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.

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 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.

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.

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.

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.

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.

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.

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.

The fate of patients with retinal artery occlusion and Hollenhorst plaque.

OBJECTIVE: Ocular symptoms and signs often herald hemispheric neurological events associated with extracranial cerebrovascular disease. However, the presence of a Hollenhorst plaque (HP) or retinal artery occlusion (RAO) and the risk of stroke is unclear. The purpose of this study was to review the outcomes of all patients who presented with a HP or RAO at a single institution. METHODS: Between 2000 and 2005, the management and outcome of 130 consecutive patients with a diagnosis of HP, central RAO, or branch RAO (ICD-9 codes 362.30 to 362.33) were reviewed. Patients with transient monocular visual loss (amaurosis fugax), retinal venous occlusion, and other ocular pathologies were excluded. Electronic and hardcopy medical records were reviewed for demographic data, clinical variables, radiological, and noninvasive vascular lab testing. Duplex and magnetic resonance angiography (MRA) of the carotid arteries were reviewed to confirm the presence of a lesion and quantify the degree of stenosis. RESULTS: During the study interval, 70 males and 60 females, with a mean age of 68 +/- 16 (+/-SD) years underwent ophthalmologic evaluation. Symptoms were present in 61% of patients and included eye pain, blurred vision, or atypical visual symptoms, while 39% were asymptomatic. Atherosclerotic risk factors in this population included the presence of hypertension (73%), diabetes (33%), hyperlipidemia (75%), and tobacco use (38%). A majority of patients underwent carotid interrogation via Duplex imaging (68%). Carotid bifurcation stenoses ipsilateral to the ocular findings were <30% in 68% of the patients, between 30 and 60% in 22% and >60% in only 8% of patients. Six patients with lesions greater than 60% went on to have either a carotid endarterectomy or carotid stenting. Follow-up data on this group ranged from 1 to 49 months (median, 22 months), with no stroke or transient ischemic attack identified. There were five deaths during follow-up; none related to stroke. Serial carotid Duplex examinations failed to identify progression of carotid stenoses in this group of patients. Overall survival was 94% at 36 months for this cohort. CONCLUSION: The presence of a HP or RAO is associated with a low prevalence of extracranial cerebrovascular disease that requires intervention. Furthermore, in contradistinction to amaurosis fugax, these ocular findings are not associated with a high risk for hemispheric neurological events.

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.

Rapamycin for treatment of refractory dysthyroid compressive optic neuropathy.

We report a patient with dysthyroid optic neuropathy refractory to steroids and orbital decompression treated with rapamycin, a fibroblast and T cell inhibitor. Symptoms, visual acuity, color plate testing, and visual fields improved. Aside from hypercholesterolemia, no complication related to this therapy was observed. By addressing the pathogenesis of thyroid eye disease, rapamycin may represent an alternative when standard treatments fail. Further investigation of rapamycin for treatment of dysthyroid orbitopathy is warranted.

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.

Branch retinal artery occlusion associated with compound heterozygous genotype for methylenetetrahydrofolate reductase.

We present a case in which mfERG and OCT helped to make a diagnosis of an old BRAO in the setting of compound heterozygous MTHFR genotype. A 44-year-old woman presented for evaluation of a 10 month history of persistently cloudy vision OS. She had been worked up previously for MS versus BRAO, and she was on coumadin, folate, and multivitamin at the time of presentation. The patient has a fraternal twin sister who was diagnosed with MS. Dilated fundus examination OS showed subtle inferior optic atrophy with slight narrowing of the inferotemporal retinal artery, and HVF test revealed a superonasal depression OS. mfERG also showed superonasal depression OS. Retinal origin of the chief complaint was further confirmed by OCT, which showed thinning of the NFL in the corresponding region of the retina OS. Coagulopathy evaluation revealed C677T/A1298C compound heterozygous genotype for MTHFR, and plasma homocysteine level after 6 months of folate and multivitamin supplementation was 10 microM (reference range 4-10 microM). The patient was diagnosed with BRAO and maintained on coumadin therapy.

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.

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.

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.