Nakazato, Yoshihiko; Shimazu, Tomokazu; Takei, Kazuo; Sugawara, Kayoko; Araki, Nobuo; Tamura, Naotoshi; Shimazu, Kunio
The incidence and mechanism of diplopia were investigated in 31 patients with Wallenberg's syndrome resulting from acute dorsolateral medullary infarction. Diplopia was found in 10 of 31 patients (32%), with 5 patients reporting vertical diplopia alone and 5 reporting vertical and horizontal diplopia. Diplopia in Wallenberg's syndrome is considered to be caused by a lesion involving the otolith-ocular system. Vertical diplopia is simply explained by ocular skew deviation due to a lesion involving the vestibular nucleus; in which the affected eye becomes deviated inferiorly. In this situation, rotation of the eye due to ocular tilt reaction also occurs. Concomitant horizontal diplopia may require involvement of the medial longitudinal fasciculus (MLF), which produces skew deviation in mirror image; the unaffected eye becomes deviated inferiorly. When downward deviation of the eye affected by dysfunction of the vestibular nucleus and that due to MLF dysfunction affecting the other eye are comparable, only horizontal diplopia becomes apparent. MLF syndrome may be accompanied by paralytic pontine exotropia (PPE) or non-paralytic pontine exotropia (NPPE), both of which may also participate in the appearance of horizontal diplopia.
Gawęcki, Maciej; Grzybowski, Andrzej
The authors present systematic review of aetiology and treatment of diplopia related to cataract surgery. The problem is set in the modern perspective of changing cataract surgery. Actual incidence is discussed as well as various modalities of therapeutic options. The authors provide the guidance for the contemporary cataract surgeon, when to expect potential problem in ocular motility after cataract surgery. PMID:26998351
Sokwala, Ahmed; Knapp, Christopher; Gottlob, Irene
Neurofibromatosis type II (NF II) is rare and most commonly presents with hearing loss, tinnitus and/or vestibular disturbance in the third decade of life. The authors describe a rare case presenting with NF II with vertical diplopia due to IV(th) nerve palsy. The patient was otherwise asymptomatic despite multiple extensive lesions on MRI.
Lipton, J R; Page, A B; Lee, J P
Diplopia in the inferior field due to orbital trauma is a notoriously difficult and disabling problem. Even if a blow-out fracture is repaired by current methods, patients may still have diplopia. There also exists another group in whom no demonstrable radiological fracture can be found, and yet there is a severe down gaze deficit. The possible mechanisms of this phenomenon are discussed and the results of horizontal muscle transposition surgery in a series of nine patients is reported. The authors conclude that this procedure is a valuable method of increasing the useful field of binocular single version in these patients.
Heinmiller, Laura J; Wasserman, Barry N
In patients with a history of strabismus, refractive surgery can result in decompensation of ocular alignment, with subsequent diplopia. Refractive surgery in the management of strabismus has been described, although it remains controversial. We present a young adult with past history of strabismus surgery and new-onset diplopia after refractive surgery. Binocular diplopia was treated surgically with laser in situ keratomileusis.
Hassen, Getaw Worku; Kalantari, Hossein
Subdural hematoma (SDH) is a rare, but life-threatening complication of spinal anesthesia. Subdural hematoma resulting from this procedure could present with vague symptoms such as chronic headache and could easily be missed. Chronic headache is one of the symptoms of chronic SDH in postpartum women. Diplopia as the presenting complaint in SDH secondary to peripartum spinal anesthesia has not, to our knowledge, been previously reported. Here, we report a case of diplopia secondary to postpartum subacute bilateral SDHs with transtentorial herniation after spinal anesthesia in a healthy primagravid 25-year-old woman. SDH can expand gradually and the initial symptoms might be subtle as in our case, despite critically high intracranial pressure. PMID:22461938
Butler, L; Yap, T; Wright, M
PurposeTo assess the diagnostic accuracy of the Edinburgh diplopia diagnostic algorithm.MethodsThis was a prospective study. Details of consecutive patients referred to ophthalmology clinics at Falkirk Community Hospital and Princess Alexandra Eye Pavilion, Edinburgh, with double vision were collected by the clinician first seeing the patient and passed to the investigators. The investigators then assessed the patient using the algorithm. An assessment of the degree of concordance between the 'algorithm assisted' diagnosis and the 'gold standard' diagnosis, made by a consultant ophthalmologist was then carried out. The accuracy of the pre-algorithm diagnosis made by the referrer was also noted.ResultsAll patients referred with diplopia were eligible for inclusion. Fifty-one patients were assessed; six were excluded. The pre-algorithm accuracy of referrers was 24% (10/41). The algorithm assisted diagnosis was correct 82% (37/45) of the time. It correctly diagnosed: cranial nerve (CN) III palsy in 6/6, CN IV palsy in 7/8, CN VI palsy in 12/12, internuclear ophthalmoplegia in 4/4, restrictive myopathy in 4/4, media opacity in 1/1, and blurred vision in 3/3. The algorithm assisted diagnosis was wrong in 18% (8/45) of the patients.ConclusionsThe baseline diagnostic accuracy of non-ophthalmologists rose from 24 to 82% when patients were assessed using the algorithm. The improvement in the diagnostic accuracy resulting from the use of the algorithm would, hopefully, result in more accurate triage of patients with diplopia that are referred to the hospital eye service. We hope we have demonstrated its potential as a learning tool for inexperienced clinicians.
Khakshoor, Hamid; Moghaddam, Ali Akbar Saber; Vejdani, Amir Hossein; Armstrong, Blair K.; Moshirfar, Majid
Foodborne botulism is a serious condition caused by Clostridium botulinum neurotoxin. Clinically, botulism presents as bilateral cranial nerve neuropathy and descending paralysis. We report a unique presentation of botulism to remind clinicians of this potentially fatal condition. In this observational case report initial evaluation showed only esodeviation. This progressed to unilateral cranial nerve six (CN VI) paresis along with systemic signs. Clinical diagnosis was made based on in-depth history and concurrent symptoms in three other patients. Foodborne botulism presenting as diplopia and unilateral motility deficits is rare and can represent a diagnostic and therapeutic challenge to the ophthalmologist. PMID:22993467
canopy acts as if it were a weak negative lens, which causes a light divergence of approximately 3.5 mrad in the forward area ( Genco , 1983, 1984; Task...AFAMRL ( Genco , 1984; Task, 1983) indicate that F-16 canopy optics can place a distant target at an optical distance of about 3.5 mrad in front of an...of America, 57, 819-830. Genco , LtCol L. V. (1983, May). Optical Interactions of aircraft windscreens and HUDs producing diplopia. in NAECON 83: Mini
Zhou, Ling-Yun; Liu, Tie-Juan; Li, Xue-Mei
AIM To estimate the adult reference values for measured deviations by a computerized diplopia test and testify the validity. METHODS Totally 391 participants were recruited and taken the computerized diplopia test. The plots and amplitude of deviations were recorded. The differences in different gender, age and visual acuity groups were analyzed respectively. Of 30 subjects were enrolled to testify the interobserver reliability. Another 46 subjects (including 26 normal subjects and 20 patients) were taken the test and theirs deviations were recorded to testify the validity of the reference value. RESULTS The max horizontal and vertical deviations were 2.55° and 0.76° with normal corrected visual acuity while 3.88° and 1.46° for subjects with poor corrected vision. The differences between age groups was insignificant (Z=3.615, 4.758; P=0.461, 0.313 for horizontal and vertical respectively). The max horizontal deviation of female was smaller than male (Z=-2.177; P=0.029), but the difference in max vertical deviation was insignificant (Z=-1.296; P=0.195). The mean difference between observers were both -0.1°, with 95% confidence limits (CI) of -1.4° and 1.6° in max horizontal deviations while -2.1° and 1.8° in max vertical deviation. The mean deviation of 26 normal subjects was 1.02°±0.84° for horizontal and 0.47°±0.30° for vertical which both within the range of reference values. The mean deviation of 20 patients was 13.51°±11.69° for horizontal and 8.34°±8.58° for vertical which both beyond the reference range. CONCLUSION The max amplitude of horizontal and vertical deviation is pointed as the numerical parameters of computerized diplopia test. The reference values are different between normal corrected visual acuity and poor corrected vision. These values may useful for evaluating patients with diplopia in veriety conditions during clinical practice. PMID:27990370
Yee, R D; Purvin, V A; Azzarelli, B; Nelson, P B
PURPOSE: Two cases illustrate the symptoms, signs, etiologies, and treatment of ocular neuromyotonia (ONM). METHODS: The histories, neuroradiologic tests, and/or biopsy revealed the etiologies of ONM in both patients. Clinical observations, videotaping, and electronic eye movement recordings documented the eye movements. RESULTS: A 72-year-old man with chronic arachnoiditis following myelography with thorium dioxide (Thorotrast) developed intermittent diplopia and a partial right third nerve palsy. Left gaze induced spasm of the right medial rectus. Right gaze produced right lateral rectus spasm. A 66-year-old woman, who had radiation treatment for a pituitary tumor and acromegaly, had intermittent spasm of the left medial rectus muscle and left esotropia. The episodes occurred spontaneously and were induced by right gaze. A left internuclear ophthalmoplegia was also found. Carbamazepine (Tegretol) abolished the ONM in both patients. CONCLUSIONS: Although ONM is an unusual cause of intermittent diplopia and strabismus, its distinctive clinical features identify it. Injury to the peripheral cranial nerves probably leads to segmental demyelination, axonal hyperexcitability, and a self-perpetuating, reverberating circuit, which causes spasms of the extraocular muscles. Images FIGURE 4 FIGURE 5 FIGURE 6 FIGURE 7 FIGURE 8 PMID:8981697
Introduction. Acute complete external ophthalmoplegia is a rare finding in clinical practice that is associated with diseases affecting the neuromuscular junction, the oculomotor nerves, or the brainstem. Ophthalmoplegia has been reported with acute ataxia in Miller Fisher syndrome (MFS) and Bickerstaff brainstem encephalitis (BBE). Up to 95% of these cases are associated with anti-GQ1b antibodies. Only a small number of cases of anti-GQ1b negative MFS have been documented in pediatric patients. This is the first case reporting a recurrence of ocular symptoms in an anti-GQ1b antibody negative patient with BBE. Case Presentation. An 8-year-old Caucasian boy presented with complete external ophthalmoplegia without ptosis, cerebellar ataxia, and a disturbance of consciousness. He had recently recovered from a confirmed Campylobacter jejuni infection. On subsequent laboratory testing he was anti-GQ1b antibody negative. He had a recurrence of diplopia at four-week follow-up. Conclusions. This patient's recurrence of diplopia was treated with a five-week course of oral corticosteroids which did not worsen his condition, and this may be a therapeutic option for similar patients. We will discuss the symptoms and treatment of reported pediatric cases of anti-GQ1b antibody negative cases of MFS and the variation between cases representing a spectrum of illness. PMID:27293928
Robert, Matthieu P; Bonci, Fabrizio; Pandit, Anand; Ferguson, Veronica; Nachev, Parashkev
Binocular diplopia is a debilitating visual symptom requiring immediate intervention for symptomatic control, whether or not definitive treatment is eventually possible. Where prismatic correction is infeasible, the current standard is occlusion, either by a patch or an opaque contact lens. In eliminating one problem—diplopia—occlusive techniques invariably create another: reduced peripheral vision. Crucially, this is often unnecessary, for the reduced spatial resolution in the periphery limits its contribution to the perception of diplopia. Here, we therefore introduce a novel soft contact lens device that instead creates a monocular central scotoma inversely mirroring the physiological variation in spatial acuity across the monocular visual field, thereby suppressing the diplopia with minimal impact on the periphery. We compared the device against standard eye patching in 12 normal subjects with prism-induced binocular diplopia and 12 patients with binocular diplopia of diverse causes. Indexed by self-reported scores and binocular perimetry, the scotogenic contact lens was comparably effective in eliminating the diplopia while significantly superior in acceptability and its impact on the peripheral visual field. This simple, inexpensive, non-invasive device may thus be an effective new tool in the treatment of a familiar but still troublesome clinical problem. PMID:25680615
Park, Min Seok; Kim, Young Joon; Kim, Hoon; Nam, Sang Hyun
Background Isolated pure blowout fractures are clinically important because they are the main cause of serious complications such as diplopia and limitation of extraocular movement. Many reports have described the incidence of blowout fractures associated with diplopia and limitation of extraocular movement; however, no studies have statistically analyzed this relationship. The purpose of this study was to demonstrate the correlation between the location of isolated pure blowout fractures and orbital symptoms such as diplopia and limitation of extraocular movement. Methods We enrolled a total of 354 patients who had been diagnosed with isolated pure blowout fractures, based on computed tomography, from June 2008 to November 2011. Medical records were reviewed, and the prevalence of extraocular movement limitations and diplopia were determined. Results There were 14 patients with extraocular movement limitation and 58 patients complained of diplopia. Extraocular movement limitation was associated with the following findings, in decreasing order of frequency: floor fracture (7.1%), extended fracture (3.6%), and medial wall (1.7%). However, there was no significant difference among the types of fractures (P=0.60). Diplopia was more commonly associated with floor fractures (21.4%) and extended type fractures (23.6%) than medial wall fractures (10.4%). The difference was statistically significant (Bonferroni-corrected chi-squared test P<0.016). Conclusions Data indicate that extended type fractures and orbital floor fractures tend to cause diplopia more commonly than medial wall fractures. However, extraocular movement limitation was not found to be dependent on the location of the orbital wall fracture. PMID:22783527
Laria, Carlos; Alió, Jorge L; Piñero, David N
We present the case of a 29 man complaining of intractable diplopia during the last 5 years. He had undergone several surgical procedures for the treatment of his infantile strabismus since age 6 years. After surgery, the patient had been treated on 4 occasions with Botox. He also performed antisuppression exercises to encourage binocular vision. On our examination, the patient showed a 20/20 visual acuity in both eyes and a strabismic dysfunction with slight alphabet pattern, which induced a disturbing constant diplopia. Several treatment options were considered as occlusion therapy or cosmetic contact lenses, but they were not used because they were not acceptable esthetically or not tolerated. Finally, an optical penalization was induced by means of a black corneal tattooing placed at the centre of the cornea. The patient was followed for a period of 18 months, showing a complete elimination of diploia with esthetical acceptance and no inflammatory signs.
Lefaucheur, Romain; Bouwyn, Jean Paul; Wallon, David; Bedat-Millet, Anne-Laure; Ahtoy, Patrick; Perot, Guillaume; Hannequin, Didier; Maltête, David
We described the case of a patient with recurrent episodes of isolated diplopia over the last 30 years. On her last event, neurological examination revealed not only a right third and sixth cranial nerves involvement, but also a right peripheral facial palsy and a motor weakness on the left ulnar territory. Electrophysiological nerves motor conduction study revealed a conduction block on the left ulnar nerve and a less severe on the right ulnar nerve. Asymmetrical upper limb sensorimotor weakness combined with conduction block and cranial nerves palsy led to a diagnosis of Lewis and Sumner syndrome (LSS). This case is unusual by the presentation of the disease and is, to our knowledge the longer natural disease course of LSS reported. Moreover, it suggests that the recurrent diplopia variant may represent a separate entity with a good prognosis even in absence of invasive treatment.
Käsmann, B; Ruprecht, K W
Cortical blindness is defined as a loss of vision due to bilateral retrogeniculate lesions (geniculocalcarine blindness). Gerstmann's syndrome is a combination of disorientation for left and right, finger agnosia, and profound agraphia, alexia, and acalculia. It is due to a lesion in the left angular gyrus, situated at the confluence of the temporal, parietal, and occipital lobes. We report on a patient who suffered from severe underdiagnosed eclampsia and who developed bilateral extensive medial temporal, parietal, and calcarine ischemic infarctions during an eclamptic fit. In addition, ischemia destroyed the left angular gyrus. The combination of these lesions led to Gerstmann's syndrome with additional cortical agnosia and cortical diplopia. For the first few months following the ischemic insult, the patient had been cortically blind. Thereafter, the patient slowly regained a visual acuity of 0.1 in both eyes. She then experienced monocular and binocular diplopia. Her ocular motility was normal; there was no phoria or tropia. Monocular and binocular diplopia slowly became less severe over the following year. Now, 2 years after the incident, the patient has a visual acuity of 0.2 in both eyes and no double vision. However, the handicapping symptoms of Gerstmann's syndrome, which make leading a normal life impossible, have persisted--the patient still cannot cope alone, mainly due to the severe disorientation for left and right. The picture of cortical agnosia, cortical diplopia, and Gerstmann's syndrome is a very rare combination. Visual recovery and rehabilitation in cortical blindness are severely affected and made difficult by the symptoms of Gerstmann's syndrome. In our case the reason for such a dramatic clinical picture was eclampsia, whose prodomes had not been diagnosed in time.
Apfelbaum, Henry L.; Ross, Nicole C.; Bowers, Alex R.; Peli, Eli
Purpose: While prisms are commonly prescribed for homonymous hemianopia to extend or expand the visual field, they cause potentially troubling visual side effects, including nonveridical location of perceived images, diplopia, and visual confusion. In addition, the field behind a prism at its apex is lost to an apical scotoma equal in magnitude to the amount of prism shift. The perceptual consequences of apical scotomas and the other effects of various designs were examined to consider parameters and designs that can mitigate the impact of these effects. Methods: Various configurations of sector and peripheral prisms were analyzed, in various directions of gaze, and their visual effects were illustrated using simulated perimetry. A novel “percept” diagram was developed that yielded insights into the patient's view through the prisms. The predictions were verified perimetrically with patients. Results: The diagrams distinguish between potentially beneficial field expansion via visual confusion and the pericentrally disturbing and useless effect of diplopia, and their relationship to prism power and gaze direction. They also illustrate the nonexpanding substitution of field segments of some popular prism designs. Conclusions: Yoked sector prisms have no effect at primary gaze or when gaze is directed toward the seeing hemifield, and they introduce pericentral field loss when gaze is shifted into them. When fitted unilaterally, sector prisms also have an effect only when the gaze is directed into the prism and may cause a pericentral scotoma and/or central diplopia. Peripheral prisms are effective at essentially all gaze angles. Since gaze is not directed into them, they avoid problematic pericentral effects. We derive useful recommendations for prism power and position parameters, including novel ways of fitting prisms asymmetrically. Translational Relevance: Clinicians will find these novel diagrams, diagramming techniques, and analyses valuable when prescribing
Fa, Bernadette Alvear; Speaker, Steven R.; Budenz, Alan W.
Complications associated with various local anesthetic techniques have been recorded in case reports and reviews. This current case reports a transient incident of blurred, double vision (diplopia) following a Gow-Gates mandibular block injection. There is descriptive discussion on possibilities associated with intra-arterial injection, intravenous injection, diffusion through tissue planes, and the autonomic nervous system pathway to lend credence suggesting the etiology of the complication. For practitioners, recognizing when a complication arises from anesthesia delivery and managing the patient in an appropriate manner is essential to an overall agreeable outcome. PMID:27585417
Ota, Tomohiro; Toda, Yusuke; Ozawa, Akiko; Yamazaki, Mineo; Kimura, Kazumi
A 66-year-old man presented with headache and ophthalmalgia. Diplopia developed, and he was hospitalized. The left eye had abducent paralysis and proptosis. We diagnosed him with Tolosa-Hunt syndrome and administered methylprednisolone at 1 g/day for 3 days. However, the patient did not respond to treatment. No abnormality was found on his MRI or cerebrospinal fluid examination. Tests showed his serum immunoglobulin G4 and antineutrophil cytoplasmic antibody titers were within normal limits. He also had untreated diabetes mellitus (HbA1c 9.2). One week after first presenting with symptoms, herpes zoster appeared on the patient's dorsum nasi, followed by keratitis and a corneal ulcer. Herpes zoster ophthalmicus with ophthalmoplegia was diagnosed. We began treatment with acyclovir (15 mg/kg) and prednisolone (1 mg/kg, decreased gradually). Ophthalmalgia and the eruption improved immediately. The eye movement disorder improved gradually over several months. It is rare that diplopia appears prior to cingulate eruption of herpes zoster ophthalmicus. We speculated that onset of the eruption was inhibited by strong steroid therapy and untreated diabetes mellitus.
Duhoux, Alexandre; Chennoufi, Mehdi; Lantieri, Laurent; Hivelin, Mikael
A 47 years old woman underwent autologous fat grafting to treat a 5×4 cm depression of the lower lid and the upper cheek secondary resection of squamous cell carcinoma and subsequent coverage by full thickness skin graft. 20 mL of autologous fat were harvested from lower abdomen, centrifuged and injected subcutaneously. The patient then gained a total of 15 kg over a period of 24 months. Eye dystopia developed while the grafted area became convex. MRI confirmed subcutaneous fat mass going to the orbital floor through the inferior septal defect. The fat excess was removed through a trans-conjonctival approach allowing for a progressive regression of diplopia after 2 months while the oedema reduced. The overall follow up from the resection-coverage and last examination was 5 years. In this case with a context of noticeable weight gain, the growth of a fat graft trapped between a sclerous plane and the eye, that penetrated the orbital cavity through a septal defect led have led to exophthalmos, ocular dystopia and diplopia. Systematic overcorrection in autologous fat grafting should be prevented, especially in functional areas and on low body mass index patient that might gain weight.
Azar, Dimitri T.
We used the ArF excimer laser to determine the feasibility of performing prismatic photoablations in model eyes (plastic spheres simulating the eye), and in rabbit corneas. This would correct diplopia and small angles of deviation, and result in minimal refractive alterations. We modified excimer laser delivery system that achieved the desired corneal contour of prismatic ablations. 193-nm argon fluoride laser was used at fluence of 160 mJ/cm2 and ablation rate 5 Hz. 5.0-mm diameter, 40 um corneal epithelial ablation were followed by 5.0- mm diameter, prismatic photokeratectomy (PPK). We were able to achieve prismatic photoablation of PMMA blocks and lenses. No other refractive changes accompanied the prismatic photoablation of PMMA blocks and lenses. No other refractive changes accompanied the prismatic effect. In rabbits re-epithelialization of the 5-mm ablations was complete by day 3, and corneal haze was not observed by gross examination. Epithelial hyperplasia and subepithelial scarring were noted at the deep edges. PPK holds important therapeutic potential for fine-tuning results of conventional strabismus surgery, and for patients with stable diplopia following nerve palsy and ocular surgery.
Claeys, A; Trullemans, F; Maes, J; Hennekes, R; Salu, P
A 37-year-old male presented a sudden diplopia. Ocular examination showed a partial paresis of the left eye (LE) on the left gaze, progressing in a few days towards a total ophthalmoplegia. Further investigation revealed an intra-orbital mass, immunohistologically diagnosed as a Diffuse Large B-cell Lymphoma (DLBCL), according to the WHO classification. Since the patient was in an advanced, disseminated stage of the disease (IVA-E), treatment was based on systemic and intrathecal chemotherapy with a pancranial radiotherapy. The clinical course was poor with only a 10-month survival. We wish to stress that the possibility of orbital malignancy in young adults with acute onset of ophthalmoplegia should be included in the differential diagnosis.
Pedemonte, C; Sáez, F; Vargas, I; González, L E; Canales, M; Salazar, K
The purpose of this study was to determine whether orbital reconstruction with customized implants can correct post-traumatic orbital deformities such as late enophthalmos and delayed diplopia. The hypothesis proposed was that an overcorrection of the orbital volume is needed to resolve enophthalmos. A retrospective observational descriptive study was conducted. Patients with a major trauma who required customized orbital implants for the delayed treatment of unilateral orbital fractures that had initially been operated on using titanium mesh and/or osteosynthesis plates were included. The orbital volumes of the unaffected contralateral side, of the affected orbit after initial reconstruction with mesh, and of the affected orbit subsequently reconstructed with the customized implant were calculated. All of the patients included in this study had diplopia in the gaze position prior to the installation of the implant. In addition, they all had severe enophthalmos. After surgery, no patient with a customized implant showed diplopia. The enophthalmos was corrected in all but one case. On average, orbits reconstructed with customized implants had lower volumes compared to the unaffected contralateral side. In cases where the enophthalmos was resolved, the volume was reduced by an average of 8.55%. Further studies using a larger number of cases and with controlled volumetric corrections using CAD/CAM are needed.
Pinto, Maria do Carmo; Limbert, Catarina; Lopes, Lurdes
Pseudotumor cerebri (PTC) is defined by clinical criteria of increased intracranial pressure, elevated intracranial pressure with normal cerebrospinal fluid (CSF) composition, and exclusion of other causes such tumors, vascular abnormalities, or infections. The association of PTC with levothyroxine (LT4) has been reported. A 12-year-old boy has been followed up for autoimmune thyroiditis under LT4. Family history was irrelevant for endocrine or autoimmune diseases. A TSH level of 4.43 μUI/mL (0.39–3.10) motivated a LT4 adjustment from 75 to 88 μg/day. Five weeks later, he developed horizontal diplopia, convergent strabismus with left eye abduction palsy, and papilledema. Laboratorial evaluation revealed elevated free thyroxine level (1.05 ng/dL [0.65–1.01]) and low TSH, without other alterations. Lumbar puncture was performed and CSF opening pressure was 24 cm H2O with normal composition. Blood and CSF cultures were sterile. Brain MRI was normal. LT4 was temporarily discontinued and progressive improvement was observed, with a normal fundoscopy at day 10 and reversion of diplopia one month later. LT4 was restarted at lower dose and gradually titrated. The boy is currently asymptomatic. This case discloses the potential role of LT4 in inducing PTC. Despite its rarity and unclear association, PTC must be seen as a potential complication of LT4, after excluding all other intracranial hypertension causes. PMID:27379191
Tripathi, Kaushalendra; Tripathi, Richa; Seraji-Bozorgzad, Navid
Sjogren's syndrome is a chronic autoimmune disorder which affects the exocrine glands with lymphocytic infiltration, and occasionally involves central nervous system. It is usually rare and manifests as a lesion in the trigeminal nerve. Our case discusses the involvement of the oculomotor and abducens nerves along with the prevalence of such cases as seen on literature review. We describe a case of a middle aged woman who presented with ophthalmoplegic symptoms. The symptoms resolved in response to steroid therapy and serum analysis was positive for anti SSA antibodies. Increasing use of imaging modalities has enabled identifying cranial nerve enhancements easily. Correlating this to serum analysis, as in our case; has helped identify more cases of third and sixth cranial nerve involvement than was previously known to occur with primary Sjogren's syndrome.
Pelluru, Pavan Kumar; Rajesh, Alugolu
Chronic compression by intracranial cystic lesions can cause cranial nerve palsies and bony changes. With the advent of imaging techniques, grossly accurate diagnosis is possible. However, few cases do surprise the clinicians both intra, and postoperatively. A 27-year-old male presented to us with complaints of double vision for 4 months followed by sharp, shooting pain in the left V1 and V2 distribution for 1-month duration, on examination, he had left lateral palsy and decreased pin prick and temperature sensation in V1 distribution. On computed tomography scan, a cystic lesion noted which is isodense in the middle cranial fossa with erosion of the underlying bone. On magnetic resonance imaging lesion was iso to hyperintense on T1-Weighted and hyperintense on T2-Weighted, brilliantly enhancing on contrast administration. Provisional diagnosis was trigeminal schwannoma, left temporal craniotomy and total excision of the cyst done. Histopathological examination showed cyst wall lined with collagen. Postoperatively patient neuralgic pain subsided with persisting sixth nerve palsy. PMID:26425164
Shen, Jianqin; Cui, Hongguang; Feng, Shi
Purpose Olfactory neuroblastoma (ONB) is a rare neuroectodermal malignant neoplasm that originates from the olfactory sensory epithelium. In reported cases involving the orbit, the primary neoplasms could invade the orbits through bone destruction or cranio-orbital communication anatomical structure. The present study reports a rare case of orbital metastatic tumor arising from an intracranial ONB in a Chinese patient, which is the first case demonstrating an uncommon route of ONB involving the orbit. Patient and methods A 62-year-old man with a right orbital tumor was diagnosed as having a secondary tumor arising from intracranial ONB. Clinical examination, including ocular imaging examination, and histopathological and immunohistochemical studies were performed. Results In the ocular examination, a focal, firm, nontender swelling with ill-defined margins in the right orbit was found on palpation. Proptosis, limited eyeball movement, and visual loss were also observed. The unenhanced computed tomography scan showed a homogeneous, isolated, ill-defined soft tissue neoplasm in the right orbit. The histopathological study revealed irregularly shaped nests of tumor cells surrounded by avascularized fibrous stroma. The monomorphic malignant tumor cells are small round blue cells that are slightly larger than mature lymphocytes, with a very high nuclear to cytoplasmic ratio. The immunohistochemical staining for tumor cells was positive for synaptophysin, chromogranin A, cluster of differentiation 56 (CD56), cytokeratin, vimentin, S-100 protein, and negative for epithelial membrane antigen, and cluster of differentiation (CD99). Conclusion This is so far the first Chinese case of orbital metastasis from intracranial ONB that has ever been reported. Metastasis might be another route of ONB invading the orbit besides direct invasion. PMID:27601919
Cioplean, E Daniela; Camburu, L Raluca
We report the case of a 9-year-old child operated for intermittent exotropia and V-pattern with a good result 2 months after bilateral Lateral Rectus Muscle Recession. The binocular vision was restored in primary position and down-gaze with excellent stereopsis at near and distance and a deviation of +4 PD in primary position. Three months later, the patient developed a consecutive esotropia of + 18 PD in primary position with diplopia in all gazes triggered by Amitriptyline treatment prescribed one month earlier for nocturnal enuresis. Diplopia was solved in time after anticholinergic medication cessation. During the recovery period, Fresnell prisms have been used in order to eliminate diplopia. Three months after diplopia onset, the binocular vision was restored showing a transitory and reversible effect of the Amitriptyline treatment. Fusion vulnerability can be a possible risk factor in developing diplopia and esotropia in patients treated with anticholinergic drugs.
Guyton, David L
Strabismus developing after retrobulbar or peribulbar anesthesia for both anterior and posterior segment eye surgery may be due to myotoxicity to an extraocular muscle from the local anesthetic agent. Initial paresis often causes diplopia immediately after surgery, but later progressive segmental fibrosis occurs, and/or hypertrophy of the muscle, producing diplopia in the opposite direction from the direction of the initial diplopia. The inferior rectus muscle is most commonly affected. Usually a large recession on an adjustable suture of the involved muscle(s) yields good alignment. Using topical anesthesia or sub-Tenon's anesthesia can avoid this complication.
Pratt-Johnson, J A
With binocular perimetry suppression was found for near vision in patients with esotropia with convergence excess, whereas fusion was present for distance. If a bitenuporal stimulation was obtained by means of base-out prisms, diplopia was elicited instead.
service. Table 15 Visual standards for retention -- Category Standard Aniseikonia Correctable with iseikonic lenses Dipiopia, Intermittent and in zone...Table 16 Visual standards for mobilization Category Standard Aniseikonia Correctable with iseikonic lenses Diplopia, Intermittent and in zone
Adulkar, Namrata; Kim, Usha; Shetty, Shashikant
Pediatric orbital trauma with fracture involving the junction of roof and medial wall leading to superior oblique entrapment is rare. Here the authors report a case of orbital fracture at the junction of roof and medial wall with entrapment of the superior oblique muscle presenting clinically as canine tooth syndrome which was surgically released. Postoperatively, the ocular motility improved, and the patient was relieved of diplopia. They recommend early surgical exploration in such cases, which lead to successful resolution of diplopia.
Peden, Marc C.; Bhatti, M. Tariq
Subperiosteal orbital hemorrhage (SPOH) following cardiac surgery has not been previously reported. We present a patient who developed diplopia and right eye proptosis immediately after cardiac surgery for a mitral valve repair and coronary artery bypass graft. A computed tomography (CT) study demonstrated a right superior SPOH. The diplopia and proptosis resolved spontaneously within 4 weeks. Follow-up CT showed complete resolution of the SPOH.
Iliff, N T
Severe midfacial trauma presents several challenges to the reconstructive surgeon. Acute rigid fixation of the facial skeleton accompanied by bone grafting to restore the confines and volume of the orbit provide the best opportunity for acceptable aesthetic results. The severity of the trauma causes the late postoperative complication of enophthalmos. Injury to orbital structures with subsequent cicatricial change results in significant alteration in extraocular motility with resultant diplopia. There are no reports in the literature which critically evaluate the effect of late enophthalmos correction on extraocular motility, diplopia, and vision in patients who have suffered Le Fort or NOE fractures. A retrospective study is presented which reviews the results of late surgery for the correction of enophthalmos in 40 patients, all of whom had severe "impure" orbital fractures. This study addresses the following questions: (1) Can the globe effectively be repositioned?, (2) Is there a change in subjective diplopia?, (3) Does a change in extraocular motility occur, and if it does, is it predictable?, (4) Is there a risk to visual acuity? and finally, (5) Do the answers to questions 1 through 4 suggest that late surgical intervention for the correction of enophthalmos should be recommended for this patient population? During a 9-year period, 44 patients with severe diplopia trauma received surgery for enophthalmos correction. A review of 40 patients on whom 56 operations were performed is presented. Thirty-eight patients had enophthalmos and 35 had inferior displacement of the globe. Medial displacement of the globe occurred in 11 patients. Twenty-nine patients had diplopia. Six patients had vision too poor on the injured side to have diplopia. Enophthalmos was improved in 32 patients. Dystopia of the globe was improved in 31 cases. However, neither enophthalmos nor dystopia of the globe could be improved with every operation. Only 35 of the 48 operations for
Cheung, Stephanie S L; Ghadiali, Larissa K; Brannagan Iii, Thomas H; Moonis, Gul; Faust, Phyllis L; Odel, Jeffrey G
A 78-year-old woman complained of gradual, painless onset of horizontal binocular diplopia associated with progressive axial weakness. Physical examination revealed esotropia that was greater at distance than at near vision, bilateral levator dehiscence, and normal abducting saccadic speeds. Given the age of the patient and compatible clinical findings, the diagnosis of Sagging Eye Syndrome (SES) was made. However, further work-up with a muscle biopsy suggested Sporadic Late-Onset Nemaline Myopathy (SLONM) as the cause of her progressive muscle weakness. Although rare, external ophthalmoplegia has been described in the literature as a presenting symptom in SLONM. To elucidate the pathological mechanism for the patient's diplopia, an MRI of the orbits was performed, which revealed findings consistent with SES. This case aims to highlight the importance of integrating clinical findings during the diagnostic process and serves as a reminder that diplopia can be a common symptom for an uncommon diagnosis.
Friedrich, Reinhard E; Heiland, Max; Kehler, Uwe; Schmelzle, Rainer
A patient with neurofibromatosis type 1 had pulsating exophthalmos of the right eye with diplopia resulting from severe dysplasia of the sphenoid bone and consecutive herniation of the right temporal lobe. The right orbital tectum was reconstructed with titanium mesh and iliac spongiosa via a lateral orbitotomy using intraoperative navigation. For intraoperative referencing a cortical fixed-reference system and a skin scanning laser device were used. Postoperatively, the diplopia was reduced, but the patient asked for further treatment and the procedure was repeated 6 months later. Intraoperatively, the previously implanted titanium mesh was deformed and most of the transplanted bone was resorbed, probably because of pressure. A more extended mesh was implanted and iliac spongiosa was placed on both sides. Intraoperative navigation was used during both procedures. The adverse effects of diplopia were minimized and follow-up computed tomography after seven months confirmed that the bone graft was in place.
Ghadiali, Larissa K.; Brannagan III, Thomas H.; Moonis, Gul; Faust, Phyllis L.; Odel, Jeffrey G.
A 78-year-old woman complained of gradual, painless onset of horizontal binocular diplopia associated with progressive axial weakness. Physical examination revealed esotropia that was greater at distance than at near vision, bilateral levator dehiscence, and normal abducting saccadic speeds. Given the age of the patient and compatible clinical findings, the diagnosis of Sagging Eye Syndrome (SES) was made. However, further work-up with a muscle biopsy suggested Sporadic Late-Onset Nemaline Myopathy (SLONM) as the cause of her progressive muscle weakness. Although rare, external ophthalmoplegia has been described in the literature as a presenting symptom in SLONM. To elucidate the pathological mechanism for the patient's diplopia, an MRI of the orbits was performed, which revealed findings consistent with SES. This case aims to highlight the importance of integrating clinical findings during the diagnostic process and serves as a reminder that diplopia can be a common symptom for an uncommon diagnosis. PMID:28182120
Peli, Eli; Satgunam, PremNandhini
Bitemporal hemianopic visual field impairment frequently leads to binocular vision difficulties. Patients with bitemporal hemianopia with pre-existing exophoria complain of horizontal diplopia, sometimes combined with vertical deviation (with pre-existing hyperphoria). The symptoms are a result of the phoria decompensating into a tropia (hemi-slide) due to the lack of retinal correspondence between the remaining nasal fields of both eyes. We measured these effects using a dichoptic perimeter. We showed that aligning the eyes with prisms could prevent diplopia if the bitemporal hemianopia is incomplete. We also describe the successful use of a novel fusion aid – the ‘stereo-typoscope’ – that utilizes midline stereopsis to prevent diplopia resulting from hemi-sliding in patients with complete bitemporal hemianopia. PMID:24588535
Cordonnier, Monique; Van Nechel, Christian
There are five possible ocular signs or complaints of a life or sight threatening neuro-ophthalmological condition: diplopia, isolated anisocoria, transient visual loss, severe pain in head or neck (with or without photophobia) and oscillopsia/nystagmus. In this review, the ocular signs and symptoms of neuro-ophthalmological emergencies are described together with their practical work-up and the risks associated with missing the diagnosis. Concerning diplopia, the associated signs pointing to a possible threatening condition are emphasized. Six focus points resuming core messages are displayed throughout this review.
... claimant has both diplopia and decreased visual acuity or visual field defect, assign a level of corrected visual acuity for the poorer eye (or the affected eye, if disability of only one eye is service-connected... adjusted level of corrected visual acuity, however, must not exceed a level of 5/200. Use the...
Moster, Stephen J.; Moster, Mark L.
Abstract A 56-year-old woman developed progressive headache, mental status changes, and diplopia after trauma. She was diagnosed with alternating skew deviation caused by intracranial hypotension. This is the first case of alternating skew deviation reported from intracranial hypotension and perhaps a differential pressure between intracranial and intraspinal spaces plays a role in the development of these findings. PMID:27928294
Al Qahtani, Elham S.; Rootman, Jack; Kersey, James; Godoy, Flavia; Lyons, Christopher J.
Thyroid orbitopathy causes a unique form of strabismus with adult-onset diplopia. Prisms can be a temporizing measure, but many patients require strabismus surgery, which can be challenging. In this article, we highlight clinical guidelines for addressing strabismus due to thyroid orbitopathy and review our surgical experience. PMID:26180468
...) Evaluation of muscle function. (1) An evaluation for diplopia will be assigned to only one eye. When a... visual acuity for the poorer eye (or the affected eye, if disability of only one eye is service-connected... visual acuity for the poorer eye (or the affected eye, if disability of only one eye is...
...) Evaluation of muscle function. (1) An evaluation for diplopia will be assigned to only one eye. When a... visual acuity for the poorer eye (or the affected eye, if disability of only one eye is service-connected... visual acuity for the poorer eye (or the affected eye, if disability of only one eye is...
...) Evaluation of muscle function. (1) An evaluation for diplopia will be assigned to only one eye. When a... visual acuity for the poorer eye (or the affected eye, if disability of only one eye is service-connected... visual acuity for the poorer eye (or the affected eye, if disability of only one eye is...
Kang, Dongwan; Lee, Joonsik; Lee, Hwa; Baek, Sehyun
A 53-year-old man presented with a palpable mass on the left lower eyelid and occasional diplopia. Under suspicion of orbital lymphoma, an excisional biopsy was performed, and histopathology revealed Castleman's disease. Castleman's disease is a rare disorder of the lymphoid system, and only a few cases of Castleman's disease in the orbit have been reported.
Murthy, Ramesh; Kesarwani, Siddharth
Acquired disruption of motor fusion is a rare condition characterized by intractable diplopia. Management of these patients is extremely difficult. Prisms in any combination or even surgery may not help relieve their symptoms. We describe a longstanding case of acquired motor fusion disruption which was managed successfully with botulinum toxin injection. PMID:19861751
The correction of astigmatism is an essential element for the refractive surgery because the majority of patients have important preoperative cylinder An uncorrected astigmatism decreases visual acuity and can also cause glare, asthenopia, headaches, monocular diplopia. It is important to remark that a complete elimination of astigmatism for the eye is very rarely achieved.
Cho, Bum-Joo; Kim, Ji-Soo; Hwang, Jeong-Min
A 55-year-old woman presented with diplopia following painful skin eruptions on the right upper extremity. On presentation, she was found to have 35 prism diopters of esotropia and an abduction limitation in the left eye. Two weeks later, she developed blepharoptosis and anisocoria with a smaller pupil in the right eye, which increased in the darkness. Cerebrospinal fluid analysis showed pleocytosis and a positive result for immunoglobulin G antibody to varicella zoster virus. She was diagnosed to have zoster meningitis with Horner's syndrome and contralateral abducens nerve palsy. After intravenous antiviral and steroid treatments, the vesicular eruptions and abducens nerve palsy improved. Horner's syndrome and diplopia resolved after six months. Here we present the first report of Horner's syndrome and contralateral abducens nerve palsy associated with zoster meningitis.
Chakor, Rahul T; Santhosh, N S
Idiopathic orbital inflammation is the third most common orbital disease, following Graves orbitopathy and lymphoproliferative diseases. We present a 11 year old girl with 15 days history of painless diplopia. There was no history of fluctuation of symptoms, drooping of eye lids or diminished vision. She had near total restricted extra-ocular movements and mild proptosis of the right eye. There was no conjunctival injection, chemosis, or bulb pain. There was no eyelid retraction or lid lag. Rest of the neurological examination was unremarkable.Erythrocyte sedimentation rate was raised with eosinophilia. Antinuclear antibodies were positive. Liver, renal and thyroid functions were normal. Antithyroid, double stranded deoxyribonucleic acid and acetylcholine receptor antibodies were negative. Repetitive nerve stimulation was negative. Magnetic resonance imaging (MRI) of the orbit was typical of orbital myositis. The patient responded to oral steroids. Orbital myositis can present as painless diplopia. MRI of orbit is diagnostic in orbital myositis.
Different methods of internal fixation of simple displaced zygomatic fracture, such as wiring, miniplate, and screw fixation, were compared for postreduction rotational stability caused by muscular forces. This study was performed to evaluate the minimally invasive therapy in cases of zygomatic bone fracture. Seventeen patients were treated by percutaneous hook reduction and miniplate fixation along the frontozygomatic suture. Postoperatively, repositioning of aesthetic and stability and also bone ends approximation were assessed clinically and radiologically. The patients were followed up for 6 to 49 months. Preoperative symptoms were subsided except the infraorbital sensitivity disturbances in one of the patients. Postoperative complications such as diplopia and hematoma were minimal and subsided by time. In this study, orbitozygomatic, commuted, and zygomatic bone fractures simultaneously with diplopia were excluded. We showed that treatment of an isolated zygomatic bone fracture according to aesthetic and functional requirements may be achieved by insertion of a single miniplate at the lateral rim of the orbit.
Ballin, Carlos R; Sava, Luiz C; Maeda, Carlos A S; Nogueira, Gustavo F; Jebahi, Yasser; Sava, Henrique W; Koladicz, Karyn R J
We present the experience of the Ear, Nose, and Throat Department of Santa Casa de Misericórdia de Curitiba and Hospital Universitário Cajuru PUC-PR in the transnasal endoscopic approach to medial orbital blowout fractures using nasal septum grafts. Seventeen patients have undergone endoscopic repair since June 2005, and septum grafts were used to maintain the orbital contents in position. All 17 patients were treated with this method. Two patients had diplopia on immediate postoperative evaluation. This symptom was corrected with orthoptic exercises in one patient, and the other had a little residual diplopia. Postoperative computed tomography scans showed anatomic reduction in 14 of 17 cases. There were no complications in these surgeries. The transnasal endoscopic approach is a reasonable method for the treatment of medial orbital blowout fractures. Use of septum graft is another surgical alternative for this technique.
Rollnik, J D; Requadt, H
Ocular myositis is a rare disease characterized by painful diplopia but loss of vision rarely occurs. The article reviews the literature focusing on the differential diagnostics. We report the case of an 80-year-old women suffering from slowly progressive loss of vision in the left eye. Diplopia was only present at the beginning and there was only moderate pain. Computed tomography and magnetic resonance imaging revealed a swelling of the left medial, lateral and inferior rectus muscles of the orbit leading to compression of the optic nerve in the orbital cone. An intravenous prednisolone stoss therapy (1000 mg per day for 3 consecutive days) was initiated, followed by oral medication of 100 mg per day then tapering over 10 weeks. Vision improved and no relapses were observed. Physicians should be aware of this rare disease to ensure quick diagnosis and treatment of ocular myositis.
Schneck, Michael; Simionescu, Monica; Bijari, Armita
A 34-year-old woman presented with a 5-month history of persistent vertigo after multiple roller coaster rides, followed by neck pain for 1 month and then 2 weeks of blurred vision related to diplopia. She was ultimately found to have bilateral cervical vertebral artery dissection. The images are described and the literature is reviewed regarding late diagnosis of vertebral dissection and prior cases of roller coaster-associated dissection.
Devastating complications can occur following an uncomplicated resection of a fronto-ethmoid mucocele, namely, total blepharoptosis, diplopia, and vertical-horizontal extraocular muscle abnormalities. Four stages of reconstructive surgery were required over a two-year period of time to arrive at an acceptable appearance and useful binocular vision. Maximum care must be exercised when resecting a fronto-ethmoid mucocele. Inadvertent trauma to the orbital anatomy may result in a multitude of serious ocular complications.
Baxstrom, Curtis R
A 14-year-old girl with diplopia and esotropia secondary to Arnold-Chiari I malformation was surgically treated with Arnold-Chiari I malformation decompression (suboccipital craniectomy), C1 and partial C2 laminectomy, and duraplasty. The residual esotropia was treated with compensatory prisms and vision therapy more than 1 year after Arnold-Chiari malformation surgery. The esotropia was resolved after approximately 3.5 months of treatment. Five years later, the patient continued to maintain fusion without compensatory prism.
Brandy-García, Anahy M; Caminal-Montero, Luis; Fernández-García, María Soledad; Saiz Ayala, Angel; Cabezas-Rodríguez, Ivan; Morante-Bolado, Isla
A 65 year-old female with a history of sarcoidosis with pulmonary and joint involvement, who after 5 years of diagnosis begins with central nervous system involvement manifesting as diplopia. She presents normal analysis results. In imaging results, a mass is identified in the right intraconal space; it depends of right optic nerve, and shows multiple lymph node involvement. Biopsy was performed diagnosed with large B-cell lymphoma, an atypical form of tumor associated with sarcoidosis.
Rhee, Seung-Hyun; Kim, Tae-Seup; Song, Jae-Min; Shin, Sang-Hoon; Lee, Jae-Yeol
Purpose: This study assessed the association between eye symptoms (enophthalmos or diplopia) and site of damage, volume, deviated inferior rectus muscle (IRM) and type of fracture with computed tomography (CT). The intent is to anticipate the prognosis of orbital trauma at initial diagnosis. Methods: Forty-five patients were diagnosed with fractures of the inferior wall of one orbit. Fracture area, volume of displaced tissue, deviated IRM, and type of fracture were evaluated from coronal CT by one investigator. The association of those variables with the occurrence of eye symptoms (diplopia and enophthalmos) was assessed. Results: Of 45 patients, 27 were symptom-free (Group A) and 18 had symptoms (Group B) of enophthalmos and/or diplopia. In Group B, 12 had diplopia, one was enophthalmos, and five had both. By CT measurement, group A mean area was 192.6 mm2 and the mean volume was 673.2 mm3. Group B area was 316.2 mm2 and volume was 1,710.6 mm3. The volume was more influential on symptom occurrence. Each patient was categorized into four grades depending on the location of IRM. Symptom occurrence and higher grade were associated. Twenty-six patients had trap-door fracture (one side, attached to the fracture), and 19 had punched-out fracture (both sides detached). The punched-out fracture was more strongly associated with symptoms and had statistically significantly higher area and volume. Conclusion: In orbital trauma, measurement of fracture area and volume, evaluation of the deviated IRM and classification of the fracture type by coronal CT can effectively predict prognosis and surgical indication. PMID:27489846
Cahill, M; Walsh, J; McAleer, A
Cyclic esotropia is a rare form of strabismus in which a convergent squint appears and disappears typically, but not always, in a regular 48-hour cycle. Characteristically, the convergent squint, when present, has a large angle with associated suppression and no binocular function. On normal or "nonsquinting" days, no manifest deviation is detectable (although in some cases there may be an esophoria). Physiologic diplopia is appreciated, whereas fusion and stereopsis are all normal. Amblyopia may occur in up to 20% of cases.
Arce-Salinas, C A; Pérez-Silva, E
This case involved a 75-year-old woman with systemic lupus erythematosus. Two months previously, she had a flare that was treated successfully by increasing the dosages of prednisone and azathioprine. A sudden onset of ocular pain, diplopia, and loss of vision suggestive of optical neuritis or vascular involvement confused the issue, and rhinocerebral zygomycosis was demonstrated later. We review the presentations of this fungal infection in patients with systemic lupus erythematosus with emphasis on its initial features.
Pearlman, Michelle; Kwong, Wilson T
We present the case of a 33-year-old man with acute onset of eye pain and diplopia as the presenting symptoms of rectal cancer with orbital metastasis. Colorectal cancer with orbital metastasis is exceedingly rare with only 7 cases worldwide despite the prevalence of colorectal cancer. The rarity of this presentation may be related to the long path through multiple vascular beds that tumor emboli from the rectum must travel in order to reach the orbit.
traumatic amnesia 3. evidence of seizure activity--eg, loss of contact, falling spells, visual or auditory hallucinations 4. motor incoordination...defined by an accompanying hearing loss and tfnnitus, sometimes with auditory recruitment and diplacusis, which may precede or follow the development...defects or diplopia, implies involvement of the visual apparatus anywhere from the eye to the occipital cortex: flying status, Class B. Hearing
Fujii, Tomoko; Norizuki, Masataro; Kobayashi, Tatsuo; Yamamoto, Makiko; Kishimoto, Mitsumasa
We report a novel case of eosinophilic orbital myositis associated with Churg-Strauss syndrome. A 56-year-old man with a 20-year history of chronic sinusitis and seasonal allergic rhinitis was admitted because of fever, swelling of cheeks and extremities, diplopia, and eosinophilia. With findings from gadolinium-enhanced FST1WI of the orbits and a muscle biopsy of the skeletal muscle, the diagnosis was made. He was treated with oral corticosteroid, and his symptoms rapidly improved.
Papapetrou, Peter D
According to a previously published theory, Socrates was afflicted with temporal lobe epilepsy since his childhood. Plato, Xenophon, and Aristoxenus described Socrates as having exophthalmos, probably diplopia, and some symptoms compatible with hyperthyroidism. Using these data, we theorize that Socrates had Graves' disease. In order to determine a cause of his temporal lobe epilepsy, we speculate that the philosopher also had autoimmune thyroiditis and Hashimoto encephalopathy during his childhood and his epilepsy may have been a sequel to this hypothesized encephalopathy.
Khanal, P.; Shrestha, A. M.; Bhattarai, S.; Sapkota, D.; Sharma, N.; Devkota, U. P.
Cerebral venous sinus thrombosis (CVST) is a rare but serious disorder that is associated with a poor clinical outcome. We report a 35-year-old man who had a severe headache and diplopia while climbing Mount Everest. His MR venography showed right transverse and right sigmoid sinus thrombosis. He improved on anticoagulant and symptomatic measures. Cerebral venous sinus thrombosis at high altitude is discussed. PMID:27872776
Hatt, Sarah R; Leske, David A; Liebermann, Laura; Holmes, Jonathan M
Purpose To evaluate changes in health-related quality of life (HRQOL) in adult strabismus patients classified as surgical failures by standard motor and diplopia criteria. Design Prospective cohort study evaluating outcomes Methods Adults undergoing strabismus surgery in a single clinical practice, with preoperative and 1-year postoperative Adult Strabismus-20 HRQOL questionnaires were included. Motor and diplopia criteria were applied to classify outcomes (success, partial success, or failure). For those classified failure, the medical record of the 1-year examination was reviewed to determine whether the patient reported subjective improvement. We evaluated improvement in HRQOL, defined as exceeding 95% limits of agreement on at least one of the four Adult Strabismus-20 domains. We compared proportions exceeding 95% limits of agreement in those reporting subjective improvement versus those who did not. Results 40 (18%) of 227 patients were classified as failure by motor and diplopia criteria, with 39 of 40 able to exceed Adult Strabismus-20 95% limits of agreement. Overall, 21 (54%) of 39 showed improved HRQOL by exceeding 95% limits of agreement on at least one of the four Adult Strabismus-20 domains (54% vs predicted 10% by chance alone; P<0.0001). 25 (64%) reported subjective improvement, of whom 16 (64%) showed improved HRQOL exceeding 95% limits of agreement.. Conclusions Many apparent surgical failures report subjective improvement, often reflected in improved HRQOL scores. We propose incorporating quantitative HRQOL criteria into the assessment of strabismus surgery outcomes, defining success as either meeting motor and diplopia criteria, or showing improvement in HRQOL beyond test-retest variability. PMID:26747379
Abrams, Michael S
Exercise-induced esotropia is a rare form of strabismus characterized by paroxysms of esotropia and diplopia only during exercise. This report describes a 15-year-old boy who presented with a 1-year history of exercise-induced esotropia. He has been asymptomatic after recession of a single medial rectus muscle. It is hypothesized that the esotropia was attributable to a malfunction in the muscle tension-control circuit and that altering the small signal gain with surgery resulted in improvement.
Chervenkoff, Jordan V; Rajak, Saul N; Selva, Dinesh; Davis, Garry
This case report discusses the case of a 23-year-old male patient who experienced retrobulbar pain, diplopia, proptosis, and mild lower eyelid bruising after consuming 3,4-methylenedioxy-methamphetamine. The symptoms settled over 10 days and vision returned to normal without intervention. The authors discuss the differential diagnosis relevant to the presenting complaints and propose several mechanisms linking 3,4-methylenedioxy-methamphetamine use to spontaneous nontraumatic intraorbital hematoma.
Yousefi, Sayedeh Reyhaneh
Headache, blurring of vision and confusion are neurologic symptoms of preeclampsia. Whereas abducens nerve palsy during pregnancy is an extremely rare condition, we report here a 40-year-old patient with diplopia, blurring of vision and abducens nerve palsy in the 39th week of pregnancy with history of hypertension (HTN). No specific pathology was found. Symptoms of abducens nerve palsy were resolved spontaneously by controlling blood pressure after delivery. PMID:28208948
Higashino, Takuya; Hirabayashi, Shinichi; Eguchi, Tomoaki; Kato, Yuki
In blow-out fractures, some nonoperative cases have a poor outcome, and a method for accurate prognosis is required. To address this need, we retrospectively reviewed blow-out fractures presenting at Teikyo University Hospital between July 2004 and May 2007 and conducted a survey regarding diplopia and enophthalmos for nonoperative cases. Computed tomographic scan findings were divided according to fracture width and the degree of protrusion of the inferior rectus muscle into the maxillary sinus. We had 106 patients presenting with blow-out fractures, and 89 patients had been treated nonoperatively. In medial orbital wall fractures, no patient had diplopia, and 1 patient had enophthalmos after nonoperative treatment. In punched-out orbital floor fractures, all cases had diplopia when the fracture width was less than half the diameter of the globe, and the protrusion of the inferior rectus muscle into the maxillary sinus was half or more of its section. Even if the fracture width was less than half the diameter of the globe, 2 of 3 patients had enophthalmos when the protrusion of the inferior rectus muscle into the maxillary sinus was half or more of its section. Among the linear orbital floor fractures, 1 case required an emergency operation. We suggest a new algorithm for treatment of blow-out fractures based on computed tomographic scan findings that can also contribute to making a prognosis.
Jellema, Hinke Marijke; Braaksma-Besselink, Yvette; Limpens, Jacqueline; von Arx, Georg; Wiersinga, Wilmar M; Mourits, Maarten P
Proposal of success criteria for strabismus surgery for patients with Graves' orbitopathy (GO) based on a systematic review of the literature. We performed a systematic search of OVID MEDLINE, OVID Embase, the Cochrane Central Register of Controlled Trials (CENTRAL) and the publisher subset of PubMed, to identify studies reporting on success criteria of strabismus surgery in GO. In addition, we handsearched several orthoptic journals and proceedings of strabismological congresses. Of the 789 articles retrieved, 42 articles described success criteria for strabismus surgery in GO. Most studies defined success in terms of a subjective diplopia-free field in primary and down gaze. Almost half of the studies used a graded scale (excellent, good, acceptable and failure) to describe the outcome of surgery. Three of the eligible studies described a tool to quantify the field of single vision in detail. Quality of life was not reported as an outcome measure in any of the published studies. In conclusion, success criteria for strabismus surgery in patients with GO are poorly defined and no consensus is available. The lack of standardization hampers comparative studies and thus the search for the best surgical treatment for diplopia in patients with GO. Therefore, we propose strict success criteria including a tool for quantification of remaining diplopia plus a disease-specific quality of life questionnaire (the GO-QoL).
Almog, Yehoshua; Ben-David, Merav; Nemet, Arie Y
Myasthenia gravis may affect any of the six extra-ocular muscles, masquerading as any type of ocular motor pathology. The frequency of involvement of each muscle is not well established in the medical literature. This study was designed to determine whether a specific muscle or combination of muscles tends to be predominantly affected. This retrospective review included 30 patients with a clinical diagnosis of myasthenia gravis who had extra-ocular muscle involvement with diplopia at presentation. The diagnosis was confirmed by at least one of the following tests: Tensilon test, acetylcholine receptor antibodies, thymoma on chest CT scan, or suggestive electromyography. Frequency of involvement of each muscle in this cohort was inferior oblique 19 (63.3%), lateral rectus nine (30%), superior rectus four (13.3%), inferior rectus six (20%), medial rectus four (13.3%), and superior oblique three (10%). The inferior oblique was involved more often than any other muscle (p<0.01). Eighteen (60%) patients had ptosis, six (20%) of whom had bilateral ptosis. Diagnosing myasthenia gravis can be difficult, because the disease may mimic every pupil-sparing pattern of ocular misalignment. In addition diplopia caused by paresis of the inferior oblique muscle is rarely encountered (other than as a part of oculomotor nerve palsy). Hence, when a patient presents with vertical diplopia resulting from an isolated inferior oblique palsy, myasthenic etiology should be highly suspected.
Wan, Kelvin H; Chong, Kelvin K L; Young, Alvin L
Post-traumatic orbital reconstruction remains a surgical challenge and requires careful preoperative planning, sound anatomical knowledge and good intraoperative judgment. Computer-assisted technology has the potential to reduce error and subjectivity in the management of these complex injuries. A systematic review of the literature was conducted to explore the emerging role of computer-assisted technologies in post-traumatic orbital reconstruction, in terms of functional and safety outcomes. We searched for articles comparing computer-assisted procedures with conventional surgery and studied outcomes on diplopia, enophthalmos, or procedure-related complications. Six observational studies with 273 orbits at a mean follow-up of 13 months were included. Three out of 4 studies reported significantly fewer patients with residual diplopia in the computer-assisted group, while only 1 of the 5 studies reported better improvement in enophthalmos in the assisted group. Types and incidence of complications were comparable. Study heterogeneities limiting statistical comparison by meta-analysis will be discussed. This review highlights the scarcity of data on computer-assisted technology in orbital reconstruction. The result suggests that computer-assisted technology may offer potential advantage in treating diplopia while its role remains to be confirmed in enophthalmos. Additional well-designed and powered randomized controlled trials are much needed.
Wan, Kelvin H.; Chong, Kelvin K. L.; Young, Alvin L.
Post-traumatic orbital reconstruction remains a surgical challenge and requires careful preoperative planning, sound anatomical knowledge and good intraoperative judgment. Computer-assisted technology has the potential to reduce error and subjectivity in the management of these complex injuries. A systematic review of the literature was conducted to explore the emerging role of computer-assisted technologies in post-traumatic orbital reconstruction, in terms of functional and safety outcomes. We searched for articles comparing computer-assisted procedures with conventional surgery and studied outcomes on diplopia, enophthalmos, or procedure-related complications. Six observational studies with 273 orbits at a mean follow-up of 13 months were included. Three out of 4 studies reported significantly fewer patients with residual diplopia in the computer-assisted group, while only 1 of the 5 studies reported better improvement in enophthalmos in the assisted group. Types and incidence of complications were comparable. Study heterogeneities limiting statistical comparison by meta-analysis will be discussed. This review highlights the scarcity of data on computer-assisted technology in orbital reconstruction. The result suggests that computer-assisted technology may offer potential advantage in treating diplopia while its role remains to be confirmed in enophthalmos. Additional well-designed and powered randomized controlled trials are much needed. PMID:26643191
Akutsu, Nobuyuki; Hosoda, Kohkichi; Ohta, Kohei; Tanaka, Hirotomo; Taniguchi, Masaaki; Kohmura, Eiji
We report an unusual case of subarachnoid hemorrhage caused by intraoperative rupture of an intracavernous carotid artery aneurysm coexisting with a prolactinoma. A 58-year-old man presenting with diplopia was found to have a left intracavernous carotid artery aneurysm encased by a suprasellar tumor on magnetic resonance imaging. His serum prolactin level was 5036 ng/mL. Proximal ligation of the left internal carotid artery with a superficial temporal artery to middle cerebral artery anastomosis was scheduled. Because the patient's diplopia had deteriorated, we started him on cabergoline at a dose of 0.25 mg once a week. One month after administration of cabergoline, the diplopia was improved to some extent and serum prolactin was decreased to 290 ng/ml. Six weeks after starting the cabergoline, the patient underwent a left frontotemporal craniotomy to treat the aneurysm. When the dura mater was opened, abnormal brain swelling and obvious subarachnoid hemorrhage were observed. Postoperative computed tomography demonstrated a thick subarachnoid hemorrhage. This case suggests that medical therapy for a pituitary adenoma should be started after treatment for a coexisting intracavernous aneurysm is completed. PMID:25083394
Akutsu, Nobuyuki; Hosoda, Kohkichi; Ohta, Kohei; Tanaka, Hirotomo; Taniguchi, Masaaki; Kohmura, Eiji
We report an unusual case of subarachnoid hemorrhage caused by intraoperative rupture of an intracavernous carotid artery aneurysm coexisting with a prolactinoma. A 58-year-old man presenting with diplopia was found to have a left intracavernous carotid artery aneurysm encased by a suprasellar tumor on magnetic resonance imaging. His serum prolactin level was 5036 ng/mL. Proximal ligation of the left internal carotid artery with a superficial temporal artery to middle cerebral artery anastomosis was scheduled. Because the patient's diplopia had deteriorated, we started him on cabergoline at a dose of 0.25 mg once a week. One month after administration of cabergoline, the diplopia was improved to some extent and serum prolactin was decreased to 290 ng/ml. Six weeks after starting the cabergoline, the patient underwent a left frontotemporal craniotomy to treat the aneurysm. When the dura mater was opened, abnormal brain swelling and obvious subarachnoid hemorrhage were observed. Postoperative computed tomography demonstrated a thick subarachnoid hemorrhage. This case suggests that medical therapy for a pituitary adenoma should be started after treatment for a coexisting intracavernous aneurysm is completed.
Yang, Yi; Su, Yi; Guo, Yi; Ding, Yao; Xu, Sha; Jiang, Yan; Wang, Shuang; Ding, Meiping
Paroxysmal kinesigenic dyskinesia (PKD) is an uncommon neurological disorder, consisting of brief attacks of involuntary movements triggered by sudden action. Patients with PKD generally respond positively to antiepileptic drugs. We compared the efficacy and tolerability of oxcarbazepine (OXC) and carbamazepine (CBZ) in the treatment of PKD, in order to find the optimal prescription. This retrospective study reviewed monotherapy use of CBZ or OXC in 28 patients with PKD during 2005-2011, dividing into two groups. The frequency and severity of attacks and adverse events were recorded. Ten patients in the OXC group and 12 in the CBZ group continued the therapy for more than 12 months. The 12-month retention rate was 76.92% and 80.00%. Both groups showed a marked reduction in attack frequency and the degree of reduction did not differ significantly between the groups. Side effects in patients with OXC included headache, diplopia, and elevated hepatic enzymes, while diplopia, nausea, and leukopenia were recorded in CBZ group. Another three cases were found with better tolerance when converted to OXC from CBZ for rash, drowsiness, diplopia, and nervousness. In conclusion, OXC and CBZ are similarly effective and tolerated in the treatment of PKD, however, more evidence from larger and blind prospective trials are needed.
Pawlak-Adamska, Edyta; Nowak, Oskar; Karabon, Lidia; Pokryszko-Dragan, Anna; Partyka, Anna; Tomkiewicz, Anna; Ptaszkowski, Jakub; Frydecka, Irena; Podemski, Ryszard; Dybko, Jaroslaw; Bilinska, Malgorzata
Multiple sclerosis (MS) is an inflammatory disease of the central nervous system (CNS), where inflammation, demyelination together with the axonopathy are the cardinal features on pathologic ground, with a combined genetic and environmental background. The associations of PD-1 single nucleotide polymorphisms (SNPs): PD-1.3 (in intron 4), PD-1.5 and PD-1.9 (both in exon 5) with clinical manifestation of MS in 479 south Polish individuals including 203 MS patients were analyzed. Presence of PD-1.5T allele was linked with the first manifestations of disease: diplopia and pyramidal signs - favored pyramidal signs but protected against of diplopia development. Farther, PD-1.3G/PD-1.5C/PD-1.9C haplotype significantly favored whereas GTC protected against diplopia. Besides, GTT haplotype strongly favored non-severe RRMS outcome and ATC haplotype was specific only for these MS patients. Our population-based case-control study, investigating selected three PD-1 SNPs: PD-1.3, PD-1.5 and PD-1.9, revealed that polymorphic variation may be rather disease-modifying than MS risk factor.
Tang, Yuan-Zhang; Wu, Bai-Shan; Yang, Li-Qiang; Yue, Jian-Ning; He, Liang-Liang; Li, Na; Ni, Jia-Xiang
Abstract To evaluate the efficacy of computed tomography (CT) guided single radiofrequency thermocoagualtion (RFT) in 1137 patients with idiopathic trigeminal neuralgia after a follow-up period of 11 years, specially focused on duration of pain relief in different branches of trigeminal nerve, side effect, and complications. Retrospective study of patients with idiopathic trigeminal neuralgia treated with a single CT guided RFT procedure between January 2002 and December 2013. The mean follow-up time was 46.14 ± 30.91 months. Immediate postprocedure pain relief was 98.4%. V2 division obtained the best pain relief rate: 91%, 89%, 80%, 72%, 60%, and 54% at 1, 3, 5, 7, 9, and 11 years, respectively. No statistical difference pairwise comparison was in other groups. The complications included masseter muscle weakness, corneitis, diplopia, ptosis, hearing loss, limited mouth opening, and low pressure headache. Masticatory weakness mostly occurred in patients with V3 branch involvement, while Corneitis and Diplopia all in patients with V1 branch involvement. No mortalities observed during or after RFT. All different branches division of trigeminal neuralgia achieved comparable satisfactory curative effect; V2 obtained the best excellent pain relief, after RFT procedure. Facial numbness is inevitable after RFT, which patients who have pain in all 3 trigeminal divisions and patients who desire no facial numbness should be cautious. Masticatory weakness is mainly related with V3 injured, while Corneitis and Diplopia in patients with V1 injured by RFT. PMID:26559288
Paridaens, D.; Verhoeff, K.; Bouwens, D.; van den Bosch, W. A
AIMS—A modified surgical technique is described to perform a one, two, or three wall orbital decompression in patients with Graves' ophthalmopathy. METHODS—The lateral wall was approached ab interno through a "swinging eyelid" approach (lateral canthotomy and lower fornix incision) and an extended periosteum incision along the inferior and lateral orbital margin. In addition, the orbital floor and medial wall were removed when indicated. To minimise the incidence of iatrogenic diplopia, the lateral and medial walls were used as the first surfaces of decompression, leaving the "medial orbital strut" intact. During 1998, this technique was used in a consecutive series of 19 patients (35 orbits) with compressive optic neuropathy (six patients), severe exposure keratopathy (one patient), or disfiguring/congestive Graves' ophthalmopathy (12 patients). RESULTS—The preoperative Hertel value (35 eyes) was on average 25 mm (range 19-31 mm). The mean proptosis reduction at 2 months after surgery was 5.5 mm (range 3-7 mm). Of the total group of 19 patients, iatrogenic diplopia occurred in two (12.5%) of 16 patients who had no preoperative diplopia or only when tired. The three other patients with continuous preoperative diplopia showed no improvement of double vision after orbital decompression, even when the ocular motility (ductions) had improved. In the total group, there was no significant change of ductions in any direction at 2 months after surgery. All six patients with recent onset compressive optic neuropathy showed improvement of visual acuity after surgery. No visual deterioration related to surgery was observed in this study. A high satisfaction score (mean 8.2 on a scale of 1 to 10) was noted following the operation. CONCLUSION—This versatile procedure is safe and efficacious, patient and cost friendly. Advantages are the low incidence of induced diplopia and periorbital hypaesthesia, the hidden and small incision, the minimal surgical
Canneti, Beatrice; Mosqueira, Antonio J; Gilo, Francisco; Carreras, Teresa; Barbosa, Antonio; Meca-Lallana, Virginia; Vivancos, José
Introduccion. El sindrome CLIPPERS (chronic lymphocytic in?ammation with pontine perivascular enhancement responsive to steroids) es un proceso inflamatorio del sistema nervioso central cuyo rasgo distintivo son las lesiones puntiformes en el troncoencefalo captantes en los estudios de resonancia magnetica. Clinicamente, cursa con disartria, ataxia y diplopia, y suele responder a corticoides. Anatomopatologicamente, aparecen infiltrados de linfocitos T en los espacios perivasculares troncoencefalicos. Caso clinico. Mujer de 40 años con cuadro de instauracion subaguda de diplopia binocular, ataxia y disartria. En la resonancia magnetica cerebral presento lesiones puntiformes hipertintensas en secuencia T2 en el tronco, cerebelo, diencefalo y areas cortico-subcorticales bihemisfericas, que realzaron con contraste. Se realizo un estudio etiologico para descartar un origen infeccioso, neoplasico o inflamatorio subyacente, que resulto negativo. La paciente recibio tratamiento en dos ocasiones con metilprednisolona, con descenso progresivo de la dosis, con buena respuesta. Conclusiones. La diplopia y la ataxia, como en nuestro caso, estan presentes practicamente siempre. Los hallazgos en la RM consisten en lesiones captantes puntiformes localizadas en la protuberancia con extension hacia el cerebelo, ganglios basales y cuerpo calloso, con gradiente de captacion menor conforme se alejan rostralmente hacia la corteza, y caudalmente hacia la medula. En el caso de nuestra paciente, este gradiente no se respeta, encontrandose una densidad similar de las lesiones a nivel supratentorial. El diagnostico diferencial es amplio y justifica un estudio diagnostico extenso, y en casos seleccionados la biopsia cerebral. El curso de la enfermedad es remitente-recurrente, y el pronostico mejora cuanto mas precoz y prolongado es el tiempo de corticoterapia.
Siatkowski, R. Michael
Purpose To describe the clinical features and response to treatment of patients with decompensated monofixation syndrome (MFS) and to propose a hypothesis for a decompensation mechanism in such patients. Methods Fourteen adults with MFS who had been symptomatically stable for a mean duration of 25 years developed diplopia in the absence of neurologic or orbital disease. After retrospective chart review, they underwent detailed orthoptic testing. Results from this cross-sectional analysis were compared with similar data from 16 control subjects with stable MFS. Results Compared to stable MFS patients, decompensated subjects had significantly poorer horizontal fusional amplitudes but greater torsional fusional amplitudes; they were also more likely to have a small vertical strabismus and to have received initial treatment later. Stable subjects, however, also had subnormal horizontal as well as torsional fusional amplitudes. There was no difference between groups with respect to refractive error, amblyopia, type or prior treatment of strabismus, stereoacuity, or angle of deviation. After treatment, all patients regained monofixational alignment, but up to one-third had continued diplopia. Symptoms recurred in two patients whose treatment was initially successful. Conclusions Patients with MFS lose fusional amplitudes over time. In some cases this results in development of sensory torsion with secondary decompensation and diplopia. The rate of decompensation averages 7% per year from ages 20 to 70. Treatment for decompensation offers excellent motor results, but sensory symptoms may persist and recurrent symptoms may develop. Monitoring and maintenance of fusional vergence amplitudes should be part of the routine care for patients with MFS. PMID:22253490
Menconi, Francesca; Leo, Marenza; Sabini, Elena; Mautone, Teresa; Nardi, Marco; Sainato, Aldo; Sellari-Franceschini, Stefano; Vitti, Paolo; Marcocci, Claudio; Marinò, Michele
Intravenous glucocorticoids are used for Graves' orbitopathy, alone or associated with/followed by additional treatments (orbital radiotherapy, orbital decompression, palpebral or eye surgery). However, the relation between associated/additional treatments and other variables with Graves' orbitopathy outcome following intravenous glucocorticoids is not clear. Thus, the present study was conducted to investigate retrospectively the impact of associated/additional treatments and other variables on Graves' orbitopathy outcome after intravenous glucocorticoids. We evaluated 226 untreated Graves' orbitopathy patients. Following first observation, patients were given intravenous glucocorticoids and re-examined after a median of 46.5 months. The end-points were the relation between Graves' orbitopathy outcome, outcome of NOSPECS score and of the single Graves' orbitopathy features with several variables, including associated/additional treatments. All Graves' orbitopathy features improved significantly after treatment. Overall, Graves' orbitopathy improved in ~60 % of patients (responders), whereas it was stable or worsened in ~40 % of patients (non-responders). Time between first and last observation and clinical activity score at first observation correlated significantly with Graves' orbitopathy outcome. The outcomes of NOSPECS, eyelid aperture, clinical activity score and diplopia correlated with time between the first and last observation. The NOSPECS outcome correlated with gender. The outcomes of proptosis, eyelid aperture and visual acuity correlated with orbital decompression. The outcome of diplopia correlated with orbital radiotherapy. Taking into account the limitations of retrospective investigations, our findings confirm that time (i.e. the natural history of Graves' orbitopathy) is a key factor in determining the long-term outcome of Graves' orbitopathy, radiotherapy is effective for diplopia, and orbital decompression is followed by an amelioration of
Kung, Nathan H; Bucelli, Robert C; McClelland, Collin M; Van Stavern, Gregory P
Ocular neuromyotonia (ONM) is a neuro-ophthalmic disorder characterized by episodic diplopia caused by contraction of one or more ocular muscles due to spontaneous excitation of the respective ocular motor nerve. We report a patient whose ocular neuromyotonia arose in the setting of a subacute demyelinating polyneuropathy consistent with chronic inflammatory demyelinating polyneuropathy (CIDP) and subsequently resolved following the initiation of intravenous immunoglobulin (IVIg) for her neuropathy. Our patient provides additional evidence towards the role of demyelination and ephaptic neurotransmission in ocular neuromyotonia and also represents the first reported case of ocular neuromyotonia associated with a systemic neurological condition.
ALBU, CARMEN VALERIA; CĂTĂLIN, B.; ZAHARIA, CORNELIA
We report the case of a 43 year old man who presented recurrent left abducens palsy. His medical history included arterial hypertension, ischemic cardiomiopathy, dyslipidemia, rhinitis, maxillary sinusitis. Physical examination revealed a overweight patient, horizontal gaze diplopia, left nerve VI paresis, mild left retro-orbital pain. The orbital MRI also didn’t offer new information: mild external edema on the left eye, with normal tendon aspect, no thickening or enhancement of the muscle belly and also normal aspect of the bony orbit. Recurrent palsy of EOMs can be caused in rare cases by ocular myositis. PMID:24791211
Ross, Jonathan J; Brown, Valerie; Fern, Alasdair I
Heroin (diamorphine) is a highly addictive opiate with potential for misuse. A small number of reports have linked the commencement of heroin misuse to acute exotropia with diplopia and subsequent withdrawal to esotropia in individuals without previous symptoms.(1-5) We describe a young adult who sought strabismus surgery to correct a large-angle exotropia. Detailed patient history and orthoptic measurements at different times of the day revealed a fluctuating angle of divergence relating to the timing of opiate ingestion, rendering surgery inappropriate. We suggest that opiate misuse, which may not willingly be disclosed by patients, should be specifically asked about before acquired-strabismus surgery is undertaken in adults.
Adachi, J; Uki, J; Kazumoto, K; Takeda, F
A 52-year-old male presented with a brainstem abscess manifesting as high fever, diplopia, and left hemiparesis. Magnetic resonance (MR) imaging with gadolinium diethylenetriamine pentaacetic acid showed the lesion as a ring-like enhanced mass consisting of a necrotic center with surrounding edema, whereas postcontrast computed tomography revealed no such confirmatory findings. He was treated with antibiotics as the lesion had been detected in the acute cerebritis stage. Serial MR images showed that the lesion decreased remarkably in size. MR imaging can detect brain abscess in the earliest inflammatory stage.
Barker, R A; Anderson, J R; Meyer, P; Dick, D J; Scolding, N J
A 50 year old woman presented with a subacute onset of vertigo and diplopia followed by an encephalopathy with confusion, spasticity, ataxia, myoclonus, and multiple branch retinal arteriolar occlusions and unilateral sensorineural deafness. Brain biopsy confirmed multiple microinfarcts with no vasculitis. After the procedure she had a right iliofemoral deep vein thrombosis and was found to be heterozygous for the factor V Leiden mutation. She was treated with anticoagulants and made a marked recovery with no relapses 6 months after presentation. This case extends the age range at which Susac's syndrome can present, and raises the possibility that the condition may be associated with abnormalities of coagulation.
Apostolopoulos, Kostas; Ferekidis, Eleftherios
We describe a rare case of an extensive primary cranial Ewing's sarcoma located in the greater wing of the sphenoid bone with extension to the orbit, the endocranium, the parapharyngeal and infratemporal space. The patient presented with diplopia, anosmia and prolapse of the left eye. He was given chemo- and radiotherapy and was free of symptoms on re-examination 1.5 years later. The prognosis of Ewing's sarcoma in the absence of surgery is uncertain, but prompt treatment appears to have a satisfactory therapeutic outcome. In the future, more cases should be studied in order to investigate the biological behaviour of a primary cranial Ewing's sarcoma.
Inaka, Yasufumi; Otani, Naoki; Nishida, Sho; Kumagai, Kohsuke; Fujii, Kazuya; Ueno, Hideaki; Tomiyama, Arata; Tomura, Satoshi; Osada, Hideo; Wada, Kojiro; Mori, Kentaro
A primary intraosseous cavernous hemangioma located at the sphenoid bone with extensive involvement of the orbital roof and the lateral wall of the orbit is very rare. A 48-year-old woman presented with progressive right exophthalmos and diplopia. CT showed a bony mass lesion in the right sphenoid bone extending to the orbital bone. MRI showed an abnormal lesion in the sphenoid bone, which was heterogeneously enhanced with gadolinium. All of the abnormal bone was surgically removed, and histological examination confirmed a cavernous angioma. We also present a brief clinical and radiological review of seven previously reported cases.
Venkatesh, Pradeep; Gogia, Varun; Gupta, Shikha; Nayak, Bhagabat
A patient with a history of vitreoretinal surgery presented with nasal dystopia, diplopia and epibulbar bluish black mass simulating a chocolate cyst in the right eye. After a non-conclusive ocular examination, he underwent CT of the orbit along with volume rendition and three-dimensional reconstruction, which demonstrated intact globe with laterally displaced band-buckle assembly along with peri-scleral buckle element (SBE) soft tissue proliferation. Imaging-assisted exploration of the lesion was performed and retained scleral buckle element (SBE) was removed in toto; thus relieving the patient long-standing dystopia.
Kumazawa, Sachiko; Ishibashi, Hironori; Takahashi, Ken; Okubo, Kenichi
Myasthenia gravis is the most common disease associated with thymoma, but it is rarely accompanied by ectopic thymoma. We describe a 47-year-old woman who presented with an ectopic cervical thymoma with myasthenia gravis. She was admitted to our neurology department with ptosis, diplopia, and mandibular muscle fatigue, and was diagnosed with myasthenia gravis. The mass was located posterior to the right lobe of thyroid gland on computed tomography and was diagnosed as ectopic thymoma on fine-needle aspiration biopsy examination. Transcervical excision of thymoma and VATET were performed. The patient has been free of neurological symptoms and has displayed no evidence of recurrent thymoma for 2 years.
Bruhnding, Aubree; Notch, Derek; Beard, Albertine
Paraneoplastic neurological syndromes are a rare complication of malignancy. Subacute cerebellar ataxia, or paraneoplastic cerebellar degeneration, usually presents in women with a subcate onset of gait instability, followed by progressive limb and trunk ataxia, dysarthria, diplopia, and dysphagia that occurs in the setting of, or precedes the diagnosis of, a gynecologic or breast malignancy and clinically stabilizes within six months. The most common autoantibody associated with PCD is purkinje cell cytoplasmic antibody type 1, also known as anti-Yo. Here we describe the first reported case of a man with anti-Yo positive paraneoplastic cerebellar degeneration in the setting of occult cholangiocarcinoma.
Duane, T D; Schatz, N J; Caputo, A R
Five patients presented with signs that were similar to but opposite from Duane's retraction syndrome. Most had a history of orbital trauma. On attempted abduction a narrowing of the palpebral fissure and retraction of the globe was observed. Diplopia with lateral gaze was present. Roentgenograms (polytomograms) showed involvement of the medial orbital wall. Forced ductuin tests were positive. Surgical repair of the fracture and release of the entrapped muscle as determined by forced duction tests and by postoperative motility led to successful results. Images FIGURE 1 A FIGURE 1 B FIGURE 2 FIGURE 3 PMID:867622
An early diagnosis of strabismus is important in order to rule out treatable organic causes and in children, if indicated, to start as early as possible with an amblyopia treatment. Early detection will also decrease the risk for accidents secondary to diplopia, to the loss of binocular vision and to the restriction of the binocular visual field in case of esodeviations. The following therapeutic options exist: in some cases the prescription of the correct refraction will be sufficient, for small deviations a prismatic correction may allow a longstanding treatment, for larger or incomitant deviations strabismus surgery will be necessary, which nowadays can be performed using minimal-invasive technique on an outpatient base.
Lascaratos, Gerassimos; McHugh, James; McCarthy, Karon; Bunting, Howard
We report a case of bilateral infero-medial orbital wall destruction, associated with loss of sinonasal architecture. The patient presented with intermittent horizontal diplopia following an acute on chronic infective sinusitis. Eight months previously the patient had developed a midline hard palate fistula for which a palatine prosthesis had been fitted. The broad differential diagnosis is discussed, though in this patient chronic cocaine abuse was identified as the underlying aetiology. Eye movement restriction worsened progressively with bilateral inflammation around the medial and inferior rectus muscles. Attempts to resolve the recurring cycle of sinus infection and inflammation by palatal fistula closure failed despite augmented techniques mobilising flaps from both nasal and palatal sides.
Phang, See Yung; Whitfield, Peter
Meningiomas are known to be more common in females than males. They are also known in rare cases to grow in size during pregnancy, which can complicate its management. We describe a 31-year-old Caucasian woman who presented with blurring of her vision and diplopia during the third trimester of her pregnancy. Magnetic resonance imaging (MRI) showed a small left cavernous sinus meningioma. The patient was treated conservatively until her uncomplicated delivery. A postpartum MRI scan showed complete regression of the suspected meningioma. Currently the patient is contemplating a further pregnancy. PMID:27066285
Abkur, Tarig M; Kearney, Hugh; Hennessy, Michael J
Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS) is a rare chronic inflammatory disorder of the central nervous system. Herein, we describe the case of a 62-year-old female who presented with right sided facial tingling, gait ataxia and diplopia. Neuroimaging revealed pontine curvilinear enhancing lesions with extension into cerebellar peduncles, characteristic of CLIPPERS. This report discusses the differential diagnosis and the importance of prolonged immunomodulatory treatment for this rare neuro-inflammatory disorder. Long-term immunosuppression appears to be mandatory in order to achieve sustained remission and prevent disability related to atrophy of the structures involved in repeated attacks.
Humayun, Hassan Nabeel; Akhtar, Shabbir; Ahmed, Shakeel
Otits media is a common problem. Some of its complications that were seen frequently in the preantibiotic era are rare today. We report a case of an 8 year boy who presented with earache, retro-orbital pain and diplopia secondary to a sixth nerve palsy--Gradenigo's syndrome. In this syndrome infection from the middle ear spreads medially to the petrous apex of the temporal bone. Work-up includes CT scan of the temporal bones. Timely management with intravenous antibiotics (+ surgery) is needed to prevent intra-cranial complications.
Berman, E L
Ocular signs and symptoms provide clinical clues to many of the more common metabolic and nutritional disorders seen in older adults. Diabetes mellitus can affect all parts of the eye and orbit. Complications include refractive visual loss, macular edema, retinopathy, increased risk of fungal infection, and diplopia. In patients with gout, urate crystals may precipitate in the eye and cause conjunctivitis, uveitis, or scleritis. Other problems are seen with Wilson's disease, hyperlipidemia, and albinism. Nutritional disorders usually arise from malabsorption, gastrointestinal surgery, and alcohol abuse. Deficiencies in vitamins A, B1 (thiamine), B12, and C may be manifest in the eye.
Sekiguchi, Kenji; Yasui, Naoko; Kowa, Hisatomo; Kanda, Fumio; Toda, Tatsushi
We herein present two cases of acute disseminated encephalomyelitis (ADEM) following vaccination against human papilloma virus (HPV). Case 1 experienced diplopia and developed an unstable gait 14 days after a second vaccination of Cervarix. Brain magnetic resonance imaging (MRI) showed an isolated small, demyelinating lesion in the pontine tegmentum. Case 2 experienced a fever and limb dysesthesia 16 days after a second vaccination of Gardasil. Brain MRI revealed hyperintense lesion in the pons with slight edema on a T2-weighted image. Both cases resolved completely. It is important to accumulate further data on confirmed cases of ADEM temporally associated with HPV vaccination.
Lee, Shin-heon; Park, Yong-sook; Kwon, Jeong-taik
A 43-year-old man was admitted for head trauma after falling backward. The patient complained of diplopia. Unilateral internuclear ophthalmoplegia (INO) was diagnosed during the neurologic examination. Initially, no specific finding was shown on T2-weighted, T1-weighted, and fluid attenuated inversion recovery brain magnetic resonance image (MRI) or brain computed tomography (CT). However, susceptibility-weighted imaging (SWI) definitively demonstrated a tiny hemorrhage at the midline of the pontomesencephalic junction. The patient's symptom improved after 12 weeks. We discuss the clinical significance of SWI when traumatic INO due to a tiny hemorrhage is suspected. PMID:27857923
Basiri, Keivan; Mohaghegh, Mohammad Reza; Teimouri, Somayyeh Sadat; Okhovat, Ali Asghar
Acute onset bulbar symptoms with respiratory failure and descending paralysis may occur in several neuromuscular disorders including variants of Guillain-Barre syndrome (GBS), diphtheria, botulism and toxins. We present a 51-year-old man who presented with complains of ptosis and dyspnea following pyrethroids spraying in an enclosed area for eradication of flea. Within 5-6 days of admission limb weakness, dysphagia, dysarthria, blurred vision, diplopia, tremor and respiratory distress added to previous symptoms. Temporal profile of events after exposure, development of similar symptoms in patient's son, electrodiagnostic findings and exclusion of other etiologies confirms intoxication etiology. We reviewed the literature and provide an extensive electrodiagnostic overview. PMID:27099845
Choi, Jae-Hwan; Cho, Jae-Wook; Lee, Jae-Hyeok; Lee, Sang Weon; Kim, Hak-Jin; Choi, Kwang-Dong
A 46-year-old man developed intermittent headache, diplopia, and visual obscuration for two months. Funduscopic examination showed optic disk swelling in both eyes. Brain MRI exhibited hydrocephalus and leptomeningeal enhancement at the prepontine cistern, left cerebellopontine angle cistern and bilateral cerebral hemisphere, and hemosiderin deposition along the cerebellar folia. CSF analysis revealed an elevated opening pressure with xanthochromic appearance and small amount of red blood cells. Antibody titer against Toxocariasis using ELISA was elevated both in blood and CSF. Obstructive hydrocephalus and hemosiderin deposition in this case may result from the active inflammatory process due to CNS toxocariasis within the subarachnoid space.
Ji, So Young; Yoo, Jae Hong; Ha, Won; Lee, Ji Won
Brown syndrome is known as limited elevation of the affected eye during adduction. It is caused by a disorder of the superior oblique tendon, which makes it difficult for the eyeball to look upward, especially during adduction. It is classified into congenital true sheath Brown syndrome and acquired simulated Brown syndrome. Acquired simulated Brown syndrome can be caused by trauma, infection, or inflammatory conditions. The surgical restoration of blowout fractures can also lead to limitations of ocular motility, including Brown syndrome. We report on three patients with acquired simulated Brown syndrome, who complained of diplopia and limitation of ocular motility after operations to treat blowout fractures. PMID:26015892
Lee, Sung Hee (Kelly); Jun, Weon; London, Richard
ABSTRACT Acquired involuntary eye movement disorders, including noncomitant strabismus, nystagmus, and saccadic dyskinesia, are common ocular manifestations of many neurodegenerative diseases. These patients may experience visual symptoms, such as blurred vision, diplopia, and oscillopsia, which can significantly impact their use of vision. The goal of the management for these patients is to reduce the visual symptoms using any combination of available management strategies. This case report discusses the effective optical management using the combination of spectacle monovision correction and yoked prism to improve visual symptoms in a patient with olivopontocerebellar atrophy. PMID:27928348
Hillman, J S; Hawkswell, A
This paper reports a study of postoperative refraction and eikonometry of 50 patients who had unilateral cataract extraction with implantation of a pupil-supported intraocular lens. The mean postoperative aniseikonia (+/- SD) was 1.97 (+/- 1.82). A statistically significant relationship was shown between anisometropia and aniseikonia. Despite aniseikonia of up to 7.8 per cent there were no diplopia problems as the visual system exhibits a high degree of tolerance. It is concluded that aniseikonia can be controlled to within clinically acceptable limits by the simple calculation of intraocular lens power for isometropia.
Alafaci, Concetta; Grasso, Giovanni; Granata, Francesca; Marino, Daniele; Salpietro, Francesco M.; Tomasello, Francesco
Background: The most common etiology of classic trigeminal neuralgia (TN) is vascular compression. However, other causes must be considered. Among these, spontaneous hematoma of the Meckel's cave (MC) causing symptomatic TN is very rare. Case Description: We present the case of a 60-year-old woman with a 2-month history of left TN and diplopia. Neuroradiological examinations revealed a well-defined hematoma in the left MC. The patient underwent surgical decompression with a progressive neurological improvement. Conclusion: Despite the number of lesions potentially affecting the MC, spontaneous hemorrhage is rare but should be taken into account in the differential diagnosis. PMID:26539319
Nekitsing, Indima; Wu, Xing; Tang, Guangyu
Central primitive neuroectodermal tumour (cPNET), a rare malignant neoplasm of embryonal origin, often occurs in children younger than 15 years. This is the first case report of the imaging findings of an infratentorial cPNET to be reported in a patient. Here, is reported the case of a 6-year-old boy presenting with symptoms of diplopia for 14 days. Magnetic resonance imaging revealed a solid mass in the fourth ventricle. The postoperative pathological diagnosis was cPNET. To conclude, whenever a child is diagnosed to have an infratentorial solid tumour in the fourth ventricle, cPNET should always be considered despite its rarity.
Marsh, R J; Dulley, B; Kelly, V
Seventy-seven new patients suffering from ophthalmic zoster and a selected group of 69 old patients were carefully examined with regard to external ocular movements. An incidence of 31% of ocular pareses was found in the new patients, and 58 in all were analysed. We were surprised to find several of these were contralateral and bilateral palsies. 28% of the palsies were asymptomatic, due to diplopia being present only in extremes of gaze and the rapid development of suppression in the affected eye. The theories of aetiology of these pareses are discussed. PMID:338027
Kimura, Yoshitaka; Asako, Kurumi; Kikuchi, Hirotoshi; Kono, Hajime
Optic perineuritis is an uncommon inflammatory disorder of the optic sheath that causes visual loss or eye pain. There are few case reports of optic perineuritis associated with granulomatosis with polyangiitis. Herein we report the case of a 37-year-old male with granulomatosis with polyangiitis and who presented with headache, blurred vision in the right eye, diplopia, and numbness in the right forehead. Brain magnetic resonance images (MRI) findings revealed hypertrophic pachymeningitis and refractory optic perineuritis. These were manageable only by means of weekly methotrexate and mycophenolate mofetil combination therapy but not with methotrexate, mycophenolate mofetil, intravenous cyclophosphamide, rituximab, azathioprine, or cyclosporine individually. PMID:28293459
Choi, Seung Woo; Lee, Jae Yeun
Purpose To evaluate the efficacy and safety of customized orbital decompression surgery combined with eyelid surgery or strabismus surgery for mild to moderate thyroid-associated ophthalmopathy (TAO). Methods Twenty-seven consecutive subjects who were treated surgically for proptosis with disfigurement or diplopia after medical therapy from September 2009 to July 2012 were included in the analysis. Customized orbital decompression surgery with correction of eyelid retraction and extraocular movement disorders was simultaneously performed. The patients had a minimum preoperative period of 3 months of stable range of ocular motility and eyelid position. All patients had inactive TAO and were euthyroid at the time of operation. Preoperative and postoperative examinations, including vision, margin reflex distance, Hertel exophthalmometry, ocular motility, visual fields, Goldmann perimetry, and subject assessment of the procedure, were performed in all patients. Data were analyzed using paired t-test (PASW Statistics ver. 18.0). Results Forty-nine decompressions were performed on 27 subjects (16 females, 11 males; mean age, 36.6 ± 11.6 years). Twenty-two patients underwent bilateral operations; five required only unilateral orbital decompression. An average proptosis of 15.6 ± 2.2 mm (p = 0.00) was achieved, with a mean preoperative Hertel measurement of 17.6 ± 2.2 mm. Ocular motility was corrected through recession of the extraocular muscle in three cases, and no new-onset diplopia or aggravated diplopia was noted. The binocular single vision field increased in all patients. Eyelid retraction correction surgery was simultaneously performed in the same surgical session in 10 of 49 cases, and strabismus and eyelid retraction surgery were performed in the same surgical session in two cases. Margin reflex distance decreased from a preoperative average of 4.3 ± 0.8 to 3.8 ± 0.5 mm postoperatively. Conclusions The customized orbital decompression procedure decreased
Kapoor, Akhil; Beniwal, Vimla; Beniwal, Surender; Mathur, Harsh; Kumar, Harvindra Singh
Metastatic lesions to the clivus have been reported in various cancers including lung cancer, prostate carcinoma, skin melanoma, and hepatocellular carcinoma. There have been only a few reports of breast cancer presenting with isolated clival metastasis. We report a case of 35-year-old lady, who was known case of breast carcinoma presented with diplopia as the only sign of clival metastasis. The etiology was established by magnetic resonance imaging which showed an enhancing lesion in the clivus. The diagnosis of clival metastasis from breast cancer was confirmed by transsphenoidal biopsy. PMID:26044482
Koukkoulli, Antigoni; Koutroumanos, Nikolas; Kidd, Desmond
ABSTRACT An 89-year-old female presented with horizontal diplopia and was diagnosed with VI nerve palsy attributed to a microvascular event. She subsequently progressed to develop an orbital apex syndrome, with neuroimaging demonstrating tumour invasion. Eighteen months earlier, she had squamous cell carcinoma of the forehead excised with clear margins. Intraneural and perineural spread of squamous carcinoma from the face to the cranial cavity is an important cause of delayed cranial nerve palsies after local excision of the skin tumour. PMID:27928347
Lin, Jonathan; German, Michael; Wong, Brian
The fractures of the orbital floor are common after craniofacial trauma. Repair with resorbable plates is a viable reconstructive option; however, there are few reports in the literature. This study describes our experience using copolymer polylactic and polyglycolic acid (PLLA/PGA) orbital reconstruction plates (LactoSorb, Lorenz Surgical, Jacksonville, FL) in 29 cases of the orbital floor fracture repair. We conducted a retrospective review of 29 orbital floor fractures at a single institution repaired through transconjunctival, preseptal dissection using PLLA/PGA plates fashioned to repair the orbital floor defect. Associated fractures included zygomaticomaxillary, LeFort, and nasoethmoid fractures. There were six patients with complications. Four patients had transient diplopia with complete resolution of symptoms within 1 year. One patient had diplopia postoperatively, but was later lost to follow-up. Two patients have had persistent enophthalmos since 1 year. In each of these cases, the floor fracture was coincident with significant panfacial or neurotrauma. We did not encounter any adverse inflammatory reactions to the implant material itself. The study concluded that orbital floor fracture repair with resorbable plates is safe, relatively easy to perform, and in the majority of cases was effective without complications. In the presence of severe orbital trauma, more rigid implant materials may be appropriate.
Couceiro, Rita; Proença, Helena; Pinto, Filomena; Fonseca, Ana; Monteiro-Grillo, Manuel
Objective: To report an unusual case of systemic non-Hodgkin lymphoma (NHL) with repeated relapse in the lacrimal glands, in spite of complete remission for several years after treatment. Methods: A 78-year-old male with small lymphocytic B cell NHL, stage IV disease (lung invasion), was submitted to surgery and chemotherapy in 2001, with complete remission of the disease. In 2003 he developed a nodular lesion in the right lacrimal fossa. Pathology results revealed a local relapse of NHL. Radiation and chemotherapy were initiated and complete remission was again achieved. In 2012 the patient developed a new nodular lesion located in the left lacrimal fossa, resulting in diplopia, ptosis and proptosis of the left eye. Orbital computerized tomography (CT), ocular ultrasound and incisional biopsy were performed. Results: Orbital CT revealed a lesion infiltrating the left lacrimal gland and encircling the globe. Biopsy results confirmed a local relapse of B cell NHL. The patient was submitted to local radiation therapy with progressive resolution of ptosis, proptosis and diplopia. Response to treatment was monitored with ocular ultrasound. Conclusions: Patients with NHL diagnosis should be immediately investigated if ophthalmic or orbital symptoms develop. NHL extension to the orbit and adnexa is infrequent (5% of NHL cases) but may occur at any stage of the disease, including as a relapse site. In such cases, radiation and chemotherapy achieve good results, inducing long periods of remission. PMID:27625948
Melo, M C; Revuelta, M E; Santeularia, T; Genové, M; Català, E
Spontaneous intracranial hypotension headache is an uncommon disease that resolves spontaneously in most of the cases and in a short period of time. The initial treatment should be symptomatic. In some patients the symptomatology is extremely disabling, and in these cases both the diagnosis and treatment may be performed by an epidural blood patch. A 49-year-old Caucasian woman, with no previous record of epidural or intrathecal puncture, consulted in the Emergency Department complaining of a 9-day history of frontal headache and diplopia, along with nausea and vomiting. The patient was diagnosed with spontaneous intracranial hypotension headache. Considering the symptomatology and the uncontrolled pain, the Pain Unit of our hospital performed an epidural blood patch. In the first 24h the patient reported a remarkable relief of both headache and diplopia but developed a left lumbar radiculopathy that was treated successfully with supportive measures. Transient lumbar radiculopathy is a common and acceptable event secondary to the use of epidural blood patch as a treatment for spontaneous intracranial hypotension headache.
Jean, Walter C; Anaizi, Amjad; DeKlotz, Timothy R
The endoscopic endonasal transclival approach has been widely described for its use to resect clivus chordomas, but there have only been isolated reports of its use for petroclival meningiomas. These tumors are most often resected utilizing open transpetrosal approaches, but these operations, difficult even in the hands of dedicated skull base surgeons, are particularly challenging if the meningiomas are medially-situated and positioned mainly behind the clivus. For this subset of petroclival meningiomas, a transclival approach may be preferable. We report a meningioma resected via an endoscopic endonasal transclival technique. The patient was a 63-year-old man who presented originally for medical attention because of diplopia related to an abducens palsy on the left. A workup at that time revealed a meningioma contained entirely in the left cavernous sinus, and this was treated with stereotactic radiosurgery. His symptoms resolved and his meningioma was stable on MRI for several years after treatment. The patient was then lost to follow-up until 13 years after radiosurgery when he experienced intermittent diplopia again. At this point, workup revealed a large petroclival meningioma compressing the brainstem. He underwent a successful endoscopic endonasal transclival resection of this tumor. A demonstration of the step-by-step surgical technique, discussion of the nuances of the operation, and a comparison with the open transpetrosal approaches are included in our report. PMID:27433420
Parkash, Vivak; Mudhar, Hardeep Singh; Wagner, Bart E.; Raoult, Didier; Batty, Ruth; Lepidi, Hubert; Burke, John; Collini, Paul; de Silva, Thushan
Purpose To describe the clinical features of a Caucasian female patient with a history of treated gastrointestinal Whipple's disease (WD) who developed new-onset diplopia, with a description of the histopathological features of the extraocular muscle biopsies. Methods A previously fit 38-year-old Caucasian female presented with acute-onset diplopia after being on a sustained medication regime for biopsy-proven gastrointestinal WD. A magnetic resonance imaging scan of her orbits with gadolinium revealed diffuse enhancement of the bellies of the extraocular muscles bilaterally, particularly the medial rectus, superior rectus, and superior oblique muscles, consistent with an infiltrative myositis. She underwent unilateral extraocular muscle biopsies. Results The extraocular muscle biopsies contained macrophages between the muscle fibres. These contained periodic acid-Schiff-positive cytoplasmic granules. Immunohistochemistry with an antibody raised to Tropheryma whipplei showed positive staining of the same macrophages. Transmission electron microscopy confirmed the presence of effete T. whipplei cell membranes in lysosomes. Conclusion This case describes bilateral WD-associated extraocular muscle myositis. The exact mechanism for this unusual presentation is unclear, but both a WD-associated immune reconstitution inflammatory syndrome and treatment failure are possibilities, with a good response observed to antibiotic therapy and adjunctive corticosteroids.
Fichter, Nicole; Guthoff, Rudolf F.
Purpose. To evaluate the effect of en bloc lateral wall decompression with additional orbital fat resection in terms of exophthalmos reduction and complications. Methods. A retrospective, noncomparative case series study from 1999 to 2011 (chart review) in Graves' orbitopathy (GO) patients. The standardized surgical technique involved removal of the lateral orbital wall including the orbital rim via a lid crease approach combined with additional orbital fat resection. Exophthalmos, diplopia, retrobulbar pressure sensation, and complications were analyzed pre- and postoperatively. Results. A total of 111 patients (164 orbits) with follow-up >3 months were analysed. Mean exophthalmos reduction was 3.05mm and preoperative orbital pressure sensation resolved or improved in all patients. Visual acuity improved significantly in patients undergoing surgery for rehabilitative or vision threatening purposes. Preoperative diplopia improved in 10 patients (9.0%) but worsened in 5 patients (4.5%), necessitating surgical correction in 3 patients. There were no significant complications; however, one patient had slight hollowing of the temporalis muscle around the scar that did not necessitate revision, and another patient with a circumscribed retraction of the scar itself underwent surgical correction. Conclusions. The study confirms the efficiency of en bloc lateral wall decompression in GO in a large series of patients, highlighting the low risk of disturbance of binocular functions and of cosmetic blemish in the temporal midface region. PMID:26221142
Hwang, Kun; Kim, Joo Ho
The aims of this study are to report a blowout fracture of the orbital floor and medial wall caused by being struck by a steering wheel knob of an automobile and to discuss the use of airbags and seatbelts as a preventive measure for orbital injuries. A 58-year-old man was struck in the left eye by a steering wheel. His car hit a telephone pole, and he had a frontal collision injury. In this frontal impact, his left eye was hit by a Brodie knob attached to the steering wheel. At the time of injury, the speed of the car was about 65 km/h. He was not wearing a seatbelt, and the airbag had not deployed. Swelling and ecchymosis were observed at the left periorbital area, and he had diplopia on a left-side gaze. A CT revealed fractures in the medial and inferior wall of the left orbit. Entrapped soft tissues were reduced, and the medial wall and floor were reconstructed with a resorbable sheet. His diplopia disappeared 12 days after surgery. To prevent the injury from the steering wheel knob, an airbag should be installed in any vehicle, which has a steering wheel knob. Legislation mandating the use of airbags as well as seatbelts in vehicles with attached steering wheel knobs should be made.
Litre, Claude Fabien Colin, Philippe; Noudel, Remy; Peruzzi, Philippe; Bazin, Arnaud; Sherpereel, Bernard; Bernard, Marie Helene; Rousseaux, Pascal
Purpose: We discuss our experiences with fractionated stereotactic radiotherapy (FSR) in the treatment of cavernous sinus meningiomas. Methods and Materials: From 1995 to 2006, we monitored 100 patients diagnosed with cavernous sinus meningiomas; 84 female and 16 male patients were included. The mean patient age was 56 years. The most common symptoms were a reduction in visual acuity (57%), diplopia (50%), exophthalmy (30%), and trigeminal neuralgia (34%). Surgery was initially performed on 26 patients. All patients were treated with FSR. A total of 45 Gy was administered to the lesion, with 5 fractions of 1.8 Gy completed each week. Patient treatment was performed using a Varian Clinac linear accelerator used for cranial treatments and a micro-multileaf collimator. Results: No side effects were reported. Mean follow-up period was 33 months, with 20% of patients undergoing follow-up evaluation of more than 4 years later. The tumor control rate at 3 years was 94%. Three patients required microsurgical intervention because FSR proved ineffective. In terms of functional symptoms, an 81% improvement was observed in patients suffering from exophthalmy, with 46% of these patients being restored to full health. A 52% improvement was observed in diplopia, together with a 67% improvement in visual acuity and a 50% improvement in type V neuropathy. Conclusions: FSR facilitates tumor control, either as an initial treatment option or in combination with microsurgery. In addition to being a safe procedure with few side effects, FSR offers the significant benefit of superior functional outcomes.
Rabinowitz, Ronen; Velez, Federico G.; Pineles, Stacy L.
Purpose To determine factors associated with surgical success in patients undergoing strabismus surgery after retinal detachment repair with scleral buckle. Methods The medical records of consecutive patients who underwent strabismus surgery after repair of retinal detachment with scleral buckle were retrospectively reviewed. A successful “motor” outcome was defined as horizontal deviation <10Δ and vertical deviation <4Δ in the primary position; successful “sensory” outcome was no diplopia in the primary position. Various factors such as removing the scleral buckle at the time of strabismus surgery, the macula structural status, size of the preoperative deviation, presence of restriction to passive movement, and whether the eye with the scleral buckle was the operated eye were compared among groups based on motor success. Results A total of 25 patients were included. The overall motor success rate was 72% after 1.8 ± 0.9 operations, with 62% of patients diplopia free in the primary position. Horizontal deviation <10Δ (P = 0.005) and minimal restriction on forced duction test were associated with motor success after the first surgery (P = 0.05). Partial or entire scleral buckle removal (n = 15) and fellow-eye surgery were not significantly correlated with motor success in our cohort. There were no retinal redetachments after scleral buckle removal. Conclusions A small preoperative horizontal deviation, and minimally restricted ocular rotations were associated with better results. Removing the scleral buckle did not improve results. PMID:24215808
Dores, Luís Almeida; Simão, Marco Alveirinho; Marques, Marta Canas; Dias, Éscar
Sphenoid sinus disease is particular not only for its clinical presentation, as well as their complications. Although rare, these may present as cranial nerve deficits, so it is important to have a high index of suspicion and be familiar with its diagnosis and management. Symptoms are often nonspecific, but the most common are headache, changes in visual acuity and diplopia due to dysfunction of one or more ocular motor nerves. The authors report a case of a 59 years-old male, who was referred to the ENT emergency department with frontal headaches for one week which had progressively worsened and were associated, since the last 12 hours, with diplopia caused by left third cranial nerve palsy. Neurologic examination was normal aside from the left third cranial nerve palsy. Anterior and posterior rhinoscopy excluded the presence of nasal masses and purulent rhinorrhea. The CT scan revealed a soft tissue component and erosion of the roof of the left sphenoid sinus. Patient was admitted for intravenous antibiotics and steroids treatment without any benefit after 48 hours. He was submitted to endoscopic sinus surgery with resolution of the symptoms 10 days after surgery. The authors present this case for its rarity focusing on the importance of differential diagnosis in patients with headaches and cranial nerves palsies.
Song, Michelle; Ahn, Sun M.; Reh, Douglas R.
Introduction: A pneumocele is an abnormal dilation of an air-containing sinus beyond the normal margins of bone, with associated bony thinning. A delayed ethmoid pneumocele after sinus surgery has not previously been reported. Methods: A case report of a patient with a delayed ethmoid pneumocele after sinus surgery. The diagnostic workup, operative approach, and postoperative results were evaluated. Results: A 57-year-old female with a history of endoscopic sinus surgery 15 years prior presented with right eye proptosis and severe orbital and facial pressure. A maxillofacial computed tomography showed a markedly expanded air-filled right anterior ethmoid space with a dehiscent lamina papyracea, consistent with a pneumocele. Marsupialization of the pneumocele as well as a revision ethmoidectomy were performed, with a visible return of the orbital contents to a more natural position. The patient experienced worsened diplopia immediately postoperatively that resolved within two weeks. Conclusions: This case demonstrates that a pneumocele can present even years after endoscopic sinus surgery, and acute but temporary development or worsening of diplopia can result from surgical decompression of the pneumocele as the eye returns to its natural position. PMID:26302735
Velez, Federico G.; Demer, Joseph L.; Pihlblad, Matthew S.; Pineles, Stacy L.
Background Rectus muscle plication is an alternative muscle-strengthening procedure to rectus muscle resection. Possible advantages of rectus muscle plication include a lower risk of “lost” muscles and anterior segment ischemia. Methods This was a retrospective case series describing a surgical procedure for rectus muscle plication using an adjustable suture technique that can be employed on any of the four rectus muscles. Results A total of 5 adult patients underwent adjustable suture plication procedures. Of these, 2 patients required suture adjustment postoperatively. At the final follow-up visit, all of the patients maintained satisfactory ocular alignment within 6Δ of orthotropia for horizontal deviations and 2Δ of orthotropia for vertical deviations. Diplopia was eliminated in all cases with preoperative diplopia. There were no postoperative complications or unexpected shifts in ocular alignment. Conclusions Rectus muscle plication using this adjustable suture technique may serve as an alternative to rectus muscle resection and may be particularly useful in patients who are at risk for anterior segment ischemia or those in whom a shorter anesthesia time is recommended. PMID:24160967
Vargas-Camaño, Eugenia; Castrejon-Vázquez, Isabel; Plazola-Hernández, Sara I.; Moguel-Ancheita, Silvia
Background Thyroid-associated orbitopathy is commonly associated with Graves' disease with lid retraction, exophthalmos, and periorbital swelling, but rarely with autoimmune thyroiditis or euthyroid state. We reviewed 3 cases from our hospital whose antibodies to anti-receptor of TSH were normal. Methods Case 1: 60 year-old non-diabetic woman with bilateral glaucoma in treatment, recurrent media otitis and euthyroidism, acute onset of painless diplopia, and lid ptosis in the left eye. MRI of orbit showed increased size of the III right cranial pair and high levels of thyroid autoantibodies (Tab) anti-tiroglobulin (ATG) 115.1, anti-thyroid peroxidase (ATPO) 1751 U/mL. She started oral deflazacort 30 mg each 3 days. Sixty days later, complete remission of eye symptoms correlated with lower auto-antibodies level (ATG 19 ATPO 117). Case 2: 10 year-old girl. At age 8, she had diplopia, lid ptosis and limitations of upper gaze in the left eye. The neurological study discarded ocular myasthenia; with thyroid goitier, and hypothyrodism, she started oral levothyroxin. At age 10 with normal IRM Botulinic toxin was injected, without change. High levels of Tab were found, ATG 2723, ATPO 10.7. She started oral deflazacort 30 mg each 3 days, azathioprin 100 mg, daily. Actually, Tab levels are almost normal, but she remains with ocular alterations. Case 3: 56 year-old woman, Grave´s disease with exophtalmos in 1990, treated with I131 and immunosupression, with good outcome; obesity, hypertension and bilateral glaucoma in treatment. She suddenly presented diplopia and IV pair paresia of the right eye. A year later, ATb were found slightly elevated, ATG 100 years ATPO 227; despite prednisone 50 mg, each 3 days and azathioprin 150 mg/daily treatment, a surgical procedure was required for relieve the ocular symptoms. Results We found only 3 cases previously reported with this type of eye thyroid disease. Is important to note that awareness of this atypical form of orbitopathy
Prakash, S; Mathew, C; Bhagat, S
Two young patients are described who made complete recovery from locked-in syndrome (LIS) after snakebites. LIS was a presenting feature in a patient of presumed snake bite who showed complete response to polyvalent Anti-snake venom (ASV). This case suggests that elapid snake bite should be suspected in unresponsive patient found in early morning in endemic areas of snake bite in monsoon season. The second case was admitted in complete LIS state with history of rapidly progressive ptosis, diplopia, ophthalmoplegia, bulbar symptoms and quadriparesis, 6 hours after snake bite. Complete improvement by ASV in second patient highlights the need of differentiation of complete LIS from coma and brain death in patient of snake bite, as former have good prognosis.
Jibia, A; Chenin, L; Lefranc, M; Peltier, J
An isolated schwannoma of the oculomotor nerve is rare in children without an associated neurofibromatosis. A 13-year-old girl, with a previous medical history of migraine, was admitted for disabling ophthalmic migraine with oblique diplopia. The clinical examination showed a right incomplete ophthalmoplegia with reduced ipsilateral visual acuity (8/10). There was no particular skin reaction. The MRI revealed a right (isosignal-T1 and isosignal-T2) nodular schwannoma located within the cisternal segment of the oculomotor nerve. The angio-CT performed later confirmed the absence of any vascular malformation. The treatment consisted of analgesics and corticotherapy, with complete regression of symptoms three weeks later and a normal MRI follow-up. Therefore, radiosurgery was not performed.
Billette de Villemeur, T; Pradel, A
Thirty cases of Creutzfeldt-Jakob disease (CJD) after cadaveric growth hormone treatment have been counted by the National Reference Center for iatrogenic CJD. The clinic presentation is homogeneous, beginning by neurological troubles (diplopia, unsteady gait) evolving rapidly in few months towards a severe neurological deterioration, insanity and death. All patients were treated between January 1984 and July 1985. The risk to transmit CJD with treatments of human origin (pituitary derived treatment, blood, placentas and corneal and dura mater graft) is analyzed. The selection of donors and techniques of purification on the one hand, the rigor of the indication and the quality of the followup on the other hand, are the only guarantees to reduce the risks secondary to utilization of products of human origin.
Marangoz, Deniz; Doğan Ekici, Işın; Çiftçi, Ferda
A 29-year-old female patient presented with a painless mass on her upper eyelid medially. She noticed the mass 4 years earlier and it had increased in size over time. She had no diplopia, eyelid swelling, skin lesion overlying the mass, or visual disturbances. On ocular examination, eye movements and funduscopy were normal. The mass was movable and painless with palpation. Magnetic resonance imaging with contrast showed a 12x8x7 mm well-circumscribed cystic lesion with no contrast dye appearance. Surgical removal was performed delicately and no capsular rupture occured. Pathological examination revealed an eccrine hidrocystoma. Our aim is to underline that eccrine hidrocystoma should be included in differential diagnosis of orbital masses. PMID:28058171
Michels, Kevin S; Ng, John D; Falardeau, Julie; Roberts, Warren G; Petersen, Bryan; Nesbit, Gary M; Barnwell, Stanley L
A 76-year-old woman presented with an acute onset of right periocular pain, diplopia, ocular injection, progressive proptosis, and periocular swelling. She had an unremarkable past medical history, and the erythrocyte sedimentation rate and complete blood count were normal. A carotid-cavernous sinus fistula was suspected, and an MRI demonstrated enlargement of the superior ophthalmic vein posterior to the globe and enlargement of the inferior ophthalmic vein throughout its entire course. Cerebral arteriography demonstrated a dural cavernous sinus fistula. The inferior ophthalmic vein was accessed via the inferonasal orbital space and was catheterized for delivery of multiple platinum coils to the cavernous sinus fistula. Follow-up venograms demonstrated occlusion of the fistula. At 2-month follow-up, there was a residual sixth nerve palsy and resolution of symptoms, including proptosis and periocular swelling.
Maurri, S; Brogelli, S; Alfieri, G; Barontini, F
After introducing the problem of blepharospasm, we report our experience on treatment with purified botulinum A toxin in 16 cases of blepharospasm, symptomatic in two and essential in 14, than had not responded to drugs. The changes in intensity and frequency of spasm after treatment were evaluated on a clinical scale and by review of videotapes. The beneficial effect appeared within a week in most patients, lasting from 6 to 28 weeks (mean 13), and reached the maximum at the third-seventh week. Mild spasms and female patients responded better. Repeated injections were followed by better response to the drug. Complications, exclusively local, were represented by transient corneal exposure, ptosis, lacrimation or diplopia.
Tiong, T S; Selva, K S
Nasopharyngeal carcinoma (NPC) is a common cancer in Malaysia. The clinical presentation in Sarawak has not been well documented. A retrospective review of 213 selected NPC cases was undertaken on the clinical records in Sarawak General Hospital, Sarawak, from June 1999 to June 2003. There were 116 patients in Kuching and 97 in Serian. There were twice as many males as females. The youngest patient was 16 and the oldest 88 years old with a mean age of 51 years. The four most common symptoms in order of frequencies were cervical lymphadenopathy, epistaxis, hearing loss and diplopia. 80.8% of the patients presented with cervical lymphadenopathy and about 85% of the patients presented in the advanced stages. Very small percentages of the patients were found to have single presenting symptoms of epistaxis (2.4%) and hearing loss (0.5%).
González Gili, Lucas O; Buffone, Ignacio R; Carrara, Laura E; Coto, María B; Fortunatti, Eliana A; Dejtera, Mabel; García Elliot, María F; Giacone, Alejandra; Luncio, Anabella C; Masnicoff, Sebastián D; Oviedo Crosta, María B; Parroua, Marianela; Romano, Mariana
Pseudotumor cerebri is a syndrome characterized by an elevated intracranial pressure greater than 20 cmH2O with ventricles and cerebrospinal fluid of normal characteristics. Consumption of minocycline have been described among the causes associated with this syndrome. We present a 13-year old female patient with a history of acne treated with minocycline who began with severe headache, diplopia and blurred vision. The diagnosis of pseudotumor cerebri was made, indicating the immediate antibiotic suspension and the beginning of the treatment with acetazolamide. Although the pathogenesis of pseudotumor cerebri is not fully known, an association with minocycline has been observed. This antibiotic is often used by health professionals for the management of acne, so it is important to consider its complications before being prescribed.
Borovac, Josip Anđelo; Božić, Joško; Žaja, Nikola; Kolić, Krešimir; Hrboka, Vedran
A case is reported of a 26-year-old primiparous woman in the 32nd week of gestation who presented to the emergency department with the symptoms of a severe headache, nausea and vomiting. The patient was diagnosed with preeclampsia that later progressed to eclampsia. This state was characterized by a sudden onset of a headache and diplopia that advanced to cortical blindness and precipitated significant alterations in mental status, most notable being global amnesia that resolved within 48 h. A post-partum magnetic resonance imaging of the brain in FLAIR mode revealed multiple cortico-subcortical areas of hyperintense signals suggestive of edematous lesions that chiefly involved occipital and parietal lobes with additional atypical manifestations. Such radiologic findings suggested a posterior reversible encephalopathy syndrome variant with the global amnesia as an extraordinary constituent. This unique feature should be acknowledged when treating a preeclamptic or hypertensive patient that exhibits neurological symptomatology and vision disturbances.
Kebede, Saba; Edmunds, Eiry; Raybould, Adrian
A 52-year-old man presented with a history of sudden onset diplopia. On neurological examination, the only abnormality was a right-sided oculomotor (third nerve) palsy. A brain CT was performed and reported as showing no abnormality. He was discharged to be investigated as an outpatient. He presented 1 month later with a new expressive dysphasia and confusional state. MRI was performed which revealed multiple cerebral infarcts. He was discharged on secondary stroke prevention medication. Six months elapsed, before a transthoracic echocardiogram was performed. This showed a large left atrial myxoma. The patient underwent an emergency resection and made a good postoperative recovery. This case report showed the importance of considering a cardiogenic source of emboli in patients who present with cerebral infarcts. Performing echocardiography early will help to detect treatable conditions such as atrial myxoma, and prevent further complications. PMID:24285802
Kaneko, Yuji; Tsurutani, Yuya; Sagawa, Naoko; Kondo, Mai; Sata, Akira; Miyao, Mariko; Azuma, Reo; Orimo, Satoshi; Mizuno, Yuzo
A 70-year-old man with a 28-year history of type 2 diabetes mellitus was admitted due to persistent vomiting and neurological abnormalities in Nov 2012. He had developed gait disturbance and diplopia for six months during antiplatelet therapy, which was initiated following the diagnosis of a cerebellar infarction in June 2012. He had nystagmus, truncal ataxia and an ocular motility disorder, and the MRI study showed increased FLAIR and DWI signals in the peri-third ventricle and periaqueductal region, in addition to the cerebellar vermis. Wernicke encephalopathy was suspected according to his symptoms, and thiamine administration dramatically improved his condition. He did not have a history of alcohol abuse or poor eating habits; however, various coexisting factors, including diabetes mellitus, pyloric stenosis and the use of antiulcer drugs and insulin, were considered to be responsible for Wernicke encephalopathy. This case demonstrates the importance of distinguishing Wernicke encephalopathy from cerebrovascular disease in elderly patients.
Alam, Md. Shahid; Agarwal, Swati
Isotretinoin a synthetic analogue of vitamin A is primarily used for cystic acne not responding to conventional treatment. Several ocular side effects including blurring of vision, decreased dark adaptation, corneal opacities and meibomian gland atrophy have been reported with prolonged use of isotretinoin. There have been reports of muscular damage caused by isotretinoin. Extra ocular myopathy as an adverse effect of long term used of isotretinoin has never been mentioned in literature. We report a case of a young male who presented to us with complaints of diplopia after using isotretinoin for a prolonged period. He was diagnosed as a case of presumed isotretinoin extraocular myopathy after imaging and other blood investigations. PMID:28163542
Sautter, T; Herzog, A; Hauri, D; Schurch, B
In the last 10 years, wound botulism has increasingly been reported and nearly all of these new cases have occurred in injecting-drug abusers. After absorption into the bloodstream, botulinum toxin binds irreversibly to the presynaptic nerve endings, where it inhibits the release of acetylcholine. Diplopia, blurred vision, dysarthria, dysphagia, respiratory failure and paresis of the limbs are common symptoms of this intoxication. Surprisingly and despite the well-known blocking action of the botulinum toxin on the autonomic nerve system, little attention has been paid to changes in the lower urinary tract following acute botulinum toxin poisoning. Here we report a case of bladder paralysis following wound botulism. Early diagnosis and adequate management of bladder paralysis following botulism is mandatory to avoid urologic complications. Accordingly, the prognosis is usually favorable and the bladder recovery complete.
Sabatino, Lorenzo; Sabatino, Francesco; Casale, Manuele; Quattrocchi, Carlo Cosimo; Zobel, Bruno Beomonte
Blepharoptosis is the drooping or inferior displacement of the upper eyelid. Blepharoptosis can be either congenital or acquired. Tumour metastasis is one of the acquired causes of blepharoptosis. The lungs, locoregional lymph nodes, bone and liver are the usual sites of metastases of renal cell carcinoma (RCC); however, unusual locations of RCC have also been reported. Herein, we describe a case of a 47-year-old man with unilateral ptosis and blurred vision due to metastatic RCC. We describe the different causes of blepharopstosis, the path that led to the diagnosis, and how RCC can metastasize to unusual anatomical regions such as the orbit. Symptoms such as exophthalmos, lid edema, diplopia, ptosis, cranial nerve paralysis or blurred vision may mime a benign disease; however, they could also be the symptoms of a systemic malignancy. PMID:28326282
AmbroŽová, Helena; DŽupová, Olga; Smíšková, Dita; Roháčová, Hana
Botulism, a life-threatening condition, is very rare in the Czech Republic. Since 1960, a total of 155 cases have been reported; between 2010 and 2012, not a single case was identified. This is a case report of familiar occurrence of botulism following consumption of home-made pork and liver pâté in three family members admitted to the Department of Infectious, Tropical and Parasitic Diseases, Na Bulovce Hospital in Prague in May 2013. The neurological symptoms were dominated by diplopia and dysarthria. After administration of an antitoxin, all patients recovered. Given the poor availability of the antitoxin, a decision was made following this small family epidemic to have an emergency reserve of life-saving anti-infective drugs for the Czech Republic in the Toxicological Information Center in Prague.
Kebede, Saba; Edmunds, Eiry; Raybould, Adrian
A 52-year-old man presented with a history of sudden onset diplopia. On neurological examination, the only abnormality was a right-sided oculomotor (third nerve) palsy. A brain CT was performed and reported as showing no abnormality. He was discharged to be investigated as an outpatient. He presented 1 month later with a new expressive dysphasia and confusional state. MRI was performed which revealed multiple cerebral infarcts. He was discharged on secondary stroke prevention medication. Six months elapsed, before a transthoracic echocardiogram was performed. This showed a large left atrial myxoma. The patient underwent an emergency resection and made a good postoperative recovery. This case report showed the importance of considering a cardiogenic source of emboli in patients who present with cerebral infarcts. Performing echocardiography early will help to detect treatable conditions such as atrial myxoma, and prevent further complications.
McNab, A A
A fistula between the paranasal sinuses and the orbit as a late complication of orbital fractures is rare and may present with intermittent symptoms due to air passing into the orbit. A case note review of two patients with sino-orbital fistula is presented. Two patients, 23- and 30-year-old males, presented with intermittent symptoms of globe displacement, diplopia or discomfort months after repair of an orbital floor fracture with a synthetic orbital floor implant. The symptoms occurred after nose blowing. They were both cured by removal of the implant and partial removal of the tissue surrounding the implant. A sino-orbital fistula may complicate the otherwise routine repair of an orbital floor fracture, but may be cured by removal of the implant and part of the surrounding pseudocapsule.
Shanker, Varshini; Nigam, Vishal
Abstract We report the case of an 11-year-old boy who presented with sudden esotropia, binocular diplopia, and blurred vision. The patient was neurologically normal. He had a large, constant, comitant, alternating esotropia associated with minimal accommodative spasm. Ocular motility and pupillary reactions were normal. He was diagnosed to have spasm of the near reflex presenting as acute onset of esotropia. The esotropia was persistent despite treatment and eventually resolved with prolonged cycloplegic therapy. This unusual case illustrates that spasm of the near reflex can have unique and variable presentations. Spasm of the near reflex needs to be considered in the differential diagnosis of every case of acute, acquired, comitant esotropia. This is the first case of spasm of the near reflex where persistent esotropia is reported in the absence of any neurological disorder. PMID:27928354
Gasparini, A; Sterlicchio, M; Castiglioni, E; Raimondi, E
The authors report a clinical case of a 48-year-old female patient admitted to the Neurological Division following acute symptoms characterised by generalised asthenia, motory disorders (incoordination, equilibrium or gait deficit) accompanied by diplopia. Instrumental (medullary and encephalic NMR) and laboratory tests revealed a malformation of the atlo-occpital hinge with basilar impression and areas of corticosubcortical demyelinisation signifying multiple sclerosis. The liquor test was also positive for the presence of oligoclonal bands of IgG with a Link index of 0.97 (lower v.n. at 0.7). The association between these two pathologies is rare, whereas the need for a differential diagnosis between them often arises. Therefore, two pathologies which are mutually exclusive in many cases were present in an associated form in this case.
Hirose, Y; Sagoh, M; Mayanagi, K; Murakami, H
A 47-year-old male presented with abducens nerve palsy due to basilar impression associated with atlanto-occipital assimilation manifesting as slowly progressive bilateral trigeminal neuralgia and diplopia in the right lateral gaze. X-ray and computed tomography of the skull confirmed the diagnosis of basilar impression and atlanto-occipital assimilation, and magnetic resonance imaging disclosed tightness of the posterior cranial fossa. Surgical suboccipital decompression resulted in gradual resolution of the patient's complaints, and no additional symptoms were recognized. Impairment of the sixth cranial nerve is a rare symptom compared to those of the fifth or the eighth cranial nerve in a patient with a craniocervical malformation. However, the present case shows the possibility of cranial nerve dysfunction due to tightness of the posterior cranial fossa, and suggests that surgical treatment for basilar impression with atlanto-occipital assimilation should be considered in patients with uncommon and unusual symptoms.
Chan, Tommy L. H.; Cartagena, Ana M.; Bombassaro, Anne Marie; Hosseini-Moghaddam, Seyed M.
Ramsay Hunt syndrome associated with varicella zoster virus reactivation affecting the central nervous system is rare. We describe a 55-year-old diabetic female who presented with gait ataxia, right peripheral facial palsy, and painful vesicular lesions involving her right ear. Later, she developed dysmetria, fluctuating diplopia, and dysarthria. Varicella zoster virus was detected in the cerebrospinal fluid by polymerase chain reaction. She was diagnosed with Ramsay Hunt syndrome associated with spread to the central nervous system. Her facial palsy completely resolved within 48 hours of treatment with intravenous acyclovir 10 mg/kg every 8 hours. However, cerebellar symptoms did not improve until a tapering course of steroid therapy was initiated. PMID:27366189
Alroughani, Raed; Thussu, Anil; Guindi, Raouf T.
Variants of Guillain-Barre syndrome such as Bickerstaff encephalitis and Miller-Fisher syndrome have been reported. We report a 15-year-old boy who presented, after a prodromal illness, with 3-day progressive limb weakness, diplopia, and acute urinary retention. Clinically, he had horizontal gaze-evoked and upbeat nystagmus, bilateral extensor plantars in addition to quadriparesis and areflexia. Magnetic resonance imaging of the brain and spine was unremarkable and cerebral spinal fluid analysis showed lymphocytic pleocytosis. Nerve conduction study revealed symmetrical axonal neuropathy. Anti-GQ1b antibody was positive. A combination of IV methylprednisone followed by IVIg was instituted which led to remarkable clinical recovery. This case underpins the importance of recognizing atypical presentations of acute autonomic dysfunction and central nervous system features such as nystagmus, which may be associated with anti-GQ1b antibody syndrome. Features mimicking myelitis and brainstem encephalitis may pose diagnostic and therapeutic dilemma among the treating physicians. PMID:26487925
Moona, Mohammad Shafi; Mehdi, Itrat
A 32 year Libyan male presented with the complaints of headache and diplopia. He was diagnosed with a cavernous sinus meningioma on the basis of MRI findings but no initial biopsy was taken. Depending on the radiologic diagnosis the patient was treated with gamma knife surgery twice, abroad. During follow up he developed left ear deafness and left cervical lymph adenopathy. An ENT evaluation with biopsy from the nasopharynx and cervical lymph node was taken. The histopathologic diagnosis of the resected tumour showed a nasopharyngeal carcinoma with cervical lymph node metastasis (poorly differentiated lympho-epithelial carcinoma). The cavernous sinus tumour which was initially treated as a meningioma was in fact metastasis from the nasopharyngeal carcinoma, making this an interesting and rare occurrence.
Benson, Rony; Mallick, Supriya; Purkait, Suvendu; Suri, Vaishali; Haresh, K P; Gupta, Subhash; Sharma, Dayanand; Julka, Pramod Kumar; Rath, Goura Kishore
Primary central nervous system lymphoma (PCNSL) is a rare disease in pediatric age group. A thirteen-year-old male child presented with complaints of headache for six months, vomiting and diplopia for three days. Magnetic resonance imaging of the brain showed a single lesion of 1.7 cm × 1.6 cm × 1.6 cm in the mid brain and tectum. He underwent a gross total resection of the tumor. The histopathological evaluation revealed B cell high grade non Hodgkin lymphoma. The patient was treated with High dose methotrexate and cranio spinal radiation. The patient was alive without disease 12 mo after completion of treatment. This case highlights importance of keeping PCNSL as differential in brain stem lesions of pediatric patients also. Radiation and chemotherapy remains the most important treatment for such patients. PMID:28035316
Ling, Helen; Kara, Eleanna; Revesz, Tamas; Lees, Andrew J; Plant, Gordon T; Martino, Davide; Houlden, Henry; Hardy, John; Holton, Janice L
We report the case of a 75-year-old ex-professional boxer who developed diplopia and eye movement abnormalities in his 60's followed by memory impairment, low mood and recurrent falls. Examination shortly before death revealed hypomimia, dysarthria, vertical supranuclear gaze palsy and impaired postural reflexes. Pathological examination demonstrated 4-repeat tau neuronal and glial lesions, including tufted astrocytes, consistent with a diagnosis of progressive supranuclear palsy. In addition, neurofibrillary tangles composed of mixed 3-repeat and 4-repeat tau and astrocytic tangles in a distribution highly suggestive of chronic traumatic encephalopathy were observed together with limbic TDP-43 pathology. Possible mechanisms for the co-occurrence of these two tau pathologies are discussed.
Ebner, R; Devoto, M H; Weil, D; Bordaberry, M; Mir, C; Martinez, H; Bonelli, L; Niepomniszcze, H
Aim: To evaluate the efficacy of periocular triamcinolone acetonide for the treatment of thyroid associated ophthalmopathy (TAO), and the presence of ocular or systemic adverse effects. Methods: A multicentre prospective pilot study was performed on patients diagnosed with Graves’ ophthalmopathy less than 6 months before entry to the study. Patients were admitted to the study and were randomised into two groups: treatment and control. The treatment group received four doses of 20 mg of triamcinolone acetate 40 mg/ml in a peribulbar injection to the inferolateral orbital quadrant. Both groups were evaluated by measuring the area of binocular vision without diplopia on a Goldmann perimeter and the size of the extraocular muscles on computed tomography (CT) scans. Ophthalmological and systemic examinations were done to rule out ocular and systemic adverse effects. Follow up was 6 months for both groups. Results: 50 patients were eligible for the study. 41 patients completed the study. There was an increase in the area of binocular vision without diplopia in the treatment group (Σ initial: mean 231.1 (SD 99.9) and final absolute change, mean 107.1 (SD 129.0)) compared to the control group (Σ initial: mean 350.7 (SD 86.5) and final absolute change, mean −4.5 (SD 67.6)). The sizes of the extraocular muscles were reduced in the treatment group (mean (inferior rectus initial values): 1.3 (0.7), final percentage change: −13.2 (25.7), medial rectus initial values: 1.2 (0.6), final percentage change: −8.2 (20.7), superior rectus-levator palpebrae initial values: 1.2 (0.6), final percentage change: −9.5 (29.1), lateral rectus initial values: 1.0 (0.4), final percentage change: −11.5 (20.6)) compared to the control group (inferior rectus initial values: 0.9 (0.3), final percentage change: −4.0 (21.5), medial rectus initial values: 0.9 (0.3), final percentage change: 0.6 (22.4), superior rectus-levator palpebrae initial values: 0.9 (0.3), final percentage change
Joo, M; Lee, G J; Koh, Y C; Park, Y K
Hemangioendothelioma is borderline or intermediate type of vascular neoplasm. Hemangioendothelioma is rare lesion that constitutes less than 0.5% of the malignant tumors of bone. We present a case of low-grade hemagioendothelioma of the skull in a 29-yr-old woman. She had pain, diplopia and exophthalmos of the left eye. Radiographic images showed a relatively well-demarcated, expansile osteolytic lesion with irregularly thickened trabeculae and calcifications in the left greater wing of sphenoid bone. Histologically, the tumor was an infiltrative vasoformative lesion. The vessels are generally well-formed with open or compressed lumina surrounded by endothelial cells showing mild atypia. It lacked frequent mitotic figures and severe atypia. Although excessive bleeding occurred during the operation, the mass was totally resected. Postoperative radiation was not necessary. She is free of disease and well 6 months postoperatively.
Nagaraj, Vaibhav; Ghosh, Abhishek; Nanjappa, Madan; Ramesh, Keerthi
Aim: To analyze the ease and surgical outcome of using sutureless transconjunctival approach for repair of infra-orbital fractures. Design: Prospective clinical case series. Materials and Methods: Totally 5 patients with infra-orbital rim or orbital floor fractures were selected and the fractures were accessed through a pre-septal transconjunctival incision. After reduction and fixation, the conjunctiva was just re-approximated and re-draped into position. Incidence of post-operative complications such as diplopia, lid retraction, eyelid dystopia, foreign body granuloma and poor conjunctival healing was assessed at intervals of 1 week, 15 days and a month post-operatively. Results: No complications were observed in any of the 5 patients. Healing was satisfactory in all patients. Conclusion: The sutureless technique appears to be a time saving and technically simpler viable alternative to multilayered suturing in orbital trauma with minimal post-operative complications. PMID:25821377
Taub, Peter J; Koch, R Michael
Patients with orbital dystopia present with symptoms of diplopia and/or facial asymmetry. Repositioning of the orbit requires multiple osteotomies around the orbit, which presents significant complications. The authors present an alternative method to restore facial symmetry in the case of a 14-year-old patient with congenital orbital dystopia due to fibrosis of the sternocleidomastoid muscle. The patient had no ocular symptoms but did have asymmetric descent of the ipsilateral brow. He refused box osteotomy and repositioning of the orbit. Unilateral brow suspension was performed using an endoscopic technique and suspension sutures fixed to resorbable screws. Adequate brow elevation was noted in the immediate postoperative period and at 6 months; it successfully restored facial symmetry.
Tan, S T; Ashworth, G; Czypionka, S; Poole, M D; Briggs, M
Many pathologic processes may lead to vertical orbital dystopia. We reviewed 47 consecutive cases seen over a 13-year period. Twenty-nine patients underwent eye leveling procedures to improve cosmesis, 2 of these by camouflage procedures and 27 by orbital translocation. Ten patients had 16 secondary operations. There was one death, serious complications occurred in 3 patients, and nuisance complications occurred in 20 others. Seven patients developed diplopia postoperatively, and in 6 patients it was troublesome. In these, it resolved fully in 2 patients, improved to be of no consequence in 2, and in the remaining 2 troublesome symptoms persisted requiring inferior oblique muscle recession in 1. Binocular vision was never restored when not present preoperatively, and in 3 patients temporary loss occurred. There was an overall modest but significant improvement in appearance after surgery. It is concluded that vertical orbital translocation is rewarding and worthwhile.
Maeda, Osamu; Yokota, Kenji; Atsuta, Naoki; Katsuno, Masahisa; Akiyama, Masashi; Ando, Yuichi
A 79-year-old man with lymph node recurrence of malignant melanoma received nivolumab, an anti-programmed death 1 (PD-1) monoclonal antibody. He had pre-existing ocular myasthenia gravis (MG) and a continued small amount of corticosteroid. Grade 3 creatine phosphokinase elevation appeared after two doses of nivolumab, and the treatment was postponed until it improved to grade 1. After three doses of nivolumab, he experienced diplopia and facial muscle weakness which were consistent with an acute exacerbation of MG, and the symptoms relieved without additional treatment for MG. He achieved shrinkage of metastasis after ten doses of nivolumab. Although a case who died due to MG after administration of nivolumab was reported recently, pre-existing MG is considered not to be always a contraindication of nivolumab.
Mano, Tomoo; Matsuo, Koji; Kobayashi, Yosuke; Kobayashi, Yasushi; Ozawa, Hiroaki; Arakawa, Toshinao
A rare cause of trigeminal neuralgia is malignant lymphoma which spread along the trigeminal nerve. We report a 79-year-old male presented with 4-month history of neuralgic pain in right cheek. He was diagnosed as classical trigeminal neuralgia. It had improved through medication of carbamazepine. Four months later, the dull pain unlike neuralgia complicated on the right cheeks, it was ineffective with the medication. Furthermore, diplopia and facial palsy as the other cranial nerve symptoms appeared. Gadolinium-enhanced magnetic resonance imaging (MRI) revealed contrast-enhanced mass lesion extend both external pterygoid muscle and brainstem through the swelling trigeminal nerve. The patient was pathological diagnosed of diffuse large B cell lymphoma by biopsy. Malignant lymphoma should be considered in the different diagnosis of cases with a minimal single cranial nerve symptom.
Smith, Rachel M; Muehlenbachs, Atis; Schaenmann, Joanna; Baxi, Sanjiv; Koo, Sophia; Blau, Dianna; Chin-Hong, Peter; Thorner, Anna R; Kuehnert, Matthew J; Wheeler, Kristina; Liakos, Alexis; Jackson, Jonathan W; Benedict, Theresa; da Silva, Alexandre J; Ritter, Jana M; Rollin, Dominique; Metcalfe, Maureen; Goldsmith, Cynthia S; Visvesvara, Govinda S; Basavaraju, Sridhar V; Zaki, Sherif
In April 2014, a kidney transplant recipient in the United States experienced headache, diplopia, and confusion, followed by neurologic decline and death. An investigation to evaluate the possibility of donor-derived infection determined that 3 patients had received 4 organs (kidney, liver, heart/kidney) from the same donor. The liver recipient experienced tremor and gait instability; the heart/kidney and contralateral kidney recipients were hospitalized with encephalitis. None experienced gastrointestinal symptoms. Encephalitozoon cuniculi was detected by tissue PCR in the central nervous system of the deceased kidney recipient and in renal allograft tissue from both kidney recipients. Urine PCR was positive for E. cuniculi in the 2 surviving recipients. Donor serum was positive for E. cuniculi antibodies. E. cuniculi was transmitted to 3 recipients from 1 donor. This rare presentation of disseminated disease resulted in diagnostic delays. Clinicians should consider donor-derived microsporidial infection in organ recipients with unexplained encephalitis, even when gastrointestinal manifestations are absent.
Selby, Kevin J; Warner, Jeremy; Klempner, Samuel; Konstantinopoulos, Panagiotis A; Hecht, Jonathan L
This is the case report of a 61-year-old woman who presented with progressive diplopia and ataxia. Her cerebrospinal fluid revealed high titers of anti-Yo (PCA-1) antibody and a magnetic resonance imaging with contrast showed cerebellar degeneration. Extensive imaging workup was negative for malignancy and she was otherwise asymptomatic. Given the association between anti-Yo antibodies and gynecologic malignancies, she underwent a bilateral salpingo-oophorectomy and cancer staging. Extensive section of the fimbriated end of the fallopian tube revealed a stage 1, microscopic serous adenocarcinoma. After surgery, her anti-YO titers fell and plans were made for adjuvant chemotherapy. Her neurologic symptoms are not expected to substantially improve, illustrating the urgent need for early surgical investigation in cases of paraneoplastic syndrome, even in the absence of imaging evidence of a lesion.
Dinu, Doina; Grigorescu, Adina; David, Roxana; Urda, S
Taking into consideration the age of the patient, the strabismus surgery targets different things. Thus, for infants, the goal of the surgical intervention for congenital esotropia, is to prevent the occurrence of amblyopia and binocular vision dysfunctions (ARC, suppression). In preschool children, we operate aiming the recovery of the binocular vision, while in children over ten years old, the surgery is done only for esthetic reasons. On the other hand, in adults, the strabismus surgery has two aspects: for esthetic reasons in monocular strabismus with amblyopia, or for diplopia treatment in strabismus of traumatic or neurological cause. To get the best results, the surgical intervention has to respect several rules, which differ with patient's age. This presentation will discuss several surgical procedures: for congenital esotropia (including its advantages and drawbacks), for preschool children strabismus and also for strabismus in adults. We will also review the surgical treatment for Duane syndrome, Ciancia syndrome and superior oblique muscle palsy.
The primary goal of strabismus treatment is to correct and reverse a pathologic condition. Successful treatment is associated with relief from visual confusion or diplopia, a very high incidence of sensory binocular vision, an expanded peripheral visual field, and improved psychosocial function. Binocularity before and after strabismus surgery should be well understood. The stereopsis or sensory fusion should be examined routinely. The measurements should be repeated with the angle of strabismus neutralized with loose prisms. Eye misalignment is measured using the alternate prism and cover test, Eye alignment is measured using the simultaneous prism and cover test. The recent find is that the maximum horizontal deviation that will support true stereopsis is 4(Delta). The functional benefit can usually be obtained in the form of improved binocularity and, consequently, better stability of ocular alignment.
Caldas, Ana Rita; Brandao, Mariana; Paula, Filipe Seguro; Castro, Elsa; Farinha, Fatima; Marinho, Antonio
Hypertrophic cranial pachymeningitis (HCP) is an uncommon disorder characterized by localized or diffuse thickening of the dura mater, and it usually presents with multiple cranial neurophaties. It has been associated with a variety of inflammatory, infectious, traumatic, toxic and neoplasic diseases, when no specific cause is found the process is called idiopathic. The infectious cases occur in patients under systemic immunosuppression, which have an evident contiguous source or those who have undergone neurosurgical procedures. We describe a case of a 62-year-old immunosuppressed woman with diabetes and rheumatoid arthritis, which had HCP and osteomyelitis of the skull base caused by pseudomonas aeruginosa, presenting with headache and diplopia. We believe this is the second documented case of pachymeningitis secondary to this microorganism. As a multifactorial disease, it is essencial to determine the specific causative agent of HCP before making treatment decisions, and great care is needed with immunocompromised patients. Keywords Pseudomonas aeruginosa; Hypertrophic pachymeningitis; Ophtalmoplegia, optical neuropathy; Osteomyelitis; Skull base PMID:22505989
Stefani, F H; Meister, P
Following attacks of maxillary sinusitis and pneumonia ocular signs (epiphora and diplopia) developed in a 39-years-old female patient. An ulceration of the skin over the left lacrimal sac with destruction of the lacrimal bone gave way to a fistula to the nose; and simultaneously a left sclerokeratitis developed. Later a similar fistula on the right side occured together with destruction of the nasal bones, left phthisis bulbi and skin ulcerations on the lower lid. Histologically there was a non-specific chronic inflammation compatible with the diagnosis granuloma gangraenescens nasi ("progressive lethal ulceration of the nose") but no evidence for Wegeners granulomatosis. Under immunosuppressive therapy the condition improved and the patient was able to return to work.
Wade, Ryckie George; Maddock, Thomas B; Ananth, Srinivasan
Orbital varices are thin walled, low flow, distensible veins which may rarely present with periorbital pain, proptosis or visual loss. Most orbital varices may be managed conservatively and only warrant surgery in the presence of recurrent thrombosis, disfiguring proptosis or acute visual loss. This report concerns an 84-year-old Caucasian woman who was admitted following a fall and noted to have isolated proptosis of the right eye, with vertical diplopia. All biochemical and haematological investigations were normal. A CT scan of the orbits demonstrated a serpiginous soft tissue mass within the superior portion of the right orbit, consistent with a thrombosed orbital varix. Conservative management was agreed with prism glasses and ophthalmological follow-up. PMID:23355578
Pesaresi, Ilaria; Sabato, Mario; Desideri, Ilaria; Puglioli, Michele; Moretti, Policarpo; Cosottini, Mirco
For the first time we describe and interpret Susceptibility Weighted Imaging (SWI) and Perfusion Weighted Imaging (PWI) findings in a case of Chronic Lymphocytic Inflammation with Perivascular Pontine Enhancement Responsive to Steroids (CLIPPERS). The diagnosis of the disease was formulated on the basis of typical Magnetic Resonance (MR) findings and its responsiveness to steroids in a 40-year-old man with acute onset of dizziness, ataxia and diplopia. The patient underwent a 3 tesla (T) MR examination including SWI and PWI sequences. SWI revealed prominent veins and multiple hypointense lesions of different size widely distributed in brainstem and cerebellum, which could be expression of iron deposition or cellular infiltrates. PWI demonstrated global infratentorial hypoperfusion. SWI and PWI provide new information on CLIPPERS that might be helpful to understand the physiopathology of the disease. Further observations are needed to evaluate if these findings are peculiar for CLIPPERS and if they might have a role in a non-invasive diagnosis of the disease.
Charakorn, Natamon; Snidvongs, Kornkiat
Chronic sphenoid rhinosinusitis is a spectrum of inflammatory diseases in isolated sphenoid sinus which may persist over a period of 12 weeks. It is a different entity from other types of rhinosinusitis because clinical presentations include headache, visual loss or diplopia, and patients may or may not have nasal obstruction or nasal discharge. Nasal endoscopic examination is useful, and computed tomography is mandatory. The disease requires comprehensive knowledge and appropriate imaging technique for diagnosis. To treat patients with chronic sphenoid rhinosinusitis, surgical treatment with endoscopic transnasal sphenoidotomy is often required. As there are no recent updated reviews of chronic sphenoid rhinosinusitis, in this article, we review the anatomy of the sphenoid sinus and its clinical relationship with the clinical signs and symptoms of the disease, the imaging findings of each diagnosis and the comprehensive surgical techniques. PMID:27877057
Kummelil, Mathew Kurian; Hemamalini, M S; Bhagali, Ridhima; Sargod, Koushik; Nagappa, Somshekar; Shetty, Rohit; Shetty, Bhujang K
Keratoconus is a progressive non-inflammatory thinning of the cornea that induces myopia and irregular astigmatism and decreases the quality of vision due to monocular diplopia, halos, or ghost images. Keratoconus patients unfit for corneal procedures and intolerant to refractive correction by spectacles or contact lenses have been implanted toric posterior chamber phakic intraocular lenses (PC pIOLs) alone or combined with other surgical procedures to correct the refractive errors associated with keratoconus as an off label procedure with special informed consent from the patients. Several reports attest to the safety and efficacy of the procedure, though the associated corneal higher order aberrations would have an impact on the final visual quality. PMID:23925337
Mathog, R H; Shibuya, T; Leider, J; Marunick, M
Surgery of benign and malignant disease of the nose and sinuses can result in disfigurement and disability. Many patients have speech, mastication, and swallowing problems, anosmia, and pain. If the orbit is retained, visual loss, dystopia, diplopia, and epiphora can occur. This study reviews the senior author's experience with 34 patients over the last 18 years. Group and case analyses demonstrate that in patients with cancer, the eye can be preserved if one anatomical boundary is retained beyond a negative frozen section margin. Dystopia can be prevented by reconstruction of the floor with skin and fascial grafts or flaps. Epiphora can be reduced by repair of the medial canthus and dacryocystorhinostomy. Evidence is presented for prosthetic management of speech and swallowing and restoration of cheek contour. Case examples are used to illustrate the surgical techniques.
Rubio-Almanza, Matilde; Cámara-Gómez, Rosa; de San Román-Mena, Laila Pérez; Simal-Julián, Juan Antonio; Ramos-Prol, Agustín; Botella-Asunción, Carlos; Merino-Torres, Juan Francisco
Macroprolactinomas may behave invasively and infiltrate the skull base, causing a subsequent thinning that can also lead to a bone defect and a direct route of entry for pathogens. We describe the case of a 34-year-old male admitted to hospital with fever (38°C), headache, stiffness in the neck, diplopia and neurological impairment. Brain magnetic resonance imaging showed two bilateral abscesses in the fronto-parietal areas with intracranial venous sinus thrombosis and a pituitary adenoma that extended from the suprasellar region, eroding the sellar floor into the sphenoid sinus. Laboratory hormone measurements showed increased levels of prolactin and low levels of FSH, LH and testosterone. The patient received antibiotic treatment and surgery was performed. The patient developed central deafness as a neurological deficit. It is advisable to include pituitary adenoma in the differential diagnosis of meningitis even though its onset as intracranial abscess and rectus sinus thrombosis is extremely rare.
Otto, C S; Nixon, K L; Mazzoli, R A; Raymond, W R; Ainbinder, D J; Hansen, E A; Krolicki, T J
Chorioretinitis sclopetaria presents a characteristic pattern of choroidal and retinal changes caused by a high velocity projectile passing into the orbit, in close proximity to the globe. While it is unlikely that a patient should completely forget the trauma causing such damage, preserved or compensated visual function may blur the patient's memory of these events over time. Characteristic physical findings help to clarify the antecedent history. Despite the lack of an acknowledged history of ocular trauma or surgery, in our case, the characteristic ocular findings discovered at presentation allowed for recognition of the underlying etiology. Because of good visual function, the patient had completely forgotten about the trauma that occurred 12 years earlier. Strabismus surgery was performed for treatment of the presenting symptomatic diplopia. The pathognomonic findings in chorioretinitis sclopetaria are invaluable in correctly diagnosing this condition, especially when a history of ocular trauma is unavailable.
Harris, Gerald J; Paul, Sean; Hunt, Bryan C
Oncocytic adenocarcinoma of the orbit is a rare tumor, with 1 case of nonlacrimal sac, nonlacrimal gland origin, and a poor outcome previously reported. An 85-year-old man with a 2-month history of left-sided epiphora, enlarging eyelid nodules, and diplopia in left gaze was found on imaging to have a poorly circumscribed, nodular mass of uniform radiodensity in the inferomedial orbit. Incisional biopsy revealed morphologic and immunohistochemical features of oncocytic adenocarcinoma with origin in the caruncle suspected, and CT of the neck, chest, abdomen, and pelvis showed no metastases or remote primary tumor source. Based on multidisciplinary consensus, orbital exenteration with adjuvant radiation therapy was performed, and there was no evidence of residual or recurrent tumor 2 years after treatment.
Jones, Jeremy; Nugent, Kenneth
The differential diagnosis for ischemic central nervous system infarcts in young patients includes paradoxic emboli through cardiac shunts, vasculitis, and vascular trauma. We report a young woman who developed headache, vomiting, diplopia, dizziness, and ataxia following neck manipulation by her chiropractor. A computed tomography scan of the head revealed an infarct in the inferior half of the left cerebellar hemisphere and compression of the fourth ventricle causing moderate acute obstructive hydrocephalus. Magnetic resonance angiography revealed severe narrowing and low flow in the intracranial segment of the left distal vertebral artery. The patient was treated with mannitol and a ventriculostomy and had an excellent functional recovery. This report illustrates the potential hazards associated with neck trauma, including chiropractic manipulation. The vertebral arteries are at risk for aneurysm formation and/or dissection, which can cause acute stroke. PMID:25552813
Singh, Swati; Mittal, Ruchi; Padhan, Prasanta; Tripathy, Devjyoti
A young healthy female presented with acute onset left ocular pain, restricted ocular motility, and binocular diplopia. CT imaging showed left lateral rectus myositis that resolved with oral corticosteroids. Two sequential relapses occurred subsequently involving the superior rectus-levator complex followed by the medial rectus. Biopsy revealed orbital inflammatory disease with lymphocytic vasculitis. Detailed systemic work up was normal. The second relapse was seen while on long-term oral methotrexate although initial disease remission had been achieved with the same drug. A changeover to oral azathioprine was able to achieve disease remission after the second relapse. All relapses involved the same side and the contralateral orbit was not affected. This report presents the curious phenomenon of unilateral migratory relapsing orbital myositis of unknown cause that recurred even while on immunosuppressant therapy. It highlights the unpredictable nature of this uncommon entity and the challenges faced in managing such cases.
Crone, R A
Ptolemy (127-148 AD) studied physiological diplopia, correspondence and the horopter. He had all the data to build a theory of depth perception through disparity detection, but left that undone. Alhazen (1000 AD) associated depth perception with the sensation of binocular convergence, just as Kepler (1611) and Descartes (1637). With the development of the concept of retinal correspondence and the fusion of the retinal images in the brain (Huygens 1667, Newton 1704) a cerebral mechanism of disparity detection became thinkable. The rise of Empiricism (Molyneux' Premise, the case of Cheselden) postponed the solution of the problem, finally reached by Wheatstone (1838). Physiological proof of Wheatstone's theory came from the experiments of Barlow et al. (1967).
Lechan, Ronald M.
Carotid-cavernous fistulas (CCFs) are rare, pathologic communications of the carotid artery and the venous plexus of the cavernous sinus. They can develop spontaneously in certain at risk individuals or following traumatic head injury. Typical clinical manifestations include headache, proptosis, orbital pain, and diplopia. We report a case of bilateral carotid-cavernous fistulas associated with these symptoms and also with pituitary enlargement and hypopituitarism, which improved following surgical intervention. Arterialization of the cavernous sinus and elevated portal pressure may interfere with normal venous drainage and the conveyance of inhibiting and releasing hormones from the hypothalamus, resulting in pituitary enlargement and hypopituitarism. This condition should be considered in the differential diagnosis of hypopituitarism associated with anterior pituitary enlargement. PMID:27651959
Krakauer, Mark; Prendes, Mark Armando; Wilkes, Byron; Lee, Hui Bae Harold; Fraig, Mostafa; Nunery, William R
Histoplasma capsulatum var capsulatum is a dimorphic fungus endemic to the Ohio and Mississippi River Valleys of the United States. In this case report, a 33-year-old woman who presented with a right orbital mass causing progressive vision loss, diplopia, and facial swelling is described. Lateral orbitotomy with lateral orbital wall bone flap was performed for excisional biopsy of the lesion. The 1.5 × 1.8 × 2.3 cm cicatricial mass demonstrated a granulomatous lesion with necrosis and positive staining consistent with Histoplasma capsulatum var capsulatum infection. To the authors' knowledge, this is the first case of orbital histoplasmosis to be reported in the United States and the first case worldwide of orbital histoplasmosis due to Histoplasma capsulatum var capsulatum.
Okudo, Jerome; Anusim, Nwabundo
Renal cell carcinoma (RCC) has unusual presentation affecting elderly males with a smoking history. The incidence of RCC varies while the incidence of spread of RCC to the clivus is rare. The typicality of RCC presentation includes hematuria, flank pain, and a palpable flank mass; however, RCC can also present with clival metastasis. The unique path of the abducens nerve in the clivus makes it susceptible to damage in metastasis. We report a case of a 54-year-old African American female that was evaluated for back pain, weakness, numbness, and tingling of bilateral lower extremities and subsequently disconjugate gaze and diplopia. Brain MRI confirmed metastasis to the clivus. She was started on radiotherapy and was planned for chemotherapy and transfer to a nursing home. When a patient presents with sudden unusual cranial nerve pathology, the possibility of metastatic RCC should be sought. PMID:27110412
Spiegel, Daniel P.; Baldwin, Alex S.; Hess, Robert F.
Brief monocular occlusion results in a transient change in ocular dominance, such that the previously patched eye makes a stronger contribution to the binocular percept after occlusion. The previously unpatched eye therefore makes a correspondingly weaker contribution to the binocular sum. To shed light on the mechanism underlying this change we investigate how the relationship between the perception of fusion, suppression, and diplopia changes after short-term monocular deprivation. Results show that fusible stimuli seen by the unpatched eye are actively suppressed as a result of patching and that this can be reversed by an interocular contrast imbalance. This suggests that dichoptic inhibition plays an important role in ocular dominance changes due to short-term occlusion, possibly by altering the contrast gain prior to binocular summation. This may help explain why this form of plasticity affects the perception of both fusible and rivalrous stimuli. PMID:28071682
Martin, Allison N.; Jones, David E.; Brenin, David R.; Lapides, David A.
Paraneoplastic cerebellar degeneration (PCD) is a rare anti-Yo mediated paraneoplastic syndromes rarely that is infrequently associated with breast cancer. We present a case of a 52-year-old female presenting with diplopia, gait instability, dysarthria, dysphagia, nystagmus, and, most notably, new onset paroxysmal episodes of uncontrollable crying concerning for pseudobulbar affect (PBA). Serologic testing showed anti-Yo antibodies. The patient was found to have stage IIIA breast cancer as the inciting cause of the paraneoplastic syndrome. The patient was treated with neoadjuvant chemotherapy, modified radical mastectomy, adjuvant Herceptin, and pertuzumab. She was given IVIG for paraneoplastic syndrome, antidepressants, and dextromethorphan-quinidine (Nuedexta), the first FDA-approved therapy for PBA. With multimodality therapy, she demonstrated significant improvement in neurologic and mood symptoms associated with PCD and PBA. PMID:28377827
Cedeño Diaz, Oderay Mabel; Leal, Roberto García; La Cruz Pelea, Cesar
Primary pineal malignant melanoma is a rare entity, with only thirteen cases reported in the world literature to date. We report a case of a 70-year-old man, who consulted with gait disturbance of six months duration, associated in the last month with dizziness, visual abnormalities and diplopia. No other additional melanocytic lesions were found elsewhere. The magnetic resonance showed a 25 mm expansive mass in the pineal gland that was associated with hydrocephaly, ventricular and transependimary oedema. The lesion was partially excised by a supracerebellar infratentorial approach. The histological examination revealed a melanoma. The patient received radiation therapy, but died of disease 16 weeks later. We herein review the literature on this rare tumour and comment on its clinical, radiological and histopathological features and differential diagnosis. PMID:24765293
Pittke, E C
The fusion amplitude of image pairs with aniseikonia was measured using the synoptophore. Aniseikonia was produced in five subjects by alteration of slide drawings. The subject's right eye was in cycloplegia after paralysis with cycloplegic eye drops. To compare these measurements with aniseikonic pictures to those using the same image pairs without aniseikonia the fusion amplitude achieved was expressed as a percentage of the aniseikonia-free fusion amplitude. The relation between aniseikonia, eccentricity of the image borders and percent fusion amplitude was described using a mathematical approach. The experimental results reveal that a minimal increase in overall aniseikonia with greater eccentricities (greater than 5 degrees) leads to a rapid decrease in the fusion amplitude and the occurrence of diplopia. The aniseikonia tolerance level of images with greater eccentricities achieved experimentally and theoretically are in good agreement with results found in unilaterally aphakic patients.
Kim, Chung-Hwan; Kim, Ungsoo Samuel
A 67-year-old woman complained of horizontal diplopia shortly following bilateral cataract surgery with intraocular lens implantation performed under retrobulbar anesthesia. Retrobulbar anesthesia was administered at an inferotemporal injection site using 1 cc lidocaine hydrochloride 2% mixed with bupivacaine hydrochloride 0.5%. The initial ophthalmologic evaluation showed a 12-prism diopter (PD) exotropia, and ocular motility evaluation revealed marked limitation of adduction without vertical limitation. One year after cataract surgery, the exodeviation increased up to 60 PD. The patient underwent an 8.0-mm recession of the right lateral rectus and a 6.0-mm recession of the left lateral rectus. Both lateral rectus muscles were biopsied, and biopsy revealed dense fibrous connective tissue without viable muscular cells. The lateral rectus muscle might be injured by retrobulbar anesthesia, and it could induce large exotropia. PMID:26953032
Muehlenbachs, Atis; Schaenmann, Joanna; Baxi, Sanjiv; Koo, Sophia; Blau, Dianna; Chin-Hong, Peter; Thorner, Anna R.; Kuehnert, Matthew J.; Wheeler, Kristina; Liakos, Alexis; Jackson, Jonathan W.; Benedict, Theresa; da Silva, Alexandre J.; Ritter, Jana M.; Rollin, Dominique; Metcalfe, Maureen; Goldsmith, Cynthia S.; Visvesvara, Govinda S.; Basavaraju, Sridhar V.; Zaki, Sherif
In April 2014, a kidney transplant recipient in the United States experienced headache, diplopia, and confusion, followed by neurologic decline and death. An investigation to evaluate the possibility of donor-derived infection determined that 3 patients had received 4 organs (kidney, liver, heart/kidney) from the same donor. The liver recipient experienced tremor and gait instability; the heart/kidney and contralateral kidney recipients were hospitalized with encephalitis. None experienced gastrointestinal symptoms. Encephalitozoon cuniculi was detected by tissue PCR in the central nervous system of the deceased kidney recipient and in renal allograft tissue from both kidney recipients. Urine PCR was positive for E. cuniculi in the 2 surviving recipients. Donor serum was positive for E. cuniculi antibodies. E. cuniculi was transmitted to 3 recipients from 1 donor. This rare presentation of disseminated disease resulted in diagnostic delays. Clinicians should consider donor-derived microsporidial infection in organ recipients with unexplained encephalitis, even when gastrointestinal manifestations are absent. PMID:28220747
Ellis, Michael J; Ritchie, Lesley; Cordingley, Dean; Essig, Marco; Mansouri, Behzad
Traumatic optic neuropathy is a rare cause of visual disturbance after head injury that can be difficult to distinguish from coexisting vestibulo-ocular dysfunction because of the overlap in presenting symptoms in patients with these conditions. We present a case report of a 13-year-old girl who sustained a head injury during a ringette game leading to blurred vision and diplopia persisting 5 months after injury. Clinical history and physical examination findings were consistent with a traumatic optic neuropathy, convergence insufficiency, and postconcussion syndrome. Neuroimaging was normal. The patient was managed using a multidisciplinary approach. At 6 months of follow-up, neuro-ophthalmological examination demonstrated evidence of permanent partial optic nerve injury, and formal neuropsychological testing fell primarily within normal limits. The patient was advised to retire from collision sports. The authors discuss the value of a comprehensive multidisciplinary approach to the evaluation and management of concussion patients presenting with persistent visual symptoms.
Leigh, R. John; Tomsak, Robert L.; Grant, Michael P.; Remler, Bernd F.; Yaniglos, Stacy S.; Lystad, Lisa; Dell'Osso, Louis F.
We injected botulinum toxin into the horizontal rectus muscles of the right eyes of two patients who had acquired pendular nystagmus with horizontal, vertical, and torsional components. This treatment successfully abolished the horizontal component of the nystagmus in the injected eye in both patients for approximately 2 months. Both patients showed a small but measurable improvement of vision in the injected eye that may have been limited by coexistent disease of the visual pathways. The vertical and torsional components of the nystagmus persisted in both patients. In one patient, the horizontal component of nystagmus in the noninjected eye increased; we ascribe this finding to plastic-adaptive changes in response to paresis caused by the botulinum toxin. Such plastic-adaptive changes and direct side effects of the injections - such as diplopia and ptosis - may limit the effectiveness of botulinum toxin in the treatment of acquired nystagmus. Neither patient elected to repeat the botulinum treatment.
Martinez-Longoria, Cesar Adrián; Rubio-Perez, Nadina Eugenia; Rios-Solis, Josue Emmanuel; Garcia-Rodriguez, Fernando
The incidences of infection with Mycobacterium tuberculosis and Cryptococcus neoformans in immunocompromised patients have increased, but there are few documented cases of their coexistence. We present the case of a 9-year-old female with systemic lupus erythematosus (SLE), treated with prednisone and cyclophosphamide, who was admitted to the emergency department with a 2-week history of fever, headache, malaise, fatigue, and diplopia 3 years after diagnosis. Physical examination showed limitation of abduction of the right eye, Kernig and Brudzinski signs, and hyporeflexia. Magnetic resonance imaging showed hyperdense lesions located in the caudate nucleus, and lumbar puncture showed pleocytosis, a low glucose level, and increased protein level. Cerebrospinal fluid culture identified C. neoformans and PCR detect M. tuberculosis. Treatment was started with isoniazid, rifampin, pyrazinamide, ethambutol, and amphotericin B. We found two similar reports in adults, but no data were found for either pediatric or SLE patients.
Weinberg, D A
A 76-year-old diabetic woman presented with progressive binocular vertical diplopia and right eye pain. Examination revealed a pupil-involving partial right third cranial nerve palsy, with development of anisocoria over the course of several hours. MRI of the brain showed no mass lesion. MRA, even with retrospective review of the images, failed to clearly identify a 1 cm right posterior communicating artery aneurysm detected by subsequent conventional cerebral angiography. While MRA has been reported to be highly sensitive in cerebral aneurysm detection at some centers, other investigators have indicated less favorable data. Standardized protocols for data acquisition and meticulous attention to proper post-processing and image interpretation are essential if MRA is to supplant invasive arteriography. Currently, conventional (x-ray) angiography remains the gold standard for aneurysm detection, while MRA possesses excellent potential in this regard.
Kuroki, K; Taguchi, H; Sumida, M
A case with non-paralytic pontine exotropia due to brainstem infarction is reported. A 58-year-old male developed sudden onset diplopia. Ocular motor findings were as follows; in forward gaze, the left eye was in abduction position. Leftward gaze, the right eye did not adduct with left beating nystagmus of left eye. Rightward gaze, both eyes could make full excursion and upward, downward gaze were possible. These findings was noted at only acute phase, 7 days later NPPE was disappeared. MRI revealed spotty lesion in the paramedian portion of the mid pontine tegmentum. It is important to observe ocular movement at acute phase because NPPE is very important sign in diagnosis of brainstem disorders.
Borovac, Josip Anđelo; Božić, Joško; Žaja, Nikola; Kolić, Krešimir; Hrboka, Vedran
A case is reported of a 26-year-old primiparous woman in the 32nd week of gestation who presented to the emergency department with the symptoms of a severe headache, nausea and vomiting. The patient was diagnosed with preeclampsia that later progressed to eclampsia. This state was characterized by a sudden onset of a headache and diplopia that advanced to cortical blindness and precipitated significant alterations in mental status, most notable being global amnesia that resolved within 48 h. A post-partum magnetic resonance imaging of the brain in FLAIR mode revealed multiple cortico-subcortical areas of hyperintense signals suggestive of edematous lesions that chiefly involved occipital and parietal lobes with additional atypical manifestations. Such radiologic findings suggested a posterior reversible encephalopathy syndrome variant with the global amnesia as an extraordinary constituent. This unique feature should be acknowledged when treating a preeclamptic or hypertensive patient that exhibits neurological symptomatology and vision disturbances. PMID:27099774
Wang, Shi; Hou, Xiaojun; Ding, Suju; Guan, Yangtai; Zhen, Huimin; Tu, Laihui; Qiu, Yiqing
A 57-year-old male patient with gastric carcinoma underwent radical distal gastrectomy type II + Braun anastomosis, and received total parenteral nutrition for 10 days after surgery, followed by small amounts of semi-liquid nutrition for 3 days and liquid nutrition for 2 days. The patient developed refractory hypotension for more than 1 week in the early course of disease, and on Day 15 after surgery presented with characteristic signs of Wernicke's encephalopathy, including diplopia and mental confusion. The hypotension did not improve despite appropriate fluid replacement soon after admission. Treatment with moderate dose of thiamine for 3 months partly relieved ophthalmoplegia and confusion, but not Korsakoff syndrome. This extraordinary presentation with refractory hypotension and the unusual course of the disease encouraged us to present this case.
Akhavan, Reza; Zandi, Behrouz; Pezeshki-Rad, Masoud; Farrokh, Donya
Colloid cysts are benign slow-growing cystic lesions located on the roof of the third ventricle that usually present with symptoms related to gradual rise of intracranial pressure. They mostly remain asymptomatic and sometimes grow progressively and cause diverse symptoms associated with increased intracranial pressure such as headache, diplopia, and sixth cranial nerve palsy. Here we report a 47-year-old female who presented to the emergency department with acute severe headache and nausea/vomiting. On MRI examination acute hydrocephaly due to hemorrhagic colloid cyst was detected. Acute hemorrhage in colloid cysts is extremely rare and may present with symptoms of acute increase in the intracranial pressure. Intracystic hemorrhage is very rarely reported as a complication of colloid cyst presenting with paroxysmal symptoms of acute hydrocephaly. PMID:28210514
Saito, Ryuta; Sonoda, Yukihiko; Kumabe, Toshihiro; Nagamatsu, Ken-ichi; Watanabe, Mika; Tominaga, Teiji
This 13-year-old boy with a history of cranial irradiation for the CNS recurrence of acute lymphocytic leukemia developed a glioblastoma in the right cerebellum. Resection and chemo- and radiotherapy induced remission of the disease. However, recurrence was noted in the brainstem region 8 months later. Because no effective treatment was available for this recurrent lesion, the authors decided to use convection-enhanced delivery (CED) to infuse nimustine hydrochloride. On stereotactic insertion of the infusion cannula into the brainstem lesion, CED of nimustine hydrochloride was performed with real-time MR imaging to monitor the co-infused chelated gadolinium. The patient's preinfusion symptom of diplopia disappeared after treatment. Follow-up MR imaging revealed the response of the tumor. The authors report on a case of recurrent glioblastoma infiltrating the brainstem that regressed after CED of nimustine hydrochloride.
Khan, Saad Akhtar; Ujjan, Badar Uddin; Salim, Adnan; Shamim, Shahzad
Background: Primitive neuroectodermal tumors (PNETs) comprise a group of aggressive, poorly differentiated embryonal tumors occurring in central nervous system as well as in peripheral locations. Primary cerebellopontine angle (CPA) PNET is an extremely rare entity. It is important to have knowledge of this pathology and to be able to differentiate it from other commonly occurring CPA tumors, such as vestibular and trigeminal schwannomas. This distinction is essential because of the difference in the overall treatment plan and prognosis. Case Description: This report describes a case of a young male presenting with diplopia and numbness of face; magnetic resonance imaging showed a CPA mass. With a provisional diagnosis of trigeminal schwannoma, the patient underwent surgery. Histopathology provided a diagnosis of PNET. Conclusion: We discuss the importance of recognizing this rare condition and how this entity differs from the commonly occurring tumors. PMID:26862446
Vaphiades, Michael S; Simmons, Debra; Archer, Robert L; Stringer, Warren
A 40-year-old woman presented with headache and diplopia after hypotension from postpartum hemorrhage. A noncontrasted cranial magnetic resonance imaging (MRI) showed an enlarged pituitary with a rim of slight increased signal. A repeat gadolinium-enhanced cranial MRI showed peripheral enhancement of the pituitary gland surrounding an isointense central area consistent with infarction of the pituitary and the clinical diagnosis of Sheehan syndrome. The patient was treated with intravenous hydrocortisone. Immediately after treatment, her symptoms remitted and the examination normalized. One month later, a gadolinium-enhanced cranial MRI was normal. The characteristic appearance of the post-gadolinium enhanced cranial MRI helped confirm the diagnosis of Sheehan syndrome and facilitate early treatment with corticosteroids.
Graber, M; Challe, G; Alexandre, M F; Bodaghi, B; LeHoang, P; Touitou, V
Although visual function is thought to be preserved in patients with locked-in syndrome (LIS), enabling them to communicate through vertical or lateral eye movements or blinking of the upper eyelid, nothing is known about the actual visual function of patients with LIS. The goal of this study is to evaluate the visual function of patients with LIS which may enable better evaluation of the state of consciousness of these patients. Patients with LIS seen in a single neurovascular unit of a tertiary center between 1997 and 2013 were retrospectively reviewed. Each patient had a specialized neuro-ophthalmological evaluation under optimal environmental conditions (light, contrast, examination distance, head position, best ergonomic adaptation, and establishment of a means of communication with help from the patient's friends/family). Visual acuity, extraocular eye movements, confrontational visual field, slit lamp and fundus examination were performed. Thirteen patients (6M/7F) were included in this study. Mean visual acuity was 20/60. Oculomotor examination was abnormal in 77%. Forty-six percent of patients presented binocular diplopia mainly related to a VIth nerve palsy. One patient presented complete ophthalmoplegia. Forty-six percent of patients had nystagmus responsible for oscillopsia (oculopalatal tremor). An abnormal visual field was observed in 17% of patients, and abnormal pupillary light response leading to photophobia was present in 22% of patients. Keratitis or dry eye syndrome was present in most patients and was a major cause of pain and visual impairment. Our results suggest that the visual function is impaired in all patients with LIS. This impairment is multifactorial including mostly binocular diplopia or oscillopsia but also refractive errors, dry eye syndrome, keratitis or visual field defect. This altered visual function may alter the ability of the patient to interact with his environment and lead to underestimation of their state of consciousness
Vecchio, Rosa Fontana Del; Pinzone, Marilia Rita; Nunnari, Giuseppe; Cacopardo, Bruno
Patient: Male, 23 Final Diagnosis: Neurocysticerosis Symptoms: Diplopia • fever • headache • insomnia • neck stiffness • vomiting Medication: Albendazole Clinical Procedure: — Specialty: Neurology Objective: Challenging differential diagnosis Background: Neurocysticercosis is a brain infection caused by the larval stage of the tapeworm Taenia (T.) solium. It is the most important parasitic disease of the human central nervous system and represents the most common cause of acquired epilepsy in developing countries. Case Report: Here, we report the case of a 23-year-old Chinese man who presented to the emergency department with a 7-day history of helmet headache radiating to the nuchal region and associated with vomiting, confusion, and fever. Cerebrospinal fluid (CSF) was clear, with increased pressure, lymphocytic pleocytosis, decreased glucose, and increased protein levels. Bacterial antigen detection test on CSF was negative, as were CSF bacterial and fungal cultures. Despite broad-spectrum antibiotic and antiviral therapy, the patient still complained of insomnia, diplopia, headache, neck stiffness, and pain in the sacral region. A second LP was performed and CSF had the same characteristics as the first LP. A brain and spinal cord MRI revealed widespread arachnoiditis and small septated cysts with CSF-like signal in the cisterna magna, within the fourth ventricle, and at the level of L3–L4. Cysticercus-specific immunoglobin G antibodies were detected by ELISA in the CSF. The patient received albendazole (15 mg/kg/day) and dexamethasone (5 mg/day) for 4 weeks, with progressive resolution of neurological symptoms. Conclusions: This case shows that, even if rare, neurocysticercosis may be responsible for meningeal symptoms and should be included in the differential diagnosis, especially in patients from endemic countries. PMID:24459541
Yim, Hye Bin; Biglan, Albert W; Cronin, Tara H
ABSTRACT Purpose To evaluate the effectiveness of graded (adjustable intraoperatively) partial vertical rectus muscle tenotomy at the insertion in correcting small degrees of hypertropia. Methods All patients with best-corrected visual acuity of better than 6/30 in both eyes who over a 30-month period underwent partial tenotomy of vertical rectus muscle(s) only (no concurrent oblique muscles) were included. Improvement was evaluated 6 weeks postoperatively as change in alignment in prism diopters (PD) in primary gaze and in the field of action of the affected rectus muscle(s). Binocular function was evaluated by Titmus stereoacuity and the Worth 4-light tests. Results All 24 patients who met criteria for inclusion had diplopia preoperatively versus seven patients (29%) postoperatively (P < .005, Student’s paired t test). Prisms were used by six preoperatively versus two postoperatively (P < .05, Student’s paired t test). The average vertical deviation in primary gaze decreased from 8 PD to 2 PD (P < .005, Student’s paired t test). In the field of action of the treated rectus muscle, hypertropia decreased from an average of 8 PD to 3 PD (P < .005, Student’s paired t test). For the preoperative and the postoperative assessments available, stereoacuity improved after 10 (56%) of the 18 procedures and Worth 4-light testing showed improvement or maintenance of fusion after 15 (79%) of 19 procedures. Conclusions Graded vertical rectus partial tenotomy can effectively reduce small degrees of hypertropia and associated diplopia, improve binocular function, and reduce or eliminate the need for prism correction. PMID:15747755
Erdoğan, Murat Faik; Demir, Özgür; Ersoy, Reyhan Ünlü; Gül, Kamile; Aydoğan, Berna İmge; Üç, Ziynet Alphan; Mete, Türkan; Ertek, Sibel; Ünlütürk, Uğur; Çakır, Bekir; Aral, Yalçın; Güler, Serdar; Güllü, Sevim; Çorapçıoğlu, Demet; Dağdelen, Selçuk; Erdoğan, Gürbüz
Background The optimal therapeutic choice for Graves' hyperthyroidism in the presence of moderate-severe Graves' orbitopathy (GO) remains controversial. Objectives We aimed to compare GO course in patients with moderate-severe GO treated with early total thyroidectomy (TTx) versus antithyroid drug (ATD) regimens, in a prospective, randomized manner. Methods Forty-two patients with moderate-severe GO were enrolled. A total of 4.5 g of pulse corticosteroids were given intravenously to all patients before randomization. Patients in the first group were given TTx, whereas patients in the second group were treated with ATDs. TSH was kept between 0.4 and 1 mIU/l. The clinical course of GO was evaluated with proptosis, lid aperture, clinical activity score (CAS), and diplopia. Results Eighteen and 24 patients were randomized to the TTx and ATD groups, respectively. Thyroid autoantibodies decreased significantly, and there were significant improvements in proptosis, lid aperture, and CAS in the TTx group. While in the ATD group the decrement in thyroid autoantibodies was not significant, there were significant improvements in proptosis and CAS. When the TTx group was compared with the ATD group, anti-TPO, anti-Tg, and TSH-receptor antibodies were significantly decreased in the TTx group (p < 0.01), but there was no significant difference with respect to proptosis, lid aperture, CAS, and diplopia between the two groups during a median (min.-max.) follow-up period of 60 months (36-72). Conclusion Although no definitive conclusions could be drawn from the study, mainly due to limited power, early TTx and the ATD treatment regimens, followed by intravenous pulse corticosteroid therapy, seemed to be equally effective on the course of GO in this relatively small group of patients with moderate-severe GO during a median (min.-max.) follow-up period of 60 months (36-72). PMID:27493884
Yoshioka, N; Tominaga, Y; Motomura, H; Muraoka, M
The authors present 2 patients with greater sphenoid wing fractures that were treated surgically. This type of fracture is classified as a blow-in fracture of the lateral orbital wall. The first patient was a 16-year-old boy who was involved in a motor vehicle accident. Computed tomography (CT) disclosed a medial displacement of the inner wall of the greater sphenoid wing of the left orbit. He was unconscious for 3 days. After he recovered consciousness, he presented limited abduction of the left eye with diplopia in all gaze directions and mild left proptosis. Although these symptoms did not improve for 1 week, displaced bone fragments of the greater sphenoid wing were removed via the lateral orbital approach. The patient had a good postoperative course with progressive improvement in eye movement over the next several weeks. The second patient was a 22-year-old man whose face was hit in a fight. CT disclosed medial displacement of the inner wall of the greater sphenoid wing of the left orbit. Although the patient also presented limited abduction of the left eye on admission, this symptom improved gradually. However, diplopia in all gaze directions and mild left proptosis did not improve. Therefore, the displaced inner wall of the greater sphenoid wing was reduced via the lateral orbital approach. The patient showed a good postoperative course with progressive improvement over the next several weeks. This type of orbital fracture, which is classified as an orbital blow-in fracture, is relatively rare. This type of greater sphenoid wing fracture is caused by buckling of the orbital wall secondary to severe compression of the orbital rim. Surgical treatment using the lateral orbital approach through a hemicoronal skin incision afforded a wider operative field and better cosmetic result.
Klingenstein, A; Kufeld, M; Wowra, B; Muacevic, A; Fürweger, C; Schaller, U C
Purpose of this study is to evaluate radiographic therapy response, clinical outcome and adverse effects of CyberKnife radiosurgery in patients suffering from orbital metastases. Sixteen orbital metastases originating from different solid cancers in fourteen patients were treated by single fraction CyberKnife radiosurgery. Radiographic response and clinical outcome were evaluated. The treated tumor volume ranged from 0.2 to 35 cm3 (median 2.3 cm3, mean 7.0 cm3, SD 6 10.4 cm3, CI 0.9-9.4 cm3). The prescription dose ranged from 16.5-21 Gy (median 18 Gy, mean 18.2 Gy, SD 6 1.2 Gy, CI 17.0-18.4 Gy). A no change situation was observed in nine lesions, partial remission in four as well as complete remission in one metastasis. Tumor growth was stabilized or regressive following CyberKnife therapy in 87% of the cases. Recurrence was observed in two cases (13%). Before therapy, three patients suffered from visual disturbance and five patients reported diplopia. Six patients had no initial symptoms. After therapy, one patient indicated improvement of the present visual deficit and two patients no change. Out of the two patients with persistent diplopia, two reported improvement after therapy and three no change. No progression of symptoms was noted in any of the cases. Fourteen out of sixteen treated lesions were stable or regressive following CyberKnife radiosurgery (87%). As no serious adverse effects were reported in this series, CyberKnife therapy was shown to be of great value for local management of orbital metastases.
Roverano, Susana; Ortiz, Alberto; Henares, Eduardo; Eletti, Mónica; Paira, Sergio
An 82-year-old woman came to consultation with sudden visual loss in her left eye. Fifteen days before, she complained of diplopia. She had doubtful symptoms of giant cell arteritis and showed a normal physical exam. Lab results showed erythrosedimentation rate (ESR) = 62 mm/1°h; uremia = 0.56 g/dl (normal <0.45); serum creatinine = 1.7 mg% (normal <1.4); low calcium and phosphorus; and normal urine calcium and serous PTH. Fundus exam and brain magnetic resonance imaging (MRI) showed normality of optic nerves, chiasma, retrochiasmatic area, ocular muscles, eyeballs, lacrimal glands, periorbital fat, cavernous sinuses, and occipital cortex. A temporal arteritis was suspected; therefore, a biopsy was carried on. It showed the presence of large calcium deposits in the artery's tunica media and internal elastic lamina, with narrowing of the lumen, with no inflammation and multinuclear giant cells. Histological diagnosis is calciphylaxis. Although calciphylaxis is a well-described entity that occurs in end-stage renal patients, many cases are due to non-uremic causes. To date, there are only six cases described in literature of calciphylaxis mimicking GCA.
Baik, Su Jung; Kim, Tae Hun; Kim, Hye In; Rhie, Jeong Yeon
Abstract Myasthenia gravis is occasionally associated with thymoma that needs surgical resection and may progress to severe respiratory failure. We experienced a rare case of myasthenia crisis during antiviral therapy for chronic hepatitis C, in whom mediastinal thymoma was discovered and successfully managed with surgical thymectomy and meticulous medical care. A 47-year-old-male patient complained of sudden diplopia 1 week after stopping 11-week administration of pegylated-interferon and ribavirin for chronic hepatitis C. Ophthalmologic examinations revealed ptosis on the right eyelid and restricted right eye movement. Myasthenia gravis was confirmed by positive repetitive nerve stimulation test and positive serum antiacetylcholine receptor antibody test, and mediastinal thymoma was found on chest CT scan. The ocular myasthenia gravis progressed to respiratory failure even after discontinuing antiviral treatment but eventually recovered with thymectomy, anticholinesterase administration, steroid pulse therapy, and prolonged ventilator care. We describe the clinical features of this life-threatening complication of interferon treatment along with previous myasthenia crisis cases by interferon for chronic hepatitis C. In patients with chronic hepatitis C who is going to receive interferon-based antiviral treatment, physicians need to keep in mind the potential life-threatening manifestations of myasthenia gravis before and during antiviral treatment especially when patients complain of muscular weakness and easy fatigability. PMID:27227948
Filippín, Federico A; Alfonso, Alejandra; López-Presas, Héctor
Introduccion. La enfermedad celiaca es una enteropatia inducida por el gluten por mecanismos inmunes, que puede presentar afectacion sistemica y producir un amplio espectro de manifestaciones neurologicas, que van desde polineuropatia periferica, ataxia, epilepsia y migraña hasta encefalitis. El objetivo es informar de un caso de posible encefalitis refractaria por enfermedad celiaca. Caso clinico. Varon de 45 años con diagnostico histopatologico de enfermedad celiaca y anticuerpos anticeliaquia negativos que intercurre con encefalitis, manifestaciones del tronco encefalico y del sistema nervioso periferico, entre las que se incluyen deterioro cognitivo y convulsiones, diplopia y ataxia, y polirradiculopatia y polineuropatia, respectivamente; liquido cefalorraquideo inflamatorio y multiples lesiones corticosubcorticales supratentoriales y en el tronco encefalico con leve realce con contraste. Los deficits neurologicos progresan a pesar de una dieta libre de gluten y el tratamiento farmacologico inmunosupresor. Durante el curso de la enfermedad se estudian patologias con afectacion simultanea intestinal y del sistema nervioso, como sarcoidosis, lupus eritematoso sistemico, enfermedad de Sjogren, sindrome paraneoplasico, sida, enfermedad de Whipple y deficit de vitamina B12. Conclusion. La encefalitis es una manifestacion clinica infrecuente en la enfermedad celiaca, con pocos casos notificados a pesar de la alta prevalencia de la enfermedad. Los diagnosticos diferenciales son dificiles y exigen una alta sospecha diagnostica, por lo que es un reto terapeutico.
Shin, Hyun-Seung; Kim, Jeong A; Kim, Dong-Seok
Chiari malformations are a congenital anomaly of the hindbrain. The most common, Chiari malformation type I (CM-I), is characterized by herniation of the cerebellar tonsils extending at least 3 mm below the plane of the foramen magnum. Consequently, CM-I is associated with hydrocephalus and symptoms involving compression of the cervicomedullary junction by ectopic tonsils. Several studies have reported the clinical symptoms associated with CM-I, including suboccipital headache, weakness in the upper extremities, facial numbness, loss of temperature sensation, ataxia, diplopia, dysarthria, dysphagia, vomiting, vertigo, nystagmus, and tinnitus. Syncope is one of the rarest presentations in patients with CM-I. There are many hypotheses regarding the causes of syncope in patients with CM-I; however, the mechanisms are not clearly understood. Although surgical decompression for CM-I in patients with syncope has yielded good clinical results in some studies, such cases are rarely reported. We report a case of orthostatic syncope in a patient with CM-I who was treated with surgical intervention. PMID:28018469
Wilson, Michelle E; Kim, Charles; Carrasco, Jacqueline
A 48-year-old smoker with a history of hyperthyroidism treated 10 years prior to presentation with radioactive iodine ablation of the thyroid gland presented to his ophthalmologist with a 2-week history of transient loss of vision in the right eye occurring for 1 to 2 hours each morning. He denied ocular pain, diplopia or change in the prominence of one or both eyes. Examination revealed 2 mm of relative proptosis on the right, bilateral temporal flare and lower lid retraction. There was minimal upper lid retraction and no evidence of lid lag. Ocular motility was full. Dilated fundoscopic examination revealed bilateral optic nerve edema, right more than left. CT of the orbit demonstrated enlargement of the extraocular muscles bilaterally with marked enlargement of the right medial rectus and left inferior rectus muscles resulting in crowding at the orbital apex bilaterally. Laboratory testing revealed the patient to be hyperthyroid. The patient was treated with high dose oral steroids followed by orbital radiation. Hyperthyroidism was managed by the patient's primary care physician. Visual symptoms rapidly improved with oral steroids and orbital radiation. Optic nerve edema completely resolved. Repeat CT imaging demonstrated a reduction in the enlargement of the extraocular muscles with relief of bilateral optic nerve compression.
Rutsch, Sebastian; Niesen, Wolf-Dirk; Meckel, Stephan; Reinhard, Matthias
The most common neurological injuries associated with roller coaster rides are subdural hematoma and cervical artery dissection. We report two cases of roller-coaster associated subarachnoid hemorrhage (SAH). A 40-year-old healthy man developed a strong, holocephalic headache during a roller coaster ride. SAH Hunt & Hess grade II and Fisher grade 3 was diagnosed. An underlying aneurysm of the anterior communicating artery was successfully treated with coil embolization. A 41-year-old female (smoker, otherwise healthy) experienced a sudden, strong headache and diplopia during a roller coaster ride. A perimesencephalic SAH (Hunt & Hess grade II, Fisher grade 3) was disclosed by a CT scan. No aneurysm was detected on angiography. Both patients were discharged without neurological disability. In conclusion, SAH is a rare but relevant differential diagnosis in cases of acute headache during roller coaster rides. Both aneurysmal and non-aneurysmal perimesencephalic SAH can occur. A combination of mechanical factors and excessive blood pressure rises in vulnerable persons is discussed.
Martinez Santos, Jaime; Kaderali, Zul; Spears, Julian; Rubin, Laurence A; Marotta, Thomas R
Intracranial aneurysms in polyarteritis nodosa (PAN) are exceedingly rare lesions with unpredictable behavior that pose real challenges to microsurgical and endovascular interventions owing to their inflammatory nature. We introduce a safe and effective alternative for treating these aneurysms using Pipeline embolization devices (PEDs). A 20-year-old man presented with diplopia, headaches, chronic abdominal pain, and weight loss. Diagnostic evaluations confirmed PAN, including bilateral giant cavernous carotid aneurysms. Cyclophosphamide and steroids achieved significant and sustained clinical improvement, with a decision to follow the aneurysms serially. Seven years later the left unruptured aneurysm enlarged, causing a sudden severe headache and a cavernous sinus syndrome. Treatment of the symptomatic aneurysm was pursued using flow diversion (PED) and the internal carotid artery was successfully reconstructed with a total of four overlapping PEDs. At 6 months follow-up, complete exclusion of the aneurysm was demonstrated, with symptomatic recovery. This is the first description of using a flow-diverting technique in an inflammatory vasculitis. In this case, PEDs not only attained a definitive closure of the aneurysm but also reconstructed the damaged and fragile arterial segment affected with vasculitis.
Santos, Jaime Martinez; Kaderali, Zul; Spears, Julian; Rubin, Laurence A; Marotta, Thomas R
Intracranial aneurysms in polyarteritis nodosa (PAN) are exceedingly rare lesions with unpredictable behavior that pose real challenges to microsurgical and endovascular interventions owing to their inflammatory nature. We introduce a safe and effective alternative for treating these aneurysms using Pipeline embolization devices (PEDs). A 20-year-old man presented with diplopia, headaches, chronic abdominal pain, and weight loss. Diagnostic evaluations confirmed PAN, including bilateral giant cavernous carotid aneurysms. Cyclophosphamide and steroids achieved significant and sustained clinical improvement, with a decision to follow the aneurysms serially. Seven years later the left unruptured aneurysm enlarged, causing a sudden severe headache and a cavernous sinus syndrome. Treatment of the symptomatic aneurysm was pursued using flow diversion (PED) and the internal carotid artery was successfully reconstructed with a total of four overlapping PEDs. At 6 months follow-up, complete exclusion of the aneurysm was demonstrated, with symptomatic recovery. This is the first description of using a flow-diverting technique in an inflammatory vasculitis. In this case, PEDs not only attained a definitive closure of the aneurysm but also reconstructed the damaged and fragile arterial segment affected with vasculitis.
López, L; Thomson, A; Rabinowicz, A L
Diplopia, blurred vision and colour disturbances are well-known side effects associated with anti-epileptic drugs (AEDs). Farnsworth-Munsell 100-hue colour test (F-100) is an accepted and sensitive tool to detect changes in colour perception. To determine the impact of AEDs upon colour vision, we evaluated 37 consecutive patients with complex partial seizures exposed to monotherapy with phenytoin (PHT, carbamazepine (CBZ) or valproic acid (VPA). All had normal IQ and no congenital disturbances in colour vision or ocular diseases. Twenty normal controls were used for statistical analysis. Thirteen patients were exposed to PHT, 12 to CBZ and 12 to VPA. Visual colour perception was impaired in 30/37 (82%) of the study group. The most significant abnormality was detected in the blue-yellow axis in 10/13 patients exposed to PHT (p < 0.02) and in 8/12 treated with CBZ (p < 0.009). In 8/12 patients taking VPA, no significant abnormality was observed (p < 0.06). None of the studied patients complained of colour vision disturbances. Our findings strongly support the negative effect of AEDs upon colour vision discrimination, most likely due to changes at the retinal processing level. F-100 proved to be very useful to assess early toxicity due to AEDs.
Kölmel, H W
Visual illusions and hallucinations may accompany a wide variety of disorders with many different aetiologies; therefore, they are non-specific phenomena. Lesions in the visual pathway may be associated with visual misperceptions. In these cases more exact information about the misperceptions--whether they are monocular or binocular, present in the whole visual field or a hemifield--may contribute to diagnostic accuracy and to a more comprehensive understanding of the patient and his state of mind. Illusions such as perseveration, monocular diplopia and polyopia, and dysmorphopsia may also occur in healthy individuals, but they are found most often in patients with epilepsy, migraine and stroke. These phenomena do not permit exact localization and definition of an aetiology, but lesions in the occipital and occipitotemporal regions near the visual pathway are involved in most cases. Hallucinations always represent a pathological form of perception. They are classified as unformed (photopsias) or formed (complex). Photopsias may be described in terms of colour, shape and brightness. Their wide variety makes it difficult, if not impossible, to arrive at an exact description of their aetiology, but it is possible to define their anatomical origin in some cases. Complex hallucinations suggest an occipitotemporal locus. Whether they appear in the whole visual field or in the hemifield may prove decisive in determining pathogenesis. A number of characteristics permit a rough classification of these phenomena. Complex hallucinations accompany physical illness and are susceptible to psychodynamic interpretation.
Menon, K Venugopal; Al Rawi, Asif Esam; Taif, Sawsan; Al Ghafri, Khalifa; Mollahalli, Kishore Kumar
Study Design Case report. Objective To report and discuss a rare complication after a patient was treated conservatively with a halo vest. Methods A 51-year-old man sustained a hangman's injury of the C2 vertebra following a motor vehicle collision. He was treated conservatively in a halo vest appliance and following mobilization was discharged from the hospital. Two weeks after discharge, the patient presented to the emergency department complaining of proptosis, ptosis, diplopia, and pin loosening. He was readmitted to the hospital, the halo vest was removed, and urgent imaging studies including computed tomography scan and magnetic resonance imaging were performed. They revealed that one of the halo pins had penetrated the orbital roof with active infection of the extraocular soft tissues. In consultation with the ophthalmologist, he was treated conservatively with antibiotics for 10 days. Results His ophthalmologic complaints resolved gradually and his eye returned to normal appearance and function. In the meantime, he was immobilized in a sterno-occipital mandibular immobilizer brace. Conclusion Though rare, penetrating injuries after cranial pin insertion can occur. Halo devices must be applied by, or under close supervision of, experienced personnel to avoid such complications, and halo vests should be reviewed frequently to detect such incidents early.
McCane, Lynn M; Sellers, Eric W; McFarland, Dennis J; Mak, Joseph N; Carmack, C Steve; Zeitlin, Debra; Wolpaw, Jonathan R; Vaughan, Theresa M
Brain-computer interfaces (BCIs) might restore communication to people severely disabled by amyotrophic lateral sclerosis (ALS) or other disorders. We sought to: 1) define a protocol for determining whether a person with ALS can use a visual P300-based BCI; 2) determine what proportion of this population can use the BCI; and 3) identify factors affecting BCI performance. Twenty-five individuals with ALS completed an evaluation protocol using a standard 6 × 6 matrix and parameters selected by stepwise linear discrimination. With an 8-channel EEG montage, the subjects fell into two groups in BCI accuracy (chance accuracy 3%). Seventeen averaged 92 (± 3)% (range 71-100%), which is adequate for communication (G70 group). Eight averaged 12 (± 6)% (range 0-36%), inadequate for communication (L40 subject group). Performance did not correlate with disability: 11/17 (65%) of G70 subjects were severely disabled (i.e. ALSFRS-R < 5). All L40 subjects had visual impairments (e.g. nystagmus, diplopia, ptosis). P300 was larger and more anterior in G70 subjects. A 16-channel montage did not significantly improve accuracy. In conclusion, most people severely disabled by ALS could use a visual P300-based BCI for communication. In those who could not, visual impairment was the principal obstacle. For these individuals, auditory P300-based BCIs might be effective.
Peyman, Amir; Walsh, Noreen; Green, Peter; Dorey, Michael W; Seamone, Christopher; Pasternak, Sylvia
A 57-year-old man presented to the ophthalmology clinic with a red right eye. He denied pain, diplopia, tearing, and blurred vision. His medical history included asymptomatic annular plaques on the trunk and extremities for at least a decade. Ophthalmological examination revealed a necrotizing scleritis of the right eye. Examination of the skin demonstrated variable sized annular plaques with central atrophy, some with prominent indurated border and yellow discoloration. No periorbital lesions were present. The ocular lesion rapidly progressed and areas of scleral melting developed in the right eye, which eventually required a scleral patch graft. The left eye also developed necrotizing scleritis with areas of scleral melting. Two sets of skin biopsies were performed a few weeks apart. An initial set of skin punch biopsies revealed extensive palisading granulomatous inflammation throughout the dermis, extending into the subcutis. The accompanying perivascular mononuclear infiltrate contained the collections of plasma cells. Scattered multinucleated giant cells were noted. The possibility of necrobiosis lipoidica diabeticorum was suggested. Subsequent skin biopsies showed more prominent and extensive necrobiosis, raising the possibility of necrobiotic xanthogranuloma. Protein electrophoresis was performed, which revealed an IgG λ monoclonal protein.
Background. Invasive fungal rhinosinusitis with orbital complications (IFSwOC) is a life-threatening condition. The incidence of mortality has been reported to be up to 80 percent. This study was conducted to determine the risk factors, presentations, clinical, and imaging findings that could help to manage this condition promptly. Methods. We conducted a case-control study of 100 patients suffering from rhinosinusitis with orbital complications. The risk factors, clinical presentations, radiological findings, medical and surgical managements, durations of hospital stay, and mortality rate data were collected. Results. Sixty-five patients were diagnosed with IFSwOC, while the other thirty-five patients composed the control group. The most important risk factor for IFSwOC was diabetes mellitus. Visual loss and diplopia were the significant symptom predictors. The significant clinical predictors were nasal crust, oculomotor nerve, and optic nerve involvement. The CT findings of IFSwOC were sinus wall erosion and hyperdensity lesions. The mortality rate was 25.71 percent in the IFSwOC group and 3.17 percent in the control group. Conclusions. Invasive fungal rhinosinusitis with orbital complications is symptomatic of a high mortality rate. The awareness of a patient's risk factors, the presenting symptoms, signs of fungal invasion, and aggressive management will determine the success of any treatment procedures. PMID:24298218
Marcus, A; Ammermann, C; Klein, M; Schmidt, M H
Wilson's disease is a hereditary autosomal recessive disorder of copper metabolism. The corresponding gene locus has been localized on the long arm of chromosome 13. Three different clinical variants of the disease can be distinguished: hepato-cerebral, abdominal/hepatic, and central nervous type. The heterogeneity of symptoms can cause problems in differential diagnosis, especially when another concordant disorder can also explain the pathogenesis of symptoms. The case report of a young man who suffered from brainstem contusion demonstrates the possibilities of misinterpretation because presenting symptoms could be attributed either to traumatic brain injury followed by adjustment disorder or Wilson's disease. Clinical signs included leftsided hemiparesis, bilateral gaze direction nystagmus, marked dysarthria with consecutive pervasive mutism, choreo-athetoid movements, spasmodic torticollis and diplopia dependent on gaze direction. Slit lamp examination showed Kayser-Fleischer's corneal ring. EEG- and computer assisted tomography investigations revealed non-specific findings. The patient was treated with D-Penicillamine. Alternative treatment with oral zinc preparations is discussed.
Bonow, Robert H; Bales, James W; Morton, Ryan P; Levitt, Michael R; Zhang, Fangyi
Intracranial hypotension is a rare condition caused by spontaneous or iatrogenic CSF leaks that alter normal CSF dynamics. Symptoms range from mild headaches to transtentorial herniation, coma, and death. Duret hemorrhages have been reported to occur in some patients with this condition and are traditionally believed to be associated with a poor neurological outcome. A 73-year-old man with a remote history of spinal fusion presented with syncope and was found to have small subdural hematomas on head CT studies. He was managed nonoperatively and discharged with a Glasgow Coma Scale score of 15, only to return 3 days later with obtundation, fixed downward gaze, anisocoria, and absent cranial nerve reflexes. A CT scan showed Duret hemorrhages and subtle enlargement of the subdural hematomas, though the hematomas remained too small to account for his poor clinical condition. Magnetic resonance imaging of the spine revealed a large lumbar pseudomeningocele in the area of prior fusion. His condition dramatically improved when he was placed in the Trendelenburg position and underwent repair of the pseudomeningocele. He was kept flat for 7 days and was ultimately discharged in good condition. On long-term follow-up, his only identifiable deficit was diplopia due to an internuclear ophthalmoplegia. Intracranial hypotension is a rare condition that can cause profound morbidity, including tonsillar herniation and brainstem hemorrhage. With proper identification and treatment of the CSF leak, patients can make functional recoveries.
Imane, Mouhoub; Asmae, Maadane; Toufik, Ramdani; Rachid, Sekhsoukh
Arnold Chiari malformation type 1 is defined as a herniation of the cerebellar tonsils into the foramen magnum of more than 5 mm. Symptoms are most commonly dominated by occipital headache, torticollis and sometimes swallowing disorders. Ophthalmologically abnormal convergences, oculomotor palsy and diplopia are the main clinical signs. We report the case of a 9 year old child, who presented with visual loss evolving since 6 months. Ophthalmologic examination showed visual acuity of 4/10 in both eyes, retained ocular motility and rotational nystagmus. The examination of the anterior segment of the eye showed megalocornea with no evidence of goniodysgenesis, iridodonesis associated with atrophy of the dilator muscle and microcoria with lazy photomotor reflex. Normal intraocular pressure was 14 mmHg. Ocular fundus examination, despite difficulties in performing it, objectified bilateral papilledema (stage II). General physical examination showed torticollis, scoliosis and a tetra-pyramidal syndrome. MRI showed Chiari malformation type I associated with hydrocephalus and syringomyelia. Neurosurgical intervention based on internal CSF drainage with occipitocervical osteo-dural decompression was proposed. The evolution was favorable with regression of clinical signs. Ophthalmologically, there was a regression of papilledema but visual acuity remained stationary. The occurrence of papilledema associated with Chiari malformation type 1 is rare, it has been only reported in 2% of symptomatic patients. Its pathophysiology is still poorly understood. The originality of our study consists in the association of cerebellar malformations with ocular malformations including megalocornea and microcoria which make ophthalmologic examination more difficult to perform.
Ekker, Merel S; Janssen, Sabine; Seppi, Klaus; Poewe, Werner; de Vries, Nienke M; Theelen, Thomas; Nonnekes, Jorik; Bloem, Bastiaan R
Patients with Parkinson's disease (PD) often compensate for their motor deficits by guiding their movements visually. A wide range of ocular and visual disorders threatens the patients' ability to benefit optimally from visual feedback. These disorders are common in patients with PD, yet they have received little attention in both research and clinical practice, leading to unnecessary - but possibly treatable - disability. Based on a literature search covering 50 years, we review the range of ocular and visual disorders in patients with PD, and classify these according to anatomical structures of the visual pathway. We discuss six common disorders in more detail: dry eyes; diplopia; glaucoma and glaucoma-like visual problems; impaired contrast and colour vision; visuospatial and visuoperceptual impairments; and visual hallucinations. In addition, we review the effects of PD-related pharmacological and surgical treatments on visual function, and we offer practical recommendations for clinical management. Greater awareness and early recognition of ocular and visual problems in PD might enable timely instalment of tailored treatments, leading to improved patient safety, greater independence, and better quality of life.
Kong, Xiangyi; Li, Dongmei; Kong, Yanguo; Zhong, Dingrong
Abstract Adenohypophysis spindle cell oncocytoma (ASCO) is a rare tumor recently reported by Roncaroli et al in 2002. This tumor is considered a grade I tumor by the World Health Organization. We report a rare case of malignant ASCO with repeating recurrences and a high Ki-67 index—a challenging diagnosis guided by clinical presentations, radiological signs, and postoperative pathological tests. We represent a 30-year-old man who had suffered from headaches, diplopia, and impaired visual field and acuity. His magnetic resonance imaging revealed an abnormal sellar mass and was originally misdiagnosed as a pituitary macroadenoma. We present detailed analysis of the patient's disease course and review relevant literature. When surgically treated, the specimen revealed a typical histopathology pattern of ASCO. The tumor recurred for several times and the patient underwent 3 surgeries and 1 γ-knife treatment, which was accompanied by a continuously increasing Ki-67 index. This is the first reported case of malignant ASCO (WHO III–IV grade). Despite its rarity, ASCO should be considered in the differential diagnosis of sellar lesions that mimic pituitary adenomas. PMID:28121922
Casimiro de Deus Cardoso, Cejana; Giordani, Adelmo Jose; Borri Wolosker, Angela Maria; Souhami, Luis; Gois Manso, Paulo; Souza Dias, Rodrigo; Comodo Segreto, Helena Regina; Araujo Segreto, Roberto
Purpose: To evaluate the clinical and radiologic response of patients with Graves' ophthalmopathy given low-dose orbital radiotherapy (RT) with a protracted fractionation. Methods and Materials: Eighteen patients (36 orbits) received orbital RT with a total dose of 10 Gy, fractionated in 1 Gy once a week over 10 weeks. Of these, 9 patients received steroid therapy as well. Patients were evaluated clinically and radiologically at 6 months after treatment. Clinical response assessment was carried out using three criteria: by physical examination, by a modified clinical activity score, and by a verbal questionnaire considering the 10 most common signs and symptoms of the disease. Radiologic response was assessed by magnetic resonance imaging. Results: Improvement in ocular pain, palpebral edema, visual acuity, and ocular motility was observed in all patients. Significant decrease in symptoms such as tearing (p < 0.001) diplopia (p = 0.008), conjunctival hyperemia (p = 0.002), and ocular grittiness (p = 0.031) also occurred. Magnetic resonance imaging showed decrease in ocular muscle thickness and in the intensity of the T2 sequence signal in the majority of patients. Treatments were well tolerated, and to date no complications from treatment have been observed. There was no statistical difference in clinical and radiologic response between patients receiving RT alone and those receiving RT plus steroid therapy. Conclusion: RT delivered in at a low dose and in a protracted scheme should be considered as a useful therapeutic option for patients with Graves' ophthalmopathy.
Neuro-ophthalmology focuses on the diagnosis and treatment of visual disorders related to the neurological system rather than the globe itself. Being a subspecialty of both neurology and ophthalmology, it requires specialized training and expertise in diseases of the eye, brain, nerves and muscles. Commonly encountered pathologies in neuro-ophthalmology include: optic neuropathies (such as optic neuritis and ischemic optic neuropathy), visual field loss (transient, constant, unexplained), transient visual loss, unspecified visual disturbances, diplopia, abnormal eye movements, thyroid eye disease, myasthenia gravis, anisocoria, and eyelid abnormalities. The current issue of "Harefuah" is dedicated to contemporary knowledge in neuro-opthalmology, and spans from studies of neuromyelitis optica (NMO), ischemic optic neuropathies, and optic neuropathies induced by phosphodiesterase inhibitors, to the management of sight-threatening carotid-cavernous fistulas, and more. These studies emphasize the importance of an interdisciplinary treatment team consisting of a neuro-ophthalmologist, a neuro-radiologist, and sometimes, even a neuro-surgeon. Such an approach may prove to be beneficial to the patient, by optimizing follow-up and treatment decisions. This issue emphasizes how a correct and timely diagnosis is of paramount significance in patients with neuro-ophthalmological disorders.
Wu, Chris Y; Archer, Steven M; Kahana, Alon
Carcinoid tumors are rare, slow-growing, low-grade neuroendocrine tumors with a propensity for orbital metastatic spread. The typical treatment paradigm for localized orbital disease involves excision, adjuvant radiotherapy, and/or receptor-targeted chemotherapy, followed by delayed evaluation for reconstructive strabismus surgery. We present a 58-year-old female patient with carcinoid tumor metastatic to the right inferior rectus muscle who presented with worsening binocular diplopia. The patient underwent coordinated "tag-team" orbital and strabismus surgeries that included excision of the right inferior rectus muscle to the annulus of Zinn followed immediately by reconstructive strabismus surgery. The patient required 1 additional strabismus surgery 1 year later. Follow up revealed no tumor recurrence at 4 years, and excellent binocular vision with good function. Deep orbital and strabismus surgeries, when performed simultaneously in a "tag-team" approach, may offer superior functional outcomes and improved patient quality of life, with expedited functional recovery. This approach may become a new treatment paradigm for surgical disease processes localized to the extraocular muscles.
Malec, Katarzyna; Gasiński, Mateusz; Kuchta, Krzysztof; Kozok, Andrzej
Gunshot injuries of the viscerocranium are rarely reported during times of peace in Europe. Penetrating wounds to the maxillofacial region pose a significant challenge for surgeons as they often comprise serious soft and bone tissue defects.We present a case report of 38-year-old male with gunshot wound to the viscerocranium after suicidal attempt. The patient's general condition was stable. The inlet wound was found in the submental region in the central line penetrating deep into the floor of the mouth, to the left, avoiding large vessels and hypoglossal nerve. No exit wound was identified. The ophthalmic examination revealed the limitation of motion in the left eyeball and diplopia in the whole field of vision. The revision was performed under general anesthesia. Control CT scan revealed the presence of one metallic fragment wedged in the hard palate. Second look of oral cavity with particular emphasis on the hard palate was performed. Shrapnel proved to be wedged in the bone of the hard palate very firmly and complete removal without damaging the function of the palate was impossible. The decision was made to withdraw from surgical removal of the remaining piece of the projectile. In most cases, it is recommended to remove all foreign material from human body. However, in the illustrated case we decided to leave small debris in the craniofacial skeleton. In our opinion, further surgical revision would result in greater tissue damage, disproportionate to the benefits of the removal of all fragments of the projectile.
Al-Harbi, Talal; Al-Sarawi, Adnan; Binfalah, Mohamed; Dermime, Said
Neuromyelitis optica (NMO), or Devic's syndrome, is an autoimmune central nervous system demyelinating disorder primarily affecting the spinal cord and the optic nerves. It is characterized by the presence of NMO antibodies, alongside clinical and radiological findings. NMO and NMO-spectrum disorders (NMO-SD) have been reported in autoimmune disorders, and are infrequently described as a paraneoplastic syndrome with cancers of lung, breast, and carcinoid tumors of the thyroid. We report a patient who presented with severe vomiting, blurring of vision, vertigo, diplopia, left hemiparesis and hemisensory loss and ataxia. She was found to have a longitudinally-extensive demyelinating lesion extending from the medulla to the upper cervical spinal cord on MRI. Her gastric endoscopy revealed carcinoid tumor of the stomach, and classic paraneoplastic antibodies in the serum were negative. She had extremely high serum gastrin level and high titer of NMO IgG autoantibody. The patient made an excellent recovery with tumor resection and immunotherapy, with both clinical and radiological improvement. On rare instances, NMO or NMO-SD may present as a paraneoplastic neurological syndrome associated with carcinoid tumor of the stomach.
Laria, Carlos; Merino-Suárez, María L; Piñero, David P; Gómez-Hurtado, Arantxa; Pérez-Cambrodí, Rafael J
We describe the case of an eight-year-old girl with complaints of headaches and blurred vision (uncorrected visual acuity: 0.1 decimal) that showed on examination miotic pupils, pseudomyopia, no ocular motility restrictions, and no associated neurological disease. After initial treatment with cyclopentolate for two months, pseudomyopia persisted with an intermittent and variable esotropia. Spectacles of +1 both eyes and atropine 1% one drop daily were then prescribed. The situation improved and remained stable for several weeks, with pseudomyopia and esotropia reappearing later. Finally, botulinum toxin (2.5 iu Botox) was injected in the medial rectus muscle on two occasions and a visual therapy program based on the stimulation of fusional divergence, diplopia, and stereopsis consciousness was recommended. This prescription was combined with the use of atropine during the first few weeks. Orthotropia and corrected distance visual acuity of 1.0 were found three months after treatment. The evolution and clinical results of this case report suggest that botulinum toxin in combination with other therapeutic alternatives may be useful in the treatment of spasm of the near reflex.
Hamad, Muddathir H; Alwadei, Ali H; Bashiri, Fahad A; Hassan, Hamdy H; Idris, Hiyam; Hassan, Saeed; Muayqil, Taim; Altweijri, Ikhlass; Salih, Mustafa A
Idiopathic intracranial hypertension (IIH) is a rare neurological disorder in children. It is characterized by raised intracranial pressure (ICP) in the absence of brain parenchymal lesion, vascular malformations, hydrocephalus, or central nervous system (CNS) infection. The diagnosis is usually confirmed by high opening pressure of cerebrospinal fluid (CSF) with exclusion of secondary causes of intracranial hypertension. If not treated properly, it may lead to severe visual dysfunction. Here we review the etiology, clinical presentation, diagnostic criteria and management of IIH in children through illustration of the clinical and radiological presentation of a 13-year-old overweight girl who presented with severe headache, diplopia and bilateral papilledema. Otherwise, she had unremarkable neurological and systemic examinations. Lumbar puncture showed a high CSF opening pressure (360–540 mmH2O). Her investigations showed normal complete blood count (CBC), normal renal, liver, and thyroid function tests. Cerebrospinal fluid (CSF) and blood chemistry were unremarkable. Magnetic resonant image (MRI) of the brain demonstrated empty sella turcica, tortuous optic nerves, and flattening of the posterior sclera. Magnetic resonant venography (MRV) showed focal narrowing of the distal transverse sinuses and absence of venous sinus thrombosis. She required treatment with acetazolamide and prednisolone. With medical treatment, weight reduction, and exercise, our patient had a remarkable improvement in her symptoms with resolution of papilledema in two months. This review highlights the importance of early recognition and management of IIH to prevent permanent visual loss. PMID:28096561
Mittal, Shivam Om; Siddiqui, Junaid; Katirji, Bashar
Isolated unilateral abducens nerve palsy is usually due to ischemia, trauma or neoplasm. Dorello's canal is the space between the petrous apex and superolateral portion of the clivus, bound superiorly by Gruber's ligament. The abducens nerve travels with inferior petrosal sinus (IPS) though the Dorello's canal before entering the cavernous sinus. A 31-year-old man presented with neck pain, and binocular horizontal diplopia, worse looking towards left and at distance. He had a history of intravenous drug abuse but no history of hypertension or diabetes. On examination, he had complete left 6th nerve palsy with normal fundi, pupils, and other cranial nerves. Methicillin-resistant Staphylococcus aureus bacteremia was detected with naïve tricuspid valve endocarditis and multiple septic emboli to lungs with infarcts. His cerebrospinal fluid was normal. MRI of the brain was normal. MRV of head and neck showed thrombosis of the left internal jugular vein, left sigmoid sinus and left inferior petrosal sinus with normal cavernous sinus and no evidence of mastoiditis. He was treated with broad spectrum antibiotics. He was not anticoagulated for fear of pulmonary hemorrhage from pulmonary infarcts. Although cerebral venous sinus thrombosis commonly presents with elevated intracranial pressure, isolated ipsilateral 6th nerve palsy from its compression in Dorello's canal due to thrombosis of the ipsilateral inferior petrosal sinus is extremely rare. To our knowledge, only two patients have been reported with isolated abducens palsy due to IPS thrombosis; one caused by septic emboli and the other developed it during IPS cortisol level sampling.
Van de Velde, Frans J.; Tassignon, Marie J.; Trau, Rene
We present a new technique, scanning laser retinoscopy, to spatially resolve in two dimensions the optical aberrations and refractive power of the ocular media. For this purpose, the Maxwellian view of a confocal scanning laser ophthalmoscope (SLO) is configured to scan simultaneously the posterior and the anterior segment of the eye at different levels of prefocussing. This set-up allows retinal imaging and psychophysics through different optical zones of the cornea and lens. In addition, the size of the anatomical pupil can be dynamically controlled by adjusting the colinear infrared and visible light intensities of the illuminating system. In retinoscopic images we can see a part of the retina superimposed by distinctive patterns of shadows in the pupillary area. The variable patterns of shadows in the retinoscopic images change with the level of prefocussing of the SLO. The patterns are the result of local variations in refraction or wavefront aberrations within the lens and cornea. In cases of excimer laser refractive surgery, for example, scanning laser retinoscopy is able to distinguish between a treated central area, transition zone and peripheral cornea. As a corollary, we can document differences between excimer laser delivery systems and also correlate the retinoscopic images with the subjective complaints of refractive surgery patients. These include monocular diplopia, glare, loss of contrast sensitivity besides reduced visual acuity.
Primary central nervous system lymphoma (PCNSL) is an extranodal Non-Hodgkin's lymphoma that is confined to the brain, eyes, and/or leptomeninges without evidence of a systemic primary tumor. Although the tumor can affect all age groups, it is rare in childhood; thus, its incidence and prognosis in children have not been well defined and the best treatment strategy remains unclear. A nine-year old presented at our department with complaints of diplopia, dizziness, dysarthria, and right side hemiparesis. Magnetic resonance image suggested a diffuse brain stem glioma with infiltration into the right cerebellar peduncle. The patient was surgically treated by craniotomy and frameless stereotactic-guided biopsy, and unexpectedly, the histopathology of the mass was consistent with diffuse large B cell lymphoma, and immunohistochemical staining revealed positivity for CD20 and CD79a. Accordingly, we performed a staging work-up for systemic lymphoma, but no evidence of lymphoma elsewhere in the body was obtained. In addition, she had a negative serologic finding for human immunodeficient virus, which confirmed the histopathological diagnosis of PCNSL. She was treated by radiosurgery at 12 Gy and subsequent adjuvant combination chemotherapy based on high dose methotrexate. Unfortunately, 10 months after the tissue-based diagnosis, she succumbed due to an acute hydrocephalic crisis. PMID:27867930
Liao, Huang-I; Wang, Chun-Chieh; Wei, Kuo-Cheng; Chang, Cheng-Nen; Hsu, Yung-Hsin; Lee, Shih-Tseng; Huang, Yin-Cheng; Chen, Hsien-Chih; Hsu, Peng-Wei
Radiosurgery has been proven to be an effective treatment for residual or recurrent pituitary adenomas after surgery. However, it causes severe complications when the optic apparatus is irradiated over the tolerance dose. In this study, we analyzed the feasibility of fractionated stereotactic radiosurgery to treat pituitary tumors close to the optic apparatus. Thirty-four patients from June 2006 to June 2011 with recurrent or residual pituitary adenomas close to (<3 mm) the optic apparatus were treated with fractionated stereotactic radiosurgery. Three fractions with a total dose of 2100 cGy were applied to the tumors. Imaging, examination of vision, and estimation of hormone level were regularly performed before and after radiosurgery. The mean tumor volume before fractioned stereotactic radiosurgery was 5.06±3.08 cm3 (range: 0.82-12.69 cm3). After a mean follow up of 36.8±15.7 months (range: 16-72 months), tumor size was reduced in seven (20.6%) patients and remained the same in the other 27 (79.4%) patients. Vision was improved in one patient and remained stable in the rest. Only one patient developed transient post-treatment diplopia. This study suggests that fractionated stereotactic radiosurgery is safe for treating pituitary adenomas close to the optic apparatus. Studies with more patients and longer follow-up are required to draw definite conclusions.
Objective. To evaluate the success rate of intra-rectal (IR) midazolam in combination with nitrous oxide/oxygen (N2O) sedation in young uncooperative dental patients when the local anesthesia is delivered by a computerized controlled local anesthetic delivery (C-CLAD). Study Design. This observational study consisted of 219 uncooperative children (age: 4.3 ± 1.69 y) who received IR midazolam (0.4 mg/kg) and N2O to complete their dental treatment. Measured variables included: child’s pain disruptive behavior during delivery of anesthesia by C-CLAD (CHEOP Scale), child behavior during treatment (Houpt scale), dental procedure performed, and side effects that appeared during treatment. Results. There was a high level of cooperation (mean score: 6.69 ± 2.1) during administration of local anesthesia. Good-to-excellent behavior was shown by 87% of the children during treatment. Planned treatment was completed by 184 (92%) patients. No statistically significant changes were noticed in the oxygen saturation levels before and after treatment. Children with side effects included 3 (1.3%) with nistagmus, 5 (2.3%) with diplopia, and 18 (8.2%) with hiccups. Three consecutive sedations decreased the overall behavior score by 5.7% compared to the first appointment (p < .05). Conclusions. IR midazolam-N2O sedation in combination with C-CLAD is very effective for delivery of dental treatment to young uncooperative children. PMID:24688838
Cannon, Richard B.; Wiggins, Richard H.; Witt, Benjamin L.; Dundar, Yusuf; Johnston, Tawni M.; Hunt, Jason P.
Objectives Low-grade sinonasal sarcoma with neural and myogenic features (LGSSNMF) is a new, rare tumor. Our goal is to describe the imaging characteristics and surgical outcomes of this unique skull base malignancy. Design Retrospective case series. Setting Academic medical center. Participants There were three patients who met inclusion criteria with a confirmed LGSSNMF. Main Outcome Measures Imaging and histopathological characteristics, treatments, survival and recurrence outcomes, complications, morbidity, and mortality. Results Patients presented with diplopia, facial discomfort, a supraorbital mass, and nasal obstruction. Magnetic resonance imaging and computed tomography imaging in all cases showed an enhancing sinonasal mass with associated hyperostotic bone formation that involved the frontal sinus, invaded the lamina papyracea and anterior skull base, and had intracranial extension. One patient underwent a purely endoscopic surgical resection and the second underwent a craniofacial resection, while the last is pending treatment. All patients recovered well, without morbidity or long-term complications, and are currently without evidence of disease (mean follow-up of 2.1 years). One patient recurred after 17 months and underwent a repeat endoscopic skull base and dural resection. Conclusions The surgical outcomes and imaging of this unique, locally aggressive skull base tumor are characterized. PMID:28229035
Shimizu, Kiyoharu; Yuki, Kiyoshi; Sadatomo, Takashi; Kurisu, Kaoru
Background: As an extremely rare subtype of sarcoidosis that develops exclusively in the nervous system, isolated neurosarcoidosis is difficult to diagnose. In addition, its exact clinical features are not known. Case Description: A 61-year-old man presented with right ear hearing loss, diplopia, and fever. Computed tomography (CT) and magnetic resonance imaging revealed mass lesions in the right cerebellum and left side body of the lateral ventricle. Neither systemic CT nor positron emission tomography revealed extracranial lesions. A neuroendoscopic biopsy was performed on the lateral ventricle lesion, and a histopathology analysis revealed epithelioid granulomatous inflammation. By systematic exclusion of other possible granulomatous diseases, isolated neurosarcoidosis was diagnosed. The lesions disappeared immediately upon corticosteroid (methylprednisolone) treatment and had not recurred as of a 12-month follow-up examination. Conclusions: Isolated neurosarcoidosis is difficult to diagnose. Successful diagnosis requires compatible clinical findings, histological demonstration of noncaseating granulomas, and exclusion of other granulomatous diseases. Isolated neurosarcoidosis has a relatively good clinical prognosis, which could be characteristic of the disease. PMID:27168947
Chuka, Okosa Michael; Darlinton, Anyiam Daniel Chukwuemeka
Introduction: Orbital masses in adults are often caused by systemic diseases or are associated with systemic manifestations. Juvenile xanthogranuloma as a cause is rare and unreported in Africa. We present clinical features, management, and outcomes of bilateral orbital adult onset juvenile xanthogranuloma. Case Presentation: A 27 year old Nigerian woman presented with bilateral upper-lid lumps having lasted 5 months. These increased in size for about 1 month and stopped. Lid swelling was preceded by itchy eyes, redness of conjunctiva, and occasional mild pain. There were no visual or systemic symptoms. The lumps were firm, slightly mobile, not tender, and not attached to skin but rather to deeper structures. There was restriction on up-gaze but no proptosis or diplopia. Hematological, biochemical, and X-ray investigations were normal. Prednisolone tablets 10 mg daily for two weeks were not useful. Tissue biopsy was invaluable in diagnosis of this rare condition and disclosed juvenile xanthogranuloma. Partial surgical excision was done under lidocaine infiltration. No recurrence has occurred in 40 months of follow-up. No systemic disease has manifested. Conclusion: Juvenile xanthogranuloma can present as bilateral superior orbital tumor in adults; functional and cosmetic aims were achieved by sub-total excision. PMID:23071425
Kazim, M.; Trokel, S.; Acaroglu, G.; Elliott, A.
AIMS—To document the successful treatment of five patients with dysthyroid optic neuropathy by orbital fat decompression instead of orbital bone decompression after failed medical therapy. METHODS—Eight orbits of five patients with dysthyroid optic neuropathy were selected for orbital fat decompression as an alternative to bone removal decompression. Treatment with systemic corticosteroids and/or orbital radiotherapy was either unsuccessful or contraindicated in each case. All patients satisfied clinical indications for orbital bone decompression to reverse the optic neuropathy. High resolution computerised tomographic (CT) scans were performed in all cases and in each case showed signs of enlargement of the orbital fat compartment. As an alternative to bone decompression, orbital fat decompression was performed on all eight orbits. RESULTS—Orbital fat decompression was performed on five patients (eight orbits) with optic neuropathy. Optic neuropathy was reversed in all cases. There were no cases of postoperative diplopia, enophthalmos, globe ptosis, or anaesthesia. All patients were followed for a minimum of 1 year. CONCLUSIONS—In a subset of patients with an enlarged orbital fat compartment and in whom extraocular muscle enlargement is not the solitary cause of optic neuropathy, fat decompression is a surgical alternative to bony decompression. PMID:10837384
Borgman, Christopher J.
Some reports have outlined many different treatment strategies for superior oblique myokymia (SOM) that attempt to reduce or eliminate patients’ symptoms of monocular oscillopsia and/or diplopia. Most treatment strategies have focused solely on oral medications or invasive surgery. The following is a current and critical review of SOM along with its clinical findings/symptoms, demographics, theories of its pathogenesis, management/treatment options, new observations in SOM, as well as a case report to highlight a relatively novel idea for the treatment of SOM: topical timolol eye drops. It also highlights evidence of a new “localized theory” regarding topical β-blockers’ mechanism of action in SOM compared to the previous systemic hypothesis proposed in 1994. The case report shows a 29-year-old female patient who suffered with SOM symptoms for 8–10 years and then experienced a worsening of her symptoms shortly postpartum. The patient was prescribed topical timolol eye drops by the author in the affected eye based on one case report12 from 1994, which completely eliminated her symptoms within 1–2 days of starting the treatment with any recurrence with the daily use of the drop at the time of this article. Given the robust effect in this case, topical timolol may be a potentially useful drug in the management of SOM given its affordability and safer side effect profile in comparison to the oral medications traditionally used in SOM. PMID:24766862
Cağdaş, Deniz; Ozgür, Tuba Turul; Asal, Gülten Türkkanı; Tezcan, Ilhan; Metin, Ayşe; Lambert, Nathalie; de Saint Basile, Geneiveve; Sanal, Ozden
Griscelli syndrome (GS) is a rare autosomal recessive disorder characterized by partial albinism. Three different types are caused by defects in three different genes. Patients with GS type 1 have primary central nervous system dysfunction, type 2 patients commonly develop hemophagocytic lymphohistiocytosis, and type 3 patients have only partial albinism. While hematopoietic stem cell transplantation is life saving in type 2, no specific therapy is required for types 1 and 3. Patients with GS types 1 and 3 are very rare. To date, only 2 patients with type 3 and about 20 GS type 1 patients, including the patients described as Elejalde syndrome, have been reported. The neurological deficits in Elejalde syndrome were reported as severe neurodevelopmental delay, seizures, hypotonia, and ophthalmological problems including nystagmus, diplopia, and retinal problems. However, none of these patients' clinical progresses were reported. We described here our two new type 1 and two type 3 patients along with the progresses of our previously diagnosed patients with GS types 1 and 3. Our previous patient with GS type I is alive at age 21 without any other problems except severe mental and motor retardation, patients with type 3 are healthy at ages 21 and 24 years having only pigmentary dilution; silvery gray hair, eye brows, and eyelashes. Since prognosis, treatment options, and genetic counseling markedly differ among different types, molecular characterization has utmost importance in GS.
Suzuki, Keisuke; Odaka, Masaaki; Tatsumoto, Muneto; Miyamoto, Tomoyuki; Takamatsu, Kazuhiro; Hirata, Koichi
An 88-year-old female with atrial fibrillation and hypertension, was admitted to our hospital with sudden onset diplopia and somnolence. She had right hemiparesis with bilateral positive Babinski's sign. Additionally, there was bilateral blepharoptosis with right esotropia. With regard to extraocular movement, the patient demonstrated conjugate upgaze palsy and left monocular down gaze palsy (vertical one-and-a-half syndrome: VOHS). Horizontal gaze in the left eye was completely impaired and there was limited abduction of the right eye. Magnetic resonance imaging of the brain showed left thalamo-mesencephalic infarction. On day 4, the vertical eye movement developed into conjugate upgaze and down- gaze palsies. Magnetic resonance imaging of the brain indicated high signal lesion extending into the dorsal portion of the midbrain. It was suggested that the pathway to contralateral downgaze neurons could have been damaged due to the unilateral (left) dosal midbrain lesion before its decussation with the unilateral interstitial nucleus of Cajal, the oculomotor nucleus and the rostral interstitial nucleus of the medial longitudinal fasciculus. This case is considered to be important because there has been no previous report of bilateral vertical gaze palsy due to VOHS in the same patient. Since there are various patterns of ocular movement disorder in the thalamo-mesencephalic portion, careful observations are required to localize the lesions.
Wali, R K; Lee, A H; Kam, J C; Jonsson, J; Thatcher, A; Poretz, D; Ambardar, S; Piper, J; Lynch, C; Kulkarni, S; Cochran, J; Djurkovic, S
We report the first case of enterovirus-D68 infection in an adult living-donor kidney transplant recipient who developed rapidly progressive bulbar weakness and acute flaccid limb paralysis following an upper respiratory infection. We present a 45-year-old gentleman who underwent pre-emptive living-donor kidney transplantation for IgA nephropathy. Eight weeks following transplantation, he developed an acute respiratory illness from enterovirus/rhinovirus that was detectable in nasopharyngeal (NP) swabs. Within 24 h of onset of respiratory symptoms, the patient developed binocular diplopia which rapidly progressed to multiple cranial nerve dysfunctions (acute bulbar syndrome) over the next 24 h. Within the next 48 h, asymmetric flaccid paralysis of the left arm and urinary retention developed. While his neurological symptoms were evolving, the Centers for Disease Control reported that the enterovirus strain from the NP swabs was, in fact, Enterovirus-D68 (EV-D68). Magnetic resonance imaging of the brain demonstrated unique gray matter and anterior horn cell changes in the midbrain and spinal cord, respectively. Constellation of these neurological symptoms and signs was suggestive for postinfectious encephalomyelitis (acute disseminated encephalomyelitis [ADEM]) from EV-D68. Treatment based on the principles of ADEM included intensive physical therapy and other supportive measures, which resulted in a steady albeit slow improvement in his left arm and bulbar weakness, while maintaining stable allograft function.
Novy, Jan; Patsalos, Philip N; Sander, Josemir W; Sisodiya, Sanjay M
Lacosamide is a new antiepileptic drug (AED) apparently devoid of major pharmacokinetic interactions. Data from a small postmarketing assessment suggest people who had lacosamide co-prescribed with a voltage-gated sodium channel (VGSC)-blocking AED seemed more likely to discontinue lacosamide because of tolerability problems. Among 39 people with refractory epilepsy who developed neurotoxicity (diplopia, dizziness, drowsiness) on lacosamide treatment given in combination with VGSC-blocking AEDs, we identified 7 (17.9%) without any changes in serum levels of other AEDs in whom the symptoms were ameliorated by dose reduction of the concomitant VGSC-blocking AED. Symptoms in these people seem to have arisen from a pharmacodynamic interaction between lacosamide and other VGSC-blocking AEDs. Slow-inactivated VGSCs targeted by lacosamide might be more sensitive to the effects of conventional VGSC-blocking AEDs. Advising people to reduce concomitantly the conventional VGSC-blocking AEDs during lacosamide uptitration in cases of neurotoxicity might improve the tolerability of combination treatment.
Le Fèvre, C; Castelli, J; Perrin, C; Hénaux, P L; Noël, G
Malignant peripheral nerve sheath tumours are extremely rare and can be associated with neurofibramatosis type 1. Their prognosis is poor and surgery remains the mainstay of therapy and should be the first line of treatment. Radiotherapy and chemotherapy are second line treatment and their effectiveness remains to demonstrate. The diagnosis is clinical, radiological, histological and immunohistochemical. Malignant peripheral nerve sheath tumours have a potential of local tumour recurrence very high and can metastasize. They often occur in extremity of the members but also rarely into brain. We report two cases of intracerebral nerve sheath tumour. The first was a 68-year-old woman who was admitted with progressive symptoms of headache and diplopia. A left frontotemporal malignant peripheral nerve sheath tumours was diagnosed and was treated by surgery and irradiation. Ten months later, she presented a local recurrence and spine bone's metastases were treated by vertebroplasty and irradiation. The patient died 15 months after the diagnosis. The second case was a 47-year-old woman who was referred because headache and vomiting symptoms. A right frontal malignant peripheral nerve sheath tumours was diagnosed and treated by surgery and irradiation. After that, the patient had three local recurrence operated and pulmonary and cranial bone's metastases. She was still alive after 20 months. We propose a literature review with 25 cases of intracerebral nerve sheath tumour identified, including the two current cases.
Gatti, C; Oelher, E; Legrand, A M
We present a retrospective study of 129 medical files concerning seafood poisonings (SFPs) registered at the central hospital of Tahiti (French Polynesia) between 1999 and 2005. Even if during that period most of the described cases (96%) concerned the ichtyosarcotoxism ciguatera, it is interesting to note that we also registered three other SFPs: tetrodotoxism, carchatoxism and lyngbyatoxism due to the consumption of tetraodon/diodon species, sharks or sea turtles, respectively. In ciguatera, cardiovascular symptoms were the primary criteria of severity with bradycardia and hypotension observed at 75% and 43%, respectively. Neurological manifestations (such as cerebellar syndrome, language troubles, diplopia or polyradiculoneuritis), trouble and/or loss of consciousness and dyspnoea were secondary criteria of severity. Body temperature was reported under 36.5 degrees C in 48 of 80 documented files. This observation, which has not previously been described in humans, may be related to possible central effects of the ingested toxin. The last remark concerns two extremely severe cases of ciguatera fish poisoning in which physicians had suspected an inflammatory neuropathy called the Guillain-Barré syndrome (GBS). Even if it is premature to conclude any correlation between the intoxication and the appearance of GBS, it is interesting to note that in both pathologies, morphological disturbances of nerve fibres have been reported.
Bansal, Sumit; Suri, Ashish; Singh, Manmohan; Kale, Shashank Sharad; Agarwal, Deepak; Sharma, Manish Singh; Mahapatra, Ashok Kumar; Sharma, Bhawani Shankar
Cavernous sinus hemangioma (CSH) is a rare extra-axial vascular neoplasm that accounts for 2% to 3% of all cavernous sinus tumors. Their location, propensity for profuse bleeding during surgery, and relationship to complex neurovascular structures are factors which present difficulty in excising these lesions. The authors describe their experience of 22 patients with CSH over 14 years at a tertiary care center. Patients were managed with microsurgical resection using a purely extradural transcavernous approach (13 patients) and with Gamma Knife radiosurgery (GKRS; Elekta AB, Stockholm, Sweden) (nine patients). Retrospective data analysis found headache and visual impairment were the most common presenting complaints, followed by facial hypesthesia and diplopia. All but one patient had complete tumor excision in the surgical series. Transient ophthalmoparesis (complete resolution in 6-8 weeks) was the most common surgical complication. In the GKRS group, marked tumor shrinkage (>50% tumor volume reduction) was achieved in two patients, slight shrinkage in five and no change in two patients, with symptom improvement in the majority of patients. To our knowledge, we describe one of the largest series of CSH managed at a single center. Although microsurgical resection using an extradural transcavernous approach is considered the treatment of choice in CSH and allows complete excision with minimal mortality and long-term morbidity, GKRS is an additional tool for treating residual symptomatic lesions or in patients with associated comorbidities making surgical resection unsuitable.
Arishima, Hidetaka; Kikuta, Ken-ichiro
If the origin of isolated abducent nerve palsy cannot be found on neuroradiological examinations, diabetes mellitus is known as a probable cause; however, some cases show no potential causes of isolated abducent nerve palsy. Here, we report a 74-year-old male who suffered from diplopia due to isolated left abducent nerve palsy. Magnetic resonance angiography and fast imaging employing steady-state acquisition imaging clearly showed a dolichoectasic vertebrobasilar artery compressing the left abducent nerve upward and outward. There were no abnormal lesions in the brain stem, cavernous sinus, or orbital cavity. Laboratory data showed no abnormal findings. We concluded that neurovascular compression of the left abducent nerve might cause isolated left abducent nerve palsy. We observed him without surgical treatment considering his general condition with angina pectoris and old age. His symptom due to the left abducent nerve palsy persisted. From previous reports, conservative treatment could not improve abducent nerve palsy. Microvascular decompression should be considered for abducent nerve palsy due to vascular compression if patients are young, and their general condition is good. We also discuss interesting characteristics with a review of the literature. PMID:28149097
Chang, Melinda Y.; Pineles, Stacy L.; Velez, Federico G.
PURPOSE To evaluate the effectiveness of adjustable small-incision selective tenotomy and plication of vertical rectus muscles in correcting vertical strabismus incomitant in horizontal gaze positions and cyclotorsion. METHODS The medical records of all patients who underwent adjustable small-incision selective tenotomy or plication of a vertical rectus muscle for correction of horizontally incomitant vertical strabismus or cyclotorsion by a single surgeon at a single eye institute from July 2013 to September 2014 were retrospectively reviewed. Selective tenotomy and plication were performed on either the nasal or temporal side of vertical rectus muscles, based on the direction of cyclotorsion and incomitance of vertical strabismus. RESULTS Of 9 patients identified, 8 (89%) had successful correction of horizontally incomitant vertical strabismus, with postoperative vertical alignment within 4Δ of orthotropia in primary position, lateral gazes, and downgaze. Of the 8 patients with preoperative cyclotorsion, 4 (50%) were successfully corrected, with <5° of cyclotorsion postoperatively. Of the 4 patients in whom cyclotorsion did not improve, 3 had undergone prior strabismus surgery, and 2 had restrictive strabismus. Eight of the 9 patients (89%) reported postoperative resolution of diplopia. CONCLUSIONS Adjustable small-incision selective tenotomy and plication effectively treat horizontally incomitant vertical strabismus. These surgeries may be less effective for correcting cyclotorsion in patients with restriction or prior strabismus surgery. Advantages are that they may be performed in an adjustable manner and, in some cases, under topical anesthesia. PMID:26486021
Singh, Achintya Dinesh; Soneja, Manish; Memon, Saba Samad; Vyas, Surabhi
A man aged 35 years presented with chronic headache and earache of 1-year duration. He had progressive vision loss and diplopia since last 9 months. He also had pain over the face and episodic profuse epistaxis. On examination, perception of light was absent in the right eye and hand movements were detected at 4 m distance in the left eye. Imaging revealed a lobulated mass in the nasopharynx extending into the bilateral cavernous sinuses and sphenoid sinus with bony erosions. Biopsy of the nasopharyngeal mass revealed pathological features which are characteristic of IgG4 disease. His serum IgG4 levels and acute inflammatory markers were also elevated. The patient was started on oral corticosteroid therapy. Fever, headache and earache resolved early and there was gradual improvement in the vision of the left eye. After 6 months, visual acuity in the left eye was 6/9, but right eye visual acuity had no change. Follow-up imaging revealed a significant reduction in the size of the mass.
Chi, Mijung; Kim, H Jane; Basham, Ryan; Yoon, Michael K; Vagefi, Reza; Kersten, Robert C
Mucormycosis is a rare often fatal opportunistic fungal infection. It is typically described in patients with diabetes in ketoacidotic status and is rare in renal transplant recipients. Calciphylaxis is a rare and highly morbid disease of vascular calcification affecting patients with end-stage renal disease (ESRD). The first case of a renal transplant recipient who was inflicted with both rhinoorbitocerebral mucormycosis and calciphylaxis is reported. A 45-year-old man presented with 2-day history of left upper blepharoptosis, periorbital pain, left-sided headache, binocular diplopia, and left V2 numbness. He had undergone renal transplant for ESRD 7 months earlier with resultant immunosuppressive therapy. MRI and nasal biopsy confirmed rhinoorbitocerebral mucormycosis. Immunosuppressive therapy was stopped and antifungal therapy begun. He had orbital exenteration for progressive rhinoorbitocerebral mucormycosis. Two months later, the patient reported new-onset intermittent bitemporal headache and bilateral swollen, tender temporal arteries. Temporal artery biopsy revealed features consistent with calciphylaxis. Clinical presentation, treatment course, and follow up are discussed.
Fan, Rui; Ji, Ruirui; Zou, Wenxin; Wang, Guoliang; Wang, Hu; Penney, Daniel James; Luo, Jin Jun; Fan, Yuxin
Andersen-Tawil syndrome (ATS) is an autosomal dominant, multisystem channelopathy characterized by periodic paralysis, ventricular arrhythmias and distinctive dysmorphic facial or skeletal features. The disorder displays marked intrafamilial variability and incomplete penetrance. Myasthenia gravis (MG) is an autoimmune disorder that demonstrates progressive fatigability, in which the nicotinic acetylcholine receptor (AChR) at neuromuscular junctions is the primary autoantigen. The present study reports a rare case of a 31-year-old woman with a history of morbid obesity and periodic weakness, who presented with hemodynamic instability, cardiogenic shock and facial anomalies. Laboratory results revealed hypokalemia and an elevated anti-AChR antibody expression levels. Electrocardiography demonstrated prolonged QT-interval, ST-elevation, and subsequent third-degree atrioventricular block. Neurological examination revealed bilateral ptosis, horizontal diplopia, dysarthria and generalized weakness. No mutations in the potassium channel inwardly rectifying subfamily J member 2 gene were detected in the present case. The patient was treated with oral potassium supplementation and an acetylcholinesterase inhibitor (pyridostigmine), after which the symptoms were improved. To the best of our knowledge, the present case report was the first to describe concomitant presentation of both ATS and MG, which represents a diagnostic and therapeutic challenge. PMID:27698745
Su, Grant W; Hong, Sang H
A 55-year-old woman presented with a 1-week history of vision loss in the right eye associated with proptosis and diplopia. Past medical history was significant for high-grade leiomyosarcoma of the uterus status post total abdominal hysterectomy and bilateral salpingo-oophorectomy and postoperative pelvic radiation 18 months prior to presentation. Staging studies at the time of initial diagnosis of uterine leiomyosarcoma showed no evidence for metastatic disease. At presentation, CT and MRI showed a well-circumscribed 3.0 cm x 3.6 cm x 2.4 cm mass centered in the right greater sphenoid wing, extending into the middle cranial fossa and the superior and lateral orbital wall. Biopsy of the orbital mass revealed a poorly differentiated high-grade leiomyosarcoma, consistent with recurrent metastatic disease from the uterus. The patient subsequently underwent radiation treatment followed by a left orbital exenteration 6 months after the orbital biopsy. A left thoracostomy was performed 8 months after the orbital biopsy for a metastatic nodule in the left lower lobe of the lung. The clinicopathologic findings of this rare metastatic orbital lesion are presented.
Kim, Yeong-Jin; Kim, Hee Kyung; Yang, Deok-Hwan; Jung, Shin; Noh, Myung-Giun; Lee, Jae-Hyuk; Lee, Kyung-Hwa; Moon, Kyung-Sub
Abstract This is the first reported case of pineal lymphoma with concomitant prolactin-producing pituitary adenoma. A 51-year-old male experienced worsening headaches accompanied by nausea, diplopia, and memory loss for 1 month. Cranial nerve examination revealed bilateral upward gaze limitation with convergence impairment, which is known as Parinaud syndrome. Magnetic resonance images revealed a mass in the pineal gland with a coexisting mass within the enlarged sella fossa. Hormone analysis revealed hyperprolactinemia. The pineal mass was removed without injuring the hypothalamus, brain stem, or any neighboring vessels. Pathology examination confirmed the diagnosis of diffuse large B-cell lymphoma (DLBCL) involving the pineal gland. After further studies, the pineal lymphoma was determined to be a secondary tumor from a gastric primary tumor. The patient died 6 months after diagnosis due to systemic progression of DLBCL. Although the mechanistic link between hyperprolactinemia and lymphoma progression has not been clarified on a clinical basis, high prolactin levels may contribute to the rapid progression and therapeutic resistance of the lymphoma. PMID:26937937
Wang, Arthur; Carberry, Nathan; Solli, Elena; Kleinman, George; Tandon, Adesh
We present an unusual case of a metastatic mantle cell lymphoma (MCL) to the pituitary gland. The patient had a known history of MCL for which she previously received chemotherapy. She presented with new-onset diplopia and confusion, and reported a history of progressive vision blurriness associated with headache, nausea, and vomiting. MRI of the brain showed an enhancing lesion within the sella turcica involving the cavernous sinuses bilaterally, extending into Meckel's cave on the left, and abutting the optic nerves bilaterally. Following surgical excision, histopathology revealed the tumor to be a MCL. Metastatic pituitary tumors are rare and have been estimated to make up 1% of tumors discovered in the sellar region. The two most common secondary metastatic lesions to the sella are breast and lung carcinoma followed by prostate, renal cell, and gastrointestinal carcinoma. Metastatic lymphoma to the pituitary gland is especially rare and is estimated to constitute 0.5% of all metastatic tumors to the sella turcica. To our knowledge, this is the first reported case of MCL metastasizing to the pituitary gland.
Tieghi, Riccardo; Malagutti, Nicola; Valente, Luisa; Carnevali, Giulia; Clauser, Luigi C
Silent Sinus Syndrome is defined as a painless spontaneous and progressive enophthalmos and hypoglobus with maxillary sinus hypoplasia and orbital floor resorption. It is caused by maxillary sinus atelectasis in a setting of ipsilateral chronic maxillary sinus hypoventilation. The syndrome was first described in 1964 by Montgomery, but the term "Silent Sinus Syndrome" was not coined until 1994 by Soparkar. The aetiology is still controversial: some authors postulate a basal hypoplastic sinus, other suggest an acquired process due to an obstruction of the ostium in the medium meatus. Silent Sinus Syndrome presents in the third to fifth decades of life, very rarely in childhood with no gender predilection and it is usually a unilateral disorder. The symptoms are not shown to be related to chronic sinuses disease. The clinical signs are: enophthalmos, hypoglobus, upper lid retraction secondary to dystopia of the globe, sinking of the eye and orbital asymmetry, deepened upper lid sulcus, disappearance of the palpebral fold line, lagophthalmos, vertical diplopia, malar depression, and facial asymmetry. Extraocular muscle function is generally preserved and usually there is no visual impairment. The diagnosis is confirmed by computed tomography scan of the orbits and paranasal sinuses. The treatment consists of orbital reconstruction and functional rehabilitation of the maxillary sinuses.
Hwang, Kun; Kim, Han Joon
We present here an exceedingly rare variant of a nonmidline basal encephalocele of the spheno-orbital type, and this was accompanied with orbital dystopia in a 56-year-old man. On examination, his left eye was located more inferolaterally than his right eye, and the patient said this had been this way since his birth. The protrusion of his left eye was aggravated when he is tired. His naked visual acuity was 0.7/0.3, and the ocular pressure was 14/12 mm Hg. The exophthalmometry was 10/14 to 16 mm. His eyeball motion was not restricted, yet diplopia was present in all directions. The distance from the midline to the medial canthus was 20/15 mm. The distance from the midline to the midpupillary line was 35/22 mm. The vertical dimension of the palpebral fissure was 12/9 mm. The height difference of the upper eyelid margin was 11 mm, and the height difference of the lower eyelid margin was 8 mm. Facial computed tomography and magnetic resonance imaging showed left sphenoid wing hypoplasia and herniation of the left anterior temporal pole and dura mater into the orbit, and this resulted into left exophthalmos and encephalomalacia in the left anterior temporal pole. To the best of our knowledge, our case is the second case of basal encephalocele and orbital dystopia.
Guéguen, Antoine; Sené, Thomas; Maillart, Elisabeth; Gout, Olivier
Neurological manifestations have been reported in Kikuchi-Fujimoto disease (KFD). Characteristics of brain lesions are not defined. In addition, no biological indexes are known to help clinicians along the diagnosis process. The authors describe encephalitis associated with KFD. Brain MRI, positron emission tomography (PET) scan and a large biological assessment including interferon α (INF-α) level measurement in cerebrospinal fluid (CSF) were performed. A 39-year-old man with chronic headaches developed diplopia, slow ideation and behavioural disturbances. MRI showed brain lesions particularly in the pontine region and internal temporal lobes with enhancement of the perivacular space and the walls of the lateral ventricle. The IFN-α level was increased in the CSF without viral infection. Cervical and mediastinal adenitis were evident as a hypermetabolic focus on a PET scan, and biopsy confirmed the diagnosis of KFD. The encephalitis spontaneously remitted. The authors characterised brain lesions especially related to KFD in association with increased of IFN-α level in the CSF.
Shi, Wenyin; Jain, Varsha; Kim, Hyun; Champ, Colin; Jain, Gaurav; Farrell, Christopher; Andrews, David W.; Judy, Kevin; Liu, Haisong; Artz, Gregory; Werner-Wasik, Maria; Evans, James J.
Purpose Data on the clinical course of irradiated facial nerve schwannomas (FNS) are lacking. We evaluated fractionated stereotactic radiotherapy (FSRT) for FNS. Methods Eight consecutive patients with FNS treated at our institution between 1998 and 2011 were included. Patients were treated with FSRT to a median dose of 50.4 Gy (range: 46.8–54 Gy) in 1.8 or 2.0 Gy fractions. We report the radiographic response, symptom control, and toxicity associated with FSRT for FNS. Results The median follow-up time was 43 months (range: 10–75 months). All patients presented with symptoms including pain, tinnitus, facial asymmetry, diplopia, and hearing loss. The median tumor volume was 1.57 cc. On the most recent follow-up imaging, five patients were noted to have stable tumor size; three patients had a net reduction in tumor volume. Additionally, six patients had improvement in clinical symptoms, one patient had stable clinical findings, and one patient had worsened House-Brackmann grade due to cystic degeneration. Conclusion FSRT treatment of FNS results in excellent control of growth and symptoms with a small rate of radiation toxicity. Given the importance of maintaining facial nerve function, FSRT could be considered as a primary management modality for enlarging or symptomatic FNS. PMID:26949592
Kim, Yeong-Jin; Kim, Hee Kyung; Yang, Deok-Hwan; Jung, Shin; Noh, Myung-Giun; Lee, Jae-Hyuk; Lee, Kyung-Hwa; Moon, Kyung-Sub
This is the first reported case of pineal lymphoma with concomitant prolactin-producing pituitary adenoma.A 51-year-old male experienced worsening headaches accompanied by nausea, diplopia, and memory loss for 1 month. Cranial nerve examination revealed bilateral upward gaze limitation with convergence impairment, which is known as Parinaud syndrome. Magnetic resonance images revealed a mass in the pineal gland with a coexisting mass within the enlarged sella fossa. Hormone analysis revealed hyperprolactinemia. The pineal mass was removed without injuring the hypothalamus, brain stem, or any neighboring vessels. Pathology examination confirmed the diagnosis of diffuse large B-cell lymphoma (DLBCL) involving the pineal gland. After further studies, the pineal lymphoma was determined to be a secondary tumor from a gastric primary tumor. The patient died 6 months after diagnosis due to systemic progression of DLBCL.Although the mechanistic link between hyperprolactinemia and lymphoma progression has not been clarified on a clinical basis, high prolactin levels may contribute to the rapid progression and therapeutic resistance of the lymphoma.
Dong, Xiaoqin; Li, Jun; Huo, Na; Wang, Yan; Wu, Zhao; Lin, Xiaohong; Zhao, Hong
Abstract Rationale: Anaplastic large cell lymphoma (ALCL) is an aggressive non-Hodgkin lymphoma. It mostly invades lymph nodes with extranodal involvement observed in the soft tissue, bone, and skin. Patient concerns: We report a 34-year-old Chinese male patient who presented with headache, diplopia, and vomit. Cerebrospinal fluid (CSF) analysis via lumbar puncture showed elevated CSF pressure, elevated CSF protein concentrations, decreased CSF glucose and chloride concentration significantly, and pleocytosis of 68 to 350 × 106/L, in which lymphocytes and monocytes were predominant. These changes could be suggestive of tuberculous (TB) meningitis. Enhanced magnetic resonance imaging of spinal cord delineated multiple enhancing nodules in spinal cord, cauda equina, and cristae membrane, and multiple abnormal enhancing lesions in bilateral lumbar intervertebral foramen. Diagnoses: Spinal dura mater biopsy and paraffin pathology examination revealed anaplastic lymphoma kinase positive ALCL. Interventions: High-dose methotrexate, cytosine arabinoside craniospinal, and radiotherapy. Outcomes: Last follow-up on September 22, 2015 showed no evidence of tumor recurrence and the lower extremity muscle strength recovered to 4/5. Lessons: ALCL of primary central nervous system is an exceedingly rare tumor, which is usually misdiagnosed as meningitis (especially TB meningitis) according to clinical manifestation and laboratory examination. Thus closely monitoring patient's conditions and timely adjusting therapeutic regimen during treatment are necessary. PMID:27930548
Bhaskar, Hemlata; Chaudhary, Rekha
Background. Vitamin B12 is vital for optimal functioning of various organ systems but more importantly the central nervous system and the hematological system. Deficiency of vitamin B12 clinically manifests as excessive daytime fatigue, memory difficulties, encephalopathy, myelopathy, peripheral neuropathy, and optic neuropathy. In occupational medicine, vitamin B12 deficiency has been reported with exposure to nitrous oxide in health care workers. However, not much is known about exposure to Freons in other industries and vitamin B12 deficiency. Aim. We are reporting a case of vitamin B12 deficiency in the setting of exposure to chlorofluorocarbon (CFC) gases. Case Report. A 55-year-old male refrigerator mechanic experienced recurrent visual symptoms, which included diplopia and blurring. A complete workup was done and was significant of vitamin B12 deficiency. However, his B12 levels were refractory to supplementation. Appropriate precautions at workplace improved patient's symptoms and were associated with significant improvement in B12 levels. Conclusion. To the best of our knowledge, this is the first reported case of vitamin B12 deficiency (that remains refractory to supplementation) in the setting of exposure to Freon gases.
Lin, Li-Mei; Colby, Geoffrey P; Jiang, Bowen; Pero, Guglielmo; Boccardi, Edoardo; Coon, Alexander L
Flow diverters are increasingly used for the endovascular treatment of cerebral aneurysms. A rare complication from flow diversion is delayed aneurysm rupture, which can lead to carotid–cavernous fistula (CCF) in the setting of cavernous carotid aneurysms (CCAs). Direct CCFs pose unique management challenges, given the lack of transarterial access to the fistula. We present two cases of direct CCFs following treatment of CCAs with the Pipeline embolization device (PED). Case 1 was a middle-aged patient with a symptomatic 10 mm wide-necked left CCA. Six weeks after PED treatment the patient developed diplopia secondary to direct CCF. Case 2 was a middle-aged patient with a symptomatic 17 mm left CCA treated with PED. One-month follow-up angiography demonstrated a direct CCF. Both patients underwent successful coil embolization of the CCF through a transvenous approach. Direct CCF formation following PED deployment for CCA is a rare complication. Parent vessel sacrifice may be avoided with transvenous occlusion of the fistula. PMID:25073525
Until the beginning of the 17th century it was held that an image is formed in the eye on the anterior surface of the crystalline lens. Ophthalmological optics as a scientific discipline only began with a discovery made by Johannes Kepler. Without performing new experiments, and solely by application of the laws of light refraction, he analyzed the path of light through the eye and demonstrated that an image is formed on the retina and that it is inverted. Acceptance of this discovery was impeded by contemporary prejudices which could imagine nothing but an upright image in the eye, even though this attitude could not explain certain phenomena. Kepler's discovery of the path of light in the eye made it possible to explain the following physical phenomena: central visual acuity, visual field, dark adaptation, and errors of refraction. Physiological diplopia and the mechanism of accommodation were discovered later. The law stating that the intensity of light decreases with the square of distance was also formulated by Johannes Kepler; this law, too, could only be demonstrated after the path of light through the eye had been discovered. In recent years the Keplerian telescope has assumed a practical significance in ophthalmological optics. As a reading aid for individuals with impaired vision it offers a significantly higher magnification than any other optical visual aid.
Berrih-Aknin, Sonia; Frenkian-Cuvelier, Mélinée; Eymard, Bruno
Myasthenia gravis is characterized by muscle weakness and abnormal fatigability. It is an autoimmune disease caused by the presence of antibodies against components of the muscle membrane localized at the neuromuscular junction. In most cases, the autoantibodies are against the acetylcholine receptor (AChR). Recently, other targets have been described such as the MuSK protein (muscle-specific kinase) or the LRP4 (lipoprotein related protein 4). Myasthenia gravis can be classified according to the profile of the autoantibodies, the location of the affected muscles (ocular versus generalized), the age of onset of symptoms and thymic abnormalities. The disease generally begins with ocular symptoms (ptosis and/or diplopia) and extends to other muscles in 80% of cases. Other features that characterize MG include the following: variability, effort induced worsening, successive periods of exacerbation during the course of the disease, severity dependent on respiratory and swallowing impairment (if rapid worsening occurs, a myasthenic crisis is suspected), and an association with thymoma in 20% of patients and with other autoimmune diseases such as hyperthyroidism and Hashimoto's disease. The diagnosis is based on the clinical features, the benefit of the cholinesterase inhibitors, the detection of specific autoantibodies (anti-AChR, anti-MuSK or anti-LRP4), and significant decrement evidenced by electrophysiological tests. In this review, we briefly describe the history and epidemiology of the disease and the diagnostic and clinical classification. The neonatal form of myasthenia is explained, and finally we discuss the main difficulties of diagnosis.
Allison, Randall Z; Kaminskas, David A; Zagorski, Natalia M; Liow, Kore K
Miller Fisher Syndrome (MFS) is a rare variant of Guillain-Barré Syndrome (GBS) that has a geographically variable incidence. It is largely a clinical diagnosis based on the cardinal clinical features of ataxia, areflexia, and opthalmoplegia, however, other neurological signs and symptoms may also be present. Serological confirmation with the anti-GQ1b antibody is available and allows for greater diagnostic certainty in the face of confounding symptoms. A self-limiting course is typical of MFS. The following case report is that of a patient who presented with generalized weakness, somatic pain, inability to walk, and diplopia following an upper respiratory illness. The patient exhibited the classic triad of ataxia, areflexia, and opthalmoplegia characteristic of MFS, but also had less typical signs and symptoms making for a more challenging diagnostic workup. Our suspected diagnosis of MFS was serologically confirmed with positive anti-GQ1b antibody titer and the patient was successfully treated with Intravenous immune globulin (IVIG). PMID:27437164
Hur, Su Won; Kim, Sung Eun; Chung, Kyu Jin; Lee, Jun Ho; Kim, Tae Gon
Background Reconstruction of combined orbital floor and medial wall fractures with a comminuted inferomedial strut (IMS) is challenging and requires careful practice. We present our surgical strategy and postoperative outcomes. Methods We divided 74 patients who underwent the reconstruction of the orbital floor and medial wall concomitantly into a comminuted IMS group (41 patients) and non-comminuted IMS group (33 patients). In the comminuted IMS group, we first reconstructed the floor stably and then the medial wall by using separate implant pieces. In the non-comminuted IMS group, we reconstructed the floor and the medial wall with a single large implant. Results In the follow-up of 6 to 65 months, most patients with diplopia improved in the first-week except one, who eventually improved at 1 year. All patients with an EOM limitation improved during the first month of follow-up. Enophthalmos (displacement, 2 mm) was observed in two patients. The orbit volume measured on the CT scans was statistically significantly restored in both groups. No complications related to the surgery were observed. Conclusions We recommend the reconstruction of orbit walls in the comminuted IMS group by using the following surgical strategy: usage of multiple pieces of rigid implants instead of one large implant, sequential repair first of the floor and then of the medial wall, and a focus on the reconstruction of key areas. Our strategy of step-by-step reconstruction has the benefits of easy repair, less surgical trauma, and minimal stress to the surgeon. PMID:26217562
Chang, Melinda Y.; Velez, Federico G.; Demer, Joseph L.; Isenberg, Sherwin J.; Coleman, Anne L.; Pineles, Stacy L.
Purpose To assess relative quality of life in patients with strabismus. Design Retrospective cohort study Methods The 25-item National Eye Institute Visual Functioning Questionnaire (NEI VFQ-25) was performed in 42 strabismic adults over the age of 50 years at a single institution. Subscale scores were compared with those of patients with other ocular diseases, including diabetic retinopathy, age-related macular degeneration (AMD), glaucoma, cataract, cytomegalovirus (CMV) retinitis, and low vision. Results Median visual acuity was 20/20 (range 20/12.5 to 20/50), and 34 patients (81%) reported diplopia. Strabismic patients performed the same or worse on nearly all vision-related subscales than did patients with diabetic retinopathy, age-related macular degeneration, glaucoma, cataract, and CMV retinitis. Additionally, strabismic patients reported significantly worse ocular pain than all comparison groups before any surgery was performed. Conclusions Strabismus impacts quality of life through both functional and psychosocial factors. Physicians treating strabismic patients should recognize these quality of life issues and address them accordingly. PMID:25498355
Fan, Rui; Ji, Ruirui; Zou, Wenxin; Wang, Guoliang; Wang, Hu; Penney, Daniel James; Luo, Jin Jun; Fan, Yuxin
Andersen-Tawil syndrome (ATS) is an autosomal dominant, multisystem channelopathy characterized by periodic paralysis, ventricular arrhythmias and distinctive dysmorphic facial or skeletal features. The disorder displays marked intrafamilial variability and incomplete penetrance. Myasthenia gravis (MG) is an autoimmune disorder that demonstrates progressive fatigability, in which the nicotinic acetylcholine receptor (AChR) at neuromuscular junctions is the primary autoantigen. The present study reports a rare case of a 31-year-old woman with a history of morbid obesity and periodic weakness, who presented with hemodynamic instability, cardiogenic shock and facial anomalies. Laboratory results revealed hypokalemia and an elevated anti-AChR antibody expression levels. Electrocardiography demonstrated prolonged QT-interval, ST-elevation, and subsequent third-degree atrioventricular block. Neurological examination revealed bilateral ptosis, horizontal diplopia, dysarthria and generalized weakness. No mutations in the potassium channel inwardly rectifying subfamily J member 2 gene were detected in the present case. The patient was treated with oral potassium supplementation and an acetylcholinesterase inhibitor (pyridostigmine), after which the symptoms were improved. To the best of our knowledge, the present case report was the first to describe concomitant presentation of both ATS and MG, which represents a diagnostic and therapeutic challenge.
Sarkar, Somenath; Das, Kapildev; Roychoudhury, Soumyajit; Shrimal, Arpit
Pseudotumor cerebri (PTC) is a rare neurological disorder characterized by increased intracranial pressure in absence of any intra-cranial space-occupying lesion. It is mostly due to impairment of drainage of CSF from arachnoid villi. Clinically pseudotumor cerebri presents with headache, diplopia, nausea, vomiting, papilloedema and if treatment is delayed, may lead to blindness. Females of childbearing age group, endocrinal abnormalities and ingestion of certain drugs have been reported to be associated with pseudotumor cerebri. However, it's occurrence in relation to acitretin ingestion has not been reported on pubmed database. Here we present a case where significant temporal association of acitretin intake with PTC was found in a child who was being treated with this medication for recalcitrant pustular psoriasis. The case is reported for its rarity in occurrence and associated significant morbidity including visual loss if not diagnosed and treated immediately. According to Naranjo ADR Causality scale of adverse drug reaction, the association of PTC due to acitretin in our case was probable. PMID:23543097
Tamura, Yoji; Yamada, Yoshitaka; Tucker, Adam; Ukita, Tohru; Tsuji, Masao; Miyake, Hiroji; Kuroiwa, Toshihiko
Pineal cysts of the third ventricle presenting with acute obstructive hydrocephalus due to internal cystic hemorrhage are a rare clinical entity. The authors report a case of a 61-year-old man taking antiplatelet medication who suffered from a hemorrhagic pineal cyst and was treated with endoscopic surgery. One month prior to treatment, the patient was diagnosed with a brainstem infarction and received clopidogrel in addition to aspirin. A small incidental pineal cyst was concurrently diagnosed using magnetic resonance (MR) imaging which was intended to be followed conservatively. The patient presented with a sudden onset of headache and diplopia. On admission, the neurological examination revealed clouding of consciousness and Parinaud syndrome. Computerized tomography (CT) scans demonstrated a hemorrhagic mass lesion in the posterior third ventricle. The patient underwent emergency external ventricular drainage with staged endoscopic biopsy and third ventriculostomy using a flexible videoscope. Histological examination revealed pineal tissue with necrotic change and no evidence of tumor cells. One year later MR imaging demonstrated no evidence of cystic lesion and a flow void between third ventricle and prepontine cistern. In patients with asymptomatic pineal cysts who are treated with antiplatelet therapy, it is important to be aware of the risk of pineal apoplexy. Endoscopic management can be effective for treatment of hemorrhagic pineal cyst with obstructive hydrocephalus.
Kodera, Toshiaki; Bozinov, Oliver; Sürücü, Oguzkan; Ulrich, Nils H; Burkhardt, Jan-Karl; Bertalanffy, Helmut
The authors present a microsurgical technique for the resection of a heterogeneous group of pineal-region tumors and discuss the key points for successfully performing this surgery. Twenty-six consecutive patients with pineal-region tumors were resected by the senior author (H.B.) and analyzed retrospectively. For all 26 patients, the operation was conducted using the infratentorial supracerebellar (ITSC) approach in the sitting (23 patients) or Concorde (three patients) positions. Twenty-five patients had symptomatic obstructive hydrocephalus and were treated with ventricular drainage, a previously inserted ventriculoperitoneal shunt, or an endoscopic third ventriculostomy before undergoing resection of the pineal-region tumor. The gross total removal of the tumor was achieved in 23 patients and subtotal removal was achieved in three patients. The tumors were pathologically diagnosed mainly as pineocytomas (10), pilocytic astrocytomas (6), or pineal cysts (4). Twenty-five of the patients clinically improved after surgery, and there was no mortality. Two patients experienced transient postoperative neurological deterioration: one patient developed Parinaud syndrome, and one patient developed intermittent diplopia. Successful surgery and patient outcome when treating tumors of the pineal region using the ITSC approach requires: (i) preservation of the venous flow of the Galenic draining system; (ii) preservation of the thick bridging veins of the tentorial surface of the cerebellum, especially the hemispheric bridging veins; and (iii) minimizing retraction of the cerebellum during surgery to avoid adverse effects caused by both direct cerebellar compression and disturbance of the venous circulation.
Kumar, Ravi; Kumar, Ramesh; Mallory, Grant W; Jacob, Jeffrey T; Daniels, David J; Wetjen, Nicholas M; Foy, Andrew B; O'Neill, Brent R; Clarke, Michelle J
OBJECT Nonpowder guns, defined as spring- or gas-powered BB or pellet guns, can be dangerous weapons that are often marketed to children. In recent decades, advances in compressed-gas technology have led to a significant increase in the power and muzzle velocity of these weapons. The risk of intracranial injury in children due to nonpowder weapons is poorly documented. METHODS A retrospective review was conducted at 3 institutions studying children 16 years or younger who had intracranial injuries secondary to nonpowder guns. RESULTS The authors reviewed 14 cases of intracranial injury in children from 3 institutions. Eleven (79%) of the 14 children were injured by BB guns, while 3 (21%) were injured by pellet guns. In 10 (71%) children, the injury was accidental. There was 1 recognized assault, but there were no suicide attempts; in the remaining 3 patients, the intention was indeterminate. There were no mortalities among the patients in this series. Ten (71%) of the children required operative intervention, and 6 (43%) were left with permanent neurological injuries, including epilepsy, cognitive deficits, hydrocephalus, diplopia, visual field cut, and blindness. CONCLUSIONS Nonpowder guns are weapons with the ability to penetrate a child's skull and brain. Awareness should be raised among parents, children, and policy makers as to the risk posed by these weapons.
Vehmeijer, Maarten; van Eijnatten, Maureen; Liberton, Niels; Wolff, Jan
Fractures of the orbital floor are often a result of traffic accidents or interpersonal violence. To date, numerous materials and methods have been used to reconstruct the orbital floor. However, simple and cost-effective 3-dimensional (3D) printing technologies for the treatment of orbital floor fractures are still sought. This study describes a simple, precise, cost-effective method of treating orbital fractures using 3D printing technologies in combination with autologous bone. Enophthalmos and diplopia developed in a 64-year-old female patient with an orbital floor fracture. A virtual 3D model of the fracture site was generated from computed tomography images of the patient. The fracture was virtually closed using spline interpolation. Furthermore, a virtual individualized mold of the defect site was created, which was manufactured using an inkjet printer. The tangible mold was subsequently used during surgery to sculpture an individualized autologous orbital floor implant. Virtual reconstruction of the orbital floor and the resulting mold enhanced the overall accuracy and efficiency of the surgical procedure. The sculptured autologous orbital floor implant showed an excellent fit in vivo. The combination of virtual planning and 3D printing offers an accurate and cost-effective treatment method for orbital floor fractures.
Prasad, Kishore Chandra; Kumar, Ashwini; Prasad, Sampath Chandra; Jain, Disha
The purpose of this article is to report a case of esthesioneuroblastoma involving the bilateral paranasal sinuses, which was excised using an endoscopic-assisted transfacial approach. A patient presented with nasal swelling and left-sided nasal obstruction, epistaxis, and diplopia. Examination revealed broadening of the nasal dorsum with a fleshy pink mass in both nasal cavities. Computed tomographic scan showed a mass involving the nasal cavity and paranasal sinuses on both sides. The tumor was diagnosed as group C esthesioneuroblastoma. The mass was excised by bilateral medial maxillectomy and bilateral frontoethmoidectomy. Using a 0 degrees endoscope, the attachment of the tumor to the cribriform plate was identified and resected using a motordrill. On Waroff staining, Hispathology slides suggested esthesioneuroblastoma. The patient was asymptomatic for 1 year, following which he developed infection of the nasal cavity for which he had no form of treatment. He subsequently developed maggots in the nasal cavity after which he died. An endoscopic resection of the cribriform plate from the nasal cavity without a formal craniofacial resection can be safely performed with oncologic safety.
Monteiro, Regina; Carneiro, José Carlos; Costa, Claúdia; Duarte, Raquel
Cerebral tuberculomas are a rare and serious form of tuberculosis (TB) due to the haematogenous spread of Mycobacterium Tuberculosis (MT). Symptoms and radiologic features are nonspecific, leading sometimes to misdiagnosis. Anti-TB drugs are essential for the successful treatment of cerebral tuberculomas but there is no agreement regarding the duration of therapy. The authors present a case of a 55 years old male, presented to the emergency room with sudden onset of diplopia. Cerebral computerized tomography revealed multiple brain lesions, with contrast enhancement and peri-lesional oedema. The patient was HIV negative and because of previous malignancy the first suspicion was metastatic disease. Cultural exam of the bronchial wash showed MT sensitive to all first-line drugs. The patient started antituberculosis treatment with 4 drugs (HRZE) for 2 months, followed by maintenance therapy (HR). Treatment was prolonged for 24 months because at 12th and 18th months of treatment one of the brain lesions, although significantly smaller, still showed contrast enhancement. Even though it is not clear if contrast enhancement lesions represent active lesions or just inflammation, continuing treatment until total resolution of the tuberculomas is probably prudent. PMID:26029627
Bibas-Bonet, Hilda; Fauze, Ricardo A; Lavado, María Graciela; Páez, Rafael O; Nieman, Judith
Garcin syndrome is characterized by a progressive ipsilateral involvement of cranial nerves, culminating in paralysis of all or at least seven of them, without sensory or motor long-tract disturbance, with no intracranial hypertension, and with osteoclastic involvement in the skull base on radiographic computed tomography. Giant cell tumor is a primary bone tumor rarely affecting the skull base. An 8-year-old female presented with a 3-month history of increasingly worsening right otalgia, tinnitus, hearing loss, right facial numbness, and diplopia. She was admitted with a 2-week history of swallowing difficulties, voice change, and right shoulder pain. Neurologic examination disclosed unilateral paralysis of the right fifth through twelfth cranial nerves, with no other abnormal neurologic findings. Skull radiographic computed tomography revealed lytic lesions in the right temporal petrous portion. Computed tomographic scan indicated a destructive mass involving the right greater wing of the sphenoid bone and temporal petrous apex. Magnetic resonance imaging demonstrated a tumor arising from the temporosphenoidal region, infiltrating neither the brain nor the brainstem. No hydrocephalus was observed. Biopsy revealed giant cell tumor. Posterior treatment consisted of radiotherapy. At an 8-year follow-up, the patient was well but with functional sequelae. There is no magnetic resonance imaging evidence of tumor growth. No other giant cell tumor presenting as Garcin syndrome is known to have been reported.
Chennupati, Sri Kiran; Norris, Robin; Dunham, Brian; Kazahaya, Ken
Osteosarcoma is the most common primary malignancy of bone in children and adolescents. Osteosarcomas are an aggressive neoplasm composed of spindle cells producing osteoid. They primarily affect the long bones, particularly after radiation or chemotherapy for other neoplasms; however, 6-7% present in the head and neck. Primary head and neck osteosarcomas in children are rare. There are few case reports and limited-sized case series in the literature. A case report presentation of a skull base osteosarcoma in a teenage female. A 14-year-old African American female presented with dysphagia, voice changes, and neck pain. On examination, she had right-sided palsies in cranial nerves X, XI, and XII. Imaging revealed partial enhancement of the clivus without bony erosion and expansion of the hypoglossal canal. There were also findings consistent with chronic denervation of her right tongue and pharynx. During the evaluation process, she developed diplopia from a right cranial nerve VI palsy. Repeat imaging revealed progression of the skull base lesion with extension into the right sphenoid sinus. An endoscopic sphenoidotomy was performed to obtain tissue. The diagnosis of high-grade osteosarcoma was made by histologic morphology and immunohistochemistry. The child was treated primarily with chemotherapy. Other adjunctive therapies are being considered. Osteosarcoma of the skull base is a rare entity. We describe a case of a high-grade clival osteosarcoma presenting primarily with lower cranial nerve palsies and pain. The rapid progression, treatment options, and prognosis are discussed.
Ashkenazi, Malka; Baniel, Anat
Objective. To evaluate the success rate of intra-rectal (IR) midazolam in combination with nitrous oxide/oxygen (N2O) sedation in young uncooperative dental patients when the local anesthesia is delivered by a computerized controlled local anesthetic delivery (C-CLAD). Study Design. This observational study consisted of 219 uncooperative children (age: 4.3 ± 1.69 y) who received IR midazolam (0.4 mg/kg) and N2O to complete their dental treatment. Measured variables included: child's pain disruptive behavior during delivery of anesthesia by C-CLAD (CHEOP Scale), child behavior during treatment (Houpt scale), dental procedure performed, and side effects that appeared during treatment. Results. There was a high level of cooperation (mean score: 6.69 ± 2.1) during administration of local anesthesia. Good-to-excellent behavior was shown by 87% of the children during treatment. Planned treatment was completed by 184 (92%) patients. No statistically significant changes were noticed in the oxygen saturation levels before and after treatment. Children with side effects included 3 (1.3%) with nistagmus, 5 (2.3%) with diplopia, and 18 (8.2%) with hiccups. Three consecutive sedations decreased the overall behavior score by 5.7% compared to the first appointment (p < .05). Conclusions. IR midazolam-N2O sedation in combination with C-CLAD is very effective for delivery of dental treatment to young uncooperative children.
Rougé, Alain; Lemarié, Jérémie; Gibot, Sébastien; Bollaert, Pierre Edouard
A 47-year-old man was admitted to the intensive care unit a few hours after presenting to emergency department with acute diplopia and dysphonia. Swallowing disorders and respiratory muscular weakness quickly required invasive ventilation. On day 3, the patient was in a “brain-death”-like state with deep coma and absent brainstem reflexes. Electroencephalogram ruled out brain death diagnosis as a paradoxical sleep trace was recorded. Cerebrospinal fluid analysis, electrophysiologic studies, and a recent history of diarrhea led to the diagnosis of Campylobacter jejuni-related fulminant Guillain-Barré syndrome (GBS) mimicking brain death. The outcome was favorable after long Intensive Care Unit and inpatient rehabilitation stays, despite persistent disability at 9 years follow-up. This case and the associated literature review of 34 previously reported fulminant GBS patients emphasize the importance of electrophysiological investigations during clinical brain-death states with no definite cause. Fulminant GBS has a worse outcome than “standard” GBS with higher rates of severe disability (about 50%). Long-term physiotherapy and specific rehabilitation programs appear essential to improve recovery. PMID:27853394
Akbari, Mahammad Reza; Jafari, Alireza Keshtcar; Ameri, Ahmed; Anvari, Faramarz; Eshraghi, Bahram; Masoomian, Babak
A 26- year-old woman who had a left head tilt since childhood was undergoing left inferior rectus resection to correct her left inferior rectus paresis. During the surgery, when the inferior rectus was isolated and engaged with an eye muscle hook, the muscle tore (snapped) completely into two pieces, 8 mm posterior to insertion. Fortunately, we were able to find the proximal portion of the muscle and, after a 3 mm resection, of the distal yet attached 8 mm portion, the proximal and distal portions were sutured together with a non-absorbable suture. After nine months followup there was significant diplopia, and the preoperative left head tilt and left hypertropia remained, so a left inferior rectus re-resection was done. At the end of 18 months followup after the second procedure there was no binocular deviation (strabismus) in primary position nor in any other gaze positions, but there was a mild ocular motility infraduction deficit present on left and down gaze.
Jung, Jae-Hyun; Peli, Eli
Prism distortions and spurious reflections are not usually considered when prescribing prisms to compensate for visual field loss due to homonymous hemianopia. Distortions and reflections in the high-power Fresnel prisms used in peripheral prism placement can be considerable, and the simplifying assumption that prism deflection power is independent of angle of incidence into the prisms results in substantial errors. We analyze the effects of high prism power and incidence angle on the field expansion, size of the apical scotomas, and image compression/expansion. We analyze and illustrate the effects of reflections within the Fresnel prisms, primarily due to reflections at the bases, and secondarily due to surface reflections. The strength and location of these effects differs materially depending on whether the serrated prismatic surface is placed toward or away from the eye, and this affects the contribution of the reflections to visual confusion, diplopia, false alarms, and loss of contrast. We conclude with suggestions for controlling and mitigating these effects in clinical practice.
Goto, Yukihiro; Furuno, Yuichi; Kawabe, Takuya; Ohwada, Kei; Tatsuzawa, Kazunori; Sasajima, Hiroyasu; Hashimoto, Naoya
A 34-year-old man with a 1-week history of diplopia was referred to the authors' hospital. Neurological examination revealed left abducens nerve palsy. Computed tomography showed a lesion in the left sphenoid sinus involving the medial wall of the left internal carotid artery (ICA) and osteolytic change at the clivus bordering the lesion. Magnetic resonance imaging demonstrated an extensive soft-tissue mass occupying the left sphenoid sinus. Surgical intervention by the endoscopic transnasal method allowed most of the lesion to be removed. Only the portion attached to the medial wall of the ICA was not removed. Postoperatively, the lesion was diagnosed as a giant cell tumor (GCT) and the patient received 120 mg of subcutaneous denosumab every 4 weeks, with additional doses on Days 8 and 15 during the first month of therapy. MRI a week after starting denosumab revealed shrinkage of the initially fast-growing residual tumor. The patient was discharged upon completion of the third denosumab administration. GCT is an aggressive stromal tumor developing mainly in young adults. Complete resection is recommended for GCT in the literature. However, size and location of the CGT often limit this approach. Various adjuvant treatments for skull base GCTs have been reported, including radiation and chemotherapy. However, the roles of adjuvant therapies have yet to be clearly defined. Denosumab, a monoclonal antibody, was recently approved for GCT in several countries. Denosumab may permit less invasive treatments for patients with GCTs while avoiding deleterious outcomes, and may also limit disease progression and recurrence.
Mitchell, Amber N; Bakhos, Charles T; Zimmerman, Earl A
Paraneoplastic neurologic syndromes (PNS) can be the first manifestations of occult malignancies. If left untreated, PNS often lead to significant morbidity and mortality. Anti-Ri (anti-neuronal nuclear antibody type 2 [ANNA-2]) autoantibodies are commonly associated with breast and small cell lung cancers. Cases of anti-Ri paraneoplastic cerebellar degeneration are reported, but few describe severe nausea and coexisting limbic encephalitis as the major presenting features. We report a 75-year-old woman with medically-intractable emesis, encephalopathy, diplopia, vertigo, and gait ataxia for 3 months. Examination revealed rotary nystagmus, ocular skew deviation, limb dysmetria, and gait ataxia. After two courses of intravenous immunoglobulin, there was minimal improvement. Anti-Ri antibodies were positive in serum only. CT scan identified a 2.0 cm left lung mass, and histopathology revealed large cell neuroendocrine carcinoma with admixed adenocarcinoma non-small cell lung carcinoma (NCSLC). Though the patient achieved nearly complete clinical recovery after tumor resection, anti-Ri levels remained high at 20 months post-resection. To our knowledge this is the first report of a paraneoplastic brainstem cerebellar syndrome with coexisting limbic encephalitis involving anti-Ri positivity and associated mixed neuroendocrine/NSCLC of the lung with marked improvement after tumor resection.
D’Adamo, Maria Cristina; Hasan, Sonia; Guglielmi, Luca; Servettini, Ilenio; Cenciarini, Marta; Catacuzzeno, Luigi; Franciolini, Fabio
Episodic ataxia type 1 (EA1) is a K+ channelopathy characterized by a broad spectrum of symptoms. Generally, patients may experience constant myokymia and dramatic episodes of spastic contractions of the skeletal muscles of the head, arms, and legs with loss of both motor coordination and balance. During attacks additional symptoms may be reported such as vertigo, blurred vision, diplopia, nausea, headache, diaphoresis, clumsiness, stiffening of the body, dysarthric speech, and difficulty in breathing. These episodes may be precipitated by anxiety, emotional stress, fatigue, startle response or sudden postural changes. Epilepsy is overrepresented in EA1. The disease is inherited in an autosomal dominant manner, and genetic analysis of several families has led to the discovery of a number of point mutations in the voltage-dependent K+ channel gene KCNA1 (Kv1.1), on chromosome 12p13. To date KCNA1 is the only gene known to be associated with EA1. Functional studies have shown that these mutations impair Kv1.1 channel function with variable effects on channel assembly, trafficking and biophysics. Despite the solid evidence obtained on the molecular mechanisms underlying EA1, how these cause dysfunctions within the central and peripheral nervous systems circuitries remains elusive. This review summarizes the main breakthrough findings in EA1, discusses the neurophysiological mechanisms underlying the disease, current therapies, future challenges and opens a window onto the role of Kv1.1 channels in central nervous system (CNS) and peripheral nervous system (PNS) functions. PMID:26347608
Huertas-González, Nuria; Hernando-Requejo, Virgilio; Luciano-García, Zaida; Cervera-Rodilla, Juan Luis
Background. Wernicke's encephalopathy (WE) is an acute neurological disorder resulting from thiamine deficiency. It is mainly related to alcohol abuse but it can be associated with other conditions such as gastrointestinal disorders. This vitamin deficiency can also present with cardiovascular symptoms, called “wet beriberi.” Association with folate deficit worsens the clinical picture. Subject. A 70-year-old man with gastric phytobezoar presented with gait instability, dyspnoea, chest pain associated with right heart failure and pericarditis, and folate deficiency. Furosemide was administered and cardiac symptoms improved but he soon developed vertiginous syndrome, nystagmus, diplopia, dysmetria, and sensitive and motor deficit in all four limbs with areflexia. Results. A cerebral magnetic resonance imaging (MRI) showed typical findings of WE. He was immediately treated with thiamine. Neurological symptoms improved in a few days and abnormal signals disappeared in a follow-up MRI two weeks later. Conclusion. Patients with malabsorption due to gastrointestinal disorders have an increased risk of thiamine deficiency, and folate deficiency can make this vitamin malabsorption worse. An established deficiency mainly shows neurological symptoms, WE, or rarely cardiovascular symptoms, wet beriberi. Early vitamin treatment in symptomatic patients improves prognosis. We recommend administration of prophylactic multivitamins supplements in patients at risk as routine clinical practice. PMID:26697247
Edmunds, Matthew R; Cikatricis, Peter; Mukherji, Subhanjan; Bowyer, Jeremy D
A previously healthy 32-year-old Caucasian female presented with sudden-onset horizontal diplopia following a paroxysm of coughing. She had recently sustained a pubic ramus fracture during an innocuous fall and had also noted a firm lump developing at the right side of her forehead. On examination, she had a right fronto-temporal mass. Visual acuities were 6/6 bilaterally. There was reduced abduction of the right eye, bilateral white, granular corneal opacities and evidence of bilateral optic disc swelling. Haematological investigations revealed normocytic anaemia, hypercalcaemia and raised erythrocyte sedimentation rate (ESR). CT showed lytic foci throughout the skull, ribs, scapulae, spine, pelvis and upper femora. Serum protein electrophoresis revealed immunoglobulin D (IgD)-kappa paraproteinaemia; urine electrophoresis showed free light chain kappa and bone marrow biopsy demonstrated 87% plasma cells. A diagnosis of IgD multiple myeloma was made, with subsequent chemotherapeutic treatment and eventual autologous stem cell transplant resulting in resolution of neuro-ophthalmic manifestations and prolonged disease remission.
Matsusaki, Takashi; Morimatsu, Hiroshi; Matsumi, Junya; Matsuda, Hiroaki; Sato, Tetsufumi; Sato, Kenji; Mizobuchi, Satoshi; Yagi, Takahito; Morita, Kiyoshi
Pituitary apoplexy occurring after surgery is a rare but life-threatening acute clinical condition that follows extensive hemorrhagenous necrosis within a pituitary adenoma. Pituitary apoplexy has been reported to occur spontaneously in the majority of cases or in association with various inducing factors. Reported is a case of pituitary apoplexy complicated by diabetes insipidus following living donor liver transplantation (LDLT). To the best of our knowledge, this has not been previously reported. A 56-year-old woman with nonalcoholic steatohepatitis underwent LDLT from her daughter. The patient also required dopamine support and transfusions because of massive intraoperative bleeding. Postoperatively, her coagulopathy continued, and she underwent a second laparotomy because of unknown bleeding on postoperative day 7, when she needed transfusions and dopamine support to maintain her vital signs. She complained of severe headache, excessive thirst, frequent urination, and diplopia from postoperative day 10. She also had polyuria greater than 300 ml/h and was diagnosed with pituitary apoplexy precipitating diabetes insipidus on postoperative day 13. She was treated conservatively without surgery because of the hormonally inactive status and slight mass effect of her tumor. It is important for anesthesiologists and critical care personnel in LDLT settings to take into consideration this complication as a differential diagnosis.
Issing, Peter R.; Hemmanouil, Ilias; Stöver, Timo; Kempf, Hans-Georg; Wilkens, L.; Heermann, R.; Lenarz, Thomas
Adenoid cystic carcinoma (ACC) is a slowly growing tumor with a particular tendency to infiltrate the surrounding tissue by perineural spread. The clinical diagnosis may prove difficult due to the submucons extension of the tumor, especially at the skull base. This article outlines the clinical characteristics, diagnostics, and treatment modalities in a series of 56 patients with an ACC in the head and neck diagnosed between 1970 and 1998 in 32 females and 24 males. The youngest patient was aged 24 years, the oldest 77 years. The average age was 54 years. In 16 patients the tumor originated in the paranasal sinuses or the nasopharynx and involved the skull base. As a rule, several months passed between the manifestation of the first symptoms such as pain, blocked nose, epistaxis, or diplopia and the initial clinical diagnosis. All patients received surgical treatment, however, complete microscopical resection could only be achieved in approximately one third of the cases. Therefore, nine patients were postoperatively treated with radiotherapy. The average survival rates of the patients with an ACC of the skull base were only 99 months as compared to 144 months in the patients without skull base involvement. ImagesFigure 1 PMID:17171116
Morita, Ken; Nakamura, Fumihiko; Kamikubo, Yasuhiko; Mizuno, Naoaki; Miyauchi, Masashi; Yamamoto, Go; Nannya, Yasuhito; Ichikawa, Motoshi; Kurokawa, Mineo
Lymphoma occurring in the pituitary gland is an exceedingly infrequent event. Here, we describe a case of pituitary lymphoma complicating recurrent pituitary adenoma. A 56-year-old male with a history of pituitary adenoma was diagnosed with diffuse large B-cell lymphoma (DLBCL) of the left ocular adnexa, which was successfully treated by standard chemotherapy and local radiotherapy. Eight months later, he complained of diplopia and bitemporal hemianopia. Brain magnetic resonance imaging detected a suprasellar tumor. Transsphenoidal biopsy of the mass was performed, and histopathological examination revealed DLBCL admixed with pituitary adenoma. On a review of the literature, we found that pituitary lymphoma developing within adenoma is a recurrent phenomenon. The composite tumor is likely to be characterized by suprasellar involvement and presentation of visual disturbances. Moreover, in the present case, the suprasellar tumor remained visible after autologous peripheral stem cell transplant, likely due to the residual pituitary adenoma. We therefore recommend that refractory pituitary lymphoma should be vigorously biopsied in search of possibly underlying adenoma.
Computer vision syndrome (CVS) is the combination of eye and vision problems associated with the use of computers. In modern western society the use of computers for both vocational and avocational activities is almost universal. However, CVS may have a significant impact not only on visual comfort but also occupational productivity since between 64% and 90% of computer users experience visual symptoms which may include eyestrain, headaches, ocular discomfort, dry eye, diplopia and blurred vision either at near or when looking into the distance after prolonged computer use. This paper reviews the principal ocular causes for this condition, namely oculomotor anomalies and dry eye. Accommodation and vergence responses to electronic screens appear to be similar to those found when viewing printed materials, whereas the prevalence of dry eye symptoms is greater during computer operation. The latter is probably due to a decrease in blink rate and blink amplitude, as well as increased corneal exposure resulting from the monitor frequently being positioned in primary gaze. However, the efficacy of proposed treatments to reduce symptoms of CVS is unproven. A better understanding of the physiology underlying CVS is critical to allow more accurate diagnosis and treatment. This will enable practitioners to optimize visual comfort and efficiency during computer operation.
León-Ruiz, Moisés; Benito-León, Julián; Sierra-Hidalgo, Fernando; García-Soldevilla, Miguel Ángel; Izquierdo-Esteban, Laura; Tejeiro-Martínez, José; Cabrera-Valdivia, Francisco; García-Albea Ristol, Esteban
Introduccion. El mieloma multiple es la neoplasia de celulas plasmaticas mas frecuente. Al ser incurable, el tratamiento persigue obtener el mayor tiempo de supervivencia libre de clinica. Constituye una causa extremadamente rara de afectacion de los nervios craneales y es producido habitualmente por un plasmocitoma intracraneal. Presentamos un caso de mieloma multiple, que asociaba un plasmocitoma intracraneal y que comenzo clinicamente con paralisis aislada, completa y fluctuante del III nervio craneal. Caso clinico. Mujer de 63 años que acudio a urgencias por presentar un cuadro clinico oscilante, consistente en diplopia binocular horizontal y, posteriormente, cefalea. La exploracion neurooftalmologica revelo una paralisis completa del III nervio craneal derecho. Se solicito una tomografia axial computarizada craneal urgente, que revelo multiples lesiones osteoliticas diploicas, asociando una de ellas componente de partes blandas en la hendidura esfenoidal derecha. La paciente fue ingresada, y se le diagnostico posteriormente un mieloma multiple IgA-kappa. Tras recibir induccion quimioterapica y ser sometida a un trasplante autologo de progenitores hematopoyeticos, alcanzo la remision completa. Conclusiones. El mieloma multiple es un trastorno raro de los nervios craneales, una causa muy infrecuente de paralisis aislada y completa del III nervio craneal y menos aun fluctuante, y no se ha encontrado ningun caso publicado con este inicio clinico. Tener en cuenta las posibles manifestaciones neurooftalmologicas del mieloma multiple puede contribuir a un diagnostico precoz y a una incidencia positiva sobre el curso de esta enfermedad.
Caltabiano, R; Serra, A; Bonfiglio, M; Platania, N; Albanese, V; Lanzafame, S; Cocuzza, S
Osteoblastoma is a solitary, benign bone tumor that is rarely localized in the frontal sinus. It consists of hypocellular mineralized tissue that may form large masses or irregular trabeculae. A 31 year old man came to our attention with a 7 month history of diplopia, photophobia, frontal headhaches and progressive exophthalmos with proptosis of the left eye. The patient was submitted to computed tomography (CT) which allowed to appraise the extension of the lesion. The mass expanded inside the left frontal sinus and the upper ethmoidal cells invading the left orbital roof. Considering the extension of the tumor, the site and the connections with contiguous structures, a combination of endoscopic endonasal technique with intraorbital approach was performed. At histological examination typical features of benign osteoblastoma were observed. The sites of predilection for the tumor are the long bones, vertebral column, and small bones of hands and feet. Its occurrence in the skull and jaw bones is relatively rare and represents only 15% of all osteoblastomas. To our knowledge, only 5 cases of osteoblastoma of the frontal sinus have been previously reported in the English-language literature. This report describes a case of benign osteoblastoma in a rare site, namely, the frontal sinus with particular attention about the differential diagnosis and the treatment.
Falcon, M I; Gomez, C M; Chen, E E; Shereen, A; Solodkin, A
Spinocerebellar ataxia 6 (SCA6), an autosomal dominant degenerative disease, is characterized by diplopia, gait ataxia, and incoordination due to severe progressive degeneration of Purkinje cells in the vestibulo- and spinocerebellum. Ocular motor deficits are common, including difficulty fixating on moving objects, nystagmus and disruption of smooth pursuit movements. In presymptomatic SCA6, there are alterations in saccades and smooth-pursuit movements. We sought to assess functional and structural changes in cerebellar connectivity associated with a visual task, hypothesizing that gradual changes would parallel disease progression. We acquired functional magnetic resonance imaging and diffusion tensor imaging data during a passive smooth-pursuit task in 14 SCA6 patients, representing a range of disease duration and severity, and performed a cross-sectional comparison of cerebellar networks compared with healthy controls. We identified a shift in activation from vermis in presymptomatic individuals to lateral cerebellum in moderate-to-severe cases. Concomitantly, effective connectivity between regions of cerebral cortex and cerebellum was at its highest in moderate cases, and disappeared in severe cases. Finally, we noted structural differences in the cerebral and cerebellar peduncles. These unique results, spanning both functional and structural domains, highlight widespread changes in SCA6 and compensatory mechanisms associated with cerebellar physiology that could be utilized in developing new therapies.
Kim, Kyoung Woo; Ha, Hyo Shin; Kim, Jae Chan
Pterygium is a common ocular surface disease characterized by triangular wing-like growth consisting of subconjunctival hypertrophic connective tissue. Pterygium is easily complicated by adhesion to the eyelid and diplopia related to motility restriction of the eyeball. Beyond the cosmetic problems, this condition has a catastrophic effect on quality of life. Post-surgical recurrence rates of pterygium excision have been reported to be very high. Therefore, identifying the distinct pathogenic pathways of the disease may lead to new therapeutic strategies with lower risk of treatment failure. Based on the relatively low vascularity and known-predominance of disease occurrence in the nasal conjunctiva of normal eyes, we proposed that hypoxic ischemic injury can elicit the development of pterygium. Here, we review hypoxia-inducible factor (HIF)-1alpha-induced activation of the stromal cell-derived factor-1 (SDF-1)/chemokine receptor type 4 (CXCR4) signaling pathway as a possible mechanism. Supporting this concept of pathogenic mechanism, we also highlight bone marrow-derived progenitor cell tropism as a main contributor to pterygium pathogenesis.
Newadkar, Ujwala Rohan; Chaudhari, Lalit; Khalekar, Yogita
Introduction: The upper jaw forms the floor of the maxillary sinus and the upper teeth are continuous with the whole midface and cranium, therefore while treating these teeth, it is important for the practitioner to consider the possibility of ocular complications. Ocular disturbances such as blurring of vision, mydriasis, ptosis, diplopia, enophthalmos, miosis, and blindness are rare complications due to intraoral local anesthesia. So far at present, the general population is having myths and misconceptions regarding the extraction of teeth and vision loss; hence, we evaluated the same. Methods: A cross-sectional prospective survey targeting the general public was conducted using a self-administered questionnaire. A total of 300 standardized self-administered questionnaires were given and the data were analyzed. Results: Out of 300 patients, 148 were educated and 152 were uneducated. The study population was analyzed based on their age, sex, and literacy, i.e. the level of education. Seventy-six percent of uneducated and 48% of educated groups had false belief of ocular complications followed by the removal of upper teeth and among them uneducated females of older age group showed higher prevalence. Conclusion: The general public's knowledge about ocular complications due to tooth extraction in our study group is not adequate and needs improvement. Although the practice of informing by dentists is satisfactory, there is a need for creating awareness in the general public against such complications.
Rajavi, Zhale; Nassiri, Nader; Azizzadeh, Monir; Ramezani, Alireza; yaseri, Mehdi
Purpose To report orthoptic changes after photorefractive keratectomy (PRK). Methods This interventional case series included 297 eyes of 150 patients scheduled for PRK. Complete ophthalmologic evaluations focusing on orthoptic examinations were performed before and 3 months after PRK. Results Before PRK, 2 (1.3%) patients had esotropia which remained unchanged; 3 (2%) patients had far exotropia which improved after the procedure. Of 12 cases (8%) with initial exotropia at near, 3 (2%) cases became orthophoric, however 6 patients (4%) developed new near exotropia. A significant reduction in convergence and divergence amplitudes (P < 0.001) and a significant increase in near point of convergence (NPC) (P < 0.006) were noticed after PRK. A reduction ≥ 10 PD in convergence amplitude and ≥ 5 PD in divergence amplitude occurred in 10 and 5 patients, respectively. Four patients had initial NPC > 10 cm which remained unchanged after surgery. Out of 9 (6%) patients with baseline stereopsis > 60 seconds of arc, 2 (1.33%) showed an improvement in stereopsis following PRK. No patient developed diplopia postoperatively. Conclusion Preexisting strabismus may improve or remain unchanged after PRK, and new deviations can develop following the procedure. A decrease in fusional amplitudes, an increase in NPC, and an improvement in stereopsis may also occur after PRK. Preoperative evaluation of orthoptic status for detection of baseline abnormalities and identification of susceptible patients seem advisable. PMID:22454717
Ortega-Porcayo, Luis Alberto; Ponce-Gómez, Juan Antonio; Martínez-Moreno, Mauricio; Portocarrero-Ortíz, Lesly; Tena-Suck, Martha Lilia; Gómez-Amador, Juan Luis
Introduction Primary ectopic craniopharyngiomas have only rarely been reported. Craniopharyngiomas involve usually the sellar and suprasellar region, but can be originated from cell remnants of the obliterated craniopharyngeal duct or metaplastic change of andenohypophyseal cells. We present the first case of a primary ectopic frontotemporal craniopharyngioma. Presentation of case A 35-year old woman presented with a one-year history of headache and diplopia. MRI showed a large frontotemporal cystic lesion. Tumor resection was performed with a keyhole endoscopic frontal lateral approach. The pathological features showed an adamantinomatous craniopharyngioma with a cholesterol granuloma reaction. Discussion There have been reported different localizations for primary ectopic craniopharyngioma. Our case presented a lobulated frontotemporal cystic mass formed by a dense eosinophilic proteinaceous material dystrophic calcifications and cholesterol crystals, with epithelial remnants. No tumor regrowth was observed in the magnetic resonance image 27 months postoperatively. Conclusion Primary ectopic craniopharyngioma is a rare entity with a pathogenesis that remains uncertain. This is an unusual anatomic location associated with unique clinical findings. PMID:25725331
Lewis, A I; Tomsick, T A; Tew, J M; Lawless, M A
Transarterial embolization of direct carotid-cavernous fistulas (CCFs) using detachable balloons is the best initial option for occlusion of the fistula and preservation of the internal carotid artery. However, the long-term safety and efficacy of this treatment is unknown. The authors reviewed the long-term outcome of 87 patients with 88 direct CCFs occluded by detachable balloons. Clinical follow up was obtained in 48 (83%) of 58 patients treated with latex balloons (mean follow-up period 10 years, range 5.9-15.5 years) and 28 (97%) of 29 patients treated with silicone balloons (mean follow-up period 4 years, range 1-6.6 years). Two patients were treated with both balloon types. There were no late recurrent symptoms of cranial bruit, proptosis, chemosis, or arterialized conjunctiva in patients treated with either latex or silicone balloons. Diplopia improved in all patients; however, five patients required shortening of the lateral rectus muscle. Delayed ischemia occurred in three patients: one patient had a transient ischemic episode 5 years after treatment with latex balloons and two patients (85 and 90 years old) who had ruptured spontaneous intracavernous aneurysms suffered cerebral infarctions 6 weeks and 4 months, respectively, after treatment with silicone balloons. There were five deaths in the series unrelated to balloon treatment. These results show that after transarterial embolization of direct CCFs using either silicone or latex detachable balloons, the long-term risks are low for fistula recurrence, symptomatic foreign body reaction, symptomatic pseudoaneurysm formation, and cerebral ischemia.
Court, Jennifer Helen; Janicek, David
A 64-year-old man presented with a 2-day history of acute onset painless left ptosis. He had no other symptoms; importantly pupils were equal and reactive and eye movements were full. There was no palpable mass or swelling. He was systemically well with no headache, other focal neurological signs, or symptoms of fatigue. CT imaging showed swelling of the levator palpebrae superioris suggestive of myositis. After showing no improvement over 5 days the patient started oral prednisolone 30 mg reducing over 12 weeks. The ptosis resolved quickly and the patient remains symptom free at 6 months follow-up. Acute ptosis may indicate serious pathology. Differential diagnoses include a posterior communicating artery aneurysm causing a partial or complete third nerve palsy, Horner’s syndrome, and myasthenia gravis. A careful history and examination must be taken. Orbital myositis typically involves the extraocular muscles causing pain and diplopia. Isolated levator myositis is rare. PMID:25564592
Rosenfeld, William; Fountain, Nathan B; Kaubrys, Gintaras; Ben-Menachem, Elinor; McShea, Cindy; Isojarvi, Jouko; Doty, Pamela
Long-term (up to 8 years of exposure) safety and efficacy of the antiepileptic drug lacosamide was evaluated in this open-label extension trial (SP615 [ClinicalTrials.gov identifier: NCT00552305]). Patients were enrolled following participation in a double-blind trial or one of two open-label trials of adjunctive lacosamide for partial-onset seizures. Dosage adjustments of lacosamide (100-800 mg/day) and/or concomitant antiepileptic drugs were allowed to optimize tolerability and seizure reduction. Of the 370 enrolled patients, 77%, 51%, and 39% had >1, >3, or >5 years of lacosamide exposure, respectively. Median lacosamide modal dose was 400mg/day. Common treatment-emergent adverse events (TEAEs) were dizziness (39.7%), headache (20.8%), nausea (17.3%), diplopia (17.0%), fatigue (16.5%), upper respiratory tract infection (16.5%), nasopharyngitis (16.2%), and contusion (15.4%). Dizziness (2.2%) was the only TEAE that led to discontinuation in >2% of patients. Ranges for median percent reductions in seizure frequency were 47-65%, and those for ≥ 50% responder rates were 49-63% for 1-, 3-, and 5-year completer cohorts. Exposure to lacosamide for up to 8 years was generally well tolerated, with a safety profile similar to previous double-blind trials, and efficacy was maintained.
Kissin, M Ya; Bondarenko, I I
The aim of the study was to evaluate the efficacy and safety of lacosamide (100-400 mg per day) as adjunctive treatment to other antiepileptic drugs (AED) in patients with uncontrolled partial epilepsy. A retrospective evaluation of efficacy and safety of lacosamide was made in 56 patients, aged 17-58 years, with focal seizures with- or without secondary localization during 6 months. Lacosamide was used with other AED (carbamazepine, oxcarbazepine, lamotrigine, valproates, levetiracetam, topiramate); in 73,2% patients it was administered with two additional AED. In 69,6% patients, the dose of lacosamide was 300 mg per day. The complete cessation of seizures was achieved in 8 (14,3%) patients, 18 (32,1%) patients had 50-75% reduction in seizures, 17 (30,4%) had 50% reduction. There were no changes in 3 (23,2) patients. Dose-dependent adverse effects were diplopia, vertigo, sleepiness, skin allergic reactions. No side-effects were caused by drug intolerance. Lacosamide is believed to open new possibilities in treatment of epilepsy.
Carlton, Jill; Kaltenthaler, Eva
Background/aims Health-related quality of life (HRQoL) measures are used in healthcare to help inform clinical decision-making and policy-making decisions. A number of disease-specific or condition-specific measures have been developed and applied in ophthalmology; however, their use in the specific fields of amblyopia and strabismus are not as established. The purpose of this study is to identify and discuss specific HRQoL instruments that may be used in the investigation and management of patients with amblyopia and/or strabismus. Methods A systematic literature review was undertaken in November 2009. The electronic databases of AMED (Allied and Complementary Medicine: 1985 to November 2009), the British Nursing Index and Archive (1985 to October 2009), Ovid Medline In-Process and Other Non-Indexed Citations and Ovid Medline (1950 to present) and PsycINFO (1806 to November Week 1 2009) were searched. No language restrictions were applied to the search. Results Four instruments were identified: the Amblyopia and Strabismus Questionnaire (A&SQ), the Amblyopia Treatment Index (ATI), the Adult Strabismus Questionnaire (AS-20) and the Intermittent Exotropia Questionnaire (IXTQ). Conclusion The use of HRQoL measures in patients with amblyopia and/or strabismus is a developing area. Further research is necessary to determine the impact of issues such as diplopia and poor cosmesis upon patient groups, and to determine the influence of ethnicity and parental reporting in these patients. PMID:20693563
Kalwani, Neil; Remenschneider, Aaron K.; Faquin, William; Ferry, Judith; Holbrook, Eric H.
Background and Importance Plasmacytomas are monoclonal proliferations of plasma cells that may arise within soft tissue or bone. The skull base is a rare site for plasmacytomas to occur, and few cases have been reported in the literature. When present in the skull base, plasmacytomas may result in cranial neuropathies and often progress to multiple myeloma more rapidly than other intracranial or skeletal plasmacytomas. Clinical Presentation A 69-year-old man presented with a primary complaint of diplopia and an examination consistent with bilateral abducens nerve palsy. No other deficits were noted. Magnetic resonance imaging of the skull base demonstrated a large T1 isointense moderately enhancing lesion centered within the clivus. Endoscopic biopsy of the mass revealed sheets and aggregates of mature monoclonal plasma cells. The patient's initial systemic work-up revealed that this was a solitary lesion, and he was treated with radiation therapy to the skull base with a durable local effect at 18-month follow-up. Unfortunately he progressed to multiple myeloma with peripheral osteolytic lesions but has been stabilized on chemotherapeutics. Conclusion The clivus is an unusual site for intracranial plasmacytomas, and enhancing lesions must be differentiated from chordoma. Characteristic findings on histopathology include an immunoglobulin light-chain restricted clonal proliferation of plasma cells. Treatment is most commonly radiotherapy with surgery reserved for biopsy and palliation. Clinicians should be aware of the increased risk of progression to multiple myeloma in skull base plasmacytomas. PMID:26251795
Liu, Jonathan; Ahmadpour, Arjang; Bewley, Arnaud F.; Lechpammer, Mirna; Bobinski, Matthew; Shahlaie, Kiarash
Background and Importance Chondroblastoma is a benign primary bone tumor that typically develops in the epiphyses of long bones. Chondroblastoma of the craniofacial skeleton is extremely rare, with most cases occurring in the squamosal portion of the temporal bone. In this report, we describe the first case of chondroblastoma of the clivus presenting with cranial neuropathy that was treated with endoscopic endonasal resection. We review the literature on craniofacial chondroblastomas with particular emphasis on extratemporal lesions. Case Presentation A 27-year-old woman presented with severe headache, left facial dysesthesias, and diplopia. Physical examination revealed hypesthesia in the left maxillary nerve dermatome, and complete left abducens nerve palsy. Imaging demonstrated an expansile intraosseous mass originating in the upper clivus with extension superiorly into the sella turcica and laterally to involve the medial wall of the left cavernous sinus. The tumor was completely resected via an endoscopic endonasal approach, with postoperative improvement in lateral gaze palsy. Histopathology was consistent with chondroblastoma. Conclusion Chondroblastoma is a rare tumor of the craniofacial skeleton that should be included in the differential diagnosis of an osteolytic lesion of the clivus. Complete surgical resection remains the mainstay of treatment. PMID:26623238
Peters, Anthea; Olateju, Tolu; Deschenes, Jean; Shankarnarayan, Santosh H; Chua, Neil; Shapiro, A M James; Senior, Peter
We report the first two cases of post-transplant lymphoproliferative disorder (PTLD) in recipients of islet transplants worldwide. First, a 44-year old recipient of 3 islet infusions developed PTLD 80 months after his initial transplant, presenting with abdominal pain and diffuse terminal ileum thickening on imaging. He was treated with surgical excision, reduction of immunosuppression and rituximab. Seven months later he developed central nervous system PTLD, presenting with vertigo and diplopia; immunosuppression was discontinued, resulting in graft loss, and he was given high-dose methotrexate and consolidative autologous stem cell transplant. He remains in remission 37 months after initial diagnosis. Second, a 58-year old female recipient of 2 islet infusions developed PTLD 24 months after initial islet infusion, presenting with pancytopenia secondary to extensive bone marrow involvement. Immunosuppression was discontinued, resulting in graft loss, and she received rituximab and chemotherapy, achieving complete remission. Both cases were monomorphic B-cell PTLD subtype by histology and negative for EBV in tissue or blood. These cases document the first occurrences of this rare complication in islet transplant, likely secondary to prolonged, intensive immunosuppression, and highlight the variable clinical manifestations of PTLD. Further studies are needed to determine incidence rate and risk factors in islet transplantation. This article is protected by copyright. All rights reserved.
Sera, I; Shibayama, K; Motomura, M; Tsujihata, M; Nagataki, S
A 46-year-old woman developed blepharoptosis, diplopia and hypogeusia in 1984, and was diagnosed as having myasthenia gravis and malignant thymoma. Her symptoms improved by thymectomy and oral prednisolone administration. In 1988 she complained of breast pain and swelling during menstruation. In 1990 her breast was enlarged to the size of an adult head, and she developed erythroderma and hypogeusia. She was admitted to Nagasaki University Hospital in August, 1990. On admission, erythema was seen in the whole body especially in the face, anterior chest, abdomen and the thigh. Her breast was the size of an adult head. Muscle strength was weak, and ocular movement was limited. She had blepharoptosis. Papillae of the tongue were atrophic. Endoclinological investigations revealed an elevation of PRL to 14.6 ng/ml (normal < 10), antibodies to the acetylcholine receptor to 118.0 nM (normal < 0.5). In TRH stimulation test the response of PRL was amplified. Four intrathoracic masses suspected of recurrence of malignant thymoma were seen in CT. Her macromastia depended upon the amount of PRL, and was reduced by bromocriptine. Erythroderma and hypogeusia were relieved by plasmaphersis. Therefore autoimmune mechanism may be related to these symptoms.
Bauer, Sebastian; Willems, Laurent M.; Paule, Esther; Petschow, Christine; Zöllner, Johann Philipp; Rosenow, Felix; Strzelczyk, Adam
Lacosamide (LCM) is approved for anticonvulsive treatment in focal epilepsy and exhibits its function through the slow inactivation of voltage-gated sodium channels (VGSCs). LCM shows comparable efficacy with other antiepileptic drugs (AEDs) licensed in the last decade: in three randomized placebo-controlled trials, significant median seizure reduction rates of 35.2% for 200 mg/day, 36.4–39% for 400 mg/day and 37.8–40% for 600 mg/day were reported. Likewise, 50% responder rates were 38.3–41.1% for 400 mg/day and 38.1–41.2% for 600 mg/day. Similar rates were reported in post-marketing studies. The main adverse events (AEs) are dizziness, abnormal vision, diplopia and ataxia. Overall, LCM is well tolerated and has no clinically-relevant drug–drug interactions. Due to the drug’s intravenous availability, its use in status epilepticus (SE) is increasing, and the available data are promising. PMID:28382109
Iwasaki, Yasushi; Saito, Yufuko; Mori, Keiko; Ito, Masumi; Mimuro, Maya; Aiba, Ikuko; Saito, Kozo; Mizuta, Ikuko; Yoshida, Tomokatsu; Nakagawa, Masanori; Yoshida, Mari
A 50-year-old Japanese man with no apparent family history noticed diplopia. He gradually showed gait disturbance and dysuria. Abducens disorder of eye movement with nystagmus, tongue atrophy with fasciculation, spastic tetraparesis, and sensory disturbance were also observed. MRI showed severe atrophy of the medulla oblongata to the cervical cord ("tadpole appearance"). Tracheotomy and gastrostomy were performed 7 years after onset due to the development of bulbar palsy. Death occurred following respiratory failure after 11 years total disease duration. The brain weighed 1,380 g. The cerebrum, cerebellum, midbrain, and upper pons were preserved from atrophy, but the medulla oblongata to the cervical cord showed severe atrophy. A few Rosenthal fibers were observed in the cerebral white matter, basal ganglia, and cerebellum, whereas numerous Rosenthal fibers were observed in the medulla oblongata to the cervical cord. Myelin loss with relatively preserved axons was extensively observed from the middle of the pons to the spinal cord. The clinicopathological diagnosis was adult-onset bulbospinal-form Alexander disease. Glial fibrillary acidic protein (GFAP) gene analysis revealed a novel mutation of S393R. Expression patterns of S393R mutant GFAP using adrenal carcinoma-derived cells (SW13 cells) showed a decreased number of filamentous structures and abnormal aggregates.
Rowe, Fiona J; Wright, David; Brand, Darren; Jackson, Carole; Harrison, Shirley; Maan, Tallat; Scott, Claire; Vogwell, Linda; Peel, Sarah; Akerman, Nicola; Dodridge, Caroline; Howard, Claire; Shipman, Tracey; Sperring, Una; Macdiarmid, Sonia; Freeman, Cicely
Aim. To evaluate the profile of ocular gaze abnormalities occurring following stroke. Methods. Prospective multicentre cohort trial. Standardised referral and investigation protocol including assessment of visual acuity, ocular alignment and motility, visual field, and visual perception. Results. 915 patients recruited: mean age 69.18 years (SD 14.19). 498 patients (54%) were diagnosed with ocular motility abnormalities. 207 patients had gaze abnormalities including impaired gaze holding (46), complete gaze palsy (23), horizontal gaze palsy (16), vertical gaze palsy (17), Parinaud's syndrome (8), INO (20), one and half syndrome (3), saccadic palsy (28), and smooth pursuit palsy (46). These were isolated impairments in 50% of cases and in association with other ocular abnormalities in 50% including impaired convergence, nystagmus, and lid or pupil abnormalities. Areas of brain stroke were frequently the cerebellum, brainstem, and diencephalic areas. Strokes causing gaze dysfunction also involved cortical areas including occipital, parietal, and temporal lobes. Symptoms of diplopia and blurred vision were present in 35%. 37 patients were discharged, 29 referred, and 141 offered review appointments. 107 reviewed patients showed full recovery (4%), partial improvement (66%), and static gaze dysfunction (30%). Conclusions. Gaze dysfunction is common following stroke. Approximately one-third of patients complain of visual symptoms, two thirds show some improvement in ocular motility.
Sarafraz, Alireza; Chamani, Mojtaba; Derakhshandeh, Hossein
Background Inadequate epidemiologic investigations of the paranasal sinuses malignancies prompted this retrospective study with special emphasis on a major group of 111 tumors. Material and Methods Clinical records of 111 patients with histologically confirmed malignant tumors of the paranasal sinuses were investigated retrospectively from April 2000 to January 2012. Collection of data included demographic information, clinical manifestations, treatment plans, and histopathology of the tumor. Results There were 69 (62.16%) male and 42 (37.83%) female patients (male-to-female ratio of 1.6:1), with a median age of 49±12.2 years (range 21 to 88 years). A high level of occurrence was noticed in the fifth (26.3%) decade of life. The most frequent histological types were squamous cell carcinoma (43.5%) and adenoid cystic carcinoma (19%). Among clinical manifestations, nasal obstruction was the most frequent followed by diplopia, and facial swelling. Fifty three patients (47.74%) were treated with combined approach of surgery and radiation therapy. Conclusions Paranasal sinuses malignancies are rare conditions with nonspecific symptoms which make early diagnosis of the lesions more challenging. The optimal therapeutic protocol for patients suffering from these tumors is still a somewhat controversial entity and requires further studies. Key words:Paranasal sinuses, malignancy, surgery,radiotherapy. PMID:27475693
Moore, Kenneth A.; Bohnstedt, Bradley N.; Shah, Sanket U.; Abdulkader, Marwah M.; Bonnin, Jose M.; Ackerman, Laurie L.; Shaikh, Kashif A.; Kralik, Stephen F.; Shah, Mitesh V.
Background: Chordomas are rare, slow-growing malignant neoplasms derived from remnants of the embryological notochord. Pediatric cases comprise only 5% of all chordomas, but more than half of the reported pediatric chordomas are intracranial. For patients of all ages, intracranial chordomas typically present with symptoms such as headaches and progressive neurological deficits occurring over several weeks to many years as they compress or invade local structures. There are only reports of these tumors presenting acutely with intracranial hemorrhage in adult patients. Case Description: A 10-year-old boy presented with acute onset of headache, emesis, and diplopia. Head computed tomography and magnetic resonance imaging of brain were suspicious for a hemorrhagic mass located in the left petroclival region, compressing the ventral pons. The mass was surgically resected and demonstrated acute intratumoral hemorrhage. Pathologic examination was consistent with chordoma. Conclusion: There are few previous reports of petroclival chordomas causing acute intracranial hemorrhage. To the authors’ knowledge, this is the first case of a petroclival chordoma presenting as acute intracranial hemorrhage in a pediatric patient. Although uncommon, it is important to consider chordoma when evaluating a patient of any age presenting with a hemorrhagic lesion of the clivus. PMID:25949851
Wang, Arthur; Carberry, Nathan; Solli, Elena; Kleinman, George; Tandon, Adesh
We present an unusual case of a metastatic mantle cell lymphoma (MCL) to the pituitary gland. The patient had a known history of MCL for which she previously received chemotherapy. She presented with new-onset diplopia and confusion, and reported a history of progressive vision blurriness associated with headache, nausea, and vomiting. MRI of the brain showed an enhancing lesion within the sella turcica involving the cavernous sinuses bilaterally, extending into Meckel's cave on the left, and abutting the optic nerves bilaterally. Following surgical excision, histopathology revealed the tumor to be a MCL. Metastatic pituitary tumors are rare and have been estimated to make up 1% of tumors discovered in the sellar region. The two most common secondary metastatic lesions to the sella are breast and lung carcinoma followed by prostate, renal cell, and gastrointestinal carcinoma. Metastatic lymphoma to the pituitary gland is especially rare and is estimated to constitute 0.5% of all metastatic tumors to the sella turcica. To our knowledge, this is the first reported case of MCL metastasizing to the pituitary gland. PMID:26933415
Kalich, Melvyn E.; Rash, Clarence E.; van de Pol, Corina; Rowe, Terri L.; Lont, Lisa M.; Peterson, R. David
Biocular helmet-mounted display (HMD) design flexibility and cost are directly related to image misalignment tolerance standards. Currently recommended tolerance levels are based on highly variable data from a number of studies. This paper presents progress of an ongoing study to evaluate optometric measures sensitive to misalignment in partial-overlap biocular optical systems like that proposed for the Comanche RAH-66 helicopter helmet integrated display sighting system (HIDSS). Horizontal divergent and relative vertical misalignments (offsets) of see-through biocular symbology viewed against a simulated daytime background were chosen for this study. Misalignments within and just beyond current tolerance recommendations were evaluated using pre, pre and post, and during measures of visual performance. Data were obtained from seven experimental and four control subjects. The diplopia responses from experimental and control subjects were essentially the same. However, accommodative facility showed a rate decrement following exposure to both types of misalignment. Horizontal heterophorias showed definite post-misalignment increases. Subject responses to questionnaires universally indicated increased adaptation to (ease with) visual tasks over the testing period.
Matsumoto, Hideyuki; Seki, Naoko; Yamamoto, Tomotaka; Oshima, Kumi; Asai, Takashi; Motokura, Toru; Ugawa, Yoshikazu; Goto, Jun; Tsuji, Shoji
A 47-year-old man, who suffered from acute lymphocytic leukemia at 45 years old and was treated with hematopoietic stem cell transplantation at 46 years old after the induction of complete remission by the standard chemotherapy, developed the symptoms of chronic graft-versus-host disease (cGVHD) such as dry eyes, dry mouth, skin thickening, skin scaling, skin pigmentation and impaired liver function. He was admitted to our hospital because of the acute development of diplopia and weakness of his left upper extremity accompanying with the exacerbation of other symptoms of cGVHD. Neurological examinations revealed the right abducens nerve palsy and asymmetric muscular weakness of the extremities; the proximal part of the left upper extremity and the distal part of the right upper extremity were markedly involved. Neurophysiological studies including magnetic motor root stimulation revealed demyelinating neuropathy specifically involving the motor nerves. On the basis of these findings, a diagnosis of peripheral neuropathy associated with cGVHD was made. Nighteen reports are available on peripheral neuropathy in cGVHD patients, but to date little is known about the pathophysiology of this condition. Most of those patients have been diagnosed as having symmetric demyelinating polyneuropathy, such as Guillain-Barré syndrome or chronic inflammatory demyelinating polyneuropathy. In this study, contrary to previous reports, the asymmetric involvement of motor nerves is noteworthy. Accumulation and further analyses of the cases like the present case are necessary to elucidate the pathogenesis of peripheral neuropathy in cGVHD.
Monteiro, Mário Luiz Ribeiro; Gonçalves, Allan Christian Pieroni; Bezerra, Alanna Mara Pinheiro Sobreira
The diagnosis of Graves' orbitopathy is usually straightforward. However, orbital diseases that mimick some clinical signs of Graves' orbitopathy may cause diagnostic confusion, particularly when associated to some form of thyroid dysfunction. This report describes the rare occurrence of localized inferior rectus muscle amyloidosis in a patient with autoimmune hypothyroidism, who was misdiagnosed as Graves' orbitopathy. A 48-year-old man complained of painless progressive proptosis on the left side and intermittent vertical diplopia for 6 months. The diagnosis of Graves' orbitopathy was entertained after magnetic resonance imaging revealing a markedly enlarged, tendon-sparing inferior rectus enlargement on the left side, and an autoimmune hypothyroidism was disclosed on systemic medical workup. After no clinical improvement with treatment, the patient was referred to an ophthalmologist and further investigation was performed. The presence of calcification in the inferior rectus muscle on computed tomography, associated with the clinical findings led to a diagnostic biopsy, which revealed amyloid deposition. This report emphasizes that a careful evaluation of atypical forms of Graves' orbitopathy may be crucial and should include, yet with rare occurrence, amyloidosis in its differential diagnosis. RESUMO O diagnóstico de orbitopatia de Graves usualmente é fácil de ser estabelecido. No entanto, doenças da órbita que simulam alguns sinais clínicos da orbitopatia de Graves podem levar à confusão diagnóstica, particularmente quando associada à alguma forma de disfunção tireoidiana. Relatamos a ocorrência rara de amiloidose localizada no músculo reto inferior em paciente com hipotireoidismo autoimune, que recebeu inicialmente o diagnóstico errôneo de orbitopatia de Graves. Paciente masculino, 48 anos, com queixa de proptose progressiva e indolor do lado esquerdo e diplopia vertical intermitente há 6 meses. O diagnóstico de orbitopatia de Graves foi
Hwang, Kun; Kim, Dong Hyun
= 198, 82.2%). At the infraorbital rim (n = 364), internal fixation was carried out in most cases (n = 257, 70.6%). At the zygomaticomaxillary buttress (n = 279), internal fixation was performed in about one third of the cases (n = 91, 32.6%). At the zygomatic arch (n = 339), only 1 case (0.3%) was fixed internally. The postoperative complication rate occurred in 88 cases (19.1%) among 461 cases operated. The most common complication was hypesthesia (50 cases, 56.8%), followed by diplopia (15 cases, 17.0%), limitation of mouth opening or closure (11 cases, 12.5%), infection (6.8%), and hematoma (4.5%). Most patients with hypesthesia improved at 2 months. About 90% of the patients with diplopia improved within 2 months. Limitation of mouth opening was improved immediately after operation in most of the cases. Our findings demonstrate significant differences in the demographics and clinical presentation that will enable a more accurate diagnosis and prediction of concomitant injuries and sequelae.
Lee, Dong Min; Yu, Seung Hee; Yoon, Hyun Hwa; Lee, Kang Lock; Eom, Young Sil; Lee, Kiyoung; Kim, Byung-Joon; Kim, Yeun Sun; Park, Ie Byung; Kim, Kwang-Won
Background Multiple endocrine neoplasia type 1 (MEN1) is a rare inherited disorder characterized by the simultaneous occurrence of endocrine tumors in target tissues (mainly the pituitary, endocrine pancreas, and parathyroid glands). MEN1 is caused by mutations in the MEN1 gene, which functions as a tumor suppressor and consists of one untranslated exon and nine exons encoding the menin protein. This condition is usually suspected when we encounter patients diagnosed with tumors in multiple endocrine organs, as mentioned above. Methods A 65-year-old woman who underwent surgery for a pancreatic tumor (serous cystadenoma) 5 years previously was referred to our hospital due to neurologic symptoms of diplopia and left ptosis. Brain magnetic resonance imaging revealed a 3.4-cm lesion originating from the cavernous sinus wall and extending into the sellar region. It was thought to be a nonfunctioning tumor from the results of the combined pituitary function test. Incidentally, we found that she also had a pancreatic tumor, indicating the necessity of genetic analysis for MEN1. Results Genomic analysis using peripheral leukocytes revealed a heterozygous c.1621G>A mutation in the MEN1 gene that was previously reported to be either a pathogenic mutation or a simple polymorphism. We pursued a stereotactic approach to the pituitary lesion, and microscopic findings of the tumor revealed it to be an intrasellar cavernous hemangioma, a rare finding in the sellar region and even rarer in relation to oculomotor palsy. The patient recovered well from surgery, but refused further evaluation for the pancreatic lesion. Conclusion There is great emphasis placed on genetic testing in the diagnosis of MEN1, but herein we report a case where it did not assist in diagnosis, hence, further discussion on the role of genetic testing in this disease is needed. Also, in cases of pituitary tumor with cranial nerve palsy, despite its low prevalence, intrasellar cavernous hemangioma could be
Bitik, Berivan; Kılıç, Levent; Küçükşahin, Orhan; Şahin, Kubilay; Tufan, Abdurrahman; Karadağ, Ömer; Pay, Salih; Ateş, Aşkın; Ucar, Murat; Tutar, Hakan; Karaaslan, Yaşar; Yilmaz, Sedat; Ertenli, A Ihsan; Konuk, Onur; Turgay, Murat; Goker, Berna
Retro-orbital granuloma is a rare and devastating component of granulomatosis with polyangiitis (GPA). Current medical treatment protocols are falling short, and outcomes are poor. The aim of the study was to investigate the frequency, clinical features, and treatment outcomes of retro-orbital granuloma in patients with GPA. This is a retrospective, multi-centre study, which involves GPA cohorts from five different clinics. Data were extracted from patient charts including history, physical examination, radiological-laboratory-histological findings, and treatment protocols. Major clinical outcome measures were changes in the volume of the granuloma on comparative MRI, and visual acuity on repeated ophthalmologic examinations. Among 141 GPA patients, nine (five females and four males) were diagnosed with a retro-orbital granuloma. Median duration of disease was 8 years. Proptosis and diplopia were the dominant presenting symptoms (77%), followed by orbital pain (55%). Three out of nine patients had isolated retro-orbital granulomas, without other organ involvement of GPA. Five patients received conventional pulse steroid and pulse cyclophosphamide (CYC) as the first-line remission induction therapy. Four of these patients had progressive disease, and a regression in granuloma size was observed in one patient using this regimen. Two patients were already receiving immunosuppressants when they were diagnosed with retro-orbital granuloma. Six patients had been treated with RTX as the second-line remission induction therapy. None of these patients had progression following RTX therapy. Three patients underwent orbital decompression surgery. The indication for the decision for surgery was either progressive loss of vision or intractable pain. Standard first-line chemotherapy (CYC and steroids) was ineffective against retro-orbital granuloma associated with GPA. RTX could be an alternative in these cases. Surgical intervention may help to decrease the morbidity. Further
Karsen, Hasan; Karahocagil, Mustafa Kasim; Irmak, Hasan; Demiröz, Ali Pekcan
Turkey is located at an endemic area for brusellosis and tuberculosis which are both important public health problems. Meningitis caused by Brucella and Mycobacterium spp. may be confused since the clinical and laboratory findings are similar. In this report, a meningitis case with Brucella and tuberculosis co-infection has been presented. A 19-years-old woman was admitted to our clinic with severe headache, fever, vomiting, meningeal irritation symptoms, confusion and diplopia. The patient was initially diagnosed as Brucella meningitis based on her history (stockbreeding, consuming raw milk products, clinical symptoms concordant to brucellosis lasting for 4-5 months), physical examination and laboratory findings of cerebrospinal fluid (CSF). Standard tube agglutination test for brucellosis was positive at 1/80 titer in CSF and at 1/640 titer in serum, whereas no growth of Brucella spp. was detected in CSF and blood cultures. Antibiotic therapy with ceftriaxone, rifampicin and doxycyclin was started, however, there was no clinical improvement and agitation and confusion of the patient continued by the end of second day of treatment. Repeated CSF examination yielded acid-fast bacteria. The patient was then diagnosed as meningitis with double etiology and the therapy was changed to ceftriaxone, streptomycin, morphozinamide, rifampicin and isoniazid for thirty days. Tuberculosis meningitis was confirmed with the growth of Mycobacterium tuberculosis on the 14th day of cultivation (BACTEC, Becton Dickinson, USA) of the CSF sample. On the 30th day of treatment she was discharged on anti-tuberculous treatment with isoniazid and rifampicin for 12 months. The follow-up of the patient on the first and third months of treatment revealed clinical and laboratory improvement. Since this was a rare case of Brucella and tuberculosis co-infection, this report emphasizes that such co-infections should be kept in mind especially in the endemic areas for tuberculosis and brucellosis.
Valles, Jennie M.; Fekete, Robert
Introduction In 1904, Giuseppe Gradenigo published his case series on the triad of ipsilateral abducens nerve palsy, facial pain in the trigeminal nerve distribution, and suppurative otitis media, which would subsequently be referred to as Gradenigo syndrome. Case Report Our patient was a 36-year-old female, 23 weeks pregnant, with a 6-day history of right-sided otalgia and hearing loss and a 4-day history of purulent otorrhea, who presented with severe, holocephalic headache, meningeal signs, fever, photophobia, and mental status decline. Lumbar puncture yielded a white blood cell count of 1,559 cells/mm3 with 95% polymorphonuclear leukocytes, a red blood cell count of 111 cells/mm3, a protein level of 61 mg/dl, and a glucose level of <40 mg/dl. Cerebrospinal fluid Gram stain showed Gram-positive diplococci, which were subsequently identified as Streptococcus pneumoniae and treated with ceftriaxone. On the second hospital day, she developed horizontal diplopia due to right abducens nerve palsy and right mydriasis. Both symptoms resolved on the third hospital day. Erosion of temporal bone and opacification of mastoid air cells was shown on CT scan. A CT venogram showed an irregularity of the left transverse and superior sagittal sinuses. She was treated with enoxaparin for possible sinus thrombosis. Discussion This case demonstrates rare but serious sequelae of otitis media and Gradenigo syndrome. Holocephalic headache from meningitis masked trigeminal pain. Involvement of the ipsilateral petrous apex and surrounding structures on imaging and clinical improvement with antibiotic treatment supports Gradenigo syndrome over intracranial hypertension due to venous sinus thrombosis as the cause of the abducens nerve palsy. PMID:25232331
Pollock, Bruce E. . E-mail: email@example.com; Stafford, Scott L.
Introduction: The purpose of this study was to evaluate the efficacy and safety of stereotactic radiosurgery as primary management for patients with imaging defined cavernous sinus meningiomas. Methods: Between 1992 and 2001, 49 patients had radiosurgery for dural-based masses of the cavernous sinus presumed to be meningiomas. The mean patient age was 55.5 years. The mean tumor volume was 10.2 mL; the mean tumor margin dose was 15.9 Gy. The mean follow-up was 58 months (range, 16-144 months). Results: No tumor enlarged after radiosurgery. Twelve of 38 patients (26%) with preexisting diplopia or facial numbness/pain had improvement in cranial nerve function. Five patients (10%) had new (n = 3) or worsened (n = 2) trigeminal dysfunction; 2 of these patients (4%) underwent surgery at 20 and 25 months after radiosurgery despite no evidence of tumor progression. Neither patient improved after partial tumor resection. One patient (2%) developed an oculomotor nerve injury. One patient (2%) had an ischemic stroke related to occlusion of the cavernous segment of the internal carotid artery. Event-free survival was 98%, 85%, and 80% at 1, 3, and 7 years after radiosurgery, respectively. Univariate analysis of patient and dosimetric factors found no analyzed factor correlated with postradiosurgical morbidity. Conclusions: Radiosurgery was an effective primary management strategy for patients with an imaging defined cavernous sinus meningioma. Except in situations of symptomatic mass effect, unusual clinical presentation, or atypical imaging features, surgery to confirm the histologic diagnosis is unlikely to provide clinical benefit.
Matis, Georgios K.; de A. Silva, Danilo O.; Chrysou, Olga I.; Karanikas, Michail A.; Birbilis, Theodossios A.
Background: Giuseppe Gradenigo (1859–1926), a legendary figure of Otology, was born in Venice, Italy. He soon became a pupil to Adam Politzer and Samuel Leopold Schenk in Vienna, demonstrating genuine interest in the embryology, morphology, physiopathology, as well as the clinical manifestations of ear diseases. In this paper, the authors attempt to highlight the major landmarks during Gradenigo's career and outline his contributions to neurosciences, which have been viewed as looking forward to the 20th century rather than awkward missteps at the end of the 19th. Methods: Several rare photographs along with many non-English, more than a century old articles have been meticulously selected to enrich this historical journey in time. Results: It was after Gradenigo that the well-known syndrome consisting of diplopia and facial pain due to a middle ear infection was named. However, Gradenigo was much more than a syndrome. Surprisingly, despite the fact that he is considered a pioneer of the Italian Otology of the late 19th and early 20th century, little is written of his life and his notable achievements in the English literature. Conclusions: Even though his name lives on nowadays only in the eponym “Gradenigo's syndrome,” his accomplishments are much wider and cast him among the emblematic figures of science. His inherent tendency for discovering the underlying mechanisms of diseases and his vision of guaranteeing quality of services, professional proficiency, respect, and dedication toward the patients is in fact what constitutes his true legacy to the next generations. PMID:23226608
AIM To evaluate the visual findings as primary manifestations in patients with intracranial tumors. METHODS The medical charts of the patients with intracranial tumors who initially admitted to the Neuro-ophthalmology and Strabismus Department with ocular complaints between August 1999 and December 2012 were reviewed retrospectively. The detailed clinical history and the findings of neuro-ophthalmologic examination were recorded. Ocular symptoms and signs, the types and locations of intracranial tumors, and the duration of symptoms before the diagnosis were evaluated. RESULTS The mean age of 11 women (61.1%) and 7 men (38.9%) was 42.2±11.0 (range 20-66y) at the time of intracranial tumor diagnosis. Initial symptoms were transient visual obscurations, visual loss or visual field defect in 16 cases (88.9%), and diplopia in 2 cases (11.1%). Neuro-ophthalmologic examination revealed normal optic discs in both eyes of 6 patients (33.3%), paleness, atrophy or edema of optic disc in 12 patients (66.7%), and sixth cranial nerve palsy in 2 patients (11.1%). Visual acuity ranged between normal vision and loss of light perception. Cranial imaging demonstrated craniopharyngioma (n=1), plasmacytoma (n=1), meningioma (n=6; olfactory groove and tuberculum sellae, pontocerebellar angle, anterior cranial fossa, frontal vertex, suprasellar region), and pituitary macroadenoma (n=10). The mean duration between the onset of visual disturbances and the diagnosis of intracranial tumor was 9.8±18mo (range 3d-6y). CONCLUSION The ophthalmologist is frequently the first physician to encounter a patient with clinical manifestations of intracranial tumors that may cause neurological and ocular complications. Neuro-ophthalmologic findings should be carefully evaluated to avoid a delay in the diagnosis of intracranial tumors. PMID:26309882
Kashima, Tomoyuki; Goldberg, Robert A; Kohn, Jocelyne C; Rootman, Daniel B
Purpose Chronic maxillary atelectasis is characterized by unilateral spontaneous enophthalmos and hypoglobus due to increased orbital volume secondary to maxillary sinus inward deformation. Reformation of the sinus architecture and reconstruction of the orbit are key to a successful outcome. Here, we introduce a one-staged surgery that addresses both these goals. Patients and methods We retrospectively reviewed 11 patients treated with one-stage orbital and sinus surgery. A transconjunctival subperiosteal approach was used to create slats in the thinned orbital floor. A nasal endoscopic approach was utilized to access the maxillary sinus and place a modified Foley catheter balloon through the enlarged maxillary ostium. A bridge graft of nasal septal, ear cartilage, or LactSorb was placed on the reconstructed and balloon-supported orbital floor. The balloon was deflated and removed at 10–14 days. All patients underwent complete ophthalmic and orbital evaluation, including standardized photography and radiologic imaging. Results Eleven patients, mean age 39.5 years, presented with diplopia in upgaze, superior sulcus deformity, and at least 2 mm of relative enophthalmos. After initial overcorrection, enophthalmos improved in all cases. Symmetry within 1 mm was accomplished in 10 of 11 cases. Follow-up time was 259±320 days. Full motility was recovered in all patients. Conclusion We describe a one-staged surgery consisting of cutting slats in the orbital floor, dilating the maxillary sinus with a balloon, and stabilizing the orbital floor with a cartilage graft placement. Our anecdotal experience suggests that this surgical approach can safely achieve normalization of the pathologic sinus outflow and restoration of the orbit anatomy. The balloon ensures orbital floor stability during the healing process, and it may act to stent open the sinus ostium during early mucosal healing. PMID:27932858
Kikuchi, A; Chida, K; Misu, T; Okita, N; Nomura, H; Konno, H; Takase, S; Takeda, A; Itoyama, Y
A 68-year-old man was hospitalized on March 4, 1998 for disturbances in consciousness. In 1995, he had received proximal subtotal gastrectomy and reconstructive surgery of the jejunal interposition for gastric cancer. Thereafter he had been taking enough food without the habit of taking liquor. In October 1997, his short term memory was becoming gradually worse. On February 12, 1998, he suffered from numbness in the feet, and then dysphagia, unsteady gait, and diplopia developed gradually. On February 26, brain MRI showed no abnormalities. On March 3, he had a fever of 38.5 degrees C and his consciousness became unclear. Neurological examination revealed semi-coma, total ophthalmoplegia, and absence of doll's eye movement. Deep tendon reflexes were absent. The serum thiamine level was 9 ng/ml (normal range: 20-50). Brain MRI demonstrated symmetrical high intensity lesions in the periaqueductal area of the midbrain, dorsomedial nuclei of bilateral thalami, and vestibular nuclei. About 30 seconds after intravenous infusion of thiamine, his consciousness improved dramatically, but returned to semi-coma after about two minutes. Wernicke-Korsakoff syndrome usually occurs acutely. In the present case, however, the disease showed slow onset, chronic progression, and then rapid worsening after fever. Reconstructive surgery of the jejunal interposition might have caused the slow onset of Wernicke-Korsakoff syndrome, and fever might have facilitated the rapid progression of the disease. An immediate high concentration of thiamine modifies the kinetics of acetylcholine receptor ion channels, thereby maintaining wakefulness, and the level of consciousness may change dramatically.
Mbizvo, Gashirai K; Dixon, Pete; Hutton, Jane L; Marson, Anthony G
The adverse effects profile of levetiracetam in epilepsy is still being fully described. We recently published a Cochrane Review evaluating the effectiveness of levetiracetam, added on to usual care, in treating drug-resistant focal epilepsy. The five most common adverse effects were reported and analysed with no scope for reporting any less common adverse effects than those. Here, we report and analyse the remaining adverse effects (including the five most common). These were (in decreasing order of frequency) somnolence; headache; asthenia; accidental injury; dizziness; infection; pharyngitis; pain; rhinitis; abdominal pain; flu syndrome; vomiting; diarrhoea; convulsion; nausea; increased cough; anorexia; upper respiratory tract infection; hostility; personality disorder; urinary tract infection; nervousness; depression; aggression; back pain; agitation; emotional liability; psychomotor hyperactivity; pyrexia; rash; ECG abnormalities; decreased appetite; nasal congestion; irritability; abnormal behaviour; epistaxis; insomnia; altered mood; anxiety; bloody urine; diplopia; dissociation; memory impairment; pruritis; increased appetite; acne; and stomach discomfort. Only somnolence and infection were significantly associated with levetiracetam. When adverse effects pertaining to infection were combined, these affected 19.7% and 15.1% of participants on levetiracetam and placebo (relative risk 1.16, CI 0.89-1.50, Chi(2) heterogeneity p = 0.13). Somnolence and infection further retained significance in adults while no single adverse effect was significant in children. This review updates the adverse effects profile data on levetiracetam use by empirically reporting its common and uncommon adverse effects and analysing their relative importance statistically using data from a group of trials that possess low Risk of Bias and high Quality of Evidence GRADE scores.
Gao, Yuping; Jiang, Jiyao; Liu, Qiang
Primary central nervous system (CNS) germ cell tumors (GCTs) are a rare heterogeneous group of lesions, which the clinicopathological features have a marked degree of heterogeneity comparing with that of gonadal GCTs. Accurately diagnosing CNS GCTs might be extremely difficult and requires immunohistochemical verification. This study was to investigate the biological feature of CNS GCTs and diagnostic value of immunohistochemical markers OCT3/4, C-kit, PLAP, and CD30 in CNS GCTs. A retrospective study was performed on 34 patients with CNS germ cell tumors between 1990 and 2014. 34 CNS GCTs account for 9.2% of all primary CNS neoplasms. The sellar region (35.3%) and pineal gland (17.6%) were the most common sites of intracranial GCTs. Hydrocephalus (82.4%) and diplopia (46.9%) were the two most common clinical presentations. The most common histological subtypes were germinoma (67.6%). PLAP, c-kit, OCT3/4 were highly expressed in gernimomas. CD30 and CK AE1/3 stainings were positive in embryonal carcinoma. Yolk sac tumor component showed positive staining for AFP and CK AE1/3. β-HCG staining was positive in choriocarcinoma and STGC. Patients with mature teratomas and germinomas had a better prognosis (a 5-year survival rate) than those with embryonal carcinoma and choriocarcinoma (a 5-year survival rates were 0). Our finding suggest that the incidences of primary CNS GCTs are higher in South China than in the West, but mixed GCTs are uncommon in our study. The judicious use of a panel of selected markers is helpful in diagnosing and predicting the prognosis for CNS GCTs.
ŞENADIM, Songül; BOZKURT, Dilek; ÇABALAR, Murat; BAJRAMI, Arsida; YAYLA, Vildan
Introduction Almost one-third of ischemic strokes has an unknown etiology and are classified as cryptogenic stroke. Paradoxical embolism because of a patent foramen ovale (PFO) is detected in 40%–50% of these patients. Recently, PFO has been reported as a risk factor for patients of all age groups. Methods In this study, 1080 ischemic stroke patients admitted to our clinic (2011–2013) were retrospectively evaluated. Age, sex, risk factors, complete blood count, vasculitis, biochemical and hypercoagulability tests, magnetic resonance imaging, magnetic resonance angiography, transthoracic echocardiography, transeosophageal echocardiography (TEE) findings, and therapeutic approaches were evaluated. Results The age range of the participants (seven male and four female patients) was 20–60 years (mean=43.09±11.13 years). Hemiparesis (n=10), diplopia (n=2), hemianopsia (n=2), and dysarthria (n=2) were the main findings of the neurological examinations. Patient medical history revealed hypertension (n=3), asthma (n=1), deep venous thrombosis (n=1), and smoking (n=4). Diffusion-weighted imaging showed middle cerebral artery (n=8) and posterior cerebral artery (n=3) infarctions. In one case, symptomatic severe carotid stenosis was detected. In eight cases, TEE showed PFO without any other abnormalities, but PFO was associated with atrial septal aneurysm in two cases, and in one case it was associated with ventricular hypokinesia and pulmonary arterial hypertension. Antiplatelet therapy was applied to nine patients and percutaneous PFO closure operation to two patients. In a 2-year follow-up, no recurrent ischemic stroke was recorded. Conclusion PFO, especially in terms of the etiology of cryptogenic stroke in young patients, should not be underestimated. We want to emphasize the importance of TEE in identifying potential cardioembolic sources not only in young but also in all ischemic stroke patients with unknown etiology; we also discuss the controversial management
Bellastella, Antonio; Bizzarro, Antonio; Coronella, Concetta; Bellastella, Giuseppe; Sinisi, Antonio Agostino; De Bellis, Annamaria
Lymphocytic hypophysitis (LYH) is an uncommon autoimmune disease in which the pituitary gland is infiltrated by lymphocytes, plasma cells and macrophages and its function is usually impaired. It has to be suspected in pregnant women and in women with recent delivery presenting with hyperprolactinemia, headache, visual field alterations and changes of one or more pituitary hormone secretions with secondary impairment of related peripheral target glands, especially when associated with other autoimmune endocrine or non-endocrine disorders. It can also occur less frequently in prepubertal or post-menopausal women and in men. Headache, visual field impairment and more rarely diplopia are due to extrasellar pituitary enlargement with optic chiasma compression and/or to invasion of cavernous sinuses. Among the 'isolated' pituitary hormone deficiencies, ACTH deficit is usually the earliest and most frequent hormonal impairment and in rare cases can induce an acute secondary hyposurrenalism as the first sign of the disease, with high mortality in affected patients. Histopathological findings from pituitary biopsy show lymphoplasmacytic infiltrate with lymphoid aggregates surrounding atropic acini of pituitary cells; immunohistochemical analysis shows numerous mast cells randomly distributed and also localized in the vicinity of capillaries, suggesting a possible influence on capillary permeability and angiogenesis, thus favoring the inflammatory and immunological aggression against pituitary cells. Nuclear magnetic resonance imaging shows uniform sellar floor depression and an extrasellar symmetrical pituitary enlargement, usually displacing the optic chiasma, which shows a rapid homogeneous enhancement after gadolinium also involving the adjacent dura (dural tail). Antipituitary antibodies have been detected in several patients with LYH but their role needs to be clarified. Since a possible spontaneous remission can occur, a careful follow-up is required in subclinical
Tambucci, Renato; Basti, Claudia; Maresca, Maria; Coppola, Giangennaro; Verrotti, Alberto
Eslicarbazepine acetate (ESL) is a once daily new third generation antiepileptic drug that shares the basic chemical structure of carbamazepine and oxcarbazepine – a dibenzazepine nucleus with the 5-carboxamide substituent, but is structurally different at the 10,11-position. ESL is a pro-drug metabolized to its major active metabolite eslicarbazepine. Despite the fact that the exact mechanism of action has not been fully elucidated, it is thought to involve inhibition of voltage-gated sodium channels (VGSC). ESL inhibits sodium currents in a voltage-dependent way by an interaction predominantly with the inactivated state of the VGSC, thus selectively reducing the activity of rapidly firing (epileptic) neurons. ESL reduces VGSC availability through enhancement of slow inactivation. In Phase III studies, adjunctive therapy with ESL 800 or 1,200 mg/day leads to a significant decrease in the seizure frequency in adults with refractory partial onset epilepsy. Based on these results, ESL has been approved in Europe (by the European Medicines Agency) and in the United States (by the US Food and Drug Administration) as add-on therapy. Data on efficacy and safety have been confirmed by 1-year extension and real life observational studies. Recently, based on results from two randomized, double-blind, historical control Phase III trials, ESL received US Food and Drug Administration approval also as a monotherapy for patients with partial onset epilepsy. In the pediatric setting, encouraging results have been obtained suggesting its potential role in the management of epileptic children. Overall ESL was generally well tolerated. The most common adverse events were dizziness, somnolence, headache, nausea, diplopia, and vomiting. Adverse events can be minimized by appropriate titration. In conclusion, ESL seems to overcome some drawbacks of the previous antiepileptic drugs, suggesting a major role of ESL in the management of focal onset epilepsy for both new onset and
Chaudhuri, Zia; Demer, Joseph L.
Objective To propose medial rectus (MR) recession to be equally as effective as lateral rectus (LR) resection, which has heretofore been the preferred treatment for divergence paralysis esotropia (DPE). Methods We examined a 17-year surgical experience comparing LR resection with MR recession in adults with DPE, defined as symptomatic distance esotropia (ET) at least double the asymptomatic ET of 10 or less prism diopters (Δ) at near. Results Twenty-four patients with DPE underwent surgery. Six patients underwent bilateral LR resection and 2 underwent unilateral LR resection (group L), while 13 underwent bilateral MR recession and 3 underwent unilateral MR recession, with the target angle double the distance ET (group M). One of 8 patients in group L and 15 of 16 patients in group M underwent intraoperative adjustable surgery under topical anesthesia. Mean (SD) preoperative central gaze ET measured 15.0 (7.7) Δ at distance and 4.1 (3.4) Δ at near in group L, but 10.4 (6.8) Δ at distance and 0.6 (1.7) Δ at near in group M (P=.15; distance, 0.003, near). Postoperatively, no patient in either group had symptomatic diplopia or convergence insufficiency in follow-up from 8.5 to 40 months. Twice the usual surgical dose of MR recession per prism diopter was required to achieve correction of the distance deviation in DPE as compared with that recommended for ET generally and also for LR resection in the same condition. Conclusions Recession of the MR provides binocular single vision in DPE without convergence insufficiency at near, and it is convenient for intraoperative adjustment under topical anesthesia. PMID:22688183
Aureli, P; Di Cunto, M; Maffei, A; De Chiara, G; Franciosa, G; Accorinti, L; Gambardella, A M; Greco, D
In the late 1996, an outbreak of botulism affected eight young people (age of patients ranged from 6 to 23 years) in Italy. The onset of the illness was the same for all of these patients: gastrointestinal symptoms (nausea and vomiting) followed by neurologic symptoms. The most common neurologic symptoms were dysphagia, respiratory failure (100%), diplopia (87%), dysarthria, ptosis (75%) and mydriasis (50%). All patients required mechanical ventilation. Botulinum toxin was detected from two of respectively five sera and six stool samples analysed, while spores of Clostridium botulinum type A were recovered from all patient' faeces. The epidemiological investigation led to suspect a commercial cream cheese ('mascarpone') as a source of botulinum toxin: indeed, it had been eaten by all the patients before onset of the symptoms, either alone or as the (uncooked) ingredient of a dessert, 'tiramisù'. Botulinum toxin type A was found in the 'tiramisù' leftover consumed by two patients and in some mascarpone cheese samples collected from the same retail stores where the other patients had previously bought their cheeses. A break in the cold-chain at the retail has likely caused germination of C. botulinum spores contaminating the products, with subsequent production of the toxin. One of the patients died, while the others recovered very slowly. Prompt international alerting and recall of the mascarpone cheese prevented the spread of the outbreak due to the wide range of distribution, demonstrating the importance of a rapid surveillance system. None of the people complaining of symptoms after the public alert resulted positive for botulinum spores and toxin.
Leffler, Christopher T.; Vaziri, Kamyar; Schwartz, Stephen G.; Cavuoto, Kara M.; McKeown, Craig A.; Kishor, Krishna S.; Janot, Adam C.
Purpose To determine predictors of reoperation and abnormal binocularity outcomes (including amblyopia and diplopia) following pediatric strabismus surgery. Design Retrospective cross-sectional study. Methods Setting: Review of a national insurance database. Study population: Children under age 18 years having strabismus procedures between 2007 and 2013. Interventions: Adjustable or fixed suture strabismus surgery, or botulinum toxin injection. Outcome measures: Reoperation or diagnosis of abnormal binocularity in the first postoperative year. Results Of 11,115 children having strabismus procedures, 851 (7.7%) underwent reoperation. The reoperation rate was 7.4% for fixed suture surgeries, 9.6% for adjustable suture surgeries (p=0.18), and 44.9% for botulinum injections (p<0.001). Age under 2 years was associated with higher reoperation and abnormal binocularity rates (p<0.001). For horizontal strabismus, the postoperative abnormal binocularity rate was 12.8% for fixed suture surgery and 26.5% for botulinum injection (p=0.005). Reoperation rates tended to be higher with adjustable sutures (odds ratio [OR] 1.69, 95% confidence interval [CI] 0.94 to 3.03, p=0.08), or botulinum toxin injection (OR 10.36, 95% CI 5.75 to 18.66, p<0.001) and lower with 3- or 4-muscle surgery (p=0.001). Esotropia, hyperopia, and botulinum injection were independently associated with higher rates of postoperative abnormal binocularity (p<=0.005). For vertical surgeries, predictors of reoperation were adjustable suture use (OR 2.51, p=0.10) and superior oblique surgery (OR 2.36, p<0.001). Conclusions Adjustable sutures were not associated with a lower reoperation rate in children. Younger age, esotropia, hyperopia, and botulinum injection were associated with postoperative abnormal binocularity. Superior oblique surgery and botulinum injection were associated with higher rates of reoperation. PMID:26548808
Wilhelm, Dirk; Reiser, Silvano; Kohn, Nils; Witte, Michael; Leiner, Ulrich; Mühlbach, Lothar; Ruschin, Detlef; Reiner, Wolfgang; Feussner, Hubertus
Though theoretically superior, 3D video systems did not yet achieve a breakthrough in laparoscopic surgery. Furthermore, visual alterations, such as eye strain, diplopia and blur have been associated with the use of stereoscopic systems. Advancements in display and endoscope technology motivated a re-evaluation of such findings. A randomized study on 48 test subjects was conducted to investigate whether surgeons can benefit from using most current 3D visualization systems. Three different 3D systems, a glasses-based 3D monitor, an autostereoscopic display and a mirror-based theoretically ideal 3D display were compared to a state-of-the-art 2D HD system. The test subjects split into a novice and an expert surgeon group, which high experience in laparoscopic procedures. Each of them had to conduct a well comparable laparoscopic suturing task. Multiple performance parameters like task completion time and the precision of stitching were measured and compared. Electromagnetic tracking provided information on the instruments path length, movement velocity and economy. The NASA task load index was used to assess the mental work load. Subjective ratings were added to assess usability, comfort and image quality of each display. Almost all performance parameters were superior for the 3D glasses-based display as compared to the 2D and the autostereoscopic one, but were often significantly exceeded by the mirror-based 3D display. Subjects performed the task at average 20% faster and with a higher precision. Work-load parameters did not show significant differences. Experienced and non-experienced laparoscopists profited equally from 3D. The 3D mirror system gave clear evidence for additional potential of 3D visualization systems with higher resolution and motion parallax presentation.
Gerbino, Giovanni; Zavattero, Emanuele; Viterbo, Stefano; Ramieri, Guglielmo
Surgical management of medial wall orbital fractures should be considered to avoid diplopia and posttraumatic enophthalmos. Treatment of these fractures remains a challenge for the maxillofacial surgeon because of complex anatomy and limited vision. This article aims to retrospectively evaluate the outcomes in the repair of medial orbital wall fractures using a retrocaruncular approach and titanium meshes, comparing the placement of the titanium mesh with three different techniques: (1) conventional free hand under direct vision, (2) with the assistance of an endoscope, and (c) with the assistance of a navigation system. Eighteen patients who underwent surgery for orbital medial wall fracture were enrolled in the study. On the basis of the implant placement technique, three groups were identified: group 1 (CONV), conventional free hand under direct vision; group 2 (ENDO), endoscopically assisted; group 3 (NAVI), a navigational system assisted (BrainLab, Feldkirchen, Germany). The postoperative quality of orbital reconstruction was assessed as satisfactory in 12 cases, good in 4 cases, and unsatisfactory in 2 cases. Particularly in group 1 (CONV) in four patients out of eight, the posterior ledge of the fracture was not reached by the implant and in one patient the mesh hinged toward the ethmoid. In group 3 (NAVI), in one patient out of five, the posterior ledge of the fracture was not reached. In conclusion, titanium orbital mesh plates and retrocaruncular approach are a reliable method to obtain an accurate orbital medial wall reconstruction. The use of endoscopic assistance through the surgical incisions improves accuracy of treatment allowing better visualization of the surgical field. Navigation aided surgery is a feasible technique especially for complex orbital reconstruction to improve predictability and outcomes in orbital repair. PMID:26576238
Matthiesen, Chance; Thompson, J. Spencer; Thompson, David; Farris, Bradley; Wilkes, Byron; Ahmad, Salahuddin; Herman, Terence; Bogardus, Carl
Purpose: To review our institutional outcomes of patients treated with radiation therapy (RT) for Graves' orbitopathy (GO), assess the role of orbital reirradiation, and identify prognostic factors of complete response (CR). Methods and Materials: This is a retrospective review of 211 patients who presented with a diagnosis of GO and received RT between January 2000-2010. RT dose was 20 Gy in 10 fractions. Patient median age was 51 years (range, 15-84 years), median follow-up was 11 months (range, 1-88 months). Patient symptoms included any combination of proptosis (90.9%), extraocular muscle dysfunction (78.9%), soft tissue signs (68.4%), and diplopia (58.4%). Corticosteroids were used as first-line therapy in 20.6% of patients. Among those who achieved either CR or partial response (PR), prognostic factors were evaluated. Results: Stabilization of disease without recurrence was clinically achieved overall in 202 patients (96.7%). At the completion of RT, 176 patients (84.2%) reported a symptomatic improvement of pretreatment symptoms. CR of GO symptoms was achieved using multiple treatment modalities, including RT by 93 patients (44.5%), of which 32 patients received RT only. Corticosteroids were discontinued in 97.8% of patients who received them as initial therapy. Surgical intervention following radiotherapy was required for 144 (68.9%) of all patients. Fourteen patients received orbital reirradiation for persistent or recurrent symptoms. Five of these achieved a CR, and the other nine achieved disease stabilization but retained persistent ocular symptoms. Long-term side effects of RT included dry eyes (12%). Of the prognostic factors we investigated, only gender predicted CR, which was less common in men (33.9%) than in women (49.7%) p = 0.0471. Conclusions: Orbital radiation for GO is an established treatment modality for patients. Orbital reirradiation is beneficial for patients who do not respond to initial RT or experience symptom recurrence without an
Sarikaya-Seiwert, Sevgi; Turowski, Bernd; Hänggi, Daniel; Janssen, Giesela; Steiger, Hans-Jakob; Stummer, Walter
Pineal cysts are benign and often asymptomatic intracranial entities. Occasionally they can lead to neurological symptoms through growth or due to intracystic hemorrhage. The purpose of the current report is to describe their clinical characteristics and treatment options. In the current study, the authors illustrate the course of disease in 3 patients who developed neurological symptoms due to hemorrhage into a pineal cyst. Two of their patients had additional cerebral disease, and regular MR imaging examinations were conducted. This circumstance allowed documentation of growth and intracystic hemorrhage. After the occurrence of new neurological symptoms with severe headache, MR images showed a fluid-fluid interface due to intracystic hemorrhage. The third patient presented with acute triventricular hydrocephalus and papilledema due to aqueductal stenosis caused by intracystic hemorrhage. In all 3 cases, excision of the pineal cysts via an infratentorial/supracerebellar approach was performed. Histological examination revealed the characteristic structure of pineal cyst in all cases, with hemorrhagic residues in the form of hemosiderin deposits. All patients recovered fully after surgical removal of the cysts. Furthermore, resolution of occlusive hydrocephalus could be demonstrated in those cases with ventricular enlargement. Pineal cysts without neurological symptoms are often discovered as incidental findings on cranial MR images. In contrast, neurological symptoms such as severe headache, diplopia, or Parinaud syndrome, may occur as a result of pineal apoplexy due to intracystic hemorrhage. The authors' cases confirm that MR imaging can identify intracystic hemorrhage by a characteristic fluid-fluid interface. Their experience suggests that microsurgical resection of cysts may be an effective and curative treatment option.
Kastrup, O; van de Nes, J; Gasser, T; Keyvani, K
The aim of the study was to further determine the pathophysiology, clinical course, MRI-features and response to therapy of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS), which has recently been proposed as a rare chronic inflammatory central nervous system disorder responsive to immunosuppressive therapy. Three patients with this rare entity underwent serial clinical and bimonthly MRI follow-up over a period of up to 16 months. Extensive laboratory work-up and brain biopsy were performed. Intravenous methylprednisolone or oral dexamethasone was administered as treatment, additionally cyclophosphamide in one patient. Clinically, diplopia, nystagmus, ataxia and facial paresthesia were the cardinal symptoms. Magnetic resonance imaging (MRI) disclosed patchy spot-like gadolinium enhancement in a "salt-and-pepper like appearance" in the pons, midbrain and cerebellum, in two cases with thalamic and in the other with spinal involvement. Brain biopsies demonstrated a predominantly angiocentric but also diffuse infiltration pattern by small mature lymphocytes. Treatment with steroids led to rapid clinical improvement and marked resolution of MRI lesions. As discontinuation of steroids led to clinical relapse, one patient was treated with a further course of steroids and the other with steroids and cyclophosphamide as immunosuppressive therapy. This led to stable remission with only mild clinical residue and normalization of MRI. Extensive laboratory and radiological work-up could not identify any other cause of the disease. Of note, in two cases a marked elevation of IgE in serum was found initially and throughout the course. CLIPPERS seems to be a distinct inflammatory central nervous system disorder. It shows characteristic MRI core features. Extrapontine involvement seems to be frequent. Histologically it is characterised by predominantly angiocentric infiltration by small mature lymphocytes. A pathogenetic
Pittock, Sean J; Debruyne, Jan; Krecke, Karl N; Giannini, Caterina; van den Ameele, Jelle; De Herdt, Veerle; McKeon, Andrew; Fealey, Robert D; Weinshenker, Brian G; Aksamit, Allen J; Krueger, Bruce R; Shuster, Elizabeth A; Keegan, B Mark
The classification and pathological mechanisms of many central nervous system inflammatory diseases remain uncertain. In this article we report eight patients with a clinically and radiologically distinct pontine-predominant encephalomyelitis we have named 'chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids' (CLIPPERS). The patients were assessed clinically, radiologically and pathologically at Mayo Clinic, USA and Ghent University Hospital, Belgium from 1999 to 2009. Median follow-up duration from clinical onset was 22 months (range 7-144 months). Patients underwent extensive laboratory (serum and cerebrospinal fluid), radiological and pathological testing (conjunctival, transbronchial and brain biopsies) to search for causes of an inflammatory central nervous system disorder. All eight patients (five female, three male) presented with episodic diplopia or facial paresthesias with subsequent brainstem and occasionally myelopathic symptoms and had a favourable initial response to high dose glucocorticosteroids. All patients had symmetric curvilinear gadolinium enhancement peppering the pons and extending variably into the medulla, brachium pontis, cerebellum, midbrain and occasionally spinal cord. Radiological improvement accompanied clinical response to glucocorticosteroids. Patients routinely worsened following glucocorticosteroid taper and required chronic glucocorticosteroid or other immunosuppressive therapy. Neuropathology of biopsy material from four patients demonstrated white matter perivascular, predominantly T lymphocytic, infiltrate without granulomas, infection, lymphoma or vasculitis. Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids is a definable, chronic inflammatory central nervous system disorder amenable to immunosuppressive treatment. The T cell predominant inflammatory pathology in affected central nervous system lesions and the clinical and radiological
Rico, María; Villafani, Javier; Tuñón, Alberto; Mateos, Valentín; Oliva-Nacarino, Pedro
Patient: Male, 31 Final Diagnosis: CLIPPERS Symptoms: Ataxia • diplopia Medication: IFNbeta 1a Clinical Procedure: — Specialty: Neurology Objective: Rare disease Background: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a recently described inflammatory disease of the central nervous system, distinguished by brainstem- and spinal cord-centered lesions with a characteristic contrast enhancement on MRI, a lymphocytic perivascular infiltrate on pathological exam, and a dramatic response to and dependence on steroids therapy. Since its initial description in 2010, different glucocorticoid-sparing agents, mostly immunosuppressant drugs, have been used to minimize the dosage, but these therapies also carry the risk of important secondary effects. We present the first reported case of CLIPPERS treated with interferon beta 1a as add-on therapy. Case Report: A previously healthy 31-year-old man presented with gait ataxia and dysarthria. MRI showed pons-centered hyperintense patchy lesions on T2-weighted images. Additional tests ruled out other possible diagnoses and symptoms reversed with intravenous methylprednisolone. Over the years the patient presented with several episodes of deterioration each year, which were partly reversed with glucocorticoid therapy, but leaving him with growing sequelae. Four years after the initial event, treatment with interferon-beta-1a was initiated, achieving reduced frequency of the relapses to 1 every 4 years, which were no longer associated to increasing disability. This allowed reducing glucocorticoids to 30 mg of Deflazacort every other day. Conclusions: Interferon beta-1a could be an alternative to corticosteroid-combined therapy in CLIPPERS and its more benign profile of secondary effects compared to immunosuppressants could make it an attractive choice. PMID:26813773
Murray, Anthony David Neil
Strabismus caused by ocular or orbital trauma can be the result of localized acute soft tissue swelling or may follow orbital fractures, partial or complete loss of extraocular muscle (EOM) and/or cranial nerve function, or damage to surrounding tissues causing mechanical restriction. The strabismus is frequently incomitant and can be difficult, if not impossible to completely correct. The resulting diplopia can affect the individual's ability to function at work, in sports and in common tasks of daily living like driving. The preoperative evaluation should include an assessment of the degree of limitation, muscle function and the condition of the surrounding tissue. In most cases, high resolution computed tomography and/or surface coil dynamic magnetic resonance imaging are required to determine the extent and nature of suspected bony or EOM injury, as well as muscle contractility. If the scan reveals an intact but paretic muscle or only minor muscle injury, surgical intervention is based on the degree of muscle recovery 6 months after the initial insult. If a rectus muscle has been lacerated, and the proximal stump is functional, retrieval should be attempted, either by a direct conjunctival approach if located anteriorly, or by an anterior medial orbitotomy if located deep in the orbit. If a damaged muscle cannot be found, recovered or repaired at any time, then muscle transposition should be considered. If multiple muscles are damaged or scar tissue is excessive, a tether procedure may be indicated. This paper will present an approach to some aspects of strabismus in this setting. PMID:26180469
Kim, Soo Jung; Ko, A Ra; Jung, Mo Kyung; Kim, Ki Eun; Chae, Hyun Wook; Kim, Duk Hee; Kim, Ho-Seong
In males, precocious puberty (PP) is defined as the development of secondary sexual characteristics before age 9 years. PP is usually idiopathic; though, organic abnormalities including tumors are more frequently found in male patients with PP. However, advanced puberty in male also can be an important clinical manifestation in tumors. We report 2 cases of rapidly progressive puberty in males, each associated with a germ-cell tumor. First, an 11-year-old boy presented with mild fever and weight loss for 1 month. Physical examination revealed a pubertal stage of G3P3 with 10-mL testes. Investigations revealed advanced bone age (16 years) with elevated basal luteinizing hormone and testosterone levels. An anterior mediastinal tumor was identified by chest radiography and computed tomography, and elevated α-fetoprotein (AFP) and β-human chorionic gonadotropin (β-hCG) levels were noted. Histopathologic analysis confirmed a yolk-sac tumor. Second, a 12-year-old boy presented with diplopia, polydipsia, and polyuria for 4 months. Physical examination revealed a pubertal stage of G3P3 with 8-mL testes. Bone age was advanced (16 years) and laboratory tests indicated panhypopituitarism with elevated testosterone level. A mixed germ-cell tumor was diagnosed with elevated AFP and β-hCG levels. Of course, these patients also have other symptoms of suspecting tumors, however, rapidly progressive puberty can be the more earlier screening sign of tumors. Therefore, in male patients with accelerated or advanced puberty, malignancy should be considered, with evaluation of tumor markers. In addition, advanced puberty in male should be recognized more widely as a unique sign of neoplasm. PMID:27104181
Kaido, Misako; Yuasa, Yoshihito; Yamamoto, Tameyoshi; Munakata, Satoru; Tagawa, Naohiro; Tanaka, Keiko
We report the case of a patient who had developed multiple cranial nerve palsies in the course of possible paraneoplastic neurological syndrome (PNS) associated with gallbladder cancer. Twelve days prior to visiting our hospital, a 69-year-old man began experiencing neurological symptoms, beginning with diplopia and progressing to ptosis of the left palpebra and subsequent complete closure of the eye within 8 days. Results of the initial medical examination indicated paresis of left oculomotor (III) and abducens (VI) nerves. MRI of the brain revealed no focal lesion that could have resulted in compression of the affected nerves, while further examination ruled out diabetes mellitus, infection, vasculitis, and other systemic autoimmune diseases as potential causes. Gadolinium-enhanced MRI revealed high intensity located in the oculomotor nerves, and steroid pulse therapy was performed based on the assumption of inflammatory diseases. Although slight improvement was observed with respect to the left extraocular paresis, subsequent emergence of bilateral facial nerve (VII) palsy, right abducens nerve palsy, and right oculomotor nerve palsy occurred in succession. PET/CT performed under suspicion of PNS, confirmed the presence of gallbladder cancer. Surgical extirpation of the cancer occurred 3 months following the appearance of left oculomotor paralysis, after which the patient underwent postoperative chemotherapy. All cranial nerve palsies resolved within 2 months after the operation, and both cancer and PNS have shown no recurrence for over 5 years. Pathological examination of the resected tumor revealed well-differentiated tubular adenocarcinoma showing some signs of epithelial-mesenchymal transition, typically an indicator of a poor prognosis. Nevertheless, lymph node metastasis did not progress beyond N2, and the cancer was completely removed by lymph node dissection. Therefore, the presence of multiple cranial palsies in this patient led to early detection of
McBain, H B; MacKenzie, K A; Hancox, J; Ezra, D G; Adams, G G W; Newman, S P
AimsTo establish the impact of adult strabismus surgery on clinical and psychosocial well-being and determine who experiences the greatest benefit from surgery and how one could intervene to improve quality of life post-surgery.MethodsA longitudinal study, with measurements taken pre-surgery and at 3 and 6 months post-surgery. All participants completed the AS-20 a disease specific quality of life scale, along with measures of mood, strabismus and appearance-related beliefs and cognitions and perceived social support. Participants also underwent a full orthoptic assessment at their preoperative visit and again 3 months postoperatively. Clinical outcomes of surgery were classified as success, partial success or failure, using the largest angle of deviation, diplopia and requirement for further therapy.Results210 participants took part in the study. Strabismus surgery led to statistically significant improvements in psychosocial and functional quality of life. Those whose surgery was deemed a partial success did however experience a deterioration in quality of life. A combination of clinical variables, high expectations, and negative beliefs about the illness and appearance pre-surgery were significant predictors of change in quality of life from pre- to post-surgery.ConclusionsStrabismus surgery leads to significant improvements in quality of life up to 6 months postoperatively. There are however a group of patients who do not experience these benefits. A series of clinical and psychosocial factors have now been identified, which will enable clinicians to identify patients who may be vulnerable to poorer outcomes post-surgery and allow for the development of interventions to improve quality of life after surgery.
Rocha, Paulo; Qanadli, Salah D.; Strumza, Pierre; Kacher, Safia; Aberkane, Linda; Aubry, Pierre; Rigaud, Michel; Lacombe, Pascal; Raffestin, Bernadette
Purpose: The common finding of thrombi between the bifoil balloons when they were extracted after mitral dilation prompted us to look for evidence of minor brain embolisms using the sensitive technique of BMRI (brain magnetic resonance T2-weighted imaging). Methods: BMRI was performed within 48 hr before and after a percutaneous mitral balloon commissurotomy (PMBC) in each of the 63 patients in this study. Results: There was evidence (hyperintensity foci: HI) of a previous asymptomatic brain embolism in 38 of 63 patients before PMBC and a new HI appeared in 18 of 63 patients after the procedure. New HI signals were found exclusively in the white matter in 8 of 18 patients and in only 3 of 18 were HI signs larger than 1 cm. One patient, with an HI signal >1 cm in the thalamus and another <1 cm in the brain stem, presented diplopia accompanied by other minor clinical signs. The differences in HI rate among four subgroups (1, older vs younger than 43 years; 2, sinus rhythm vs atrial fibrillation; 3, echo score <8 vs >8; 4, patients from western countries vs the others) were not statistically significant, probably because the number of patients in each subgroup was low. Patients in atrial fibrillation had slightly more (not significant) HI before PMBC (15/20, 75%) than patients in sinus rhythm (23/43, 53%), but after PMBC their HI frequencies were similar (atrial fibrillation: 5/20, 25%; sinus rhythm: 13/43, 30%). Conclusion: Brain microembolism is frequent during PMBC, but is often anatomically limited and free from clinical signs in most cases. Brain embolism seems to be related mainly to the procedure itself and not the features of the patient.
Nakamura, Yuko; Sawada, Mikio; Ikeguchi, Kunihiko; Nakano, Imaharu
Botulism is a neuroparalytic disease caused by neurotoxins produced by Clostridium botulinum. Food-borne botulism is a kind of exotoxin-caused food intoxication. Although this disease is rarely reported in Japan now, it is a cause of great concern because of its high mortality rate, and botulism cases should be treated as a public health emergency. Botulism classically presents as acute symmetrical descending flaccid paralysis. Its diagnosis is based on the detection of botulinum toxins in the patient's serum or stool specimens. Electrophysiologic tests of such patients show reduced compound muscle action potentials (CMAPs), low amplitudes and short durations of motor unit potentials (MUPs), and mild waning in repetitive low-frequency stimulations. Single fiber electromyography (EMG) is particularly useful for the diagnosis of botulism. We report a case of food-borne botulism that we had encountered. An 83-year-old man with rapidly progressive diplopia, dysphagia, and tetraplegia was hospitalized; he required intensive care, including artificial ventilatory support. Electrophysiologic tests yielded findings compatible with botulism. We made a clinical diagnosis of food-borne botulism and administered antitoxin on the seventh disease day. The patient's motor symptoms started ameliorating several days after the antitoxin injection. Subsequently, botulinum toxin type A was detected in the patient's serum specimen by using a bioassay, and the type A gene and silent B gene were detected in his serum specimen by using polymerase chain reaction (PCR). C. botulinum was also obtained from stool culture on the 17th and 50th disease days. Botulism is a curable disease if treated early. Although it is a rare condition, it should always be considered in the differential diagnosis of patients with rapid onset of cranial nerve and limb muscle palsies.
Leske, David A.; Hatt, Sarah R.; Liebermann, Laura; Holmes, Jonathan M.
Purpose We compare two methods of analysis for Rasch scoring pre- to postintervention data: Rasch lookup table versus de novo stacked Rasch analysis using the Adult Strabismus-20 (AS-20). Methods One hundred forty-seven subjects completed the AS-20 questionnaire prior to surgery and 6 weeks postoperatively. Subjects were classified 6 weeks postoperatively as “success,” “partial success,” or “failure” based on angle and diplopia status. Postoperative change in AS-20 scores was compared for all four AS-20 domains (self-perception, interactions, reading function, and general function) overall and by success status using two methods: (1) applying historical Rasch threshold measures from lookup tables and (2) performing a stacked de novo Rasch analysis. Change was assessed by analyzing effect size, improvement exceeding 95% limits of agreement (LOA), and score distributions. Results Effect sizes were similar for all AS-20 domains whether obtained from lookup tables or stacked analysis. Similar proportions exceeded 95% LOAs using lookup tables versus stacked analysis. Improvement in median score was observed for all AS-20 domains using lookup tables and stacked analysis (P < 0.0001 for all comparisons). Conclusions The Rasch-scored AS-20 is a responsive and valid instrument designed to measure strabismus-specific health-related quality of life. When analyzing pre- to postoperative change in AS-20 scores, Rasch lookup tables and de novo stacked Rasch analysis yield essentially the same results. Translational Relevance We describe a practical application of lookup tables, allowing the clinician or researcher to score the Rasch-calibrated AS-20 questionnaire without specialized software. PMID:26933524
Eum, Sun Jung
diplopia compared with LR recession alone. PMID:27980365
Dedushi, Kreshnike; Kabashi, Serbeze; Ugurel, Mehmet Sahin; Ramadani, Naser; Mucaj, Sefedin; Zeqiraj, Kamber
Background: The purpose of this study is to investigate the MRI features of central neurocytoma. Case report: A 45 year old man with 3 months of worsening daily headaches. These headaches were diffuse, lasted for several hours, and mostly occurred in the morning. She was initially diagnosed and treated for migraines but later he had epileptic attack and diplopia and neurolog recomaded MRI. Methods: precontrast MRI; TSE/T2Wsequence in axial/coronal planes; 3D–Hi-resolution T1W sagittal; FLAIR/T2W axial; FLAIR/T2W and Flash/T2W oblique coronal plane (perpendicular to temporal lobes) GRE/T2W axial plane for detection of heme products. Post-contrast TSE/T1W sequence in axial, coronal and sagittal planes. Diffusion weighted and ADC mapping MRI images for EPI sequence in axial plane. Results: A 23x12mm heterogeneous mass within aqueductus cerebri, with calcified and hemorrhagic foci and extending downwards till fourth ventricle. It’s originating from the right paramedian posterior aqueductal wall (tectum), and also extending to and involving the tegmentum of mesencephalon at its right paramedian aspect. CSF flow obstruction secondary to described aqueductal mass, with resultant triventricular hydrocephalus). Marked transependymal CSF leak can be noted at periventricular white matter, secondary to severe hydrocephalus. After IV injection of contrast media, this mass shows mild-to-moderate heterogenous speckled enhancement. Conclusion: MRI is helpful in defining tumor extension, which is important in preoperative planning. Although IN is a relatively rare lesion, it should be considered in the differential diagnosis of intraventricular lesions in the presence of such typical MR findings. However, a definitive diagnosis requires immunochemical study and electron microscopy. PMID:28077908
Chung, S A; Kim, W K; Moon, J W; Yang, H; Kim, J K; Lee, S B; Lee, J B
Purpose To evaluate the impact of myopic keratorefractive surgery on ocular alignment. Methods This prospective study included 194 eyes of 97 myopic patients undergoing laser refractive surgery. All patients received a complete ophthalmic examination with particular attention to ocular alignment before and 3 months after surgery. Results Patients with a mean age of 26.6 years and a mean refractive error of −4.83 diopters (D) myopia were treated. Asymptomatic ocular misalignment was present preoperatively in 46 (47%) patients: a small-angle heterophoria (1–8 prism diopters, PD) in 36% and a large-angle heterophoria (>8 PD)/heterotropia in 11%. Postoperatively, the change in angles of 10 PD or greater occurred in 3% for distance and 6% for near fixation: in 7% of the patients with orthophoria, in 3% of those with a small-angle heterophoria, and in 18% of those with a large-angle heterophoria/heterotropia. No patient developed diplopia. The preoperative magnitude of myopia or postoperative refractive status was not related to the change in ocular alignment. The higher anisometropia was associated with a decrease in deviation (P=0.041 for distance and P=0.002 for near fixation), whereas the further near point of convergence tended to be related with an increase in near deviation (P=0.055). Conclusions Myopic refractive surgery may cause a change in ocular alignment, especially in cases with a large-angle heterophoria/heterotropia. There is also a chance of improvement of misalignment in patients with anisometropia. PMID:25190533
Bharadwaj, Shrikant R.; Hoenig, Pia M.; Sivaramakrishnan, C. V.; Karthikeyan, B.; Simonian, Donna; Mau, Katie; Rastani, Sally; Schor, Clifton M.
Purpose The maximum binocular vertical disparity that can be fused with disparity vergence (vertical-fusion amplitude or VFA), varies with convergence angle. VFA is larger for convergence responses to near than to far viewing distances; however the clinical norms for changes in VFA with convergence have not been established. VFA at several convergence angles was measured to obtain a quantitative description of the changes in VFA with convergence. Methods 56-adults took part in the study. Horizontal and vertical disparity stimuli were presented on a computer monitor using the red-green anaglyphic technique. Stimulus to convergence was altered either by changing horizontal disparity on the computer monitor (experiment I: 9 horizontal disparities: 1.2 –22.5 Δ) or by changing the binocular viewing distance (experiment II: 5 viewing distances: 25 – 300 cms). Convergence was held constant during an experimental session while vertical disparity was incremented in steps of 0.05 Δ after a subjective report of fusion until the subject reported diplopia. The maximum vertical disparity that could be fused was defined as the VFA. Results VFA increased linearly over the range of convergence stimuli (y = 0.10x + 1.62) and inter-subject variability of VFA increased marginally with the amount of convergence. Linear regression equations with similar slopes and y-intercepts were observed in experiments I and II. Conclusions The results of our experiments provide a quantitative description of a linear relationship between VFA and convergence. The linear regression equation could be employed in a clinical setting to establish norms and screen for vertical vergence abnormalities. PMID:17389488
Hoy, Sheridan M
Lacosamide (Vimpat(®)) is a functionalized amino acid available orally (as a syrup or tablet) and as an intravenous infusion. It is believed to exert its antiepileptic effect by selectively enhancing the slow inactivation of voltage-gated sodium channels. Lacosamide is approved in several countries worldwide as an adjunctive therapy for the treatment of partial-onset seizures; however, prescribing regulations differ between countries. This article reviews the use of lacosamide as indicated in adults and adolescents (aged 16-18 years) in the EU, where it is approved in this patient population as an adjunctive therapy to other AEDs in the treatment of partial-onset seizures, with or without secondary generalization. In three randomized, double-blind, placebo-controlled, multicentre studies in adults and adolescents (aged 16-18 years) with partial-onset seizures, adjunctive therapy with oral lacosamide (administered for an initial titration period followed by 12 weeks' maintenance therapy) generally reduced the frequency of seizures to a significantly greater extent than placebo, with antiepileptic efficacy sustained following longer-term treatment (up to 8 years) in this patient population. Oral and intravenous lacosamide were generally well tolerated in clinical studies, with the majority of adverse events being mild or moderate in severity. Very common adverse reactions following adjunctive therapy with oral lacosamide included diplopia, dizziness, headache and nausea; the tolerability profile of intravenous lacosamide appeared consistent with that of oral lacosamide, although intravenous administration was associated with local adverse events, such as injection site discomfort or pain, irritation and erythema. Thus, oral and intravenous lacosamide as an adjunctive therapy to other AEDs provides a useful option in the treatment of patients with partial-onset seizures.
Tambucci, Renato; Basti, Claudia; Maresca, Maria; Coppola, Giangennaro; Verrotti, Alberto
Eslicarbazepine acetate (ESL) is a once daily new third generation antiepileptic drug that shares the basic chemical structure of carbamazepine and oxcarbazepine - a dibenzazepine nucleus with the 5-carboxamide substituent, but is structurally different at the 10,11-position. ESL is a pro-drug metabolized to its major active metabolite eslicarbazepine. Despite the fact that the exact mechanism of action has not been fully elucidated, it is thought to involve inhibition of voltage-gated sodium channels (VGSC). ESL inhibits sodium currents in a voltage-dependent way by an interaction predominantly with the inactivated state of the VGSC, thus selectively reducing the activity of rapidly firing (epileptic) neurons. ESL reduces VGSC availability through enhancement of slow inactivation. In Phase III studies, adjunctive therapy with ESL 800 or 1,200 mg/day leads to a significant decrease in the seizure frequency in adults with refractory partial onset epilepsy. Based on these results, ESL has been approved in Europe (by the European Medicines Agency) and in the United States (by the US Food and Drug Administration) as add-on therapy. Data on efficacy and safety have been confirmed by 1-year extension and real life observational studies. Recently, based on results from two randomized, double-blind, historical control Phase III trials, ESL received US Food and Drug Administration approval also as a monotherapy for patients with partial onset epilepsy. In the pediatric setting, encouraging results have been obtained suggesting its potential role in the management of epileptic children. Overall ESL was generally well tolerated. The most common adverse events were dizziness, somnolence, headache, nausea, diplopia, and vomiting. Adverse events can be minimized by appropriate titration. In conclusion, ESL seems to overcome some drawbacks of the previous antiepileptic drugs, suggesting a major role of ESL in the management of focal onset epilepsy for both new onset and
Göçmen, Emine Seyhan; Atalay, Yonca; Evren Kemer, Özlem; Sarıkatipoğlu, Hikmet Yavuz
A 46-year-old male patient was referred to our clinic with complaints of diplopia and esotropia in his right eye that developed after a car accident. The patient had right esotropia in primary position and abduction of the right eye was totally limited. Primary deviation was over 40 prism diopters at near and distance. The patient was diagnosed with sixth nerve palsy and 18 months after trauma, he underwent right medial rectus muscle recession. Ten months after the first operation, full-thickness tendon transposition of the superior and inferior rectus muscles (with Foster suture) was performed. On the first postoperative day, slit-lamp examination revealed corneal edema, 3+ cells in the anterior chamber and an irregular pupil. According to these findings, the diagnosis was anterior segment ischemia. Treatment with 0.1/5 mL topical dexamethasone drops (16 times/day), cyclopentolate hydrochloride drops (3 times/day) and 20 mg oral fluocortolone (3 times/day) was initiated. After 1 week of treatment, corneal edema regressed and the anterior chamber was clean. Topical and systemic steroid treatment was gradually discontinued. At postoperative 1 month, the patient was orthophoric and there were no pathologic symptoms besides the irregular pupil. Anterior segment ischemia is one of the most serious complications of strabismus surgery. Despite the fact that in most cases the only remaining sequel is an irregular pupil, serious circulation deficits could lead to phthisis bulbi. Clinical properties of anterior segment ischemia should be well recognized and in especially risky cases, preventative measures should be taken. PMID:28182149
Deguchi, K; Takeuchi, H; Touge, T; Tsukaguchi, M; Sasaki, I; Ishibashi, T; Nishioka, M
We report a patient with pontine infarction who showed non-paralytic pontine exotropia (NPPE) with alternating exotropia. An 81-year old woman developed diplopia and gait disturbance. On the fourth day, she was admitted to our hospital. Oculomotor findings on admission showed that the ocular position of the left eye on forward gaze was fixed at the midline, while the right eye was abducted. When she was instructed to use the right eye for forward gaze, the left eye deviated outward. On right gaze, the left eye was not abducted and monocular nystagmus was noted in the abducted right eye. Upper gaze was limited in the right eye and convergence was not possible. The remaining ocular movements were intact. On cranial CT performed on eye closure, the left eye was abducted and the right eye was fixed at the midline. Brain MRI showed cerebral infarction located in the left paramedian portion of the middle pontine tegmentum with involvement of the medial longitudinum fasciculus (MLF) and in the ventral site of the upper pons. On the 16th day, right exotropia disappeared on eye opening without forward gaze, but forward gaze easily induced outward deviation of the right eye. On the 39th day, right exotropia on forward gaze had almost disappeared. However, left MLF syndrome and abnormal convergence were persistent when she discharged on the 76th day. In conjunction with oculomotor and neuroradiological findings, the NPPE with alternating exotropia in our patient may be due to severe MLF damage and secondary hyperactivity of the contralateral paramedian pontine reticular formation.
Budenz, Donald L.; Feuer, William J.; Barton, Keith; Schiffman, Joyce; Costa, Vital P.; Godfrey, David G.; Buys, Yvonne M.
PURPOSE To compare the late complications in the Ahmed Baerveldt Comparison Study during 5 years of follow-up. DESIGN Multicenter, prospective, randomized clinical trial. METHODS SETTINGS Sixteen international clinical centers. STUDY POPULATION Two hundred seventy six subjects aged 18 to 85 years with previous intraocular surgery or refractory glaucoma with intraocular pressure of > 18 mmHg. INTERVENTIONS Ahmed Glaucoma Valve FP7 or Baerveldt Glaucoma Implant BG 101-350. MAIN OUTCOME MEASURES Late postoperative complications (beyond 3 months), reoperations for complications, and decreased vision from complications. RESULTS Late complications developed in 56 subjects (46.8 ± 4.8 5 year cumulative % ± SE) in the Ahmed Glaucoma Valve group and 67 (56.3 ± 4.7 5 year cumulative % ± SE) in the Baerveldt Glaucoma Implant group (P = 0.082). The cumulative rates of serious complications were 15.9% and 24.7% in the Ahmed Glaucoma Valve and Baerveldt Glaucoma Implant groups respectively (P = 0.034) although this was largely driven by subjects who had tube occlusions in the two groups (0.8% in the Ahmed Glaucoma Valve group and 5.7% in the Baerveldt Glaucoma Implant group, P = 0.037). Both groups had a relatively high incidence of persistent diplopia (12%) and corneal edema (20%), although half of the corneal edema cases were likely due to pre-existing causes other than the aqueous shunt. The incidence of tube erosion was 1% and 3% in the Ahmed Glaucoma Valve and Baerveldt Glaucoma Implant groups, respectively (P = 0.04). CONCLUSIONS Long term rates of vision threatening complications and complications resulting in reoperation were higher in the Baerveldt Glaucoma Implant than the Ahmed Glaucoma Valve group over 5 years of follow-up. PMID:26596400
Qureshi, Adnan I.; Khan, Asif A.; Capistrant, Rachel; Qureshi, Mushtaq H.; Xie, Kevin; Suri, M. Fareed K.
OBJECTIVE To report upon technique of concurrent placement of angioplasty balloon at the internal jugular vein and sigmoid venous sinus junction to facilitate stent delivery in two patients in whom stent delivery past the jugular bulb was not possible. CLINICAL PRESENTATION A 21-year-old woman and a 41-year-old woman with worsening headaches, visual obscuration or diplopia were treated for pseudotumor cerebri associated with transverse venous stenosis. Both patients had undergone primary angioplasty, which resulted in improvement in clinical symptoms followed by the recurrence of symptoms with restenosis at the site of angioplasty. INTERVENTION After multiple attempts at stent delivery through jugular venous bulb were unsuccessful, a second guide catheter was placed in the ipsilateral internal jugular vein through contralateral femoral venous approach. A 6 mm × 20 mm (left) or 5 × 15 mm (right) angioplasty balloon was placed across the internal jugular vein and sigmoid sinus junction and partially inflated until the inflation and relative straightening of the junction was observed. In both patients, the internal jugular vein and sigmoid sinus junction was successfully traversed by the stent delivery system in a parallel alignment to inflated balloon. Balloon mounted stent was deployed at the site of restenosis with near complete resolution of lumen narrowing delivery and improvement in clinical symptoms. CONCLUSION We report a technique for realignment and diameter change with concurrent placement and partial inflation of angioplasty balloon at the jugular venous bulb to facilitate stent delivery into the sigmoid and transverse venous sinuses in circumstances where multiple attempts at stent delivery are unsuccessful. PMID:27829971
Johnston, Christopher I.; O'Leary, Margaret A.; Brown, Simon G. A.; Currie, Bart J.; Halkidis, Lambros; Whitaker, Richard; Close, Benjamin; Isbister, Geoffrey K.
Background Death adders (Acanthophis spp) are found in Australia, Papua New Guinea and parts of eastern Indonesia. This study aimed to investigate the clinical syndrome of death adder envenoming and response to antivenom treatment. Methodology/Principal Findings Definite death adder bites were recruited from the Australian Snakebite Project (ASP) as defined by expert identification or detection of death adder venom in blood. Clinical effects and laboratory results were collected prospectively, including the time course of neurotoxicity and response to treatment. Enzyme immunoassay was used to measure venom concentrations. Twenty nine patients had definite death adder bites; median age 45 yr (5–74 yr); 25 were male. Envenoming occurred in 14 patients. Two further patients had allergic reactions without envenoming, both snake handlers with previous death adder bites. Of 14 envenomed patients, 12 developed neurotoxicity characterised by ptosis (12), diplopia (9), bulbar weakness (7), intercostal muscle weakness (2) and limb weakness (2). Intubation and mechanical ventilation were required for two patients for 17 and 83 hours. The median time to onset of neurotoxicity was 4 hours (0.5–15.5 hr). One patient bitten by a northern death adder developed myotoxicity and one patient only developed systemic symptoms without neurotoxicity. No patient developed venom induced consumption coagulopathy. Antivenom was administered to 13 patients, all receiving one vial initially. The median time for resolution of neurotoxicity post-antivenom was 21 hours (5–168). The median peak venom concentration in 13 envenomed patients with blood samples was 22 ng/mL (4.4–245 ng/mL). In eight patients where post-antivenom bloods were available, no venom was detected after one vial of antivenom. Conclusions/Significance Death adder envenoming is characterised by neurotoxicity, which is mild in most cases. One vial of death adder antivenom was sufficient to bind all circulating venom. The
Wang, Hui; Pan, Yunzhu; Zhang, Zaiqiang; Li, Xingang; Xu, Zhe; Suo, Yue; Li, Wei; Wang, Yongjun
Familial cerebral cavernous malformation (FCCM) is a vascular malformation disorder that closely associated with three identified genes: KRIT1/CCM1, MGC4607/CCM2, and PDCD10/CCM3. Here, we present a Chinese family affected by FCCM due to a novel KRIT1/CCM1 insertion mutation. The proband was hospitalized for sudden unconsciousness and underwent surgical treatment. The section of lesions showed classical cavernous-dilated vessels without intervening brain parenchyma, and hemosiderin-laden macrophages were accumulated in the surrounding tissue. In addition, magnetic resonance imaging (MRI) showed severe multiple cerebral cavernous malformation (CCM) lesions in cerebrum, brainstem, and cerebellum in other affected subjects. Especially, for the proband's mother, hundreds of lesions were presented, and a few lesions were found in the expanded lateral ventricle (Evans' index =0.33). Moreover, she showed the similar symptoms of hydrocephalus, including headache, dizziness, and diplopia. It was extremely rare in previous reports. To date, the genetic alterations leading to FCCM in Chinese population remain largely unknown. We investigated genetic defects of this family. Sequence analyses disclosed a novel heterozygous insertion mutation (c.1896_1897insT; p.Pro633SerfsTer22) in KRIT1/CCM1. Moreover, our real-time PCR results revealed that the mRNA level of KRIT1/CCM1 were significantly decreased in FCCM subjects (CCM family =0.42 ± 0.20 vs. healthy control =1.01 ± 0.16, P = 0.004). It indicated that this mutation could cause KRIT1/CCM1 functional mRNA deficiency. It may be closely related with the pathogenesis of FCCM. Our findings provided a new gene mutation profile which will be of great significance in early diagnosis and appropriate clinical surveillance of FCCM patients.
Ganguly, Anasua; Ramarao, Kesarpu; Mohapatra, Samir; Rath, Suryasnata
Purpose: To report the anatomical and cosmetic outcome of transconjunctival dacryocystorhinostomy (TDCR) in an Asian Indian population. Methods: TDCR was initially performed in cadaver eyes followed by patients with primary acquired nasolacrimal duct obstruction (NLDO). This was a prospective noncomparative case series of all consecutive TDCRs performed between April 2013 and June 2015. Outcome measures were anatomical patency, epiphora, presence of diplopia, aesthetic outcome, and health status. Results: A total of 17 (18 eyes) patients with a mean age 43.9 ± 11.8 years (range, 32–75) were included in the study. Eight were males, and one patient underwent TDCR in both eyes. TDCR was successfully performed in 15/18 (82%) eyes under local anesthesia. Procedure converted to transcutaneous external DCR in two and dacryocystectomy in one patient each. Mean duration of surgery was 52.6 (range, 29–110) min. Anatomical patency and relief from epiphora was achieved in all (15/15) eyes after TDCR at a median follow-up of 15.5 months. At final follow-up, objective assessment of the cosmetic outcome graded the surgical scar at the lateral canthus as invisible in all except one and conjunctival fornix as visible only after eyelid eversion in all patients. Disturbance of the medial fat pad was not seen in any patient. A questionnaire-based health status evaluation showed marked improvement in anxiety/depression before and after TDCR with an overall well-being score of 88 on a scale of 0–100 (worst–best) after TDCR. Conclusions: TDCR offers a promising aesthetic approach in patients with primary acquired NLDO and gives excellent functional and cosmetic outcome. PMID:28112129
Harris, G J; Garcia, G H; Logani, S C; Murphy, M L; Sheth, B P; Seth, A K
BACKGROUND/PURPOSE: Although the management of orbital blow-out fractures was controversial for many years, refined imaging with computed tomography (CT) helped to narrow the poles of the debate. Many orbital surgeons currently recommend repair if fracture size portends late enophthalmos, or if diplopia has not substantially resolved within 2 weeks of the injury. While volumetric considerations have been generally well-served by this approach, ocular motility outcomes have been less than ideal. In one series, almost 50% of patients had residual diplopia 6 months after surgery. A fine network of fibrous septa that functionally unites the periosteum of the orbital floor, the inferior fibrofatty tissues, and the sheaths of the inferior rectus and oblique muscles was demonstrated by Koornneef. Entrapment between bone fragments of any of the components of this anatomic unit can limit ocular motility. Based on the pathogenesis of blow-out fractures, in which the fibrofatty-muscular complex is driven to varying degrees between bone fragments, some measure of soft tissue damage might be anticipated. Subsequent intrinsic fibrosis and contraction can tether globe movement, despite complete reduction of herniated orbital tissue from the fracture site. We postulated that the extent of this soft tissue damage might be estimated from preoperative imaging studies. METHODS: Study criteria included: retrievable coronal CT scans; fractures of the orbital floor without rim involvement, with or without extension into the medial wall; preoperative diplopia; surgical repair by a single surgeon; complete release of entrapped tissues; and postoperative ocular motility outcomes documented with binocular visual fields (BVFs). Thirty patients met all criteria. The CT scans and BVFs were assessed by different examiners among the authors. Fractures were classified into 3 general categories and 2 subtypes to reflect the severity of soft tissue damage within each category. "Trap-door" injuries
[Transethmoidal decompressive orbitotomy in the patients presenting with endocrine ophthalmopathy at the stage of remission: peculiarities of the surgical intervention and the results of exophthalmos correction].
Kochetkov, P A
The objective of the present study was to analyze the original results of the transethmoidal decompressive surgical interventions on the eye orbits of the patients presenting with endocrine ophthalmopathy (EOP) including the evaluation of regress of exophthalmos during the post-operative period. The study included 51 patients with endocrine ophthalmopathy at the stage of remission or pharmacotherapeutic compensation of the underlying process. A total of 80 eye-socket were treated in 29 patients with unilateral exophthalmos and 22 ones having bilateral exophthalmos. All the patients underwent transethmoidal decompression of the orbit (TEDO) with the use of the endonasal approach. The examination before and after surgery included the standard ophthalmological evaluation (such as the external inspection, visiometry, tonometry, computed perimetry, biomicroscopy, and ophthalmoscopy) as well as additional studies including exophthalmometry and orbitometry. The activity of endocrine ophthalmopathy was estimated with use of the clinical activity score scale. The main indication for surgery was cosmetically unacceptable exophthalmos. The results of the analysis of the surgical techniques employed in the present study, anatomical features revealed intra-operatively, methods of post-operative care for the patients, and final outcomes of the treatment taken together have demonstrated the high effectiveness of transethmoidal decompressive surgery. Not a single case of visual acuity deterioration was documented during the follow-up period. Persistent convergent strabismus and post-operative diplopia developed in 18% of the patients. Regress of exophthalmos following transethmoidal decompressive orbitotomy varied from 3.5 to 6.5 mm and averaged 5.1 mm in comparison with the mean initial value of 23.9 mm. In means that transethmoidal decompressive orbitotomy ensured the overall regress of exophthalmos by 18.8 mm (p<0.05). The multispiral CT study revealed the significant reduction
Sperling, Michael R; Abou-Khalil, Bassel; Harvey, Jay; Rogin, Joanne B; Biraben, Arnaud; Galimberti, Carlo A; Kowacs, Pedro A; Hong, Seung Bong; Cheng, Hailong; Blum, David; Nunes, Teresa; Soares-da-Silva, Patrício
Objective To evaluate the efficacy and safety of adjunctive eslicarbazepine acetate (ESL) in patients with refractory partial-onset seizures. Methods This randomized, placebo-controlled, double-blind, parallel-group, phase III study was conducted at 173 centers in 19 countries, including the United States and Canada. Eligible patients were aged ≥16 years and had uncontrolled partial-onset seizures despite treatment with 1–2 antiepileptic drugs (AEDs). After an 8-week baseline period, patients were randomized to once-daily placebo (n = 226), ESL 800 mg (n = 216), or ESL 1,200 mg (n = 211). Following a 2-week titration period, patients received ESL 800 or 1,200 mg once-daily for 12 weeks. Seizure data were captured and documented using event-entry or daily entry diaries. Results Standardized seizure frequency (SSF) during the maintenance period (primary end point) was reduced with ESL 1,200 mg (p = 0.004), and there was a trend toward improvement with ESL 800 mg (p = 0.06), compared with placebo. When data for titration and maintenance periods were combined, ESL 800 mg (p = 0.001) and 1,200 mg (p < 0.001) both reduced SSF. There were no statistically significant interactions between treatment response and geographical region (p = 0.38) or diary version (p = 0.76). Responder rate (≥50% reduction in SSF) was significantly higher with ESL 1,200 mg (42.6%, p < 0.001) but not ESL 800 mg (30.5%, p = 0.07) than placebo (23.1%). Incidence of treatment-emergent adverse events (TEAEs) and TEAEs leading to discontinuation increased with ESL dose. The most common TEAEs were dizziness, somnolence, nausea, headache, and diplopia. Significance Adjunctive ESL 1,200 mg once-daily was more efficacious than placebo in adult patients with refractory partial-onset seizures. The once-daily 800 mg dose showed a marginal effect on SSF, but did not reach statistical significance. Both doses were well tolerated. Efficacy assessment was not affected by
Shew, Matthew; Carlisle, Michael P; Lu, Guanning Nina; Humphrey, Clinton; Kriet, J David
Orbital fractures are a common result of facial trauma. Sequelae and indications for repair include enophthalmos and/or diplopia from extraocular muscle entrapment. Alloplastic implant placement with careful release of periorbital fat and extraocular muscles can effectively restore extraocular movements, orbital integrity, and anatomic volume. However, rare but devastating complications such as retrobulbar hematoma (RBH) can occur after repair, which pose a risk of permanent vision loss if not addressed emergently. For this reason, some surgeons take the precaution of admitting patients for 24-hour postoperative vision checks, while others do not. The incidence of postoperative RBH has not been previously reported and existing data are limited to case reports. Our aim was to examine national trends in postoperative management and to report the incidence of immediate postoperative complications at our institution following orbital repair. A retrospective assessment of orbital blowout fractures was undertaken to assess immediate postoperative complications including RBH. Only patients treated by a senior surgeon in the Department of Otolaryngology were included in the review. In addition, we surveyed AO North America (AONA) Craniomaxillofacial faculty to assess current trends in postoperative management. There were 80 patients treated surgically for orbital blowout fractures over a 9.5-year period. Nearly all patients were observed overnight (74%) or longer (25%) due to other trauma. Average length of stay was 17 hours for those observed overnight. There was one (1.3%) patient with RBH, who was treated and recovered without sequelae. Results of the survey indicated that a majority (64%) of responders observe postoperative patients overnight. Twenty-nine percent of responders indicated that they send patients home the same day of surgery. Performance of more than 20 orbital repairs annually significantly increased the likelihood that faculty would manage patients on
Hellenbroich, Y; Gierga, K; Reusche, E; Schwinger, E; Deller, T; de Vos, R A I; Zühlke, C; Rüb, U
Spinocerebellar ataxia type 4 (SCA4), also known as 'hereditary ataxia with sensory neuropathy', represents a very rare, progressive and untreatable form of an autosomal dominant inherited cerebellar ataxia (ADCA). Due to a lack of autopsy cases, no neuropathological or clinicopathological studies had yet been performed in SCA4. In the present study, the first available cerebellar and brainstem tissue of a clinically diagnosed and genetically-confirmed German SCA4 patient was pathoanatomically studied using serial thick sections. During this systematic postmortem investigation, along with an obvious demyelinization of cerebellar and brainstem fiber tracts we observed widespread cerebellar and brainstem neurodegeneration with marked neuronal loss in the substantia nigra and ventral tegmental area, central raphe and pontine nuclei, all auditory brainstem nuclei, in the abducens, principal trigeminal, spinal trigeminal, facial, superior vestibular, medial vestibular, interstitial vestibular, dorsal motor vagal, hypoglossal, and prepositus hypoglossal nuclei, as well as in the nucleus raphe interpositus, all dorsal column nuclei, and in the principal and medial subnuclei of the inferior olive. Severe neuronal loss was seen in the Purkinje cell layer of the cerebellum, in the cerebellar fastigial nucleus, in the red, trochlear, lateral vestibular, and lateral reticular nuclei, the reticulotegmental nucleus of the pons, and the nucleus of Roller. In addition, immunocytochemical analysis using the anti-polyglutamine antibody 1C2 failed to detect any polyglutamine-related immunoreactivity in the central nervous regions of this SCA4 patient studied. In view of the known functional role of affected nuclei and related fiber tracts, the present findings not only offer explanations for the well-known disease symptoms of SCA4 patients (i.e. ataxic symptoms, dysarthria and somatosensory deficits), but for the first time help to explain why diplopia, gaze-evoked nystagmus
Adham, Marlinda; Kurniawan, Antonius N; Muhtadi, Arina Ika; Roezin, Averdi; Hermani, Bambang; Gondhowiardjo, Soehartati; Tan, I Bing; Middeldorp, Jaap M
Among all head and neck (H&N) cancers, nasopharyngeal carcinoma (NPC) represents a distinct entity regarding epidemiology, clinical presentation, biological markers, carcinogenic risk factors, and prognostic factors. NPC is endemic in certain regions of the world, especially in Southeast Asia, and has a poor prognosis. In Indonesia, the recorded mean prevalence is 6.2/100 000, with 13 000 yearly new NPC cases, but otherwise little is documented on NPC in Indonesia. Here, we report on a group of 1121 NPC patients diagnosed and treated at Dr. Cipto Mangunkusumo Hospital, Jakarta, Indonesia between 1996 and 2005. We studied NPC incidence among all H&N cancer cases (n=6000) observed in that period, focusing on age and gender distribution, the ethnic background of patients, and the disease etiology. We also analyzed most prevalent signs and symptoms and staging of NPC patients at first presentation. In this study population, NPC was the most frequent H&N cancer (28.4%), with a male-to-female ratio of 2.4, and was endemic in the Javanese population. Interestingly, NPC appeared to affect patients at a relatively young age (20% juvenile cases) without a bimodal age distribution. Mostly, NPC initiated in the fossa of Rosenmuller and spreaded intracranially or locally as a mass in the head. Occasionally, NPC developed at the submucosal level spreading outside the anatomic limits of the nasopharynx. At presentation, NPC associated with hearing problems, serous otitis media, tinnitus, nasal obstruction, anosmia, bleeding, difficulty in swallowing and dysphonia, and even eye symptoms with diplopia and pain. The initial diagnosis is difficult to make because early signs and symptoms of NPC are not specific to the disease. Early-age Epstein-Barr virus (EBV) infection combined with frequent exposure to environmental carcinogenic co-factors is suggested to cause NPC development. Undifferentiated NPC is the most frequent histological type and is closely associated with EBV
Adham, Marlinda; Kurniawan, Antonius N.; Muhtadi, Arina Ika; Roezin, Averdi; Hermani, Bambang; Gondhowiardjo, Soehartati; Tan, I Bing; Middeldorp, Jaap M.
Among all head and neck (H&N) cancers, nasopharyngeal carcinoma (NPC) represents a distinct entity regarding epidemiology, clinical presentation, biological markers, carcinogenic risk factors, and prognostic factors. NPC is endemic in certain regions of the world, especially in Southeast Asia, and has a poor prognosis. In Indonesia, the recorded mean prevalence is 6.2/100 000, with 13 000 yearly new NPC cases, but otherwise little is documented on NPC in Indonesia. Here, we report on a group of 1121 NPC patients diagnosed and treated at Dr. Cipto Mangunkusumo Hospital, Jakarta, Indonesia between 1996 and 2005. We studied NPC incidence among all H&N cancer cases (n=6000) observed in that period, focusing on age and gender distribution, the ethnic background of patients, and the disease etiology. We also analyzed most prevalent signs and symptoms and staging of NPC patients at first presentation. In this study population, NPC was the most frequent H&N cancer (28.4%), with a male-to-female ratio of 2.4, and was endemic in the Javanese population. Interestingly, NPC appeared to affect patients at a relatively young age (20% juvenile cases) without a bimodal age distribution. Mostly, NPC initiated in the fossa of Rosenmuller and spreaded intracranially or locally as a mass in the head. Occasionally, NPC developed at the submucosal level spreading outside the anatomic limits of the nasopharynx. At presentation, NPC associated with hearing problems, serous otitis media, tinnitus, nasal obstruction, anosmia, bleeding, difficulty in swallowing and dysphonia, and even eye symptoms with diplopia and pain. The initial diagnosis is difficult to make because early signs and symptoms of NPC are not specific to the disease. Early-age Epstein-Barr virus (EBV) infection combined with frequent exposure to environmental carcinogenic co-factors is suggested to cause NPC development. Undifferentiated NPC is the most frequent histological type and is closely associated with EBV
Bartley, G B
Among incident cases of GO in Olmsted County, Minnesota: GO affected females six times more frequently than males (86% versus 14% of cases, respectively). The age-adjusted incidence rate was 16 cases per 100,000 population per year for females and 2.9 cases per 100,000 population for males. The peak incidence rates were bimodal, occurring in the age groups 40 to 44 years and 60 to 64 years in females and 45 to 49 years and 65 to 69 years in males. Among patients with GO, approximately 90% had Graves' hyperthyroidism, 1% had primary hypothyroidism, 3% had Hashimoto's thyroiditis, and 5% were euthyroid. Eyelid retraction was the most common ophthalmic feature of autoimmune thyroid disease, being present either unilaterally or bilaterally in more than 90% of patients at some point in their clinical course. Exophthalmos of one or both eyes affected approximately 60% of patients, restrictive extraocular myopathy was apparent in about 40% of patients, and optic nerve dysfunction occurred in either one or both eyes in 6% of patients with autoimmune thyroid disease. Only 5% of patients had the complete constellation of classic findings: eyelid retraction, exophthalmos, optic nerve dysfunction, extraocular muscle involvement, and hyperthyroidism. Upper eyelid retraction, either unilateral or bilateral, was documented in approximately 75% of patients at the time of diagnosis of GO. Lid lag also was a frequent early sign, being present either unilaterally or bilaterally in 50% of patients at the initial examination. At the time of diagnosis of GO, the most frequent ocular symptom was pain or discomfort, which affected 30% of patients. Some degree of diplopia was noted by approximately 17% of patients, lacrimation or photophobia was present in about 15% to 20% of patients, and 7.5% of patients complained of blurred vision. Decreased vision attributable to optic neuropathy was present in less than 2% of eyes at the time of diagnosis of GO. Thyroid dermopathy and acropachy
Šuchaň, M; Horňák, M; Kaliarik, L; Krempaská, S; Koštialová, T; Kovaľ, J
Orbital complications categorised by Chandler are emergency. They need early diagnosis and agresive treatment. Stage and origin of orbital complications are identified by rhinoendoscopy, ophtalmologic examination and CT of orbite and paranasal sinuses. Periorbital cellulitis and early stage of orbital cellulitis can be treated conservatively with i. v. antibiotics. Monitoring of laboratory parameters and ophtalmologic symptoms is mandatory. Lack of improvement or worsening of symptoms within 24-48 hours and advanced stages of orbital complications are indicated for surgery. The purpose of the study is to evaluate epidemiology, clinical features and management of sinogenic orbital complications. Retrospective data of 8 patients with suspicion of orbital complication admited to hospital from 2008 to 2013 were evaluated. Patients were analyzed in terms of gender, age, CT findings, microbiology, clinical features, stage and treatment. Male and female were afected in rate 1,66:1. Most of patients were young adult in 3rd. and 4th. decade of life (62,5 %). Acute and chronic sinusitis were cause of orbital complication in the same rate. The most common origin of orbital complication was ethmoiditis (62,5 %), than maxillary (25 %) and frontal (12,5 %) sinusitis. Polysinusitis with affection of ethmoidal, maxillary and frontal sinuses (75 %) was usual CT finding. Staphylococcus epidermidis and Staphylococcus aureus were etiological agens in half of cases. Periorbital oedema (100 %), proptosis, chemosis (50 %), diplopia and glaucoma (12,5 %) were observed. Based on examinations, diagnosis of periorbital oedema/preseptal cellulitis was made in 3 (37,5 %), orbital cellulitis in 3 (37,5 %) and subperiosteal abscess in 2 cases (25 %). All patients underwent combined therapy - i. v. antibiotics and surgery within 24 hours. Eradication of disease from ostiomeatal complex (OMC), drainage of affected sinuses and drainage of subperiosteal abscess were done via fuctional endonasal
Hajnsek, Sanja; Paladino, Josip; Gadze, Zeljka Petelin; Nanković, Sibila; Mrak, Goran; Lupret, Velimir
In the last 20 years neurological and neurosurgical follow up of our patients with pineal region expansions (118 patients) pointed to certain clinical and neurophysiological regularities. We performed retrospective study which included 84 patients with pineal region expansions in the period from 1992 to 2009. The study included 55 women and 29 men, mean age 30.08 +/- 13.93 years, with positive brain magnetic resonance imaging (MRI)--70 patients (83.4%) had simple pineal gland cysts, and 14 patients (16.67%) had expansive process in pineal region with compressive effect. All patients had headache, while 32 patients (38%) had epileptic phenomena--primary generalized seizures. Patients had common electroencephalography (EEG) pattern with paroxysmal discharges of 3Hz (or more than 3 Hz) spike-and-wave complexes. Operation with supracerebellar infratentorial approach was performed in 70 patients. In most of our patients indication for the operation was established based on the size of the cyst (15 mm or more), with the signs of compression on the quadrigeminal plate and compression of the surrounding veins, which could result in seizures and EEG changes verified in our group of patients. Pathohistological analysis revealed pineocytomas in 11 cases (15.71%), pinealoblastomas in 2 cases (2.86%), one case of teratoma (1.43%), while 56 patients had pineal gland cysts (80%). Following surgery clinical condition improved in all patients--patients became seizure-free and headaches significantly decreased. Other symptoms including diplopiae, nausea, vomiting, vertigo as well as blurred vision also disappeared. There were no complications after surgical procedures. This study points to often appearance of seizures that clinically and neurophysiologically present as primary generalized epilepsy in patients with pineal region expansions. Our hypotheses are that mass effect on the surrounding veins that affects normal perfusion, compressive effect on the quadrigeminal plate and the
Hwang, Kun; Yeom, Seung Han; Hwang, Suk Hyun
The aim of this study was to perform a systematic review of the treatment of nasal bone fractures. The search terms ("nasal bone fracture" AND complication) and ("nasal bone fracture" AND [anosmia OR olfaction OR olfactory nerve OR smell]) and (anosmia AND ["nasal preparation" OR "nasal antiseptics"]) were used to search PubMed and SCOPUS. Of the 500 titles, 40 full papers were reviewed. One paper was excluded, and 3 mined papers were added. Ultimately, 12 papers were analyzed. The overall deformity rate was 10.4% ± 4.8%. No significant differences were found between patients who underwent closed reduction (14.7% ± 7.3%) and those who underwent open reduction (9.4% ± 4.4%), between those who underwent local anesthesia (5.8% ± 4.5%), and those who underwent general anesthesia (8.8% ± 3.8%), or between those who received timely treatment (5.7%) and those whose treatment was delayed (9.0%). Septal deviation occurred in 10.0% of patients as a sequela of nasal bone fracture. The nasal obstruction rate was 10.5% ± 5.3%. Fewer patients of nasal obstruction occurred in the open reduction patients (6.9% ± 4.4%) than in the closed reduction patients (15.2%). One patient of epiphora and 1 patient of diplopia were reportedAmong the 77 patients with nasal bone fractures, 29 (37.7% ± 11.3%) complained of olfactory disturbances. No significant associations were found between the type of fracture and the presence of olfactory disturbances. It is recommended for providers to explain to patients that approximately one-tenth of nasal bone fractures exhibit deformity, septal deviation, or nasal obstruction after surgery. Surgeons should take considerable care to avoid the olfactory mucosa during reduction surgery.
Nampoory, M R; Johny, K V; Gupta, R K; Constandi, J N; Nair, M P; al-Muzeiri, I
Idiopathic intracranial hypertension is a disorder of intracerebral pressure regulation and patients run the risk of permanent visual loss. Intracranial hypertension (IH) has been reported rarely in systemic lupus erythematosus (SLE). We reviewed the medical records of 127 patients with lupus nephritis (LN) who were followed up from 1987 to 1996 in our unit. There were six patients with IH which gave a disease prevalence of 4.7% in those with LN. All were females giving a disease prevalence of 5.2% for that sex, a high rate of occurrence of IH in patients with LN. Their age ranged from 22 to 34 y (27.8 +/- 3.6 y). Headache, vomiting and diplopia were the common presenting symptoms and had started 7.3 +/- 4.4 weeks prior to the diagnosis of IH. The cerebrospinal (CSF) opening pressure (413.3 +/- 77.0 mmH2O) was raised in all cases. Biochemical and cytological analyses of CSF were normal. The only abnormal radiological finding was partially empty sella in one patient on magnetic resonance imaging (MRI) (performed in three patients) or computed tomography (CT) (performed in all patients). All patients had serological evidences of active lupus disease at the time of diagnosis of IH. The renal histology was WHO type IV in four cases and III and V in one each indicating severe renal involvement. Laboratory evidences of procoagulant activity were found in the form of positive anticardiolipin antibody (aCL) in two patients, lupus anticoagulant (LA) in two and an otherwise unexplained isolated prolongation of activated partial thromboplastin time (APTT) in the other two. Clinically, one or more episodes of symptomatic venous or arterial thrombosis had occurred in all subjects. In addition to symptomatic measures, all subjects were treated with prednisolone, azathioprine, cyclophosphamide and plasmapheresis according to the protocol of our unit. One patient who did not receive plasmapheresis and cyclophosphamide had a relapse while all others recovered completely. None
Ellis, Michael J; Cordingley, Dean M; Vis, Sara; Reimer, Karen M; Leiter, Jeff; Russell, Kelly
OBJECTIVE There were 2 objectives of this study. The first objective was to identify clinical variables associated with vestibulo-ocular dysfunction (VOD) detected at initial consultation among pediatric patients with acute sports-related concussion (SRC) and postconcussion syndrome (PCS). The second objective was to reexamine the prevalence of VOD in this clinical cohort and evaluate the effect of VOD on length of recovery and the development of PCS. METHODS A retrospective review was conducted for all patients with acute SRC and PCS who were evaluated at a pediatric multidisciplinary concussion program from September 2013 to May 2015. Acute SRS was defined as presenting < 30 days postinjury, and PCS was defined according to the International Classification of Diseases, 10th Revision criteria and included being symptomatic 30 days or longer postinjury. The initial assessment included clinical history and physical examination performed by 1 neurosurgeon. Patients were assessed for VOD, defined as the presence of more than 1 subjective vestibular and oculomotor complaint (dizziness, diplopia, blurred vision, etc.) and more than 1 objective physical examination finding (abnormal near point of convergence, smooth pursuits, saccades, or vestibulo-ocular reflex testing). Poisson regression analysis was used to identify factors that increased the risk of VOD at initial presentation and the development of PCS. RESULTS Three hundred ninety-nine children, including 306 patients with acute SRC and 93 with PCS, were included. Of these patients, 30.1% of those with acute SRC (65.0% male, mean age 13.9 years) and 43.0% of those with PCS (41.9% male, mean age 15.4 years) met the criteria for VOD at initial consultation. Independent predictors of VOD at initial consultation included female sex, preinjury history of depression, posttraumatic amnesia, and presence of dizziness, blurred vision, or difficulty focusing at the time of injury. Independent predictors of PCS among
Kollewe, Katja; Mohammadi, Bahram; Köhler, Steffen; Pickenbrock, Heidrun; Dengler, Reinhard; Dressler, Dirk
Botulinum toxin (BT) therapy is the treatment of choice for blepharospasm (BPS). Currently available BT type A drugs include Botox(®), Dysport(®) and Xeomin(®). Until now, there are few long-term studies on BT therapy for BPS. This is the first long-term study comparing all three major BT drugs. We collected treatment, efficacy and adverse effect data on BPS patients treated with either Botox(®), Dysport(®) or Xeomin(®) for at least eight consecutive treatments. Two hundred and eighty-eight patients (208 females, 80 males, age 62 ± 12 years) were included in this study. The treatment time was 11.2 ± 4.1 years covering 10,701 injection series. Doses were 47 ± 10 MU for Botox(®), 120 ± 35 MU for Dysport(®) and 62 ± 11 MU for Xeomin(®) (Botox(®) dose vs Xeomin(®) dose: p < 0.001, unpaired t test). 85 % of all patients had stable doses. The onset of the therapeutic effect was after 6.1 ± 3.3 days and its duration lasted 10.2 ± 3.5 weeks. The Global Clinical Improvement (GCI, 0 = no, 1 = slight, 2 = moderate, 3 = marked improvement in severity and function) as estimated by the patient was 2.5 ± 0.6. It was stable in 90% of the patients. Adverse effect frequency was 3.0% (ptosis 2.3%, dry eye 0.5%, diplopia 0.2%). None of these findings was significantly different between Botox(®), Dysport(®) and Xeomin(®). Our study, one of the largest studies on BT therapy of BPS and the study with the longest follow-up, confirms that BT therapy produces robust clinical improvement which is stable throughout the treatment time. Therapeutic effects start after 6.1 days and last for about 10 weeks before they start to vanish. With this, they are approximately 2 weeks shorter than the recommended inter-injection interval. Adverse effects were rare, mild and always transient. BT therapy is a safe and effective treatment for BSP. Shorter inter-injection intervals may improve therapeutic results.
Graillon, T; Fuentes, S; Metellus, P; Adetchessi, T; Gras, R; Dufour, H
Advances in transsphenoidal surgery and endoscopic techniques have opened new perspectives for cavernous sinus (CS) approaches. The aim of this study was to assess the advantages and disadvantages of limited endoscopic transsphenoidal approach, as performed in pituitary adenoma surgery, for CS tumor biopsy illustrated with three clinical cases. The first case was a 46-year-old woman with a prior medical history of parotid adenocarcinoma successfully treated 10 years previously. The cavernous sinus tumor was revealed by right third and sixth nerve palsy and increased over the past three years. A tumor biopsy using a limited endoscopic transsphenoidal approach revealed an adenocarcinoma metastasis. Complementary radiosurgery was performed. The second case was a 36-year-old woman who consulted for diplopia with right sixth nerve palsy and amenorrhea with hyperprolactinemia. Dopamine agonist treatment was used to restore the patient's menstrual cycle. Cerebral magnetic resonance imaging (MRI) revealed a right sided CS tumor. CS biopsy, via a limited endoscopic transsphenoidal approach, confirmed a meningothelial grade 1 meningioma. Complementary radiosurgery was performed. The third case was a 63-year-old woman with progressive installation of left third nerve palsy and visual acuity loss, revealing a left cavernous sinus tumor invading the optic canal. Surgical biopsy was performed using an enlarged endoscopic transsphenoidal approach to the decompress optic nerve. Biopsy results revealed a meningothelial grade 1 meningioma. Complementary radiotherapy was performed. In these three cases, no complications were observed. Mean hospitalization duration was 4 days. Reported anatomical studies and clinical series have shown the feasibility of reaching the cavernous sinus using an endoscopic endonasal approach. Trans-foramen ovale CS percutaneous biopsy is an interesting procedure but only provides cell analysis results, and not tissue analysis. However, radiotherapy and
Kuhnt, T; Müller, A C; Janich, M; Gerlach, R; Hädecke, J; Duncker, G I W; Dunst, J
Graves' ophthalmopathy (GO) is the most frequent extrathyroidal manifestation of Graves' disease, an autoimmune disorder of the thyroid, whereas the precise pathogenesis still remains unclear. In Hashimoto's thyroiditis the occurrence of proptosis is an extremely rare event. The therapy for middle and severe courses of GO shows in partly disappointing results, although several therapy modalities are possible (glucocorticoid therapy, radiotherapy, antithyroid drug treatment, surgery). All these therapies lead in only 40 - 70 % to an improvement of the pathogenic symptoms. An intensive interdisciplinary cooperation is necessary to satisfy the requirements for the treatment of Graves' ophthalmopathy. As a consequence of the very different results of the few of clinical studies that were accomplished with reference to this topic, treatment by radiotherapy in the management of the disease is presently controversially discussed. In the German-speaking countries the radiotherapy is, however, firmly established as a therapy option in the treatment of the moderate disease classes (class 2-5 according to NO SPECS), especially if diplopia is present. This article describes the sequences, dosages and fractionation schemes as well as the risks and side effects of the radiotherapy. Altogether, radiotherapy is assessed as an effective and sure method. The administration of glucocorticoids can take place before the beginning of or during the radiotherapy. For the success of treatment the correct selection of patients who may possibly profit from a radiotherapy is absolutely essential. By realising that GO proceeds normally over a period of 2-5 years, which is followed by a period of fibrotic alteration, the application of the radiotherapy in the early, active phase is indispensable. A precise explanation for the effects of radiotherapy in treatment of the GO does not exist at present. The determination of the most effective irradiation doses was made from retrospectively evaluated