Comitant Ocular Deviation in Myasthenia Gravis.

BACKGROUND: Occurrence of comitant ocular deviation in myasthenia gravis (MG) is not well described. METHODS: A retrospective analysis of patients with ocular or generalized MG evaluated at a neuro-ophthalmology clinic for a 6-year period. Comitant ocular deviation was defined as magnitude of deviations in all planes varying by <20% from the measurement in the primary position. RESULTS: Among the 120 patients included, 89 patients had ocular and 31 patients generalized MG. At the initial strabismus testing, comitant ocular deviation was present in 27 (22.5%) patients. Among the 16 patients who had a follow-up, ocular deviation remained comitant in 6 patients and converted to incomitant or no ocular deviation in 10 patients. An additional 7 patients demonstrated comitant ocular deviation at follow-up. Brain MRI was performed in 18 patients with comitant ocular deviation, and none showed abnormalities in the brainstem or cerebellum. CONCLUSION: Comitant ocular deviation can be an ocular manifestation of MG. Its presence does not necessarily indicate a central etiology in patients with MG neither excluding a MG diagnosis.

Optic Neuropathy and Stroke Secondary to Invasive Aspergillus in an Immunocompetent Patient.

Angioinvasive aspergillosis is an aggressive fungal infection that is potentially life threatening without prompt treatment. Optic nerve involvement of Aspergillus can mimic optic neuritis commonly seen in demyelinating and other inflammatory conditions. Treatment of Aspergillus infection with steroids may worsen the clinical course. We describe a unique case of disseminated central nervous system aspergillosis, initially presenting as an optic neuropathy, with subsequent stroke in multiple vascular territories.

Idiopathic intracranial hypertension: pseudotumor cerebri.

BACKGROUND: Idiopathic intracranial hypertension (IIH) is most often diagnosed in young obese females of childbearing years. The diagnosis is made based on the modified Dandy criteria and the exclusion of alternate causes of raised intracranial pressure. RESULTS: The focus of this review is to provide an overview of the diagnosis and treatment options for patients with IIH. There are long-term consequences for patients experiencing IIH, with visual loss being the most serious. CONCLUSION: We conclude that the diagnosis of IIH is not usually difficult. An ophthalmologic examination is essential in patients with IIH to monitor visual function. A neurologist or neurosurgeon may be needed at some point for medical and/or surgical intervention.

Crossover trial of gabapentin and memantine as treatment for acquired nystagmus.

We conducted a masked, crossover, therapeutic trial of gabapentin (1,200mg/day) versus memantine (40 mg/day) for acquired nystagmus in 10 patients (aged 28-61 years; 7 female; 3 multiple sclerosis [MS]; 6 post-stroke; 1 post-traumatic). Nystagmus was pendular in 6 patients (4 oculopalatal tremor; 2 MS) and jerk upbeat, hemi-seesaw, torsional, or upbeat-diagonal in each of the others. For the group, both drugs reduced median eye speed (p < 0.001), gabapentin by 32.8% and memantine by 27.8%, and improved visual acuity (p < 0.05). Each patient improved with 1 or both drugs. Side effects included unsteadiness with gabapentin and lethargy with memantine. Both drugs should be considered as treatment for acquired forms of nystagmus.

Selected lid problems in neurologic practice.

Ptosis is a common clinical finding. The many causes of ptosis include involutional (aging), myopathic, neuropathic, congenital, infectious, tumorous, traumatic, and inflammatory processes. This article reviews recent literature on some causes of ptosis and other lid conditions.

Nonuniform pressure generation in the optic chiasm may explain bitemporal hemianopsia.

PURPOSE: To measure the pressure in the temporal and central aspects of the chiasm simultaneously during compression of the optic chiasm from below with an expanding simulated tumor. DESIGN: Experimental study. METHODS: Craniotomies were performed on 5 unfixed cadaveric specimens ranging in age from 49 to 89 years, and the optic chiasm was exposed. After a pediatric gauge Foley catheter was inserted into the sellar region, the temporal side of the chiasm (temporal aspect uncrossed fibers) was impaled with a 30-gauge needle connected to a pressure transducer. In like fashion, the central portion of the chiasm (crossed fibers) was impaled with another 30-gauge needle connected to a pressure transducer on a separate channel. The Foley catheter was inflated for a period of 30 seconds to 1 minute and the pressure in each channel continuously monitored. Multiple trials were performed on each of the 5 specimens. MAIN OUTCOME MEASURE: Pressure change between the temporal and nasal aspects of the optic chiasm. RESULTS: In all cases in which intrachiasmal pressure could be measured, higher pressures were always generated in the central aspect of the chiasm than in the temporal aspect of the chiasm. In cadaver 1, the mean central pressure was 19.75 mmHg, whereas the mean temporal pressure was 6 mmHg. In cadaver 5, the mean central pressure was 4 mmHg, whereas the mean temporal pressure was zero. Donors 70 and older demonstrated abnormally thin and frail chiasms that were unable to support a sustained pressure increase during simulated tumor expansion. CONCLUSIONS: During deformation of the optic chiasm from below by a radially expanding mass analogous to a pituitary tumor, the central aspect of the optic chiasm consistently manifests a higher pressure than the temporal aspect. It is hypothesized that the peculiar geometry of the optic chiasm renders the crossing nasal fibers more prone to a deformation stress exerted from below. Nonuniform pressure generation between the central and temporal aspects of the chiasm results in a greater effective stress on the crossing fibers of the chiasm and may be responsible for the clinical phenomenon of bitemporal hemianopsia.

My, what asthenia you have.

A 59-year-old woman noted intermittent ptosis, diplopia, dysphagia, and proximal muscle weakness for several years. She had a strong family history of myasthenia gravis. Chest computed tomography and sternotomy revealed a micronodular spindle cell thymoma. Electromyography and antibody testing was negative for myasthenia gravis. Genetic testing confirmed a diagnosis of oculopharyngeal muscular dystrophy.

Prevalence of optic nerve edema in patients on peripheral hemodialysis.

PURPOSE: Cerebral venous hypertension with optic nerve edema has been reported in patients with peripheral arteriovenous hemodialysis shunts. This study aimed to estimate the prevalence of optic nerve edema in patients with peripheral arteriovenous accesses and to evaluate the value of ophthalmic examination and surveillance in this study population. DESIGN: Cross-sectional observation case series. PARTICIPANTS: Forty-four patients with peripheral arteriovenous shunts for hemodialysis. METHODS: A cross-sectional observation was done of all patients with peripheral arteriovenous shunts presenting to our outpatient hemodialysis unit on 2 consecutive days. Using indirect ophthalmoscopy, the presence or absence of optic nerve edema was recorded. Patients also were asked to record any symptoms suggestive of intracranial hypertension and/or papilledema such as headache, decreased visual acuity, or an abnormal visual phenomenon. The 95% confidence interval (CI) was calculated to estimate the prevalence of optic nerve edema in patients with peripheral arteriovenous accesses. A literature search also was conducted to obtain prior reports of optic nerve edema and ophthalmic complications in patients with peripheral arteriovenous accesses. MAIN OUTCOME MEASURES: Presence or absence of optic nerve edema. RESULTS: Among our series of 44 patients with peripheral arteriovenous shunts for hemodialysis, no case of optic nerve edema was observed and no patient reported any headache, decrease in vision, or visual phenomenon. The 95% CI for the estimated prevalence of optic nerve edema was 0% to 8.0%. A literature review revealed 7 reports of symptomatic ophthalmic complications in patients with peripheral arteriovenous accesses. CONCLUSIONS: Although cases of papilledema in patients with peripheral arteriovenous shunts have been reported in the literature, the occurrence appears to be low, and routine ophthalmic surveillance is probably unnecessary in asymptomatic patients.

Conjunctival involvement in Churg-Strauss syndrome.

PURPOSE: To describe an unusual case of Churg-Strauss syndrome (CSS) that presented with a conjunctival nodule and was successfully treated with oral corticosteroids. METHODS: Case report. RESULTS: A 30-year-old woman with a history of adult-onset asthma, seasonal allergies, and a lung mass presented with a nodular elevation of the conjunctiva. Excisional biopsy demonstrated necrotizing eosinophilic granulomas. Systemic evaluation revealed peripheral eosinophilia and elevated IgE, consistent with Churg-Strauss syndrome. The patient's symptoms and eosinophilia resolved after an increase in the dose of oral corticosteroids. CONCLUSIONS: This case describes a unique presentation of conjunctival involvement in CSS that differs from prior case reports by having a small, well-demarcated nodule and lacking signs of active inflammation.

Isolated optic nerve sarcoidosis.

Objective: To report three cases of sarcoidosis confined to the optic nerve. Methods: Chart review of clinical, laboratory, imaging, and optic nerve biopsy findings and a review of the literature. Results: All three cases presented with progressive visual loss and showed enhancement of the intraorbital optic nerve on magnetic resonance imaging. There was no evidence for systemic disease, including a negative workup for sarcoidosis or other infiltrative pathologies. Optic nerve biopsy in each case showed non-caseating granulomas consistent with sarcoidosis. Conclusions: Sarcoidosis confined to the optic nerve is a rare phenomenon but should still be considered in the differential diagnosis of progressive optic neuropathy, even in the absence of systemic disease. (Sarcoidosis Vasc Diffuse Lung Dis 2017; 34: 179-183).

Optic neuropathy in vitamin B12 deficiency.

There are many neurological manifestations of vitamin B(12) deficiency. Optic neuropathy is a rare, but important, manifestation of vitamin B(12) deficiency that should be suspected in patients with risk factors for malnutrition. We present a case of a 68-year-old male who presented with bilateral decreased central vision for months and was found to have a low vitamin B(12) level. After a few months, his vision improved with parenteral vitamin B(12) supplementation. Vitamin B(12) optic neuropathy is a reversible, treatable cause of vision loss and may be a harbinger for other manifestations of the disease.

Ocular ethambutol toxicity.

Ethambutol is an antimicrobial agent used frequently to treat tuberculosis. The most commonly recognized toxic effect of ethambutol is optic neuropathy, which generally is considered uncommon and reversible in medical literature. We describe a 43-year-old man who developed signs and symptoms of bilateral optic neuropathy during treatment with ethambutol. This case and a review of the literature show the severe and unpredictable nature of ethambutol toxicity and its potential for irreversible vision loss despite careful ophthalmologic monitoring.

Gabapentin attenuates superior oblique myokymia.

PURPOSE: To investigate therapeutic effects of oral gabapentin therapy on superior oblique myokymia. DESIGN: Observational case series with measurement of visual acuity and eye movements before, during, and after therapy. METHODS: Two adult patients with superior oblique myokymia, refractory to other therapies, were treated with gabapentin orally after informed consent was obtained. Eye movements were measured using the magnetic search coil technique. RESULTS: Superior oblique myokymia completely resolved after starting gabapentin. CONCLUSION: Gabapentin may be an effective treatment for superior oblique myokymia; a double-blind study seems justified.

Chest computerized tomography in the evaluation of uveitis in elderly women.

PURPOSE: To describe the usefulness of chest computerized tomography (CT) in the evaluation of uveitis in elderly women and the clinical characteristics of patients with an abnormal chest CT scan. DESIGN: Prospective noncomparative case series. METHODS: We evaluated 30 elderly women, aged 61-83 years, with chronic iritis, vitritis, or choroiditis and with no definitive cause for their uveitis. All patients underwent a battery of diagnostic laboratory studies and chest CT. RESULTS: The diagnostic examination in most patients included serum angiotensin converting enzyme level, serum lysozyme, rapid plasma reagin level, fluorescent treponemal antibody-absorption test, purified protein derivative skin test, and chest x-rays. Chest CT performed on all patients showed parenchymal, mediastinal, and/or hilar adenopathy in 17 patients (57%). Histopathologic confirmation of sarcoidosis with noncaseating granulomas in the biopsy specimens was obtained in 14 patients: eight by mediastinoscopy, two by bronchoscopy, two by conjunctival biopsy, one by nasal biopsy, and one by vitreous biopsy. CONCLUSIONS: Chest CT can be useful in elderly female patients with chronic uveitis for identifying mediastinal lymphadenopathy and other lesions suggestive of sarcoidosis, as well as to help guide tissue confirmation and to rule out other diagnoses including lymphoma.

Prevalence of psychosocial disturbances in children with nonorganic visual loss.

PURPOSE: To report on the prevalence of psychiatric disease and psychosocial stress in children with nonorganic visual loss. MATERIALS AND METHODS: Case series of 71 consecutive pediatric patients with a variety of nonorganic visual signs and symptoms. Chart review of ophthalmologic findings and details of medical and social history with main outcome measures of prevalence of psychiatric disease and psychosocial stress. RESULTS: Psychological/psychiatric disturbances in the form of anxiety, depression, and attention deficit hyperactivity disorder had been previously diagnosed in 19 (26.7%) patients. We had a very high index of suspicion of psychiatric illness in another four patients. Furthermore, we uncovered significant home and school stress in 22 (31%) patients. Sixteen (22.5%) patients wanted glasses and in 10 (14.1%) patients no cause for the behavior could be determined. Of those patients who wanted glasses, girls outnumbered boys 3:1. CONCLUSIONS: An underlying psychiatric or psychosocial disturbance should be ruled out in children who present with nonorganic visual loss.

A pain in the eye.

A 38-year-old woman presented with chronic left retro-orbital pain and photophobia. Palpation of the left occipital prominence revealed tenderness in the area of the greater occipital nerve and reproduced the eye pain. The diagnosis of cervicogenic headache was confirmed by symptom resolution following left greater occipital nerve blockade. The functional association between the greater occipital nerve and the trigeminal nerve provides a potential mechanism for this case of cervicogenic eye pain.

Can't hear, can't see, and too sore to play.

A 52-year-old man developed transient, migratory polyarthralgias in the presence of hearing loss. He then developed persistent leukocytosis and thrombocytosis. His initial transient, bilateral visual obscurations happened in context with bilateral disk edema and an enlarged blind spot. Visual symptoms progressed to vision loss and multiple branch retinal artery occlusions. It was not until later in the disease progression that gastrointestinal symptoms occurred. Electron microscopy of duodenal biopsies confirmed a diagnosis of Whipple disease.