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1

Voxel-based morphometry and intellectual assessment in patients with congenital bilateral perisylvian syndrome.  

PubMed

Congenital bilateral perisylvian syndrome (CBPS) presents with heterogeneous clinical manifestations such as pseudobulbar palsy, language disorder, variable cognitive deficits, epilepsy, and perisylvian abnormalities (most frequently polymicrogyria) on imaging studies. We investigated the relationship between seizures and extent of gray matter (GM) and white matter (WM) abnormalities using voxel-based morphometry (VBM) of brain magnetic resonance imaging (MRI) as well the association between seizures, structural abnormalities and cognitive ability. In this cross-sectional study, we evaluated 51 healthy volunteers and 18 patients with CBPS with epilepsy (seizure group, n = 7) and without (non-seizure group, n = 11). We used VBM (SPM8/DARTEL) to investigate areas with excess and atrophy of both gray and white matter, comparing groups of patients with controls. Intellectual ability of patients was assessed by the WISC-III or WAIS-III. Both groups with CBPS and the control group were homogeneous with respect to gender (p = 0.07) and age (p = 0.065). Besides perisylvian polymicrogyria, the seizure group exhibited areas with GM and WM reduction including temporal, frontal, parietal and occipital lobes. In contrast, we identified fewer areas with GM and WM reduction in the non-seizure group. The seizure group presented worse intellectual performance (performance IQ and global IQ) than the non-seizure group. The seizure group presented with a more widespread pattern of cortical and sub-cortical abnormalities, as well as worse cognition. Our results suggest that patients with CBPS and epilepsy appear to have widespread neuronal damage that goes beyond the areas with MRI-visible perisylvian polymicrogyria. PMID:24781839

Yasuda, Clarissa L; Guimarães, Catarina A; Guerreiro, Marilisa M; Boscariol, Mirela; Oliveira, Ecila P M; Teixeira, Karine C; Costa, André L F; Beltramini, Guilherme C; Cendes, Fernando

2014-07-01

2

Anterior corpus callosotomy combined with anterior temporal resection with amygdalohippocampectomy: outcome in a patient with congenital bilateral perisylvian syndrome.  

PubMed

Congenital bilateral perisylvian syndrome (CBPS) is characterized by epilepsy, cognitive deficits, pseudobulbar palsy and diplegia of the facial, pharyngeal and masticatory muscles. Epilepsy has been described in nearly 90% of affected patients. The epilepsy is usually severe and pharmacoresistant in about 55 percent of CBPS patients. Until now, only 12 cases of surgical treatment on CBPS have been reported; the surgical treatment is usually corpus callosotomy. In this paper, we describe a previously unreported combination of anterior corpus callosotomy plus anterior temporal lobectomy with amygdalohippocampectomy for a patient with CBPS, resulting in a satisfactory clinical outcome. Based on this case, we suggest that palliative focal resective surgery combined with anterior corpus callosotomy should be considered when a predominance of the epileptiform discharges suggests focal onset in patients with CBPS. Meanwhile, the clinical decision to adopt this combination surgery must be based on a thorough pre-surgical evaluation, and should take into account the clinical, radiological, and EEG features. PMID:24535795

Junming, Zhu; Yuanyuan, Zhao; Fang, Feng; Weiming, Fu; Ryan, Hays; Jianmin, Zhang; Li, Feng; Xiao, Jin; Shuda, Chen

2014-01-01

3

Worster-Drought syndrome (congenital bilateral perisylvian syndrome) with posterior pituitary ectopia, pituitary hypoplasia, empty sella and panhypopituitarism: a patient report.  

PubMed

Worster-Drought syndrome (WDS) (congenital bilateral perisylvian syndrome, congenital pseudobulbar paresia) is characterized by neuronal migration defect, pseudobulbar paralysis, epilepsy, neuromotor retardation and perisylvian dysplasia. We report a patient with WDS associated with posterior pituitary ectopia, pituitary hypoplasia, partial empty sella and panhypopituitarism, not previously reported in the literature. The 16.4 year-old female patient had severe growth retardation with height SDS -4.5, delayed puberty, microcephaly, pes equinovarus deformity, developmental delay, speech disorder and epilepsy. Laboratory findings, which revealed abnormal electroencephalography and bilateral perisylvian cortical dysplasia on cranial magnetic resonance imaging (MRI) were consistent with WDS. Endocrinological evaluation revealed secondary hypothyroidism and combined deficiency of adrenocorticotropin, gonadotropin and growth hormone (GH). Sella MRI showed congenital empty sella, anterior pituitary hypoplasia, ectopic neurohypophysis, and stalk agenesis. Appropriate replacement therapy was started. GH treatment resulted in a final height of 150.3 cm, appropriate for her target height. This is the first reported patient with WDS associated with congenital structural hypothalamic-pituitary abnormalities, including empty sella, pituitary hypoplasia, posterior pituitary ectopia, stalk agenesis and panhypopituitarism. GH has been successful in the treatment of her short stature. PMID:16759040

Ba?, Firdevs; Darendeliler, Feyza; Yapici, Zuhal; Gökalp, Selman; Bundak, Rüveyde; Saka, Nurçin; Günöz, Hülya

2006-04-01

4

A clinically recognizable neuronal migration disorder: congenital bilateral perisylvian syndrome. Case report with long-term clinical and EEG follow-up.  

PubMed

Congenital bilateral perisylvian syndrome (CBPS) is a recently described, neuronal migration disorder, characterized by pseudobulbar palsy, epilepsy and mental retardation and bilateral perisylvian dysplasia. A 15-year-old boy was diagnosed with CBPS according to the typical clinical, and magnetic resonance imaging (MRI) features. The patient was suffering from atypical absence seizures, repeating daily in spite of antiepileptic drug therapy, since age 7 years. He had also experienced rare generalized tonic-clonic seizures and complex partial seizures. Neurological examination showed severe restriction of tongue movements, severe dysarthria, dysphagia, facial diplegia, mild pyramidal signs and moderate mental retardation. A computed tomographic (CT) scan demonstrated bilateral perisylvian enlargement. The diagnosis was corrected with MRI after six years. Frequent irregular generalized spike and wave abnormalities and focal sharp and slow waves over the posterior regions of both hemispheres were shown by electroencephalograms (EEG). The patient was treated with Na-Valproate, carbamazepine and lamotrigine but did now show any significant change in seizure frequency in the eight-year follow-up period. Intractable seizures, mental retardation and particularly congenital pseudobulbar palsy suggest this congenital entity. Those patients who exhibit these typically clinical features, must have MRI. PMID:9530947

Baykan-Kurt, B; Sarp, A; Gökyi?it, A; Tunçay, R; Cali?kan, A

1997-12-01

5

Intractable epilepsy in Turner syndrome associated with bilateral perisylvian hypoplasia: one case report  

Microsoft Academic Search

Turner syndrome (TS) is the most frequent sex abnormality in females, generally associated with a 45,X0 karyotype. Although neurological complications are frequently part of the clinical picture, serious brain abnormalities are quite rare in TS.Epilepsy in TS is not frequent and so far only few cases have been reported, usually associated with cortical dysplasias. We report a Turner patient showing

Salvatore Striano; Pasquale Striano; Fabio Tortora; Andrea Elefante

2005-01-01

6

Verbal and gestural communication in children with bilateral perisylvian polymicrogyria.  

PubMed

We assessed intelligence and receptive and expressive language skills in 6 children, ages 7 years 9 months to 12 years 4 months, with bilateral perisylvian polymicrogyria of variable extent and with dysarthria of different severity. In view of the recent findings of a close relationship between word and gesture, we also examined the communicative use of gesture. We found that mental retardation was related to the extent of cortical malformation; lexical comprehension, but not morphosyntactic comprehension, and verbal production were more compromised than expected from nonverbal intellectual abilities; lack of verbal language was not compensated by the use of referential gestures. Results are discussed suggesting that compromised verbal and gestural communication in bilateral perisylvian polymicrogyria are not due simply to mental retardation and/or dysarthria but also to dysfunction of Sylvian fissure areas concerned with the totality of language processing. PMID:17890406

Saletti, Veronica; Bulgheroni, Sara; D'Incerti, Ludovico; Franceschetti, Silvana; Molteni, Bruna; Airaghi, Gloria; Pantaleoni, Chiara; D'Arrigo, Stefano; Riva, Daria

2007-09-01

7

A Locus for Bilateral Perisylvian Polymicrogyria Maps to Xq28  

PubMed Central

Polymicrogyria (PMG) is one of a large group of human cortical malformations that collectively account for a significant percentage of patients with epilepsy, congenital neurological deficits, and intellectual disability. PMG is characterized by an excess of small gyri and abnormal cortical lamination. The most common distribution is bilateral, symmetrical, and maximal, in the region surrounding the sylvian fissures, and is known as “bilateral perisylvian polymicrogyria” (BPP). Most cases are sporadic, although several families have been observed with multiple affected members, usually following an X-linked inheritance pattern. Here we report the first genetic locus for BPP mapped by linkage analysis in five families. Linkage places the critical region for BPP at Xq28 (LOD score 3.08 in Xq28, distal to DXS8103 by multipoint analysis). We suggest that this region contains a gene that is necessary for correct neuronal organization and that the identification of this gene will both enhance our understanding of normal cortical development and accelerate the identification of other genes responsible for PMG.

Villard, Laurent; Nguyen, Karine; Cardoso, Carlos; Martin, Christa Lese; Weiss, Ann M.; Sifry-Platt, Mara; Grix, Arthur W.; Graham, Jr., John M.; Winter, Robin M.; Leventer, Richard J.; Dobyns, William B.

2002-01-01

8

The Syndrome of Perisylvian Polymicrogyria with Congenital Arthrogryposis  

PubMed Central

Background Bilateral perisylvian polymicrogyria (BPP) is a well-recognized malformation of cortical development commonly associated with epilepsy, cognitive impairment, and oromotor apraxia. Reports have suggested the association of BPP with arthrogryposis multiplex congenita. We sought to investigate the clinical, electrophysiological, and neuroradiological features of this combined syndrome to determine if there are unique features that distinguish BPP with arthrogryposis from BPP alone. Methods Cases of BPP with congenital arthrogryposis were identified from a large research database of individuals with polymicrogyria. Clinical features (including oromotor function, seizures, and joint contractures), MR brain imaging, and results of neuromuscular testing were reviewed. Results Ten cases of BPP with congenital arthrogryposis were identified. Most cases had some degree of oromotor apraxia. Only a few had seizures, but a majority of cases were still young children. Electrophysiological studies provided evidence for lower motor neuron or peripheral nervous system involvement. On brain imaging, bilateral polymicrogyria (PMG) centered along the Sylvian fissures was seen, with variable extension frontally or parietally; no other cortical malformations were present. We did not identify obvious neuroimaging features that distinguish this syndrome from that of BPP without arthrogryposis. Conclusions The clinical and neuroimaging features of the syndrome of BPP with congenital arthrogryposis appear similar to those seen in cases of isolated BPP without joint contractures, but electrophysiological studies often demonstrate coexistent lower motor neuron or peripheral nervous system pathology. These findings suggest that BPP with arthrogryposis may have a genetic etiology with effects at two levels of the neuraxis.

Poduri, Annapurna; Chitsazzadeh, Vida; D'Arrigo, Stefano; Fedrizzi, Ermellina; Pantaleoni, Chiara; Riva, Daria; Busse, Claudia; Kuster, Helmut; Duplessis, Adre; Gaitanis, John; Sahin, Mustafa; Garganta, Cheryl; Topcu, Meral; Dies, Kira A.; Barry, Brenda J.; Partlow, Jennifer; Barkovich, A. James; Walsh, Christopher A.; Chang, Bernard S.

2009-01-01

9

Worster-Drought and congenital perisylvian syndromes-a continuum?  

PubMed

A 5-year-old female was evaluated because of severe speech and expressive language delay. On examination, she could hardly speak and communicated through gestures. She manifested severe dysarthria and difficulty in protruding and moving her tongue laterally. She lacked coordination of the swallowing process, with drooling and an increased mental reflex. Her cognitive development was normal, and no associated neurologic dysfunction of the limbs was noted. On follow-up, the child experienced two episodes of seizures at 6 years of age. Magnetic resonance imaging of the brain demonstrated perisylvian and frontal polymicrogyria. Clinical and radiologic findings demonstrated a similarity and continuum between congenital suprabulbar paresis (Worster-Drought syndrome) and perisylvian syndrome. PMID:11275467

Nevo, Y; Segev, Y; Gelman, Y; Rieder-Grosswasser, I; Harel, S

2001-02-01

10

Bilateral Subclavian Steal Syndrome  

PubMed Central

Bilateral subclavian steal syndrome is a rare condition. It is usually due to reversal of vertebral blood flow in the setting of bilateral proximal subclavian or left subclavian plus innominate artery severe stenosis or occlusion. This finding may cause cerebral ischemia related to upper extremities exercise. We report a case of bilateral subclavian steal secondary to total occlusion of the innominate and left subclavian arteries in a patient who presented with cardiomyopathy and flow reversal in the right carotid and bilateral vertebral arteries.

Amini, Reza; Gornik, Heather L.; Gilbert, Leslie; Whitelaw, Sue; Shishehbor, Mehdi

2011-01-01

11

Bilateral nevus comedonicus syndrome.  

PubMed

Nevus comedonicus is an uncommon skin abnormality characterized by an aggregation of dilated follicular orifices filled with keratinous material. Nevus comedonicus is occasionally complicated with other conditions including cataracts, skeletal defects, central nervous system abnormalities or other extra-cutaneous diseases (nevus comedonicus syndrome). Although most cases of nevus comedonicus occur unilaterally on the face, neck and chest, the lesions occasionally show a bilateral distribution (bilateral nevus comedonicus). We report here an unusual case of bilaterally disseminated nevus comedonicus with various systemic complications. A 62-year-old Japanese man presented with a 50-year history of numerous keratotic papules and comedo-like lesions, which gradually worsened with time. Physical examination revealed that the papules were skin-colored and 1-4 mm in diameter. Some papules had dark-black keratinous materials on their surface, giving them a comedo-like appearance. The lesions were located predominantly on the face, head, neck and trunk with symmetric distribution, following Blaschko's lines. The patient's past medical history was noteworthy; he had undergone surgical treatments for thyroid cancer, pneumothorax and schwannoma in the cauda equina. He also suffered from scoliosis, cervical spondylosis and atrial fibrillation. Histopathologic examination revealed dilated and invaginated follicular structures filled with lamellar keratin, compatible with nevus comedonicus. Our patient's case did not fit with any previously reported diseases, and we thought a diagnosis of "bilateral nevus comedonicus syndrome" was the most appropriate for our patient's condition. He was treated with topical retinoic acid and activated vitamin D3 ointment for 3 months each, but the lesions remained unchanged. PMID:24031154

Ito, Takamichi; Mitamura, Yasutaka; Tsuji, Yayoi; Harada, Kayo; Urabe, Kazunori

2013-06-01

12

Anatomical predictors of aphasia recovery: a tractography study of bilateral perisylvian language networks.  

PubMed

Stroke-induced aphasia is associated with adverse effects on quality of life and the ability to return to work. For patients and clinicians the possibility of relying on valid predictors of recovery is an important asset in the clinical management of stroke-related impairment. Age, level of education, type and severity of initial symptoms are established predictors of recovery. However, anatomical predictors are still poorly understood. In this prospective longitudinal study, we intended to assess anatomical predictors of recovery derived from diffusion tractography of the perisylvian language networks. Our study focused on the arcuate fasciculus, a language pathway composed of three segments connecting Wernicke's to Broca's region (i.e. long segment), Wernicke's to Geschwind's region (i.e. posterior segment) and Broca's to Geschwind's region (i.e. anterior segment). In our study we were particularly interested in understanding how lateralization of the arcuate fasciculus impacts on severity of symptoms and their recovery. Sixteen patients (10 males; mean age 60 ± 17 years, range 28-87 years) underwent post stroke language assessment with the Revised Western Aphasia Battery and neuroimaging scanning within a fortnight from symptoms onset. Language assessment was repeated at 6 months. Backward elimination analysis identified a subset of predictor variables (age, sex, lesion size) to be introduced to further regression analyses. A hierarchical regression was conducted with the longitudinal aphasia severity as the dependent variable. The first model included the subset of variables as previously defined. The second model additionally introduced the left and right arcuate fasciculus (separate analysis for each segment). Lesion size was identified as the only independent predictor of longitudinal aphasia severity in the left hemisphere [beta = -0.630, t(-3.129), P = 0.011]. For the right hemisphere, age [beta = -0.678, t(-3.087), P = 0.010] and volume of the long segment of the arcuate fasciculus [beta = 0.730, t(2.732), P = 0.020] were predictors of longitudinal aphasia severity. Adding the volume of the right long segment to the first-level model increased the overall predictive power of the model from 28% to 57% [F(1,11) = 7.46, P = 0.02]. These findings suggest that different predictors of recovery are at play in the left and right hemisphere. The right hemisphere language network seems to be important in aphasia recovery after left hemispheric stroke. PMID:24951631

Forkel, Stephanie J; Thiebaut de Schotten, Michel; Dell'Acqua, Flavio; Kalra, Lalit; Murphy, Declan G M; Williams, Steven C R; Catani, Marco

2014-07-01

13

Congenital polymicrogyria including the perisylvian region in early childhood.  

PubMed

Six pediatric cases including four infants with congenital polymicrogyria including the perisylvian region are presented herein. Their clinical features were analyzed and compared with patients suffering from congenital bilateral perisylvian syndrome (CBPS). Two specific abnormalities were diagnosed as accompanying disorders in two cases, namely Kabuki syndrome and Peters' anomaly. In the other four cases, the pathogenetic etiology was not elucidated. Subtle symptoms, such as choking and drooling became detectable in one case each, and expressive language development was delayed in two patients. A developmental delay became apparent in five cases during the follow-up period, and epilepsy was observed in one patient with onset at 12 years of age. Our results indicate that the presence of perisylvian polymicrogyria may not always result in the development of oropharyngoglossal dysfunction or dysarthria, although most patients tend to gradually show the onset of developmental disorders. To support cognitive and psychosocial development, an early integrated approach, including not only conventional speech and language therapy, but also various communication methods is essential for patients with congenital polymicrogyria including the perisylvian region. PMID:20201970

Takano, Tomoyuki; Matsuwake, Kumiko; Yoshioka, Seiichirou; Takeuchi, Yoshihiro

2010-03-01

14

Bilateral Congenital Lacrimal Fistula in Down Syndrome  

PubMed Central

Congenital lacrimal fistulae are rare in Down syndrome and bilateral presentation is very unusual. It can be associated with nasolacrimal duct obstruction. We report a 3-year-old female with Down syndrome who presented with watering and discharge from both eyes and bilateral fistulous openings present inferonasal to the medial canthus. Upon examination, the lacrimal sac regurgitation test was positive on both sides. Our case report documents a distinctive case of bilateral congenital lacrimal fistulae in association with Down syndrome. It was managed successfully by primary fistulectomy and nasolacrimal duct probing.

Singh, Manpreet; Singh, Usha

2013-01-01

15

A new case of megalencephaly and perisylvian polymicrogyria with post-axial polydactyly and hydrocephalus: MPPH syndrome.  

PubMed

We report a new case of megalencephaly and polymicrogyria with post-axial polydactyly and hydrocephalus (MPPH syndrome) in an 18-month-old girl. She was the first child of healthy non-consanguineous parents and measurements at birth were +3 standard deviations (S.D.) for weight, +2 S.D. for length and +4 S.D. for OFC. Ultrasound scan at 24 weeks of gestation (WG) showed mild ventriculomagaly with unique umbilical artery and dacryocystocele. Clinical examination at birth revealed macrosomia with macrocephaly, facial dysmorphism, post-axial polydactyly at the right hand and both feet, and axial hypotonia with hypertonic arms and legs. At 18 months of age, weight was +2 S.D., length was +2 S.D. and OFC was +4 S.D. She remained hypertonic, could not sit and had no hand use. Cerebral magnetic resonance imaging showed stable ventriculomegaly and polymicrogyria located on the frontal and perisylvian region with white matter hypersignal on T2-weighted images. There was no associated visceral malformation. Standard and high-resolution cytogenetic examination, telomeric FISH and array-CGH studies were normal. This case represents the sixth observation of MPPH syndrome as described by Mirzaa et al. in 2004. We provide further neurological follow-up since three out of five index patients were aged 6 months or less. We postulate that macrosomia at birth might be a major feature (five/six cases), with advanced bone age in the two/two investigated cases. White matter abnormalities might be an occasional feature of this syndrome (three/six cases), as well as dacryocystocele, if not coincidental (one/six case). The mode of inheritance of this syndrome remains unknown since there was no significant family history in all reported cases. The search for infracytogenetic chromosomal imbalance was unsuccessful. PMID:16807158

Colombani, Marina; Chouchane, Mondher; Pitelet, Gaelle; Morales, Laure; Callier, Patrick; Pinard, Jean-Pierre; Lion-François, Laurence; Thauvin-Robinet, Christel; Mugneret, Francine; Huet, Frédéric; Guibaud, Laurent; Faivre, Laurence

2006-01-01

16

Bilateral Compartment Syndrome Complicating Prolonged Lithotomy Position  

Microsoft Academic Search

Summary A case of bilateral compartment syndrome after prolonged Lloyd-Davies lithotomy position is described. The diagnosis was made early, despite effective extradural bupivacaine- fentanyl analgesia. The aetiology, diagnosis, pathology and treatment of compartment syn- drome are described. Complications of the syndrome may be life-threatening and perma- nently disabling. The anaesthetist should be aware of the potential complications of the operative

J. TUCKEY

1997-01-01

17

Gorlin syndrome and bilateral ovarian fibroma  

PubMed Central

INTRODUCTION Gorlin syndrome (GS), also known as nevoid basal cell carcinoma syndrome (NBCCS), is a rare hereditary, autosomal dominant disease that affects various systems. Its prevalence is estimated at 1/57,000 to 1/256,000 of the population. It is characterized by basal cell carcinomas, multiple odontogenic keratocysts, skeletal abnormalities and ovarian fibroma, among other disorders. PRESENTATION OF CASE To report the case of a young patient with Gorlin syndrome and bilateral ovarian fibroma. DISCUSSION A 20-year old patient with Gorlin syndrome presented with facial asymmetry, broad nasal root, dental abnormalities, micrognathism, convergent strabismus, multiple pigmented lesions on the trunk and face, pectus excavatum, kyphoscoliosis and a palpable mass in the abdomen occupying the entire pelvic region. CONCLUSION Gorlin–Goltz syndrome is a hereditary pathology that includes numerous clinical manifestations. Diagnosis is clinical and genetic confirmation is unnecessary.

Pirschner, Fernanda; Bastos, Pollyana Marcal; Contarato, George Luiz; Bimbato, Anna Carolina Bon Lima; Filho, Antonio Chambo

2012-01-01

18

Bilateral piriformis syndrome after total hip arthroplasty  

Microsoft Academic Search

We present the case of a 39-year-old man with bilateral piriformis syndrome 4 and 6 years after two cementless total hip\\u000a arthroplasties, respectively. During surgical exploration, each sciatic nerve was found to be entrapped by a tense piriformis\\u000a muscle and hypertrophic posterior hip capsule. The sciatic-type pain was relieved after sectioning each piriformis muscle\\u000a with external neurolysis. This is the

Y. Uchio; U. Nishikawa; M. Ochi; N. Shu; K. Takata

1998-01-01

19

Atypical Cogan's syndrome presenting as bilateral endogenous endophthalmitis.  

PubMed

We report a case of atypical Cogan's syndrome presenting as bilateral endogenous endophthalmitis in a woman with ovarian cancer. A 62-year-old woman with ovarian cancer developed bilateral interstitial keratitis and panuveitis accompanied by bilateral sensorineural hearing loss and chondritis. Auricular cartilage biopsy ruled out relapsing polychondritis and the diagnosis of atypical Cogan's syndrome was set clinically. PMID:22908938

Georgakopoulos, Constantine D; Makri, Olga E; Exarchou, Artemis M; Pharmakakis, Nikolaos

2014-01-01

20

Megacystis microcolon intestinal hypoperistalsis syndrome with bilateral duplicated systems.  

PubMed

A 29-year-old multigravida woman presented for her second prenatal ultrasound evaluation at 30 weeks of gestation. The study showed a female fetus, bilateral duplicated systems with severe hydronephrosis in the upper pole moieties and a massively distended bladder. Initial interpretation suggested ectopic/obstructing bilateral ureteroceles. To evaluate these findings further, a prenatal magnetic resonance imaging scan was obtained, documenting the absence of ureteroceles. The presumptive diagnosis of megacystis microcolon intestinal hypoperistalsis syndrome was made. After birth, contrast enema confirmed the presence of microcolon. This appears to be the first reported case of megacystis microcolon intestinal hypoperistalsis syndrome with bilateral duplex systems evaluated with prenatal magnetic resonance imaging. PMID:12837448

Lorenzo, Armando J; Twickler, Diane M; Baker, Linda A

2003-07-01

21

Recurrent Bilateral Occipital Infarct with Cortical Blindness and Anton Syndrome  

PubMed Central

Bilateral cortical blindness and Anton syndrome, are most commonly caused by ischaemic stroke. In this condition, patients have loss of vision but deny their blindness despite objective evidence of visual loss. We report a case of a patient with multiple cardiovascular risk factors who developed recurrent bilateral occipital lobe infarct with Anton syndrome. A suspicion of this condition should be raised when the patient has denial of blindness in the presence of clinical and radiological evidence of occipital lobe injury. Management of this condition should focus on the underlying cause, in which our patient requires secondary stroke prevention and rehabilitation.

Kwong Yew, Kiu; Abdul halim, Sanihah; Tharakan, John

2014-01-01

22

Acute bilateral forearm compartment syndrome of unknown aetiology.  

PubMed

This is the first reported case of non-traumatic, acute bilateral forearm compartment syndrome. Despite a delay of over 24 hours until surgical decompression and 50% muscle fibre necrosis in the histopathological examination, the clinical outcome was excellent after fasciotomy, delayed primary wound closure and early institution of a range of motion exercise programme. The literature on non-traumatic causes of compartment syndrome is reviewed. PMID:17405196

O'Leary, E J P; Bulstrode, N W; Gschwind, C

2006-01-01

23

Bilateral acute lupus pneumonitis in a case of rhupus syndrome  

PubMed Central

Rhupus syndrome, the overlap of rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE), is an extremely uncommon condition. Organ damages found due to SLE are usually mild in rhupus. Lupus pneumonitis in rhupus syndrome has not been reported worldwide. We are reporting a 23-year-old female with bilateral symmetric erosive arthritis, oral ulcer, alopecia, polyserositis, anemia, leucopenia, positive RA-factor, anti nuclear antibody (ANA) and anti ds-DNA. She presented with acute onset dyspnea, high fever, chest pain, tachycardia, tachypnea, hypoxia and respiratory alkalosis. High resolution computed tomography (HRCT)-thorax showed bilateral, basal consolidation with air bronchogram. Repeated sputum and single broncho alveolar lavage (BAL) fluid examination revealed no organism or Hemosiderin-laden macrophage. The diagnosis of rhupus was confirmed by combined manifestations of RA and SLE, and the diagnosis of acute lupus pneumonitis was established by clinico-radiological picture and by excluding other possibilities.

Sarkar, Supriya; Saha, Kaushik

2012-01-01

24

A case of bilateral frontal tumors without "frontal syndrome".  

PubMed

We report the longitudinal case study of a right-handed patient harboring two frontal tumors that benefited from bilateral simultaneous surgery. The tumors were WHO Grade II gliomas located in the left inferior frontal area (including the cingulate gyrus) and the right anterior superior frontal gyrus. The double tumor resection was guided by direct electrical stimulation of brain areas while the patient was awake. Neuropsychological assessments were administered before and after the surgery to analyse how the brain functions in the presence of two frontal gliomas that affect both hemispheres and reacts to a bilateral resection, which can brutally compromise the neuronal connectivity, progressively established during the infiltrating process. We showed that both the tumor infiltration and their bilateral resection did not lead to a "frontal syndrome" or a "dysexecutive syndrome" predicted by the localization models. However, a subtle fragility was observed in fine-grain language, memory and emotional skills. This case study reveals the significance of brain plasticity in the reorganization of cognitive networks, even in cases of bilateral tumors. It also confirms the clinical relevance of hodotopical brain models, which considers the brain to be organized in parallel-distributed networks around cortical centers and epicenters. PMID:23962155

Plaza, M; du Boullay, V; Perrault, A; Chaby, L; Capelle, L

2014-12-01

25

Bilateral axillary arterial aneurysms in siblings with Marfan syndrome.  

PubMed

Axillary arterial aneurysm associated with Marfan syndrome is an extremely rare and dangerous disease because of its potential risk for rupture and impairment of neurologic function in the upper extremities. Treatment of large axillary aneurysms can be surgically challenging because the axillary artery is anatomically located under the clavicle bones and the brachial plexus runs dorsal to the aneurysm. We report 2 cases of bilateral axillary arterial aneurysms in brothers with Marfan syndrome. Revival of a novel approach and cautious attempts during the procedures are described in both cases. PMID:24378247

Saito, Yoshiaki; Taniguchi, Satoshi; Watanabe, Kenichi; Fukui, Kozo; Fukuda, Ikuo

2014-04-01

26

Bilateral gluteal compartment syndrome after total hip arthroplasty under epidural anesthesia.  

PubMed

The gluteal compartment syndrome is uncommon and is discussed in only a few published case reports. The simultaneous bilateral gluteal compartment syndrome is exceptionally rare and is tackled in only 4 case reports to date. We report a case of bilateral gluteal compartment syndrome after total hip arthroplasty under epidural anesthesia and discuss its management. PMID:16376268

Somayaji, H Sadashiva; Hassan, A N A; Reddy, K; Heatley, F W

2005-12-01

27

Congenital bilateral perislyvian syndrome: case report and review of literature.  

PubMed

Congenital bilateral Perislyvian syndrome (CBPS) is a rare structural malformation of the brain in which the underlying anomaly is polymicrogyria. Polymicrogyria is a malformation of cortical development that is characterized by abnormal arrangement and excessive folding of cerebral cortical cell layers, often with fusion of the gyral surfaces, which can be focal or regional or involve the whole cortical mantle. Clinical manifestations depend upon the anatomical region of the brain involved. We report a case of 7-year-old male child with magnetic resonance imaging findings of CBPS. PMID:24404535

Gowda, Anand K; Mane, Ranoji Shivaji; Kumar, Ashok

2013-10-01

28

Non-syndromic bilateral dentigerous cysts associated with permanent second premolars  

PubMed Central

The dentigerous cyst is one of the most common developmental odontogenic cysts in the jaw. Occurrence of the bilateral dentigerous cyst is uncommon, and frequently associated with syndromes like basal cell nevus syndrome or cleidocranial dysplasia. There are few reports on the presence of bilateral dentigerous cyst in nonsyndromic patients, and most of these are associated with first and third molars. The reported case in this paper is bilateral dentigerous cysts associated with mandibular permanent second premolars, in the absence of any signs of syndrome. To our knowledge bilateral dentigerous cysts in these locations have not been previously reported.

Shirazian, Shiva; Agha-Hosseini, Farzaneh

2011-01-01

29

Bilateral Type-I Duane Syndrome with Multiple Anamolies: A Case Report  

PubMed Central

The Duane syndrome is a strabismus syndrome which is characterized by congenital non-progressive horizontal ophthalmoplegia which primarily affects the abducens nerve. Approximately 70% of the individuals with the Duane syndrome have an isolated disease. We have described here, a case of bilateral Duane syndrome with associated anamolies.

Varma, Chaitanya; Aroor, Shrikiran; Mundkur, Suneel C; Annamalai, Karthick

2012-01-01

30

Coexisting first and bilateral second branchial fistulas in a child with nonfamilial branchio-otic syndrome.  

PubMed

We describe what we believe is only the third reported case of coexisting first and bilateral second branchial fistulas associated with nonfamilial branchio-otic syndrome. The patient was a 6-year-old girl who presented with bilaterally draining anterior neck puncta, a preauricular sinus, and moderately severe bilateral hearing loss. She had no family history of branchial anomalies. Compared with branchial cysts and sinuses, branchial fistulas are rare. Even more rare are bilateral second branchial fistulas coexisting with first branchial anomalies, as only 10 cases have been previously reported in the English-language literature. Of these 10 cases, 5 were associated with either branchio-otic syndrome or branchio-oto-renal syndrome; 2 patients had familial branchio-otic syndrome, 2 had nonfamilial branchio-otic syndrome, and 1 had nonfamilial branchio-oto-renal syndrome. PMID:23904306

Lapeña, Jose F; Jimena, Genilou Liv M

2013-07-01

31

Laparoscopic detorsion for bilateral ovarian torsion in a singleton pregnancy with spontaneous ovarian hyperstimulation syndrome  

PubMed Central

A 26-year-old primigravida with a singleton pregnancy of 9 weeks gestation presented with severe lower abdominal pain, following spontaneous hyperstimulation of the ovaries in a natural conception. Emergency laparoscopy was done and bilateral ovarian torsion with retained vascularity was noted. Bilateral detorsion with ovarian puncture and ovariopexy was performed. A review of international literature suggests that this is the first case reported with bilateral ovarian torsion following spontaneous ovarian hyperstimulation syndrome in a singleton pregnancy.

Munshi, Sujal; Patel, Azadeh; Banker, Manish; Patel, Pravin

2014-01-01

32

Thrombocytopenia and absent radii (TAR) syndrome associated with bilateral congenital cataract: a case report  

PubMed Central

Introduction Thrombocytopenia with absent radii is a rare congenital defect with hypomegakaryocytic thrombocytopenia and bilateral radial aplasia that may have additional anomalies. We report the case of a girl baby with thrombocytopenia and absent radii syndrome and bilateral congenital cataract. This anomaly has not been previously reported in the children of a non- consanguineous marriage. Case presentation This case report describes a two-day-old girl baby of Arab origin with thrombocytopenia and absent radii syndrome and bilateral congenital cataract. Conclusions This report describes a finding of bilateral congenital cataract associated with thrombocytopenia and absent radii syndrome that has been reported only once before in the literature. This case report highlights a new ocular manifestation in one of the bone marrow failure syndromes.

2012-01-01

33

Compound heterozygosity in GPR56 with bilateral frontoparietal polymicrogyria.  

PubMed

Polymicrogyria is caused by a diverse etiology, one of which is gene mutation. At present, only one gene (GPR56) is known to cause polymicrogyria, which leads to a distinctive phenotype termed bilateral frontoparietal polymicrogyria (BFPP). BFPP is an autosomal recessive inherited human brain malformation with abnormal cortical lamination. Here, we identified compound heterozygous GPR56 mutations in a patient with BFPP. The proband was a Japanese female born from non-consanguineous parents. She presented with mental retardation, developmental motor delay, epilepsy exhibiting the feature of Lennox-Gastaut syndrome, exotropia, bilateral polymicrogyria with a relatively spared perisylvian region, bilateral patchy-white-matter MRI signal changes, and hypoplastic pontine basis. GPR56 sequence analysis revealed a c.107G>A substitution leading to a p.S36N, and a c.113G>A leading to a p.R38Q. Although affected individuals with compound heterozygosity in GPR56 have not been previously described, we presume that compound heterozygosity of these two mutations in a ligand binding domain within the extracellular N-terminus of protein could result in BFPP. In addition, we observed unusually less involvement of perisylvian cortex for polymicrogyria, and Lennox-Gastaut syndrome for epilepsy, which are likely common features in patients with BFPP caused by GPR56 mutations. PMID:23981349

Fujii, Yuji; Ishikawa, Nobutsune; Kobayashi, Yoshiyuki; Kobayashi, Masao; Kato, Mitsuhiro

2014-06-01

34

Bilateral Laparoscopic Gonadectomy in a Patient With Complete Androgen Insensitivity Syndrome and Bilateral Sertoli-Leydig Cell Tumor: A Case Report and Brief Review of the Literature  

PubMed Central

Introduction: Complete androgen insensitivity syndrome (previously called testicular feminization) is specified by a 46 XY karyotype and negative sex chromatin, bilateral undescended testes, female genitalia appearance, and lack of mullerian derivatives. Case Presentation: A 28-year-old woman with complete (severe) androgen resistance underwent prophylactic laparoscopic bilateral gonadectomy because of the eventually increased risk of gonadal malignancy. Although the gonads appeared grossly normal, microscopic examination revealed bilateral well differentiated sertoli–leydig cell tumor (SLCT). Discussion: Our Medline search revealed that this is the first reported case of bilateral sertoli–leydig cell tumor (SLCT) in androgen insensitivity syndrome.

Asl Zare, Mohammad; Kalantari, Mahmood Reza; Asadpour, Amir Abbas; Kamalati, Ali

2014-01-01

35

Non-syndrome patient with bilateral supernumerary teeth: Case report and 9-year follow-up  

PubMed Central

The presence of supernumerary teeth, also known as hyperdontia, is the condition of having teeth in addition to the regular number of teeth. The occurrence of multiple supernumerary teeth is often found in association with syndromes such as Gardner syndrome, Anderson-Fabry disease, Ellis-van Creveld syndrome, Ehlers-Danlos syndrome, incontinentia pigmenti, and Tricho-rhino-phalangeal syndrome. Only a few examples of nonsyndromic multiple supernumerary teeth have been reported in literature. In this report, we present the unusual case and 9-year follow up of a non-syndrome female patient with bilateral supernumerary teeth that occurred with an interval of several years.

Akgun, Ozlem Marti; Sabuncuoglu, Fidan; Altug, Ayberk; Altun, Ceyhan

2013-01-01

36

Non-syndrome patient with bilateral supernumerary teeth: Case report and 9-year follow-up.  

PubMed

The presence of supernumerary teeth, also known as hyperdontia, is the condition of having teeth in addition to the regular number of teeth. The occurrence of multiple supernumerary teeth is often found in association with syndromes such as Gardner syndrome, Anderson-Fabry disease, Ellis-van Creveld syndrome, Ehlers-Danlos syndrome, incontinentia pigmenti, and Tricho-rhino-phalangeal syndrome. Only a few examples of nonsyndromic multiple supernumerary teeth have been reported in literature. In this report, we present the unusual case and 9-year follow up of a non-syndrome female patient with bilateral supernumerary teeth that occurred with an interval of several years. PMID:23408107

Akgun, Ozlem Marti; Sabuncuoglu, Fidan; Altug, Ayberk; Altun, Ceyhan

2013-01-01

37

Bilateral squamosal suture synostosis: A rare form of isolated craniosynostosis in Crouzon syndrome  

PubMed Central

Craniosynostosis is a pathologic condition which is characterized by the premature fusion of cranial sutures. It may occur alone or in association with other anomalies making up various syndromes. Crouzon syndrome is the most common craniosynostosis syndrome. Bicoronal sutures fusion is most commonly involved in Crouzon syndrome. There have only been a handful of cases of squamosal suture synostosis described in the surgery literature with the few ones described in Crouzon syndrome associated with other types of craniosynostosis. To the best of our knowledge, we are presenting the first case of isolated bilateral squamosal suture synostosis in a patient with Crouzon syndrome in a radiology journal with emphasis on its radiological appearance.

Tandon, Yasmeen K; Rubin, Michael; Kahlifa, Mohamed; Doumit, Gaby; Naffaa, Lena

2014-01-01

38

Bilateral compartment syndrome of the anterior thigh following functional fitness exercises: a case report.  

PubMed

We present a case of delayed, acute bilateral exertional compartment syndrome of the anterior thigh induced by callisthenic exercise. Symptoms consisted of pain out of proportion to examination findings, inability to ambulate, and severe pain with knee flexion. Treatment consisted of bilateral thigh fasciotomies and supportive therapy for concomitant rhabdomyolysis. Full strength, range of motion, and return to all military duties were achieved by 4 months postinjury. PMID:22934383

McDonald, Lucas S; Mitchell, Ronald J; Deaton, Travis G

2012-08-01

39

Simultaneous isolated bilateral superior orbital vein thrombosis as a presenting feature of antiphospholipid syndrome.  

PubMed

Abstract Purpose: To report a rare case of isolated bilateral simulataneous superior ophthalmic vein occlusions secondary to antiphospholipid syndrome. Case Report: We report the case of a 77-year-old female who presented with a one week history of right relative proptosis, conjunctival injection and optic disc swelling. Magnetic resonance imaging revealed bilateral orbital masses which were diagnosed as superior ophthalmic vein occlusion. The patient was anticoagulated and the lesions resolved completely. System cardiovascular and haematological work-up revealed the patient was suffering from anti-phospholipid syndrome. Conclusion: Although superior ophthalmic vein occlusion is rare, it is an important differential as orbital biopsy is to be avoided. This is only the second reported case secondary to antiphospholipid syndrome and the first such case with bilateral occlusions. PMID:24568210

Idrees, Zubair; Dooley, Ian; Jackson, Aoife; Roche, Claire; Fahy, Gerry

2014-06-01

40

[Compartment syndrome in bilateral lower legs after total cystectomy: a case report].  

PubMed

We report a case of compartment syndrome in bilateral lower legs after total cystectomy with urethrectomy and ileal conduit diversion. A 64-year-old man who had diabetes mellitus for 20 years underwent an operation for invasive bladder cancer. He was placed in the lithotomy position and both lower legs were protected with an elastic stocking and intermittent pneumatic compression for prevention of deep vein thrombosis during the operation. Seven hours postoperatively, he complained of bilateral calf pain. Eleven hours postoperatively, skin redness, swelling, movement and sensory disorder of bilateral lower legs were found. Contrasting computed tomography (CT) of lower legs showed the swelling of bilateral soleus muscles and gastrocnemius muscles without any contrasting effect. Creatinine phosphokinase (CPK) increased to 46, 740 IU/l and the intramuscular pressure was 50 mmHg. He was diagnosed with compartment syndrome, in bilateral lower legs and emergent fasciotomy was performed. Bilateral calf pain was improved immediately after fasciotomy and could walk on his own after rehabilitation. Lower leg compartment syndrome is an uncommon disease but may require lower leg amputation or result in death if the treatment is delayed. Urologists should recognize this disease as a complication after prolonged operation in the lithotomy position. PMID:21412041

Sumiyoshi, Takayuki; Utsunomiya, Noriaki; Segawa, Takehiko; Muguruma, Koei; Ichikawa, Koichi; Kawakita, Mutsushi

2011-02-01

41

Cerebral fat embolism syndrome after simultaneous bilateral total knee arthroplasty: a case series.  

PubMed

Total knee arthroplasty (TKA), particularly when performed as a simultaneous bilateral procedure, theoretically increases the risk for entry of fat globules into the blood stream. The frequency of cerebral fat embolism syndrome (CFES) was retrospectively investigated among 2345 simultaneous bilateral TKA procedures performed from August 2006 to May 2010. During that period, 9 patients presented with neurologic deficits after surgery and underwent brain magnetic resonance imaging. For identification of CFES among them, we used both magnetic resonance imaging findings and clinical criteria modified from the original one of Gurd and Wilson (J Bone Joint Surg Br 1974; 56B:408). Four patients fulfilled the modified criteria. The overall incidence of CFES occurring after simultaneous bilateral TKA was 0.17%. Cerebral fat embolism syndrome should be ruled out, although rare, in patients who present with neurologic impairment after TKA. PMID:21820848

Lee, Su Chan; Yoon, Ji Yeol; Nam, Chang Hyun; Kim, Taek Keun; Jung, Kwang Am; Lee, Dong Won

2012-03-01

42

Trigeminal sensory neuropathy and bilateral carpal tunnel syndrome: the initial manifestation of mixed connective tissue disease  

Microsoft Academic Search

A patient is described who devloped a bilateral carpal tunnel syndrome and a trigeminal sensory neuropathy as the initial manifestations of mixed connective tissue disease. Either condition can occur in other connective tissue diseases, but this combination has not been reported previously in mixed connective tissue disease, in which, however, trigeminal sensory neuropathy is frequently seen.

F M Vincent; R N Van Houzen

1980-01-01

43

Massive bilateral phaeochromocytomas in association with Von Hippel Lindau syndrome in pregnancy.  

PubMed

We report a rare case of large bilateral phaeochromocytomas in pregnancy, found coincidentally by ultrasonography at 26 weeks' gestation, in a woman with a family history of Von Hippel Lindau syndrome. Further, we report maternal and fetal serum and amniotic fluid phenoxybenzamine levels from this case. PMID:10554962

Kothari, A; Bethune, M; Manwaring, J; Astley, N; Wallace, E

1999-08-01

44

Cerebellar Ataxia with Bilateral Vestibulopathy: Description of a Syndrome and Its Characteristic Clinical Sign  

ERIC Educational Resources Information Center

We report four patients with the syndrome of cerebellar ataxia with bilateral vestibulopathy (CABV) and, using search coil oculography, we validate its characteristic clinical sign, namely impairment of the visually enhanced vestibulo-ocular reflex (VVOR) or doll's head reflex. In our four patients, CABV began in the sixth decade of life; they are…

Migliaccio, Americo A.; Halmagyi, G. Michael; McGarvie, Leigh A.; Cremer, Phillip D.

2004-01-01

45

Bilateral anterior compartment syndrome after routine coronary artery bypass surgery and severe hypothyroidism.  

PubMed

Compartment syndrome is a very rare complication of coronary artery bypass grafting and previously it has only been described unilaterally. We describe the development of compartment syndrome in bilateral anterior compartments of the lower leg after vein harvest for coronary artery bypass grafting. We describe a series of predisposing factors contributing to this condition and its delayed diagnosis, including severe undiagnosed hypothyroidism. We advise a high index of suspicion in patients postvein harvest and recommend thyroid function testing for all patients who have compartment syndrome develop. PMID:20868840

Mills, Jane; Pretorius, Victor; Lording, Tim; Hardikar, Ashutosh; Murton, Mark

2010-10-01

46

Weaver syndrome associated with bilateral congenital hip and unilateral subtalar dislocation  

PubMed Central

Background: Weaver syndrome is a congenital paediatric syndrome characterized by mental, respiratory and musculoskeletal manifestations. The coexisting deformities of the skull, the face, fingers and toes are typical. We report a case of a girl with Weaver syndrome associated with rare bilateral congenital dislocation of the hips associated with congenital hypoplastic talus and subtalar dislocation of her ankle joint. Case Report: A 3-year old girl was admitted in our department with typical manifestations of Weaver syndrome, associated with congenital dislocation of bilateral hips, hypoplastic talus and subtalar dislocation of her right ankle. She was in pain while standing upright and incapable of independent walking. Both hips were treated operatively with open reduction and bilateral iliac osteotomy. Two years afterwards she had an open reduction of her talus and extraarticular arthrodesis of her subtalar joint in her right ankle. Six years postoperatively after the hip operations and four years after the ankle operation the girl is ambulant with a painless independent and unaided walking with a mild limp and full range of movements in all the operated joints. Conclusions: We suggest that children with Weaver syndrome and disabling musculosceletal deformities, particularly affecting their ability to stand up and walk should be treated early, before bone maturity, in order to achieve the best potential musculoskeletal as well as developmental outcome.

Mikalef, P; Beslikas, T; Gigis, I; Bisbinas, I; Papageorgiou, T; Christoforides, I

2010-01-01

47

[Mucosynechial conjunctivitis and bilateral corneal ulcers in Lyell's syndrome].  

PubMed

The patient, aged 44, presented fever and chills with altered general condition and received an analgesic-antipyretic treatment with salicylic acid and pyrazolon derivatives. A bullous eruption that followed was labelled incipient Lyell syndrome. Both cornea presented ulcers with a tendency to perforation and mucosynechial conjunctivitis. Pathogenic Staphylococcus albus was isolated from the conjunctival secretion. The ocular phenomena improved under mydriatic treatment and trophic medication. Lyell's syndrome is the result of a severe medicamentous toxemia. Stress is laid on the role of Staphylococcus aureus, phage type 71, in the process of epidermal necrosis with detachment of the epithelium and ulceration of the mucosa. PMID:2533371

Talea, L

1989-01-01

48

Bilateral Radial Artery Aneurysms in the Anatomical Snuff Box Seen in Marfan Syndrome Patient: Case Report and Literature Review  

PubMed Central

We describe the first Marfan syndrome case of non-traumatic bilateral radial artery aneurysms in the anatomical snuff box. A 74-year-old woman with Marfan syndrome had a pulsatile mass in her bilateral anatomical snuff box. The color Doppler ultrasonography showed an aneurysm of radial artery located in the bilateral anatomical snuff box. Resection of the right radial artery aneurysm was completed without complications. Histopathological analysis showed a true aneurysm with atherosclerotic changes in the arterial wall. We review the literature on non-traumatic or bilateral radial artery aneurysm in the anatomical snuff box, and discuss the clinical presentation and surgical management.

Matsuno, Yukihiro; Imaizumi, Matsuhisa; Mori, Yoshio; Iwata, Hitoshi; Takiya, Hiroshi

2010-01-01

49

A unique case of tessier 30 and bilateral cleft lip and palate: a new clinical syndrome?  

PubMed

Background : Tessier 30 cleft is rare and sparsely reported in the literature. A unique case of an infant with a Tessier 30 cleft, bilateral cleft lip and palate, and other anomalies is presented. In addition to craniofacial anomalies, he had cardiac, gastrointestinal, and genitourinal defects. The constellation of these findings suggests the possibility of a new clinical syndrome. We present these findings and postoperative results following surgical treatment. Patient and Methods : A 37-week gestational male infant with multiple congenital anomalies is presented. Findings on clinical exam were notable for Tessier 30 median mandibular cleft, bilateral cleft of the lip and palate, and bifid tongue. Further workup revealed levocardia, perimembranous moderate-to-large ventricular septal defect, patent foramen ovale, double outlet right ventricle, intestinal malrotation, and bilateral undescended testicles. There were no extremity anomalies, and cytogenetic studies for 22q deletion were negative. Results : The preoperative, intraoperative, and postoperative findings and images are discussed. Conclusion : We present a unique case of a child with a Tessier 30 cleft associated with bilateral cleft lip and palate in the absence of intraoral masses or limb anomalies. Previous reports of median facial clefts have occurred either in the presence of intraoral hamartomas, suggesting the palatal defects are a result of sequence abnormalities, or in association with extremity findings consistent with the spectrum of orofaciodigital syndrome. The case we present is distinct and may represent a new clinical syndrome. PMID:22034959

Adetayo, Oluwaseun A; Martin, Mark C

2013-01-01

50

Bilateral striatal necrosis in two subjects with Aicardi-Goutières syndrome due to mutations in ADAR1 (AGS6).  

PubMed

Aicardi-Goutières syndrome (AGS) is a genetic inflammatory disease. The classic neuroradiological picture mimics that of congenital infections in that Aicardi-Goutières syndrome is characterized by leukoencephalopathy, brain atrophy and intracranial calcifications. To date, bilateral striatal necrosis has not been reported in patients with AGS. We report on two patients with clinical diagnosis of Aicardi-Goutières syndrome in which brain MRI and CT scans demonstrated bilateral striatal necrosis. The diagnosis of Aicardi-Goutières syndrome in these two patients was genetically confirmed after the recent discovery that mutations in the ADAR1 (AGS6) gene may cause Aicardi-Goutières syndrome. This is the first report of bilateral striatal necrosis in association with Aicardi-Goutières syndrome. These results expand the neuroradiological phenotype of Aicardi-Goutières syndrome. PMID:24376015

La Piana, Roberta; Uggetti, Carla; Olivieri, Ivana; Tonduti, Davide; Balottin, Umberto; Fazzi, Elisa; Orcesi, Simona

2014-03-01

51

Long-term follow-up after bilateral Artisan aphakia intraocular lens implantation in two children with Marfan syndrome.  

PubMed

We present two patients with Marfan syndrome and bilateral crystalline lens dislocation who underwent bilateral lens extraction and Artisan aphakia intraocular lens (IOL) implantation. With a follow-up of more than 12 years, we found a good visual outcome, no serious IOL-related complications, and endothelial cell densities within the expected range for eyes without cataract surgery. PMID:22370675

Sminia, Marije L; Odenthal, Monica T P; Prick, Liesbeth J J M; Cobben, Jan M; Mourits, Maarten P; Völker-Dieben, Hennie J

2012-02-01

52

Bilateral pulmonary artery banding for hypoplastic left heart syndrome and related anomalies  

Microsoft Academic Search

Objective  Bilateral pulmonary artery banding is considered an option for initial palliation in high-risk patients with hypoplastic left\\u000a heart syndrome or related anomalies. However, there are potential interim morbidities, including ductal constriction, compromised\\u000a growth of the ascending aorta, and pulmonary artery stenosis at the banding site. In addition, there are still controversies\\u000a regarding the optimal timing and choice of the second-stage

Takashi Sasaki; Yukihiro Takahashi; Makoto Ando; Naoki Wada; Yasuhiro Kawase; Hiroshi Seki

2008-01-01

53

Early results of bilateral pulmonary artery banding for hypoplastic left heart syndrome  

Microsoft Academic Search

Objective: To compare the haemodynamics and perioperative course of initial palliation with bilateral pulmonary artery banding (PAB) and the Norwood procedure. Methods: Between April 2004 and December 2007, 43 consecutive children with hypoplastic left heart syndrome (HLHS) or a variant underwent initial palliation (PAB, n=18; Norwood, n=25). Clinical perioperative data were analysed. In the PAB group, lipo-prostaglandin E1 administration was

Takahisa Sakurai; Hideaki Kado; Toshihide Nakano; Kazuhiro Hinokiyama; Akira Shiose; Masaki Kajimoto; Kunihiko Joo; Yuichi Ueda

2009-01-01

54

Bronchial fistula after lobar size reduction for bilateral lung transplantation in Kartagener's syndrome: a surgical challenge  

PubMed Central

Bilateral lung transplantation was performed in a 52-year old man with end-stage Kartagener's syndrome. A postimplantation right lower lobectomy was required for volume reduction and dextrocardia. A bronchial fistula developed with an intractable colonized residual pleural cavity. Closure was obtained successfully with multiple-stage procedures including decortication, muscle flap and an open-window thoracostomy without modification of the immunosuppressive protocol.

Brioude, Geoffrey; D'journo, Xavier Benoit; Reynaud-Gaubert, Martine; Thomas, Pascal Alexandre

2013-01-01

55

Bilateral De Quervain Syndrome after Aromatase Inhibitor Administration: A Case Report and Review of the Literature  

PubMed Central

Aromatase inhibitors are widely used as one of the main treatment options of both early and advanced hormone receptor-positive breast cancer in postmenopausal women. Unfortunately, musculoskeletal symptoms are often presented in patients treated with aromatase inhibitors (AIs), and, although the pathogenesis is unknown, postulated mechanisms have been described. Herein, to our knowledge, we present the first report of bilateral De Quervain syndrome related with AIs therapy with a review of the relevant literature.

Papadimitriou, Konstantinos; Kountourakis, Panteleimon; Morakis, Emmanouil; Vassiliou, Vassilios; Barbounis, Vasileios; Ardavanis, Alexandros

2012-01-01

56

Bilateral Lung Transplantation in a Patient With Vascular Ehlers-Danlos Syndrome.  

PubMed

We describe the case of a 29-year-old woman with end-stage chronic obstructive pulmonary disease secondary to vascular Ehlers-Danlos syndrome. Because of critical deterioration, respiratory arrest, and complete lung failure, she required urgent implantation of a venovenous extracorporeal membrane oxygenator as a bridge to lung transplantation. After 6 days of extracorporeal life support, a successful bilateral sequential lung transplantation was performed. This is the first case of lung transplantation in a patient with a diagnosis of chronic obstructive pulmonary disease secondary to Ehlers-Danlos syndrome. PMID:24792277

García Sáez, Diana; Mohite, Prashant N; Zych, Bartlomiej; Sabashnikov, Anton; Moza, Ajay; Carby, Martin; Simon, André R

2014-05-01

57

Posterior fossa surgery complicated by a pseudomeningocele, bilateral subdural hygromata and cerebellar cognitive affective syndrome.  

PubMed

We report the case of a 51-year-old woman who underwent excision of a left postero-inferior, para-vermian cerebellar hemangioblastoma and foramen magnum decompression for an associated acquired Chiari I malformation. Two weeks postoperatively she developed a pseudomeningocele, bilateral supra- and infratentorial subdural hygromata, and a clinical disorder compatible with the cerebellar cognitive affective syndrome. There was no response to drainage of the left supratentorial collection. CSF aspiration from a pseudomeningocele lead to resolution of her syndrome and the subdural effusions. The relationships between the subdural hygromas, pseudomeningocele, location of the lesion within the cerebellum, cerebellar oedema, and the cause of her cognitive-affective syndrome are discussed. PMID:18224531

Stavrinos, N G; Taylor, R; Rowe, A; Whittle, I R

2008-02-01

58

Bilateral and Simultaneous Central Retinal Vein Occlusion in a Patient with Obstructive Sleep Apnea Syndrome  

PubMed Central

Purpose To describe a case of bilateral and simultaneous central retinal vein occlusion (RVO) in a young patient diagnosed with obstructive sleep apnea syndrome (OSAS). Case Report A 38-year-old man with morbid obesity and daytime sleepiness presented with a history of bilateral vision loss. His visual acuity (VA) was hand movements, and fundus examination (FE) revealed bilateral central RVO. General medical examination revealed untreated hypertension and type II respiratory failure. Laboratory tests for thrombophilia showed increased hematocrit (59%) and high levels of fibrinogen and C-reactive protein. Other causes of congenital and acquired hypercoagulability were ruled out. Pathologic polysomnography led to the diagnosis of OSAS. The patient was treated with antihypertensive drugs and continuous positive air pressure. In addition, he received intravitreal ranibizumab. At 10 months after presentation, his VA was no light perception in the right eye and hand movements in the left eye. FE revealed bilateral retinal and optic nerve atrophy, and the occurrence of a nonarteritic anterior ischemic neuropathy in the right eye was considered.

Govetto, Andrea; Dominguez, Ramon; Rojas, Laura; Pereiro, Maria; Lorente, Ramon

2014-01-01

59

Non-Syndromic Bilateral Supplemental Maxillary Lateral Incisors: A Rare Case  

PubMed Central

Supernumerary teeth or hyperdontia are defined as the teeth that exceeds the normal dental formula, regardless of their location and morphology. A supernumerary tooth is a developmental anomaly and it has been argued to arise from multiple aetiologies. Supernumerary teeth with orthodox shapes and sizes, that resemble the normal dentition are called ‘supplemental teeth’. The supplemental teeth are often overlooked because of their normal shapes and sizes. Supplemental lateral incisors are rare and bilateral cases are even rarer, with only two cases being reported in the literature till date. Therefore, we are describing a rare case of non–syndromic, bilateral, supplemental, maxillary, lateral incisors and we have discussed the management of supernumerary teeth.

Nagpal, Ajay; Hans, Manoj Kumar; Shetty, Shashit; Kaur, Navpreet; Kumar, Sunil

2013-01-01

60

Non-syndromic bilateral supplemental maxillary lateral incisors: a rare case.  

PubMed

Supernumerary teeth or hyperdontia are defined as the teeth that exceeds the normal dental formula, regardless of their location and morphology. A supernumerary tooth is a developmental anomaly and it has been argued to arise from multiple aetiologies. Supernumerary teeth with orthodox shapes and sizes, that resemble the normal dentition are called 'supplemental teeth'. The supplemental teeth are often overlooked because of their normal shapes and sizes. Supplemental lateral incisors are rare and bilateral cases are even rarer, with only two cases being reported in the literature till date. Therefore, we are describing a rare case of non-syndromic, bilateral, supplemental, maxillary, lateral incisors and we have discussed the management of supernumerary teeth. PMID:24086923

Nagpal, Ajay; Hans, Manoj Kumar; Shetty, Shashit; Kaur, Navpreet; Kumar, Sunil

2013-08-01

61

Bilateral Internal Carotid Artery Occlusion Associated with the Antiphospholipid Antibody Syndrome  

PubMed Central

A 39-year-old woman presented with a right-hemispheric stroke 1 year after she had suffered a left-hemispheric stroke. Her diagnostic workup was notable for bilateral occlusions of the internal carotid arteries at their origins and a positive lupus anticoagulant antibody test. There was no evidence of carotid dissection or another identifiable cause for her carotid occlusions. These findings suggest that the antiphospholipid antibody syndrome may be implicated in the pathological changes that resulted in occlusions of the extracranial internal carotid arteries. Young stroke patients who present with unexplained internal carotid artery occlusions may benefit from testing for the presence of antiphospholipid antibodies.

Anand, Pria; Mann, Sharan K.; Fischbein, Nancy J.; Lansberg, Maarten G.

2014-01-01

62

Bilateral stellate ganglion blockade for recalcitrant oral pain from Burning Mouth Syndrome: a case report.  

PubMed

Burning Mouth Syndrome (BMS) is a chronic painful disorder characterized by unremitting bilateral burning oral pain often associated with taste abnormalities and complaints of dry mouth. The diagnosis is made by history and symptom presentation in the absence of an identifiable cause or oral lesion. It is commonly seen in perimenopausal women but is also seen in men, and is considered a small-fiber neuropathy. Management can be challenging and few effective treatments are available. This article presents a case report of stellate ganglion blockade as a treatment for recalcitrant pain from BMS. PMID:24822241

Walega, David R; Smith, Clark; Epstein, Joel B

2014-01-01

63

Thrombolysis for central venous occlusion causing bilateral chylothorax in a patient with down syndrome.  

PubMed

A 20-year-old woman with Down syndrome (trisomy 21) and acute lymphoblastic leukemia presented with severe respiratory compromise secondary to bilateral chylothorax as a result of central venous thrombosis and extensive upper-limb deep venous thrombosis. The chylothorax was successfully managed by catheter-directed thrombolysis and angioplasty of the venous occlusions. The development of venous thrombosis was likely to have been multifactorial. It is recognized that there is an increased incidence of congenital lymphatic anomalies in Down syndrome, which may have been a contributing factor in the development of chylothorax in this patient. This report illustrates the angiographic findings, demonstrates the successful vascular recanalization, and discusses the etiology and management of central venous thrombosis and chylothorax. The case is also presented to contribute to the expanding evidence in support of catheter-directed venous thrombolysis in selected clinical circumstances. PMID:15126664

Manghat, Nathan; Hancock, John; Walsh, Mark; Puckett, Mark; Noble, Richard; Travis, Simon

2004-05-01

64

Bilateral abducens palsy in a case of cytomegalovirus-associated Guillain-Barré syndrome.  

PubMed

Ophthalmoparesis in cytomegalovirus (CMV)-associated Guillain-Barré syndrome (GBS) is rare. We treated a 37-year-old woman with CMV-GBS who presented with an acute onset of generalized weakness and numbness in the extremities, followed by facial diplegia, which led to mechanical ventilation. She had increased IgM and IgG-type antibodies against CMV in the serum and increased IgM-type serum anti-GM2 ganglioside antibody was also noted, whereas anti-GQ1b ganglioside antibody was not found. She then developed the syndrome of inappropriate secretion of antidiuretic hormone (SIADH). After recovery of consciousness due to SIADH, she exhibited bilateral abducens palsy, together with the recurrence of limb weakness and facial diplegia. Her neurological signs gradually recovered after high-dose intravenous administrations of immunoglobulin. CMV infection should be listed in the differential diagnosis of GBS patients who present with ophthalmoparesis. PMID:21833717

Kishi, Masahiko; Sakakibara, Ryuji; Ogawa, Emina; Tateno, Fuyuki; Takahashi, Osamu; Koga, Michiaki

2011-12-01

65

'Eye is a window to the pulse': bilateral ocular ischaemic syndrome as a presenting manifestation of Takayasu arteritis.  

PubMed

A 22-year-old lady presented with gradually progressive visual loss and chronic pain in both the eyes of 3 months duration. The clinical examination and fundus fluorescein angiography were suggestive of bilateral ocular ischaemic syndrome. Her upper limb pulses were feeble and blood pressure was not recordable in the same. Carotid Doppler revealed a bilateral carotid artery occlusion. Systemic markers for inflammation were elevated. Diagnosis of Takayasu arteritis was made on the basis of clinical and laboratory findings. Takayasu arteritis should be considered in the differential diagnosis of ocular ischaemic syndrome, particularly in young Asian women. PMID:23632189

Shailaja, S; Vivek, G; Shetty, Ranjan; Kamath, Yogish

2013-01-01

66

Unilateral or bilateral vagotomy induces ovulation in both ovaries of rats with polycystic ovarian syndrome  

PubMed Central

Background Injecting estradiol valerate (EV) to pre-pubertal or adult female rat results in effects similar to those observed in women with polycystic ovarian syndrome (PCOS). One of the mechanisms involved in PCOS development is the hyperactivity of the sympathetic nervous system. In EV-induced PCOS rats, the unilateral sectioning of the superior ovarian nerve (SON) restores ovulation of the innervated ovary. This suggests that, in addition to the sympathetic innervation, other neural mechanisms are involved in the development/maintenance of PCOS. The aims of present study were analyze if the vagus nerve is one of the neural pathways participating in PCOS development. Methods Ten-day old rats were injected with EV dissolved in corn oil. At 24-days of age sham-surgery, unilateral, or bilateral sectioning of the vagus nerve (vagotomy) was performed on these rats. The animals were sacrificed at 90–92 days of age, when they presented vaginal estrous preceded by a pro-estrus smear. Results In EV-induced PCOS rats, unilateral or bilateral vagotomy restored ovulation in both ovaries. Follicle-stimulating hormone (FSH) levels in PCOS rats with unilateral or bilateral vagotomy were lower than in control rats. Conclusions This result suggests that in EV-induced PCOS rats the vagus nerve is a neural pathway participating in maintaining PCOS. The vagus nerve innervates the ovaries directly and indirectly through its synapsis in the celiac-superior-mesenteric ganglion, where the somas of neurons originating in the SON are located. Then, it is possible that vagotomy effects in EV-induced PCOS rats may be explained as a lack of communication between the central nervous system and the ovaries.

2013-01-01

67

Bilateral occipital calcification, epilepsy and coeliac disease: clinical and neuroimaging features of a new syndrome.  

PubMed Central

Twenty patients affected by bilateral occipital cortical-subcortical calcification (BOC) are described, 19 (95%) had epilepsy. In 8 of 16 cases studied, intestinal biopsy revealed coeliac disease. Fourteen patients had occipital partial epilepsy with a relatively benign outcome, while 4 patients were affected by a severe form of epilepsy, with very frequent, drug-resistant, generalised and partial seizures with mental deterioration. One patient had a single episode of convulsive status epilepticus at four months of age. The neurological examination was normal in all patients. CT showed flocculo-nodular, cortico-subcortical BOC, without enhancement and without lobar or hemispheric atrophy. MRI was normal. The clinical and neuroimaging features of these patients are different therefore from those with the Sturge-Weber Syndrome. The study confirms a high prevalence of coliac disease in patients with BOC, but the relationship between these two pathologies still needs to be clarified. Images

Magaudda, A; Dalla Bernardina, B; De Marco, P; Sfaello, Z; Longo, M; Colamaria, V; Daniele, O; Tortorella, G; Tata, M A; Di Perri, R

1993-01-01

68

Bilateral dysgerminoma in a patient with a previous diagnosis of Swyer syndrome.  

PubMed

A 16-year-old girl was referred to our center by her general physician because of primary amenorrhea. Her family history revealed an older sister with Swyer syndrome and gonadectomy at another institution. After thorough evaluation she received the same diagnosis, but unlike her sister, she refused gonadectomy. Four years later she presented with abdominal discomfort and a complex pelvic mass. She underwent exploratory laparotomy and histological examination revealed bilateral dysgerminoma without capsular invasion. The tumor was classified as stage IB. After surgery she underwent adjuvant chemotherapy with three cycles of BEP (bleomycin + etoposide + cisplatin). The present case emphasizes the importance of familial screening with a karyotype study in pure gonadal dysgenesis to prevent gonadal malignancy. PMID:22176344

Moreira, Ana Isabel Duarte Mendonça; Silva, José Carlos; Ferreira, Maria Soledade; Lanhoso, António

2012-02-01

69

High le fort I and bilateral split sagittal osteotomy in crouzon syndrome.  

PubMed

Crouzon syndrome is a rare, autosomal dominant disease from a fibroblast growth factor receptor 2 gene mutation, characterized by premature craniosynostosis, hypertelorism, orbital proptosis, psittichorina, hypoplastic maxilla, and mandibular prognathism. We present an adult 32-year-old Crouzon syndrome patient who underwent an elective High Le Fort I and bilateral split sagittal osteotomy for midface advancement with a background of jaw malocclusion and obstructive respiratory symptoms. The operation features a potential dynamic movement of the secured airway in the surgical field and close proximity to exposed ocular structures. Permissive hypotensive anesthesia was employed to improve the surgical field and reduce intraoperative blood loss and dose of long-acting opioids. He was extubated at the end of an uneventful surgery and was monitored in the high dependency overnight before he was discharged to the general ward. Perioperative issues include potential difficult airway management; ocular, auditory, and neurological injury prevention; surgery-specific anesthetic technique; and postoperative analgesia. Understanding the multisystemic issues facilitates the dynamic anesthetic management during surgery. Good communication among the multidisciplinary team is essential to ensure a successful operation and uneventful recovery. PMID:23714981

Tay, Yoong Chuan; Tan, Kian Hian; Yeow, Vincent Kok-Leng

2013-05-01

70

ACTH-Independent Cushing's Syndrome with Bilateral Micronodular Adrenal Hyperplasia and Ectopic Adrenocortical Adenoma  

PubMed Central

Context: Bilateral micronodular adrenal hyperplasia and ectopic adrenocortical adenoma are two rare causes of ACTH-independent Cushing’s syndrome. Objective: The aim of the study was to evaluate a 35-yr-old woman with ACTH-independent hypercortisolism associated with both micronodular adrenal hyperplasia and ectopic pararenal adrenocortical adenoma. Design and Setting: In vivo and in vitro studies were performed in a University Hospital Department and academic research laboratories. Intervention: Mutations of the PRKAR1A, PDE8B, and PDE11A genes were searched for in leukocytes and adrenocortical tissues. The ability of adrenal and adenoma tissues to synthesize cortisol was investigated by immunohistochemistry, quantitative PCR, and/or cell culture studies. Main Outcome Measure: Detection of 17?-hydroxylase and 21-hydroxylase immunoreactivities, quantification of CYP11B1 mRNA in adrenal and adenoma tissues, and measurement of cortisol levels in supernatants by radioimmunological assays were the main outcomes. Results: Histological examination of the adrenals revealed nonpigmented micronodular cortical hyperplasia associated with relative atrophy of internodular cortex. No genomic and/or somatic adrenal mutations of the PRKAR1A, PDE8B, and PDE11A genes were detected. 17?-Hydroxylase and 21-hydroxylase immunoreactivities as well as CYP11B1 mRNA were detected in adrenal and adenoma tissues. ACTH and dexamethasone activated cortisol secretion from adenoma cells. The stimulatory action of dexamethasone was mediated by a nongenomic effect involving the protein kinase A pathway. Conclusion: This case suggests that unknown molecular defects can favor both micronodular adrenal hyperplasia and ectopic adrenocortical adenoma associated with Cushing’s syndrome.

Louiset, Estelle; Gobet, Francoise; Libe, Rossella; Horvath, Anelia; Renouf, Sylvie; Cariou, Juliette; Rothenbuhler, Anya; Bertherat, Jerome; Clauser, Eric; Grise, Philippe; Stratakis, Constantine A.; Kuhn, Jean-Marc; Lefebvre, Herve

2010-01-01

71

[A male case of acute sarcoidosis with fever, polyarthralgia, erythema nodosum, and bilateral hilar lymphadenopathy: Löfgren's syndrome].  

PubMed

A 27-year-old man initially had low back pain and ankle arthralgia. He was admitted because fever, cough, and polyarthralgia developed and continued for three months. Chest X-ray and CT revealed bilateral hilar and mediastinal lymphadenopathy with pulmonary lesions. Furthermore, elevated serum-ACE level and noncaseating epitheloid cell granuloma obtained by TBLB confirmed the diagnosis of sarcoidosis. After hospitalization, erythema nodosum appeared and ocular involvement was demonstrated. As a result, this case fulfilled the criteria of Löfgren's syndrome (arthritis, erythema nodosum, and BHL). Löfgren's syndrome is not uncommon in European countries, but is extremely rare in Japan. So far, only six cases with Löfgren's syndrome were reported in Japan, and all were female cases. This is the first male case in Japan. Löfgren's syndrome is usually a self-limiting disease. We used steroids for this case and remission has been maintained after the beginning of the treatment for the past one year. PMID:16457340

Izumo, Mayu; Sekiya, Kiyoshi; Sakai, Toshihiko; Tojima, Hirokazu

2005-12-01

72

Bilateral Asynchronous Adrenocortical Adenoma in a Girl with Beckwith-Wiedemann Syndrome  

PubMed Central

We report a case of asynchronous occurrence of bilateral adrenocortical adenoma in a 13-yr-old girl with Beckwith-Wiedemann syndrome. A right virilizing adrenal adenoma was surgically removed at age 6, following clinical manifestation of virilization such as acne, voice change, clitoris hypertrophy and overgrowth. Histopathological examination of the resected specimen revealed an adrenocortical adenoma predominantly composed of eosinophilic tumor cells expressing all the steroidogenic enzymes. High serum levels of DHEA-S (6,380 ng/ml) and testosterone (547 ng/dl) were noted prior to the operation. Postoperative course was unremarkable. Menstruation started at age 11, with a regular interval. At the age of 13 yr old, a high serum level of DHEA-S (8,250 ng/ml) was detected. In contrast to the episode of virilization at age 6, however, the serum testosterone level was not so high (122 ng/dl), and no clinical symptoms of virilization were apparent. Abdominal ultrasonography demonstrated the presence of a left adrenocortical adenoma. Pathological examination of the resected specimen revealed a circumscribed and well encapsulated tumor with essentially the same histological features as the tumor previously removed, except that the tumor cells showed a more prominent morphological similarity to the fetal adrenal cortex and did not express 3? HSD. The absence of virilization at the second episode was due to the relatively low serum level of testosterone compared with that of DHEA-S.

Mizota, Michiyo; Tamada, Izumi; Hizukuri, Kazuko; Otsubo, Kiyoko; Arima, Siu; Kawano, Yoshifumi; Ono, Seigo; Hayashida, Yoshihiro; Kaji, Tatsuru; Takamatsu, Hideo; Sasano, Hironobu

2005-01-01

73

Bilateral stenosis of carotid siphon in Hutchinson-Gilford progeria syndrome.  

PubMed

Hutchinson-Gilford progeria syndrome (HGPS) is a rare premature aging disease, caused by a de novo mutation of lamin-A gene, LMNA G608G. Accumulation of abnormal lamin-A (progerin) compromises nuclear membrane integrity and results in the accelerated senescence. Affected patients show a typical feature of birdlike face, alopecia, sclerotic skin, loss of subcutaneous fat, and short stature with advancing years. Neonatal scleroderma is the first presentation, although early diagnosis is challenging. The leading cause of death is cardio-/cerebro-vascular accidents associated with atherosclerosis. However, not all findings may recapitulate the aging process. We herein report a 9-year-old Japanese male with HGPS who developed cerebral infarction. The genetic study of peripheral blood-derived DNA determined a heterozygous c.1824C>T mutation, p.G608G. Telomere length of lymphocytes was normal. Bilateral stenosis of carotid siphons was prominent, while systemic arteriosclerosis was unremarkable assessed by the ankle-brachial index, carotid ultrasound imaging and funduscopic study. HGPS patients have marked loss and functional defects in vascular smooth muscle cells, leading to the vulnerability to circulatory stress. Symmetrical stenosis of siphons might occur as a distinctive cerebral vasculopathy of HGPS, rather than simple vascular senescence. Peripheral blood study on LMNA G608G and telomere length could screen progerias in infancy for early therapeutic intervention. PMID:23141186

Narazaki, Ryo; Makimura, Mika; Sanefuji, Masafumi; Fukamachi, Shigeru; Akiyoshi, Hidetaka; So, Hidenori; Yamamura, Kenichiro; Doisaki, Sayoko; Kojima, Seiji; Ihara, Kenji; Hara, Toshiro; Ohga, Shouichi

2013-08-01

74

The Role of Left Perisylvian Cortical Regions in Spelling  

ERIC Educational Resources Information Center

In order to examine the role of left perisylvian cortex in spelling, 13 individuals with lesions in this area were administered a comprehensive spelling battery. Their spelling of regular words, irregular words, and nonwords was compared with that of individuals with extrasylvian damage involving left inferior temporo-occipital cortex and normal…

Henry, Maya L.; Beeson, Pelagie M.; Stark, Amy J.; Rapcsak, Steven Z.

2007-01-01

75

Club feet with congenital perisylvian polymicrogyria possibly due to bifocal ischemic damage of the neuraxis in utero.  

PubMed

Club foot is a common congenital deformity, for which a neurogenic process in utero has been proposed in some severe forms, but in most cases its cause remain uncertain. We report on four patients with an unilateral (three cases) or bilateral (one case) clubfoot and a bilateral perisylvian cortical dysplasia. All had severe dysarthria with mild mental retardation, epilepsy occurred in three cases. Direct evidence of fetal lesions of the spinal cord was occasionally present, such as signs of motor axonopathy in two cases analyzed by electrophysiological methods and syringomyelic cavitation at the thoracic level in one case. Even though the sensitivity of the investigations to demonstrate microcopic scars in the spinal cord remains weak, the presence of polymicrogyric rearrangements in the perisylvian cortex, known to proceed from a transient ischemic process occurring in the carotid territory during mid-gestation, strongly suggests that a similar mechanism occurred in the spinal cord. In fact, the foot deformity cannot be viewed as the consequence of lesions to brain regions that do not control the foot motility in the fetus. Extraneurological lesions such as jejunal atresia, possibly proceeding from localized vascular compromise, were also encountered. In one sibship, one sister was found to have a severe developmental anomaly of one foot, suggesting that genetic factors may be involved. PMID:15057985

Kammoun, Fatma; Tanguy, Alain; Boesplug-Tanguy, Odile; Bensahel, Henri; Khouri, Nejib; Landrieu, Pierre

2004-04-15

76

Colonic adenocarcinoma and bilateral malignant ovarian sex cord tumor with annular tubules in Peutz-Jeghers syndrome.  

PubMed

Peutz-Jeghers syndrome is characterized by multiple polyps throughout the gastrointestinal tract in association with mucocutaneous pigmentation. Although Peutz-Jeghers syndrome polyps are hamartomas, frequent association of this syndrome with both gastrointestinal and non-gastrointestinal tumours had led to reassessment of the cancer risk in this hereditary disorder. The most common gynaecological tumors in this syndrome are adenoma malignum of the uterine cervix and ovarian sex cord tumor, particularly sex cord tumor with annular tubules. The question of malignant change in a polyp or of the association of gastro intestinal carcinomas still discuss. The authors report a case of Peutz-Jeghers syndrome in a young patient who developed a colonic adenocarcinoma in a hamartomatous polyp together with an incidentally discovered bilateral malignant sex cord tumours. We discuss its association with certain benign and malignant tumors and the risk of rare complications of these hamartomatous polyps. Although malignant tumors are increasingly reported in association with the Peutz-Jeghers syndrome, to our knowledge, there have been no previous reports of such an association in the literature. PMID:15524052

Ayadi-Kaddour, A; Bouraoui, S; Bellil, K; Bellil, S; Kchir, N; Zitouna, M M; Haouet, S

2004-06-01

77

Antiphospholipid Antibody Syndrome Associated with Graves' Disease Presenting As Inferior Vena Cava Thrombosis with Bilateral Lower Limb DVT  

PubMed Central

We report a case of a 60-year-old lady who presented with bilateral lower limb swelling and a thyroid swelling with clinical features consistent with thyrotoxicosis. Investigations revealed the presence of a thrombus in bilateral external, internal iliac veins, and inferior vena cava extending up to its infrahepatic part. Hormone profile and radioiodine uptake scan confirmed the diagnosis of Graves’ disease. Further workup revealed the presence of antiphospholipid antibodies (confirmed after a repeat test at 12 weeks). The patient was treated with antithyroid drugs and anticoagulants. The patient improved with normalization of thyroid function and partial recanalization of the infrahepatic part of inferior vena cava. Hyperthyroidism has been implicated as a potential hypercoagulable state; however, the association of Graves’ disease with antiphospholipid antibody syndrome is limited to isolated case reports. This case highlights a new mechanism underlying hypercoagulability associated with Graves’ disease.

Jain, Ankur

2014-01-01

78

Antiphospholipid Antibody Syndrome Associated with Graves' Disease Presenting As Inferior Vena Cava Thrombosis with Bilateral Lower Limb DVT.  

PubMed

We report a case of a 60-year-old lady who presented with bilateral lower limb swelling and a thyroid swelling with clinical features consistent with thyrotoxicosis. Investigations revealed the presence of a thrombus in bilateral external, internal iliac veins, and inferior vena cava extending up to its infrahepatic part. Hormone profile and radioiodine uptake scan confirmed the diagnosis of Graves' disease. Further workup revealed the presence of antiphospholipid antibodies (confirmed after a repeat test at 12 weeks). The patient was treated with antithyroid drugs and anticoagulants. The patient improved with normalization of thyroid function and partial recanalization of the infrahepatic part of inferior vena cava. Hyperthyroidism has been implicated as a potential hypercoagulable state; however, the association of Graves' disease with antiphospholipid antibody syndrome is limited to isolated case reports. This case highlights a new mechanism underlying hypercoagulability associated with Graves' disease. PMID:24812529

Jain, Ankur

2014-01-01

79

Spontaneous Bilateral Corneal Perforation in Stevens- Johnsons Syndrome-A Challenge in Management  

PubMed Central

A 42-year-old man from Ghana presented with bilateral painful corneal perforations following ingestion of a sulphur-based antibiotic. Emergency bilateral penetrating keratoplasty was performed, with restoration of globe integrity. However, surgical complications arose such as non-healing epithelial defect, secondary infection, graft dehiscence, and mounting intraocular pressure. This case illustrates the challenges faced in managing corneal grafts in patients with already compromised ocular surfaces.

Md Noh, Umi Kalthum; Then, Kong Yong

2013-01-01

80

[A case of wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) syndrome with cerebellar ataxia and facial dysesthesia].  

PubMed

We report an 85-year-old man presenting with wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) syndrome with cerebellar ataxia and facial dysesthesia. He experienced an abrupt onset of double vision and exotropia of the right eye with unsteady gait and dysesthesia around upper lip. He was admitted to our hospital ten days after the onset of the double vision. On admission, he presented with WEBINO, left limb ataxia, and dysesthesia around upper lip on the right side. His exotropia was prominent on the right side. Diffusion weighted images of MRI revealed a high intensity lesion in the paramedian pontine tegmentum involving bilateral medial longitudinal fasciculus (MLF), consistent with acute ischemic lesion. Four months after the onset, the WEBINO persisted, without cerebellar ataxia and facial dysesthesia. Putative lesions of the WEBINO, cerebellar ataxia and facial dysesthesia were bilateral MLF, left superior cerebellar peduncle and trigeminothalamic tract, respectively, which were broader than the MRI lesion. Neurological examination is critical for evaluation of accurate ischemic area. PMID:24807275

Kadoya, Masato; Onoue, Hiroyuki; Kadoya, Akiko; Higashihara, Mana; Ikewaki, Katsunori; Kaida, Kenichi

2014-01-01

81

Pure sensory syndromes and post-stroke pain secondary to bilateral thalamic lacunar infarcts: a case report  

PubMed Central

Introduction Patients often complain about sensory symptoms that appear to the doctor as harmless, and reassurances are often given. Sensory strokes may easily be ignored. Case presentation A 48-year-old Caucasian woman with insulin-dependent diabetes and hyperlipidemia experienced symptoms that progressed within hours to a complete left-sided hemisensory syndrome. This was caused by a lacunar infarct in the ventral posterior tier nuclei of the right thalamus. A few days later she gradually developed an almost identical, but incomplete hemisensory syndrome on the opposite side caused by a corresponding lacune in the left thalamus. Severe persistent and paroxysmal pain on both sides of the body became disabling. Conclusion Small strokes only affecting the somatosensory system should not be underestimated. Neuropathic pain may result. Probably unique in the present case is the demonstration of bilateral thalamic pain secondary to two almost identical thalamic infarcts. Small vessel disease (microatheroma or lipohyalinosis) was the most likely cause of the lacunes. One can only speculate if there was an occlusion in two separate thalamic perforators, or in a single dominant artery supplying the bilateral thalami.

2012-01-01

82

Bone-anchored maxillary expansion and bilateral interoral mandibular distraction osteogenesis in adult with severe obstructive sleep apnea syndrome.  

PubMed

Severe obstructive sleep apnea syndrome (OSAS) threatens patients' lives. To solve ventilation problem, snoring, and avoid another orthognathic surgery for mandibular advancement, bone-anchored rapid maxillary expansion and bilateral interoral mandibular distraction osteogenesis were tried on a 20-year-old Chinese male patient with severe skeletal class II malocclusion and OSAS.The patient had polysomnography (apnea-hypopnea index 54.2), body mass index measurement (19.7 kg/m), and cephalometry before the treatment. Bone-anchored rapid maxillary expansion was performed for the correction of maxillary transverse and minor sagittal deficiency and the improvement of nasal airflow by decreasing nasal resistance. Bilateral interoral mandibular distraction osteogenesis was operated to lengthen the small, retruded mandible by 15 mm. Orthodontic treatment after the maxillary expansion and mandibular distraction osteogenesis can help obtain stable occlusion.The Epworth Sleepiness Scale, a questionnaire for temporomandibular joint, cephalometric analysis, polysomnography, acoustic rhinometry, and multislice spiral computed tomography were performed to evaluate changes from the treatment. All the results showed that the patient had a significantly alleviated OSAS. In addition, an acceptable occlusion was also obtained. PMID:23714918

Nie, Ping; Zhu, Min; Lu, Xiao-Feng; Fang, Bing

2013-05-01

83

Homozygous FOXE3 mutations cause non-syndromic, bilateral, total sclerocornea, aphakia, microphthalmia and optic disc coloboma  

PubMed Central

Purpose To investigate the genetic basis of recessively-inherited congenital, non syndromic, bilateral, total sclerocornea in two consanguineous pedigrees, one from the Punjab province of Pakistan and the other from the Tlaxcala province of Mexico. Methods Ophthalmic examinations were conducted on each family member to confirm their diagnosis and magnetic resonance imaging (MRI) or ultrasonography of the eyes was performed on some family members. Genomic DNA was analyzed by homozygosity mapping using the Affymetrix 6.0 SNP array and linkage was confirmed with polymorphic microsatellite markers. Candidate genes were sequenced. Results A diagnosis of autosomal recessive sclerocornea was established for 7 members of the Pakistani and 8 members of the Mexican pedigrees. In the Pakistani family we established linkage to a region on chromosome 1p that contained Forkhead Box E3 (FOXE3), a strong candidate gene since FOXE3 mutations had previously been associated with various anterior segment abnormalities. Sequencing FOXE3 identified the previously reported nonsense mutation, c.720C>A, p.C240X, in the Pakistani pedigree and a novel missense mutation which disrupts an evolutionarily conserved residue in the forkhead domain, c.292T>C, p.Y98H, in the Mexican pedigree. Individuals with heterozygous mutations had no ocular abnormalities. MRI or ultrasonography confirmed that the patients with sclerocornea were also aphakic, had microphthalmia and some had optic disc coloboma. Conclusions This is the fourth report detailing homozygous FOXE3 mutations causing anterior segment abnormalities in human patients. Previous papers have emphasized aphakia and microphthalmia as the primary phenotype, but we find that the initial diagnosis – and perhaps the only one possible in a rural setting – is one of non-syndromic, bilateral, total sclerocornea. Dominantly inherited anterior segment defects have also been noted in association with heterozygous FOXE3 mutations. However the absence of any abnormalities in the FOXE3 heterozygotes described suggests that genetic background and environmental factors plays a role in the penetrance of the mutant allele.

Buentello-Volante, Beatriz; McKibbin, Martin; Rocha-Medina, J. Alberto; Fernandez-Fuentes, Narcis; Koga-Nakamura, Wilson; Ashiq, Aruna; Khan, Kamron; Booth, Adam P.; Williams, Grange; Raashid, Yasmin; Jafri, Hussain; Rice, Aine; Inglehearn, Chris F.; Zenteno, Juan Carlos

2010-01-01

84

First Report of Bilateral Pheochromocytoma in the Clinical Spectrum of HIF2A-Related Polycythemia-Paraganglioma Syndrome  

PubMed Central

Context: Molecular genetic research has so far resulted in the identification of 10 well-characterized susceptibility genes for hereditary pheochromocytoma (PHEO) or paraganglioma (PGL). Recently, a new syndrome characterized by multiple PGLs and somatostatinomas associated with congenital polycythemia due to somatic mutations in HIF2A has been reported. Objective: The aim of the study was to define the genetic defect in a new case of bilateral PHEO and multiple PGLs associated with congenital polycythemia. Patient: A female patient presented with neonatal polycythemia (treated by phlebotomies, 1 session approximately every 4 mo), mildly enlarged cerebral ventricles, and bilateral PHEO and multiple PGLs. There was no family history of any neuroendocrine tumor or polycythemia. Surgical removal of the tumors only temporarily normalized plasma erythropoietin (Epo) levels and discontinued phlebotomies. No germline mutations were initially detected in the SDHB, SDHC, SDHD, VHL, and PHD2 genes, known to be associated with polycythemia. The PHEOs presented with a typical noradrenergic biochemical phenotype. Results: A heterozygous missense mutation (c.1589C>T) was identified in exon 12 of HIF2A, resulting in an alanine 530 substitution in the HIF-2? protein with valine (A530V). This somatic mutation was detected in the tissue from 1 PHEO and 1 PGL, with no HIF2A germline mutation found. This mutation led to stabilization of HIF-2? and hence a gain-of-function phenotype, as in previously published studies. Conclusion: This case represents the first association of a somatic HIF2A gain-of-function mutation with PHEO and congenital polycythemia, and it alerts physicians to perform proper genetic screening in patients presenting with multiple norepinephrine-producing PHEOs and polycythemia. This report also extends the previous findings of a new syndrome of only multiple PGLs, somatostatinomas, and polycythemia to multiple PHEOs.

Taieb, David; Yang, Chunzhang; Delenne, Blandine; Zhuang, Zhengping; Barlier, Anne; Sebag, Frederic

2013-01-01

85

Unbalanced der(5)t(5;20) translocation associated with Megalencephaly, perisylvian Polymicrogyria, Polydactyly and Hydrocephalus  

PubMed Central

The combination of megalencephaly, perisylvian polymicrogyria, polydactyly and hydrocephalus (MPPH) is a rare syndrome of unknown cause. We observed two first cousins affected by an MPPH-like phenotype with a submicroscopic chromosome 5q35 deletion as a result of an unbalanced der(5)t(5;20)(q35.2;q13.3) translocation, including the NSD1 Sotos syndrome locus. We describe the phenotype and the deletion breakpoints of the two MPPH-like patients and compare these with five unrelated MPPH and Sotos patients harboring a 5q35 microdeletion. Mapping of the breakpoints in the two cousins was performed by MLPA, FISH, high density SNP-arrays and Q-PCR for the 5q35 deletion and 20q13 duplication. The 5q35 deletion area of the two cousins almost completely overlaps with earlier described patients with an atypical Sotos microdeletion, except for the DRD1 gene. The five unrelated MPPH patients neither showed submicroscopic chromosomal aberrations nor DRD1 mutations. We reviewed the brain MRI of 10 Sotos patients and did not detect polymicrogyria in any of them. In our two cousins, the MPPH-like phenotype is probably caused by the contribution of genes on both chromosome 5q35 and 20q13. Some patients with MPPH may harbor a submicroscopic chromosomal aberration and therefore high-resolution array analysis should be part of the diagnostic workup.

Verkerk, Annemieke J.M.H.; Schot, Rachel; van Waterschoot, Laura; Douben, Hannie; Poddighe, Pino J.; Lequin, Maarten H.; de Vries, Linda S.; Terhal, Paulien; Hahnemann, Johanne M.D.; de Coo, Irenaeus F.M.; de Wit, Marie-Claire Y.; Wafelman, Leontien S.; Garavelli, Livia; Dobyns, William B.; Van der Spek, Peter J.; de Klein, Annelies; Mancini, Grazia M.S.

2010-01-01

86

EFFECT OF BILATERAL OOPHORECTOMY ON ADRENOCORTICAL FUNCTION IN WOMEN WITH POLYCYSTIC OVARY SYNDROME (PCOS)  

PubMed Central

Objective To determine the impact of ovary-secreted products on adrenocortical function in women with PCOS by studying the adrenocortical response to acute adrenocorticotropic-stimulating hormone (ACTH) stimulation before and after bilateral oophorectomy. Design Prospective study. Setting Tertiary care medical center Participants Fourteen women with PCOS scheduled for bilateral oophorectomy for benign indications, on transdermal estradiol (E2) postoperatively. Interventions Physical exam, blood sampling before and after oophorectomy, measurement of hormone levels. Basal (Steroid0), maximum stimulated (Steroid60), and net increment (?Steroid) levels of androstenedione (A4), dehydroepiandrosterone (DHEA), and cortisol (F) before and after ACTH-1–24 stimulation were assessed. Main Outcome Measures Pre- and post-operative basal and ACTH(1–24)-stimulated hormonal levels. Results Total testosterone, free testosterone, and estrone levels decreased, and FSH levels increased significantly following oophorectomy. No significant differences in E2, DHEA sulfate (DHEAS) or sex hormone binding globulin levels were detected. Basal and ACTH-stimulated A4 levels decreased significantly following oophorectomy, and ?A4 was significantly increased. No significant differences in DHEA0, DHEA60, or F0 levels were detected; F60 and ?F levels tended to increase following oophorectomy, but the differencesdid not reach significance. Conclusions Ovarian factors do not appear to contribute significantly to the adrenocortical dysfunction of PCOS.

Azziz, Ricardo; Chang, Wendy Y.; Stanczyk, Frank Z.; Woods, Keslie

2012-01-01

87

[A neonatal case of anterior spinal artery syndrome presenting with bilateral arm paresis].  

PubMed

Anterior spinal artery syndrome is rare in children, especially in neonates. We present a girl with hydrops fetalis and hypothyroidism who developed flaccid paresis of both arms in the neonatal period (around day 25). MRI of the spine performed on day 52 revealed atrophic changes at C5-Th1 without Gd-DTPA-induced enhancement. Nerve conduction studies were also helpful in the diagnosis;in the upper limbs, motor potential was not elicited, while sensory nerve conduction velocity was normal. These clinical and laboratory findings suggested an atypical case of anterior spinal artery syndrome. PMID:15675362

Suzuki, Yasuhiro; Toribe, Yasuhisa; Idoguchi, Rie; Ogawa, Kana; Mano, Toshiyuki

2005-01-01

88

Late onset Li-Fraumeni Syndrome with bilateral breast cancer and other malignancies: case report and review of the literature  

PubMed Central

Background Li-Fraumeni-Syndrome (LFS) is an autosomal-dominant, inherited tumour predisposition syndrome associated with heterozygous germline mutations in the TP53 gene. Patients with LFS are at a high risk to develop early-onset breast cancer and multiple malignancies, among which sarcomas are the most common. A high incidence of childhood tumours and close to 100% penetrance has been described. Knowledge of the genetic status of the TP53 gene in these patients is critical not only due to the increased risk of malignancies, but also because of the therapeutic implications, since a higher rate of radiation-induced secondary tumours in these patients has been observed. Case report We report a patient with LFS harbouring heterozygous, pathogenic TP53 germline mutation, who was affected by four synchronous malignancies at the age of 40: a myxofibrosarcoma of the right upper arm, bilateral breast cancer and a periadrenal liposarcoma. Radiological treatments and a surveillance program were adjusted according to recommendations for LFS patients. Conclusion Management of tumour treatment of patients with LFS is different to the general population because of their risk for secondary cancers in the radiation field. Screening procedures should take a possibly elevated risk for radiation induced cancer into account.

2012-01-01

89

Thermal capsulorraphy of bilateral glenohumeral joints in a pediatric patient with Ehlers-Danlos syndrome  

Microsoft Academic Search

Ehlers-Danlos syndrome (EDS) is a heterogeneous collection of inherited connective tissue disorders characterized by hypermobility of the joints and hyperextensibility and fragility of the skin. For many patients, the hypermobile joints become problematic. To date, the mainstay of surgical treatment for EDS-related joint laxity has been open surgical capsulorraphy, which, although usually effective, confers significant morbidity to the patient. We

Julian M. Aldridge; John J. Perry; Daryl C. Osbahr; Kevin P. Speer

2003-01-01

90

Recurrent Bilateral Retinal Vasculitis as a Manifestation of Post-streptococcal Uveitis Syndrome  

PubMed Central

We report a case of post-streptococcal uveitis mainly presenting with bilateral recurrent retinal vasculitis in Korea. A 14-year-old Asian female presented with decreased visual acuity of 20 / 30 in the right eye and 20 / 25 in the left eye. The patient had a history of glomerulonephritis nine months before onset of uveitis. The manifestation of uveitis was predominantly retinal vasculitis. We presumed post-streptococcal uveitis because probable streptococcal infection was confirmed by anti-streptolysin O titer elevation. With topical and oral steroid treatments, the patient experienced complete vision recovery. Post-streptococcal uveitis occurs rarely and mostly involves young patients in the form of non-granulomatous anterior uveitis. However, as this case shows, it may primarily involve the posterior uvea without anterior inflammation and may recur.

Han, Jinu; Lee, Sung Chul

2012-01-01

91

Thermal capsulorraphy of bilateral glenohumeral joints in a pediatric patient with Ehlers-Danlos syndrome.  

PubMed

Ehlers-Danlos syndrome (EDS) is a heterogeneous collection of inherited connective tissue disorders characterized by hypermobility of the joints and hyperextensibility and fragility of the skin. For many patients, the hypermobile joints become problematic. To date, the mainstay of surgical treatment for EDS-related joint laxity has been open surgical capsulorraphy, which, although usually effective, confers significant morbidity to the patient. We present the case of a 9-year-old girl diagnosed with a variant of EDS and severely disabled from multidirectional instability of her shoulders and recurrent dislocations of her hips. After 1 year of nonoperative treatment (physical therapy, bracing, and activity restriction) failed, we performed a sequential arthroscopic thermal capsulorraphy of both shoulders. At a 2-year follow-up, the patient has no instability in the left shoulder and only occasional subluxations of the contralateral shoulder. We believe that thermal capsulorraphy is a viable addition to the shoulder surgeon's armamentarium in treating multidirectional instability in children with EDS. PMID:12724665

Aldridge, Julian M; Perry, John J; Osbahr, Daryl C; Speer, Kevin P

2003-01-01

92

A new multiple malformation syndrome of Müllerian dysgenesis and conductive hearing loss with facial hypoplasia, bilateral forearm deformity, brachydactyly, spinal stenosis and scoliosis.  

PubMed

Multiple congenital malformations in a young girl with bilateral conductive hearing loss are described. Facial dysmorphic features include prominent supraorbital ridges, facial hypoplasia, facial asymmetry, downward-slanting palpebral fissures, high prominent nasal bridge with bifid nasal tip and a small lower jaw, and hypoplastic ear lobules with bilaterally narrow and oblique external auditory canals. Recognisable skeletal abnormalities include hypoplastic facial bones, hypoplastic clavicles, narrow and anteriorly sloping shoulders, bowing of both forearm bones, brachydactyly due to short metacarpals and hypoplastic terminal phalanges, thoracolumbar kyphoscoliosis, narrow transverse measurements of most vertebrae with prominent coccyx, spinal canal narrowing, hypoplasia of lower ilia, medially bowed femora, tibiae and fibulae and brachysyndactyly of the second, third and fourth toes bilaterally. Gynaecological evaluation revealed abnormalities of the Mullerian duct structures: urogenital sinus, a vestigial uterus, a posteriorly placed small but patent vagina and a septum at the vaginal introitus. The pattern of MCA probably refers to a new syndrome within the "community of syndromes" involving anomalies of the Mullerian duct structures, limbs, spine and external ears. PMID:9272710

Kumar, D; Masel, J P

1997-07-01

93

Which approach is better: bilateral versus unilateral thalamic deep brain stimulation in patients with fragile X-associated tremor ataxia syndrome.  

PubMed

Fragile X-associated tremor ataxia syndrome (FXTAS) is a relatively recently described condition that is frequently misdiagnosed as essential tremor and then occasionally treated as such with deep brain stimulation (DBS) to the nucleus ventralis intermedius of the thalamus (Vim). Reports of ataxia worsening after bilateral Vim DBS in FXTAS patients are conflicting, and only five FXTAS patients treated with Vim DBS for intractable tremor have been reported in the literature, three of whom having undergone a bilateral procedure. We report a patient who underwent a staged Vim DBS procedure, with excellent contralateral hand tremor control and no worsening of ataxia after the first procedure, but immediate worsening of his ataxia after the second one, arguing in favor of a unilateral surgical approach for intractable tremor in FXTAS. PMID:24122741

Mehanna, R; Itin, I

2014-04-01

94

Methylxanthines Reverse the Adipsic and Aphagic Syndrome Induced by Bilateral 6Hydroxydopamine Lesions of the Nigrostriatal Pathway in Rats  

Microsoft Academic Search

This study investigated whether methylxanthines (caffeine and theophylline) would restore food and water intake in rats made aphagic and adipsic by bilateral 6-hydroxydopamine lesions of the nigrostriatal bundle, and these results were compared with the effects of d-amphetamine, the dopamine D1 agonist SKF 38393, and the D2\\/3 agonist quinpirole. In a separate experiment, we investigated whether the selective D1 antagonist,

M Casas; G Prat; P Robledo; M Barbanoj; J Kulisevsky; F Jané

2000-01-01

95

A life-threatening respiratory syncytial virus infection: a previously healthy infant with bilateral spontaneous pneumothorax and acute respiratory distress syndrome.  

PubMed

Respiratory syncytial virus (RSV) is the leading cause of viral respiratory tract infections in infants and young children. Although the course of RSV infection is usually benign, a small proportion of infants require mechanical ventilation for respiratory failure. We describe an eight-month-old previously healthy female who developed bilateral pneumothorax and acute respiratory distress syndrome (ARDS) secondary to RSV infection. Because of the severe hypoxemia, three doses of surfactant were administered and prone positioning was implemented for nine days. After a prolonged course of mechanical ventilation, she was extubated at day 21 and discharged from the pediatric intensive care unit four days later. We conclude that RSV infections can be severe, and some patients may require mechanical ventilation. Supportive therapies like surfactant replacement therapy and prone positioning can be beneficial in patients with ARDS in whom severe hypoxemia persists despite high levels of positive end-expiratory pressure (PEEP) and plateau pressures. PMID:24382538

Odek, Ca?lar; Kendirli, Tan?l; Yaman, Ayhan; Aldemir-Kocaba?, Bilge; Ince, Erdal

2013-01-01

96

Recurrent Bilateral Branch Retinal Artery Occlusion with Hearing Loss and Encephalopathy: The First Case Report of Susac Syndrome in Korea  

PubMed Central

We report the first case of Susac syndrome in Koreans, in a 23-yr-old female patient who presented with sudden visual loss and associated neurological symptoms. Ophthalmic examination and fluorescein angiography showed multiple areas of branch retinal artery occlusion, which tended to recur in both eyes. Magnetic resonance imaging showed dot-like, diffusion-restricted lesions in the corpus callosum and left fornix, and audiometry showed low-frequency sensory hearing loss, compatible with Susac syndrome. She received immunosuppressive therapy with oral steroid and azathioprine. Three months later all the symptoms disappeared but obstructive vasculitis have been relapsing. This patient demonstrated the entire clinical triad of Susac syndrome, which tends to occur in young females. Although this disorder has rarely been reported in Asian populations, a high index of suspicion is warranted for early diagnosis and timely treatment.

Joe, Soo Geun; Kim, June-Gone; Kwon, Sun Uck; Lee, Choong Wook; Lim, Hyun Woo

2011-01-01

97

[A Study of the Usefulness of Flow-adjustable Bilateral Pulmonary Artery Banding (FABPAB) for Hypoplastic Left Heart Syndrome( HLHS) or HLHS Variant].  

PubMed

Backgrounds:Bilateral pulmonary artery banding( BPAB), a relatively minimally invasive procedure, has begun to be undertaken as the initial operation for the treatment of hypoplastic left heart syndrome (HLHS) or HLHS variant. In the opinion of the present authors, it is practicable to avoid stepwise operations with cardiopulmonary bypass (CPB) in newborn patients by conducting an initial operation consisting of flow-adjustable bilateral pulmonary artery banding (FABPAB) combined with transcatheter pulmonary artery balloon dilatation( PABD), followed in a subsequent treatment course by transcatheter PABD, which would allow for more minimally invasive adjustment of the pulmonary artery bloodflow. This study was conducted as a retrospective evaluation of the usefulness of this therapeutic strategy. Methods:Fourteen patients who underwent BPAB as the initial operation for HLHS/HLHS variant between April 2008 and October 2013 were included in this study. Stepwise surgical repair including BPAB was performed via a median sternotomy as the initial operation, without the use of CPB. And 7patients underwent transcatheter PABD successfully. Results:In this series, 7 patients developed a significant pulmonary artery index (PAI) after PABD (pre PABD:148.3±63.9 vs. post PABD:232.5±73.2, p<0.05). No need of the pulmonary artery reconstruction at the 2nd operation. One patient failed to survive the operation, in which difficulty in adjustment of atrioventricular valvular regurgitation was encountered after the Norwood procedure. There were 3 patients who underwent the Norwood combined BDG at the mean age of 4.6 months and themean body weight of 5.5 kg. Fontan procedure was accomplished in 3 of the 6 patients of the survivor, while 3 patients were on standby for the Fontan procedure. Conclusion:We performed BPAB as the initial operation combined with PABD in the treatment of HLHS/HLHS variant, which enabled avoidance of the initial operation with cardiopulmonary bypass at the neonatal stage and developed a significant pulmonary artery index after PABD. PMID:24917154

Miyamoto, Takashi

2014-04-01

98

Bilateral anterior lenticonus in a case of Alport syndrome: a clinical and histopathological correlation after successful clear lens extraction.  

PubMed

A 29-year-old woman presented with symptoms of gradual, progressive, painless diminution of vision in both eyes for past 8-10?years. On examination, uncorrected visual acuity was 20/60 in the right eye and 20/126 in the left eye. Anterior segment examination was essentially normal except for a conical protrusion in the pupillary axis of the anterior lens capsule with a clear underlying lens. During surgery, central protruded fragile lens capsule was meticulously handled to create well-centered continuous curvilinear capsulorhexis by using paediatric rhexis forceps. Histopathology of excised capsule showed markedly thinned lens capsule with normal epithelium. Electron microscopy of the anterior lens capsule showed multiple linear and irregular zones of dehiscence. Few of these had fibrillar, irregular electron-dense material and vacuoles; and adjacent cells were irregular suggestive of Alport syndrome. Postoperative course was uneventful and visual acuity improved to 20/20 unaided. Clear lens extraction is an effective means of rehabilitating visually symptomatic anterior lenticonus of Alport syndrome. PMID:24969069

Sonarkhan, Shilpa; Ramappa, Muralidhar; Chaurasia, Sunita; Mulay, Kaustubh

2014-01-01

99

[Impacts of Bilateral Pulmonary Artery Banding on the Surgical and Long-term Outcomes in Hypoplastic Left Heart Syndrome( HLHS) and its Variants].  

PubMed

Although Norwood-type operation is a fundamental procedure for hypoplastic left heart syndrome and its variants, the surgical risk is still high even in the high volume center. Bilateral pulmonary artery (PA) banding( BPAB) is an alternative procedure to avoid the risk in the neonatal period. We clarified the impacts of BPAB on the surgical and long-term outcomes in HLHS and its variants. Consecutive 85 patients( classical HLHS:52, variant:33) who underwent surgical intervention between July, 1993 and December, 2012 were evaluated. Fetal diagnosis was done in 37 patients (44%). Forty-two patients underwent primary Norwood operation[ Gp N, Blalock-Taussig( BT):29, right-ventricle( RV)-PA conduit:13] and the remaining 43 patients underwent BPAB followed by Norwood operation. Among them, 4 patients did not reach Norwood [9%, atrioventricular valve regurgitation (AVVR):1, infection:1, preoperative shock:2). The survived 39 patients were divided into 2 groups;Gp BN (BPAB ? Norwood+BT/RV-PA conduit, n=22), Gp BG [BPAB ? Norwood+bidirectional Glenn procedure(BDG), n=17]. BPAB for HLHS and its variants is useful in terms of lower mortality. The 2ndstage palliation is so important that modified Norwood procedure with BT shunt or RV-PA conduit contribute to the lower morbidity including neurological outcome. Norwood+BDG strategy might be beneficial for the high risk patients such as with poor cardiac function or low body weight. PMID:24917157

Sakamoto, Takahiko; Harada, Yorikazu

2014-04-01

100

Volume reductions in frontopolar and left perisylvian cortices in methamphetamine induced psychosis.  

PubMed

Consumption of methamphetamine disturbs dopaminergic transmission and sometimes provokes schizophrenia-like-psychosis, named methamphetamine-associated psychosis (MAP). While previous studies have repeatedly reported regional volume reductions in the frontal and temporal areas as neuroanatomical substrates for psychotic symptoms, no study has examined whether such neuroanatomical substrates exist or not in patients with MAP. Magnetic resonance images obtained from twenty patients with MAP and 20 demographically-matched healthy controls (HC) were processed for voxel-based morphometry (VBM) using Diffeomorphic Anatomical Registration using Exponentiated Lie Algebra. An analysis of covariance model was adopted to identify volume differences between subjects with MAP and HC, treating intracranial volume as a confounding covariate. The VBM analyses showed significant gray matter volume reductions in the left perisylvian structures, such as the posterior inferior frontal gyrus and the anterior superior temporal gyrus, and the frontopolar cortices, including its dorsomedial, ventromedial, dorsolateral, and ventrolateral portions, and white matter volume reduction in the orbitofrontal area in the patients with MAP compared with the HC subjects. The smaller regional gray matter volume in the medial portion of the frontopolar cortex was significantly correlated with the severe positive symptoms in the individuals with MAP. The volume reductions in the left perisylvian structure suggest that patients with MAP have a similar pathophysiology to schizophrenia, whereas those in the frontopolar cortices and orbitofrontal area suggest an association with antisocial traits or vulnerability to substance dependence. PMID:23688384

Aoki, Yuta; Orikabe, Lina; Takayanagi, Yoichiro; Yahata, Noriaki; Mozue, Yuriko; Sudo, Yasuhiko; Ishii, Tatsuji; Itokawa, Masanari; Suzuki, Michio; Kurachi, Masayoshi; Okazaki, Yuji; Kasai, Kiyoto; Yamasue, Hidenori

2013-07-01

101

Analysis of the subcomponents and cortical terminations of the perisylvian superior longitudinal fasciculus: a fiber dissection and DTI tractography study.  

PubMed

The anatomy of the perisylvian component of the superior longitudinal fasciculus (SLF) has recently been reviewed by numerous diffusion tensor imaging tractography (DTI) studies. However, little is known about the exact cortical terminations of this tract. The aim of the present work is to isolate the different subcomponents of this tract with fiber dissection and DTI tractography, and to identify the exact cortical connections. Twelve postmortem human hemispheres (6 right and 6 left) were dissected using the cortex-sparing fiber dissection. In addition, three healthy brains were analyzed using DTI-based tractography software. The different components of the perisylvian SLF were isolated and the fibers were followed until the cortical terminations. Three segments of the perisylvian SLF were identified: (1) anterior segment, connecting the supramarginal gyrus and superior temporal gyrus with the precentral gyrus, (2) posterior segment, connecting the posterior portion of the middle temporal gyrus with the angular gyrus, and (3) long segment of the arcuate fasciculus that connects the middle and inferior temporal gyri with the precentral gyrus and posterior portion of the inferior and middle frontal gyri. In the present study, three different components of the perisylvian SLF were identified. For the first time, our dissections revealed that each component was connected to a specific cortical area within the frontal, parietal and temporal lobes. By accurately depicting not only the trajectory but also cortical connections of this bundle, it is possible to develop new insights into the putative functional role of this tract. PMID:22422148

Martino, Juan; De Witt Hamer, Philip C; Berger, Mitchel S; Lawton, Michael T; Arnold, Christine M; de Lucas, Enrique Marco; Duffau, Hugues

2013-01-01

102

The Relationship between Perisylvian Morphology and Verbal Short-Term Memory Functioning in Children with Neurodevelopmental Disorders  

ERIC Educational Resources Information Center

Although children with neurodevelopmental disorders frequently present with reduced short-term memory functioning, the relationship between perisylvian morphology and verbal short-term memory functioning has received limited attention. Thus, examining this relationship in children with neurodevelopmental disorders was the focus of this exploratory…

Kibby, Michelle Y.; Kroese, Judith M.; Morgan, Allison E.; Hiemenz, Jennifer R.; Cohen, Morris J.; Hynd, George W.

2004-01-01

103

Parallel versus Serial Processing Dependencies in the Perisylvian Speech Network: A Granger Analysis of Intracranial EEG Data  

ERIC Educational Resources Information Center

In this work, we apply Granger causality analysis to high spatiotemporal resolution intracranial EEG (iEEG) data to examine how different components of the left perisylvian language network interact during spoken language perception. The specific focus is on the characterization of serial versus parallel processing dependencies in the dominant…

Gow, David W., Jr.; Keller, Corey J.; Eskandar, Emad; Meng, Nate; Cash, Sydney S.

2009-01-01

104

Genetic mapping to 10q23.3-q24.2, in a large Italian pedigree, of a new syndrome showing bilateral cataracts, gastroesophageal reflux, and spastic paraparesis with amyotrophy.  

PubMed Central

We have recently observed a large pedigree with a new rare autosomal dominant spastic paraparesis. In three subsequent generations, 13 affected individuals presented with bilateral cataracts, gastroesophageal reflux with persistent vomiting, and spastic paraparesis with amyotrophy. Bilateral cataracts occurred in all affected individuals, with the exception of one patient who presented with a chorioretinal dystrophy, whereas clinical signs of spastic paraparesis showed a variable expressivity. Using a genomewide mapping approach, we mapped the disorder to the long arm of chromosome 10 on band q23.3-q24.2, in a 12-cM chromosomal region where additional neurologic disorders have been localized. The spectrum of phenotypic manifestations in this family is reminiscent of a smaller pedigree, reported recently, confirming the possibility of a new syndrome. Finally, the anticipation of symptoms suggests that an unstable trinucleotide repeat may be responsible for the condition.

Seri, M; Cusano, R; Forabosco, P; Cinti, R; Caroli, F; Picco, P; Bini, R; Morra, V B; De Michele, G; Lerone, M; Silengo, M; Pela, I; Borrone, C; Romeo, G; Devoto, M

1999-01-01

105

Bilateral odontogenic keratocyst of the mandible.  

PubMed

Odontogenic keratocyst (OKC) is a cyst of dental origin with an aggressive clinical behavior, having high recurrence rate. Multiple cysts are associated with bifid-rib basal cell nevus syndrome (Gorlin syndrome). We present a case of bilateral odontogenic keratocyst in a cleft lip patient. PMID:25018611

Ram, Hari; Mohammad, Shadab; Husain, Nuzhat; Gupta, Shalini; Kumar, Ajay

2014-09-01

106

Mapping phonemic processing zones along human perisylvian cortex: an electro-corticographic investigation.  

PubMed

The auditory system is organized such that progressively more complex features are represented across successive cortical hierarchical stages. Just when and where the processing of phonemes, fundamental elements of the speech signal, is achieved in this hierarchy remains a matter of vigorous debate. Non-invasive measures of phonemic representation have been somewhat equivocal. While some studies point to a primary role for middle/anterior regions of the superior temporal gyrus (STG), others implicate the posterior STG. Differences in stimulation, task and inter-individual anatomical/functional variability may account for these discrepant findings. Here, we sought to clarify this issue by mapping phonemic representation across left perisylvian cortex, taking advantage of the excellent sampling density afforded by intracranial recordings in humans. We asked whether one or both major divisions of the STG were sensitive to phonemic transitions. The high signal-to-noise characteristics of direct intracranial recordings allowed for analysis at the individual participant level, circumventing issues of inter-individual anatomic and functional variability that may have obscured previous findings at the group level of analysis. The mismatch negativity (MMN), an electrophysiological response elicited by changes in repetitive streams of stimulation, served as our primary dependent measure. Oddball configurations of pairs of phonemes, spectro-temporally matched non-phonemes, and simple tones were presented. The loci of the MMN clearly differed as a function of stimulus type. Phoneme representation was most robust over middle/anterior STG/STS, but was also observed over posterior STG/SMG. These data point to multiple phonemic processing zones along perisylvian cortex, both anterior and posterior to primary auditory cortex. This finding is considered within the context of a dual stream model of auditory processing in which functionally distinct ventral and dorsal auditory processing pathways may be engaged by speech stimuli. PMID:23708059

Molholm, Sophie; Mercier, Manuel R; Liebenthal, Einat; Schwartz, Theodore H; Ritter, Walter; Foxe, John J; De Sanctis, Pierfilippo

2014-07-01

107

Temporal dynamics of perisylvian activation during language processing in children and adults  

PubMed Central

The perisylvian region of the human cortex is known to play a major role in language processing. Especially the superior temporal cortex (STC) and the inferior frontal cortex (IFC) have been investigated with respect to their particular involvement in language comprehension. In the present research, the timing of recruitment of these language-related brain areas in both hemispheres was examined as a function of age using functional imaging data of 6-year-old children and adults with a special focus on blood oxygenation level dependent (BOLD) response time courses. The results show that children’s activation time courses differ from that of adults. First, children show an overall later peak of BOLD responses. Second, children’s IFC responds much later than their STC, while in adults the difference between both regions is less pronounced. Within the STC, both groups show similar regionally U-shaped activation patterns with fastest peaks in voxels at the STC’s mid-portion around Heschl’s gyrus and longer latencies in anterior and posterior directions, suggesting a coarsely similar information flow in adults and children in the temporal region. Finally, children in contrast to adults, display a temporal primacy of right over left hemispheric activation. The observed overall latency differences between children and adults are in line with the assumption of ongoing maturation in perisylvian brain regions and the connections between them. A functional perspective on BOLD timing argues for a developmental change from higher processing costs in children compared to adults due to slower and less automatic language processes, in particular those located in the IFC. The observed hemispheric differences are discussed in the context of developmental models assuming a high reliance on right-hemisphere-based suprasegmental information processing during language comprehension in childhood.

Brauer, Jens; Neumann, Jane; Friederici, Angela D.

2008-01-01

108

Temporal dynamics of perisylvian activation during language processing in children and adults.  

PubMed

The perisylvian region of the human cortex is known to play a major role in language processing. Especially the superior temporal cortex (STC) and the inferior frontal cortex (IFC) have been investigated with respect to their particular involvement in language comprehension. In the present research, the timing of recruitment of these language-related brain areas in both hemispheres was examined as a function of age using functional imaging data of 6-year-old children and adults with a special focus on blood oxygenation level dependent (BOLD) response time courses. The results show that children's activation time courses differ from that of adults. First, children show an overall later peak of BOLD responses. Second, children's IFC responds much later than their STC, while in adults the difference between both regions is less pronounced. Within the STC, both groups show similar regionally U-shaped activation patterns with fastest peaks in voxels at the STC's mid-portion around Heschl's gyrus and longer latencies in anterior and posterior directions, suggesting a coarsely similar information flow in adults and children in the temporal region. Finally, children in contrast to adults, display a temporal primacy of right over left hemispheric activation. The observed overall latency differences between children and adults are in line with the assumption of ongoing maturation in perisylvian brain regions and the connections between them. A functional perspective on BOLD timing argues for a developmental change from higher processing costs in children compared to adults due to slower and less automatic language processes, in particular those located in the IFC. The observed hemispheric differences are discussed in the context of developmental models assuming a high reliance on right-hemisphere-based suprasegmental information processing during language comprehension in childhood. PMID:18479941

Brauer, Jens; Neumann, Jane; Friederici, Angela D

2008-07-15

109

Segregation of Lexical and Sub-Lexical Reading Processes in the Left Perisylvian Cortex  

PubMed Central

A fundamental issue in cognitive neuroscience is the existence of two major, sub-lexical and lexical, reading processes and their possible segregation in the left posterior perisylvian cortex. Using cortical electrostimulation mapping, we identified the cortical areas involved on reading either orthographically irregular words (lexical, “direct” process) or pronounceable pseudowords (sublexical, “indirect” process) in 14 right-handed neurosurgical patients while video-recording behavioral effects. Intraoperative neuronavigation system and Montreal Neurological Institute (MNI) stereotactic coordinates were used to identify the localization of stimulation sites. Fifty-one reading interference areas were found that affected either words (14 areas), or pseudo-words (11 areas), or both (26 areas). Forty-one (80%) corresponded to the impairment of the phonological level of reading processes. Reading processes involved discrete, highly localized perisylvian cortical areas with individual variability. MNI coordinates throughout the group exhibited a clear segregation according to the tested reading route; specific pseudo-word reading interferences were concentrated in a restricted inferior and anterior subpart of the left supramarginal gyrus (barycentre x?=??68.1; y?=??25.9; z?=?30.2; Brodmann’s area 40) while specific word reading areas were located almost exclusively alongside the left superior temporal gyrus. Although half of the reading interferences found were nonspecific, the finding of specific lexical or sublexical interferences is new evidence that lexical and sublexical processes of reading could be partially supported by distinct cortical sub-regions despite their anatomical proximity. These data are in line with many brain activation studies that showed that left superior temporal and inferior parietal regions had a crucial role respectively in word and pseudoword reading and were core regions for dyslexia.

Roux, Franck-Emmanuel; Durand, Jean-Baptiste; Jucla, Melanie; Rehault, Emilie; Reddy, Marion; Demonet, Jean-Francois

2012-01-01

110

Does bilateral pulmonary banding in comparison to Norwood procedure improve outcome in neonates with hypoplastic left heart syndrome beyond second-stage palliation? A review of the current literature.  

PubMed

Best evidence protocol was applied and the question addressed, whether there is an outcome advantage of a hybrid approach including bilateral pulmonary banding (BPB) and ductal stenting over Norwood procedure (NP) to maintain systemic circulation and to restrict pulmonary blood flow in neonates with hypoplastic left heart syndrome (HLHS). Out of 80 articles published since the original description of BPB in 2002, eight were selected to answer the clinical question. All studies were retrospective case-series after BLB, only three compared results with NP controls, but not in a randomized fashion. Only three chose high-risk patients. Mean follow-up interval was mentioned only in one study. Reported mean hospital, interstage, and stage 2 mortalities after BPB was 17.3, 12.4, and 25.3%, respectively. Estimated transplant-free survival beyond stage 2 palliation ranged from 7 to 80%, with no significant differences to NP controls in three studies. Reintervention rate was high, ranging from 20 to 42% at different sites. Due to study design or inhomogeneity of patient groups, current literature does not show evidence that bilateral pulmonary banding improves outcome with respect to survival beyond second-stage HLHS palliation. PMID:22215495

Photiadis, Joachim; Sinzobahamvya, Nicodème; Hraška, Viktor; Asfour, Boulos

2012-04-01

111

Neuroimaging Aspects of Aicardi Syndrome  

PubMed Central

Aicardi syndrome is a rare neurodevelopmental disorder characterized by congenital chorioretinal lacunae, corpus callosum dysgenesis, seizures, polymicrogyria, cerebral heterotopias, intracranial cysts, and costovertebral defects. Cerebellar abnormalities have been described occasionally. Aicardi syndrome is sporadic and has been observed only in females and 47,XXY males. Therefore, it is thought to result from a mutation in an X-linked gene. Improved definition of the clinical phenotype should focus the selection of functional candidate genes for mutation analysis. Because central nervous system abnormalities are the most prominent component of the phenotype, we performed a detailed characterization of abnormalities identified on magnetic resonance neuroimaging studies from 23 girls with Aicardi syndrome, the largest cohort to undergo such review by a single group of investigators. All patients had polymicrogyria that was predominantly frontal and perisylvian and often associated with underopercularization. Periventricular nodular heterotopias, present in all patients, were more frequent than previously reported; 10 had single and 11 had multiple intracranial cysts. Posterior fossa abnormalities were also more frequent than previously described. Cerebellar abnormalities were noted in 95% of studies where they could be evaluated. As a novel finding, we noted tectal enlargement in 10 patients. Since mildly-affected girls with variable callosal dysgenesis have now been reported, the constellation of frontal-dominant and perisylvian polymicrogyria, periventricular nodular heterotopias, intracranial cysts, and posterior fossa abnormalities, including tectal enlargement, should prompt consideration of the diagnosis of Aicardi syndrome. We further propose that improved characterization of the neurological phenotype will benefit the selection of candidate genes for mutation analysis.

Hopkins, Bobbi; Sutton, V. Reid; Lewis, Richard Alan; Van den Veyver, Ignatia; Clark, Gary

2008-01-01

112

Decreased left perisylvian GABA concentration in children with autism and unaffected siblings.  

PubMed

Imbalanced levels of excitation and inhibition (E/I) have been proposed to account for various behavioral and electrophysiological phenotypes in autism. Although proton magnetic resonance spectroscopy ((1)H-MRS) studies have been published on various metabolite levels in autism, including glutamate, the major excitatory neurotransmitter, few (1)H-MRS studies have yet been conducted the major inhibitory neurotransmitter GABA. Seventeen individuals with autism spectrum disorders (ASD) participated in a single-voxel, point resolved spectroscopy (PRESS) study conducted on a 3T magnet. Data were also acquired on 14 unaffected siblings of children with autism, and 17 age- and gender-matched healthy control subjects. GABA concentration was measured along with Creatine (Cr) in a single voxel aligned with the auditory cortex in the perisylvian region of the left hemisphere. The ratio of GABA to Cr was significantly lower in the ASD group than the control subjects. Siblings also exhibited lower GABA/Cr ratios compared to controls. Cr concentration did not differ between groups. The volumes of gray matter, white matter and CSF did not differ between groups in the whole brain or within the spectroscopy voxel. Reduced auditory GABA concentration in ASD is consistent with one previous MRS study of GABA concentration in the frontal lobe in autism, suggesting that multiple neocortical areas may be involved. Lower GABA levels are consistent with theories of ASD as a disorder involving impaired inhibitory neurotransmission and E/I imbalance. The reduction in unaffected siblings suggests that it may be a heritable biomarker, or endophenotype, of autism. PMID:23370056

Rojas, Donald C; Singel, Debra; Steinmetz, Sarah; Hepburn, Susan; Brown, Mark S

2014-02-01

113

Traumatic bilateral vertebral artery dissection.  

PubMed

Traumatic vertebral artery dissection is not often seen by forensic pathologists, and cases investigated are scarce in the forensic literature. We present the case of a 40-year-old woman cyclist who was struck by a car while wearing a helmet, and was neurologically near normal immediately thereafter at Emergency. She presented 48 h later with acute right hemiparesis, decreasing level of consciousness, and unsteadiness. CT revealed massive cerebellar infarction. CT angiography was normal. The patient died in coma 7 days after injury and autopsy revealed bilateral edematous cerebellar infarction and bilateral vertebral artery dissection. Rotational neck injury and mural tear in the wall of the Atlantic parts of both vertebral arteries is suggested as the possible mechanism of the arterial injury. Head and neck injuries are reported as a precipitating cause of vertebral artery injury. The possible influence of trauma may be further underestimated if longer intervals between vessel dissection and ischemia occur. The current case illustrates that "talk-and-die" syndrome may be due to occult vertebral artery dissection, possibly bilateral. In forensic cases of delayed death after mild trauma to the head and neck, the vertebral arteries should be examined for the cause of death. PMID:21798679

Galtés, Ignasi; Borondo, Juan Carlos; Cos, Mònica; Subirana, Mercè; Martin-Fumadó, Carles; Martín, Carles; Castellà, Josep; Medallo, Jordi

2012-01-10

114

Bilateral taurodontism in primary dentition with hypodontia.  

PubMed

Taurodontism is a rare dental anomaly in which there is an enlarged pulp chamber at the expense of roots with apical displacement of the pulpal floor, giving it a rectangular shape. It is caused by the failure of Hertwig's epithelial root sheath to invaginate at the proper horizontal level. Taurodontism has been reported as an intraoral feature of several syndromes like Down syndrome, Klinefelter syndrome, Smith-Magenis syndrome, Hurler syndrome, etc. Association of taurodontism with hypodontia in permanent dentition has also been reported. Taurodontism in primary dentition and its association with hypodontia is very rarely reported in the literature. The present case illustrates bilateral taurodontism of primary mandibular molars with hypodontia in maxilla. PMID:23345504

Surendar, Marappan Natarajan; Pandey, Ramesh Kumar; Khanna, Richa

2013-01-01

115

Bilateral Adrenal Incidentalomas: A Case Report and Review of Diagnostic Challenges  

PubMed Central

Incidentally discovered adrenal masses (incidentalomas) are common and present challenges both in diagnosis and management. When incidentally discovered adrenal masses are bilateral, a refined diagnostic approach is warranted since bilateral disease is more likely to be pathologic. We review a case of a 34-year-old man with incidentally discovered bilateral adrenal nodules. A comprehensive diagnostic strategy led to the diagnosis of bilateral pheochromocytoma caused by von Hippel-Lindau syndrome. He was successfully treated with bilateral laparoscopic adrenalectomy and has recovered well. While the initial diagnostic approach is similar to the unilateral incidentaloma, additional testing and/or genetic testing should be considered in the case of the bilateral adrenal mass.

Carlson, Anders L.; Marney, Annis M.; Anderson, Scott R.; Gilbert, Matthew P.

2013-01-01

116

Bilateral Optic Nerve Meningioma  

Microsoft Academic Search

A case of bilateral optic nerve meningioma is reported. The onset of the clinical symptoms, at age 27, resembled unilateral optic neuritis with papilledema, leading to bilateral amaurosis with optic atrophy 4 years later. Skull X-ray revealed a ‘blistering’ type of bone reaction. In the carotid angiogram, the ophthalmic artery appeared quite enlarged and displaced. The CT scan showed a

H. Liaño; C. Garcia-Alix; M. Lousa; M. Marquez; L. Nombela; J. de Miguel

1982-01-01

117

Bilateral Occipital Condyle Fracture  

PubMed Central

Occipital condyle fractures are a rare finding in trauma victims. Bilateral fractures are even more unusual and have typically been reported in autopsy studies. We treated two patients with bilateral occipital condyle fractures who had only minor symptoms. Anderson and Montesano's classification,1 possible cranial nerve palsies, diagnosis, and treatment of this rare fracture are discussed. ImagesFigure 1Figure 2

Schrodel, Markus H.; Kestlmeier, Ralph; Trappe, Anna E.

2002-01-01

118

Bilateral cochlear implantation.  

PubMed

Cochlear implantation (CI) is the standard of care for the treatment of children and adults with bilateral severe-to-profound sensorineural hearing loss. Because the ultimate and continuous goal of CI teams is to improve patient performance, a potential method is bilateral CI. The potential benefits of bilateral CI include binaural summation, squelch, equivalent head shadow for each ear, improved hearing in noise, sound localization ability, and spatial release from masking. The potential disadvantages include additional or prolonged surgical procedure, unproven cost/benefit profile, and the elimination of the ability to use future technologies and/or medical therapies in the implanted ear. PMID:22115683

Wanna, George B; Gifford, René H; McRackan, Theodore R; Rivas, Alejandro; Haynes, David S

2012-02-01

119

Role of bilateral adrenalectomy in Cushing's disease  

Microsoft Academic Search

Of 206 patients with Cushing's syndrome observed from 1975 through 1991, 144 (69.9%) had pituitary-dependent Cushing's disease. Of the 110 patients who underwent pituitary surgery, 31 (28%) developed an early recurrence and 23 patients (20%) had a late relapse 1 to 5 years after primary pituitary exploration. We performed a one- or two-step total bilateral adrenalectomy in 43 patients—9 men

Gennaro Favia; Marco Boscaro; Franco Lumachi; Davide F. D'Amico

1994-01-01

120

Bilateral acromial dimples: a case report and review of the literature.  

PubMed

Bilateral acromial dimples are uncommon in pediatric dermatology. They are usually found as a sporadic finding with limited clinical symptoms but may cause concern for parents. They can occur spontaneously or be inherited. Bilateral acromial dimples may occasionally be present as part of more complex syndromes. This article reports an 18-month-old girl with congenital bilateral acromial dimples and presents a classification of this unusual entity. A literature review of English and non-English publications was performed. We report the second case of bilateral acromial dimples seen in the context of maternal cocaine use during pregnancy. Our case is probably sporadic and nonsyndromal. We suggest that bilateral acromial dimples be classified as syndromal or nonsyndromal and that nonsyndromal cases be subdivided into inherited and sporadic. Although bilateral acromial dimples can be seen in 18q deletion syndrome, Apert syndrome, Say syndrome, and a recently described syndrome in Brazil, our review of the literature does not support the association with trisomy 9 syndrome. Dermatologists need to be aware of this unusual cutaneous finding and potential syndromal associations. The significance of the association with fetal exposure to cocaine during pregnancy has yet to be determined. The biological mother was not available for assessment for bilateral acromial dimples. PMID:23171068

Debosz, Joanna; Haber, Richard M

2014-05-01

121

Bilateral lunate intraosseous ganglia.  

PubMed

An intraosseous ganglion is a relatively uncommon, benign cystic lesion that occurs in young and middle-aged adults. Bilateral and symmetrical lesions of the wrist are rare. Intraosseous ganglia of the carpal bones are uncommon causes of chronic wrist pain. Isolated cases of intraosseous ganglion have been reported most commonly in the lunate and scaphoid. The lunate was most frequently affected, followed by the capitate, scaphoid, and triquetrum bones. Radiolucent lesions in the carpal bones are not uncommon and are often seen incidentally in asymptomatic patients. The differential diagnosis of a lytic lesion in a carpal bone includes unicameral bone cyst, degenerative cyst, fibrous developmental defect, osteomyelitis, and intraosseous ganglion cyst. This article describes a case of bilateral lunate intraosseous ganglia. A review of the literature revealed that bilateral and symmetrical intraosseous ganglia of the wrist are rare, with only 3 other reported cases of bilateral lunate lesions. PMID:20608626

Kural, Cemal; Sungur, Ibrahim; Cetinus, Ercan

2010-07-01

122

Bilateral Parotid Tuberculosis  

PubMed Central

Tuberculosis of parotid is a rare clinical entity, and cases of bilateral tubercular parotitis are even rarer. We present a case of bilateral primary parotid tuberculosis in a 49-year-old female. The patient received anti-tuberculosis treatment for six months, resulting in complete resolution of the disease. We also review the theories related to the pathogenesis of tubercular parotitis, and propose a novel hypothesis about greater involvement of parotid gland as compared to other salivary glands in primary tuberculosis.

Thakur, JS; Thakur, A; Mohindroo, NK; Mohindroo, S; Sharma, DR

2011-01-01

123

Echinoderms Have Bilateral Tendencies  

PubMed Central

Echinoderms take many forms of symmetry. Pentameral symmetry is the major form and the other forms are derived from it. However, the ancestors of echinoderms, which originated from Cambrian period, were believed to be bilaterians. Echinoderm larvae are bilateral during their early development. During embryonic development of starfish and sea urchins, the position and the developmental sequence of each arm are fixed, implying an auxological anterior/posterior axis. Starfish also possess the Hox gene cluster, which controls symmetrical development. Overall, echinoderms are thought to have a bilateral developmental mechanism and process. In this article, we focused on adult starfish behaviors to corroborate its bilateral tendency. We weighed their central disk and each arm to measure the position of the center of gravity. We then studied their turning-over behavior, crawling behavior and fleeing behavior statistically to obtain the center of frequency of each behavior. By joining the center of gravity and each center of frequency, we obtained three behavioral symmetric planes. These behavioral bilateral tendencies might be related to the A/P axis during the embryonic development of the starfish. It is very likely that the adult starfish is, to some extent, bilaterian because it displays some bilateral propensity and has a definite behavioral symmetric plane. The remainder of bilateral symmetry may have benefited echinoderms during their evolution from the Cambrian period to the present.

Zhao, Wenchan; Wang, Sishuo; Lv, Jianhao

2012-01-01

124

Echinoderms have bilateral tendencies.  

PubMed

Echinoderms take many forms of symmetry. Pentameral symmetry is the major form and the other forms are derived from it. However, the ancestors of echinoderms, which originated from Cambrian period, were believed to be bilaterians. Echinoderm larvae are bilateral during their early development. During embryonic development of starfish and sea urchins, the position and the developmental sequence of each arm are fixed, implying an auxological anterior/posterior axis. Starfish also possess the Hox gene cluster, which controls symmetrical development. Overall, echinoderms are thought to have a bilateral developmental mechanism and process. In this article, we focused on adult starfish behaviors to corroborate its bilateral tendency. We weighed their central disk and each arm to measure the position of the center of gravity. We then studied their turning-over behavior, crawling behavior and fleeing behavior statistically to obtain the center of frequency of each behavior. By joining the center of gravity and each center of frequency, we obtained three behavioral symmetric planes. These behavioral bilateral tendencies might be related to the A/P axis during the embryonic development of the starfish. It is very likely that the adult starfish is, to some extent, bilaterian because it displays some bilateral propensity and has a definite behavioral symmetric plane. The remainder of bilateral symmetry may have benefited echinoderms during their evolution from the Cambrian period to the present. PMID:22247765

Ji, Chengcheng; Wu, Liang; Zhao, Wenchan; Wang, Sishuo; Lv, Jianhao

2012-01-01

125

[Bilateral Wilms tumor].  

PubMed

The incidence of bilateral involvement it is generally estimated to be 5% to 10%. It shows association with certain congenital anomalies and it has an increased occurrence of familial cases. The records of 9 children (5 boys, 4 girls) diagnosed at Vall d'Hebron Hospital with bilateral Wilms tumor between 1976-1995 were analyzed. Six patients had synchronous tumors and 3 had metachronous lesions. Genitourinary malformations were present in 4 children and another had hemihypertrophy. Two children were brothers. Eight patients underwent pre-operative radiation therapy and/or chemotherapy. Five patients had nephrectomy on one side (3 of them had metachronous presentation) and partial nephrectomy on the other side. The other children had bilateral partial nephrectomy or tumorectomy. Seven out of the nine patients are alive (78%). The two children who died presented with stage IV tumors and high grade malignant. One boy suffers cardiomyopathy. All survivors have normal renal function. With the proven efficacy of chemotherapy, bilateral renal salvage procedures were demonstrated to be effective in controlling disease without compromising renal function or survival. The innovative approaches developed for the treatment of bilateral Wilms tumor may influence the treatment of unilateral Wilms. PMID:9376233

Presedo, A; Martínez Ibáñez, V; Marqués, A; Sánchez de Toledo, J; Boix Ochoa, J

1997-07-01

126

Bilateral medial medullary infarction: a systematic review.  

PubMed

Bilateral infarction of the medial medulla (MMI) is rare. Limited information is available on clinical characteristics, etiology, and prognosis. High-resolution neuroimaging has a major role in elucidating the underlying stroke mechanism. The aim of this systematic review was to analyze the clinical presentations, stroke mechanisms, and outcomes in patients with bilateral MMI. We performed a systematic review of the literature from 1992-2011 that reported on clinical presentations, stroke mechanism, and/or outcomes in patients with magnetic resonance imaging-proven bilateral MMI. Medline, EMBASE, and Web of Science Scholars Portal were searched without language restriction. Two reviewers independently assessed identified studies to determine eligibility, validity, and quality. The primary outcome was inpatient mortality; a secondary outcome was case fatality at 12 months. We identified 138 articles from Medline, EMBASE, and Scholars Portal including the MeSH terms "brainstem infarction," "medulla," and "bilateral." Twenty-nine articles met our inclusion criteria, including a total of 38 cases with bilateral MMI, and included in our study. These 38 patients had a mean age of 62.2 years and were predominately male (74.2%). The most common clinical presentations were motor weakness in 78.4%, dysarthria in 48.6%, and hypoglossal palsy in 40.5%. The most common vascular pathology was vertebral artery atherosclerosis, in 38.5%. The clinical outcome was poor (mortality, 23.8%; dependency, 61.9%). Bilateral medial medullary infarction is a rare stroke syndrome. Clinical presentations were mostly rostral medullary lesions. Large-artery atherosclerosis and branch disease were the most common stroke mechanisms. The clinical outcome was usually poor. PMID:22541608

Pongmoragot, Jitphapa; Parthasarathy, Sujatha; Selchen, Daniel; Saposnik, Gustavo

2013-08-01

127

Surgical treatment of bronchiectasis in Kartagener's syndrome.  

PubMed

A case of Kartagener's syndrome in which bilateral partial pulmonary resection was performed is reported. The literature concerning the surgical treatment of severe bronchiectasis is reviewed. PMID:1084204

Michel, L; Lantin, F; Vandeperre, J

1976-06-01

128

Bilateral globus pallidus lesions.  

PubMed

Following a hypoxic-ischemic insult, the globus pallidus is selectively spared from ischemic injury in contrast to the caudate and putamen. The known causes for hemorrhagic and necrotic lesions selective for injuring the globus pallidus are varied but few. The most widely known etiology is in fatal cases of carbon monoxide poisoning. Additionally reported associations include fatalities involving 3,4-methylenedioxymethamphetamine; cocaine; opiates; and cyanide poisoning. These bilateral globus palladus necrotic lesions have been reported to occur in 5-10% of opiate addicts. In this article, we present a striking photo of selective symmetrical bilateral globus pallidus necrosis following cocaine and opiate abuse. PMID:22866355

Alquist, Caroline Raasch; McGoey, Robin; Bastian, Frank; Newman, William

2012-01-01

129

Bilateral lichen striatus.  

PubMed

We describe a very rare case of bilateral lichen striatus on the lower extremities with a history of more than ten years. Histopathologically, the lesions demonstrated a lichenoid tissue reaction with foci of spongiosis and perivascular inflammatory cell infiltration. In addition, the finding of lymphocytic infiltrations around the eccrine duct was observed. They were treated successfully with topical application of corticosteroid ointment. To the best of our knowledge, no other lichen striatus case has been reported with bilateral distribution and such long-term persistence. PMID:15160869

Kurokawa, Motoki; Kikuchi, Hidezumi; Ogata, Katsumi; Setoyama, Mitsuru

2004-02-01

130

Bilateral ovarian Burkitt's lymphoma.  

PubMed

Primary ovarian lymphoma is a rare entity. We submit a case of a 34-year-old black patient presenting with a bilateral adnexal tumor. She underwent hysterectomy with double salpingo-oophorectomy followed by polychemotherapy treatment. Histology confirmed Epstein-Barr virus-positive bilateral Burkitt's lymphoma. The patient died from septic shock after a month of treatment. Endemic Burkitt's lymphoma has a predilection for the female genital tract, manifesting itself clinically as a pelvic mass and less frequently as a menstrual disorder. It is a rare entity in our environment but should be kept in mind when treating patients of African origin. PMID:19480266

Gutiérrez-García, L; Medina Ramos, N; García Rodríguez, R; Barber, M A; Arias, M D; García, J A

2009-01-01

131

Bilateral Bowen's disease.  

PubMed

Multiple Bowen's disease may be difficult to differentiate from bowenoid papulosis because of its clinicopathological resemblance to bowenoid papulosis. We experienced a case of bilaterally and symmetrically developed multiple bowenoid lesions in a 71-year-old man previously diagnosed as having chronic lymphocytic lymphoma (CLL). Based on histological findings and the results of human papillomavirus examinations, we finally diagnosed this case as bilateral Bowen's disease. We speculate that the underlying immunosuppressive state due to CLL may have been associated with onset of the disease. We report the unique clinical picture, the differential diagnosis and the aetiology. PMID:15270896

Nishimura, Y; Kishigawa, T; Tanaka, T

2004-07-01

132

An atypical patient with Cowden syndrome and PTEN gene mutation presenting with cortical malformation and focal epilepsy.  

PubMed

We report the case of a girl with Cowden syndrome (CS) presenting with unilateral perisylvian dysplasia and with drug resistant focal seizures carrying a novel missense mutation 385G>A (G129R) in the PTEN gene. CS has been rarely reported in association with a cortical malformation or epilepsy. These cases suggest that cortical dysplasia needs to be suspected when a CS patient presents with drug-resistant seizures. PMID:22469695

Elia, Maurizio; Amato, Carmelo; Bottitta, Maria; Grillo, Lucia; Calabrese, Giuseppe; Esposito, Maria; Carotenuto, Marco

2012-11-01

133

Bilateral vocal fold chondromas.  

PubMed

Laryngeal chondromas are uncommon, benign, slow-growing neoplasms with few reports in the literature. Vocal fold chondromas are even more rare, and all reported cases are unilateral. Here, we present the first case of bilateral vocal fold chondromas. Detailed evaluation, careful resection with phonomicrosurgery technique, and perioperative voice therapy are considered essential for the management. PMID:23406842

Lai, Ying-Ta; Petty, Brian E; Huang, Wei; Dailey, Seth H

2013-03-01

134

Treatment of bilateral urolithiasis.  

PubMed

Patients with bilateral urolithiasis diagnosed at their first visit were followed for at least one year after the start of treatment. In this retrospective study of 123 patients, the basic metabolic workup revealed no specific underlying cause of simultaneously occurring bilateral nephrolithiasis, and none of the currently used therapeutic regimes proved to be efficient in attaining clinically acceptable stone-free rates at the 3, 6 and 12-month follow-ups. Of 38 patients treated with extracorporeal shockwave lithotripsy (SWL), 21 underwent treatment of both kidneys and 10 (48%) were free of stones bilaterally after 12 months. All of the 17 patients treated with unilateral SWL failed to achieve a stone-free state on the contralateral side. In another group, treated with medication alone to minimize risks of stone recurrence, only 3 of 26 (11.5%) patients were stone-free on both sides 12 months after the start of medication. Since we achieved a stone-free state of both kidneys in no more than 12% of the non-medicated cases, it seems warranted to treat bilateral urolithiasis with SWL more frequently, particularly when patients cannot return regularly to the stone clinic for a longterm follow-up. PMID:1343424

Matsushita, K; Tanikawa, K; Masuda, A; Matsunaga, J; Matsuzaki, S

1992-12-01

135

Bilateral intraocular dirofilariasis  

PubMed Central

Ocular dirofilariasis mostly presents as a subconjunctival or eyelid lesion.[1] Intraocular dirofilarial infestation is rare.[23] We report a case of a young woman who was accidentally detected to have a live motile worm in the anterior segment in one eye and a cystic lesion on the optic disc in the other eye. To our knowledge, bilateral intraocular dirofilariasis has never been reported.

Gupta, Viney; Sankaran, Preeti; Mohanraj; Samantaray, Jyotish Chandra; Menon, Vimla

2014-01-01

136

Isolated severe bilateral bronchomalacia.  

PubMed

Airway malacia is uncommon condition having symptoms similar to common respiratory illnesses. Any child having persistent wheeze during infancy should be evaluated for airway malacia. The authors report a case of isolated severe bilateral bronchomalacia managed with tracheostomy and continuous positive pressure ventilation. PMID:23715795

Saikia, Bhaskar; Sharma, Pradeep Kumar; Sharma, Rachna; Gagneja, Vikram; Khilnani, Praveen

2014-07-01

137

Somato-motor haptic processing in posterior inner perisylvian region (SII/pIC) of the macaque monkey.  

PubMed

The posterior inner perisylvian region including the secondary somatosensory cortex (area SII) and the adjacent region of posterior insular cortex (pIC) has been implicated in haptic processing by integrating somato-motor information during hand-manipulation, both in humans and in non-human primates. However, motor-related properties during hand-manipulation are still largely unknown. To investigate a motor-related activity in the hand region of SII/pIC, two macaque monkeys were trained to perform a hand-manipulation task, requiring 3 different grip types (precision grip, finger exploration, side grip) both in light and in dark conditions. Our results showed that 70% (n?=?33/48) of task related neurons within SII/pIC were only activated during monkeys' active hand-manipulation. Of those 33 neurons, 15 (45%) began to discharge before hand-target contact, while the remaining neurons were tonically active after contact. Thirty-percent (n?=?15/48) of studied neurons responded to both passive somatosensory stimulation and to the motor task. A consistent percentage of task-related neurons in SII/pIC was selectively activated during finger exploration (FE) and precision grasping (PG) execution, suggesting they play a pivotal role in control skilled finger movements. Furthermore, hand-manipulation-related neurons also responded when visual feedback was absent in the dark. Altogether, our results suggest that somato-motor neurons in SII/pIC likely contribute to haptic processing from the initial to the final phase of grasping and object manipulation. Such motor-related activity could also provide the somato-motor binding principle enabling the translation of diachronic somatosensory inputs into a coherent image of the explored object. PMID:23936121

Ishida, Hiroaki; Fornia, Luca; Grandi, Laura Clara; Umiltà, Maria Alessandra; Gallese, Vittorio

2013-01-01

138

Somato-Motor Haptic Processing in Posterior Inner Perisylvian Region (SII/pIC) of the Macaque Monkey  

PubMed Central

The posterior inner perisylvian region including the secondary somatosensory cortex (area SII) and the adjacent region of posterior insular cortex (pIC) has been implicated in haptic processing by integrating somato-motor information during hand-manipulation, both in humans and in non-human primates. However, motor-related properties during hand-manipulation are still largely unknown. To investigate a motor-related activity in the hand region of SII/pIC, two macaque monkeys were trained to perform a hand-manipulation task, requiring 3 different grip types (precision grip, finger exploration, side grip) both in light and in dark conditions. Our results showed that 70% (n?=?33/48) of task related neurons within SII/pIC were only activated during monkeys’ active hand-manipulation. Of those 33 neurons, 15 (45%) began to discharge before hand-target contact, while the remaining neurons were tonically active after contact. Thirty-percent (n?=?15/48) of studied neurons responded to both passive somatosensory stimulation and to the motor task. A consistent percentage of task-related neurons in SII/pIC was selectively activated during finger exploration (FE) and precision grasping (PG) execution, suggesting they play a pivotal role in control skilled finger movements. Furthermore, hand-manipulation-related neurons also responded when visual feedback was absent in the dark. Altogether, our results suggest that somato-motor neurons in SII/pIC likely contribute to haptic processing from the initial to the final phase of grasping and object manipulation. Such motor-related activity could also provide the somato-motor binding principle enabling the translation of diachronic somatosensory inputs into a coherent image of the explored object.

Ishida, Hiroaki; Fornia, Luca; Grandi, Laura Clara; Umilta, Maria Alessandra; Gallese, Vittorio

2013-01-01

139

Congenital diaphragmatic eventration and bilateral ureterohydronephrosis in a patient with neonatal Marfan syndrome caused by a mutation in exon 25 of the FBN1 gene and review of the literature  

Microsoft Academic Search

Neonatal Marfan syndrome, the most severe presentation of Marfan syndrome phenotypes (MIM 154700), is characterised mainly by joint contractures, arachnodactyly, loose skin, crumpled ears, severe atrioventricular valve dysfunction and pulmonary emphysema. Death usually occurs within the first 2 years of life from congestive heart failure. We describe here a newborn male with many typical characteristics of neonatal Marfan syndrome associated

Nicole Revencu; Geneviève Quenum; Thierry Detaille; Gaston Verellen; Anne De Paepe; Christine Verellen-Dumoulin

2004-01-01

140

Susac syndrome  

Microsoft Academic Search

PURPOSE: To report the first Japanese case of the Susac syndrome characterized by microangiopathy of the brain, retina, and cochlea.METHODS: Case report. A 36-year-old Japanese man underwent extensive clinical and laboratory examination for encephalopathy, bilateral recurrent branch retinal arterial occlusion, and hearing loss.RESULTS: This case was initially diagnosed as acute disseminated encephalomyelitis. Despite exhaustive clinical and laboratory examination of the

Yasuhiro Murata; Koichiro Inada; Akira Negi

2000-01-01

141

Bilateral Suspected Tuberculous Empyema Thoracis  

PubMed Central

Empyema thoracis is a well known complication following para-pneumonic effusions in paediatric age group. Usually it is unilateral but rarely could be bilateral. Herein we report a case of bilateral tuberculous empyema thoracis in a 12 years old, unvaccinated girl with a positive history of contact with tuberculosis. She was managed conservatively with tube thoracostomies and anti-tuberculous drugs. Emphasis is on the conservative approach and patience in management of patients with bilateral empyema thoracis.

2012-01-01

142

A 52-Year-Old Male with Bilaterally Duplicated Collecting Systems with Obstructing Ureteral Stones: A Case Report  

PubMed Central

Collecting system duplication is marked by a variety of clinical syndromes. Bilateral and obstructed duplicated systems, particularly with asymmetric levels of duplication, are rare and typically due to ureteric bud development anomalies. The infrequency with which this condition exists makes it a formidable challenge for physicians and patients. To our knowledge, we present the first case report of bilateral obstruction of bilaterally duplicated collecting systems. In our case, a 52-year-old male complaining of low back pain, constipation, urinary urgency and hematuria was found to have bilateral obstructing stones as well as asymmetrical bilateral collecting system duplication. We discuss the natural history of this condition, its consequences and identification.

Scantling, Dane; Ross, Curtis; Altman, Howard

2013-01-01

143

Bilateral macrostomia associated with aqueductal stenosis and glial heterotopias.  

PubMed

We report on an Italian boy, born to normal and nonconsanguineous parents with a prenatal diagnosis of ventriculomegaly and subependymal glial heterotopias. At birth bilateral macrostomia was diagnosed without other evident facial anomalies. Magnetic resonance imaging (MRI) showed triventricular hydrocephalus and aqueductal stenosis and confirmed the nodules of glial heterotopia. The bilateral macrostomia was surgically corrected with the vermilion square flap method and W-plasty technique and follow up MRI at 6 months showed mild increase of ventricular dilatation without signs of active hydrocephalus. The association between macrostomia and hydrocephalus has been reported only in rare cases of complex malformative syndromes but never with isolated macrostomia. PMID:17760484

Pepe, Ernesto; Petricig, Paola; Peretta, Paola; Cinalli, Giuseppe

2007-09-01

144

[Ophthalmoplegia with bilateral mydriasis].  

PubMed

We report the case history of a 41-year-old patient who, following bronchial infection, presented with progressive ophthalmoplegia in both eyes, dilated pupils unresponsive to light or convergence and ataxia. The suspected diagnosis of Miller-Fisher syndrome was confirmed by the detection of anti-ganglioside GQ1b antibodies. PMID:22130723

Jehle, T; Puvanachandra, N

2012-01-01

145

[Diagnosing bilateral papilledema].  

PubMed

Bilateral optic disc swelling requires following a number of steps from discovery to causal diagnosis. First, it is necessary to differentiate between true optic disc swelling and disc elevation without true swelling. Then fundus examination, visual acuity and visual field, fluorescein angiography, and optical coherence tomography are performed in order to differentiate papilledema secondary to increased intracranial pressure from optic disc swelling secondary to optic neuropathy. Even if the most frequent etiology is idiopathic intracranial hypertension, the clinician must check for the absence of any signs or symptoms related to hypertension secondary to a cerebral tumor or to cerebral venous thrombosis. Fortunately, modern imaging techniques have facilitated the differential diagnoses of optic disc swelling, and the combination of magnetic resonance imaging (MRI) and magnetic resonance venography appears to be necessary each time the diagnosis of idiopathic hypertension is suggested. PMID:20493584

Rougier, M-B

2010-06-01

146

Bilateral transaxillary endoscopic total thyroidectomy  

Microsoft Academic Search

Background\\/PurposeMinimal-access thyroid surgery using various techniques is well described. The present study reviews our initial experience with total thyroidectomy using a robotic-assisted bilateral transaxillary endoscopic approach (R-BAEA) and a non–robotic-assisted bilateral transaxillary endoscopic approach (BAEA) to assess it's safety and feasibility.

Go Miyano; Thom E. Lobe; Simon K. Wright

2008-01-01

147

Potter syndrome with an unusual cardiac anomaly  

PubMed Central

Potter syndrome is a congenital anomaly characterised by bilateral renal agenesis, pulmonary hypoplasia, cardiac, skeletal abnormalities and maternal oligohydramnios. Here we report a case of Potter syndrome with bilateral renal agenesis, pulmonary hypoplasia and complete transposition of the great vessels, which had been identified during a post-mortem examination. Although cardiac anomalies are known to exist with Potter syndrome, complete transposition of the great vessels has not been reported in the literature.

Prabhu, Savit; Sigamani, Elanthenral; Das, Prasenjit; Sasi, Arun; Safaya, Rajni

2009-01-01

148

Glycopyrrolate induced bilateral angle closure glaucoma after cervical spine surgery.  

PubMed

To report a case of bilateral acute angle closure glaucoma (AACG) that occurred after cervical spine surgery with the use of glycopyrolate. A 59-year-old male who presented with severe bilateral bifrontal headache and eye pain that started 12 h postextubation from a cervical spine surgery. Neostigmine 0.05 mg/kg (4.5 mg) and glycopyrrolate 0.01 mg/kg (0.9 mg) were used as muscle relaxant reversals at the end of the surgery. Ophthalmic examination revealed he had bilateral AACG with plateau iris syndrome that was treated medically along with laser iridotomies. Thorough examination of anterior chamber should be performed preoperatively on all patients undergoing surgeries in the prone position and receiving mydriatic agents under general anesthesia. PMID:23741140

Jaroudi, Mahmoud; Fadi, Maalouf; Farah, Fadi; El Mollayess, Georges M

2013-01-01

149

Bilateral pseudogout 8 years after bilateral total knee arthroplasty.  

PubMed

This article presents the clinical features of crystal arthropathy after knee replacement. The current literature on pseudogout and gout after both total and partial knee replacement is summarized. A case of bilateral pseudogout 8 years after initial total knee arthroplasty (TKA) is used to highlight the clinical characteristics and treatment options for this underrecognized condition. Presentation mimicked a late septic joint arthroplasty with sudden onset of pain and effusion. The patient was treated successfully with an arthrotomy, debridement, synovectomy, polyethylene insert exchange, oral steroids, and nonsteroidal anti-inflammatories. There are no other reported cases of bilateral pseudogout after bilateral TKA. PMID:23102423

Levi, Gabriel S; Sadr, Kamran; Scuderi, Giles R

2012-11-01

150

Cerebral fat embolism after bilateral total knee replacement arthroplasty -A case report-  

PubMed Central

Fat embolism syndrome is a rare and potentially lethal complication most commonly seen in long bone fractures and intramedullary manipulation. The clinical triad of fat embolism syndrome consists of mental confusion, respiratory distress, and petechiae. This study reports a case of cerebral fat embolism syndrome following elective bilateral total knee replacement. After an uneventful anesthesia and initial recovery, the patient developed neurologic symptoms nine hours postoperatively.

Chang, Ri-Na; Lee, Heeseung; Baik, Hee-Jung; Chung, Rack Kyung; Kim, Chi Hyo; Hwang, Tae-Hu

2010-01-01

151

Acute silicosis with bilateral pneumothorax.  

PubMed

We present a case of acute silicosis with bilateral pneumothorax of a 28-year-old man working at a stone crusher factory for 1?year. He presented to the emergency department with cough, respiratory distress and diffuse chest pain. The patient was managed with bilateral intercostal tube drainage under water seal, oxygen inhalation and conservative therapy. On follow-up he showed improvement of resting dyspnoea and was doing well. This case is being reported because of the rare complications of acute silicosis as bilateral pneumothorax. PMID:24862410

Srivastava, G N; Prasad, Rajniti; Meena, Manoj; Hussain, Moosa

2014-01-01

152

Bilateral hypoglossal nerve injury after bilateral carotid endarterectomy.  

PubMed

A case of severe bilateral injury to the hypoglossal nerves after two-stage carotid endarterectomy is described. Injury to the hypoglossal nerve occurs in up to 20% of patients undergoing carotid endarterectomy and may result in mild or unnoticed deficits. These injuries must be carefully searched for in patients who will undergo a similar procedure on the opposite side since a bilateral deficit of the hypoglossal nerve is poorly tolerated, causing potentially serious impairment of speech and risk of aspiration. PMID:3278413

Gutrecht, J A; Jones, H R

1988-02-01

153

Severe megacystis and bilateral hydronephrosis in a female fetus.  

PubMed

The differential diagnosis for antenatal bladder dilation and hydronephrosis is extensive, ranging from posterior urethral valves to neurogenic bladder. We present a case of a female fetus diagnosed antenatally with severe megacystis and bilateral hydroureteronephrosis. The patient was found to have megacystis-microcolon-intestinal hypoperistalsis syndrome, a rare, congenital, and generally fatal disorder in which there is functional obstruction of the bladder and intestine. PMID:12385942

Bloom, Tammy L K; Kolon, Thomas F

2002-10-01

154

Orbital emphysema following removal of bilateral midfacial implants.  

PubMed

A 36-year-old man with Crouzon syndrome was referred post-operatively with unilateral painful limitation of eye movements and proptosis following removal of bilateral malar and orbital rim implants. Further investigation revealed right inferior orbital emphysema, caused by the intra-operative entrance of hydrogen peroxide through a suspected perforation of the orbital septum. The symptoms settled with conservative management. PMID:21977963

Brettell, Daniel; Ooi, Kenneth G-J; Bank, Allan; Gianoutsos, Mark P

2011-12-01

155

Spontaneous bilateral internal carotid artery dissection  

PubMed Central

Spontaneous internal carotid artery dissection is not an uncommon cause of ischaemic stroke in younger patients, but multiple cervical arterial dissections at presentation are uncommon. Recurrence of dissection in a previously normal artery is common. In this case report we review the history, clinical findings and management of a 42-year-old woman who presented with stroke and Horner syndrome and was found to have spontaneous bilateral internal carotid artery dissection. She was not anticoagulated due to concerns relating to the size of her infarct. She was treated with a combination of aspirin and clopidogrel. We use dual antiplatelets for the management of cervical dissections as a part of the CADISS trial. The patient made good progress with the multidisciplinary team and was discharged on day 22 with support from the community stroke team.

Ardhalapudi, Srujan; Addy, Victoria; Da Costa, David

2009-01-01

156

Bilateral internal laryngoceles mimicking asthma  

PubMed Central

Laryngocele is an air-filled, abnormal dilation of the laryngeal saccule that extends upward within the false vocal fold, in communication with the laryngeal lumen. A case of 43-year-old male with bilateral internal laryngoceles, who has been treated as asthma for 4 years, is presented. The patient had dyspnea, cough, and excessive phlegm for a month and a late onset stridor. Flexible nasopharyngolaryngoscopy showed bilateral cystic enlargements of the false vocal folds and true vocal folds could not be visualized. Laryngeal CT without contrast enhancement showed bilateral internal laryngoceles. Submucosal total excision of bilateral cystic masses including parts of false vocal folds was performed. The symptoms resolved immediately after surgery. Although the incidence of internal laryngocele is rare, it should be remembered in the differential diagnosis of upper airway problems and diagnostic flexible nasopharnygolaryngoscopy is routinely indicated for airway evaluation in at-risk patients.

Aksoy, Elif A.; Elsurer, Cagdas; Serin, Gediz M.; Unal, O. Faruk

2013-01-01

157

[Bilateral sarcoidosis of parotid glands].  

PubMed

We describe an unusual case of sarcoidosis in which the patient presented with a bilateral swelling of the parotid salivary glands and no other manifestation of the disease. Sarcoidosis is a multisystem granulomatous disorder of unknown cause in which there may be multiple exocrine involvement, including the salivary glands. This case emphasises the importance of including sarcoidosis in the differential diagnosis of bilateral parotid swelling. PMID:22533935

Hahn, Pernille; Krogdahl, Annelise; Godballe, Christian

2012-04-23

158

Diffuse and bilateral lichen striatus.  

PubMed

A 5-year-old girl had a papulo-squamous dermatosis arranged in a Blaschko line pattern of 1 month's duration. The skin lesions were asymmetrically distributed bilaterally on the face, neck, trunk, and limbs. Histologically there was a lichenoid infiltrate with foci of spongiosis. The dermatosis cleared spontaneously with hypopigmentation 16 months later. Lichen striatus usually occurs in a solitary and unilateral fashion. Its diffuse and bilateral distribution is an uncommon presentation. PMID:9050762

Aloi, F; Solaroli, C; Pippione, M

1997-01-01

159

Bilateral Polydactyly in a foal  

PubMed Central

The following case report describes the diagnosis and surgery of bilateral polydactyly of unknown origin in a colt. A 7-month-old Berber colt was referred for cosmetic and curative excision of supernumerary digits. Radiographic examination revealed bilateral polydactyly and well-developed first carpal bones. Surgery consisted of an osteotomy of both second metacarpal bones combined with an amputation of the supernumerary digits. The follow-up at 18 months after surgery revealed a sound horse with an excellent cosmetic outcome.

Abitbol, Marie; Desbois, Christophe

2007-01-01

160

Bilateral progressive hearing loss and vestibular dysfunction with inner ear antibodies  

Microsoft Academic Search

Autoimmune inner ear disease (AIED) is a clinical syndrome of uncertain etiology. We present the neuro-otological findings of 2 cases of bilateral hearing loss, dizziness and the antibody profiles of the inner ears. Case 1 had bilateral progressive hearing loss, vestibular dysfunction and abnormal eye movement as the disease progressed. She had inner ear antibodies against 42 and 58kDa protein

Kumiko Yukawa; Akira Hagiwara; Yasuo Ogawa; Nobuhiro Nishiyama; Shigetaka Shimizu; Sachie Kawaguchi; Mari Nakamura; Hiroyuki Ito; Shunichi Tomiyama; Mamoru Suzuki

2010-01-01

161

Löfgren's syndrome misdiagnosed as cellulitis.  

PubMed

Löfgren's syndrome is an acute-onset presentation of sarcoidosis that can be easily missed in an ED setting. A case is reported of Löfgren's syndrome presenting as erythema nodosum with bilateral ankle oedema. Although rare, this diagnosis should be considered when examining a patient with erythema nodosum and articular symptoms. PMID:21668726

Cheng, Daryl Ray; Maini, Amit

2011-06-01

162

Unilateral hyperhydrosis in Pourfour du Petit syndrome  

Microsoft Academic Search

Upper limp hyperhydrosis is an idiopathic disease with bilateral involvement. However, Pourfour du Petit syndrome, the opposite of Horner syndrome, may result in unilateral upper limb hyperhydrosis. It occurs following hyperactivity of the sympathetic cervical chain as a consequence of irritation secondary to trauma. We report herein two cases with Pourfour du Petit syndrome showing unilateral upper limb hyperhydrosis. The

Murat Kara; Erkan Dikmen; Cengiz Akarsu; Ahu Birol

2004-01-01

163

Unusual bilateral dentigerous cysts in a nonsyndromic patient assessed by cone beam computed tomography  

PubMed Central

In the absence of syndromes, bilateral dentigerous cysts (DC) located on the jaws are unusual. In English based language literature review, we only found eight reports of nonsyndromic bilateral dentigerous cyst associated with mandibular third molars. Therefore, we report the unusual occurrence of sizable nonsyndromic bilateral DC associated with mandibular impacted third molars in a 42-year-old Caucasian woman. The lesions were assessed by cone beam computed tomography (CBCT) the right lesion showed approximately 23.64 mm and the left one, 16.57 mm diameter, both located intimately next to the mandibular canal. Bilateral surgical enucleation, related teeth excision of both third molars and plate for fixation placement on the right and bigger lesion, under general anesthesia was the final treatment choice. Clinical, radiographic and histopathological features confirmed diagnose of bilateral dentigerous cyst. Now-a-days, the patient is on 18 months radiograph follow-up with favorable osseous formation with no evidence of recurrence of the cysts.

Imada, Thais Sumie Nozu; Neto, V. Tieghi; Bernini, G. F.; Silva Santos, P. S.; Rubira-Bullen, I. R. F.; Bravo-Calderon, D.; Oliveira, D. T.; Goncales, E. S.

2014-01-01

164

Retinal Infarcts in a Patient with an Acute Confusional Syndrome  

Microsoft Academic Search

A 46-year-old man presented with acute confusional syndrome, ataxia, dysarthria, and right hemiparesthesia. Brain MRI showed small bilateral infarcts and fluorescein angiography revealed multiple peripheral retinal infarcts bilaterally. No visual loss was present, and no other organs were involved. The diagnosis of Susac syndrome (microangiopathy of the brain, retina and cochlea) was made and immunosuppressive therapy begun.

Elena Bitrian; Bernardo Sanchez-Dalmau; Molly E. Gilbert; Alfredo Adan; John O. Susac

2009-01-01

165

Achondroplasia Associated with Bilateral Keratoconus  

PubMed Central

We report a rare case of bilateral keratoconus in association with achondroplasia. A 26-year-old male, with a known case of achondroplasia, complained of bilateral gradual deterioration in vision for the past few years. Slit lamp biomicroscopy showed bilateral central corneal protrusion and stromal thinning at the apex consistent with keratoconus. a trial of hard contact lens fitting failed to improve VA in the left eye (LE). Right eye (RE) improved to 20/25. The patient underwent penetrating keratoplasty (PKP) in his LE. Twenty-seven months postoperatively, uncorrected visual acuity (UCVA) was 20/30. Ophthalmologists should be aware that patients with achondroplasia who complain of poor vision should be suspected of having keratoconus once other more common conditions are ruled out.

Al Mahmood, Ammar M.; Al Katan, Hind M.; Al Bin Ali, Ghada Y.; Al-Swailem, Samar A.

2012-01-01

166

Congenital patellar syndrome.  

PubMed

Congenital patellar syndrome is bilateral isolated absence of patella. Congenital patellar aplasia or hypoplasia associated with genetic disorders belongs to a clinically diverse and genetically heterogeneous group of lower limb malformations. Absence of patella as an isolated anomaly is extremely rare and we discuss such a case in a 9-year-old boy. PMID:19434325

Jerome, J Terrence Jose; Varghese, M; Sankaran, B

2009-01-01

167

Syndrome In Question*  

PubMed Central

The authors present a male 40-year-old patient with established diagnosis of Behçet's disease which had evolved to recurrent bilateral auricular polychondritis crises. MAGIC syndrome (mouth and genital ulcers with inflamed cartilage) is rare and groups together patients with this clinical picture without necessarily fulfilling the clinical criteria for Behçet's disease or relapsing polychondritis, demonstrating an independent disorder.

do Nascimento, Ana Claudia Mendes; Gaspardo, Daniela Barros Cortez; Cortez, Tatiana Mimura; Miot, Helio Amante

2014-01-01

168

Carpal Tunnel Syndrome as Manifestation of Sarcoidosis.  

National Technical Information Service (NTIS)

Two patients with bilateral carpal tunnel syndrome are reported. One had coeistent psoriasis raising the question as to the etiology of his joint, muscle, and carpal tunnel manifestations. The other had mild diabetes mellitus. In both, evidence favored th...

G. E. Shambaugh W. J. Cirksena K. L. Newcomer

1964-01-01

169

The first Susac's syndrome case in Turkey  

Microsoft Academic Search

Susac's syndrome is a vasospastic disease resulting from bilateral microangiopathy of the brain, cochlea and retina. It is characterized by encephalopathy, bilateral sensorineural fluctuating hearing loss and visual loss. It is very uncommon and usually affects women during young adulthood. Since all three symptoms of the triad may not be present, the clinical diagnosis is difficult. Therefore, neuroimaging, particularly magnetic

Sevda Erer; Meral Boz; Ozlem Taskapilioglu; Bahattin Hakyemez; Berkant Kaderli; Mehmet Zarifoglu

2006-01-01

170

[Bilateral carotid endarterectomy for patients with bilateral carotid artery stenosis].  

PubMed

In a couple of recent randomized trials, the benefits of unilateral carotid endarterectomy (CEA) have been reevaluated in symptomatic patients with severe stenosis. In contrast, the operative indication, procedure, and perioperative management of bilateral CEAs for patients with bilateral carotid artery stenosis are still controversial. In this report, we reviewed 7 patients who underwent bilateral CEAs at out institute during the last 10 years, with regard to the clinical feature, angiographical findings, operative procedure, surgical results and long-term prognosis. The patients ranged from 52 to 73 years in age, and included six males and one female. Clinical symptoms were asymptomatic in 1 patient, transient ischemic attack in 2, reversible ischemic neurological deficits in 2, minor completed stroke in 1, and major completed stroke in 1. The angiographical carotid artery stenosis in the dominant side of symptomatic cases was 50% in 3, 70% in 1, 90% in 2, and ulceration in 4 cases. The stenosis in the non-dominant side of symptomatic cases was 60% in 1, 70% in 3, 90% in 2, and 4 cases with ulceration. One case among the asymptomatic cases had bilateral 80-90% stenosis. We staged bilateral CEAs, in the dominant side first except in one case among the symptomatic cases and on the more severely stenotic side first in the asymptomatic cases. During CEA, an external shunt was placed in 1 case, but no internal shunt was used in any of the cases. Perioperative complications were found in 2 patients, transient bilateral hypoglossal nerve palsy and local hemorrhage in the other case. Totally, all of 7 cases (14 consecutive CEAs) have been performed with satisfactory results. No mortality and no permanent morbidity has resulted. In the follow-up period (mean: 38.3 month), 1 patient was found to have developed cerebral infarction in the ipsilateral carotid artery territory. From our own small experience and from that in the literature, CEAs for bilateral carotid artery stenosis should be performed in the dominant side first. Then, after a certain period, from 2 to 6 weeks, the CEA should be performed in the non-dominant side. PMID:8914146

Sato, M; Nishizaka, T; Endo, Y; Maeno, K; Takahagi, S

1996-10-01

171

Chylothorax in POEMS syndrome.  

PubMed

Chylothorax results from various causes, such as malignancy, trauma, or infection. POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) is a multisystemic syndrome that is associated with plasma cell disorder. Pleural effusion is a common manifestation of POEMS syndrome, but the association of POEMS syndrome with chylothorax has not been reported. We report on a 61-year-old female patient who initially presented with dyspnea and bilateral leg edema. Importantly, the patient had normal renal function. Her chest X-ray and computed tomographic imaging showed bilateral pleural effusion, and her chest drainage revealed chylothorax. Detailed examination failed to reveal the definitive cause of the chylothorax. She received several treatments for chylothorax, namely, a low-fat diet or fasting, total parenteral nutrition, a somatostatin analog (octreotide), thoracic duct ligation by video-assisted thoracic surgery, and pleurodesis. However, further examination revealed endocrinopathy, monoclonal plasma cell disorder, peripheral neuropathy, and elevation of the serum level of vascular endothelial growth factor. The patient's condition was consequently diagnosed as POEMS syndrome. Eventually, her chylothorax was controlled by pleurodesis, and she was transferred to another hospital for stem cell transplantation. Herein, we report on the apparent first case of POEMS syndrome with chylothorax. In some cases of idiopathic chylothorax, the underlying primary disease may be latent, such as in the present patient. POEMS syndrome is rare, but this syndrome should be included in the differential diagnosis of chylothorax with unexplained etiology. PMID:24477187

Kudo, Yujin; Miura, Hiroyuki; Nakajima, Eiji; Takahashi, Hidenobu; Aoki, Akiko; Ikeda, Norihiko

2014-01-01

172

Bilateral and symmetrical tinea mammae.  

PubMed

Tinea corporis has rarely been reported in some locations such as on the breast skin as unilaterally. Herein, we present a case of bilateral tinea mammae, which has not been reported before in English language literature to our knowledge. PMID:24050298

Yilmaz, Murat; Kavak, Ayse; Yamaner, Nalan Jale

2013-09-01

173

Bilateral inferior petrosal sinus sampling.  

PubMed

Bilateral inferior petrosal sinus sampling, the gold standard assay in diagnosing pituitary secretion of adrenocorticotropic hormone in Cushing disease, is highly accurate and safe when performed by experienced interventionalists. We review the anatomic and technical considerations essential for safe and reliable practice. PMID:21990499

Deipolyi, Amy R; Hirsch, Joshua A; Oklu, Rahmi

2012-05-01

174

Middlemen in Bilateral Search Markets  

Microsoft Academic Search

This article examines the role of middlemen in bilateral search markets (e.g., employment agencies, real estate brokers). It is shown that the middleman narrows the set of buyer (firm) and seller (worker) types who search; seller types with high valuations and buyer types with low valuations drop out of the search market and instead trade through the middleman. The middleman

Abdullah Yavas

1994-01-01

175

Cerebral cortical dysplasia: assessment by MRI and SPECT  

Microsoft Academic Search

The objective of this study was to establish correlations between image findings and pathologic deficits in patients with cerebral cortical malformations. The results of magnetic resonance imaging (MRI) and single-photon emission computed tomography (SPECT) in addition to clinical data for 15 patients with cerebral cortical malformations were reviewed retrospectively. MRI led to the diagnoses of bilateral perisylvian syndrome, hemimegalencephaly, focal

Kyoko Sasaki; Yukiko Ohsawa; Masayuki Sasaki; Makiko Kaga; Sachio Takashima; Hiroshi Matsuda

2000-01-01

176

Giant cell arteritis causing symmetric bilateral posterior circulation infarcts.  

PubMed

An 82-year-old woman presented with bilateral, symmetric posterior circulation infarctions secondary to giant cell arteritis (GCA). Her atypical clinical presentation included a lack of headache and fever, but she exhibited signs of systemic illness including generalized weakness, cachexia, apathy, and anemia. Laboratory testing revealed a markedly elevated erythrocyte sedimentation rate, but only a borderline elevated C-reactive protein. Head and neck vascular imaging demonstrated a pattern of vertebral arterial narrowing consistent with GCA-a diagnosis confirmed by temporal artery biopsy. Her unusual symptomatic, laboratory, and imaging presentation highlights the importance of considering GCA in the differential diagnosis of unusual bilateral stroke syndromes, where early treatment decreases morbid outcomes. PMID:24048110

Mackay, Devin D; Huesmann, Graham R; Wu, Roseann I; Stone, James R; Pless, Misha L

2013-10-01

177

Bilateral symptomatic snapping biceps femoris tendon due to fibular exostosis.  

PubMed

We present an unusual case of an athletic 17-year-old male cyclist with bilateral chronic dislocating biceps femoris tendons. On flexion of the knee, the biceps tendon subluxed over a large exostosis, creating a snapping sound. Snapping of tendons is common around the hip, ankles, shoulder, and elbow, but rare at the knee. When it does occur, snapping about the knee can be due to discoid meniscus, rheumatoid nodules, synovial plicae, iliotibial band syndrome, congenital snapping knee, and snapping tendons. Research revealed only 5 previous cases due to subluxation of the biceps femoris tendon. The case we present is the only one due to an exostosis, as well as the only one that required bilateral surgical repair. The patient presented when his pain became significant enough to interfere with his ability to continue competitive cycling. PMID:18300673

Fung, Daniel A; Frey, Steven; Markbreiter, Lance

2008-01-01

178

Transnasal endoscopic marsupialization of bilateral nasoalveolar cysts.  

PubMed

Nasoalveolar cysts are uncommon nonodontogenic and developmental cysts with an uncertain pathogenesis. This cyst has been reported to occur bilaterally in 10-11% of cases. This case report describes a 45-year-old woman in whom the clinicopathologic findings were consistent with bilateral nasoalveolar cysts. The authors report a new, less invasive method for transnasal endoscopic marsupialization of bilateral nasoalveolar cysts. Transnasal endoscopic marsupialization has benefits for the treatment of bilateral and unilateral nasoalveolar cysts. PMID:19604675

Sazgar, A A; Sadeghi, M; Yazdi, A K; Ojani, L

2009-11-01

179

Bilateral spermatocytic seminoma: a case report  

PubMed Central

Spermatocytic seminoma (SS) is a rare entity, accounting for 2%–12% of all seminomas; amongst those, fewer than 10% are bilateral. These may occur synchronously or metachranously. We report here a case of bilateral SS in a 63-year-old patient, who initially presented with bilateral testicular masses. In our search of the literature, this represents the fifth documented case of synchronous, bilateral SS.

Narins, Hadley; Chevli, Kent; Gilbert, Richard; Duff, Michael; Toenniessen, Andrew; Hu, Yan

2014-01-01

180

Cognitive and behavioral deficits following bilateral thalamic stroke: A longitudinal study.  

PubMed

We describe behavioral and neuropsychological outcome of a patient (N.S.), who showed a bilateral paramedian thalamic ischemic lesion, with particular reference to the longitudinal evolution of topographical disorientation (TD) and confabulations. We report clinical neuropsychological/behavioral data over a 43-month follow-up. The results show early after the stroke a severe amnesic-confabulatory syndrome with dysexecutive deficits, associated with memory disorders both for visuo-spatial and verbal materials and TD both for known and new places. Behavioral disinhibition and anosognosia for cognitive deficits were also observed. All cognitive impairments have been recovered during the long-term follow-up. Bilateral paramedian thalamic infarcts often lead to severe and long-lasting neurological and cognitive impairments. Only a few cases showed good recovery. Our patient represents an interesting and uncommon case of bilateral paramedian thalamic syndrome with a significant neuropsychological recovery. PMID:23980818

Rusconi, Maria Luisa; Carelli, Laura; Stampatori, Chiara; Mattioli, Flavia

2014-10-01

181

Bilateral suborbital rash: A dermatologic manifestation of neuropsychiatric disease in a pediatric patient.  

PubMed

A 10-year-old girl presented with a new onset bilateral suborbital rash. Dermatologic examination revealed violaceous, non-tender, well-demarcated patches with an atypical distribution and pigmentation. After further investigation, a diagnosis of Munchausen syndrome was made and the patient was referred to her primary care provider for further management. PMID:24852783

Tong, Lana X; Wang, Yen Tun; Beynet, David

2014-01-01

182

[Bilateral adnexal masses during pregnancy].  

PubMed

The presence of adnexal masses during pregnancy is a rare situation but due to a broader use of sonography; its occurrence is increasing. Their management essentially relies on imagery. We report the case of an 18 weeks-pregnant women presenting bilateral teratomas visualized by RMI. She was successfully treated by laparoscopy. This paper approaches the management of adnexal masses during pregnancy. The epidemiology, possible complications, diagnostic approach and the therapeutic management are discussed. PMID:24908949

Liefferinckx, C; Hottat, N; Simon, P

2014-01-01

183

Pneumomediastinum and bilateral pneumothorax following near drowning in shallow water  

PubMed Central

We report pneumomediastinum, bilateral pneumothorax and acute respiratory distress syndrome in a victim of near drowning who was intoxicated and did not have thoracic or neck trauma. Chest radiograph revealed the above findings, later confirmed by computed tomography. He was in shock and also had gastrointestinal (GI) bleeding and renal dysfunction. With adequate resuscitative measures including fluids, blood transfusions, intercostal tube drainage and mechanical ventilation he made a complete recovery. Good prognostic indicators in near drowning patients include higher Glasgow Coma Scale, short submersion time and quick resuscitative measures even in the presence of serious cardiorespiratory or hemodynamic compromise.

Govindaraj, Santhiya; Viswanathan, Stalin

2011-01-01

184

Laparoscopic Cortical Sparing Adrenalectomy for Pediatric Bilateral Pheochromocytoma: Anesthetic Management  

PubMed Central

Introduction: Pheochromocytoma is a catecholamine-secreting tumor, which is seen rarely in children. These tumors predominantly secrete norepinephrine and epinephrine. They might be familial and associated with hereditary tumors such as Von Hippel-Lindau syndrome and multiple endocrine neoplasia type II. Case Presentation: The child might present with a spectrum of clinical manifestation including hypertension, headache, visual disturbances, and behavioral problems. A meticulous preoperative preparation is essential for a stable intraoperative and postoperative outcome Conclusions: We described successful perioperative management of a child who underwent bilateral laparoscopic cortical sparing adrenalectomy and a repeated surgery for the residual tumor removal.

Rajappa, Geetha Chamanhalli; Anandaswamy, Tejesh Channasandra

2014-01-01

185

Selective cognitive patterns resulting from bilateral hippocampal ischemia  

PubMed Central

A 54-year-old diabetic, hypertensive man with poorly controlled moderate-severe sleep apnea presented with acute onset of severe anterograde amnesia and well preserved remote memory without additional cognitive impairment. Investigations, including a lumbar puncture, electroencephalogram (EEG) and serology testing ruled out infectious, neoplastic and epilleptogenic causes. MRI taken 10 days after symptom onset, was suggestive of sequential ischemic damage to both hippocampal formations. Neuropyschological evaluation suggested a focal and dense amnestic syndrome with little improvement over time. The bilateral nature of hippocampal ischemia though has been reported, is rare.

Cachia, David; Swearer, Joan; Ferguson, Warren; Moonis, Majaz

2011-01-01

186

A case of relapsing flitting bilateral idiopathic orbital inflammation.  

PubMed

Idiopathic orbital inflammation (IOI) is defined as a benign non-infective clinical syndrome characterized by features of non-specific inflammation of the orbit without identifiable local or systemic causes. This can be called orbital myositis if the inflammation is predominantly in the orbital muscles. It is a diagnosis of exclusion based on clinical, radiological, and if necessary, histological findings. The most commons symptoms are swelling, ptosis, proptosis and painful eye movements. To our knowledge, this patient is the first with IOI to demonstrate relapsing flitting bilateral involvement of several individual extra-ocular muscles. PMID:19760410

Browne, Michelle Ann; O'Keefe, Michael; Twomey, Eilish; Donoghue, Veronica; Ryan, Stephanie

2009-12-01

187

Common and uncommon bilateral adult renal masses  

PubMed Central

Abstract Masses can involve the kidney unilaterally or bilaterally. The purpose of this article is to review common and uncommon adult renal masses that present bilaterally. Clinical and imaging findings are described. Renal masses that present in a bilateral fashion can have particular clinical and imaging characteristics and knowledge of their presentation enables appropriate diagnosis and management, especially in a multidisciplinary care setting. More commonly found bilateral renal masses that are discussed include metastasis, lymphoproliferative disorders, adult polycystic kidney disease, angiomyolipomas, renal infracts and renal abscesses. Less common bilateral renal masses include transitional cell carcinoma, oncocytoma, and hematomas.

Roy, Anjali; Silverman, Paul M.; Kundra, Vikas

2012-01-01

188

Compensation following bilateral vestibular damage.  

PubMed

Bilateral loss of vestibular inputs affects far fewer patients than unilateral inner ear damage, and thus has been understudied. In both animal subjects and human patients, bilateral vestibular hypofunction (BVH) produces a variety of clinical problems, including impaired balance control, inability to maintain stable blood pressure during postural changes, difficulty in visual targeting of images, and disturbances in spatial memory and navigational performance. Experiments in animals have shown that non-labyrinthine inputs to the vestibular nuclei are rapidly amplified following the onset of BVH, which may explain the recovery of postural stability and orthostatic tolerance that occurs within 10?days. However, the loss of the vestibulo-ocular reflex and degraded spatial cognition appear to be permanent in animals with BVH. Current concepts of the compensatory mechanisms in humans with BVH are largely inferential, as there is a lack of data from patients early in the disease process. Translation of animal studies of compensation for BVH into therapeutic strategies and subsequent application in the clinic is the most likely route to improve treatment. In addition to physical therapy, two types of prosthetic devices have been proposed to treat individuals with bilateral loss of vestibular inputs: those that provide tactile stimulation to indicate body position in space, and those that deliver electrical stimuli to branches of the vestibular nerve in accordance with head movements. The relative efficacy of these two treatment paradigms, and whether they can be combined to facilitate recovery, is yet to be ascertained. PMID:22207864

McCall, Andrew A; Yates, Bill J

2011-01-01

189

Compensation Following Bilateral Vestibular Damage  

PubMed Central

Bilateral loss of vestibular inputs affects far fewer patients than unilateral inner ear damage, and thus has been understudied. In both animal subjects and human patients, bilateral vestibular hypofunction (BVH) produces a variety of clinical problems, including impaired balance control, inability to maintain stable blood pressure during postural changes, difficulty in visual targeting of images, and disturbances in spatial memory and navigational performance. Experiments in animals have shown that non-labyrinthine inputs to the vestibular nuclei are rapidly amplified following the onset of BVH, which may explain the recovery of postural stability and orthostatic tolerance that occurs within 10?days. However, the loss of the vestibulo-ocular reflex and degraded spatial cognition appear to be permanent in animals with BVH. Current concepts of the compensatory mechanisms in humans with BVH are largely inferential, as there is a lack of data from patients early in the disease process. Translation of animal studies of compensation for BVH into therapeutic strategies and subsequent application in the clinic is the most likely route to improve treatment. In addition to physical therapy, two types of prosthetic devices have been proposed to treat individuals with bilateral loss of vestibular inputs: those that provide tactile stimulation to indicate body position in space, and those that deliver electrical stimuli to branches of the vestibular nerve in accordance with head movements. The relative efficacy of these two treatment paradigms, and whether they can be combined to facilitate recovery, is yet to be ascertained.

McCall, Andrew A.; Yates, Bill J.

2011-01-01

190

Bilateral nephrolithiasis: simultaneous operative management.  

PubMed

A total of 14 patients with extensive bilateral nephrolithiasis underwent simultaneous bilateral lithotomy, in most instances through a single transabdominal incision. Anatrophic nephrolithotomy was performed on 25 kidneys, while 3 kidneys were approached in other ways without formal hypothermia and ischemia. There was no statistically significant change in the average preoperative and postoperative serum creatinine values (p greater than 0.1). There were residual stone fragments in 2 of the 28 kidneys (7 per cent) and stones recurred in 3 others (11 per cent) during the followup period (average 12 months). Of the 14 patients 10 (71 per cent) had infected urine preoperatively and 9 (64 per cent) have been free of infection postoperatively. There were no operative deaths and the average postoperative hospital stay was 17.6 days. We herein demonstrate that bilateral renal surgery for stone removal in 1 operative session can be performed safely with results comparable to those of unilateral staged procedures using other approaches. The advantages of this type of surgical management are discussed. PMID:6834484

Demler, J W; Dennis, M A; Finlayson, B

1983-02-01

191

Multiresolution Bilateral Filtering for Image Denoising  

PubMed Central

The bilateral filter is a nonlinear filter that does spatial averaging without smoothing edges; it has shown to be an effective image denoising technique. An important issue with the application of the bilateral filter is the selection of the filter parameters, which affect the results significantly. There are two main contributions of this paper. The first contribution is an empirical study of the optimal bilateral filter parameter selection in image denoising applications. The second contribution is an extension of the bilateral filter: multiresolution bilateral filter, where bilateral filtering is applied to the approximation (low-frequency) subbands of a signal decomposed using a wavelet filter bank. The multiresolution bilateral filter is combined with wavelet thresholding to form a new image denoising framework, which turns out to be very effective in eliminating noise in real noisy images. Experimental results with both simulated and real data are provided.

Zhang, Ming; Gunturk, Bahadir K.

2008-01-01

192

[Roussy-Levy's syndrome: clinical aspects (author's transl)].  

PubMed

A case of non familial Roussy-Lévy syndrome is presented: optic atrophy, gait ataxia, bilateral hypotrophy of thenar and hypothenar muscles, weak deep tendon reflexes of the upper limbs, weak knee jerks, absent ankle jerks, bilateral Babinski, anapallaesthesia from the bisiliac spine distally, bilateral pes cavus. The literature on Roussy-Lévy syndrome is discussed and whether this syndrome should be considered as a precise entity among heredo-degenerative diseases. The overlapping of differing clinical features may suggest an intermediate form between Friedreich's and Charcot-Marie-Tooth's diseases. PMID:121167

Ravetti, C; Palmucci, L; Brignolio, F; Schiffer, D

1979-01-01

193

Bilateral diffuse uveal melanocytic proliferation with multiple iris cysts.  

PubMed

IMPORTANCE Bilateral diffuse uveal melanocytic proliferation (BDUMP) is a rare paraneoplastic syndrome with characteristic findings, including exudative retinal detachment, rapid cataract formation, and uveal melanocytic tumors. We report a case notable for bilateral iris and ciliary body cysts-a rare presentation of the disease. OBSERVATIONS A woman in her 50s presented with bilateral decreased vision. Her medical history was significant for clear cell adenocarcinoma of the endometrium. Slitlamp examination revealed a contiguous ring of pigmented translucent iris cysts at the pupillary margin of each eye, confirmed with ultrasound biomicroscopy. Ophthalmoscopic examination of the left eye showed a geographic patch of subretinal fluid temporal to the macula that was associated with orange polygonal pigment. The patient underwent periocular injection of triamcinolone acetonide, with resolution of the subretinal fluid. Recurrent fluid was treated successfully with a second injection of triamcinolone. CONCLUSIONS AND RELEVANCE Our case of BDUMP appears to be the first to demonstrate multiple iris and ciliary body cysts with high-quality color photography and ultrasound biomicroscopy. Involvement of the anterior uveal tract may be more common than reported in the literature because of its occult nature. Ultrasound biomicroscopy and anterior segment optical coherence tomography may be useful in patients with suspected BDUMP to identify anterior uveal tract involvement. PMID:24763691

Joseph, Anthony; Rahimy, Ehsan; Sarraf, David

2014-06-01

194

Primary amyloidosis presenting as "dropped head syndrome".  

PubMed

A 77-year-old man, with a history of lymphoma, presented with isolated neck extensor weakness and a 2-year history of bilateral carpal tunnel syndrome (CTS). Needle electromyography showed myopathic changes, and biopsy of cervical paraspinal muscles showed amyloid deposition in blood vessels. Amyloidosis should be considered in the differential diagnosis of dropped head syndrome. PMID:21607974

Chuquilin, Miguel; Al-Lozi, Muhammad

2011-06-01

195

An Animal Model of Tourette's Syndrome  

Microsoft Academic Search

Method: Sera from 12 patients with Tourette's syndrome with high levels of antineural or antinuclear antibodies were in- fused bilaterally into the ventrolateral striatum of rats. Sera from 12 additional Tourette's syndrome patients and 12 nor- mal subjects (both groups with low levels of autoantibodies) were infused for comparison. Rates of oral stereotypies were recorded by observers who were blind

J. R. Taylor; Plunket KD; Lehmann JM; Syed A. Morshed; Salina Parveen; Marcos T. Mercadante; Bradley S. Peterson; Robert A. King; James F. Leckman; Paul J. Lombroso

2002-01-01

196

[Cockayne syndrome].  

PubMed

Cockayne syndrome is a rare autosomal recessive disease. This paper reports a case of Cockayne syndrome confirmed by gene analysis. The baby (male, 7 years old) was referred to Peking University Third Hospital with recurrent desquamation, pigmentation and growth and development failure for 6 years, and recurrent dental caries and tooth loss for 2 years. Physical examination showed very low body weight, body length and head circumference, yellow hair, a lot of fawn spots on the face, skin dry and less elastic, and subcutaneous lipopenia. He had an unusual appearance with sunken eyes, sharp nose, sharp mandible, big auricle and dental caries and tooth loss. Crura spasticity and ataxia with excessive tendon reflexion, and ankle movement limitation while bending back were observed. He had slured speech. The level of serum insulin like growth factor I was low, and the results of blood and urinary amino acid analysis suggested malnutrition. The results of blood growth hormone, thyroxin, parathyroxin, liver function, renal function, lipoprotein profile and blood glucose and electrolytes were all within normal limit. An electronic hearing examination showed moderate neural hearing loss. The sonogram of eyes revealed small eye axis and vitreous body opacity of right side. MRI of brain revealed bilateral calcification of basal ganglia and generalized cerebral and cerebellar atrophy, and brainstem and callus were also atrophic. Genetic analysis confirmed with CSA gene mutation. So the boy was definitely diagnosed with Cockayne syndrome. He was discharged because of no effective treatment. PMID:21342626

Wang, Xue-Mei; Cui, Yun-Pu; Liu, Yun-Feng; Wei, Ling; Liu, Hui; Wang, Xin-Li; Zheng, Zhuo-Zhao

2011-02-01

197

Moebius syndrome with Dandy-Walker variant and agenesis of corpus callosum.  

PubMed

Moebius syndrome is a rare congenital neurological disorder. The most frequent mode of presentation is facial diplegia with bilateral lateral rectus palsy, but there are variations. Here, we report a rare case of Moebius syndrome in a 15-month-old child with unilateral facial palsy, bilateral abducens nerve palsy with Dandy Walker variant, and complete agenesis of corpus callosum. PMID:24470815

John, Jomol Sara; Vanitha, R

2013-09-01

198

The first Susac's syndrome case in Turkey.  

PubMed

Susac's syndrome is a vasospastic disease resulting from bilateral microangiopathy of the brain, cochlea and retina. It is characterized by encephalopathy, bilateral sensorineural fluctuating hearing loss and visual loss. It is very uncommon and usually affects women during young adulthood. Since all three symptoms of the triad may not be present, the clinical diagnosis is difficult. Therefore, neuroimaging, particularly magnetic resonance imaging, has an important role in establishing the diagnosis. In this case report, we present a young woman who had all the symptoms of Susac's syndrome. This is the first Susac's syndrome case reported in Turkey. PMID:17109892

Erer, Sevda; Boz, Meral; Taskapilioglu, Ozlem; Hakyemez, Bahattin; Kaderli, Berkant; Zarifoglu, Mehmet

2006-12-21

199

Chronic lymphangiectasis in Turner's syndrome  

Microsoft Academic Search

A 3 1\\/2-year-old female presented with Turner's syndrome and Nonne-Milroy-Meige disease. Ocular findings included strabismus and bilateral chemosis which was unchanging and persisted throughout the four years the patient was followed up. Histopathological findings included diffuse lymphangiectasia and dense connective tissue surrounding the dilated lymph channels. Although the association between congenital lymphoedema and Turner's syndrome is common, the lymphoedema usually

H D Perry; A J Cossari

1986-01-01

200

Bilateral zosteriform extragenital lichen sclerosus.  

PubMed

A 35-year-old man presented with asymptomatic eruption on both forearms and lower aspects of the legs for 6 months. The lesions first appeared on his inner aspects of the wrist, the dorsal surface of the hands, and legs and progressed to involve proximal aspects of the extremities. There was no significant past history. On examination, multiple pearly white papules and depigmented atrophic plaques were found bilaterally on the flexors of the arms and the extensors of the legs. The lesions were arranged in a linear manner, following the lines of Blaschko (Figures 1 and 2). The surface of the atrophic plaques was notable for prominent telangiectasia, giving an erythematous appearance. The genitalia, oral cavity, palms, and soles were spared. Systemic examination was noncontributory. Lichen striatus and extragenital lichen sclerosus (ELS) were considered the differential diagnosis. Clinically, the age of the patient, the absence of scaling, and the presence of atrophic plaques and telangiectasia were in favor of ELS. A punch biopsy from an atrophic plaque was performed, and it revealed hyperkeratosis, atrophic epidermis, basal layer vacuolar degeneration, mild lymphocytic infiltration in the dermis, edema, and homogenization of collagen of the upper portion of the dermis (Figures 3 and Figure 4). Histopathologic findings were consistent with lichen sclerosus. A diagnosis of bilateral zosteriform ELS was made. PMID:24933856

Kumar, Piyush; Jha, Abhijeet Kumar; Mallik, Sambeet Kumar; Raihan, Mohammed

2014-01-01

201

Bilateral facial synkinesis in leprosy  

PubMed Central

Leprosy is an important cause of cranial nerve palsy in endemic areas where it may be seen in upto 17.6% patients. The authors herein describe a rare case of bilaterally symmetrical facial synkinesis with video documentation and modified blink reflex. A 35-year-old gentleman presented with numbness involving right half of his face for 8 months and abnormal stretching sensations over both sides of his nose for one and a half months. Sensory and motor involvement of the right trigeminal nerve was detected along with bilaterally symmetrical facial synkinesis involving orbicularis oculi and nasalis. R1 and R2 responses consistent with mis-reinnervation were recorded on the left-side using orbicularis oculi and nasalis muscles. Skin biopsy revealed acid-fast bacilli and sural nerve biopsy, the presence of granulomas. After 3 months of follow-up on WHO multi-drug therapy, an improvement in facial sensations was observed but without any change in facial synkinetic movements.

Malhotra, Hardeep Singh; Garg, Ravindra Kumar; Goel, Madhu Mati; Jain, Amita; Gupta, Arvind; Lalla, Rakesh; Singh, Gyan Prakash

2012-01-01

202

Sequential bilateral retinal artery occlusion  

PubMed Central

An 86 year old woman experienced a sequential bilateral loss of vision over a period of less than 24 hours. Clinical findings and complementary studies suggested a bilateral atherogenic embolic event. Initially, she presented a superior branch retinal artery occlusion in her right eye followed by a central retinal artery occlusion with cilioretinal artery sparing in her left eye. Some conservative maneuvers performed did not improve visual acuity in the left eye. Supra-aortic Doppler ultrasonography revealed mild right internal carotid artery stenosis and moderate left internal carotid artery stenosis with a small, smooth, and homogeneous plaque. The transthoracic echocardiography showed a severe calcification of the mitral valve with a mild-moderate rim of stenosis. Central retinal artery occlusion and branch retinal artery occlusion are characterized by painless monocular loss of vision. Clinical approach and management attempt to treat the acute event, find the source of the vascular occlusion, and prevent further vascular events from occurring. Giant cell arteritis is a potentially treatable cause of central retinal artery occlusion and should be excluded in every single patient over 50 years old.

Padron-Perez, Noel; Arones, Janny Rosario; Munoz, Silvia; Arias-Barquet, Luis; Arruga, Jorge

2014-01-01

203

Focal orchitis presenting as bilateral testicular masses  

Microsoft Academic Search

A 14-year-old male presented with bilateral scrotal pain. Ultrasound with Doppler revealed multiple bilateral testicular masses that were hypoechoic and hypervascular. Tumor markers and endocrinologic work-up were normal. Bilateral inguinal exploration of the testicles was done and biopsy revealed inflammation with no evidence of malignancy. The patient's pain resolved a few weeks postoperatively. Follow-up ultrasound revealed resolution of the masses.

Jose A. Karam; Linda A. Baker

2007-01-01

204

A clinical analysis of bilateral orbital fracture.  

PubMed

Although bilateral orbital fracture can cause serious eyeball and facial skeletal problems, few reports have been issued on the topic. We analyzed the clinical features of bilateral orbital fracture by reviewing the medical records of 147 patients and compared bilateral and unilateral fractures by reviewing the literature.Bilateral orbital fracture was most common in men aged between 50 and 59 years. A traffic accident was the leading cause of trauma, and average time between trauma and surgery was 12.2 days. Bilateral medial fracture accompanied by nasal fracture accounted for the overwhelming majority, and impure blowout fracture in at least 1 eye occurred in 69.4% of the 147 patients. Associated ocular injuries seemed to be similar for bilateral and unilateral fracture. Thirty-five patients (23.8%) had other multiple traumas affecting other than the eyes, and this significantly increased the need for surgery (P < 0.05). Of the 48 patients who underwent surgery, including 4 cases of bilateral surgery, 21 patients who had ocular motility restriction with central diplopia within 30 degrees almost completely recovered. No significant relation between the timing of surgery and improvement was found. Although unilateral surgery was performed in most cases, facial asymmetry related to enophthalmos was unclear at 6 months postoperatively.In summary, bilateral orbital fracture was found to be clinically distinguishable from unilateral fracture in several aspects. We hope these findings provide a reference guide to the approach and management of bilateral orbital fracture. PMID:24514894

Roh, Joon Ho; Jung, Jee Woong; Chi, Mijung

2014-03-01

205

Acute bilateral mydriasis associated with anti-GQ1b antibody.  

PubMed

Miller Fisher syndrome (MFS) is an autoimmune neuropathy characterized by external ophthalmoplegia, ataxia and areflexia. Mydriasis is present in 35% of typical MFS. We report five patients with acute bilateral mydriasis, either isolated or associated with external ophthalmoplegia for which the presumed diagnosis of "atypical MFS" was confirmed by the positivity of anti-GQ1b antibodies. Acute bilateral mydriasis raises important differential diagnoses in clinical practice. This report demonstrates that acute mydriasis can be autoimmune mediated and that anti-GQ1b antibodies are useful to confirm the diagnosis in unexplained cases. PMID:20093031

Fleury, V; Aqallal, A; Lagrange, E; Besson, G; Caudie, C

2010-04-01

206

Bilateral Ureteral Tapering and Secondary Ureteroneocystostomy for Late Stenosis in a Patient with Bladder Extrophy  

PubMed Central

Objective To report a case of severe hydronephrosis 20 years after bladder exstrophy (BE) repair, managed by bilateral ureteral tapering and secondary ureteroneocystostomy. Case Presentation A 21-year-old woman with a history of BE and ureteral reimplantation, presented with hematuria-dysuria syndrome and recurrent febrile urinary tract infections. After counselling, she elected to undergo bilateral ureteral tapering and second ureteroneocystostomy. Conclusion Hydronephrosis secondary to ureteralvesical stricture in BE patients can be successfully managed with ureteral reimplantation associated to ureteral tapering even after a prior reimplantation.

Altobelli, Emanuela; Bove, Alfredo M.; Sergi, Federico; Buscarini, Maurizio

2013-01-01

207

Unusual bilateral cervical metastases as first clinical evidence of lung cancer.  

PubMed

We report on an unusual case of laterocervical bilateral metastatatic masses with unknown clinical, radiological or computer tomographicic detected primary site of origin. Cancer of an unknown primary site is a clinical syndrome, accounting for 2%-5% of patients with cancer. The peculiarities of our case are its evolution as fast-growing bilateral tumor masses with involvement of other neck structures and its unexpected origin from the lung, certified by complementary immunohistochemical tests following surgery, in the absence of any other clinical signs or any detectable lung tumor mass by radiological or computer tomographic tests. PMID:23606699

Radu, Boia Eugen; Marioara, Poenaru; Constantin, Balica Nicolae; Maria, Cimpean Anca; Raluca, Ceausu Amalia; Marius, Raica; Caius, Doros

2013-01-01

208

Skin involvement in Susac's syndrome  

Microsoft Academic Search

Susac's syndrome (SS) is a rare microangiopathy affecting the precapillary arterioles of the brain, retina and inner ear, presumably resulting from an autoimmune endotheliopathy. We report the first case of SS with histologically proven skin involvement, in a 24-year-old male who presented a subacute encephalopathy, branch retinal artery occlusions and bilateral hearing loss, two weeks after the onset of a

Guillaume Turc; Dominique Monnet; Nicolas Dupin; Frédéric Beuvon; Vincent Guiraud; Mejdi Ben Amor; Emmanuel Touzé

2011-01-01

209

Neuromyelitis optica — Devic's syndrome, update  

Microsoft Academic Search

Neuromyelitis optica of Devic (NMO) is a syndrome which combines transverse myelitis and optic neuritis. For many years it was assumed that transverse myelitis and optic neuritis should be simultaneous, that optic neuritis should be bilateral, and that transverse myelitis must be complete. NMO was considered to be very rare. Through the 20th century there was a debate on whether

Raul N. Mandler

2006-01-01

210

Asian paralysis syndrome.  

PubMed

We report 20 children admitted to the paediatric ward of a public general hospital for acute flaccid paralysis, which was bilaterally symmetrical in all cases and was associated with bulbar involvement in eight of them. Recovery was partial. Nerve conduction studies showed motor axonal neuropathy. This new disease, variously termed as non-inflammatory neuropathy/Chinese paralysis syndrome must be differentiated from Guillain-Barré syndrome (GBS) and poliomyelitis. Both GBS and Asian paralysis syndrome have bilaterally symmetrical flaccid paralysis but GBS tends to have sensory involvement, full recovery occurs in 90% of cases and nerve conduction shows demyelinating neuropathy. Asian paralysis syndrome and poliomyelitis are pure motor lesions without sensory changes and partial recovery, but poliomyelitis differs in that paralysis is asymmetrical and unequal, muscle spasm is always present in the initial stage and there are prodromal symptoms. Nerve conduction studies show anterior horn cell disease. This new entity, common in Asian populations, assumes public health importance when it mimics poliomyelitis in a country that has tried to eliminate poliomyelitis by universal immunization. To the best of our knowledge, this is the first report of Asian paralysis syndrome in children in our area. PMID:10716023

Phadke, M A; Gambhir, P S; Deshpande, A S; Kurlekar, S U; Godbole, K G

1999-12-01

211

Hypoplastic left heart syndrome in PAGOD syndrome.  

PubMed

Chromosomal abnormalities as well as non-cardiac anomalies have been identified as independent risk factors for surgical morbidity and mortality in Fontan palliation. The combination of malformations consisting of pulmonary hypoplasia, agonadism (sex reversal), omphalocele, and diaphragmatic defect is compatible with pulmonary artery and lung hypoplasia, agonadism, omphalocele, and diaphragmatic defect (PAGOD). Most cases have been associated with cardiac disease, particularly hypoplastic left heart syndrome (HLHS) that is potentially destined for Fontan palliation. Reported herein is the case of a Japanese female infant diagnosed with PAGOD syndrome along with HLHS (mitral atresia and aortic atresia), in whom intractable respiratory failure manifested as bilateral eventration of the diaphragm and presumed right lung hypoplasia. These characteristic pulmonary lesions associated with the syndrome precluded use of the Fontan pathway. PMID:24894929

Takahashi, Kazuhiro; Miyake, Akira; Nakayashiro, Mami

2014-06-01

212

An evaluation of surgical outcome of bilateral cleft lip surgery using a modified Millard's (Fork Flap) technique  

PubMed Central

Background The central third of the face is distorted by the bilateral cleft of the lip and palate and restoring the normal facial form is one of the primary goals for the reconstructive surgeons. The history of bilateral cleft lip repair has evolved from discarding the premaxilla and prolabium and approximating the lateral lip elements to a definitive lip and primary cleft nasal repair utilising the underlying musculature. The aim of this study was to review surgical outcome of bilateral cleft lip surgery (BCLS) done at the Lagos University Teaching Hospital. Materials and Methods A review of all cases of BCLS done between January 2007 and December 2012 at the Lagos University Teaching Hospital was done. Data analysis included age and sex of patients, type of cleft deformity and type of surgery (primary or secondary) and whether the cleft deformity was syndromic and non-syndromic. Techniques of repair, surgical outcome and complications were also recorded. Results A total of 39 cases of BCLS involving 21 males and 18 females were done during the period. This constituted 10% (39/390) of all cases of cleft surgery done during the period. There were 5 syndromic and 34 non-syndromic cases. Age of patients at time of surgery ranged between 3 months and 32 years. There were 24 bilateral cleft lip and palate deformities and 15 bilateral cleft lip deformities. Thirty-one of the cases were primary surgery, while 8 were secondary (revision) surgery. The most common surgical technique employed was modified Fork flap (Millard) technique, which was employed in 37 (95%) cases. Conclusion Bilateral cleft lip deformity is a common cleft deformity seen in clinical practice, surgical repair of which can be a challenge to an experienced surgeon. A modified Fork flap for repair of bilateral cleft lip is a reliable and versatile technique associated with excellent surgical outcome.

Adeyemo, W. L.; James, O.; Adeyemi, M. O.; Ogunlewe, M. O.; Ladeinde, A. L.; Butali, A.; Taiwo, O. A.; Emeka, C. I.; Ayodele, A. O. S; Ugwumba, C. U.

2014-01-01

213

Loss of Heterozygosity in Bilateral Breast Cancer  

Microsoft Academic Search

Women who develop bilateral breast cancer at an early age are likely to harbour germline mutations in breast cancer susceptibility genes. The aim of this study was to test for concordant genetic changes in left and right breast cancer of young women (age <50) with bilateral breast cancer that may suggest an inherited breast cancer predisposition. Microsatellite markers were used

J. Kollias; S. Man; M. Marafie; K. Carpenter; S. Pinder; I. O. Ellis; R. W. Blamey; G. Cross; J. D. Brook

2000-01-01

214

Secondary polycythaemia associated with bilateral renal lymphocoeles.  

PubMed Central

A patient with a 15 year history of secondary polycythaemia due to renal erythropoietin hypersecretion is presented. Subsequent spontaneous development of bilateral renal lymphocoeles, which contained high erythropoietin levels, was shown by computerized tomography. The lymphocoeles were successfully treated by bilateral peritoneal marsupialization. No cause for the persistent polycythaemia or lymphocoeles was found at laparotomy or on renal biopsy. Images Figure 1

Burton, I. E.; Sambrook, P.; McWilliam, L. J.

1994-01-01

215

CHINA AND ASEAN5 BILATERAL TRADE RELATIONSHIP  

Microsoft Academic Search

This paper examines the China-ASEAN bilateral trade relationship, using the Gravity model where the per capita income difference is included in the specification for testing the hypothesis. The empirical results based on Bounds test proposed by Pesaran et al . (2001) reveal that the transportation cost and real GDP are statistically significant determinants of bilateral trade. The trade distance remains

SIAH KIM LAN

216

Recurrent Bilateral Breast Abscesses after Sternotomy  

PubMed Central

Median sternotomy is the most commonly used incision in cardiothoracic procedures. Development of breast abscess after sternotomy is a very rare situation. We present a case of sternal wound infection with recurrent bilateral breast abscess after sternotomy. Our case is the first and only case in the literature due to the presence of sternal wound infection with recurrent bilateral breast abscess after sternotomy.

Cinar, Hamza; Ulusoy, Ali Naki; Kaya, Emir Fatih; Lap, Gokhan; Karabulut, Kagan; Polat, Ayfer Kamal?; Selcuk Ozbalci, Gokhan

2012-01-01

217

Multiresolution Bilateral Filtering for Image Denoising  

Microsoft Academic Search

The bilateral filter is a nonlinear filter that does spatial averaging without smoothing edges; it has shown to be an effective image denoising technique. An important issue with the application of the bilateral filter is the selection of the filter parameters, which affect the results significantly. There are two main contributions of this paper. The first contribution is an empirical

Ming Zhang; Bahadir K. Gunturk

2008-01-01

218

Myoclonus-induced bilateral acetabular fracture dislocations.  

PubMed

An uncommon case of myoclonus-induced bilateral central acetabular fracture-dislocation is presented. Although different fracture types due to forceful muscular contractions have been reported, this is an exceedingly uncommon injury pattern. Bilateral total hip arthroplasty, in combination with a reinforcement ring and autologous bone grafting, was performed after open reduction and internal fixation. PMID:18703309

Balcarek, Peter; Dresing, Klaus; Walde, Tim Alexander; Tezval, Mohammad; Stürmer, Klaus M

2009-08-01

219

Bilateral adrenal myelolipomas in a woman with chronic anticoagulation, postmenopausal uterine bleeding, primary hyperparathyroidism and hyperthyroidism.  

PubMed

Adrenal myelolipomas are rare, usually benign unilateral tumors. Their management has controversially been discussed. The authors here present a 53-year-old African American female Jehovah's witness with postmenopausal uterine bleeding on chronic anticoagulation and episodic right flank pain who was found to have bilateral myelolipomas in addition to primary hyperparathyroidism. In collaboration with gynecology, midline laparotomy was performed to remove uterus and both ovaries in addition to the right adrenal gland for a 62 × 79 mm myelolipoma. An open biopsy of the left adrenal mass measuring 42 × 43 mm revealed myelolipoma and ruled out malignancy. Pathology confirmed bilateral myelolipomas, endometrial polyps, and leiomyomata uteri. After an uneventful recovery, the patient then underwent a right inferior parathyroidectomy for parathyroid adenoma. This case illustrates the challenges in deciding when to surgically intervene for bilateral adrenal myelolipoma and, for the first time, associates various other endocrinopathies, although no known endocrine neoplasia syndrome variant was present. PMID:23426084

Saunders, Rachel N; Koch, Christian A; Brown, Kathryn B; Hairston, Frances J; Daley, William P; Ahmed, Naveed; Vanderlan, Wesley B

2013-07-01

220

Chronic lymphangiectasis in Turner's syndrome.  

PubMed

A 3 1/2-year-old female presented with Turner's syndrome and Nonne-Milroy-Meige disease. Ocular findings included strabismus and bilateral chemosis which was unchanging and persisted throughout the four years the patient was followed up. Histopathological findings included diffuse lymphangiectasia and dense connective tissue surrounding the dilated lymph channels. Although the association between congenital lymphoedema and Turner's syndrome is common, the lymphoedema usually disappears by the first year of life. The persistence of the lymphoedema beyond this age is rare, as is the presence of the persistent chemosis. This report represents the first histopathological documentation of congenital lymphangiectasis in association with Turner's syndrome. PMID:3964640

Perry, H D; Cossari, A J

1986-05-01

221

Bilateral Diaphragmatic Hernia Due to Blunt Trauma; a Case Report.  

National Technical Information Service (NTIS)

A case of bilateral diaphragmatic hernia due to blunt trauma with clinical manifestations similar to those of cardiac tamponade was treated successfully by bilateral thoracotomy and laparotomy. Two types can be distinguished: (1) True bilateral diaphragma...

W. Enyu

1980-01-01

222

[Cryptorchidism with didymo-epididymal dissociation and Robinow's syndrome: 2 case reports].  

PubMed

Two brothers of normal not consanguineous parents, with bilateral intrabdominal cryptorchidism, were admitted to our Institution. Both children had short stature, limb and hand malformations and craniofacial patterns of Robinow syndrome. During the orchidopexy, bilateral epididymal and vasal abnormalities were found in both of them. This anomaly associated with Robinow syndrome has never been reported before. These two cases provides the Authors with the opportunity of reviewing clinical features, genetics and radiological patterns of this rare syndrome. PMID:9312747

Fabbro, M A; D'Agostino, S; Costa, L; Musi, L; Cappellari, F

1997-01-01

223

Megacystis, microcolon, intestinal hypoperistalsis syndrome and bilateral streak gonads  

PubMed Central

Megacystis, microcolon, intestinal hypoperistalsis is an uncommon condition presenting in neonatal age with features of intestinal obstruction and bladder evacuation abnormalities. We present here an infant girl with the diagnosis consistent with this entity.

Mantan, M.; Singhal, K. K.; Sethi, G. R.; Aggarwal, S. K.

2011-01-01

224

Cancer problem in Peutz-Jeghers syndrome  

PubMed Central

Peutz–Jeghers syndrome is a rare autosomal dominantly inherited condition, characterized by the presence of hamartomatous gastrointestinal polyps and mucocutaneous pigmentation. Patients with this syndrome can be associated with other neoplasms such as ovarian neoplasms known as sex-cord tumor with annular tubules that are associated in one third of the cases with this syndrome and other types of malignancies. We report a 42-year-old woman with a history of Peutz–Jeghers Syndrome and bilateral breast cancer that presented with abnormal uterine bleeding. Total abdominal hysterectomy with bilateral salpino-oophorectomy was done and an ovarian sex cord tumor with annular tubules was incidentally diagnosed. By reviewing literatures and in agreement with previous studies we suggest routine screening for malignancies in patients with Peutz–Jeghers syndrome.

Taheri, Diana; Afshar-Moghadam, Noushin; Mahzoni, Parvin; Eftekhari, Amin; Hashemi, Seyed Mozafar; Emami, Mohammad Hasan; Fesharakizadeh, Mehdi; Ghasemi-basir, Hamid Reza

2013-01-01

225

Bilateral sudden hearing loss following habitual abortion: a case report and review of literature  

PubMed Central

Sudden sensorineural hearing loss (SSNHL) is usually unilateral and can be associated with tinnitus and vertigo. The most common causes of this disease are known to be the vascular and viral agents, but immune disorders are involved in the development of sudden deafness. The antiphospholipid syndrome (APS) is an acquired autoimmune system disorder, which is defined as the presence of antiphospholipid antibodies (APA) in the patient’s blood, then cause venous and/or arterial thrombosis in various organs of the body, for example, thrombosis can occur in the placenta and/or the inner ear. As a result, it can cause abortion and/or sudden deafness. Bilateral SSNHL following habitual abortion is a rare clinical event. Here, we report a case of 32-year-old woman who presented with bilateral sudden hearing loss following recurrent pregnancy loss (RPL) as the first manifestation of primary antiphospholipid syndrome. Combine the literature, the diagnosis, clinical implication and treatment are discussed.

Yin, Tuanfang; Huang, Fengying; Ren, Jihao; Liu, Wei; Chen, Xing; Li, Lihua; Xie, Dinghua; Lu, Yongde

2013-01-01

226

[Bilateral carotid artery dissection in a severe preeclamptic setting: an unusual cause of postpartum headache].  

PubMed

A 30-year-old woman with severe preeclampsia presented at 27 weeks of amenorrhea with left headache, neck pain, blurred vision and numbness of left hemiface that resolved spontaneously within 2 hours. A week later, hypertension remained poorly controlled despite combination of nicardipine and labetalol intravenous therapy; an urgent caesarean section was eventually performed due to onset of HELLP syndrome. At day 5 postpartum, the patient had a Horner syndrome with right ipsilateral disabling tinnitus. A CT-angiography of supra-aortic trunks was performed urgently; it showed a bilateral carotid arterial dissection without stroke, which was subsequently confirmed by MRI angiography. The patient was transferred in neurovascular intensive care unit. Anticoagulant therapy was implemented to prevent cerebral and retinal ischemic lesions. Symptoms resolved quickly and the patient was discharged at day 7 postpartum. MR-angiography performed 4 months later showed a full resolution of the bilateral carotid dissection. Anticoagulant therapy was therefore discontinued. PMID:23498556

Hoffmann, C; Augé, M; Falzone, E; Martel-Jacob, S; Mercier, F J

2013-04-01

227

Bilateral symmetry across Aphrodite Terra  

NASA Technical Reports Server (NTRS)

There are three main highland areas on Venus: Beta Regio, Ishtar Terra and Aphrodite Terra. The latter is least known and the least mapped, yet existing analyses of Aphrodite Terra based on available Pioneer-Venus orbiter data suggest that it may be the site of extensive rifting. Some of the highest resolution (30 km) PV data (SAR) included most of the western half of Aphrodite Terra. Recent analysis of the SAR data together with Arecibo range-doppler topographic profiling (10 X 100 km horizontal and 10 m vertical resolution) across parts of Aphrodite, further characterized the nature of possible tectonic processes in the equatorial highlands. The existence of distinct topographic and radar morphologic linear discontinuities across the nearly east-west strike of Aphrodite Terra is indicated. Another prominent set of linear features is distinctly parallel to and orthogonal to the ground tracks of the PV spacecraft and are not included because of the possibility that they are artifacts. Study of the northwest trending cross-strike discontinuities (CSD's) and the nature of topographic and morphologic features along their strike suggest the presence of bilateral topographic and morphologic symmetry about the long axis of Aphrodite Terra.

Crumpler, L. S.; Head, J. W.; Campbell, D. B.

1987-01-01

228

Bilateral internuclear ophthalmoplegia associated with pediatric brain tumor progression: a case series and review of the literature.  

PubMed

Internuclear ophthalmoplegia (INO) is a rare disorder of conjugate lateral gaze that has been described in a number of neurologic conditions including multiple sclerosis, stroke and less commonly brain tumors. We describe a series of 3 boys (11, 12, 15 years) diagnosed with primary central nervous system tumors (pilomyxoid variant astrocytoma, anaplastic oligoastrocytoma, gliomatosis cerebri) who developed bilateral INO as a manifestation of progressive disease. Time from diagnosis to development of bilateral INO ranged from 13-36 months. All children died of their disease 1-9 months following diagnosis of bilateral INO and had significant dorsal pontine invasion on magnetic resonance imaging at progression. Only one child had brainstem involvement at diagnosis. Our case series highlights this rare ophthalmologic syndrome of bilateral INO in association with tumor progression and provides a literature review of brain tumor associations with INO. PMID:24048548

Rismanchi, Neggy; Crawford, John R

2013-12-01

229

Bilateral cataract surgery: A controlled clinical trial  

Microsoft Academic Search

Purpose  To evaluate the efficacy and safety of simultaneous bilateral cataract surgery with respect to patient satisfaction, outcomes,\\u000a and complication rates.\\u000a \\u000a \\u000a \\u000a Methods  We conducted a prospective study of consecutive patients who had simultaneous bilateral cataract surgery on the same day or\\u000a separate bilateral cataract surgery with an interval of 2 days between operations. The changes in refraction, visual acuity,\\u000a degree of anisometropia,

J. K. Chung; Song Hee Park; Woo Jin Lee; Sung Jin Lee

2009-01-01

230

Fibromuscular Dysplasia Presenting with Bilateral Renal Infarction  

SciTech Connect

Fibromuscular dysplasia (FMD) describes a group of conditions which cause nonatheromatous arterial stenoses, most commonly of the renal and carotid arteries, typically in young women. We report a rare case of bilateral segmental renal infarction secondary to FMD in a young male patient. His initial presentation with loin pain and pyrexia resulted in a delay in the definitive diagnosis of FMD. He was successfully treated with bilateral balloon angioplasty. The delayed diagnosis in this patient until the condition had progressed to bilateral renal infarcts highlights the need for prompt investigation and diagnosis of suspected cases of FMD.

Doody, O., E-mail: orla_doody@hotmail.co [Alfred Hospital, Department of Radiology (Australia); Adam, W. R. [University of Melbourne and Goulburn Valley Health, School of Rural Health (Australia); Foley, P. T.; Lyon, S. M. [Alfred Hospital, Department of Radiology (Australia)

2009-03-15

231

Bilateral dacryocystoceles in a pregnant woman.  

PubMed

The authors describe, for the first time, bilateral, sequential large dacryocystoceles during pregnancy and review the literature for this presentation. A 26-year-old, 15-week pregnant woman presented with OD epiphora, diplopia, and pain in the setting of an inferomedial orbital mass. Surgical exploration and histopathology were consistent with a dacryocystocele, and a dacryocystorhinostomy was curative. She returned at 34-week gestation, with an identical presentation on the left side. Review of the literature reveals that dacryocystoceles occasionally present in adults; however, bilateral involvement may be unusual. Bilateral dacryocystoceles have not been previously reported in a pregnant woman. PMID:24025998

Hirabayashi, Kristin E; Yang, Elizabeth; Echegoyen, Julio; Yoon, Steven J; Tao, Jeremiah P

2014-01-01

232

Acquisition of motor skill after bilateral medial temporal-lobe excision  

Microsoft Academic Search

The acquisition of selected motor skills was studied in a 40-year-old man with a severe amnestic syndrome resulting from a bilateral medial temporal-lobe resection carried out 13 years before. On Rotary Pursuit, Bimanual Tracking, and Tapping, his scores improved from session to session, and on the one task where it was feasible to retest several days after the end of

Suzanne Corkin

1968-01-01

233

[Oculocephalomotor disorders and bilateral thalamo-subthalamic infarction (author's transl)].  

PubMed

A 33-year-old woman on oral contraceptives suddenly developed neurological disorders characterised by decreased alertness, absence of motor and verbal spontaneity, anterograde amnesia with difficulties in verbal and visual learning. She also presented paralysis of upwards and downwards ocular movements affecting mainly saccadic movements while convergence was normal. An initial almost total absence of spontaneous head movements and later an impossibility to carry out downwards movements of the head. This apparently represents a syndrome still not reported in human pathology. The clinical course was favourable. CT scan suggested the presence of a paramedian infarct of the region supplied by the thalamosubthalamic artery of the type II b of Percheron. An analogy is suggested between these disorders of oculocephalomotor behaviour and those observed by Denny-Brown in experimental bilateral retrorubric lesions. The possibility of a lesion in the internal thalamic nucleis and the adjacent subthalamic region is considered. PMID:7336023

Petit, H; Rousseaux, M; Clarisse, J; Delafosse, A

1981-01-01

234

Trans-radial bilateral iliac artery stenting.  

PubMed

We describe a step-by-step instructional 'how to' case of trans-radial bilateral iliac stenting using a 5-Fr guide sheath in a symptomatic patient to illustrate an alternative to common femoral artery access. PMID:23945043

Harruna, Shayibu; Jacobs, Evan S; White, Christopher J

2013-08-01

235

Neuroma in bilateral upper limb amputation.  

PubMed

To evaluate the prevalence of neuroma in bilateral upper limb amputees and investigate the effect of level of amputation on their pain, 86 patients with bilateral upper limb amputation were thoroughly examined by an orthopedic surgeon. Of 172 bilateral amputated upper limbs (86 victims of war) 17.1+/-6.1 years after injury, physical examination revealed that 26.2% had moderate to severe stump pain and clinical signs suggestive of neuroma. Statistical analysis showed no relation between level of amputation, prosthesis usage, and occurrence of neuroma. Although high occurrence of neuroma among traumatic bilateral upper limb amputation had no significant effects on wearing prosthesis, its treatment can deeply influence alleviation of their pain and subsequently their quality of life. PMID:19226072

Soroush, Mansoor; Modirian, Ehsan; Soroush, Mohamadreza; Masoumi, Mahdi

2008-12-01

236

Bilateral thoracic paravertebral block: potential and practice.  

PubMed

Paravertebral nerve blocks (PVBs) can provide excellent intraoperative anaesthetic and postoperative analgesic conditions with less adverse effects and fewer contraindications than central neural blocks. Most published data are related to unilateral PVB, but its potential as a bilateral technique has been demonstrated. Bilateral PVB has been used successfully in the thoracic, abdominal, and pelvic regions, sometimes obviating the need for general anaesthesia. We have reviewed the use of bilateral PVB in association with surgery and chronic pain therapy. This covers 12 published studies with a total of 538 patients, and with varied methods and outcome measures. Despite the need for relatively large doses of local anaesthetics, there are no reports of systemic toxicity. The incidence of complications such as pneumothorax and hypotension is low. More studies on the use of bilateral PVB are required. PMID:21233114

Richardson, J; Lönnqvist, P A; Naja, Z

2011-02-01

237

38 CFR 4.26 - Bilateral factor.  

Code of Federal Regulations, 2010 CFR

...Thus with a compensable disability of the right thigh, for example, amputation, and one of the left foot, for example, pes planus, the bilateral factor applies, and similarly whenever there are compensable disabilities affecting use of paired...

2010-07-01

238

Bilateral, Zero-Impedance Static Semiconductor Switch  

NASA Technical Reports Server (NTRS)

Static semiconductor switching circuit eliminates the undesirable features of electromechanical relays and conventional semiconductor switching circuits. There is a net zero voltage drop at the terminals and thus a zero impedance for bilateral currents there.

Doughman, C. L.

1968-01-01

239

Bilateral persistent pupillary membranes associated with cataract  

PubMed Central

Summary Exuberant persistent pupillary membranes (PPM) are rare in adult eyes. We report the case of a 53-year-old man diagnosed with bilateral, profuse, persistent pupillary membranes and unilateral cataract.

Ahmad, Syed Shoeb; Binson, Caroline; Lung, Chong Ka; Ghani, Shuaibah Abdul

2011-01-01

240

Bilateral Hippocampal Dysfunction in Schizophrenia  

PubMed Central

The hippocampus has long been known to be important for memory, with the right hippocampus particularly implicated in nonverbal/visuo-spatial memory and left in verbal/narrative or episodic memory. Despite this hypothesized lateralized functional difference, there has not been a single task that has been shown to activate both the right and left hippocampus differentially, dissociating the two, using neuroimaging. The transverse patterning (TP) task is a strong candidate for this purpose, as it has been shown in human and nonhuman animal studies to theoretically and empirically depend on the hippocampus. In TP, participants choose between stimuli presented in pairs, with the correct choice being a function of the specific pairing. In this project, TP was used to assess lateralized hippocampal function by varying its dependence on verbal material, with the goal of dissociating the two hippocampi. Magnetoencephalographic (MEG) data were collected while controls performed verbal and nonverbal versions of TP in order to verify and validate lateralized activation within the hippocampi. Schizophrenia patients were evaluated to determine whether they exhibited a lateralized hippocampal deficit. As hypothesized, patients’ mean level of behavioral performance was poorer than controls’ on both verbal and nonverbal TP. In contrast, patients had no decrement in performance on a verbal and nonverbal non-hippocampal-dependent matched control task. Also, controls but not patients showed more right hippocampal activation during nonverbal TP and more left hippocampal activation during verbal TP. These data demonstrate the capacity to assess lateralized hippocampal function and suggest a bilateral hippocampal behavioral and activation deficit in schizophrenia.

Hanlon, Faith M.; Houck, Jon M.; Pyeatt, Clinton J.; Lundy, S. Laura; Euler, Matthew J.; Weisend, Michael P.; Thoma, Robert J.; Bustillo, Juan R.; Miller, Gregory A.; Tesche, Claudia D.

2014-01-01

241

Postpubertal cherubism with Noonan syndrome.  

PubMed

Cherubism is a self limiting, autosomal dominant, fibro-osseous lesion of the maxillo facial region affecting the young adults. The etiology is considered to be a mutation of the SH3BP2 gene from chromosome 4p16.3. Sporadic non-familial cases have also been reported. The affected subjects usually present with simultaneous, bilateral swellings of the maxillae and/or mandible, premature loss of primary teeth, malocclusion due to disturbed eruption pattern of permanent teeth and a characteristic chubby faced angelic eyed appearance. This usually presents as an isolated finding, however, it can exist in association with syndromes like Noonan's syndrome, Jaffe syndrome, Gardener syndrome and Ollier's disease. Treatment is focused on the exact genetic diagnosis and management of symptomatic conditions utilizing cosmetic surgery and orthodontics. Genetic counselling of the affected families may help to decrease its incidence. This report presents a rare case of cherubism with associated features of Noonan syndrome. PMID:24718001

Ahmed, Bilal; Amin, Muhammad

2014-03-01

242

Bilateral Acute Epidural Hematoma with Good Outcome  

PubMed Central

Epidural haematomas are one of the most common complicated closed-head injuries, but they, rarely show any bilateral localization. We are reporting here a case of a man found unconscious with Glasgow Coma Scale score; 8/15. Computed tomography of skull revealed bilateral epidural hematoma. Two emergency craniotomies were performed simultaneously, with satisfactory radiological control and neurological outcome. We discussed the aspects of a etiology and treatment about this unusual condition.

Paiva, Wellingson Silva; Andrade, Almir Ferreira De; Alves, Aderaldo Costa Junior; Ribeiro, Iuri Neville; Teixeira, Manoel Jacobsen

2013-01-01

243

Bilateral bifid mandibular canal: a case report.  

PubMed

The objective of this case report is to help clinicians identify bifid mandibular canals on panoramic radiographs and subsequently use the information in the modification of dental treatment planning. A 45-year old man was referred to the service of Oral Diagnosis and Radiology. Routine panoramic radiography, suggested the presence of bilateral bifid mandibular canals (BMC). Mandibular computed tomography revealed a clear view of bilateral mandibular canals. BMC can be detected on a panoramic radiograph. PMID:19218898

Miloglu, Ozkan; Yilmaz, Ahmet Berhan; Caglayan, Fatma

2009-05-01

244

Bilateral eventration of sciatic nerve.  

PubMed

During routine dissection of a 60 years male cadaver, it was observed that the two divisions of sciatic nerve were separate in the gluteal region on both the sides with the tibial nerve passing below the piriformis and the common peroneal nerve piercing the piriformis muscle. The abnormal passage of the sciatic nerve (SN), the common peroneal nerve (CPN), and the tibial nerve (TN), either through the piriformis or below the superior gemellus may facilitate compression of these nerves. Knowledge of such patterns is also important for surgeons dealing with piriformis syndrome which affects 5-6% of patients referred for the treatment of back and leg pain. A high division may also account for frequent failures reported with the popliteal block. PMID:22049898

Sharma, T; Singla, R K; Lalit, M

2010-01-01

245

Bilateral Keratectasia 34 Years after Corneal Transplant  

PubMed Central

We report the clinical findings of a patient with severe bilateral keratectasia 34 years after a penetrating keratoplasty (PK) in both eyes. An otherwise healthy 67-year-old man complained of deterioration of the eyesight in both eyes over the last 6 months. The patient was diagnosed with bilateral keratoconus at the age of 32 years, and he underwent a bilateral PK. At presentation, visual acuity was 20/200 in the right eye and light perception in the left eye. A Pentacam pachymetric map revealed a central pachymetry of 720 ?m in the right eye and of 710 ?m in the left eye, as well as an average paracentral pachymetry of 436 and 270 ?m in the 9-mm zone in the right and the left eye, respectively. Corneal topography revealed bilateral irregular and asymmetric bowing with generalized steepening and high corneal power. We describe a case of bilateral keratectasia 34 years after PK in a patient who was originally diagnosed with bilateral keratoconus.

Valldeperas, Xavier; Angi, Martina; Romano, Vito; Romano, Mario R.

2010-01-01

246

Bilateral Carotid Paraganglioma: Surgery and Radiotherapy  

PubMed Central

BACKGROUND Paragangliomas are relatively rare vascular tumors that develop from the neural crest cells of carotid bifurcation. They usually present as slow-growing, painless unilateral neck masses; bilateral presentation is rare and is mostly associated with familial forms. Bilateral total resection is not always possible for high-grade bilateral tumors, and radiotherapy is a good alternative, with cure rates similar to surgery. CASE REPORT A 35-year-old female patient was admitted with a chief complaint of a bilateral, painless mass located on her neck. Subsequent magnetic resonance imaging (MRI) and angiographic imaging revealed bilateral hypervascular masses surrounding her carotid at 360°, and they were interpreted as stage 3 carotid paragangliomas according to the Shamblin classification protocol. Surgery was carried out on the left carotid paraganglioma and the mass was totally resected. It was thought that the patient could not tolerate bilateral surgery. Primary radiotherapy was planned on the right carotid paraganglioma: 59.8 gray (Gy) conformal, Linac-based multileaf collimator radiotherapy with a 180 cGy daily dosage, and five fractions per week were planned. RESULTS Follow-up at 3 months following the conclusion of radiotherapy revealed no significant regression. A follow-up MRI 6 months and 24 months later revealed 59% regression. Grade 2 esophagitis and minimal neck edema were the only complications noted during the course of radiotherapy and during the 24-month follow-up period. No complications or relapse were observed except for edema following neck surgery.

Kiziltan, Huriye S; Ozucer, Berke; Eris, Ali H; Veyseller, Bayram

2014-01-01

247

Bilateral microvascular second toe transfer for bilateral post-traumatic thumb amputation  

PubMed Central

In bilateral thumb amputations, the functional impairment is serious and every attempt should be made to reconstruct the thumb. We report a case of bilateral post traumatic thumb amputation, reconstructed with bilateral second toe transfer. Only two such cases have been reported in literature so far. Though there are various modalities for the reconstruction of thumb, microvascular toe transfer has its own merits. The convalescent period is minimal with excellent function. It is bilaterally symmetric and aesthetically superior to the osteoplastic reconstruction. The technical details are discussed, and the long term functional and aesthetic results are presented.

Nehete, Rajendra; Nehete, Anita; Singla, Sandeep; Adhav, Harshad

2012-01-01

248

Severe chemosis in a patient with nephrotic syndrome  

Microsoft Academic Search

Chemosis is a rare and reversible complication of nephrotic syndrome in children. However, it is not a threatening condition\\u000a as there are no complications of its own. We report a 15-year-old girl with steroid-resistant nephrotic syndrome with bilateral\\u000a chemosis recovering along with the disappearance of the anasarca.

Augustina Jankauskiene; Inga Buteikiene

2009-01-01

249

Major vascular complications in Beh?et's syndrome  

PubMed Central

Two patients with Behçet's syndrome are described. One patient developed spontaneous bilateral popliteal artery aneurysms and the other spontaneous thrombosis of the superior vena cava. These cases emphasize that primary inflammatory lesions of large vessels, with arterial aneurysm formation and thrombosis may be a feature of the syndrome. ImagesFig. 1Fig. 2Fig. 3Fig. 4Fig. 5Fig. 6Fig. 7

Enoch, B. Anthony; Khoo, T. C. L.; Castillo-Olivares, J. L.; Grainger, R. G.; Henry, L.

1968-01-01

250

Sjögren-Larsson syndrome--unusual presentation with pathological femoral neck fracture: a case report.  

PubMed

Patients with Sjögren-Larsson syndrome, an autosomal recessive syndrome characterized by ichthyosis, spastic diplegia/quadriplegia, mental retardation and ocular features that include pigmentary changes in the retina, rarely present to the orthopaedic surgeon. We detail a rare and unusual presentation of Sjögren-Larsson syndrome in an adolescent boy with bilateral femoral neck fractures. PMID:22828186

Balakumar, Balasubramanian; Arora, Sanjay; Palocaren, Thomas

2012-11-01

251

Bilateral anterior shoulder dislocation with bilateral fractures of the greater tuberosity: A case report  

PubMed Central

Bilateral anterior dislocation of the shoulders with fractures of both greater tuberosities is very rare. A 76-year-old woman sustained a bilateral anterior dislocation of her shoulders with fractures of the greater tuberosity on both sides after a fall on stairs. Her arms were abducted and externally rotated. Radiological examination revealed the bilateral anterior dislocation and also the bilateral fractures of the greater tuberosity. Prompt closed reduction followed by a 3 weeks immobilization and subsequent rehabilitation allows a good outcome. Results at one-year follow-up were satisfactory with normal range of motion and no redislocations occurring. To our knowledge, this is the first reported case of bilateral anterior shoulder dislocation associated with fractures of both greater tuberosities in elderly woman.

Dlimi, F.; Mahfoud, M.; Lahlou, A.; El Bardouni, A.; Berrada, M.S.; El Yaacoubi, M.

2012-01-01

252

Transareola single-site laparoendoscopic bilateral thyroidectomy.  

PubMed

Abstract Background: Transareola single-site endoscopic thyroidectomy has been successfully established as a surgical approach. This study investigated the feasibility and safety of transareola single-site endoscopic thyroidectomy for bilateral thyroid disease. Patients and Methods: Twelve patients who underwent bilateral thyroidectomy were enrolled in this study. The surgical outcomes were analyzed, including operation time, intraoperative bleeding volume, postoperative pain score, and cosmetic satisfaction score. Results: All patients underwent successful transareola single-site endoscopic bilateral thyroidectomy, and no patient was semiconverted to three-port endoscopic surgery or open surgery. Seven patients underwent bilateral partial thyroidectomy, and 5 patients underwent subtotal thyroidectomy plus contralateral partial thyroidectomy. The mean operation time was 165±23.8 minutes (range, 142-185 minutes). The mean intraoperative bleeding volume was 27.3±12.3?mL (range, 20-45?mL). The mean postoperative wound drainage was 121±45.8?mL (range, 85-137?mL). The drainage tube was removed 3-4 days after surgery. The mean visual analog scale score was 3.3±2.5 (range, 1-5) at 24 hours postoperatively. The patients were followed up for 2 month with no complaint of chest wall wound pain and numbness. The mean cosmetic satisfaction score was 9.55±0.8 (range, 8-10). Conclusions: Transareola single-site endoscopic bilateral thyroidectomy is feasible and safe and has the advantages of high cosmetic satisfaction. PMID:24785137

Zhu, Guanghui; Zhang, Ming; Zhang, Xueli; Zhou, Lianming; Wang, Shiguang; Tang, Zhiqiang; Shan, Yuanzhou

2014-06-01

253

[High-degree bilateral carotid stenoses].  

PubMed

The indication for carotid endarterectomy in patients with unilateral stenotic lesions as well as the operative risk in patients with bilateral disease is still controversial among experts. We performed a retrospective analysis in our patients with bilateral carotid endarterectomy. 578 patients underwent carotid endarterectomy at our institution from 1986-1992: 54 patients (9.3%) had bilateral carotid disease. 30% of these patients were asymptomatic and 16% had symptoms from both sides. Surgical results concerning the optimal restoration of blood supply (75%) and the occurrence of recurrent stenosis (8%) were comparable for patients with unilateral and bilateral carotid endarterectomy. On the contrary, in patients with bilateral carotid disease, both the operative morbidity (2.8%) and mortality (1.8%) were increased as compared to the total study population (1.9% and 0.45% resp.). Carotid endarterectomy is very effective in preventing stroke especially in patients with multiple vessel disease as compared to the natural history of these lesions. PMID:8036836

Fraunhofer, S; Kiossis, D; Helmberger, H; von Sommoggy, S; Maurer, P C

1994-01-01

254

Adult-onset bulbar ptosis in Joubert syndrome  

PubMed Central

In this case report, we describe a case of adult-onset bulbar ptosis in a patient with Joubert syndrome. Joubert syndrome is a rare neurodevelopmental disorder with malformations in cerebellum and brainstem. Many ocular abnormalities have been noted in Joubert syndrome, but the association of this syndrome with adult-onset ptosis has not been described to date. This 24-year-old Joubert patient developed a cerebrospinal fluid cyst in her midbrain. She had signs of bilateral third nerve palsy and abducens palsy in the left eye. The bilateral central third nerve palsy causing functional blindness secondary to severe bilateral levator palsy was treated successfully with silicone sling frontalis suspension, as the seventh nerve nucleus was not involved.

Burt, Benjamin; Levine, Johanan; Le, Kim

2012-01-01

255

Fragile lung in the Marfan syndrome.  

PubMed Central

Two cases of the Marfan syndrome presented with spontaneous pneumothorax. Both had chest radiographs showing bilateral bullae in the upper lung zones and pulmonary function tests consistent with mild emphysema. There were dereases in forced expiratory flow rates at low lung volumes, carbon monoxide transfer factor, and lung elastic recoil. It is suggested that pneumothorax and bullous emphysema in this syndrome are caused by a weakness in the pulmonary connective tissue framework.

Turner, J A; Stanley, N N

1976-01-01

256

Langerhans Cell Histiocytosis in Bilateral Mastoid Cavity  

PubMed Central

A 39-year-old male was admitted to our clinic with symptoms of headache, dizziness, nausea, otalgia, otorrhea, tinnitus, and hearing loss in both ears for 3 weeks. Physical examination revealed edema in the tympanic membrane and external ear canal, and pain by palpation in the mastoid area bilaterally. There was no nystagmus, and the rest of the physical examination was otherwise normal. Temporal bone high resolution computed tomography (CT) showed a lesion causing erosion in the mastoid cortex, tegmen tympani, ossicles, and in the bone covering the sigmoid sinus bilaterally. There was also erosion in the superior semicircular canal and petrous bone on the left side. Cortical mastoidectomy was performed under general anesthesia. Histopathologic examination of the tissue revealed Langerhans cell histiocytosis (LCH). In this paper a case with LCH, presenting with bilateral mastoid involvement which has been rarely reported in the literature, is discussed with the existing literature.

Bozdemir, Kaz?m; Tarlak, Behcet; Cakar, Hasan; Doblan, Ahmet; Kutluhan, Ahmet; Dilek, Imdat; Ad?yaman Sungu, Nuran

2013-01-01

257

Bilateral Absence of the Superior Vena Cava  

PubMed Central

Bilateral absence of the superior vena cava (SVC) is a very rarely detected, mainly asymptomatic congenital vascular anomaly. Though usually innocent, this anomaly may complicate cardiothoracic surgery and certain procedures like central venous catheter insertion. This SVC anomaly is poorly known, and we assume that its incidence in the general population may be higher than detected. In this paper, we summarize current knowledge on this anomaly and its clinical implications. In addition, we present a neonatal case with bilateral absence of the SVC associated with a fetal cystic hygroma. Conclusion. Totally absent SVC can cause unexpected problems during cardiothoracic surgery. Suspicion of SVC absence should arise in basic echocardiography. Our paper suggests that, like other congenital anomalies, bilateral absent SVC may be associated with a fetal cyctic hygroma.

Ylanen, Kaisa; Poutanen, Tuija; Savikurki-Heikkila, Paivi; Uotila, Jukka; Korppi, Matti; Eerola, Anneli

2012-01-01

258

Bilateral representation in the deep cerebellar nuclei  

PubMed Central

The cerebellum is normally assumed to represent ipsilateral movements. We tested this by making microelectrode penetrations into the deep cerebellar nuclei (mainly nucleus interpositus) of monkeys trained to perform a reach and grasp task with either hand. Following weak single electrical stimuli, many sites produced clear bilateral facilitation of multiple forelimb muscles. The short onset latencies, which were similar for each side, suggested that at least some of the muscle responses were mediated by descending tracts originating in the brainstem, rather than via the cerebral cortex. Additionally, cerebellar neurones modulated their discharge with both ipsilateral and contralateral movements. This was so, even when we carefully excluded contralateral trials with evidence of electromyogram modulation on the ipsilateral side. We conclude that the deep cerebellar nuclei have a bilateral movement representation, and relatively direct, powerful access to limb muscles on both sides of the body. This places the cerebellum in an ideal position to coordinate bilateral movements.

Soteropoulos, Demetris S; Baker, Stuart N

2008-01-01

259

Bilateral adrenal histoplasmosis in immunocompetent patients.  

PubMed

Histoplasmosis is a fungal infection caused by Histoplasma capsulatum, which commonly presents as transient pulmonary infection that usually subsides without treatment. Disseminated histoplasmosis may affect almost all systems, including the reticuloendothelial system, lungs, gastrointestinal tract, renal tract, central nervous system, bone marrow, and adrenal glands. Adrenal gland is frequently involved in disseminated histoplasmosis but commonly present as unilateral mass; bilateral involvement is rare. It is also rare in immunocompetent hosts and only few cases have been reported in past. We report four cases of adrenal histoplasmosis with bilateral involvement. These cases were initially diagnosed cytopathologically on fine needle aspirations performed on adrenal glands. All these patients were immunocompetent and two of them also had associated adrenal insufficiency. Diagnosis of adrenal histoplasmosis can be easily made on fine needle aspiration cytology. Adrenal histoplasmosis can occur in immunocompetent individuals and should be considered in differential diagnosis of bilateral adrenal masses in immunocompetent individuals. PMID:20607678

Rana, Chanchal; Krishnani, Narendra; Kumari, Niraj

2011-04-01

260

Asperger Syndrome  

MedlinePLUS

NINDS Asperger Syndrome Information Page Condensed from Asperger Syndrome Fact Sheet Table of Contents (click to jump to sections) ... Trials Organizations Additional resources from MedlinePlus What is Asperger Syndrome? Asperger syndrome (AS) is a developmental disorder. ...

261

Cushing's Syndrome  

MedlinePLUS

NINDS Cushing's Syndrome Information Page Synonym(s): Hypercortisolism Table of Contents (click to jump to sections) What is Cushing's Syndrome? Is ... is being done? Clinical Trials Organizations What is Cushing's Syndrome? Cushing's syndrome, also called hypercortisolism , is a rare ...

262

Fanconi syndrome  

MedlinePLUS

De Toni-Fanconi syndrome ... Fanconi syndrome can be caused by faulty genes, or it may result later in life due to kidney damage. Sometimes the cause of Fanconi syndrome is unknown. Common causes of Fanconi syndrome ...

263

GPR56-related bilateral frontoparietal polymicrogyria: further evidence for an overlap with the cobblestone complex.  

PubMed

GPR56 mutations cause an autosomal recessive polymicrogyria syndrome that has distinctive radiological features combining bilateral frontoparietal polymicrogyria, white matter abnormalities and cerebellar hypoplasia. Recent investigations of a GPR56 knockout mouse model suggest that bilateral bifrontoparietal polymicrogyria shares some features of the cobblestone brain malformation and demonstrate that loss of GPR56 leads to a dysregulation of the maintenance of the pial basement membrane integrity in the forebrain and the rostral cerebellum. In light of these findings and other data in the literature, this study aimed to refine the clinical features with the first description of a foetopathological case and to define the range of cobblestone-like features in GPR56 bilateral bifrontoparietal polymicrogyria in a sample of 14 patients. We identified homozygous GPR56 mutations in 14 patients from eight consanguineous families with typical bilateral bifrontoparietal polymicrogyria and in one foetal case, out of 30 patients with bifrontoparietal polymicrogyria referred for molecular screening. The foetal case, which was terminated at 35 weeks of gestation in view of suspicion of Walker Warburg syndrome, showed a cobblestone-like lissencephaly with a succession of normal, polymicrogyric and 'cobblestone-like' cortex with ectopic neuronal overmigration, agenesis of the cerebellar vermis and hypoplastic cerebellar hemispheres with additional neuronal overmigration in the pons and the cerebellar cortex. The 14 patients with GPR56 mutations (median 8.25 years, range 1.5-33 years) were phenotypically homogeneous with a distinctive clinical course characterized by pseudomyopathic behaviour at onset that subsequently evolved into severe mental and motor retardation. Generalized seizures (12/14) occurred later with onset ranging from 2.5 to 10 years with consistent electroencephalogram findings of predominantly anterior bursts of low amplitude ?-like activity. Neuroimaging demonstrated a common phenotype with bilateral frontoparietally predominant polymicrogyria (13/13), cerebellar dysplasia with cysts mainly affecting the superior vermis (11/13) and patchy to diffuse myelination abnormalities (13/13). Additionally, the white matter abnormalities showed a peculiar evolution from severe hypomyelination at 4 months to patchy lesions later in childhood. Taken as a whole, these observations collectively demonstrate that GPR56 bilateral bifrontoparietal polymicrogyria combines all the features of a cobblestone-like lissencephaly and also suggest that GRP56-related defects produce a phenotypic continuum ranging from bilateral bifrontoparietal polymicrogyria to cobblestone-like lissencephaly. PMID:20929962

Bahi-Buisson, Nadia; Poirier, Karine; Boddaert, Nathalie; Fallet-Bianco, Catherine; Specchio, Nicola; Bertini, Enrico; Caglayan, Okay; Lascelles, Karine; Elie, Caroline; Rambaud, Jérôme; Baulac, Michel; An, Isabelle; Dias, Patricia; des Portes, Vincent; Moutard, Marie Laure; Soufflet, Christine; El Maleh, Monique; Beldjord, Cherif; Villard, Laurent; Chelly, Jamel

2010-11-01

264

Generalized bilateral control of robot arms  

NASA Technical Reports Server (NTRS)

This paper summarizes the theory of a new form of bilateral or force-reflecting manual control of remote robot (slave) arms. The mathematics of workspace representation, command structure, kinematic and dynamic control coordination required for implementing the new form of generalized bilateral control through computer programs is presented. The paper concludes with a brief description of implementation organization of the new system using a six-degree-of-freedom backdrivable hand controller, a PUMA 560 robot arm and a distributed net of microcomputers organized in two groups: one at the remote work site and one at the control station.

Bejczy, A. K.; Lee, S.

1984-01-01

265

Bilateral Simultaneous Stage 1 Macular Hole  

PubMed Central

The authors describe two cases of bilateral simultaneous stage 1 macular hole diagnosed via optical coherence tomography (OCT). Vitreomacular traction, foveal pseudo cysts, and outer retinal changes were present initially. Resolution of the foveal pseudo cysts and outer retinal changes occurred in cases where a complete posterior vitreous detachment was noted. The initial step in the pathogenesis of macular holes is “traction” from the detaching posterior hyaloid. However, there are missing links in the exact inciting events and in the progression of the disease. The development of bilateral stage 1 macular holes simultaneously is unique and interesting because an unknown common inciting factor might be at play in these cases.

Alwassia, Ahmad A.; Adhi, Mehreen; Duker, Jay S.

2014-01-01

266

Bilateral congenital third cranial nerve palsy.  

PubMed

We describe a patient in whom bilateral congenital third cranial nerve palsy was diagnosed at 2 weeks of age. The ptosis was treated at 3 weeks with bilateral eyelid suspension surgery, and surgical repair of the exotropia was done at 4 months. To our knowledge this is the fourth reported case of this condition. It was probably caused by a single lesion involving the oculomotor and possibly the trochlear nuclei in the brain stem. Magnetic resonance imaging scans supported this hypothesis and suggested other central nervous system anomalies. PMID:2713752

Flanders, M; Watters, G; Draper, J; O'Gorman, A

1989-02-01

267

Bilateral Hypertrophic Olivary Degeneration in Wilson Disease  

PubMed Central

Hypertrophic olivary degeneration resulting from lesions of the dento-rubro-olivary pathway, also called Guillain-Mollaret-triangle, has been described previously in a number of cases. Reports about bilateral hypertrophic olivary degeneration of the inferior olivary nuclei are very limited, and the magnetic resonance imaging findings of hypertrophic olivary degeneration in Wilson disease have not yet been described to the best of our knowledge. Herein, we present the first report of bilateral hypertrophic olivary degeneration diagnosed by magnetic resonance imaging in a patient suffering from Wilson disease.

Guenther, Peter; Hoffmann, Karl-Titus

2013-01-01

268

Bilateral vision loss responsive to corticosteroids.  

PubMed

A 48-year-old woman presented with painless bilateral vision loss that began in the left eye and responded to steroids, followed by vision loss in the right eye one day after completing her steroid taper. Diagnosis was complicated by a positive screening test for Leber hereditary optic neuropathy and a negative workup for demyelinating disease. Steroid-dependent optic neuropathies such as autoimmune optic neuropathy and chronic relapsing inflammatory optic neuropathy were considered in the differential. Seven months after initial presentation, the patient developed a new periventricular white matter lesion, lesions on her cervical and thoracic spinal cord, bilateral leg weakness, and sensory loss consistent with multiple sclerosis. PMID:23153758

Lee, Michele D; Song, Brian J; Odel, Jeffrey G; Sadun, Alfredo A

2013-01-01

269

Prune belly syndrome.  

PubMed

The majority of paediatric surgeons will encounter a patient with prune belly syndrome (PBS) only a few times in their clinical practice. There have been many opposing views in the literature regarding the pathogenesis and management of this complex condition. A detailed review was conducted using PubMed to identify key publications involving PBS. This article discusses the evolution of our understanding of the pathogenesis and diagnosis of PBS, including its typical characteristics. We describe the management options available for bilateral intra-abdominal testes, the deficient abdominal wall, the dilated urinary system and examine the evidence base used to support the current approaches employed. PMID:22198807

Hassett, S; Smith, G H H; Holland, A J A

2012-03-01

270

[Psychological characteristics of Cushing's syndrome].  

PubMed

Psychological state of 15 patients with Cushing's syndrome was studied, including 8 subjects with bilateral adrenocortical hyperplasia, 4 with adrenocortical adenoma, 2 with hypophyseal adenoma and 1 with adrenocortical cancer. The weakened intellect was noted in patients, suffering from adrenocortical hyperplasia. Low emotional stability, anxiety, suspiciousness and hesitation were characteristic of the patients under study. Psychic stability was lowered. A possible role of endogenous and exogenic factors of depression, as well as the conditions of hospitalization for such patients are discussed. PMID:7156071

Alvarez, M A; Navarro, D

1982-01-01

271

Lymphatic abnormalities in Noonan's syndrome.  

PubMed

Five boys who had Noonan's syndrome and lymphatic abnormalities are reported. The youngest boy had clinical lymphoedema and the other four showed dermal backflow after interdigital injection of Patent Blue indicating impairment of flow along the superficial lymphatics. One boy had severe bilateral chylothorax. The lymphographic findings in four of these boys are reported. Patients with the Noonan syndrome frequently have oedema of the hands and feet at birth, which decreases during the first years of life [10]. It has been demonstrated by lymphography that similar peripheral oedema in patients with the Turner's syndrome is due to lymphatic hypoplasia [1, 3]. We report certain lymphatic abnormalities diagnosed by lymphography in four out of five patients with Noonan's syndrome. PMID:673526

Lanning, P; Similä, S; Suramo, I; Paavilainen, T

1978-06-19

272

Neglected surgically intervened bilateral congenital dislocation of knee in an adolescent  

PubMed Central

Neglected bilateral congenital dislocation of knee is unusual. A 12 year old boy presented with inability to walk due to buckling of the knee. The symptoms were present since the child learnt walking. He preferred not to walk. Bilateral supracondylar femoral osteotomy was done at the age of 6 years. Patient had a fixed flexion deformity of both knees, 30° in the right (range of flexion from 30° to 45°) and 45° fixed flexion deformity in left knee respectively (range of flexion from 45° to 65°) when presented to us. The radiological examination revealed bilateral congenital dislocation of knee (CDK). No syndromic association was observed. He was planned for staged treatment. In stage I, the knee joints were distracted by Ilizarov ring fixators and this was followed by open reduction of both the knee joints in stage II. A bilateral supracondylar extension osteotomy was done 18 months after the previous surgery (stage III). The final followup visit at 4 years the patient presented with range of motion 5-100° and 5-80° on the right and left knee respectively with good functional outcome. The case is reported in view of lack of treatment guidelines for long standing neglected CDK in an adolescent child.

Kumar, Jaswant; Dhammi, Ish Kumar; Jain, Anil K

2014-01-01

273

Neglected surgically intervened bilateral congenital dislocation of knee in an adolescent.  

PubMed

Neglected bilateral congenital dislocation of knee is unusual. A 12 year old boy presented with inability to walk due to buckling of the knee. The symptoms were present since the child learnt walking. He preferred not to walk. Bilateral supracondylar femoral osteotomy was done at the age of 6 years. Patient had a fixed flexion deformity of both knees, 30° in the right (range of flexion from 30° to 45°) and 45° fixed flexion deformity in left knee respectively (range of flexion from 45° to 65°) when presented to us. The radiological examination revealed bilateral congenital dislocation of knee (CDK). No syndromic association was observed. He was planned for staged treatment. In stage I, the knee joints were distracted by Ilizarov ring fixators and this was followed by open reduction of both the knee joints in stage II. A bilateral supracondylar extension osteotomy was done 18 months after the previous surgery (stage III). The final followup visit at 4 years the patient presented with range of motion 5-100° and 5-80° on the right and left knee respectively with good functional outcome. The case is reported in view of lack of treatment guidelines for long standing neglected CDK in an adolescent child. PMID:24600070

Kumar, Jaswant; Dhammi, Ish Kumar; Jain, Anil K

2014-01-01

274

Neonatal status epilepticus controlled with levetiracetam at Sturge Weber syndrome.  

PubMed

Sturge-Weber syndrome is a rare, sporadic, congenital neurocutaneous syndrome characterized by facial cutaneous vascular malformation, leptomeningeal angioma and eye abnormalities. Seizures develop during the first year of life, may become refractory to multiple anticonvulsants and status epilepticus may develop. A rare subtype of Sturge-Weber syndrome with bilateral facial vascular malformation, unilateral cerebral involvement and neonatal status epilepticus is reported here. Neonatal status epilepticus was successfully controlled with intravenous levetiracetam infusion. PMID:22804835

Tanriverdi, Sema; Terek, Demet; Koroglu, Ozge Altun; Yalaz, Mehmet; Tekgul, Hasan; Kultursay, Nilgun

2013-04-01

275

Opercular syndrome: A case report and review  

PubMed Central

We present a 9-year-old boy with history of perinatal asphyxia and neonatal seizures; who presented with delayed development of speech, with predominant dysarthria, dysphagia, and drooling of saliva and unable to protrude tongue along with delayed motor and mental milestones. He had complex partial seizures since last 3 years requiring multiple anti-epileptic drugs. He had dysarthria, nasal twang, and drooling of saliva with difficulty in chewing and swallowing. Hearing and understanding were normal. Bilateral trigemino-facio-linguo-pharyngeal palsy was noticed on voluntary movements with normal jaw jerk with preserved automatic and emotional motor movements. Electroencephalography revealed focal left fronto-temporal epileptiform discharges and brain imaging was suggestive of bilateral cortical and subcortical region encephalomalacia, predominantly involving bilateral opercular region. The clinical and neuroimaging features correspond to bilateral opercular syndrome which could have resulted from the perinatal insult in this case.

Desai, Soaham Dilip; Patel, Dipen; Bharani, Sheela; Kharod, Nikhil

2013-01-01

276

Opercular syndrome: A case report and review.  

PubMed

We present a 9-year-old boy with history of perinatal asphyxia and neonatal seizures; who presented with delayed development of speech, with predominant dysarthria, dysphagia, and drooling of saliva and unable to protrude tongue along with delayed motor and mental milestones. He had complex partial seizures since last 3 years requiring multiple anti-epileptic drugs. He had dysarthria, nasal twang, and drooling of saliva with difficulty in chewing and swallowing. Hearing and understanding were normal. Bilateral trigemino-facio-linguo-pharyngeal palsy was noticed on voluntary movements with normal jaw jerk with preserved automatic and emotional motor movements. Electroencephalography revealed focal left fronto-temporal epileptiform discharges and brain imaging was suggestive of bilateral cortical and subcortical region encephalomalacia, predominantly involving bilateral opercular region. The clinical and neuroimaging features correspond to bilateral opercular syndrome which could have resulted from the perinatal insult in this case. PMID:24082930

Desai, Soaham Dilip; Patel, Dipen; Bharani, Sheela; Kharod, Nikhil

2013-05-01

277

Retinal vasculitis in Susac syndrome: case report  

Microsoft Academic Search

A 29 year old male was admitted at the emergency room suffering from gradually worsening headache followed by nausea. In the hospital patient presented with lethargy, reduction of consciousness level and bilateral hypoacusis. Ophthalmic examination and fluorescein angio- graphy showed retinal vasculitis. This finding was crucial to the diag- nosis of Susac syndrome, a rare disease characterized by vasculopathy of

Francisco Azevedo Marquardt; Heriberto Pinto; Guimarães Neto; Daniel Azevedo Marquardt

278

Goldenhar Syndrome: A Report of 3 Cases  

PubMed Central

We report here 3 cases with the classic signs of Goldenhar syndrome in the form of multiple accessory tragi, bilateral ocular dermoids, mandibular hypoplasia (micrognathia), and facial microsomia. One of the patients also had vitiligo, which is yet to be reported as an association.

Gaurkar, Sudarshan P; Gupta, Khushboo D; Parmar, Kirti S; Shah, Bela J

2013-01-01

279

[Diagnostic image (70). A man with fever, swollen joints and erythema nodosum. Loefgren's syndrome].  

PubMed

A 39-year-old man presented with fever, arthritis of knees and wrists, periarticular ankle inflammation, erythema nodosum, bilateral hilar adenopathy and diffuse pulmonary parenchymal changes, due to Löfgren's syndrome. PMID:11802332

Hofstee, H M A; Folmer, S C C Reinders

2002-01-01

280

Effective Treatment with Topical Cyclosporin A of a Patient with Cogan Syndrome  

Microsoft Academic Search

The purpose of this report is to describe the effective treatment of severe anterior segment inflammation due to Cogan syndrome through the use of topical administration of cyclosporin A. A 47-year-old female patient had been experiencing headaches and difficulties with her vision. Subsequent examination revealed the sudden onset of bilateral conjunctival injection and swelling of bilateral auricles. Despite the multiple

Masahiko Shimura; Kanako Yasuda; Nobuo Fuse; Mitsuru Nakazawa; Makoto Tamai

2000-01-01

281

Successful management of a bilateral diaphragmatic lymphangioma.  

PubMed

A 5-year-old boy developed two episodes of massive chylothorax due to a huge bilateral diaphragmatic lymphangioma. Two episodes of chylothorax were well controlled only after parenternal octreotide. The lymphangioma showed more than 90% shrinkage after intralesional sclerotherapy with bleomycin. PMID:17043877

Ming, Yung-Ching; Wong, Kin-Sun; Wang, Chao-Jan; Lai, Jin-Yao

2007-06-01

282

Successful management of a bilateral diaphragmatic lymphangioma  

Microsoft Academic Search

A 5-year-old boy developed two episodes of massive chylothorax due to a huge bilateral diaphragmatic lymphangioma. Two episodes\\u000a of chylothorax were well controlled only after parenternal octreotide. The lymphangioma showed more than 90% shrinkage after\\u000a intralesional sclerotherapy with bleomycin.

Yung-Ching Ming; Kin-Sun Wong; Chao-Jan Wang; Jin-Yao Lai

2007-01-01

283

Bilateral adrenal lymphoma presenting as Addison's disease  

Microsoft Academic Search

We describe an unusual case of non-Hodgkin's lymphoma of the adrenals which presented as Addison's disease. Examination of tissue taken by computed tomography guided biopsy revealed a high grade B cell centroblastic lymphoma. The patient was treated with chemotherapy but died from invasive aspergillosis. Autopsy confirmed bilateral adrenal involvement by lymphoma.

A. Pagliuca; D. S. Gillett; J. R. Salisbury; R. N. Basu; G. J. Mufti

1989-01-01

284

BILATERAL MONOPOLIES AND INCENTIVES FOR MERGER  

Microsoft Academic Search

We analyze a duopoly in which firms acquire inputs through bilateral monopoly relations with suppliers. We combine a bargaining model with a duopoly model to examine how input prices and profits are affected by the structures of the upstream and downstream industries, by the demand relations among the final products, and by the nature of bargaining between suppliers and firms.

Henrick Horn; Asher Wolinsky

1988-01-01

285

Silicosis presenting as bilateral hilar lymphadenopathy.  

PubMed Central

Classical radiographic features of patients presenting with silicosis are diffuse interstitial shadowing with subsequent enlargement of hilar nodes, sometimes with "eggshell" calcification. Five case histories are described of workers who were exposed to silica and presented initially with bilateral hilar lymphadenopathy without radiographic evidence of interstitial lung disease. One case progressed to show features of silicosis. Images

Baldwin, D R; Lambert, L; Pantin, C F; Prowse, K; Cole, R B

1996-01-01

286

Hypersexuality following bilateral thalamic infarction: case report  

Microsoft Academic Search

Hypersexuality is a rare but well recognized condition following brain injury. It has been described secondarily to dysfunction in the hypothalamus, the temporal and frontal lobes. We re p o rt a 63 y e a r-old man that developed neuropsychological disturbances with hypersexuality as a prominent feature , disinhibition and moderate memory loss, hypersomnia and irritability after a bilateral

Eduardo G. Mutarelli; Antonio M. P. Omuro; Tarso Adoni

2006-01-01

287

"Ostrich sign" indicates bilateral vertebral artery dissection.  

PubMed

Vertebral artery dissections (VADs) comprise about 2% of ischemic strokes and can be associated with trauma, chiropractic manipulation, motor vehicle collisions, whiplash, amusement park rides, golfing, and other motion-induced injuries to the neck. We present a case of bilateral extracranial VAD as a complication of conducting an orchestra. To our knowledge, this has not been documented in the literature. Conceivably, vigorous neck twisting in an inexperienced, amateur conductor may place excessive rotational forces upon mobile portions of the verterbral arteries, tear the intima, deposit subintimal blood that extends longitudinally, and cause neck pain and/or posterior fossa ischemic symptoms. Magnetic resonance angiography examinations of axially oriented slices of bilateral VADs resemble the face of an ostrich. This observation is similar to the "puppy sign," in which bilateral internal carotid artery dissections resemble the face of a dog. Craniocervical dissections of either the carotid or vertebral arteries have the potential to form an aneurysm, cause artery-to-artery embolism, or completely occlude the parent artery, resulting in an ischemic stroke. Because bilateral VADs in axial magnetic resonance angiographic sections stand out like the eyes of an ostrich, and because the fast identification of VADs is so critical, we eponymize this image the "ostrich sign." PMID:21440457

Rose, David Z; Husain, M Rizwan

2012-11-01

288

Bilateral central scotomata due to intracranial tumour.  

PubMed Central

Bilateral centrocaecal scotomata have been recognised as a sign of intrinsic optic nerve disease, usually associated with hereditary optic neuropathy, and nutritional or toxic amblyopias. This report describes four patients with central scotomata due to intracranial masses, three of whom recovered after surgical intervention. The clinician should be alerted to the association in patients with headaches, other neurological signs, and central visual loss. Images

Page, N G; Sanders, M D

1984-01-01

289

Bilateral scalp necrosis with giant cell arteritis.  

PubMed

We present a patient with bilateral scalp necrosis caused by giant cell arteritis (temporal arteritis). A 67-year-old woman, who had been treated with 5 mg of oral prednisolone every other day for polymyalgia rheumatica, developed painful egg-sized regions of necrosis on both of her temples. Doppler pulsemetory revealed bilateral obstruction of the temporal arteries. Biopsy revealed ischemic necrosis of the skin and necrotic angiitis of the temporal arteries with giant cell infiltration. Bilateral stenosis of the internal carotid arteries and moderate retinal bleeding were revealed by angiography. Daily administration of prednisolone (20 mg/day) with intravenous and topical limaprost alphadex markedly improved her condition. The ulcers reepithelized without surgical treatment. There are few reports of bilateral scalp necrosis. Rapid and complete obstruction of the temporal artery may result in this condition. Simultaneous development of two ulcerative lesions in the ventro-parietal cranial regions is thought to correspond to systemic arterial involvement, including involvement of the internal carotid arteries. PMID:12692357

Matsushima, Midori; Yamanaka, Keiichi; Mori, Hitoshi; Murakami, Takaaki; Hakamada, Arata; Isoda, Ken-ichi; Mizutani, Hitoshi

2003-03-01

290

Synchronous bilateral breast cancer in a male.  

PubMed

Male breast cancer, which represents only 1% of all breast cancers, is occasionally associated with a family history of breast cancer. Sporadic male breast cancers presenting with another primary breast cancer are extremely rare. In this article, we report on a 70-year-old male patient with bilateral multifocal and synchronous breast cancer and without a family history of breast cancer. PMID:24319497

Rubio Hernández, María Caridad; Díaz Prado, Yenia Ivet; Pérez, Suanly Rodríguez; Díaz, Ronald Rodríguez; Aleaga, Zaili Gutiérrez

2013-01-01

291

Bilateral Synergy: A Framework for Post-Stroke Rehabilitation  

PubMed Central

Background Unilateral stroke produces debilitating deficits in voluntary control in the contralesional arm, and significant motor coordination deficits in the ipsilesional arm. In addition, patients tend to avoid bilateral arm patterns and during performance of activities of daily living. Nevertheless, upper extremity physical rehabilitation predominantly focuses on motor training activities with only the paretic arm. This can be limiting because of persistent deficits in the ipsilesional arm, and because of the tendency of patients to avoid spontaneous bilateral arm patterns. Proposition Rehabilitation should focus on bilateral training to advance recovery of function in both arms of stroke patients, as well as to facilitate spontaneous bilateral arm use. This paper reviews the rationale for this approach, citing evidence for significant hemisphere specific bilateral motor deficits in stroke patients, which affect both the contralesional and the ipsilesional arm. The rationale for, and advantages of, training both arms simultaneously through bilateral tasks is reviewed. Although bilateral training has been employed to treat stroke patients previously, this has tended to focus on bimanual ‘coupling’ as a rationale for performing parallel, but not cooperative bilateral tasks. Bilateral synergy provides a more functional framework for structuring post-stroke upper extremity rehabilitation. Conclusion Bilateral synergy may be causally linked to spontaneous bilateral arm use, suggesting that rehabilitation should be focused on bilateral cooperative tasks, such as bilateral object transport. Further research is required to determine whether this approach could be efficacious for patients with hemiparesis, and whether both left and right hemisphere strokes can benefit from such intervention.

Sainburg, RL; Good, D; Przybyla, A

2014-01-01

292

[Sturge-Weber syndrome].  

PubMed

Sturge-Weber syndrome (SWS) is a rare congenital disease which affects the brain, the skin and the eyes. It is a sporadically occurring neurocutaneous syndrome affecting the intracerebral veins (venous angiomatosis). The frequency is estimated to be 1 in 50,000 births [1]. The main symptom is intracranial leptomeningeal angiomatosis which mostly affects the occipital and posterior parietal lobes and can occur unilaterally and also bilaterally. Facial cutaneous vascular alterations occur ipsilaterally in the form of port wine stains (nevus flammeus) which are normally found in the catchment area of the trigeminal branch VI. Other clinical symptoms associated with SWS are seizures, glaucoma, headaches, transient neurological stroke-like episodes and cognitive impairment. Neurological cortical symptoms often include hemiparesis, hemiatrophy and hemianopsia. PMID:24292369

Reith, W; Yilmaz, U; Zimmer, A

2013-12-01

293

Increased cardiovascular mortality following early bilateral oophorectomy  

PubMed Central

Objective To investigate the mortality associated with cardiovascular diseases and the effect of estrogen treatment in women who underwent unilateral or bilateral oophorectomy before menopause. Design We conducted a cohort study with long-term follow-up of women in Olmsted County, MN, who underwent either unilateral or bilateral oophorectomy before the onset of menopause from 1950 through 1987. Each member of the oophorectomy cohort was matched by age to a referent woman from the same population who had not undergone any oophorectomy. We studied the mortality associated with cardiovascular disease in a total of 1,274 women with unilateral oophorectomy, 1,091 women with bilateral oophorectomy, and 2,383 referent women. Results Women who underwent unilateral oophorectomy experienced a reduced mortality associated with cardiovascular disease compared with referent women (hazard ratio [HR], 0.82; 95% confidence interval [CI], 0.67–0.99; P = 0.04). By contrast, women who underwent bilateral oophorectomy before age 45 years experienced an increased mortality associated with cardiovascular disease compared with referent women (HR, 1.44; 95% CI, 1.01–2.05; P = 0.04). Within this age stratum, the HR for mortality was significantly elevated in women who were not treated with estrogen through age 45 years or longer (HR, 1.84; 95% CI, 1.27–2.68; P = 0.001) but not in women treated (HR, 0.65; 95% CI, 0.30–1.41; P = 0.28; test of interaction, P = 0.01). Mortality was further increased after excluding deaths associated with cerebrovascular causes. Conclusions Bilateral oophorectomy performed before age 45 years is associated with increased cardiovascular mortality, especially with cardiac mortality. However, estrogen treatment may reduce this risk.

Rivera, Cathleen M.; Grossardt, Brandon R.; Rhodes, Deborah J.; Brown, Robert D.; Roger, Veronique L.; Melton, L. Joseph; Rocca, Walter A.

2008-01-01

294

Bilateral Simultaneous Disc Edema and Cataract Associated with Albright Hereditary Osteodystrophy  

PubMed Central

A 16-year-old female presented with poor vision in both eyes. On clinical examination, she had bilateral cataracts and optic disc edema bilaterally on ultrasound examination. Extensive intracranial calcification was evident on computerized tomography. Physical examination revealed short stature, rounded chubby face, dental abnormalities, brachydactyly, and obesity. Laboratory evidence of hypocalcemia, hyperphosphatemia, elevated parathyroid hormone level (indicative of pseudohypoparathyroidism) along with the constellation of phenotypical characteristics lead to a diagnosis of Albright's hereditary osteodystrophy. This case is being presented to increase awareness regarding presence of coexisting and previously undiagnosed hypocalcemic syndromes in pediatric cataracts. The role of an ophthalmologist may be pivotal in diagnosing such an entity as documented in the present case.

Sengupta, Sabyasachi; Ravindran, Ravilla D.; Kannusamy, Veena; Tamrakar, Varsha

2012-01-01

295

Management of Simultaneous Bilateral Neck of Femur Fractures in an Elderly Patient  

PubMed Central

Simultaneous bilateral neck of femur fracture is rare. There have been few reports of such a condition in the literature. This uncommon pattern of injury has been associated with high-energy trauma, underlying bone disease, and seizure disorders. We describe our experience of such a condition involving an elderly patient with significant cardiovascular comorbidity. The operative approach involved single-stage repair of both the involved joints. Bilateral uncemented hemi-arthroplasty was performed using a single tray of sterile surgical instruments and 2 sterile drapes. We report a satisfactory outcome. Uncemented arthroplasty should be considered in such a case so as to minimize the risk of a possible bone cement implantation syndrome.

McGoldrick, Niall P.; Dodds, Michael K.; Green, Connor; Synnott, Keith

2013-01-01

296

Sporadic bilateral synchronous multicentric papillary renal cell carcinoma masquerading as bilateral multifocal pyelonephritis.  

PubMed

Pyelonephritis is defined as an inflammation of the kidney and renal pelvis. The diagnosis is usually clinical. Acute multifocal bacterial nephritis is a rare form of pyelonephritis that is more severe and sepsis is more common. We report a patient who presented with fever and right-sided abdominal pain associated with right flank tenderness, suggesting right acute pyelonephritis. Bilateral multifocal pyelonephritis was diagnosed on ultrasonography, radionuclide renal scintigraphy and computed tomography. However, owing to non-resolution of symptoms, a biopsy was performed, which showed bilateral papillary renal cell carcinoma (PRCC). PRCC is known to exhibit multicentricity. To our knowledge, a case of bilateral multicentric PRCC masquerading as bilateral multifocal pyelonephritis has not been reported in the English literature. This case highlights the need to be vigilant while treating patients with focal lesions of the kidney as an inflammatory condition lest a malignancy should be missed. PMID:24992402

Karthikeyan, Vs; Dorairajan, Ln; Kumar, S; Vijayakumar, Ar; Ramesh, A; Ganesh Rajesh, N; Halanaik, D; Gupta, A

2014-07-01

297

Congenital glaucoma as an ophthalmic manifestation of Frank-Ter Haar syndrome.  

PubMed

We report on a patient with Frank-Ter Haar syndrome that is associated with high intraocular pressures. A 21-day-old male patient was referred to our clinic for surgical treatment of congenital glaucoma. On ophthalmic examination, he had buphthalmos, mild corneal edema and high IOP readings in both eyes. The patient underwent uneventful trabeculotomy surgery, bilaterally. Marked bilateral anterior iris insertion was noted during the surgery. Childhood glaucoma may be associated with Frank-Ter Haar syndrome. PMID:23709337

Aktas, Zeynep; Karaca, Emine Esra; Dogan, Nurcan; Çakmak, Tugba; Unlu, Metin; Tok, Levent; Hasanreisoglu, Murat

2014-04-01

298

Adams-Oliver syndrome associated with cutis marmorata telangiectatica congenita and congenital cataract: a case report.  

PubMed

A female infant presented with Adams-Oliver syndrome (AOS), intrauterine growth retardation, severe cutis marmorata telangiectatica congenita, bilateral congenital cataract, and periventricular lesions. The here-reported association of bilateral congenital cataract with AOS is original. Adams-Oliver syndrome is a genetic defect that causes a vasculopathy and leads to a variety of phenotypes. This observation further supports the current understanding of the physiopathology of AOS. PMID:16586236

Fayol, Laurence; Garcia, Patricia; Denis, Danièle; Philip, Nicole; Simeoni, Umberto

2006-04-01

299

A review of bilateral training for upper extremity hemiparesis.  

PubMed

Upper extremity hemiparesis is the most common post-stroke disability. Longitudinal studies have indicated that 30-66% of stroke survivors do not have full arm function 6 months post-stroke. The current gold standard for treatment of mild post-stroke upper limb impairment is constraint-induced therapy but, because of the inclusion criteria, alternative treatments are needed which target more impaired subjects. Bilateral arm training has been investigated as a potential rehabilitation intervention. Bilateral arm training encompasses a number of methods including: (1) bilateral isokinematic training; (2) mirror therapy using bilateral training; (3) device-driven bilateral training; and (4) bilateral motor priming. Neural mechanisms mediating bilateral training are first reviewed. The key bilateral training studies that have demonstrated evidence of efficacy will then be discussed. Finally, conclusions are drawn concerning clinical implications based on the reviewed literature. PMID:19517519

Stoykov, Mary Ellen; Corcos, Daniel M

2009-01-01

300

Prenatal diagnosis of VACTERL syndrome and partial caudal regression syndrome: a previously unreported association.  

PubMed

We describe a case of VACTERL syndrome associated with type 1 unilateral caudal regression syndrome. The abnormal sonographic findings at 26 weeks included hemivertebrae, scoliosis, hypoplastic and deformed lumbar spine and sacrum, preaxial polydactyly on the left hand, duplicated hallux on the left foot and hemihypoplasia of the left lower limb, bilateral club foot, and single umbilical artery. Postmortem examination confirmed prenatal sonographic findings with additional findings of supernumerary rib at the lumbar level and anal atresia. PMID:19536863

Gedikbasi, Ali; Yararbas, Kanay; Yildirim, Gokhan; Yildirim, Dogukan; Arslan, Oguz; Gul, Ahmet; Ceylan, Yavuz

2009-10-01

301

Nongranulomatous anterior uveitis in a patient with Usher syndrome  

PubMed Central

A 34-year-old female with Usher syndrome, but no family history of similar illness, presented with complaints of vision reduction, redness, and photophobia. Biomicroscopic examination showed mildly injected conjunctivae bilateral, small, round keratic precipitates; bilateral +2 cells with no flare reaction in the anterior chamber; and bilateral posterior subcapsular cataracts. No associated posterior synechiae, angle neovascularization, or iris changes were detected; normal intraocular pressures were obtained. Fundus examination demonstrated waxy pallor of both optic nerves, marked vasoconstriction in retinal vessels, and retinal bone spicule pigment formation, with a normal macula. Electroretinography confirmed the diagnosis of retinitis pigmentosa, optical coherent tomography was normal and otolaryngology consultation was conducted. To our knowledge, an association between Usher syndrome and bilateral nongranulomatous anterior uveitis has not been previously reported, and our purpose is to report this association.

Alzuhairy, Sultan Abdulaziz S.; Alfawaz, Abdullah

2013-01-01

302

Sequential Bilateral Cochlear Implantation in a Patient with Bilateral Meniere's Disease  

PubMed Central

This case study describes a 45 year old female with bilateral, profound sensorineural hearing loss due to Meniere’s disease. She received her first cochlear implant in the right ear in 2008 and the second cochlear implant in the left ear in 2010. The case study examines the enhancement to speech recognition, particularly in noise, provided by bilateral cochlear implants. Speech recognition tests were administered prior to obtaining the second implant and at a number of test intervals following activation of the second device. Speech recognition in quiet and noise as well as localization abilities were assessed in several conditions to determine bilateral benefit and performance differences between ears. The results of the speech recognition testing indicated a substantial improvement in the patient’s ability to understand speech in noise and her ability to localize sound when using bilateral cochlear implants compared to using a unilateral implant or an implant and a hearing aid. In addition, the patient reported considerable improvement in her ability to communicate in daily life when using bilateral implants versus a unilateral implant. This case suggests that cochlear implantation is a viable option for patients who have lost their hearing to Meniere’s disease even when a number of medical treatments and surgical interventions have been performed to control vertigo. In the case presented, bilateral cochlear implantation was necessary for this patient to communicate successfully at home and at work.

Holden, Laura K.; Neely, J. Gail; Gotter, Brenda D.; Mispagel, Karen M.; Firszt, Jill B.

2012-01-01

303

Bilateral posterior ischemic optic neuropathy after spinal surgery  

Microsoft Academic Search

PURPOSE: To report the association between bilateral posterior ischemic optic neuropathy and spinal surgery.METHOD: Case report.RESULTS: After prone-position spinal surgery of 8 hours’ duration, a 68-year-old woman was completely blind in both eyes. Moderate periorbital edema and temporal conjunctival chemosis were present bilaterally. Ophthalmic examination disclosed normal-appearing optic nerve heads, except for bilateral nasal fullness related to bilateral optic nerve

George Alexandrakis; Byron L. Lam

1999-01-01

304

A new case of Martsolf syndrome.  

PubMed

Martsolf syndrome is an autosomal recessive syndrome characterized by microcephaly, mental retardation, cataract, hypogonadism and short stature. A seven-year-old boy was admitted to the hospital with growth retardation and difficulties in walking. His parents were first cousins. Bilateral lens extraction was performed during infancy because of congenital cataract. On physical examination he had short stature, microcephaly, micropthalmia, hypogonadism, mental retardation. Brain magnetic resonance imaging revealed alterations in the white matter. Up to date very few cases with this syndrome have been reported. This is the first case described in the Turkish population and may add valuable information to the literature. PMID:17515302

Bora, E; Cankaya, T; Alpman, A; Karaca, E; Cogulu, O; Tekgul, H; Ozkinay, F

2007-01-01

305

Cryptogenic organising pneumonia presenting with bilateral hilar and mediastinal lymphadenopathy.  

PubMed

Cryptogenic organising pneumonia is not considered in the differential diagnosis of bilateral hilar and mediastinal lymphadenopathy. We submitted a patient presenting with bilateral hilar and mediastinal lymphadenopathy. We suspected diagnosis of sarcoidosis, but the patient was diagnosed as cryptogenic organising pneumonia with the histological result. This is the second case report of cryptogenic organising pneumonia presenting with bilateral hilar and mediastinal lymphadenopathy. PMID:23761506

Kahraman, Hasan; Tokur, Mahmut; Sayar, Hamide; Inci, Mehmet Fatih

2013-01-01

306

Bilateral staged thoracotomy for multiple lung hydatidosis  

PubMed Central

Background Hydatid cyst disease is still a problem in many countries. Surgical removal is currently the generally accepted choice of treatment for lung hydatidosis. However, operating on bilateral widespread lung hydatidosis is still controversial. The aim of this retrospective study was to evaluate the results of surgical treatment in bilateral multiple hydatid disease of the lung. Methods In this study, we reviewed our experience in the surgical treatment of 17 (3.7%) patients with bilateral, and at least three, lung hydatid cysts. These 17 patients (8 male, 9 female), with an average age of 34.6 years (range 12–58 years), underwent bilateral staged thoracotomy. Results In total 105 lung cysts were removed from 17 patients who underwent staged thoracotomies. The mean count of cysts was 6.7 (range 3–20 cysts). Most of the cysts (38.2%) were located in the right lower lobe. The mean interval between thoracotomies was 4.2 (range 3–5) days. Two patients (11.7%) had cysts associated with hepatic hydatidosis and one (5.8%) had cysts associated with the spleen; they were treated via phrenotomy during thoracotomies. All cysts were removed without lung resection. We observed some complications such as prolonged air leaks (n?=?2), atelectasis (n?=?3) and empyema (n?=?2). No further surgery was required for management of complications. The mean hospital stay was 9.3 days. (range 7–23 days). Oral albendazole was started on the 2nd post operative day after the first thoracotomy in the dose of 10–20 mg/kg and was continued for 3 months with a gap of 1 week after each 21 days. No recurrences or deaths occured during the follow-up period. Conclusions Although staged thoracotomy applied in 3–5 days after the initial thoracotomy increases the total hospital stay, it decreases the chance of possible complications can occur in cysts in the other lung when long intervals are preferred between the first and the second thoracotomy. In our experience, bilateral staged thoracotomy is an appropriate surgical option because morbidity rates are minimal and the hospital stay is acceptable for the treatment of bilateral widespread lung hydatidosis, even in patients who had a total of 20 hydatid cysts.

2013-01-01

307

Atypical herpes simplex encephalitis presenting as operculum syndrome  

Microsoft Academic Search

This case report demonstrates the course of herpes simplex virus cerebritis in a patient aged 7 years 2 months who presented\\u000a with non-specific symptoms followed by an epileptic attack. Subcortical, bilateral opercular and bilateral thalamic lesions\\u000a were detected, but the temporal and inferior frontal lobes were spared. The patient developed anarthria, impairment of mastication\\u000a and swallowing consistent with operculum syndrome.

Rainer W. Wolf; Detlev Schultze; Christian Fretz; Markus Weissert; Peter Waibel

1999-01-01

308

[Cushing syndrome: Physiopathology, etiology and principles of therapy].  

PubMed

The most frequent cause of Cushing's syndrome is iatrogenic, as Cushing's syndrome is the unavoidable consequence of long-term glucocorticoid treatment using more than 7.5 mg prednisone per day. The most frequent cause of endogenous Cushing's syndrome is Cushing's disease (CD), which is an ACTH dependent hypercortisolism linked to a pituitary corticotroph adenoma. This adenoma is often very small, its diagnosis may require bilateral inferior petrosal sinus sampling and the first line treatment of CD is transsphenoidal surgery by an expert neurosurgeon. The second line treatments include drugs that can act either on the pituitary adenoma or on adrenal steroidogenesis, pituitary radiotherapy or bilateral adrenalectomy. Ectopic ACTH dependent Cushing's syndrome is linked either to poorly differentiated endocrine tumors with a very poor prognosis, such as small cell lung cancer, or to well differentiated endocrine tumors, such as bronchial carcinoid tumors, which have a good prognosis when treated by surgery, but may be very difficult to localize. Adrenal Cushing's syndromes, which are independent of pituitary ACTH secretion, include adrenal cortex carcinoma, which requires abdominal surgery with extended adrenalectomy by an expert surgeon, adrenal adenoma which is treated by laparoscopic unilateral adrenalectomy and bilateral macronodular hyperplasia, whose surgical treatment may require unilateral or bilateral adrenalectomy. Treatment of Cushing's syndrome generally leads to spectacular clinical results, which must not hide the fact that the reversibility of some signs is actually incomplete. This underlines the need for a timely multidisciplinary management of the patients by an expert team. PMID:24656295

Chabre, Olivier

2014-04-01

309

Right and left perisylvian cortex and left inferior frontal cortex mediate sentence-level rhyme detection in spoken language as revealed by sparse fMRI.  

PubMed

In this study, we used functional magnetic resonance imaging to investigate the neural basis of auditory rhyme processing at the sentence level in healthy adults. In an explicit rhyme detection task, participants were required to decide whether the ending syllable of a metrically spoken pseudosentence rhymed or not. Participants performing this task revealed bilateral activation in posterior-superior temporal gyri with a much more extended cluster of activation in the right hemisphere. These findings suggest that the right hemisphere primarily supports suprasegmental tasks, such as the segmentation of speech into syllables; thus, our findings are in line with the "asymmetric sampling in time" model suggested by Poeppel (: Speech Commun 41:245-255). The direct contrast between rhymed and nonrhymed trials revealed a stronger BOLD response for rhymed trials in the frontal operculum and the anterior insula of the left hemisphere. Our results suggest an involvement of these frontal regions not only in articulatory rehearsal processes, but especially in the detection of a matching syllable, as well as in the execution of rhyme judgment. PMID:22711328

Hurschler, Martina A; Liem, Franziskus; Jäncke, Lutz; Meyer, Martin

2013-12-01

310

[Bilateral cerebellar hematoma after supratentorial glioma surgery].  

PubMed

We present a case of bilateral hematoma in cerebellar hemispheres in a 30-year-old man after surgical treatment of extensive left frontal glioma. 16 hours after surgery the patient lost consciousness. An immediate CT revealed hematoma in both cerebellar hemispheres. The hematoma was subsequently removed via bilateral suboccipital craniectomy. After the operation the clinical status of the patient gradually improved - he was discharged in a good general condition. In the presented case the hematoma developed presumably as a consequence of extensive cerebrospinal fluid (CSF) loss (670 ml) via postoperative wound drainage. The resulting cerebellar displacement caused strain of the draining veins, affecting blood outflow, and causing parenchymal hemorrhage. In order to prevent the complication, massive CSF loss during and after operation should be avoided. Careful monitoring of the patient's condition in the postoperative period, even if the general status is good, is important because only an immediate intervention may prevent the development of irreversible consequences of cerebellar hematoma formation. PMID:15354240

Czepko, Ryszard; Kwinta, Borys; Uhl, Henryka; Urbanik, Andrzej; Libionka, Witold; Pietraszko, Wojciech

2004-01-01

311

Amyloidosis in bilateral external auditory canals.  

PubMed

We report a 76-year-old man, a retired farmer, presenting with bilateral external auditory canal obstruction. Skin examination revealed multiple pruritic nodules and periorbital purpura. Ear canal opening surgery was performed. Skin and ear canal biopsy revealed extensive amyloid depositions. Immunoelectrophoresis for urinary Bence-Jones protein was positive, and bone marrow examination showed plasma cell dyscrasia. He received chemotherapy for amyloid light-chain amyloidosis secondary to light-chain multiple myeloma but died from myeloma progression. This case demonstrated how infiltrative conditions such as amyloidosis can lead to bilateral auditory canal obstruction and that the diagnosis of amyloidosis can be missed if a clinician focuses solely on a single organ. PMID:24825551

Hosoi, Takahiro; Dhaliwal, Gurpreet; Tokuda, Yasuharu

2014-01-01

312

[Primary hyperparathyroidism in patients with bilateral nephrolithiasis].  

PubMed

Experience in the diagnosis and treatment of the renal form of primary hyperparathyroidism in 57 patients with bilateral nephrolithiasis was summed up. The main diagnostic criterion was the detection of biochemical changes in the blood and urine (hypercalcemia, hypophosphatemia, hypercalciuria) and the use of some tests (Howard's test and parathyroidin test). Parathyroidectomy was performed after establishing diagnosis. A new stage in therapy of such patients was a study of renal function and phosphocalcium metabolism after parathyroidectomy. The improvement of some indices (an increase in glomerular filtration, urea excretion with urine and relative urine density, and a decrease in hypercalciuria and hyperphosphaturia) indicated the effectiveness of surgical intervention for primary hyperparathyroidism in patients with bilateral nephrolithiasis. It was also confirmed by a decrease in lithogenic relapse after parathyroidectomy. PMID:2587527

Serniak, P S; Guzenko, V N; Berko, E M

1989-01-01

313

Bilateral Macular Hole Following Myopic Photorefractive Keratectomy  

PubMed Central

A 42-year-old man was admitted to our clinic complaining of visual distortion in his left eye two months after bilateral myopic photorefractive keratectomy (PRK). Macular optical coherence tomography (OCT) showed a stage II macular hole in the left eye. Simultaneous OCT in the right eye showed vitreous traction and distortion of the outer retina. One month later, the patient underwent vitrectomy for the left eye, and the macular hole was closed. Two months after that, the patient complained of visual distortion in the right eye, and OCT revealed increased traction and accentuated outer retinal distortion indicating a stage IB macular hole. Traction attenuated later without any intervention. The short interval between PRK and hole formation, bilateral involvement, and the moderate refractive error in this case highlight the possible role of PRK in aggravating vitreoretinal interface abnormalities. We recommend the addition of PRK to the list of procedures that may be associated with the formation of a macular hole.

Shoeibi, Nasser; Abrishami, Majid; Ansari-Astaneh, Mohammad-reza

2014-01-01

314

Bilateral brachial plexus compressive neuropathy (crutch palsy).  

PubMed

Brachial plexus compressive neuropathy following the use of axillary crutches (crutch palsy) is a rare but well-recognized entity. Most reported cases involve the posterior cord of the brachial plexus in children and have resolved spontaneously within 8-12 weeks. We recently treated a 36-year-old man who was using axillary crutches for mobilization after a supracondylar femoral fracture. Bilateral posterior cord (predominantly radial nerve) compressive neuropathy subsequently developed, with lesser involvement of the ulnar and median nerves. The patient had little to no improvement clinically 8 weeks after the estimated onset of the palsy, and an electromyogram at that time confirmed the presence of a severe axonotmesis lesion of the radial, median, and ulnar nerves bilaterally. The patient was treated with static cock-up wrist splinting and discontinuation of the axillary crutches. Return of sensory and motor function was delayed but occurred within 9 months. PMID:9057152

Raikin, S; Froimson, M I

1997-01-01

315

[Bilateral hypoglossal nerve palsy following intubation].  

PubMed

Hypoglossal nerve palsy following intubation is a rare complication that can be reversible depending on the extent of nerve damage. A 63-year-old male with a sigma carcinoma was repeatedly intubated orotracheally due to postoperative complications. After the fourth intubation, bilateral, complete hypoglossal nerve palsy with severe dysarthria and swallowing disability was observed. A percutaneous endoscopic gastrostomy tube was inserted for nutrition and to prevent aspiration. Cerebral MRI showed no pathological findings, particularly in the brainstem. Electromyographic studies revealed pathological spontaneous activity of both glossal muscles without any motor unit potential consistent with an axonal lesion of both hypoglossal nerves. Nevertheless, complete clinical and electromyographical recovery occurred within 7 months. The bilateral hypoglossal nerve palsy in our patient was probably due to mechanical alteration during intubation, leading to axonotmesis. Hypoglossal nerve palsy following intubation might have a favourable prognosis as long as continuity of the nerve sheath is maintained. PMID:16133427

Bramer, S; Koscielny, S; Witte, O W; Terborg, C

2006-02-01

316

Bilateral Breast Masses with a Rare Etiology  

PubMed Central

Breast masses have a variety of benign and malignant etiologies. We present the case of a 28-year-old woman with bilateral large painful breast masses that developed rapidly in the three weeks before first presentation. Further investigation revealed bilateral ovarian masses. Biopsies of both ovarian masses were taken, and the pathology reported Burkitt's lymphoma. Additional staging with a PET scan was suggestive of bone marrow involvement, but bone marrow biopsy was negative. Examination of the cerebrospinal fluid did not identify malignant cells. The patient underwent CODOX-M/IVAC chemotherapy, and a complete response was demonstrated after one cycle of treatment. Six months after finishing chemotherapy the patient remained in complete remission. To our knowledge this is the first case reporting simultaneous involvement of breast, ovaries, and bones in Burkitt's lymphoma. Gynecologists and oncologists should be aware of this pattern. Polychemotherapy treatment must be initiated rapidly with curative intent.

Sartorius, Gideon; Kalf, Katrin; Heinzelmann, Viola

2013-01-01

317

Total ocular akinesis: Miller Fisher or Guillain-Barré syndrome?  

PubMed

Total, bilateral ophthalmoplegia is very rare. More than 50% of cases are Miller Fisher (MFS) and Guillain-Barré (GBS) syndromes. There is a correlation of MFS with anti-GQ1b antibodies. High levels of GQ1b gangliosides are found in myelin sheathes of cranial nerves supplying the extraocular muscles. This may explain the association of anti-GQ1b antibodies with ophthalmoplegia. Anti-GQ1b were also found in cases of GBS accompanied by ophthalmoplegia, atypical MFS (MFS without ataxia), MFS/GBS overlap syndromes and Bickerstaff brainstem encephalitis. This has led some authors to classify them as 'anti-GQ1b syndromes'. In this article we describe a diagnostically difficult case of a patient with a very rare, total bilateral paralysis of all ocular muscles, accompanied by bilateral ptosis, diminished tendon reflexes of upper extremities, paresis and hypoesthesia of the left upper extremity. PMID:21866487

Rajska, Kinga; Ro?niecki, Jacek; Loba, Piotr; Zieli?ska, Ma?gorzata; Broniarczyk-Loba, Anna

2011-01-01

318

Systemic effects of bilateral tibial versus bilateral femoral shaft fractures. Is there a difference?  

PubMed

The authors investigated the prevalence and the difference in the severity of systemic complications following intramedullary nailing of bilateral tibial and femoral shaft fractures. A retrospective chart analysis of 12 consecutive patients with bilateral tibial shaft fractures (TF) and 14 patients with bilateral femoral shaft fractures (FF) was performed. The incidences of bilateral tibial fractures and bilateral femoral shaft fractures were 3.8% and 4.6% respectively. The median Injury Severity Score (ISS) in TF group was 13 (9-29) compared to 16 (9-34) in the FF group (p = 0.169). The mean resuscitation requirements were 4.2 (3-11) litres of colloids and crystalloids and 1.7 (0-10) units of blood in the TF group and 10.6 (6-16) litres of colloids and crystalloids and 9.2 (5-25) units of blood in the FF group (p = 0.002). In the TF group there was 1 death compared to 2 in the FF group. In the TF group, there were 2 cases of ARDS, 4 cases of deep sepsis and 3 above knee amputations. In the FF group, there were 6 cases of ARDS (p = 0.04), 1 case of deep sepsis and 1 above knee amputation. Patients with bilateral tibial shaft fractures revealed lower ISS, resuscitation requirements, ARDS, associated injuries, and mortality when compared to bilateral femoral shaft fractures. This is probably due to the anatomical difference in the morphology of the bones, volume of liberated intravascular marrow fat, organisation and layout of the venous capillary network and severity of associated injuries. PMID:15165015

Raman, Raghu; Sidhom, Sameh; Pape, Hans C; Giannoudis, Peter V

2004-04-01

319

Storage Detrusor Pressure in Bilateral Hydroureteronephrosis  

Microsoft Academic Search

Objectives: To determine the detrusor pressure (Pdet) during the storage phase associated with upper urinary tract (UUT) dilatation.Subjects and Methods: Twenty–seven patients with bilateral hydroureteronephrosis, neither due to organic obstruction nor vesicoureteral reflux, were included. Water cystometry was done according to the recommendations of the International Continence Society [Abrams P., et al.: Scand J Urol Nephrol 1988(suppl 114):5–19]. Pdet at

Ammar G. Ghobish

2001-01-01

320

Fibromuscular Dysplasia Presenting with Bilateral Renal Infarction  

Microsoft Academic Search

Fibromuscular dysplasia (FMD) describes a group of conditions which cause nonatheromatous arterial stenoses, most commonly\\u000a of the renal and carotid arteries, typically in young women. We report a rare case of bilateral segmental renal infarction\\u000a secondary to FMD in a young male patient. His initial presentation with loin pain and pyrexia resulted in a delay in the definitive\\u000a diagnosis of

O. Doody; W. R. Adam; P. T. Foley; S. M. Lyon

2009-01-01

321

Balo's concentric sclerosis involving bilateral thalami.  

PubMed

Balo's concentric sclerosis (BCS) is a rare inflammatory demyelinating disease of central nervous system, pathologically characterized by alternate bands of demyelination and preserved myelin tissue. Before the era of magnetic resonance imaging (MRI), most cases of BCS were diagnosed on postmortem examination. MRI allows for noninvasive diagnosis by demonstrating characteristic changes which closely parallels the histopathological features of BCS. We report a case of 26-year-old female with BCS involving bilateral thalami, with typical MRI appearance. PMID:21891941

Badar, Farheen; Azfar, Shah F; Ahmad, Ibne; Kirmani, Sanna; Rashid, Muddassir

2011-01-01

322

Unilateral headache with bilateral internal ophthalmoplegia.  

PubMed

We describe the case of a young woman suffering from migraine. After some years, the headache started to be accompanied by internal ophthalmoplegia. Mydriasis was unilateral and after a few months became bilateral. The ophthalmoplegic migraine is a rare type of headache and it is characterized by paresis of one or more of the third, fourth or sixth cranial nerves. Migraine attacks associated with mydriasis (internal ophthalmoplegia) are extremely rare. Triptan therapy resolved mydriasis, but not the headache. PMID:22183266

Simonetto, Marco; Zanet, Luca; Capozzoli, Francesca; Gelli, Andrea; Masè, Giovanni

2012-10-01

323

Postpartum anuria caused by silent bilateral nephrolithiasis.  

PubMed

A case of remarkable silent anuria caused by bilateral nephrolithiasis in the post-partum period is reported. The anuria was not associated with any kind of pain and the diagnosis was not revealed until three days postpartum when an intravenous pyelography was carried out. Differential diagnosis and management of nephrolithiasis are discussed. Ultrasound scanning is recommended as a routine procedure for all patients with signs and symptoms of renal dysfunction during pregnancy. PMID:3425236

Jakobson, J; Norman, M; Ulmsten, U; Westgren, M

1987-01-01

324

Frequency and causes of bilateral occular trauma.  

PubMed

Objective: To determine the frequency and causes of bilateral ocular trauma. Design: A descriptive case series. Place and Duration of Study: Khyber Institute of Ophthalmic Medical Sciences, Hayatabad Medical Complex, Peshawar from October 1999 to September 2006. Patients and Methods: All patients coming to the hospital with bilateral eye trauma and requiring admission were recruited into the study. The details of patients' demographics, risk factors, ocular examination, treatment offered and final visual acuity were noted and described as frequency and percentages. Results: Out of a total of 1551 patients of hospitalized ocular trauma, 46 (2.9%, 92 eyes) had bilateral ocular trauma. The majority (54.3%) were due to landmine blast injuries followed by dynamite blast in 10.8%, coalmine blast and firearm injury in 6.5% each. Pressure cooker explosion and road traffic accident was the cause in 4.3% each. Gas cylinder and automobile battery explosion, alkali and acid burn, assault and incidental trauma occurred in 2.1%. Sixty three percent were between 16 and 40 years of age. Males were affected in 93.4%. Corneal and / or scleral repair was done in 58.6%, conjunctival and or corneal foreign body removal in 26% and extracapular cataract extraction with intraocular lens implantation in 16.3%. The visual acuity was in the range of 6/60 and perception of light in 54.3%, while in 21.7%, there was no perception of light at the time of admission. Due to severity of injury, the final visual acuity was poor and only 28.2% regained vision between 6/18 and 6/60. Conclusion: In this series, landmine, dynamite and coalmine blasts were the major causes of bilateral ocular trauma. Victims were usually young males. Due to severity of ocular trauma, majority had poor visual outcome. PMID:18070576

Babar, Tariq Farooq; Khan, Mohammad Naeem; Jan, Sana Ullah; Shah, Shafqat Ali; Zaman, Mir; Khan, Mohammad Daud

2007-11-01

325

Second-Side Surgery in Superior Canal Dehiscence Syndrome  

PubMed Central

Objective Bilateral superior canal (SC) dehiscence syndrome poses a challenge because bilateral SC dehiscence (SCD) plugging might be expected to result in oscillopsia and disability. Our aims were as follows: 1) to evaluate which symptoms prompted patients with bilateral SCD syndrome (SCDS) to seek second-side surgery, and 2) to determine the prevalence of disabling imbalance and oscillopsia after bilateral SC plugging. Study Design Prospective observational study. Setting Tertiary referral center. Patients Five patients with bilateral SCDS based on history, audiometric and physiologic testing, and computed tomographic findings. This includes all of our patients who have had second-side plugging surgery to date. Intervention(s) Bilateral sequential middle fossa craniotomy and plugging of SCs. Main Outcome Measure(s) Cochleovestibular symptoms, cervical and ocular vestibular-evoked myogenic potential testing, dizziness handicap inventory, short-form 36 Health Survey, dynamic visual acuity testing. Results The most common symptoms prompting second-side surgery were sound- and pressure-induced vertigo and autophony. Three of the 5 patients reported that symptoms shifted to the contralateral ear immediately after plugging the first side, whereas in 2 patients, contralateral symptoms developed several years after the first SC plugging. Two of 4 patients experienced ongoing oscillopsia after bilateral SCDS surgery; however, all patients reported relief from their SCD symptoms and were glad that they had pursued bilateral surgery. Conclusion In patients with bilateral SCDS, sound- and pressure-induced vertigo most commonly prompted second-side surgery. Despite some degree of oscillopsia after bilateral SCDS surgery, patients were very satisfied with second-side surgery, given their relief from other SCDS symptoms.

Agrawal, Yuri; Minor, Lloyd B.; Schubert, Michael C.; Janky, Kristen L.; Davalos-Bichara, Marcela; Carey, John P.

2014-01-01

326

Endovascular treatment in two cases of bilateral ischemic stroke.  

PubMed

Acute bilateral intracranial large artery occlusion is a rare occurrence that can lead to bihemispheric ischemia and potentially devastating functional consequences. We discuss two cases that initially presented with unilateral symptoms and did not show signs of bilateral arterial occlusions on the initial MRI imaging. This became evident while performing the angiographic study at the beginning of the endovascular procedure. Both patients were successfully treated with bilateral mechanical thrombectomy as a complement to intravenous thrombolysis. We describe our method of simultaneous sequential bilateral thrombectomy. To our knowledge, this is the first report of successful endovascular therapy in bilateral ischemic stroke. PMID:24091758

Pop, Raoul; Manisor, Monica; Wolff, Valerie; Habashy, Mohamed; Rouyer, Olivier; Kehrli, Pierre; Marescaux, Christian; Beaujeux, Remy

2014-06-01

327

Bilateral limbic system destruction in man  

PubMed Central

We report here a case study of a rare neurological patient with bilateral brain damage encompassing a substantial portion of the so-called “limbic system.” The patient, Roger, has been studied in our laboratory for over 14 years and the current article presents his complete neuroanatomical and neuropsychological profiles. The brain damage occurred in 1980 following an episode of herpes simplex encephalitis. The amount of destroyed neural tissue is extensive and includes bilateral damage to core limbic and paralimbic regions, including the hippocampus, amygdala, parahippocampal gyrus, temporal poles, orbitofrontal cortex, basal forebrain, anterior cingulate cortex, and insular cortex. The right hemisphere is more extensively affected than the left, although the lesions are largely bilateral. Despite the magnitude of his brain damage, Roger has a normal IQ, average to above average attention, working memory, and executive functioning skills, and very good speech and language abilities. In fact, his only obvious presenting deficits are a dense global amnesia and a severe anosmia and ageusia. Roger's case presents a rare opportunity to advance our understanding of the critical functions underlying the human limbic system, and the neuropsychological and neuroanatomical data presented here provide a critical foundation for such investigations.

Feinstein, Justin S.; Rudrauf, David; Khalsa, Sahib S.; Cassell, Martin D.; Bruss, Joel; Grabowski, Thomas J.; Tranel, Daniel

2010-01-01

328

Diagnosis and management of bilateral nasolabial cysts  

PubMed Central

Nasolabial cysts are painless, submucosal, non-odontogenic jaw cysts presenting as soft tissue swellings in the maxillary anterior mucolabial fold lateral to midline, leading to elevation of nasal ala. Present case documents bilateral nasolabial cysts in a 69-year-old Asian female patient. In the present case, extraoral swelling of maxillary lip and elevation of nasal ala was observed on right side of the face. Intraorally, soft and fluctuant bilateral cysts were observed. Straw-colored fluid was aspirated from the right cyst. Radiographically, erosion of bone in a “cupping” fashion was observed in the region of left cyst. The cysts were enucleated using intraoral approach. Histopathology of the right-sided cyst revealed a cystic cavity lined by stratified squamous cells along with a few mucosal cells. At few places, stratified squamous and pseudostratified columnar epithelia with many cilia and goblet cells were also evident. Capsule was loosely arranged with fibrous tissue and chronic inflammatory infiltrate. Left-sided cystic specimen showed two or more layered stratified squamous lining epithelium with thin capsule. Diagnosis of bilateral nasolabial/nasoalveolar cysts was confirmed.

Parwani, Rajkumar; Parwani, Simran; Wanjari, Sangeeta

2013-01-01

329

High Performance, Three-Dimensional Bilateral Filtering  

SciTech Connect

Image smoothing is a fundamental operation in computer vision and image processing. This work has two main thrusts: (1) implementation of a bilateral filter suitable for use in smoothing, or denoising, 3D volumetric data; (2) implementation of the 3D bilateral filter in three different parallelization models, along with parallel performance studies on two modern HPC architectures. Our bilateral filter formulation is based upon the work of Tomasi [11], but extended to 3D for use on volumetric data. Our three parallel implementations use POSIX threads, the Message Passing Interface (MPI), and Unified Parallel C (UPC), a Partitioned Global Address Space (PGAS) language. Our parallel performance studies, which were conducted on a Cray XT4 supercomputer and aquad-socket, quad-core Opteron workstation, show our algorithm to have near-perfect scalability up to 120 processors. Parallel algorithms, such as the one we present here, will have an increasingly important role for use in production visual analysis systems as the underlying computational platforms transition from single- to multi-core architectures in the future.

Bethel, E. Wes

2008-06-05

330

Williams syndrome  

MedlinePLUS

Williams-Beuren syndrome ... Williams syndrome is a rare condition caused by missing a copy of several genes. Parents may not ... history of the condition. However, a person with Williams syndrome has a 50% chance of passing the ...

331

Metabolic Syndrome  

MedlinePLUS

... page from the NHLBI on Twitter. What Is Metabolic Syndrome? Metabolic (met-ah-BOL-ik) syndrome is the ... three metabolic risk factors to be diagnosed with metabolic syndrome. A large waistline. This also is called abdominal ...

332

Usher Syndrome  

MedlinePLUS

... syndrome. Top What are the characteristics of the three types of Usher syndrome? Type 1 Children with ... RP often not apparent until the teens. Type 3 Children with type 3 Usher syndrome have normal ...

333

Unilateral versus bilateral clubfoot: an analysis of severity and correlation.  

PubMed

This study compares the severity of unilateral and bilateral clubfoot, and the correlation between right and left feet of bilateral cases. Sixty-six unilateral and 75 bilateral clubfoot patients were assessed for severity using the Pirani score at an average age of 12.9 days (SD 9 days). In bilateral cases, the severity of right and left feet was highly correlated (r=0.68). The odds of being very severe were 2.6 (95% confidence interval 1.3-5.1) times higher in bilateral cases (P=0.007). Bilateral and unilateral clubfeet present with differing severity. Right and left feet from bilateral cases are highly correlated. Researchers need to address these issues during study design and analysis. PMID:24869905

Gray, Kelly; Barnes, Elizabeth; Gibbons, Paul; Little, David; Burns, Joshua

2014-09-01

334

Branchiootic syndrome--a clinical case report and review of the literature.  

PubMed

Branchiootoic syndrome is part of the spectrum of brachiootorenal disorders. Brachiootorenal disorder is a rare autosomal dominant condition, characterized by malformations of the outer, middle, and inner ear, which are associated with branchial and renal anomalies. We describe a case of bilateral branchiootoic syndrome and discuss the anatomy of second branchial cleft fistulae and the surgical management of this uncommon condition. We report the case of a 6-year-old girl referred to our department with bilateral intermittently discharging neck swellings. Clinical examination revealed bilateral branchial fistulae and preauricular sinuses, on a background of a positive family history of branchial fistulae. A magnetic resonance imaging scan confirmed the diagnosis of bilateral second branchial cleft fistulae. In view of her symptoms, she underwent bilateral branchial fistula excision and tonsillectomy with an uneventful postoperative recovery. PMID:22901925

Amer, Imad; Falzon, Adam; Choudhury, Natasha; Ghufoor, Khalid

2012-08-01

335

Neu-Laxova syndrome: a case report.  

PubMed

Neu-Laxova syndrome (NLS) is a rare lethal syndrome found in both consanguinous and non-consanguinous couple. This is characterized by terrible face with unusual craniofacial appearance with exophthalmos, spectrum of central nervous system malformation, like microcaphaly, hypoplastic cerebellum, cleft lip/palate, ichthyosis and oedema. The diagnosis is made on the basis of clinical parameter. We report a 4 hour old male term newborn with IUGR of Neu-Laxova syndrome presented with anencephaly, rudimentary cerebellum, exophthalmos of right eye, bilateral cleft lip and palate and cryptorchidism. Anencephaly and cryptorchidism are two recently reported findings of NLS. We are presenting this case in addition from Bangladesh to lend further support to those two new findings as component of Neu-Laxova syndrome. Outcome of this syndrome is not good. Most of the patients are died of infection within hours to days. PMID:24584393

Roy, S; Begum, J; Sharifunnaher, B; Saha, A K; Afroza, S; Islam, M Z

2014-01-01

336

Bilateral breast reconstruction using bilateral anterolateral thigh flaps: a case report.  

PubMed

Options for autologous reconstruction have been limited in some patients by previous abdominal surgeries, and by lack of adequate abdominal tissue. The anterolateral thigh (ALT) flap has previously been described as an alternate donor site for autologous breast reconstruction when abdominal tissue is unavailable or unsuitable.We describe our experience with a 41-year-old low body mass index (19.8 kg/m) patient with previous suction-assisted lipectomy underwent bilateral breast reconstruction using bilateral ALT flaps.At a follow-up of 2 years, the patient was delighted with her reconstructed breasts and despite her athletic build was able to fill a B cup bra.ALT flap has the advantages of a long pedicle, adequate soft adipose tissue, and also allowing supine positioning with a 2-team approach. The anterolateral flap is a credible alternative that may be considered for bilateral autologous breast reconstruction in selected patients. PMID:19158519

Bernier, Christina; Ali, Rozina; Rebecca, Alanna; Cheng, Ming-Huei

2009-02-01

337

[Bilateral Pulmonary Artery Banding using Ligation Clips and Facile Norwood-Glenn Procedure].  

PubMed

Bilateral pulmonary artery banding( BPAB), though a less-invasive surgical option for hypoplastic left heart syndrome (HLHS), entails considerable risk of residual pulmonary artery stenosis after de-banding. Autologous aortic reconstruction in Norwood procedure is attractive in terms of growth potential, but technically demanding. To overcome these drawbacks, we modified the 2 techniques. Eightpatients with HLHS underwent BPAB whereby ligation clips were half-closed into rhombic shape to deform bilateral pulmonary arteries. The arterial duct was kept patent by prostaglandin E1 infusion. One patient died of sepsis( age 8 months), while the 7 survivors underwent Norwood-Glenn procedure. Both pulmonary arteries were excised from the pulmonary trunk with minimal cuffs. Resultant defect in the pulmonary trunk was longitudinally closed. After arterial duct excision, pulmonary trunk-todescending aorta continuity was reconstructed by end-to-end anastomosis. Ascending aorta-to-aortic arch complex was anastomosed to the pulmonary trunk in a side-to-side fashion. After bilateral pulmonary artery continuity was reconstructed, Glenn anastomosis was made. One patient died of pneumonia(age 5 months). Currently, the 6 surviving patients(age 4?30 months), enjoy good health. Four of them have completed Fontan procedure. Our modified techniques are facile, reproducible, and pose low risk of residual pulmonary artery stenosis or aortic stenosis. PMID:24917155

Kaneko, Yukihiro; Achiwa, Ikuya; Morishita, Hiroyuki; Shibata, Miyuki

2014-04-01

338

Generalization of visuomotor learning between bilateral and unilateral conditions.  

PubMed

A long history of behavioral and physiological research has suggested that bilateral coordination invokes unique neural processes that are not involved in unilateral movements. This hypothesis predicts that motor learning should show limited transfer between unilateral and bilateral conditions, which is consistent with a recent finding that indicated partial, but not complete, transfer of learning between the two conditions. However, during learning of new motor skills, transformations must also be made between visual and proprioceptive coordinate systems, a process that may occur upstream to the processes that differentiate bilateral from unilateral movements. We now investigate whether visuomotor adaptations are shared between unilateral and bilateral movement conditions. Our results indicate substantial transfer from bilateral to subsequent unilateral conditions for both arms. Interestingly, whereas the nondominant arm never showed complete adaptation to visual rotation under bilateral conditions, this interference, or lack of improvement, in bilateral performance did not disturb the visuomotor adaptation process or transfer, as reflected by superb unilateral performances immediately following the bilateral conditions. These findings unambiguously indicate that visuomotor adaptation can extensively generalize between bilateral and unilateral conditions, thus suggesting a substantial overlap in the neural processes underlying visuomotor transformations between the two movement conditions. Our findings provide support for a two-stage model of motor planning, in which the visuomotor transformation process precedes the processes that convert the visuomotor plan into effector-specific commands that incorporate bilateral synergies and that result in the forces that determine motion. PMID:19759325

Wang, Jinsung; Sainburg, Robert L

2009-11-01

339

A Bilateral Advantage for Storage in Visual Working Memory  

PubMed Central

Various studies have demonstrated enhanced visual processing when information is presented across both visual hemifields rather than in a single hemifield (the bilateral advantage). For example, reported that observers were able to track twice as many moving visual stimuli when the tracked items were presented bilaterally rather than unilaterally, suggesting that independent resources enable tracking in the two visual fields. Motivated by similarities in the apparent capacity and neural substrates that mediate tracking and visual working memory (WM), the present work examined whether or not a bilateral advantage also arises during storage in visual WM. Using a recall procedure to assess working memory for orientation information, we found a reliable bilateral advantage; recall error was smaller with bilateral sample displays than with unilateral displays. To demonstrate that the bilateral advantage influenced storage per se rather than just encoding efficiency, we replicated the observed bilateral advantage using sequentially presented stimuli. Finally, to further characterize how bilateral presentations enhanced storage in working memory, we measured both the number and the resolution of the stored items and found that bilateral presentations lead to an increased probability of storage, rather than enhanced mnemonic resolution. Thus, the bilateral advantage extends beyond the initial selection and encoding of visual information to influence online maintenance in visual working memory.

Umemoto, Akina; Drew, Trafton; Ester, Edward F.; Awh, Edward

2010-01-01

340

Learning about Duane Syndrome  

MedlinePLUS

... Duane syndrome? Duane syndrome (DS) is a rare, congenital (present from birth) eye movement disorder. Most patients ... or DR syndrome), Eye Retraction Syndrome, Retraction Syndrome, Congenital retraction syndrome and Stilling-Turk-Duane Syndrome. In ...

341

Bilateral Wilms' tumors: changing concepts in management  

SciTech Connect

Bilaterality is uncommon in Wilms' tumor, being present in 4% to 8% of the cases. We report the combined experience of two children's hospitals in one city over a 20-year period. We encountered nine cases of synchronous bilateral nephroblastoma (National Wilms' Tumor Study 3, stage V). Age at diagnosis ranged from 9 to 41 months (mean 23 months). There were five girls and four boys. Associated findings include nephroblastomatosis in three cases (33%), one of which also had a familial history; undescended testis in two cases; and minor anomalies in two other cases. Surgical treatment consisted of unilateral nephrectomy with contralateral partial nephrectomy or tumorectomy in six cases, nephrectomy with contralateral biopsy only in two cases, and the other patient had bilateral biopsies initially, followed at a later date by partial nephrectomy on one side. All patients received chemotherapy; actinomycin D (AMD) only was used in the oldest case, vincristine and AMD in five cases, to which was added cyclophosphamide in one case and adriamycin in two. Seven patients received radiation therapy. Seven out of the nine patients survived more than 2 years (77%); five are well, off chemotherapy, with no evidence of disease from 4 to 11 years after diagnosis. Two patients suffered from chronic renal failure and one died from complications after renal transplantation more than 19 years after diagnosis. The two patients who died from their disease presented with more advanced tumor. Therefore, the agressiveness of multimodal therapy can be tailored according to stage and histology, and effective chemotherapy allows maximal preservation of renal parenchyma in patients with stage I and II tumors.

Laberge, J.M.; Nguyen, L.T.; Homsy, Y.L.; Doody, D.P.

1987-08-01

342

Mechanism of highly synchronized bilateral hippocampal activity.  

PubMed

In vivo studies of epileptiform discharges in the hippocampi of rodents have shown that bilateral seizure activity can sometimes be synchronized with very small delays (<2 ms). This observed small time delay of epileptiform activity between the left and right CA3 regions is unexpected given the physiological propagation time across the hemispheres (>6 ms). The goal of this study is to determine the mechanisms of this tight synchronization with in-vitro electrophysiology techniques and computer simulations. The hypothesis of a common source was first eliminated by using an in-vitro preparation containing both hippocampi with a functional ventral hippocampal commissure (VHC) and no other tissue. Next, the hypothesis that a noisy baseline could mask the underlying synchronous activity between the two hemispheres was ruled out by low noise in-vivo recordings and computer simulation of the noisy environment. Then we built a novel bilateral CA3 model to test the hypothesis that the phenomenon of very small left-to-right propagation delay of seizure activity is a product of epileptic cell network dynamics. We found that the commissural tract connectivity could decrease the delay between seizure events recorded from two sides while the activity propagated longitudinally along the CA3 layer thereby yielding delays much smaller than the propagation time between the two sides. The modeling results indicate that both recurrent and feedforward inhibition were required for shortening the bilateral propagation delay and depended critically on the length of the commissural fiber tract as well as the number of cells involved in seizure generation. These combined modeling/experimental studies indicate that it is possible to explain near perfect synchronization between the two hemispheres by taking into account the structure of the hippocampal network. PMID:24262205

Wang, Y; Toprani, S; Tang, Y; Vrabec, T; Durand, D M

2014-01-01

343

Bilateral symmetric stress fractures in a toddler.  

PubMed

Stress fractures are a common injury among adolescent athletes and military recruits. The increase in child participation in organized sport activities has contributed to the inclusion of the skeletally immature age group among those who may suffer from this problem. Bilateral simultaneous symmetric tibial stress fractures that are infrequent in older children are even more rare in toddlers. This entity may cause a diagnostic problem as it must be differentiated from infectious disease, acute trauma or even from the result of a battered child. PMID:11269816

Keret, D; Segev, E; Hayek, S; Lokiec, F; Wientroub, S

2001-01-01

344

Bilateral Ossiculoplasty in 1 Case of Achondroplasia  

PubMed Central

Achondroplasia is the most common skeletal dysplasia and it combines various complications with normal longevity. Hearing disturbance due to otitis media or an ossicular anomaly is one of the most common complications. Conductive hearing loss is suggested as the most common form of hearing loss. Temporal bone and middle ear structures are distorted in achondroplasia because of rotational change of the skull base. Authors experienced a case of an achondroplastic patient with bilateral hearing disturbance. We faced making a potential mistake during the previous operation but a favorable postoperative result occurred. Our experience could be helpful to other clinicians who face achondroplastic patients.

Jung, Jongyoon; Yang, Chulwon; Lee, Sunkyu

2013-01-01

345

Bilateral TMJ Involvement in Rheumatoid Arthritis  

PubMed Central

Rheumatoid arthritis (RA) is a systemic inflammatory, slowly progressive disease that results in cartilage and bone destruction. Temporomandibular joint (TMJ) involvement is not uncommon in RA, and it is present in about more than 50% of patients; however, TMJ is usually among the last joints to be involved and is associated with many varied clinical signs and symptoms. Hence, RA of TMJ presents to the dentist with great diagnostic challenges. This report presents a case of RA with bilateral TMJ involvement with its classical radiographic findings and review literature.

Ruparelia, Pritesh B.; Shah, Deep S.; Ruparelia, Kosha; Sutaria, Shreyansh P.; Pathak, Deep

2014-01-01

346

Prophylactic bilateral mastectomy and contralateral prophylactic mastectomy.  

PubMed

With increasing public awareness of the risk for breast cancer and modern techniques of reconstruction, the option of surgical prophylaxis for risk reduction is becoming increasingly popular. Bilateral prophylactic mastectomy for women at increased risk of developing breast cancer and contralateral prophylactic mastectomy for those with unilateral breast cancer seeking symmetry, risk reduction, and ease of follow-up are acceptable options for many women. However, prophylactic surgery is not an inconsequential decision, and careful consideration should be given to the risks and benefits of such procedures. PMID:24882342

Chagpar, Anees B

2014-07-01

347

Bilateral retroesophageal course of the carotid arteries.  

PubMed

Many variations in the anatomy of the carotid artery are described in the literature. There are few case reports of hypoplasia or the retropharyngeal course of the internal carotid artery. We describe the unique case of a 72-year-old woman with bilateral complete retroesophageal common carotid arteries and concomitant hypoplasia of the right carotid system. Anatomical detection of such a variation is critical because the misidentification of a retroesophageal carotid artery carries the risk of potential injury during orotracheal intubation and oropharyngeal procedures. PMID:23601592

Virvilis, Dimitrios; Koullias, George; Labropoulos, Nicos

2013-05-01

348

Bilateral ossiculoplasty in 1 case of achondroplasia.  

PubMed

Achondroplasia is the most common skeletal dysplasia and it combines various complications with normal longevity. Hearing disturbance due to otitis media or an ossicular anomaly is one of the most common complications. Conductive hearing loss is suggested as the most common form of hearing loss. Temporal bone and middle ear structures are distorted in achondroplasia because of rotational change of the skull base. Authors experienced a case of an achondroplastic patient with bilateral hearing disturbance. We faced making a potential mistake during the previous operation but a favorable postoperative result occurred. Our experience could be helpful to other clinicians who face achondroplastic patients. PMID:24653923

Jung, Jongyoon; Yang, Chulwon; Lee, Sunkyu; Choi, June

2013-12-01

349

Bilateral Hydronephrosis and Cystitis Resulting from  

PubMed Central

Ketamine associated urinary dysfunction has become increasingly more common worldwide. Point-of-care ultrasound (POCUS) is an established modality for diagnosing hydronephrosis in the emergency department. We describe a case of a young male ketamine abuser with severe urinary urgency and frequency in which POCUS performed by the emergency physician demonstrated bilateral hydronephrosis and a focally thickened irregular shaped bladder. Emergency physicians should consider using POCUS evaluate for hydronephrosis and bladder changes in ketamine abusers with lower urinary tract symptoms. The mainstay of treatment is discontinuing ketamine abuse.

Abuse, Chronic Ketamine; Tran, Vu Huy; Nelson, Mathew; Nogar, Joshua; Bramante, Robert M.

2014-01-01

350

Bilateral recurrent self-induced keratoconus.  

PubMed

The observational case describes bilateral recurrent keratoconus in corneal transplants performed in a patient with self-induced keratoconus secondary to compulsive eye rubbing. Slitlamp findings demonstrated corneal stromal thinning and scarring in the patient's right eye and temporal corneal hydrops in his left eye. Videokeratography of the right eye confirmed the presence of corneal steepening and irregular astigmatism, consistent with the diagnosis of keratoconus involving each transplant. Together with the history of ongoing compulsive eye rubbing, these findings support the concept that chronic mechanical trauma to the cornea may contribute to the development of keratoconus. PMID:18997547

Koenig, Steven B

2008-11-01

351

Progressive bilateral thinning of the parietal bones  

SciTech Connect

Observation of a case of progressive bilateral parietal thinning within a period of 14 years induced us to study skull films of 3 636 consecutive patients. Parietal thinning was found in 86 patients (2.37%). It was more common in women, with a sex ratio of 1:1.9. The mean age of the females was 72 years, and that of the males 63 years. Previous skull films of 25 of these patients were available and showed progression in 10. It is concluded that parietal thinning is a slowly progressive disease of middle-aged and old patients and is not an anatomical variant or congenital dysplasia of the dipole.

Cederlund, C.G.; Andren, L.; Olivecrona, H.

1982-03-01

352

Metabolic Syndrome  

MedlinePLUS

... you know? One in five Americans has the metabolic syndrome. WhO is At risk fOr the metAbOlic syndrOme? The syndrome runs in families and is more ... High blood pressure Most people who have the metabolic syndrome feel healthy and may not have any signs ...

353

Fregoli syndrome.  

PubMed

Fregoli syndrome is the delusional belief that one or more familiar persons, usually persecutors following the patient, repeatedly change their appearance. This syndrome has often been discussed as a variant of the Capgras syndrome in the literature, but these two syndromes have different phenomenological structures and age and sex distributions. The author presents a review of 34 cases of Fregoli syndrome in the English and French language literature, discussing the syndrome's definition, aetiology and course. It is suggested that although an organic substrate may be found in some cases, it is the dominant psychotic theme which determines the content of the syndrome. PMID:7893241

Mojtabai, R

1994-09-01

354

Frontal syndrome as a consequence of lesions in the pedunculopontine tegmental nucleus: A short theoretical review  

Microsoft Academic Search

In this review, it is argued that the consequence of bilateral damage to the pedunculopontine tegmental nucleus (PPTg) in experimental animals is the production of a form of frontal syndrome. Frontal syndrome is a term used to describe the behavioural consequences of damage to the frontal lobes in human patients. These behavioural changes can be classified as disinhibition of behaviour

Philip Winn

1998-01-01

355

Urgent Bilateral Endoscopic Marsupialization for Respiratory Distress due to Bilateral Dacryocystitis in a Newborn  

PubMed Central

Abstract We describe an infant with respiratory distress due to bilateral dacryocystoceles and dacryocystitis who was successfully treated with urgent bilateral endoscopic marsupialization. A male infant was brought to our outpatient department 7 days after birth, with red, acutely inflamed swellings near the medial canthal area of both eyes. From birth, there had been bluish swelling near the medial canthal area, and redness and swellings developed within 3 days. On physical examination, the child was afebrile but showed respiratory distress with coarse breathing sound. That day, the infant was admitted and treated with intravenous cefotaxime 150 mg. After withholding oral intake for appropriate preoperative fasting, urgent bilateral probing with endoscopy was done. On endoscopy, huge bilateral congenital dacryocystoceles were found. Because of its huge size, the inferior surface of the cyst was touching the nasal floor, which made probe unable to perforate the wall of dacryocystocele. Therefore, an endoscopy-assisted marsupialization of dacryocystoceles and bicanalicular silicone intubation were performed. Both swellings and erythema subsided within 48 hours postoperatively, and the patient was discharged after 72 hours from treatment.

Kim, Hochang; Park, Jongyeop; Jang, Jaeho; Chun, Junwoo

2014-01-01

356

[Bilateral ankylosing paraarticular ossifications of the hip joint with involvement of the sciatic nerve].  

PubMed

In December 1997 a 48 year-old man was treated by resection of the bilateral paraarticular ankylosing ossifications that developed after 6 weeks of unconsciousness after necrotic pancreatitis. The ossifications on the posterior part of the hip joint involved a highly separated sciatic nerve. There was a bilateral osseous ankylosis of the hip joint and total denervation of sciatic nerve. In April 1999 the ossifications on the right side were removed. The splitted sciatic nerve was in two osseous channels. The head of the femur was vital and immediately following the operation, flexion of the hip joint was possible to 90 degrees. The same procedure was performed on the left hip joint in October 1999. In April 2000, a re-occurance of the paraarticular ossification on the ventral part of the right hip joint was removed from an ilioinquinal approach. In June 2002 the patient was able to stand and walk without crutches. ROM of the knee joints improved bilaterally to 120 degrees of flexion. The flexion of the right hip joint is 70 degrees and in the left hip is 80 degrees. 3 years after decompression of both sciatic nerves involved in heterotopic bone established significant reinervation. The motor function of the proximal femoral muscles and the function of the tibial nerve was renewed. Complete motor denervation syndrom persists in common peroneal nerve. Sensitive inervation was renewed as in n. tibialis as in common peroneal nerve except acral parts of the foot a toes. Concerning function of the sciatic nerves, the overall results can be assessed as partially succesfull because of partial denervation syndrome of sciatic nerves. In definite clinical improvement the recovery of the neurologic status is very important besides the mechanical improvement of range of motion of the hip joint. PMID:15600127

St?drý, V; Zv?rina, E; Chomiak, J

2004-01-01

357

[Cushing syndrome in children].  

PubMed

In pediatric patients, endogenous Cushing syndrome is an infrequent condition almost always due to one of two conditions. 1) Adrenal gland tumors account for 70% of Cushing syndromes in young pediatric patients. They cause rapidly progressive hypercorticism not due to increased ACTH production (elevated plasma and urine cortisol levels, very low ACTH and LPH levels unchanged by dexamethasone, metyrapone or CRH). Imaging techniques determine the side and spread of the tumor and look for metastases. Following surgical removal, patients with indicators of malignant disease (tumor weight above 30 g, extracapsular spread or metastases, independently from pathological data) are given op'DDD. 2) Cushing disease occurs in peripubertal patients and causes overweight with delayed statural gain. ACTH production is increased (positive dexamethasone suppression test and provocative metopirone and CRH tests) as a result of a pituitary adenoma which should be looked for by magnetic resonance imaging and whose removal ensures recovery in 50% of cases. Other therapeutic tools include op'DDD, radiation to the pituitary, and bilateral adrenalectomy as the last resort given the high risk of post-adrenalectomy pituitary tumor (50% of pediatric patients). Other causes are exceedingly rare: primary nodular hyperplasia of the adrenal glands and production of ACTH by a nonpituitary tumor. Corticosteroid treatment is the most common cause of Cushing syndrome in children. PMID:8239397

Raux-Demay, M C; Girard, F

1993-09-01

358

Bilateral bifid mandibular condyle: a case report.  

PubMed

Bilateral bifid mandibular condyle is a rarely seen malformation. The aetiology of bifid condyle is not completely understood, although developmental anomaly, traumas, condylar fracture, teratogenic embryopathy and surgical condylectomy may all be causative factors. Although a few studies on human dried skulls tried to shed light on this entity it remains obscure. As most bifid condyle subjects have no complaint related to temporamandibular joint(TMJ), the cases are generally diagnosed through incidental radiographic findings. The case of a 54-year-old female is presented. In a panoramic radiograph obtained after a clinical examination, bilateral bifid mandibular condyle was observed. The open-closed lateral radiograph of the TMJ (obtained using the TMJ-specific program of the panoramic device) demonstrated duplication of the right and left condyles. In order to better evaluate the TMJ morphology and to eliminate pathologies such as fractures that might be missed with conventional radiographs, a computed tomography scan was also obtained. The joint head orientation was observed in the mediolateral direction. The case is discussed in the context of the relevant literature. Until large population-based studies are undertaken and further experimental studies are performed, bifid condyle will remain an incidental finding of anatomic variation rather than a clinically informative observation. PMID:16938108

Açikgöz, A

2006-10-01

359

Renal tubular defects in recurring bilateral nephrolithiasis.  

PubMed

A metabolic study was performed in 28 patients with bilateral recurrent calcium-containing renal staghorn calculi and chronic pyelonephritis ("obstructive nephropathy"). Fourteen had normal GFR and 14 mild renal insufficiency. Ten normal subjects were used as controls. Under basal conditions, polyuria and negative sodium balance were commonly observed in patients with obstructive nephropathy and normal renal function. After an acute acid load (NH4Cl) an acidifying defect, i.e. high values for urine pH and reduced excretion of titratable acid and ammonium, was observed in 64% of patients with normal GFR and in 71.4% of those with renal insufficiency. During intravenous infusion with neutral sodium phosphate, the urine pH changed little but the rate of excretion of titratable acid increased in direct proportion to that of urinary phosphate in both groups of patients. These results, associated with the finding of normal blood pH in almost all patients, lead to the conclusion that an incomplete Type 1 or "distal" renal tubular acidosis is a frequent complication of obstructive nephropathy secondary to bilateral nephrolithiasis. The anatomical abnormalities of renal tubules and collecting ducts and the superimposed interstitial nephritis might be the pathogenetic factors responsible for the acidifying defect and for the impairment in sodium and water conservation. PMID:954242

D'Angelo, A; Pagano, F; Ossi, E; Lupo, A; Valvo, E; Messa, P; Tessitore, N; Maschio, G

1976-08-01

360

Bilateral tension pneumothorax following equipment improvisation.  

PubMed

This case report describes an unexpected event that took place as a result of using improvised equipment. The patient, a 16-year-old female undergoing complex oral surgery, suffered bilateral pneumothorax following the improper use of an airway support device. During the immediate postoperative period with the patient still intubated, oxygen tubing was attached to a right angle elbow connector with the port closed and 10 L/minute oxygen flow was administered to the patient in a manner that did not allow the patient to exhale. Within seconds, pneumothorax was apparent as the patient's vital signs deteriorated, visible swelling was noted in the shoulders and neck, and there was an absence of breath sounds on auscultation. This case study has application beyond the immediate discussion of bilateral pneumothorax, serving as a caution about the unintended consequences of equipment improvisation. In addition to highlighting the hazards of providing patient care with a non-standard device, this study also provides a powerful example of the human factors that can contribute to medical errors in the healthcare setting. PMID:24654348

Zambricki, Christine; Schmidt, Carol; Vos, Karen

2014-02-01

361

Noninvasive testing of asymptomatic bilateral hilar adenopathy.  

PubMed

The diagnostic strategy for asymptomatic patients with persistent bilateral bilar adenopathy often involves invasive procedures. The authors used Bayesian analysis to: 1) estimate the relative prevalences of diseases causing bilateral bilar adenopathy; 2) assess changes in the prevalence of disease by race, the presence of other clinical symptoms, and geography; and 3) determine the value of relevant noninvasive tests, including the angiotensin-converting enzyme (ACE) assay, gallium scan, and purified protein derivative (PPD), in order to assess when a strategy of watchful waiting is appropriate. The analysis indicated that the ACE assay, particularly when paired with the PPD, can identify many patients who might safely be managed without immediate invasive biopsy. Patients who are ACE+ and PPD- have an estimated probability of sarcoidosis of 0.95 or greater; patients who are ACE- and PPD+ have a probability of tuberculosis of 0.86 if black, 0.79 if white. In contrast, gallium scanning has no diagnostic role in this clinical situation. Bronchoscopic or mediastinoscopic biopsy has a limited role for patients who are ACE+ PPD- or ACE- PPD+ because of limited sensitivity. Patients who are both ACE- and PPD-, particularly if white, may have a high enough risk of lymphoma to consider invasive biopsy. PMID:2156032

Carr, P L; Singer, D E; Goldenheim, P; Bernardo, J; Mulley, A G

1990-01-01

362

Anaesthetic consideration during laparoscopic bilateral simultaneous nephrectomy  

PubMed Central

STUDY OBJECTIVE: To assess outcome from anaesthesia during laparoscopic bilateral simultaneous nephrectomy. DESIGN: Retrospective study. MEASUREMENTS: Preoperative Hb%, serum potassium, coagulation profile electrocardiography (ECG) changes, 2D Echography, x-ray chest, haemodynamic changes, end-tidal carbon dioxide (EtCO2), fluid management and postoperative analgesia. RESULTS: The mean age was 24.75 ± 14.35 years. The mean duration of surgery was 120 ± 80 minutes. The Hb%, serum creatinine and serum potassium were 9.4 ± 1.04%, 6.79 ± 4.91 meq/L and 3.61 ± 0.51 meq/L, respectively. Pulse rate mean blood pressure and EtCO2 were recorded after creation of pneumoperitoneum and at 15, 30, 45 and after exsufflation of pneumoperitoneum. After pneumoperitoneum, there was increase in pulse rate, systolic blood pressure, diastolic blood pressure and EtCO2. After 30 minutes and throughout the surgery, these variables remained stable. Four patients required nitroglycerine infusion for intraoperative hypertention. Only one patient required packed cell volume (PCV) transfusion and total intravenous fluid was 1 ± 0.5 L. At the time of exsufflation, there was decrease in pulse rate, systolic and diastolic blood pressure and EtCO2. CONCLUSION: Because of advancement in anaesthetic agents and muscle relaxant, there is safe outcome from anaesthesia during laparoscopic bilateral simultaneous nephrectomy.

Modi, Manisha P; Vora, Kalpana S; Parikh, Geeta P; Modi, Pranjal R; Shah, Veena R

2014-01-01

363

A case of Gerstmann-Sträussler-Scheinker syndrome.  

PubMed

Gerstmann-Sträussler-Scheinker syndrome (GSS syndrome) is a rare hereditary disorder caused by prion protein gene mutation. We present the case of a 31-year-old man, whose signs and symptoms gradually progressed from loss of attention while driving at onset to headache, dysarthria, night sweat, fatigue, and dysgraphia. Diffusion-weighted imaging (DWI) of the brain after admission showed high signal intensities in the bilateral caudate nuclei, bilateral thalami, and cerebral cortices that suggested transmissible spongiform encephalopathy. The patient was diagnosed with GSS syndrome on genetic study. Magnetic resonance (MR) imaging of the entire period of sickbed showed gradually changing signal intensities and cerebral atrophy. We present a series of images and discuss the reasons for the abnormal intensities in GSS syndrome that vary among reported cases. PMID:17510542

Irisawa, Momoko; Amanuma, Makoto; Kozawa, Eito; Kimura, Fumiko; Araki, Nobuo

2007-01-01

364

Pediatric bilateral large concurrent thalamic glioblastoma: An unusual case report  

PubMed Central

Bilateral thalamic tumors are rare. Pediatric bilateral thalamic glioblastomas are even rarer, only five cases reported in the English literature till date. The clinical presentation, natural history, and prognosis of pediatric thalamic tumors are still relatively obscure. In this article, we report an 8-year-old patient with large bilateral thalamic glioblastomas and briefly discuss its clinical presentation, possible modalities of management, and prognosis, in the light of available literature.

Pandey, Nityanand; Singh, Pankaj Kumar; Mahapatra, Ashok K.; Kakkar, Aanchal; Sharma, Bhawani Shankar

2014-01-01

365

Bilateral synchronous squamous cell tonsil carcinoma treated with chemoradiotherapy.  

PubMed

The incidence of numerous head and neck tumours is a known issue though bilateral synchronous tonsillar carcinoma reports are so uncommon that only 20 cases were found in a literature review. Most of these patients were treated with bilateral tonsillectomy followed by adjuvant radiotherapy. We report, to our knowledge, the first case of bilateral synchronous tonsillar squamous cell carcinoma treated only with chemoradiotherapy without tonsillectomy. PMID:24864648

Bakkal, Bekir Hakan; Ugur, Mehmet Birol; Bahadir, Burak

2014-04-01

366

Pediatric bilateral large concurrent thalamic glioblastoma: An unusual case report.  

PubMed

Bilateral thalamic tumors are rare. Pediatric bilateral thalamic glioblastomas are even rarer, only five cases reported in the English literature till date. The clinical presentation, natural history, and prognosis of pediatric thalamic tumors are still relatively obscure. In this article, we report an 8-year-old patient with large bilateral thalamic glioblastomas and briefly discuss its clinical presentation, possible modalities of management, and prognosis, in the light of available literature. PMID:24891914

Pandey, Nityanand; Singh, Pankaj Kumar; Mahapatra, Ashok K; Kakkar, Aanchal; Sharma, Bhawani Shankar

2014-01-01

367

Bilateral thalamic infarctions due to occlusion of artery of Percheron.  

PubMed

The artery of Percheron is an uncommon anatomic variant in which a solitary trunk originates from either posterior cerebral artery and provides arterial supply to bilateral paramedian thalami and the rostral midbrain (Fig. 1). Recognition of this anatomical variant is essential in establishing the stroke mechanism in bilateral paramedian thalamic infarcts. We describe the clinical presentation and neuroimaging characteristics in a patient with bilateral thalamic infarctions due to occlusion of the artery of Percheron. PMID:20417939

Teoh, Hock Luen; Ahmad, Aftab; Yeo, Leonard L L; Hsu, Eugene; Chan, Bernard P L; Sharma, Vijay K

2010-06-15

368

Bilateral pneumothoraces complicating reduction mammoplasty: a case report  

PubMed Central

Background Bilateral pneumothoraces after cosmetic breast surgery are rare and sporadically reported in the literature. Case presentation A 65-year-old female patient developed bilateral pneumothoraces after bilateral breast reduction surgery. Emergent chest tube thoracostomy was performed on both sides. The chest drains were removed on the fourth day (left side) and sixth day (right side), and the patient was discharged after 7 days of hospitalization without any further complications. Conclusion To our knowledge, the English-language literature contains no other reports of bilateral pneumothoraces after reduction mammoplasty.

2013-01-01

369

Exceptional bilateral adrenalectomy after secondaries from colorectal cancer.  

PubMed

We report a very uncommon case of bilateral adrenal metastasis treated at our institution. The patient was 65 years old, with a history of low anterior resection for colorectal cancer in 2001. One year later, he was diagnosed with bilateral adrenal metastasis, based on the results of abdominal computed tomography-scan. A bilateral adrenalectomy extended to distal pancreatectomy and splenectomy was performed. Postoperative course was uneventful. He is alive and free of disease 12 months after adrenalectomy. We conclude adrenal metastasis from colorectal cancer should be managed surgically, even if they are bilateral. PMID:15011840

Moreno-Elola, A; Moreno-Gonzalez, E; Alonso-Casado, O; Meneu-Diaz, J C; García-García, I; Abradelo-Usera, M

2004-01-01

370

"Mirror-image" bilateral giants: intracavernous carotid artery aneurysms.  

PubMed

Summary: The literature on the incidence of "mirror image" bilateral giant intracavernous aneurysms, their symptoms and their association with other entities is reviewed, with a brief comment on their evolution and treatment. A case of "mirror image" bilateral giant intracavernous aneurysms in a 76-year-old man who presented a sudden diplopia with pupillary sparing is reported. A CT scan showed parasellar images and dolichomega circle of Willis arteries that enhanced with endovenous contrast. MRI and angiography disclose bilateral aneurysms in detail, associated with an anomalous origin of the left common carotid artery and bilateral renal artery stenosis. PMID:20569579

Díaz, M B; Mercado, F C; Lemme Plaghos, L A

2006-09-15

371

"Mirror-Image" Bilateral Giants: Intracavernous Carotid Artery Aneurysms  

PubMed Central

Summary The literature on the incidence of "mirror image" bilateral giant intracavernous aneurysms, their symptoms and their association with other entities is reviewed, with a brief comment on their evolution and treatment. A case of "mirror image" bilateral giant intracavernous aneurysms in a 76-year-old man who presented a sudden diplopia with pupillary sparing is reported. A CT scan showed parasellar images and dolichomega circle of Willis arteries that enhanced with endovenous contrast. MRI and angiography disclose bilateral aneurysms in detail, associated with an anomalous origin of the left common carotid artery and bilateral renal artery stenosis.

Diaz, M.B.; Mercado, F.C.; Lemme Plaghos, L.A.

2006-01-01

372

Kearns-Sayre syndrome: An unusual ophthalmic presentation.  

PubMed

Kearns-Sayre syndrome (KSS) belongs to the group of neuromuscular disorders known as mitochondrial encephalomyopathies. It has characteristic syndromal features, which include: chronic progressive external ophthalmoplegia, bilateral atypical pigmentary retinopathy, and cardiac conduction abnormalities. So far, only a single case has been reported where a patient with KSS had a normal retina. Herein, we report this extremely rare variant of KSS, which not only presented later than the normal age of presentation, but also had minimal pigmentary retinopathy. PMID:22993469

Ahmad, Syed S; Ghani, Shuaibah A

2012-05-01

373

Interocular symmetry analysis of bilateral eyes.  

PubMed

Abstract The purpose of this study was to evaluate the interocular symmetry of several biometric parameters between both eyes. The symmetry between the right and left eye of 397 subjects in 14 biometric parameters, spherical equivalent of refractive error (SE), Jackson crossed cylinder power of refractive error astigmatism with axes at 90° and 180° (RJ0) and at 45° and 135° (RJ45), best-corrected visual acuity (BCVA), average corneal curvature (CC), Jackson crossed cylinder power of corneal astigmatism (CJ0 and CJ45), corneal asphericity coefficient (Q), intraocular pressure (IOP), central corneal thickness (CCT), axial length (AL), anterior chamber depth (ACD), lens thickness (LT) and vitreous chamber depth (VCD), was assessed by comparative data analysis. Aside from RJ0 (p?=?0.00), RJ45 (p?=?0.02) and Q (p?=?0.00), the overall interocular differences of other biometric parameters between fellow eyes were not significant (p?>?0.05). The interocular correlation and Bland-Altman plots showed a good agreement between fellow eyes in 14 biometric parameters. Correlations between interocular differences in SE and that in RJ0 (p?=?0.03), CC (p?=?0.00), AL (p?=?0.00) and VCD (p?=?0.00) were statistically significant. There were similar strong linear relationships between refractive error astigmatism vectors and corneal astigmatism vectors in bilateral eyes. There were negative correlations of RJ45 and CJ45 between bilateral eyes. A potentially clinically important interocular symmetry in SE, BCVA, CC, CJ0, CJ45, IOP, CCT, AL, ACD, LT and VCD is found in this research, while the differences of RJ0, RJ45 and Q between left and right eyes seem a bit large. The negative interocular relationships of RJ45 and CJ45 demonstrate moderate mirror symmetry exists among fellow eyes. High interocular symmetry in bilateral eyes may be helpful in intraocular lens power calculation, intraocular pressure evaluation, post-operative visual acuity and refraction prediction at the time the fellow eye is undergoing refractive surgery. PMID:24734876

Li, Yi; Bao, Fang Jun

2014-05-01

374

Bilateral peroneal nerve palsy after simultaneous bilateral total knee arthroplasty. Report of a case with rheumatoid arthritis.  

PubMed

In this study, we report on a 46-year-old female patient with bilateral severe gonarthrosis due to rheumatoid arthritis. Simultaneous bilateral cemented total knee arthroplasty was performed. Bilateral common peroneal nerve palsy was observed on the second postoperative day. The electromyographic diagnosis was "bilateral axonotmesis". Complete clinical and electromyographic motor recoveries were seen on both sides within 6 months postoperatively. The sensorial deficit was still present on one side at 2 years postoperatively. Preoperative severe flexion contracture and epidural anesthesia were assumed as the risk factors for the development of the nerve palsy in this patient. PMID:11522079

Omeroglu, H; Ozçelik, A; Turgut, A

2001-07-01

375

Cat eye syndrome.  

PubMed

A full-term female baby, a product of non-consanguineous marriage, was born at 37?weeks of gestation with a birth weight of 2.08?kg. Antenatal scan at 31?weeks revealed complex congenital heart disease with a hypoplastic right ventricle, pulmonary atresia and an intact septum. Immediately after birth, the infant was shifted to the nursery and was started on intravenous fluids and infusion prostaglandin E1 (Alprostidil). On examination, she had microcephaly, periorbital puffiness, a long philtrum, a broad nasal bridge and retrognathia, up slanting palpebral fissures, widely spaced nipples, a sacral dimple and right upper limb postaxial polydactyly. Postnatal echocardiography confirmed a large ostium secundum atrial septal defect with left to right shunt, right ventricle hypoplasia, pulmonary atresia with an intact septum and a large vertical patent ductus arteriosus. Ophthalmological examination showed a bilateral chorioretinal coloboma sparing disc and fovea. Karyotyping showed an extra small marker chromosome suggestive of the Cat eye syndrome. PMID:24842361

Sharma, Deepak; Murki, Srinivas; Pratap, Tejo; Vasikarla, Madhavi

2014-01-01

376

A case of bilateral presumed chikungunya neuroretinitis  

PubMed Central

Chikungunya fever is a relatively rare from of vector-borne viral fever caused by chikungunya virus and spread by bites of the Aedes aegypti and Aedes albopictus mosquito. Epidemics of chikungunya fever have been reported in the past from different parts of the world. Although the virus had been passive for quite some time, recent reports of outbreaks of chikungunya fever in several parts of Southern India have confirmed the re-emergence of this virus. Symptoms of this infection include abrupt onset of fever, chills, and headache, rash, severe joint pain, conjunctival injection and photophobia. Ocular manifestations have been recently reported with this infection. We report a case of a 48-year-old female patient, who presented with defective vision two weeks after a serology proven chikungunya infection. There was bilateral neuroretinitis with peripapillary cotton wool spots. These findings should be kept in mind as an ocular manifestation of chikungunya virus infection.

G, Mahesh; Giridhar, A; Shedbele, Archis; Kumar, Ram; Saikumar, S J

2009-01-01

377

Bilateral pneumothorax during subdural-peritoneal shunting.  

PubMed

Pneumothorax is a very rare complication of ventriculoperitoneal shunting in children. We report a case of an iatrogenic bilateral tension pneumothorax during the placement of a subdural-peritoneal shunting. After the placement of peritoneal catheter, oxygen saturation of the patient quickly decreased, hypotension and bradycardia occurred. Intraoperative x-rays showed the pneumothorax. A thoracostomy tube was inserted and attached to an underwater seal. Vital signs improved in a short time period. The radiological improvement had been achieved in four days. Early diagnosis and prompt intervention are life-saving for this complication. To avoid this complication, the tip of the shunt tunneler should be always palpable during the placement of the peritoneal catheter, especially in children's shunt surgery. PMID:21845583

Solmaz, Ilker; Tehli, Ozkan; Kaya, Serdar; Erdogan, Ersin; Izci, Yusuf

2011-01-01

378

Cerebral ischaemic stroke and bilateral pheochromocytoma  

PubMed Central

We report a case of a 10-year-old girl who presented with an acute neurological deficit preceded by a 2 month history of headaches. The patient was treated for acute viral encephalitis because of the presence of inflammation. A computed tomography (CT) scan of her brain scan showed a cerebral ischaemic stroke, and paroxysmal arterial hypertension led us to suspect pheochromocytoma. The diagnosis was confirmed by elevated urinary catecholamine and by imaging (abdominal x-ray and abdominal/pelvic CT scan). Bilateral adrenal gland tumours were successfully removed. This report illustrates an uncommon presentation of pheochromocytoma in children and shows the importance of a careful investigation for pheochromocytoma as a cause of hypertension in children with cerebral ischaemic stroke.

Abourazzak, Sana; Atmani, Samir; Arqam, Larbi El; Chaouki, Sanae; Labib, Smail; Harrandou, Moustapha; Tizniti, Siham; Bouabdellah, Youssef; Bouharrou, Abdelhak; Hida, Moustapha

2010-01-01

379

Central masking with bilateral cochlear implants  

PubMed Central

Across bilateral cochlear implants, contralateral threshold shift has been investigated as a function of electrode difference between the masking and probe electrodes. For contralateral electric masking, maximum threshold elevations occurred when the position of the masker and probe electrode was approximately place-matched across ears. The amount of masking diminished with increasing masker-probe electrode separation. Place-dependent masking occurred in both sequentially implanted ears, and was not affected by the masker intensity or the time delay from the masker onset. When compared to previous contralateral masking results in normal hearing, the similarities between place-dependent central masking patterns suggest comparable mechanisms of overlapping excitation in the central auditory nervous system.

Lin, Payton; Lu, Thomas; Zeng, Fan-Gang

2013-01-01

380

Bilateral xanthine nephrolithiasis in a dog.  

PubMed

Xanthinuria is an uncommon metabolic disorder clinically manifested as urolithiasis. There are two forms of the disease, congenital and iatrogenic. The former was diagnosed in a four-year-old dachshund bitch. The patient was presented with signs of terminal chronic renal failure. Urine was bacteriologically sterile with massive amorphous crystalluria. Bilateral nephrolithiasis was diagnosed at necropsy. The kidneys were shrunken with marked atrophy of the renal cortex and medulla. Infrared spectrometry revealed that the stones comprised 100 per cent xanthine. Later, two of the patient's siblings were examined for urine xanthine content; both showed higher concentrations of xanthine than a control dog. The described case is compared with seven known published cases of congenital xanthinuria. PMID:9239633

Kucera, J; Bulková, T; Rychlá, R; Jahn, P

1997-07-01

381

[Bilateral fracture of the pisiform bone].  

PubMed

Fracture of the pisiform bone is exceptional compared with the other carpal bones. Such lesions may be missed in the emergency room because of the complex anatomy of the carpal region. The usual circumstances leading to fracture of the pisiform bone include fall with reception on the hyperextended wrist, traffic accidents, and blunt trauma. The purpose of this work was to present a case of bilateral fracture of the pisiform bones. A 34-year-old woman presented with pain along the ulnar borders of both wrists after a fall. The mechanism of fracture was direct trauma on both hands after reception in radial inclination. The diagnosis was established late and conservative treatment was applied. After simple immobilization, the clinical and functional outcome was excellent. The anatomic position of the pisiform bone exposes it to direct trauma. Late diagnosis can favor osteoarthritic sequelae involving the pisotriquetral joint. PMID:18166960

Ozalp, T; Kurt, C; Coskunol, E; Ozdemir, O; Bégué, T

2007-12-01

382

Computed tomography findings in bilateral perinephric lymphangiomatosis  

PubMed Central

Perinephric lymphangioma is rare disorder that may be confused with various forms of renal cystic diseases and urinomas. In this disorder a developmental malformation results in failure of developing lymphatic tissue to establish normal communication with the rest of lymphatic system. Once there is restricted drainage of lymphatic fluid the lymphatic channels dilate to form cystic masses that may be unilocular or multilocular and may be seen unilaterally or bilaterally .This condition presents with various signs and symptoms or can be just an incidental finding which in presence of misleading clinical history may be confused with other diseases. CT scan with delayed cuts and USG guided aspiration with biochemical analysis of fluid will help us in arriving to final diagnosis.

Hakeem, Aijaz; Gojwari, Tariq A; Reyaz, Sheikh; Rasool, Shubana; Shafi, Hakim; Mufti, Shahida

2010-01-01

383

An unusual case of bilateral granulomatous mastitis.  

PubMed

Idiopathic granulomatous mastitis (IGM) is an uncommon benign disorder of the breast. At clinical examination, IGM is characterized by an inflammatory process of the breast, usually unilateral. Possible clinical findings are palpable mass with erythematous skin, pain, sterile abscesses, fistula and nipple retraction. Mammography and ultrasound findings are not specific for IGM. Magnetic resonance imaging (MRI) is a useful tool for the differential diagnosis; it is also necessary to delineate the exact extension of the disease and to plan the correct treatment. Final diagnosis is histological. We described an unusual case of IGM with bilateral involvement in a patient with history of pacemaker implantation and IGM typical clinical symptoms. Mammography, ultrasound, and MRI examinations were performed to identify the inflammatory disorder and to plan the correct therapy. Imaging features were correlated with final histological diagnosis of IGM. PMID:23781373

Pistolese, C A; Di Trapano, R; Girardi, V; Costanzo, E; Di Poce, I; Simonetti, G

2013-01-01

384

An Unusual Case of Bilateral Granulomatous Mastitis  

PubMed Central

Idiopathic granulomatous mastitis (IGM) is an uncommon benign disorder of the breast. At clinical examination, IGM is characterized by an inflammatory process of the breast, usually unilateral. Possible clinical findings are palpable mass with erythematous skin, pain, sterile abscesses, fistula and nipple retraction. Mammography and ultrasound findings are not specific for IGM. Magnetic resonance imaging (MRI) is a useful tool for the differential diagnosis; it is also necessary to delineate the exact extension of the disease and to plan the correct treatment. Final diagnosis is histological. We described an unusual case of IGM with bilateral involvement in a patient with history of pacemaker implantation and IGM typical clinical symptoms. Mammography, ultrasound, and MRI examinations were performed to identify the inflammatory disorder and to plan the correct therapy. Imaging features were correlated with final histological diagnosis of IGM.

Pistolese, C. A.; Di Trapano, R.; Girardi, V.; Costanzo, E.; Di Poce, I.; Simonetti, G.

2013-01-01

385

A very rare bilateral adrenal tumor.  

PubMed

We report a case of very rare adrenal tumor. A 54-year-old patient was classified as affected by bilateral adrenal incidentaloma that surprisingly, on histology resulted solitary fibrous tumors. Solitary fibrous tumor (SFT) is an uncommon mesenchymal neoplasm. Only five cases of localization of SFT in adrenal gland are reported in the literature, while the frequency of retroperitoneum localization is more frequent, about 30 cases. Immunohistochemically, SFT can be positive for CD34 antigen, vimentin, CD99, and bcl-2 and usually negative for cytokeratins, chromogranin A, NSE, neurofilaments, synoptophysin, and S-100. Surgical excision remains the main treatment in fact the recurrence is locoregional and correlated with positive margins due to incomplete excision, while distant metastases are correlated with atypical or malignant features. PMID:24146413

Toniato, Antonio; Boschin, Isabella Merante; Pelizzo, Maria Rosa

2014-04-01

386

Bilateral stony lung: pulmonary alveolar microlithiasis  

PubMed Central

A 40-year-old male paddy field worker was referred for exertional shortness of breath and non-productive cough for 4 years. He had been treated for pulmonary tuberculosis twice. Chest radiograph showed extensive bilateral nodular opacities (“sandstorm-like”) in the middle and lower lobe. Pulmonary function tests revealed a restrictive ventilatory defect. High resolution CT showed widespread nodular infiltration with “crazy paving” appearance and interrupted black pleura sign. This was confirmed as pulmonary alveolar microlithiasis (PAM) by trans-bronchial lung biopsy, which showed normal respiratory lining epithelium with dilated alveolar spaces containing many calcific bodies, some of which showed concentric calcification. The possibilities of silicosis (due to exposure to husk) and tuberculosis, both of which can mimic PAM clinically and radiologically, made this case a diagnostic challenge.

Chandra, Subhash; Mohan, Anant; Guleria, Randeep; Das, Prasenjit; Sarkar, Chitra

2009-01-01

387

Tuberculosis of bilateral first costotransverse joints.  

PubMed

Involvement of posterior spine constitutes to 1-6% of the cases of spinal tuberculosis (TB). To the best of our knowledge, TB of costotransverse joints has only been reported once in the literature. The purpose of this study is to describe a case of TB arthritis of costotransverse joints. A 22-year-old immunocompetent patient presented with a 3-month history of pain in the neck and upper back along with a swelling on the left side of the base of the neck. On performing an MRI of the cervicodorsal spine, there was evidence of destructive collections at the bilateral first and right-sided second costotransverse joints. The patient was diagnosed as a case of tubercular pathology and was managed successfully with antitubercular chemotherapy. PMID:23907973

Gupta, Mayank; Jain, Vijay Kumar; Naik, Ananta Kumar; Arya, Rajendera Kumar

2013-01-01

388

Subacute bilateral visual loss in methylmalonic acidemia.  

PubMed

A 23-year-old woman known to have methylmalonic acidemia (MMA) since birth suffered bilateral visual loss within 5 days. Multiple sclerosis, Leber hereditary optic neuropathy, vasculitis, infections (in particular treponema), and vitamin deficiency were ruled out. The optic nerve head changed from normal in appearance to atrophic. Treatment attempts with high-dose intravenous steroids and coenzyme Q10 combined with vitamin E were ineffective. The patient's underlying disease was metabolically well controlled by strict diet and carnitine supplementation. Toxic damage of both optic nerves due to MMA is the most likely mechanism. MRI showed moderate enhancement of both optic nerves. To our knowledge, this is the first report of a morphological correlate on MRI. PMID:21873889

Traber, Ghislaine; Baumgartner, Matthias R; Schwarz, Urs; Pangalu, Athina; Donath, Marc Y; Landau, Klara

2011-12-01

389

Outcome study of bilateral lateral rectus recession for intermittent exotropia in children.  

PubMed Central

PURPOSE: This paper reports an outcome study of 52 consecutive children treated by bilateral lateral rectus recession for intermittent exotropia over a 9 year period (1981-90) with a minimum follow up of 6 months. METHODS: Successful alignment was defined as the absence of any postoperative intermittent or constant tropia in any position of gaze. The study examined the variables that might be predictive of successful alignment. The charts were abstracted for age at initial surgery, quantity of initial deviation, initial refraction, motor alignment at 1 week and 6 months, final alignment, secondary surgery results and the incidence of a monofixation syndrome result. RESULTS: Motor tests demonstrated that 32 (62%) of the patients were successfully aligned by the initial procedure performed for a mean of 25 prism diopters of preoperative deviation while viewing distant targets at a mean age of 4 years 8 months, followed for a mean of 4 years, 4 months. The incidence of undercorrections and overcorrections were approximately equal in quantity suggesting that the current surgical dosage was adequate, but the age at initial surgery, initial deviation, initial refraction and 1 week postoperative alignment results were not predictive of success. Alignment at 6 months, however, was highly correlated with successful alignment by the end of the study (p = 0.002). Secondary surgery was performed for 11 patients and 5 patients were found to have a monofixation syndrome result. CONCLUSION: Successful alignment was achieved in the majority of children treated for intermittent exotropia by an initial bilateral lateral rectus recession. However, 20% of the patients received secondary surgery for a residual deviation, and the study confirmed a previously reported 10% incidence of monofixation syndrome result in children surgically treated for this type of strabismus.

Ing, M R; Nishimura, J; Okino, L

1997-01-01

390

Mohr syndrome in two sisters: prenatal diagnosis in a 22-week-old fetus with post-mortem findings in both.  

PubMed

Oral-facial digital syndrome type II (OFP syndrome II; orofaciodigital syndrome II) is a rare autosomal recessive syndrome, first described by Mohr (1941). We present two sisters with Mohr syndrome from a consanguineous family. One is a three-day-old female patient, the other is 22-week-old fetus. Polydactyly with bifid thumbs in both hands, bilateral polysyndactyly of halluces, lateral polysyndactyly and bilateral pes equinovarus were demonstrated in the fetus sonographically. Corpus callosum agenesis, congenital heart disease, bilateral bifid thumbs and halluces and polydactyly were seen in both patients. In addition, post-mortem findings showed absence of olfactory nerve, single atrium. VSD, abnormal lung lobulation and natal teeth in the fetus. Absence of olfactory nerve and natal teeth have not been reported previously in Mohr syndrome. PMID:10521839

Balci, S; Güler, G; Kale, G; Söylemezo?lu, F; Besim, A

1999-09-01

391

Unilateral coronal craniosynostosis and Down syndrome.  

PubMed

There is no known correlation between Down syndrome and craniosynostosis. The authors report 2 infants with trisomy 21 and right unilateral coronal craniosynostosis. Both patients were clinically asymptomatic but displayed characteristic craniofacial features associated with each disorder. One patient underwent a bilateral fronto-orbital advancement and the other underwent an endoscopically assisted strip craniectomy with postoperative helmet therapy. Both patients demonstrated good cosmesis at follow-up. PMID:24635134

Siu, Alan; Rogers, Gary F; Myseros, John S; Khalsa, Siri S; Keating, Robert F; Magge, Suresh N

2014-05-01

392

Jugular Foraminal Stenosis in Crouzon Syndrome  

Microsoft Academic Search

We describe a 4-year-old boy with Crouzon syndrome, with associated acanthosis nigricans and Chiari-I malformation, who developed increased intracranial pressure necessitating posterior cranial expansion. Postoperatively, an arteriovenous fistula appeared over the mastoid region. Cerebral angiography demonstrated bilateral atresia of the jugular veins and occlusion of the left sigmoid and right transverse sinuses. We propose that increased intracranial venous pressure, secondary

Dolores Martinez-Perez; Douglas L. Vander Woude; Patrick D. Barnes; Michael Scott; John B. Mulliken

1996-01-01

393

Miliary sarcoidosis with secondary Sjogren's syndrome.  

PubMed

We report a case of miliary sarcoidosis with secondary Sjogren's in a 45-year-old male who presented with symptoms of sicca syndrome in the form of dryness of eyes and mouth with parotid swelling. Computed tomography thorax showed mediastinal and hilar lymphadenopathy, bilateral miliary opacities in lung parenchyma. Whole body FDG PET/CT showed involvement of both parotids, liver, diffuse uptake in lungs, mediastinal and retroperitoneal lymph nodes. Patient is on treatment with prednisolone and has responded well. PMID:24772762

Kumar, Puneet; Jaco, M J; Pandit, A G; Shanmughanandan, K; Jain, Anurag; Rajeev; Ravina, M

2013-07-01

394

Ocular findings in arteriohepatic dysplasia (Alagille's syndrome).  

PubMed

The ocular findings in a father and two offspring with arteriohepatic dysplasia are reported in this paper. All three people had bilateral posterior embryotoxon, Axenfeld's anomaly and a pigmentary retinopathy. Other ocular findings were exotropia, an ectopic pupil, band keratopathy, choroidal folds, anomalous optic discs and infantile myopia. Persons with arteriohepatic dysplasia typically present with prolonged neonatal jaundice. Cognizance of the associated eye findings helps distinguish this syndrome from other types of familial intrahepatic cholestasis. PMID:6796244

Romanchuk, K G; Judisch, G F; LaBrecque, D R

1981-04-01

395

A case of prune belly syndrome.  

PubMed

The prune belly syndrome was first described in 1839 by Frolich. Till now about 300 cases have been reported in literature. The complete form of the syndrome is presented by a classic triad that is seen only in boys and is incompatible with life. The etiology of the syndrome is uncertain. Genetic factors, intrauterine infections, adverse mechanical factors have been implicated. The prenatal diagnosis relies on the ultrasonographic findings of oligohydramnion, renal anomalies, and non-immune fetal hydropsy. The present case concerns a newborn of male sex suffering from the most severe and rare form of the syndrome. The child died 5 minutes after his birth. The pathological examination found the patient devoid of striated muscles anterior abdominal wall, urethral atresia, hyperthrophy of the urinary bladder, bilateral hydroureter and hydronephrosis and coinciding cryptorchidism. The case indicates the PBS as one of the possible reasons for non-immune fetal hydropsy. PMID:10979181

Peshev, Z V; Krusteva, M B; Danev, V H

2000-01-01

396

[XX 'pure' gonadal dysgenesis and XYY syndrome].  

PubMed

XX 'pure' gonadal dysgenesis is a disease related to Turner's syndrome. Patients of this disease are characterized by normal female external genitalia, bilateral streak gonads, amenorrhea and sexual infantilism. Recently, it has been reported that point mutations of the FSH receptor gene may be one of cause of this disease. The relationship between criminal behavior and XYY syndrome is still controversial. Increased incidence of disomic sperm in 47,XYY males has been reported by fluorescent in situ hybridization(FISH). Genetic counseling should be done when they undergo intracytoplasmic sperm injection. PMID:14968536

Itoh, Naoki; Tsukamoto, Taiji

2004-02-01

397

Paroxysmal nocturnal hemoglobinuria presenting as moyamoya syndrome.  

PubMed

We report an 11-year-old girl who has paroxysmal nocturnal hemoglobinuria (PNH) and was admitted because of recurrent cerebrovascular accidents (CVA) and intermittent hemoglobinuria. Internal carotid angiography revealed bilateral typical moyamoya patterns. Although CVA due to arterial thrombosis may occur in PNH, the basal moyamoya vessels were never mentioned in case reports yet. The moyamoya syndrome has been reported in a variety of diseases and represents the nonspecific response to an impairment of arterial flow at specific sites in the brain. Our case discloses that PNH may present as moyamoya syndrome. PMID:8733912

Lin, H C; Chen, R L; Wang, P J

1996-01-01

398

[Fahr syndrome discovered following a bacterial meningitis].  

PubMed

Fahr's disease refers to a rare syndrome characterized by symmetrical and bilateral intracranial calcifications. The basal ganglia and dentate nucleus are the most common site of involvement and most cases present extrapyramidal symptoms. This disease is mostly associated with a phosphocalcic metabolism disorder, especially to hypoparathyroidism. The authors report a case of Fahr syndrome (FS) discovered when a young patient with hypocalcemia and bacterial meningitis had a cerebral CT scan disclosing intracerebral calcifications. She fully recovered after both meningitis and hypocalcemia were treated. PMID:18329142

Sbai, H; Smail, L; Hamdani, S; Essatara, Y; Harrandou, M; Khatouf, M; Kanjaa, N

2008-05-01

399

Management of IRVAN Syndrome With Observation.  

PubMed

A 7-year-old girl with IRVAN (idiopathic retinal vasculitis, aneurysms, and neuroretinitis) syndrome was monitored for 9 years. The patient had symmetric multiple aneurysmal dilations, retinal vasculitis, and severe hard exudation in the macula bilaterally. Long-term visual acuity in her untreated right eye was 20/25. In the left eye, treated with laser panretinal photocoagulation and intravitreal vascular endothelial growth factor inhibitor therapy, visual acuity decreased to 20/200 from macular hard exudates and central fibrovascular tissue. Observation is a consideration in IRVAN syndrome if the vision remains good with hard exudation. [Ophthalmic Surg Lasers Imaging Retina. 2014;45:e18-e22.]. PMID:24766157

Basha, Madhi; Brown, Gary C; Palombaro, Geremie; Shields, Carol L; Shields, Jerry A

2014-01-01

400

The Deficiency of PIP 2 5Phosphatase in Lowe Syndrome Affects Actin Polymerization  

Microsoft Academic Search

Lowe syndrome is a rare X-linked disorder characterized by bilateral congenital cataracts, renal Fanconi syndrome, and mental retardation. Lowe syndrome results from mutations in the OCRL1 gene, which encodes a phospha- tidylinositol 4,5 bisphosphate 5-phosphatase located in the trans-Golgi network. As a first step in identifying the link between ocrl1 deficiency and the clinical disorder, we have identified a reproducible

Sharon F. Suchy; Robert L. Nussbaum

2002-01-01

401

Johanson-Blizzard syndrome: A challenge in nasal reconstruction  

PubMed Central

Johanson-Blizzard syndrome is rare and involves multiple congenital anomalies, including bilateral absence or deficiency of the nasal alae, giving the affected infant a characteristic appearance. Two cases are described that illustrate the difficulties of nasal reconstruction in young children. Surgical options and the timing of intervention are discussed.

Timoney, Norma; Weinberg, Michael J; Ross, David A; Thomson, Hugh G

2004-01-01

402

Dance Therapy with Physical Therapy for Children with Down Syndrome.  

ERIC Educational Resources Information Center

This study sought to investigate effects of a dance program on bilateral toe-standing balance and single-point static balance skills of a group of children with Down Syndrome. Thirteen experimental and 10 control group students between the ages of 3 and 13 years were assessed on toe-standing balance and single-point standing balance on the right…

Dupont, Blanche Burt; Schulmann, Diana

403

An unusual ocular presentation of acquired immune deficiency syndrome  

PubMed Central

A 50-year-old male who presented with bilateral keratomalacia and on subsequent evaluation was found to be human immunodeficiency virus (HIV) positive is being reported. A MEDLINE search of the literature did not reveal any report of keratomalacia as the initial presenting feature of HIV/ acquired immune deficiency syndrome.

Arunachalam, Cynthia; Hegde, Vidya; D?Souza, Nameeth

2008-01-01

404

Vocal cord paralysis in the Shy-Drager syndrome  

Microsoft Academic Search

Eight out of 12 unselected patients with Shy-Drager syndrome were found to have severe bilateral paresis of vocal cord abduction by fibre-optic laryngoscopy. This commonly presented as increased snoring followed by episodes of inspiratory and expiratory stridor and sometimes by sleep apnoea. Respiratory failure eventually developed in four cases and was reversed by tracheostomy. In another patient tracheostomy relieved severe

A Williams; D Hanson; D B Calne

1979-01-01

405

Bilateral hemorrhagic pleural effusion due to kerosene aspiration  

PubMed Central

Kerosene ingested, intentionally or accidentally, is toxic. Data is scarce on complications and outcomes of hydrocarbon poisoning following kerosene aspiration in adults and there has been no known case of bilateral hemorrhagic effusion occurring due to it in literature. We, hereby, report a case of a bilateral hemorrhagic pleural effusion secondary to hydrocarbon aspiration in a 40-year old adult.

Prasad, Rajendra; Karmakar, Saurabh; Sodhi, Rakhee; Karmakar, Shilpi

2011-01-01

406

Bilateral breast carcinomas: Do they have similar mammographic features?  

Microsoft Academic Search

AIMS: This study compared the mammographic appearance, site and histological features of synchronous and metachronous bilateral breast carcinomas.METHODS: Site, type of abnormality and background pattern seen on the diagnostic mammograms of 63 women with bilateral breast carcinoma were compared. The histological type and grade of all the carcinomas were verified by histological review.RESULTS: There was a tendency for the contralateral

N. J Hall; A. J Evans; J Kollias; H Denley; S. E Pinder; I. O Ellis; R. W Blamey; A. R. M Wilson

1999-01-01

407

Parkinsonism associated with autosomal dominant bilateral striopallidodentate calcinosis  

Microsoft Academic Search

Bilateral striopallidodentate calcinosis (BSPDC, also known as Fahr's disease, a misnomer), is a rare disorder where bilateral, almost symmetric, calcium and other mineral deposits occur in subcortical nuclei and white matter. Neurological manifestations vary but movement disorders are the most common. Of the movement disorders, parkinsonism predominates. We describe 6 patients with BSPDC associated with parkinsonism. Of the 6 patients,

B. V. Manyam; A. S. Walters; I. A. Keller; M. Ghobrial

2001-01-01

408

Bilateral thalamic infarction and DSA demonstrated AOP after thrombosis.  

PubMed

Bilateral paramedian thalamic stroke is a special ischemic pattern that results from occlusion of the artery of Percheron (AOP), a rare anatomic variant of the paramedian arteries. We report a case of bilateral thalamic infarctions, with a dramatic improvement after thrombolysis. DSA demonstrated recanalization of AOP with possible unreported variation. PMID:23986825

Cao, Wenjie; Dong, Qiang; Li, Linxin; Dong, Yi

2012-01-01

409

Bilateral thalamic infarction and DSA demonstrated AOP after thrombosis  

PubMed Central

Bilateral paramedian thalamic stroke is a special ischemic pattern that results from occlusion of the artery of Percheron (AOP), a rare anatomic variant of the paramedian arteries. We report a case of bilateral thalamic infarctions, with a dramatic improvement after thrombolysis. DSA demonstrated recanalization of AOP with possible unreported variation.

Cao, Wenjie; Dong, Qiang; Li, Linxin; Dong, Yi

2012-01-01

410

Meningeal carcinomatosis causing isolated bilateral symmetric progressive hearing loss  

Microsoft Academic Search

A 53-year-old man presented with isolated bilateral progressive hearing loss from about 5 months. Fourteen months after onset of this isolated symptom a diagnosis of meningeal carcinomatosis (MC) was made. Isolated bilateral deafness is a rare first manifestation of MC.

S. Testoni; A. Pirodda; M. Pastore Trossello; E. Minguzzi; R. D’Alessandro

2005-01-01

411

Prenatal bilateral extravaginal testicular torsion—a case presentation  

Microsoft Academic Search

A newborn male presented at birth with findings consistent with bilateral testicular torsion. Preoperative ultrasound demonstrated no flow to either testicle, and he underwent surgery, during which bilateral extravaginal testicular torsion was confirmed. The right testicle was grossly necrotic and orchidectomy was performed, whereas the left testicle was indeterminate and underwent detorsion and orchidopexy. At 6-month follow-up, the left testicle

Mathew D. Sorensen; Amanda M. Striegl; Martin A. Koyle

2004-01-01

412

Modern tenets for repair of bilateral cleft lip.  

PubMed

The understanding of the bilateral cleft lip and associated nasal deformity has evolved over the last 30 years to a point where there now exists general agreement regarding the goals, principles, and strategies for operative repair. This article presents modern tenets for repair of bilateral cleft lip and describes a logical approach to correction of the different possible subtypes. PMID:24607187

Allori, Alexander C; Marcus, Jeffrey R

2014-04-01

413

Androgen insensitivity syndrome.  

PubMed

The androgen insensitivity syndromes (AIS) fall within the generic category of 46,XY DSD (disorder of sex development) and present as phenotypes associated with complete or partial resistance to the action of androgens. Three categories are recognized: complete androgen insensitivity syndrome (CAIS), partial androgen insensitivity syndrome (PAIS), mild androgen insensitivity syndrome (MAIS). The androgen receptor (AR) is encoded by an 8 exon gene on the X chromosome long arm. More than 800 mutations in the AR gene have been reported in AIS patients (www.androgendb.mcgill.ca/). They are distributed throughout the gene with a preponderance located in the ligand binding domain. The most severe mutations are generally associated with a CAIS phenotype, but the correlation is less defined in PAIS. CAIS presents typically as primary amenorrhoea in an adolescent female and less commonly in infancy with bilateral inguinal/labial swellings due to testes. The differential diagnosis in CAIS is limited, whereas in PAIS, numerous other causes of DSD can also produce the typical phenotype of micropenis, severe hypospadias and bifid scrotum. Management issues in CAIS involve timing of gonadectomy, appropriate hormone replacement therapy and assessment of the need for vaginal dilation or rarely, vaginal surgery. The risk of gonadal germ cell tumor is low during childhood and adolescence but increases in later adulthood. Expert psychological counseling is mandatory to manage the disconnect between chromosomal, gonadal and phenotypic sex and to choreograph the evolving process of disclosure from late childhood through to maturity. It is implicit that management in AIS requires a multidisciplinary team and engagement with patient advocacy groups. PMID:23044881

Hughes, Ieuan Arwel; Werner, Ralf; Bunch, Trevor; Hiort, Olaf

2012-10-01

414

Goldenhar and cri-du-chat syndromes: a contiguous gene deletion syndrome?  

PubMed

We report a full-term male infant born to nonconsanguinous parents who had clinical features of Goldenhar syndrome and cri du chat syndrome. At birth, the infant was noted to have dysmorphic features with bilateral preauricular tags, rotated ears, bilateral epicanthic folds, a left epibulbar lipodermoid, and an accessory left nipple. After he was assessed for feeding difficulty and tachypnea, he was found to have esophageal atresia with tracheoesophageal fistula. In addition, he had a high-pitched, cat-like cry, characteristic of cri-du-chat syndrome. He also failed a hearing test. Chromosomal analysis and fluorescence in situ hybridisation studies showed an unbalanced karyotype with a terminal deletion of the segment p14 on the short arm of chromosome 5, which is consistent with the cri-du-chat locus. The association of Goldenhar syndrome and cri-du-chat syndrome in this patient suggests that the chromosome 5p14 locus may harbor a gene implicated with Goldenhar syndrome. PMID:12825068

Choong, Yee Fong; Watts, Patrick; Little, Elizabeth; Beck, Lyn

2003-06-01

415

Learning about Down Syndrome  

MedlinePLUS

... genetic terms used on this page Learning About Down Syndrome What is Down syndrome? What are the ... syndrome Additional Resources for Down Syndrome What is Down syndrome? Down syndrome is a chromosomal condition related ...

416

Moebius Syndrome  

MedlinePLUS

... disorders. NIH Patient Recruitment for Moebius Syndrome Clinical Trials At NIH Clinical Center Throughout the U.S. and Worldwide NINDS Clinical Trials Organizations Column1 Column2 Moebius Syndrome Foundation P.O. ...

417

Trichorhinophalangeal syndrome.  

PubMed

Trichorhinophalangeal syndrome type 1 is an autosomal dominant variety of peripheral dysostosis. I have reviewed nine cases of this syndrome and have described two of these cases. I have also presented an extensive review of the literature. PMID:1925732

Burgess, R C

1991-10-01

418

Hunter syndrome  

MedlinePLUS

... form: Mild to no mental deficiency Both forms: Carpal tunnel syndrome Coarse features of the face Deafness (gets ... Airway obstruction Carpal tunnel syndrome Hearing loss that gets ... of ability to complete daily living activities Joint stiffness ...

419

Metabolic syndrome  

MedlinePLUS

Metabolic syndrome is a name for a group of risk factors that occur together and increase the risk ... Metabolic syndrome is becoming more and more common in the United States. Researchers are not sure whether the ...

420

Compartment syndrome  

MedlinePLUS

Compartment syndrome is a serious condition that involves increased pressure in a muscle compartment. It can lead to ... need to be amputated. Swelling that leads to compartment syndrome occurs from trauma such as a car accident ...

421

Dravet Syndrome  

MedlinePLUS

... Syndrome Information Page Synonym(s): Severe Myoclonic Epilepsy of Infancy (SMEI) Table of Contents (click to jump to ... Dravet syndrome, also called severe myoclonic epilepsy of infancy (SMEI), is a severe form of epilepsy. It ...

422

Bilateral thoracic ganglion cyst : a rare case report.  

PubMed

Ganglion cysts usually arise from the tissues around the facet joints. It is usually associated with degenerative cahanges in facet joints. Bilateral thoracic ganglion cysts are very rare and there is no previous case that located in bilateral intervertebral foramen compressing the L1 nerve root associated with severe radiculopathy. We report a 53 years old woman who presented with bilateral groin pain and severe numbness. Magnetic resonance imaging revealed bilateral cystic mass in the intervertebral foramen between 12th thoracal and 1st lumbar vertebrae. The cystic lesions were removed after bilateral exposure of Th12-L1 foramens. The result of hystopathology confirmed the diagnosis as ganglion cyst. The ganglion cyst may compromise lumbar dorsal ganglion when it located in the intervertebral foramen. The surgeon should keep this rare entity in their mind for differential diagnosis. PMID:23908708

Kazanci, Burak; Tehli, Ozkan; Türkoglu, Erhan; Guclu, Bulent

2013-05-01

423

A New Image Denoising Framework Based on Bilateral Filter  

PubMed Central

The bilateral filter is a nonlinear filter that does spatial averaging without smoothing edges; it has shown to be an effective image denoising technique in addition to some other applications. There are two main contributions of this paper. First, we provide an empirical study of the optimal parameter selection for the bilateral filter in image denoising applications. Second, we present an extension of the bilateral filter: multi-resolution bilateral filter, where bilateral filtering is applied to low-frequency subbands of a signal decomposed using an orthogonal wavelet transform. Combined with wavelet thresholding, this new image denoising framework turns out to be very effective in eliminating noise in real noisy images. We provide experimental results with both simulated data and real data.

Zhang, Ming; Gunturk, Bahadir K.

2010-01-01

424

Bilateral Thoracic Ganglion Cyst : A Rare Case Report  

PubMed Central

Ganglion cysts usually arise from the tissues around the facet joints. It is usually associated with degenerative cahanges in facet joints. Bilateral thoracic ganglion cysts are very rare and there is no previous case that located in bilateral intervertebral foramen compressing the L1 nerve root associated with severe radiculopathy. We report a 53 years old woman who presented with bilateral groin pain and severe numbness. Magnetic resonance imaging revealed bilateral cystic mass in the intervertebral foramen between 12th thoracal and 1st lumbar vertebrae. The cystic lesions were removed after bilateral exposure of Th12-L1 foramens. The result of hystopathology confirmed the diagnosis as ganglion cyst. The ganglion cyst may compromise lumbar dorsal ganglion when it located in the intervertebral foramen. The surgeon should keep this rare entity in their mind for differential diagnosis.

Kazanci, Burak; Tehli, Ozkan; Guclu, Bulent

2013-01-01

425

Cervical diastematomyelia in cervico-oculo-acoustic (Wildervanck) syndrome: MRI findings.  

PubMed

Cervico-oculo-acoustic (COA) or Wildervanck syndrome is characterized by the triad of Klippel-Feil anomaly, bilateral abducens palsy with retracted bulbs (Duane 'syndrome') and hearing loss. The clinical findings of this syndrome have been well documented. A few case reports with MRI findings have appeared in the literature showing brainstem and cerebellar hypoplasia and vertebral segmentation anomalies. Our case is unique in that diastematomyelia of the lower medulla and cervical cord was accompanied by vermian hypoplasia, tonsillar herniation and resulting triventricular hydrocephalus in a child with Wildervanck syndrome. This case is presented with MR images. Children with Wildervanck syndrome should be investigated for craniospinal abnormalities with MR imaging. PMID:12002143

Balci, Sevim; Oguz, Kader Karli; Firat, M Murat; Boduroglu, Koray

2002-04-01

426

Aase syndrome  

MedlinePLUS

Aase-Smith syndrome; Hypoplastic anemia/Triphalangeal thumb syndrome ... Jones KL, ed. Aase syndrome. In: Smith's Recognizable Patterns Of Human Malformation. 6th ed. Saunders. 2005. Clinton C, Gazda HT. Diamond-Blackfan Anemia. 2009 Jun 25 [Updated 2013 Jul ...

427

Velocardiofacial Syndrome  

ERIC Educational Resources Information Center

Velocardiofacial syndrome (VCFS), also known as DiGeorge, conotruncal anomaly face, and Cayler syndromes, is caused by a microdeletion in the long arm of Chromosome 22. We review the history of the syndrome from the first clinical reports almost half a century ago to the current intriguing molecular findings associating genes from the…

Gothelf, Doron; Frisch, Amos; Michaelovsky, Elena; Weizman, Abraham; Shprintzen, Robert J.

2009-01-01

428

Gardner's syndrome  

Microsoft Academic Search

In recent years, a number of comprehensive reviews have been written on inherited intestinal polyposis syndromes (1–7), but none has dealt specifically with Gardner's syndrome and none has focused on basic research being carried out in an attempt to under-stand this syndrome and to improve the medical management of affected patients. A better understanding of this rare genetic disorder is

Edwin W. Naylor; Emanuel Lebenthal

1980-01-01

429

Velocardiofacial Syndrome  

Microsoft Academic Search

Velocardiofacial syndrome (VCFS), also known as DiGeorge, conotruncal anomaly face, and Cayler syndromes, is caused by a microdeletion in the long arm of Chromosome 22. We review the history of the syndrome from the first clinical reports almost half a century ago to the current intriguing molecular findings associating genes from the microdeletion region and the physical and neuropsychiatric phenotype

Doron Gothelf; Amos Frisch; Elena Michaelovsky; Abraham Weizman; Robert J. Shprintzen

2009-01-01

430

Medullary thyroid carcinoma with ectopic adrenocorticotropic hormone syndrome.  

PubMed

Ectopic adrenocorticotropic hormone (ACTH) syndrome is caused most frequently by a bronchial carcinoid tumor or by small cell lung cancer. Medullary thyroid carcinoma (MTC) is a rare etiology of ectopic ACTH syndrome. We describe a case of Cushing syndrome due to ectopic ACTH production from MTC in a 48-year-old male. He was diagnosed with MTC 14 years ago and underwent total thyroidectomy, cervical lymph node dissection and a series of metastasectomies. MTC was confirmed by the pathological examination of the thyroid and metastatic mediastinal lymph node tissues. Two years after his last surgery, he developed Cushingoid features, such as moon face and central obesity, accompanied by uncontrolled hypertension and new-onset diabetes. The laboratory results were compatible with ectopic ACTH syndrome. A bilateral adrenalectomy improved the clinical and laboratory findings that were associated with Cushing syndrome. This is the first confirmed case of ectopic ACTH syndrome caused by MTC in Korea. PMID:24741461

Choi, Hong Seok; Kim, Min Joo; Moon, Chae Ho; Yoon, Jong Ho; Ku, Ha Ra; Kang, Geon Wook; Na, Im Il; Lee, Seung-Sook; Lee, Byung-Chul; Park, Young Joo; Kim, Hong Il; Ku, Yun Hyi

2014-03-01

431

Wolfram syndrome maps to distal human chromosome 4p  

SciTech Connect

Wolfram syndrome (MIM 222300) is an autosomal recessive disorder defined by the occurrence of diabetes mellitus and progressive bilateral optic atrophy. Wolfram syndrome homozygotes develop widespread nervous system abnormalities; in particular, they exhibit severe behavioral difficulties that often lead to suicide attempts or psychiatric hospitalizations. The Wolfram syndrome gene also predisposes heterozygous carriers to psychiatric disorders. Since these heterozygotes are common in the general population, the Wolfram syndrome gene may contribute significantly to the overall burden of psychiatric illness. Based on a linkage analysis of 11 families segregating for this syndrome, using microsatellite repeat polymorphisms throughout the human genome, we found the Wolfram syndrome gene to be linked to markers on the short arm of human chromosome 4, with Zmax=6.46 at {theta}=0.02 for marker D4S431.

Polymeropoulos, M.H. [National Center for Human genome Research, Bethesda, MD (United States); Swift, R.; Swift, M. [New York Medical College, Hawthorne, NY (United States)

1994-09-01

432

Harlequin syndrome as a complication of epidural anesthesia.  

PubMed

Harlequin syndrome is a rare neurological condition that results in unilateral facial flushing and sweating. Although the syndrome is generally a benign condition with complete resolution if appropriate treatment is initiated, unilateral facial flushing can be a sign of several serious conditions and should be thoroughly investigated. Sudden onset of facial flushing related to harlequin syndrome developed in a patient who had bilateral lung transplant with postoperative epidural anesthesia for pain control. Differential diagnosis includes neurovascular disease (acute stroke), malignant neoplasm of brain or lung, Horner syndrome, idiopathic hyperhidrosis, and Frey syndrome. Harlequin syndrome is often easily treated by discontinuing the anesthetic or adjusting placement of the epidural catheter. (Critical Care Nurse. 2014;34[3]:57-61). PMID:24882829

Boling, Bryan; Key, Christopher; Wainscott, Justin; Rebel, Annette

2014-06-01

433

Bilateral cochlear implantation: current concepts, indications, and results.  

PubMed

The optimal treatment for bilateral hearing loss continues to evolve as cochlear implant (CI) and hearing aid technologies advance, as does our understanding of the central auditory system. Ongoing discussions continue on the validity and feasibility of bilateral CI in terms of performance, justification of need, medical/surgical safety concerns, and economics. The purpose of this review article is to provide an update on the advantages and disadvantages of bilateral CI and to provide a discussion on timing (simultaneous vs. sequential), technology (bimodal vs. binaural) and feasibility. Binaural advantages are found in both adult and pediatric bilateral CI recipients, the greatest being the head shadow effect and improvements in localization and loudness summation. This theoretically offers an advantage over their unilateral implanted counterparts in terms of improved sound localization and enhanced speech perception under noisy conditions. Most investigators agree that bilateral stimulation during critical periods of development is paramount for optimizing auditory functioning in children. Currently, bilateral CI is widely accepted as a safe and effective means of bilateral auditory stimulation. PMID:19894280

Basura, Gregory J; Eapen, Rose; Buchman, Craig A

2009-12-01

434

Morphologic types of breast cancer: age, bilaterality, and family history.  

PubMed

Morphologic type is not generally included in the study of cancer risk factors. In breast cancer, attention is being given to age at diagnosis. In this study cases were analyzed by morphologic type as well as age at diagnosis for occurrence of bilateral disease and for family history of breast cancer. Of the morphologic types which were more frequent in younger patients--lobular in situ, medullary, and intraductal comedo--only lobular in situ had an increased frequency of bilaterality (fourfold increase). Of these, only patients with intraductal comedo reported familial breast cancer more frequently than the average. Lobular infiltrating carcinoma is diagnosed more frequently in elderly patients and is associated with 2.6 times more bilaterality and increased familial risk. Diagnosis at age 45 to 54 is also associated with increased bilaterality, but the diagnosis of lobular carcinoma has much stronger association with bilaterality than early age at diagnosis. Because of the high risk of bilaterality in lobular neoplasia and in those with a family history of cancer occurring before the menopause, bilateral biopsies and subsequent screening for malignancy should be done in these women. PMID:6243191

Erdreich, L S; Asal, N R; Hoge, A F

1980-01-01

435

M?bius Syndrome: Surgical Treatment for Eyelid Dysfunction  

PubMed Central

Introduction Möbius syndrome is a heterogeneous congenital disorder that is linked to bilateral palsies of the cranial nerves VI and VII, resulting in congenital facial paralysis sometimes associated with impaired ocular abduction. Case Report We present the case of a 44-year-old woman with Möbius syndrome and inferior recurrent keratitis secondary to scleral show in both eyes. We decided to use a cartilage graft from the ear in the inferior eyelid to avoid eyelid retraction and scleral show. Discussion Patients with Möbius syndrome have a severe dysfunction of their facial mimic. Their treatment must be individualized, depending on their age, clinical examination and symptoms.

Lopez-Valverde, Gloria; Jarrin-Hernandez, Elena; Cruz-Gonzalez, Fernando; Mateos-Sanchez, Encarnacion

2013-01-01

436

Proteus Syndrome: Report of a Case with Developmental Glaucoma  

PubMed Central

The purpose of this study was to report developmental glaucoma and pseudopapilledema in a patient with Proteus syndrome. We defined the presence of developmental glaucoma, right pseudopapilledema and myopia in a 4.5-year-old patient with Proteus syndrome. Marked right hemihypertrophy, lipoma, macrodactyly, and asymmetry of the limbs were observed on systemic examination. A cavernoma was also detected in magnetic resonance imaging of the brain. The patient underwent bilateral goniotomy surgery due to glaucoma. The surgical outcomes were satisfactory in both eyes. In conclusions developmental glaucoma and pseudopapilledema might be associated with Proteus syndrome.

Yuksel, Nursen; Sarman, Hakan; Bayramgurler, Dilek

2014-01-01

437

Proteus syndrome: report of a case with developmental glaucoma.  

PubMed

The purpose of this study was to report developmental glaucoma and pseudopapilledema in a patient with Proteus syndrome. We defined the presence of developmental glaucoma, right pseudopapilledema and myopia in a 4.5-year-old patient with Proteus syndrome. Marked right hemihypertrophy, lipoma, macrodactyly, and asymmetry of the limbs were observed on systemic examination. A cavernoma was also detected in magnetic resonance imaging of the brain. The patient underwent bilateral goniotomy surgery due to glaucoma. The surgical outcomes were satisfactory in both eyes. In conclusions developmental glaucoma and pseudopapilledema might be associated with Proteus syndrome. PMID:24882963

Sarman, Zuleyha Sik; Yuksel, Nursen; Sarman, Hakan; Bayramgurler, Dilek

2014-06-01

438

Möbius syndrome with oculomotor nerve paralysis without abducens paralysis.  

PubMed

Möbius syndrome is typified by bilateral facial nerve palsies, usually with abducens palsies. We examined an infant with Möbius syndrome who had bifacial weakness and third nerve palsies, but intact abduction of both eyes. Lower cranial nerve involvement, leading to respiratory, swallowing, and cardiac difficulties, was also present. Pathologic examination of the brainstem showed absent or hypoplastic third, seventh, tenth, and twelfth nerve nuclei. The fourth, fifth, sixth, and eighth nerve nuclei were intact. In Möbius syndrome with ocular motor palsies, rarely the sixth nerve may be spared. PMID:9858013

Tran, D B; Wilson, M C; Fox, C A; Clancy, R; Teener, J W; Golden, J A; Liu, G T

1998-12-01

439

Wiedemann-Rautenstrauch syndrome: report of a variant case.  

PubMed

Wiedemann-Rautenstrauch syndrome (WRS) is a rare autosomal recessive disorder that includes premature aging phenotype at birth. The condition is also known as a neonatal progeroid syndrome. Up to now only a few published case reports have been documented. The syndrome is characterized by progeroid appearance, decreased subcutaneous fat, hypotrichosis, macrocephaly, and in some natal teeth. We describe a new patient with features of bilaterally pelvicalyceal ectasia and partial syndactyly on 2th and 3th toes, not previously described, to our knowledge. PMID:22585414

Kiraz, Aslihan; Ozen, Samim; Tubas, Filiz; Usta, Yusuf; Aldemir, Ozgur; Alanay, Yasemin

2012-06-01

440

From bilateral to multi-countries cooperation  

NASA Astrophysics Data System (ADS)

In 2010, the Office for Science and Technology of the French Embassy in Washington DC provided seed funding to enable a new scientific collaboration between the US and France. The work primarily focuses on the understanding of the interaction of particles with the DNA. The National Society of Black Physicists managed the funds. This so-called G4TRAP work is an extension of the ESA-funded G4DNA project that uses the Geant4 Monte Carlo toolkit and is spearheaded by the University of Bordeaux 1 in France. This initiative spawns to new areas (ultrafast lasers, nuclear/high energy physics, radiation biology and space science) and includes US industries, thanks to the interdisciplinary activities needed to advance this project. In addition, the bi-lateral collaboration was expanded to two other countries: Canada and Senegal. Subsequent funding requests were made to US agencies (NSF and DoE) as well as other French programs. More are anticipated that will also include NIH, NASA and the Canadian programs. A review of this multidisciplinary effort will be presented along with the present status on the various aspects of the G4TRAP project.

Gueye, Paul

2011-04-01

441

Bilateral symmetry in crayfish behavioral reactions.  

PubMed

The crayfish, Procambarus cubensis, placed in the central area of a plus-maze preferred to go forward in about 50% of trials; they chose the right or left arm in 20.7 and 18.9% of the trials, respectively. In a T-maze, the difference between right and left directions was also insignificant. When exploring a plus-maze, the crayfish turned to 180 degrees at the end of the arms, then turned to 90 degrees going to the next arm. The mean difference between the right and left U-turns, and the right and left turns was insignificant though some animals demonstrated a left or right preference. There was a strong correlation between the direction of U-turns and following turns ensuring the clockwise or anti-clockwise movements of the crayfish. Also we examined a possible preference of the right or left claw in the feeding behavior of the crayfish. The crayfish caught a small bloodworm given from above equally with the right or left claw. The crayfish conditioned to take a bloodworm with a claw did not demonstrate any stable preference of left or right claw in the course of the experiments. The question of bilateral asymmetries within the decapod crustaceans is discussed. PMID:18652389

Shuranova, Zhanna

2008-01-01

442

Bilateral, atraumatic, proximal tibiofibular joint instability.  

PubMed

Dislocation of the tibiofibular joint is rare and usually results from a traumatic event. Only 1 case of atraumatic proximal tibiofibular joint instability in a 14-year-old girl has been reported in the literature, however this condition might occur more frequently than once thought. A wide range of treatment options exist for tibiofibular dislocations. Currently, the first choice is a conservative approach, and when this fails, surgical means such as resection of the fibula head, arthrodesis, and reconstruction are considered. However, no consensus exists on the most effective treatment. This article reports a unique case of bilateral, atraumatic, proximal tibia and fibular joint instability involving a 30-year-old man with a 20-year history of pain and laxity in the right knee. The patient had no trauma to his knees; he reported 2 immediate family members with similar complaints, which suggests that this case is likely congenital. After conservative approaches proved to be ineffective, the patient underwent capsular reconstruction using free autologous gracilis tendon. At 6-month postoperative follow-up, the patient was pain free with no locking and instability. He then underwent surgery on the left knee. At 1-year follow-up after the second surgery, the patient had no symptoms or restrictions in mobility. We provide an alternative surgical approach to arthrodesis and resection for the treatment of chronic proximal tibiofibular instability. In the treatment of chronic tibiofibular instability, we believe that reconstruction of the tibiofibular joint is a safe and effective choice. PMID:21323280

Morrison, Troy D; Shaer, James A; Little, Jill E

2011-02-01

443

[Refractory status epilepticus presenting as shaken baby syndrome].  

PubMed

INTRODUCTION. A convulsive status in infants is usually triggered by a febrile syndrome secondary to an intercurrent infection or an infection affecting the central nervous system. Shaken baby syndrome is characterised by its association with bilateral or multifocal haemorrhage, retinal haemorrhage and encephalopathy. Children under one year old are the group with the highest risk, with a maximum incidence reaching a peak between 10 and 16 weeks of age. Intercurrent processes, such as baby colic or febrile syndromes that tend to step up crying, are usually precipitating factors of shaking. CASE REPORTS. We present the cases of two infants who began with a status epilepticus within a context of a febrile syndrome. Imaging tests revealed bilateral subdural haematomas in different stage of progress and bilateral retinal haemorrhages were observed in the fundus oculi of both children. CONCLUSIONS. In a child with an unspecific febrile process that develops a convulsive status, the professional should suspect, in addition to more usual problems, shaken baby syndrome as a possible causation, above all if the child is under six months old. PMID:24915031

Fernandez-Menendez, A; Buenache-Espartosa, R; Coca-Perez, A; Morillo-Carnero, P; Martinez-San Millan, J; Lorenzo-Sanz, G

2014-06-16

444

Delleman Oorthuys syndrome: 'Oculocerebrocutaneous syndrome'  

PubMed Central

Delleman Oorthuys syndrome (oculocerebrocutaneous syndrome) is a rare, congenital sporadic disorder affecting the skin and central nervous system. We present the case of a one-month-old male who presented with an orbital cyst in the left eye since birth along with other manifestations of this syndrome. The manifestations of this syndrome resemble other developmental disorders like Goldenhar and Goltz syndrome. Conservative management of the orbital cyst in these cases have been described. The need to diagnose this rare congenital anomaly with cerebral malformations as a separate entity is crucial in the management of these children.

Arora, Vipul; Kim, Usha R; Khazei, Hadi M

2009-01-01

445

Geleophysic dysplasia associated with bilateral angle closure glaucoma  

PubMed Central

In this case report, we present occurrence of bilateral angle closure glaucoma in a 9-year-old girl with geleophysic dysplasia. Bilateral YAG laser iridotomy was applied, but intraocular pressure (IOP) remained at high levels, necessitating bilateral trabeculectomy with mitomycin C. On her follow-up examinations for 3 years, IOP remained in the mid-20s with no need for further intervention or antiglaucoma medication. There are few reports describing the ocular findings of geleophysic dysplasia in literature. To our knowledge, this is the first case report describing an application of glaucoma surgery and its results at geleophysic dysplasia.

Saricaoglu, Murat Sinan; Guven, Dilek; Karakurt, Ahmet; Hasiripi, Hikmet

2013-01-01

446

A rare case of bilateral orbital castleman disease.  

PubMed

Abstract Castleman disease is a non-neoplastic cause of lymphadenopathy, first described in 1956 by Dr. Benjamin Castleman. Orbital involvement in Castleman disease is extremely rare. We report a case of bilateral orbital Castleman disease in a 48-year-old Asian male who presented with bilateral inferior dystopia. MRI revealed bilateral extraconal superior orbital mass. Histopathology and immunohistochemistry of the mass revealed features of Castleman disease of hyaline vascular type. Castleman disease should be a differential in suspected idiopathic orbital inflammatory disease and lymphoproliferative disorders. PMID:24831817

Mukherjee, Bipasha; Alam, Mohammad Shahid; Krishnakumar, S

2014-08-01

447

Simultaneous Bilateral Vertebral Artery 3D Rotational Angiography  

PubMed Central

Summary We describe the first reported use of simultaneous bilateral vertebral artery rotational angiography to visualize an aneurysm. Images acquired from 3D rotational angiography with a single vertebral injection provided insufficient anatomic detail in two cases with vertebrobasilar junction aneurysms associated with fenestration. Therefore, simultaneous bilateral vertebral artery 3D rotational runs were performed. Anatomic detail was superior with the simultaneous injection and this allowed for the appropriate plan of care to be instituted. In the rare cases of vertebrobasilar junction aneurysm associated with fenestration, simultaneous bilateral vertebral artery 3D rotational angiography may provide the most useful anatomic detail when evaluating a patient for possible endovascular intervention.

Reavey-Cantwell, J.F.; Lewis, S.B.; Firment, C.

2005-01-01

448

Bilateral eyelid erythema associated with false eyelash glue.  

PubMed

We report an unusual case of bilateral eyelid erythema caused by eyelash glue. A 22-year-old woman presented with a 3-day history of bilateral eyelid dermatitis after attaching false eyelashes by using latex-containing glue. Slit-lamp examination revealed erythema and swelling of the upper lids of both eyes. The skin prick test was positive for eyelash glue and her total tear IgE score was high. To the best of our knowledge, this is the first report of bilateral eyelid dermatitis caused by eyelash glue. PMID:22519514

Mimura, Tatsuya

2013-03-01

449

Neurological impairment in congenital bilateral ptosis with ophthalmoplegia.  

PubMed

A case is described of congenital bilateral ptosis and ophthalmoplegia due to incomplete bilateral paralysis of the third cranial nerve associated with dysmorphisms, brain malformations and epileptiform EEG abnormalities. We hypothesize that in our case the ophthalmological disturbance is due to mesencephalic impairment. In literature there are few reports of congenital bilateral paralysis of the third cranial nerve and they lack detailed MRI findings. We stress in patients with congenital third cranial nerve palsy the importance of thorough neurological investigations including prolonged wake-sleep EEG monitoring as well as CT scan and MRI to establish the origin of the disorder. PMID:1621924

Parmeggiani, A; Posar, A; Leonardi, M; Rossi, P G

1992-03-01

450

Simultaneous bilateral spontaneous pneumothorax with congenital pleuro-pleural communication  

PubMed Central

A single pleural space can lead to serious simultaneous bilateral pneumothorax in cases of congenital or acquired pleuro-pleural communication. Here we report a 35-year-old man with bilateral pneumothorax. Chest computed tomography scans revealed a small air space between the esophagus and aorta, suggesting pleuro-pleural communication. Bilateral thoracoscopic bullectomy was performed. Repeated inspection revealed a 2-cm-long pleural window between the aorta and esophagus, which was closed with a collagen patch. Although congenital pleuro-pleural communication has been regarded as rare, as many as nine patients have been reported (if suspected cases are included). The lower middle mediastinum should be carefully inspected.

Suzuki, Takashi; Yokoi, Masahide; Yoshida, Shuji; Takeyama, Teruaki; Nakazaki, Haruhiro; Goto, Hidenori; Sato, Fumitomo; Takagi, Keigo; Otsuka, Hajime

2013-01-01

451

Spheniodal mucocele causing bilateral optic neuropathy and ophthalmoplegia.  

PubMed

Sphenoid sinus mucocele comprises only 2% of all paranasal sinus mucoceles. In literature, there is a case report on sphenoidal mucocele causing bilateral optic neuropathy, with unilateral partial recovery and cranial nerve palsy, but we did not come across any literature with bilateral optic neuropathy and ophthalmoplegia together caused by spheno-ethmoidal mucocele. We present such a rare case of spheno-ethmoidal mucocele causing bilateral optic neuropathy and unilateral sixth nerve palsy who had postsurgery, unilateral good vision recovery, and complete resolution of sixth nerve palsy. PMID:23571243

Selvakumar, Ambika; Mahalaxmi, Balasubramanyam; Ananth, V; Gautam, Cugati

2014-04-01

452

Imaging of acute bilateral paramedian thalamic and mesencephalic infarcts.  

PubMed

Thalami and midbrain arterial supply arises from many perforating blood vessels with a complex distribution for which many variations have been described. One rare variation, named the "artery of Percheron," is a solitary arterial trunk that arises from one of the proximal segments of a posterior cerebral artery and supplies the paramedian thalami and the rostral midbrain bilaterally. Occlusion of this artery results in bilateral thalamic and mesencephalic infarctions. We describe three patients with a presumed occlusion of the artery of Percheron in whom MR imaging showed characteristic symmetrical bilateral paramedian thalamic and mesencephalic infarctions. PMID:14625223

Matheus, M Gisele; Castillo, Mauricio

2003-01-01

453

A 72-year-old patient with bilateral Maisonneuve fractures  

PubMed Central

Maisonneuve fractures result from a disruption of the medial ankle structures and a proximal fibular fracture. Patient complaints can be misleading and there i