significant loss of right cerebral parenchyma and no evidence of cerebrospinal fluid obstruction. Histologic examination of the remaining cerebral parenchyma revealed a moderate, multifocal, cellular infiltrate; encephalomalacia; fibrosis; and hemosiderosis in tissue adjacent to the distended ventricles. Other findings included hepatic vacuolar degeneration. Diagnostic imaging and postmortem findings were consistent with a diagnosis of hydrocephalus ex vacuo. To our knowledge, this is the first report of hydrocephalus in an Amazon parrot as well as the first report of hydrocephalus in any avian species associated with long-term follow-up and prolonged corticosteroid treatment.

Stereotactic CO2 laser therapy for hydrocephalus

NASA Astrophysics Data System (ADS)

Kozodoy-Pins, Rebecca L.; Harrington, James A.; Zazanis, George A.; Nosko, Michael G.; Lehman, Richard M.

1994-05-01

A new fiber-optic delivery system for CO2 radiation has been used to successfully treat non-communicating hydrocephalus. This system consists of a hollow sapphire waveguide employed in the lumen of a stereotactically-guided neuroendoscope. CO2 gas flows through the bore of the hollow waveguide, creating a path for the laser beam through the cerebrospinal fluid (CSF). This delivery system has the advantages of both visualization and guided CO2 laser radiation without the same 4.3 mm diameter scope. Several patients with hydrocephalus were treated with this new system. The laser was used to create a passage in the floor of the ventricle to allow the flow of CSF from the ventricles to the sub-arachnoid space. Initial postoperative results demonstrated a relief of the clinical symptoms. Long-term results will indicate if this type of therapy will be superior to the use of implanted silicone shunts. Since CO2 laser radiation at 10.6 micrometers is strongly absorbed by the water in tissue and CSF, damage to tissue surrounding the lesion with each laser pulse is limited. The accuracy and safety of this technique may prove it to be an advantageous therapy for obstructive hydrocephalus.

Idiopathic ophthalmodynia and idiopathic rhinalgia: two topographic facial pain syndromes.

PubMed

Pareja, Juan A; Cuadrado, María L; Porta-Etessam, Jesús; Fernández-de-las-Peñas, César; Gili, Pablo; Caminero, Ana B; Cebrián, José L

2010-09-01

To describe 2 topographic facial pain conditions with the pain clearly localized in the eye (idiopathic ophthalmodynia) or in the nose (idiopathic rhinalgia), and to propose their distinction from persistent idiopathic facial pain. Persistent idiopathic facial pain, burning mouth syndrome, atypical odontalgia, and facial arthromyalgia are idiopathic facial pain syndromes that have been separated according to topographical criteria. Still, some other facial pain syndromes might have been veiled under the broad term of persistent idiopathic facial pain. Through a 10-year period we have studied all patients referred to our neurological clinic because of facial pain of unknown etiology that might deviate from all well-characterized facial pain syndromes. In a group of patients we have identified 2 consistent clinical pictures with pain precisely located either in the eye (n=11) or in the nose (n=7). Clinical features resembled those of other localized idiopathic facial syndromes, the key differences relying on the topographic distribution of the pain. Both idiopathic ophthalmodynia and idiopathic rhinalgia seem specific pain syndromes with a distinctive location, and may deserve a nosologic status just as other focal pain syndromes of the face. Whether all such focal syndromes are topographic variants of persistent idiopathic facial pain or independent disorders remains a controversial issue.

Pediatric Hydrocephalus in Ethiopia: Treatment Failures and Infections: A Hospital-Based, Retrospective Study.

PubMed

Laeke, Tsegazeab; Tirsit, Abenezer; Biluts, Hagos; Murali, Deeptha; Wester, Knut

2017-04-01

Treatment of pediatric hydrocephalus in sub-Saharan countries is associated with significant complication rates. The objective of this study is to analyze the management of hydrocephalus and complication rates of surgical intervention in the Ethiopian setting to improve future quality. A retrospective cohort study was conducted in a neurosurgical teaching hospital. Two cohorts separated by 2.5 years were analyzed. A total of 128 (58.6% male and 41.4% female) children with isolated hydrocephalus, meningomyelocele (MMC)-related hydrocephalus, or MMC without hydrocephalus were included. Their age ranged from 1 day to 5 years, for a mean age of 7.2 months (median age 2 months). One hundred thirteen patients had hydrocephalus, of whom 57 (44.5.3%) had isolated hydrocephalus and 56 (43.8%) had hydrocephalus associated with MMC. Seventy-seven (74.7%) patients underwent ventriculoperitoneal (VP) shunting, whereas 24 (23.3%) underwent endoscopic third ventriculostomy (ETV). The incidence of shunt infection was 23.4%. Reoperation was needed in 54 (52.4%) patients, with the most common indication being shunt failure. ETV failed in 14 (58.3%) of the 24 patients undergoing ETV. VP shunt insertions had unacceptably high infection rate despite the presence of a protocol for the procedure. Intraoperative guidelines should be developed further and followed strictly to reduce infections. Such measures should include restricting the number of surgeons performing the procedure. In our opinion, one should avoid insertion of a VP shunt as the primary treatment. ETV has proved to be a good alternative in other studies and the decreasing pattern of ETV failure in our study also suggest ETV as a better alternative to VP shunt. Copyright © 2016 The Author(s). Published by Elsevier Inc. All rights reserved.

The Risk Factors for Hydrocephalus and Subdural Hygroma after Decompressive Craniectomy in Head Injured Patients.

PubMed

Ki, Hee Jong; Lee, Hyung-Jin; Lee, Hong-Jae; Yi, Jin-Seok; Yang, Ji-Ho; Lee, Il-Woo

2015-09-01

The present study aims to investigate 1) the risk factors for hydrocephalus and subdural hygroma (SDG) occurring after decompressive craniectomy (DC), and 2) the association between the type of SDG and hydrocephalus. We retrospectively reviewed the clinical and radiological features of 92 patients who underwent DC procedures after severe head injuries. The risk factors for developing post-traumatic hydrocephalus (PTH) and SDG were analyzed. Types of SDGs were classified according to location and their relationship with hydrocephalus was investigated. Ultimately, 26.09% (24/92) of these patients developed PTH. In the univariate analyses, hydrocephalus was statically associated with large bone flap diameter, large craniectomy area, bilateral craniectomy, intraventricular hemorrhage, contralateral or interhemisheric SDGs, and delayed cranioplasty. However, in the multivariate analysis, only large craniectomy area (adjusted OR=4.66; p=0.0239) and contralateral SDG (adjusted OR=6.62; p=0.0105) were significant independent risk factors for developing hydrocephalus after DC. The incidence of overall SDGs after DC was 55.43% (51/92). Subgroup analysis results were separated by SDG types. Statistically significant associations between hydrocephalus were found in multivariate analysis in the contralateral (adjusted OR=5.58; p=0.0074) and interhemispheric (adjusted OR=17.63; p=0.0113) types. For patients who are subjected to DC following severe head trauma, hydrocephalus is associated with a large craniectomy area and contralateral SDG. For SDGs after DC that occur on the interhemispherical or controlateral side of the craniectomy, careful follow-up monitoring for the potential progression into hydrocephalus is needed.

Compensatory parameters of intracranial space in giant hydrocephalus.

PubMed

Cieślicki, Krzysztof; Czepko, Ryszard

2009-01-01

The main goal of the present study is to examine compensatory parameters of intracranial space in giant hydrocephalus. We also assess the early and late outcome and analyse complications in shunted cases. Nine cases of giant hydrocephalus characterised by the value of Evans ratio > 0.5, ventricular index > 1.5, and the width of the third ventricle > 20 mm were considered. Using the lumbar infusion test and developed software we analysed the intracranial compensatory parameters typical for hydrocephalus. Based on the Marmarou model, the method depended on a repeated search for the best fitting curve corresponding to the progress of the test was used. Eight out of nine patients were therefore shunted. Patients were followed up for 9 months. Five out of eight shunted patients undoubtedly improved in a few days after surgery (62%). Complications (subdural hygromas/haematomas and intracerebral haematoma) developed in 5 (62%) cases in longer follow-up. A definite improvement was noted in 4 out of 8 operated cases (50%). To get the stable values of compensatory parameters, the duration of the infusion test must at least double the inflexion time of the test curve. All but one considered cases of giant hydrocephalus were characterized by lack of intracranial space reserve, significantly reduced rate of CSF secretion and by various degrees of elevated value of the resistance to outflow. Due to the significant number of complications and uncertain long-term improvement, great caution in decision making for shunting has to be taken.

Morphological and Behavioral Changes in the Pathogenesis of a Novel Mouse Model of Communicating Hydrocephalus

PubMed Central

McMullen, Allison B.; Baidwan, Gurlal S.; McCarthy, Ken D.

2012-01-01

The Ro1 model of hydrocephalus represents an excellent model for studying the pathogenesis of hydrocephalus due to its complete penetrance and inducibility, enabling the investigation of the earliest cellular and histological changes in hydrocephalus prior to overt pathology. Hematoxylin and eosin staining, immunofluorescence and electron microscopy were used to characterize the histopathological events of hydrocephalus in this model. Additionally, a broad battery of behavioral tests was used to investigate behavioral changes in the Ro1 model of hydrocephalus. The earliest histological changes observed in this model were ventriculomegaly and disorganization of the ependymal lining of the aqueduct of Sylvius, which occurred concomitantly. Ventriculomegaly led to thinning of the ependyma, which was associated with periventricular edema and areas of the ventricular wall void of cilia and microvilli. Ependymal denudation was subsequent to severe ventriculomegaly, suggesting that it is an effect, rather than a cause, of hydrocephalus in the Ro1 model. Additionally, there was no closure of the aqueduct of Sylvius or any blockages within the ventricular system, even with severe ventriculomegaly, suggesting that the Ro1 model represents a model of communicating hydrocephalus. Interestingly, even with severe ventriculomegaly, there were no behavioral changes, suggesting that the brain is able to compensate for the structural changes that occur in the pathogenesis of hydrocephalus if the disorder progresses at a sufficiently slow rate. PMID:22291910

Post-hemorrhagic hydrocephalus and diabetes insipidus in preterm infants.

PubMed

Borenstein-Levin, Liron; Koren, Ilana; Kugelman, Amir; Bader, David; Toropine, Arina; Riskin, Arieh

2014-11-01

We present two cases of transient central diabetes insipidus in preterm neonates with post-hemorrhagic hydrocephalus. Although the association between intraventricular hemorrhage and diabetes insipidus has been described in preterm infants, the association between diabetes insipidus and hydrocephalus, and the fact that such central diabetes insipidus could be reversible with the reduction of ventricular size, either because of spontaneous resolution or the placement of ventriculo-peritoneal shunt is first described here in neonates.

Idiopathic toe walking.

PubMed

Oetgen, Matthew E; Peden, Sean

2012-05-01

Toe walking is a bilateral gait abnormality in which a normal heel strike is absent and most weight bearing occurs through the forefoot. This abnormality may not be pathologic in patients aged <2 years, but it is a common reason for referral to an orthopaedic surgeon. Toe walking can be caused by several neurologic and developmental abnormalities and may be the first sign of a global developmental problem. Cases that lack a definitive etiology are categorized as idiopathic. A detailed history, with careful documentation of the developmental history, and a thorough physical examination are required in the child with a primary report of toe walking. Treatment is based on age and the severity of the abnormality. Management includes observation, stretching, casting, bracing, chemodenervation, and surgical lengthening of the gastrocnemius-soleus complex and/or Achilles tendon. An understanding of idiopathic toe walking as well as treatment options and their outcomes can help the physician individualize treatment to achieve optimal results.

FSI simulation of CSF hydrodynamic changes in a large population of non-communicating hydrocephalus patients during treatment process with regard to their clinical symptoms

PubMed Central

2018-01-01

3D fluid-structure interaction modelling was utilized for simulation of 13 normal subjects, 11 non-communicating hydrocephalus (NCH) patients at pre-treatment phase, and 3 patients at five post-treatment phases. Evaluation of ventricles volume and maximum CSF pressure (before shunting) following results validation indicated that these parameters were the most proper hydrodynamic indices and the NCH type doesn’t have any significant effect on changes in two indices. The results confirmed an appropriate correlation between these indices although the correlation decreased slightly after the occurrence of disease. NCH raises the intensity of vortex and pulsatility (2.4 times) of CSF flow while the flow remains laminar. On day 18 after shunting, the CSF pressure decreased 81.0% and all clinical symptoms of patients vanished except for headache. Continuing this investigation during the treatment process showed that maximum CSF pressure is the most sensitive parameter to patients’ clinical symptoms. Maximum CSF pressure has decreased proportional to the level of decrease in clinical symptoms and has returned close to the pressure range in normal subjects faster than other parameters and simultaneous with disappearance of patients’ clinical symptoms (from day 81 after shunting). However, phase lag between flow rate and pressure gradient functions and the degree of CSF pulsatility haven’t returned to normal subjects’ conditions even 981 days after shunting and NCH has also caused a permanent volume change (of 20.1%) in ventricles. Therefore, patients have experienced a new healthy state in new hydrodynamic conditions after shunting and healing. Increase in patients’ intracranial compliance was predicted with a more accurate non-invasive method than previous experimental methods up to more than 981 days after shunting. The changes in hydrodynamic parameters along with clinical reports of patients can help to gain more insight into the pathophysiology of NCH

Continuous Minor Bleeding from Tumor Surface in Patients with Craniopharyngiomas: Case Series of Nonobstructive Hydrocephalus.

PubMed

Shoji, Takuhiro; Kawaguchi, Tomohiro; Ogawa, Yoshikazu; Watanabe, Mika; Fujimura, Miki; Tominaga, Teiji

2018-06-05

Nonobstructive hydrocephalus in patients with craniopharyngiomas is uncommon. We describe our surgical series of 25 consecutive patients with craniopharyngioma who presented with hydrocephalus. Obstructive hydrocephalus was evident in most cases, and nonobstructive hydrocephalus was revealed in three cases. Even after improvement of cerebrospinal fluid (CSF) pathway obstruction by tumor removal, 10 patients (40%) required CSF diversion. Preoperative imaging study revealed thin intraventricular hemorrhage or superficial siderosis in five cases, and CSF examination revealed hemosiderin-laden phagocytes in one case. These findings indicate continuous bleeding into the CSF that might be associated with CSF malabsorption. We also describe a representative case of craniopharyngioma associated with nonobstructive hydrocephalus due to continuous minor bleeding from the tumor surface in a 62-year-old man with a complaint of disorientation and a decline in daily living activity.Our study demonstrated that minor bleeding into the CSF is a possible mechanism of the development of nonobstructive hydrocephalus in patients with craniopharyngiomas. Georg Thieme Verlag KG Stuttgart · New York.

Ventriculosubgaleal shunts for posthemorrhagic hydrocephalus in premature infants.

PubMed

Willis, Brian K; Kumar, Cherukuri Ravi; Wylen, Esther L; Nanda, Anil

2005-01-01

The early management of posthemorrhagic hydrocephalus in premature infants is challenging and controversial. These infants need a temporary cerebrospinal fluid (CSF) diversion procedure until they gain adequate weight, and the blood and protein levels in CSF are reasonably low before permanent shunt can be placed. Various options are available with their associated advantages and disadvantages. Ventriculosubgaleal shunts have been recommended as a more physiologic and less invasive means of achieving this goal. We have performed this procedure in 6 premature infants to evaluate their effectiveness and complications. Six consecutive premature infants with posthemorrhagic hydrocephalus underwent placement of ventriculosubgaleal shunts over a 1-year period of time. We reviewed their clinical and imaging progress to assess the ability of the shunt to control hydrocephalus and the complication rates. In all 6 patients, the ventriculosubgaleal shunt controlled the progression of hydrocephalus as assessed by clinical and imaging parameters. A permanent shunt was avoided in 1 patient (16.6%). However, 4 patients developed shunt infections, 1 involving the ventriculosubgaleal shunt itself, and 3 immediately after conversion to ventriculoperitoneal shunt. The total infection rate of the series was 66.6%. All infections were caused by staphylococcus species. There was only a 1% shunt infection rate in our institution for all nonventriculosubgaleal shunts during the same period of time. Placement of ventriculosubgaleal shunts for interim CSF diversion in neonates with posthemorrhagic hydrocephalus is effective as a temporary method of CSF diversion. However, our experience has shown that it is associated with a unacceptably high CSF infection rate. A potential cause for infection is CSF stasis just beneath the extremely thin skin of the premature infants, promoting colonization by skin flora. CSF sampling before conversion to a permanent shunt and replacement of the proximal

Computational modeling of venous sinus stenosis in idiopathic intracranial hypertension

PubMed Central

Levitt, Michael R; McGah, Patrick M; Moon, Karam; Albuquerque, Felipe C; McDougall, Cameron G; Kalani, M Yashar S; Kim, Louis J; Aliseda, Alberto

2016-01-01

Background and Purpose Idiopathic intracranial hypertension has been associated with dural venous sinus stenosis in some patients, but the hemodynamic environment of the dural venous sinuses has not been quantitatively described. Here, we present the first such computational fluid dynamics model using patient-specific blood pressure measurements. Materials and Methods Six patients with idiopathic intracranial hypertension and at least one stenosis or atresia at the transverse-sigmoid sinus junction underwent MRV followed by cerebral venography and manometry throughout the dural venous sinuses. Patient-specific computational fluid dynamics models were created using MRV anatomy, with venous pressure measurements as boundary conditions. Blood flow and wall shear stress were calculated for each patient. Results Computational models of dural venous sinuses were successfully reconstructed in all six patients with patient-specific boundary conditions. Three patients demonstrated a pathologic pressure gradient (≥ 8 mm Hg) across four dural venous sinus stenoses. Small sample size precludes statistical comparisons, but average overall flow throughout the dural venous sinuses of patients with pathologic pressure gradients was higher than in those without (1041.00 ± 506.52 vs. 358.00 ± 190.95 mL/min). Wall shear stress was also higher across stenoses in patients with pathologic pressure gradients (37.66 ± 48.39 vs 7.02 ± 13.60 Pa). Conclusion The hemodynamic environment of the dural venous sinuses can be computationally modeled using patient-specific anatomy and physiological measurements in patients with idiopathic intracranial hypertension. There was substantially higher blood flow and wall shear stress in patients with pathological pressure gradients. PMID:27197986

Hydrocephalus research funding from the National Institutes of Health: a 10-year perspective.

PubMed

Gross, Paul; Reed, Gavin T; Engelmann, Rachel; Kestle, John R W

2014-02-01

Funding of hydrocephalus research is important to the advancement of the field. The goal of this paper is to describe the funding of hydrocephalus research from the National Institutes of Health (NIH) over a recent 10-year period. The NIH online database RePORT (Research Portfolio Online Reporting Tools) was searched using the key word "hydrocephalus." Studies were sorted by relevance to hydrocephalus. The authors analyzed funding by institute, grant type, and scientific approach over time. Over $54 million was awarded to 59 grantees for 66 unique hydrocephalus proposals from 48 institutions from 2002 to 2011. The largest sources of funding were the National Institute of Neurological Disease and Stroke and the National Institute of Child Health and Human Development. Of the total, $22 million went to clinical trials, $15 million to basic science, and $10 million to joint ventures with small business (Small Business Innovation Research or Small Business Technology Transfer). Annual funding varied from $2.3 to $8.1 million and steadily increased in the second half of the observation period. The number of new grants also went from 15 in the first 5 years to 27 in the second 5 years. A large portion of the funding has been for clinical trials. Funding for shunt-device development grew substantially. Support for training of hydrocephalus investigators has been low. Hydrocephalus research funding is low compared with that for other conditions of similar health care burden. In addition to NIH applications, researchers should pursue other funding sources. Small business collaborations appear to present an opportunity for appropriate projects.

Periodic oscillation of intracranial pressure in ventricular dilation: a preliminary report.

PubMed

Kuchiwaki, H; Misu, N; Kageyama, N; Ishiguri, H; Takada, S

1987-12-01

Artificial pressure waves (PWs) were generated by manual inflation of a balloon in the trigonum of the lateral ventricle in seven adult mongrel dogs with normal cerebrospinal fluid (CSF) circulation. In 14 of 16 series of continuous appearances of artificial PWs, local shifts of the brain were successfully monitored using small strain-gauge sensors at the periventricular structures in these animals. Of the 14 series, 13 showed displacements of the periventricular structures, suggesting ventricular dilation. These results did not always correlate with macroscopic findings. They are thought to be due largely to periventricular oedemas and, in part, non-uniform dilations of the ventricles during PWs. We conclude that a water hammer formed by reflection of an increased pulse pressure of PWs at the site of CSF absorption causes a shift of CSF from the ventricle to the periventricular structures through the wall of the ventricle. This phenomenon appears amplified in patients with impaired CSF absorption. Thus, PWs have a pathological role in the progress of ventricular dilation in patients with normal pressure hydrocephalus.

Impact of timing of cranioplasty on hydrocephalus after decompressive hemicraniectomy in malignant middle cerebral artery infarction.

PubMed

Finger, Tobias; Prinz, Vincent; Schreck, Evelyn; Pinczolits, Alexandra; Bayerl, Simon; Liman, Thomas; Woitzik, Johannes; Vajkoczy, Peter

2017-02-01

Patients with malignant middle cerebral artery infarction frequently develop hydrocephalus after decompressive hemicraniectomy. Hydrocephalus itself and known shunt related complications after ventriculo-peritoneal shunt implantation may negatively impact patientś outcome. Here, we aimed to identify factors associated with the development of hydrocephalus after decompressive hemicraniectomy in malignant middle cerebral artery infarction. A total of 99 consecutive patients with the diagnosis of large hemispheric infarctions and the indication for decompressive hemicraniectomy were included. We retrospectively evaluated patient characteristics (gender, age and selected preoperative risk factors), stroke characteristics (side, stroke volume and existing mass effect) and surgical characteristics (size of the bone flap, initial complication rate, time to cranioplasty, complication rate following cranioplasty, type of implant, number of revision surgeries and mortality). Frequency of hydrocephalus development was 10% in our cohort. Patients who developed a hydrocephalus had an earlier time point of bone flap reimplantation compared to the control group (no hydrocephalus=164±104days, hydrocephalus=108±52days, p<0.05). Additionally, numbers of revision surgeries after cranioplasty was associated with hydrocephalus with a trend towards significance (p=0.08). Communicating hydrocephalus is frequent in patients with malignant middle cerebral artery infarction after decompressive hemicraniectomy. A later time point of cranioplasty might lead to a lower incidence of required shunting procedures in general as we could show in our patient cohort. Copyright © 2016 Elsevier B.V. All rights reserved.

The influence of thermoplastic thoraco lumbo sacral orthoses on standing balance in subjects with idiopathic scoliosis.

PubMed

Khanal, Minoo; Arazpour, Mokhtar; Bahramizadeh, Mahmood; Samadian, Mohammad; Hutchins, Stephen W; Kashani, Reza Vahab; Mardani, Mohammad A; Tari, Hossein Vahid; Aboutorabi, Atefeh; Curran, Sarah; Sadeghi, Heidar

2016-08-01

Idiopathic scoliosis patients have postural equilibrium problems. The objective of this study was to assess postural control in subjects with idiopathic scoliosis following a 4-month intervention in an unbraced position. Quasi-experimental. Eight healthy girls and eight girls with idiopathic scoliosis took part. A Kistler force platform was used with a frequency of 100 Hz for recording data. The center of pressure was recorded in different positions out of brace for scoliosis and healthy subjects. Test conditions were single limb and double limb stance, with eyes open and closed, and foam and rigid surfaces. The data reflected a weak balance of idiopathic scoliosis subjects compared to healthy subjects. After 1 and 4 months of wearing the brace, center of pressure and center of gravity sway increased in the majority of the tests, although there were no significant differences in any of the test conditions (p > 0.05). While the center of pressure sway in medio-lateral direction decreased after 4 months of wearing a brace, in other variables center of pressure and center of gravity sway increased. Idiopathic scoliosis patients have weak balance in comparison to healthy subjects. In addition, following a period of 4 months of wearing a brace, balance parameters in the scoliosis subjects did not improve. The results show that we need more follow-up of orthoses wearing in idiopathic scoliosis subjects and suggest more studies at least 1-year follow-up to identify the efficiency of brace wear on balance. Scoliosis can alter postural stability and balance performance during quiet standing. Spinal deformity can alter a subject's ability to compensate for postural changes and cause gait deviations. This study investigated balance differences between the healthy and idiopathic scoliosis patients and the results of thoraco lumbo sacral orthosis brace wear. It might provide some new insight into the conservative treatment of idiopathic scoliosis patients for

Plasma lipid profile in Nigerians with high--normal blood pressure.

PubMed

Saidu, Hadiza; Karaye, Kamilu Musa; Okeahialam, Basil N

2014-12-18

High blood pressure levels have been associated with elevated atherogenic blood lipid fraction, but epidemiological surveys often give inconsistent results across population sub-groups. To determine the extent to which there are differences in lipid profile based on blood pressure levels, we assessed lipid profile of subjects with high-normal blood pressure and compared with those of hypertensives and optimally normal blood pressure. The study was a cross-sectional comparative study conducted at Aminu Kano Teaching Hospital, Kano, Nigeria. Fasting lipid levels were examined among randomly selected patients with optimally normal blood pressure (group 1), high-normal blood pressure (group 2) and those with hypertension (group 3). Optimal blood pressure was defined as systolic blood pressure (SBP) of <120 mmHg/or diastolic blood pressure (DBP) of <80 mmHg; and high-normal blood pressure as SBP of 130-139 mmHg and/or DBP of 85-89 mmHg. A total of 300 subjects were studied, 100 in each group. The mean age of subjects in group 1 was 27.32±8.20 years and 60% were female, while that of group 2 was 34.04±6.25 years, and 53% were female, and that for group 3 was 52.81±13.3 years and 56% were female. The mean total cholesterol (TC) for subjects in group1 (3.96±0.40 mmol/L) was significantly lower than levels in group2 (4.55±1.01 mmol/L); P=<0.001. Subjects in group 3 (5.20±1.88 mmol/L), however had statistically significant higher mean TC when compared with group 2; (P=0.03). The difference between the groups for low density lipoprotein cholesterol (LDL-C) and triglycerides (TG) followed the same pattern as that of TC, with statistically significant increasing trend across the blood pressure categories. Levels of high density lipoprotein cholesterol (HDL-C) were however similar across the three groups (group 2 versus group 1; P=0.49, group 2 versus group 3; P=0.9). Increased TC (>5.2 mmol/L) was absent in group1, but found among 11% of group2 subjects and 40% of

Effects of irregular cerebrospinal fluid production rate in human brain ventricular system

NASA Astrophysics Data System (ADS)

Hadzri, Edi Azali; Shamsudin, Amir Hamzah; Osman, Kahar; Abdul Kadir, Mohammed Rafiq; Aziz, Azian Abd

2012-06-01

Hydrocephalus is an abnormal accumulation of fluid in the ventricles and cavities in the brain. It occurs when the cerebrospinal fluid (CSF) flow or absorption is blocked or when excessive CSF is secreted. The excessive accumulation of CSF results in an abnormal widening of the ventricles. This widening creates potentially harmful pressure on the tissues of the brain. In this study, flow analysis of CSF was conducted on a three-dimensional model of the third ventricle and aqueduct of Sylvius, derived from MRI scans. CSF was modeled as Newtonian Fluid and its flow through the region of interest (ROI) was done using EFD. Lab software. Different steady flow rates through the Foramen of Monro, classified by normal and hydrocephalus cases, were modeled to investigate its effects. The results show that, for normal and hydrocephalus cases, the pressure drop of CSF flow across the third ventricle was observed to be linearly proportionally to the production rate increment. In conclusion, flow rates that cause pressure drop of 5 Pa was found to be the threshold for the initial sign of hydrocephalus.

At what age is hydrocephalus detected, and what is the role of head circumference measurements?

PubMed

Breuning-Broers, Jacqueline M; Deurloo, Jacqueline A; Gooskens, Rob H; Verkerk, Paul H

2014-02-01

To investigate at what age hydrocephalus is detected and to assess the role of head circumference measurements in detecting hydrocephalus, we performed a retrospective chart review in children with hydrocephalus treated in a tertiary paediatric hospital in the Netherlands. The study group contained 146 patients; 38 patients (31%) were referred because of abnormalities in head circumference. Eighty-nine per cent of the patients were detected in the first year of life. After this period, no patients were referred because of an abnormal head circumference. Therefore, head circumference measurements seem to have little value for detecting hydrocephalus after the first year of life.

Intracranial pressure monitoring in pediatric and adult patients with hydrocephalus and tentative shunt failure: a single-center experience over 10 years in 146 patients.

PubMed

Sæhle, Terje; Eide, Per Kristian

2015-05-01

OBJECT In patients with hydrocephalus and shunts, lasting symptoms such as headache and dizziness may be indicative of shunt failure, which may necessitate shunt revision. In cases of doubt, the authors monitor intracranial pressure (ICP) to determine the presence of over- or underdrainage of CSF to tailor management. In this study, the authors reviewed their experience of ICP monitoring in shunt failure. The aims of the study were to identify the complications and impact of ICP monitoring, as well as to determine the mean ICP and characteristics of the cardiac-induced ICP waves in pediatric versus adult over- and underdrainage. METHODS The study population included all pediatric and adult patients with hydrocephalus and shunts undergoing diagnostic ICP monitoring for tentative shunt failure during the 10-year period from 2002 to 2011. The patients were allocated into 3 groups depending on how they were managed following ICP monitoring: no drainage failure, overdrainage, or underdrainage. While patients with no drainage failure were managed conservatively without further actions, over- or underdrainage cases were managed with shunt revision or shunt valve adjustment. The ICP and ICP wave scores were determined from the continuous ICP waveforms. RESULTS The study population included 71 pediatric and 75 adult patients. There were no major complications related to ICP monitoring, but 1 patient was treated for a postoperative superficial wound infection and another experienced a minor bleed at the tip of the ICP sensor. Following ICP monitoring, shunt revision was performed in 74 (51%) of 146 patients, while valve adjustment was conducted in 17 (12%) and conservative measures without any actions in 55 (38%). Overdrainage was characterized by a higher percentage of episodes with negative mean ICP less than -5 to -10 mm Hg. The ICP wave scores, in particular the mean ICP wave amplitude (MWA), best differentiated underdrainage. Neither mean ICP nor MWA levels showed any

Clinical analysis of aqueductal stenosis in patients with hydrocephalus in a Kenyan setting.

PubMed

Kaur, Loyal Poonamjeet; Munyiri, Nderitu Joseph; Dismus, Wekesa Vincent

2017-01-01

Aqueductal stenosis is the commonest cause of congenital hydrocephalus. The scope of this paper is to highlight the disease burden of hydrocephalus attributed to aqueductal stenosis which still remains unknown in our setting. In a descriptive cross-sectional study, 258 records of patients diagnosed with hydrocephalus were analyzed after ethical approval from Kenyatta National Hospital- University of Nairobi (KNH-UON) ethics and research committee from January 2010 to May 2016. Patients with a diagnosis of hydrocephalus due to aqueductal stenosis were included in this study. Patients age, sex, mode of delivery, associated comorbidities, presenting complaints, neurosurgical intervention performed, Kafarnosky score were recorded. Data were divided into 2 sets based on the patient's age i.e. whether < 1 year or > 12 years. Data were recorded on google data collection form and analyzed using Google spreadsheets. Out of 258 cases of hydrocephalus, 52 had aqueductal stenosis. Male to female sex ratio for this condition was 3:2. There were 25 cases < 1year and 27 cases > 12 years old who were diagnosed with hydrocephalus due to aqueductal stenosis. Associated conditions were bilateral congenital talipes equinovarus, spina bifida, Arnold Chairi malformations, meningitis and HIV. The presenting complaints differed according to the age groups. Neurosurgical interventions included Endoscopic Third Ventriculostomy (ETV) in 21 cases, insertion of Ventriculoperitoneal (VP) shunt and ETV were done in 3 cases while the rest had only insertion of VP shunt. The Kafanosky score improve from < 50 pre-op to 19 cases achieving a score of 100, six months post-op. Aqueductal stenosis contributes a significant burden of morbidity in patients with hydrocephalus. Clinical presentation differs according to patients age. Accurate diagnosis and treatment remain a cardinal to improving patient outcome.

Diffusion tensor imaging study of pediatric patients with congenital hydrocephalus: 1-year postsurgical outcomes.

PubMed

Mangano, Francesco T; Altaye, Mekibib; McKinstry, Robert C; Shimony, Joshua S; Powell, Stephanie K; Phillips, Jannel M; Barnard, Holly; Limbrick, David D; Holland, Scott K; Jones, Blaise V; Dodd, Jonathan; Simpson, Sarah; Mercer, Deanna; Rajagopal, Akila; Bidwell, Sarah; Yuan, Weihong

2016-09-01

OBJECTIVE The purpose of this study was to investigate white matter (WM) structural abnormalities using diffusion tensor imaging (DTI) in children with hydrocephalus before CSF diversionary surgery (including ventriculoperitoneal shunt insertion and endoscopic third ventriculostomy) and during the course of recovery after surgery in association with neuropsychological and behavioral outcome. METHODS This prospective study included 54 pediatric patients with congenital hydrocephalus (21 female, 33 male; age range 0.03-194.5 months) who underwent surgery and 64 normal controls (30 female, 34 male; age range 0.30-197.75 months). DTI and neurodevelopmental outcome data were collected once in the control group and 3 times (preoperatively and at 3 and 12 months postoperatively) in the patients with hydrocephalus. DTI measures, including fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (AD), and radial diffusivity (RD) values were extracted from the genu of the corpus callosum (gCC) and the posterior limb of internal capsule (PLIC). Group analysis was performed first cross-sectionally to quantify DTI abnormalities at 3 time points by comparing the data obtained in the hydrocephalus group for each of the 3 time points to data obtained in the controls. Longitudinal comparisons were conducted pairwise between different time points in patients whose data were acquired at multiple time points. Neurodevelopmental data were collected and analyzed using the Adaptive Behavior Assessment System, Second Edition, and the Bayley Scales of Infant Development, Third Edition. Correlation analyses were performed between DTI and behavioral measures. RESULTS Significant DTI abnormalities were found in the hydrocephalus patients in both the gCC (lower FA and higher MD, AD, and RD) and the PLIC (higher FA, lower AD and RD) before surgery. The DTI measures in the gCC remained mostly abnormal at 3 and 12 months after surgery. The DTI abnormalities in the PLIC were

Idiopathic restrictive cardiomyopathy is part of the clinical expression of cardiac troponin I mutations

PubMed Central

Mogensen, Jens; Kubo, Toru; Duque, Mauricio; Uribe, William; Shaw, Anthony; Murphy, Ross; Gimeno, Juan R.; Elliott, Perry; McKenna, William J.

2003-01-01

Restrictive cardiomyopathy (RCM) is an uncommon heart muscle disorder characterized by impaired filling of the ventricles with reduced volume in the presence of normal or near normal wall thickness and systolic function. The disease may be associated with systemic disease but is most often idiopathic. We recognized a large family in which individuals were affected by either idiopathic RCM or hypertrophic cardiomyopathy (HCM). Linkage analysis to selected sarcomeric contractile protein genes identified cardiac troponin I (TNNI3) as the likely disease gene. Subsequent mutation analysis revealed a novel missense mutation, which cosegregated with the disease in the family (lod score: 4.8). To determine if idiopathic RCM is part of the clinical expression of TNNI3 mutations, genetic investigations of the gene were performed in an additional nine unrelated RCM patients with restrictive filling patterns, bi-atrial dilatation, normal systolic function, and normal wall thickness. TNNI3 mutations were identified in six of these nine RCM patients. Two of the mutations identified in young individuals were de novo mutations. All mutations appeared in conserved and functionally important domains of the gene. PMID:12531876

Correlation of circular RNA abundance with proliferation--exemplified with colorectal and ovarian cancer, idiopathic lung fibrosis, and normal human tissues.

PubMed

Bachmayr-Heyda, Anna; Reiner, Agnes T; Auer, Katharina; Sukhbaatar, Nyamdelger; Aust, Stefanie; Bachleitner-Hofmann, Thomas; Mesteri, Ildiko; Grunt, Thomas W; Zeillinger, Robert; Pils, Dietmar

2015-01-27

Circular RNAs are a recently (re-)discovered abundant RNA species with presumed function as miRNA sponges, thus part of the competing endogenous RNA network. We analysed the expression of circular and linear RNAs and proliferation in matched normal colon mucosa and tumour tissues. We predicted >1,800 circular RNAs and proved the existence of five randomly chosen examples using RT-qPCR. Interestingly, the ratio of circular to linear RNA isoforms was always lower in tumour compared to normal colon samples and even lower in colorectal cancer cell lines. Furthermore, this ratio correlated negatively with the proliferation index. The correlation of global circular RNA abundance (the circRNA index) and proliferation was validated in a non-cancerous proliferative disease, idiopathic pulmonary fibrosis, ovarian cancer cells compared to cultured normal ovarian epithelial cells, and 13 normal human tissues. We are the first to report a global reduction of circular RNA abundance in colorectal cancer cell lines and cancer compared to normal tissues and discovered a negative correlation of global circular RNA abundance and proliferation. This negative correlation seems to be a general principle in human tissues as validated with three different settings. Finally, we present a simple model how circular RNAs could accumulate in non-proliferating cells.

Passive flow regulators for drug delivery and hydrocephalus treatment

NASA Astrophysics Data System (ADS)

Chappel, E.; Dumont-Fillon, D.; Mefti, S.

2014-03-01

Passive flow regulators are usually intended to deliver or drain a fluid at a constant rate independently from pressure variations. New designs of passive flow regulators made of a stack of a silicon membrane anodically bonded to a Pyrex substrate are proposed. A first design has been built for the derivation of cerebrospinal fluid (CSF) towards peritoneum for hydrocephalus treatment. The device allows draining CSF at the patient production rate independently from postural changes. The flow rate is regulated at 20 ml/h in the range 10 to 40 mbar. Specific features to adjust in vivo the nominal flow rate are shown. A second design including high pressure shut-off feature has been made. The intended use is drug delivery with pressurized reservoir of typically 100 to 300 mbar. In both cases, the membrane comprises several holes facing pillars in the Pyrex substrate. These pillars are machined in a cavity which ensures a gap between the membrane and the pillars at rest. The fluid in the pressurized reservoir is directly in contact with the top surface of the membrane, inducing its deflection towards Pyrex substrate and closing progressively the fluidic pathway through each hole of the membrane. Since the membrane deflection is highly non-linear, FEM simulations have been performed to determine both radial position and diameter of the membrane holes that ensure a constant flow rate for a given range of pressure.

Insole-pressure distribution for normal children in different age groups.

PubMed

Liu, Xue-Cheng; Lyon, Roger; Thometz, John G; Curtin, Brian; Tarima, Serge; Tassone, Channing

2011-09-01

In measuring plantar pressures during gait, earlier methods have used a platform system that does not take into account the interactions feet have with orthotics and shoe wearing. The purpose of the study was to provide normal insole plantar pressure parameter data during stance phase using the Pedar pressure insole system. Twenty-nine normal children, age 6 to 16 years, were recruited and walked along the 25 m walkway at self-selected speeds. Patients were divided into 2 separate groups for statistical analysis--juniors (< 12 y old) and teenagers (> 13 y old). The pressure map was divided into 8 regions (masks) determined by anatomic landmarks and a total of 7 pressure parameters were analyzed of each mask. We did not detect significant differences in foot pressures between juniors and teenagers when regarding sex, or left and right feet for 7 parameters measured. This normative data will provide a basis with which to more accurately assess pediatric pathologic foot deformities and to distinguish dynamic foot deformities from anatomic foot deformities. THE LEVEL OF EVIDENCE: Level II.

Idiopathic pulmonary fibrosis misdiagnosed as sputum-negative pulmonary tuberculosis.

PubMed

Isah, Muhammad Danasabe; Abbas, Aminu; Abba, Abdullahi A; Umar, Mohammed

2016-01-01

Idiopathic pulmonary fibrosis (IPF), also known as cryptogenic fibrosing alveolitis, is one of a spectrum of idiopathic interstitial pneumonia. IPF is an increasingly common condition which poses many diagnostic and therapeutic challenges leading to misdiagnosis and mismanagement. We presented a 55-year-old male textile trader who was initially managed as sputum-negative pulmonary tuberculosis before histology report. He presented to our clinic with Breathlessness and cough of 3 years and 2.5 years, respectively. He had commenced anti-tuberculosis two months before presentation without significant relief. General Physical examination and vital signs were essentially normal. SPO2 was 96% on room air. Chest Examination revealed end-inspiratory bi-basal velcro-like crackles. Other systemic examinations were normal. Radiological examination by way of chest X- ray and chest CT showed features suggestive of IPF. The patient also had open Lung biopsy for histology and spirometry which demonstrated restrictive ventilatory function pattern. A diagnosis of Interstitial lung disease probably Idiopathic Pulmonary Fibrosis was entertained. He was commenced on Tab prednisolone, Tab Rabeprazole, with minimal improvement. IPF have often been misdiagnosed and treated as pulmonary tuberculosis with unfavorable outcome.

Idiopathic anaphylaxis.

PubMed

Fenny, Nana; Grammer, Leslie C

2015-05-01

Idiopathic anaphylaxis is a diagnosis of exclusion after other causes have been thoroughly evaluated and excluded. The pathogenesis of idiopathic anaphylaxis remains uncertain, although increased numbers of activated lymphocytes and circulating histamine-releasing factors have been implicated. Signs and symptoms of patients diagnosed with idiopathic anaphylaxis are indistinguishable from the manifestations of other forms of anaphylaxis. Treatment regimens are implemented based on the frequency and severity of patient symptoms and generally include the use of epinephrine autoinjectors, antihistamines, and steroids. The prognosis of idiopathic anaphylaxis is generally favorable with well-established treatment regimens and effective patient education. Copyright © 2015 Elsevier Inc. All rights reserved.

Head circumference: the forgotten tool for hydrocephalus management. A reference interval study in the Spanish population.

PubMed

Poca, Maria A; Martínez-Ricarte, Francisco R; Portabella, Mireia; Torné, Ramon; Fuertes, Maria L; González-Tartiere, Pilar; Sahuquillo, Juan

2013-11-01

In children, deviations from the normal range of head circumference (HC) have traditionally been related with CSF dynamics abnormalities. In adults, this neglected parameter is helpful in the diagnosis and understanding of the pathophysiology of some CSF abnormalities. It has been demonstrated that HC is related to height. Because humans have increased in stature dramatically during the last 50 years, pediatric charts for head growth physiology and normal HC values in adults should be reevaluated. The main aim of the present study was to assess HC in a series of 270 normal healthy Spanish adults and to determine any differences between sexes and age groups. A secondary aim was to discuss the relevance of this parameter in the management of hydrocephalus in adult people. HC measurements were taken using a measuring tape placed over the greatest frontal and occipital protuberances. The reference interval and the upper and lower thresholds for HC were calculated by 3 different methods: normal distribution, the non-parametrical percentile method, and the "robust method". The results were consistent and showed that Spanish adult men with a HC greater than 60 cm, and Spanish adult women with a HC greater than 58 cm should be considered macrocephalic. Microcephaly should be considered when HC is 53.6 cm in men and 51.3 cm in women. Adult age groups of either sex do not present any statistically significant differences in HC. HC obtained in Spanish adult people are greater than those reported in the classical Nellhaus graphs in both men and women aged 18. These findings should be considered in the management of hydrocephalus in adults today. Copyright © 2013 Elsevier B.V. All rights reserved.

Communicating Hydrocephalus Associated with Small- to Medium-Sized Vestibular Schwannomas: Clinical Significance of the Tumor Apparent Diffusion Coefficient Map.

PubMed

Taniguchi, Masaaki; Nakai, Tomoaki; Kohta, Masaaki; Kimura, Hidehito; Kohmura, Eiji

2016-10-01

The etiology of hydrocephalus associated with the small- to medium-sized vestibular schwannomas is still controversial. We investigated tumor-specific factors related to the association of hydrocephalus with small- to medium-sized vestibular schwannomas. Among the 77 patients with vestibular schwannoma smaller than 30 mm, 9 patients demonstrated associated communicating hydrocephalus. Patient medical records, radiologic data, and histopathologic specimens were reviewed retrospectively. The age of the patients, and size, mean apparent diffusion coefficient (ADC) value, and histologic features of the tumors were compared with those of patients without hydrocephalus. The symptoms related to hydrocephalus improved in all patients after tumor removal. Both the mean size and ADC values exhibited a statistically significant difference between the tumors with and without hydrocephalus (P < 0.01). The size and ADC value of the tumor were significantly related to the association with hydrocephalus. The increased tumor ADC value was considered to be the result of degenerative change and suggested the involvement of protein sloughing in the etiology of the associated hydrocephalus. Copyright © 2016 Elsevier Inc. All rights reserved.

Social media networking in pediatric hydrocephalus: a point-prevalence analysis of utilization.

PubMed

Elkarim, Ghassan Awad; Alotaibi, Naif M; Samuel, Nardin; Wang, Shelly; Ibrahim, George M; Fallah, Aria; Weil, Alexander G; Kulkarni, Abhaya V

2017-08-01

OBJECTIVE A recent survey has shown that caregivers of children with shunt-treated hydrocephalus frequently use social media networks for support and information gathering. The objective of this study is to describe and assess social media utilization among users interested in hydrocephalus. METHODS Publicly accessible accounts and videos dedicated to the topic of hydrocephalus were comprehensively searched across 3 social media platforms (Facebook, Twitter, and YouTube) throughout March 2016. Summary statistics were calculated on standard metrics of social media popularity. A categorization framework to describe the purpose of pages, groups, accounts, channels, and videos was developed following the screening of 100 titles. Categorized data were analyzed using nonparametric tests for statistical significance. RESULTS The authors' search identified 30 Facebook pages, 213 Facebook groups, 17 Twitter accounts, and 253 YouTube videos. These platforms were run by patients, caregivers, nonprofit foundations, and patient support groups. Most accounts were from the United States (n = 196), followed by the United Kingdom (n = 31), Canada (n = 17), India (n = 15), and Germany (n = 12). The earliest accounts were created in 2007, and a peak of 65 new accounts were created in 2011. The total number of users in Facebook pages exceeded those in Facebook groups (p < 0.001). The majority of users in Facebook groups were in private groups, in contrast to public groups (p < 0.001). The YouTube videos with the highest median number of views were for surgical products and treatment procedures. CONCLUSIONS This study presents novel observations into the characteristics of social media use in the topic of hydrocephalus. Users interested in hydrocephalus seek privacy for support communications and are attracted to treatment procedure and surgical products videos. These findings provide insight into potential avenues of hydrocephalus outreach, support, or advocacy in social media.

Everolimus Alleviates Obstructive Hydrocephalus due to Subependymal Giant Cell Astrocytomas.

PubMed

Moavero, Romina; Carai, Andrea; Mastronuzzi, Angela; Marciano, Sara; Graziola, Federica; Vigevano, Federico; Curatolo, Paolo

2017-03-01

Subependymal giant cell astrocytomas (SEGAs) are low-grade tumors affecting up to 20% of patients with tuberous sclerosis complex (TSC). Early neurosurgical resection has been the only standard treatment until few years ago when a better understanding of the molecular pathogenesis of TSC led to the use of mammalian target of rapamycin (mTOR) inhibitors. Surgical resection of SEGAs is still considered as the first line treatment in individuals with symptomatic hydrocephalus and intratumoral hemorrhage. We describe four patients with symptomatic or asymptomatic hydrocephalus who were successfully treated with the mTOR inhibitor everolimus. We collected the clinical data of four consecutive patients presenting with symptomatic or asymptomatic hydrocephalus due to a growth of subependymal giant cell atrocytomas and who could not undergo surgery for different reasons. All patients experienced a clinically significant response to everolimus and an early shrinkage of the SEGA with improvement in ventricular dilatation. Everolimus was well tolerated by all individuals. Our clinical series demonstrate a possible expanding indication for mTOR inhibition in TSC, which can be considered in patients with asymptomatic hydrocephalus or even when the symptoms already appeared. It offers a significant therapeutic alternative to individuals that once would have undergone immediate surgery. Everolimus might also allow postponement of a neurosurgical resection, making it elective with an overall lower risk. Copyright © 2016 Elsevier Inc. All rights reserved.

Obstructive hydrocephalus secondary to odontoid pannus: case report and review of literature.

PubMed

Soon, Wai Cheong; Thanabalasundaram, Gopiga; Thant, Kyaw Zayar; Ogbonnaya, Ebere Sunny; Harrisson, Stuart Edward

2018-04-01

Odontoid pannus or periodontoid pseudotumour is associated with a variety of rheumatological conditions. We report a case of an 80-year old man who presented with acute hydrocephalus following an emergency operation to amputate his infected left big toe. Imaging revealed a large tissue mass causing severe compression at the cervico-medullary junction and obstruction of CSF flow. The acute hydrocephalus was presumed to be related to neck manoeuvring during general anaesthesia. Following an initial emergency CSF diversion through external ventricular drainage catheter insertion, the patient subsequently underwent ventriculo-peritoneal insertion and posterior spinal decompression and fixation. To our knowledge, there have been no reported cases of tophaceous gout of the odontoid pannus causing acute hydrocephalus.

Successful treatment of Dandy-Walker syndrome by endoscopic third ventriculostomy in a 6-month-old girl with progressive hydrocephalus: a case report and literature review.

PubMed

Hu, Chih-Fen; Fan, Hueng-Chuen; Chang, Cheng-Fu; Wang, Chih-Chien; Chen, Shyi-Jou

2011-02-01

Dandy-Walker syndrome (DWS) is a congenital brain malformation involving the cerebellum and fourth ventricle. We report a 6-month-old girl with DWS presenting an initially normal ventricular system and mild cyst-like lesion over the posterior fossa as assessed by postnatal brain sonography. However, symptoms and signs of increased intracranial cerebral pressure in terms of frequent vomiting and tense anterior fontanel developed, and these were associated with mild hypotonia and poor neck support, and upward-gaze palsy at the age of 6 months. Magnetic resonance imaging revealed a huge cystic lesion of the fourth ventricle, which filled the posterior fossa and ventricular dilatation. The tentorium was progressively displaced upward by the cyst. A nearly complete agenesis of the cerebellar vermis was also confirmed. After a successful endoscopic third ventriculostomy, a series of brain magnetic resonance imaging scans, taken during a follow-up survey, showed normal lateral and third ventricles. Consequently, symptoms of intracranial cerebral pressure resolved, and a developmental milestone was achieved. In conclusion, DWS can be confirmed postpartum, and endoscopic third ventriculostomy was found to be a preferential operative procedure for DWS with hydrocephalus. It may be effective for patients younger than 1 year. Copyright © 2011. Published by Elsevier B.V.

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