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.

A Case Report of Drug-Induced Myopathy Involving Extraocular Muscles after Combination Therapy with Tremelimumab and Durvalumab for Non-Small Cell Lung Cancer.

Recently developed anti-tumour therapies targeting immune checkpoints include tremelimumab and durvalumab. These agents have incompletely characterised side effect profiles. The authors report a 68-year-old man treated for non-small cell lung cancer (NSCLC) with a combination of tremelimumab and durvalumab. After treatment he developed diplopia, ptosis, fatigue, weakness, and an inflammatory myopathy affecting the extraocular muscles requiring hospitalisation. Electromyography (EMG) testing and muscle biopsy suggested inflammatory myopathy without sign of myasthenia. Within 1 month of withdrawal of cancer therapies and initiation of oral steroid therapy, ocular and systemic symptoms had resolved. This notable adverse effect has not been previously described for these drugs administered singly or in combination, and ophthalmologists should be aware of this presentation in patients treated with these agents.

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.

The Microbiome of Temporal Arteries.

OBJECTIVE: A role for microorganisms in giant cell arteritis (GCA) has long been suspected. We describe the microbiomes of temporal arteries from patients with GCA and controls. METHODS: Temporal artery biopsies from patients suspected to have GCA were collected under aseptic conditions and snap-frozen. Fluorescence in situ hybridization (FISH) and long-read 16S rRNA-gene sequencing was used to examine microbiomes of temporal arteries. Taxonomic classification of bacterial sequences was performed to the genus level and relative abundances were calculated. Microbiome differential abundances were analyzed by principal coordinate analysis (PCoA) with comparative Unifrac distances and predicted functional profiling using PICRUSt. RESULTS: Forty-seven patients, including 9 with biopsy-positive GCA, 15 with biopsy-negative GCA and 23 controls without GCA, were enrolled. FISH for bacterial DNA revealed signal in the arterial media. Beta, but not alpha, diversity differed between GCA and control temporal arteries (P = 0.042). Importantly, there were no significant differences between biopsy-positive and biopsy-negative GCA (P > 0.99). The largest differential abundances seen between GCA and non-GCA temporal arteries included Proteobacteria (P), Bifidobacterium (g), Parasutterella (g), and Granulicatella (g) [Log 2-fold change ≥ 4]. CONCLUSION: Temporal arteries are not sterile, but rather are inhabited by a community of bacteria. We have demonstrated that there are microbiomic differences between GCA and non-GCA temporal arteries, but not between biopsy-positive and biopsy-negative GCA.

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.

Retinal manifestations of cerebroretinal vasculopathy.

We report a case of a 42-year-old woman who presented with a slowly progressive decrease in visual acuity and memory loss. Dilated fundus exam showed cotton-wool spots and parafoveal telangiectasias. Fluorescein angiography revealed foveal capillary nonperfusion, which was very similar to that of her father, who passed away from a similar illness. These two patients share the same clinical, angiographic and pathologic features as a pedigree that had autosomal dominant cerebroretinal vasculopathy. Hereditary small vessel disease of the brain and retina is a spectrum of different entities that has many manifestations in end organs outside of the central nervous system.

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).

Development and validation of a computerized expert system for evaluation of automated visual fields from the Ischemic Optic Neuropathy Decompression Trial.

BACKGROUND: The objective of this report is to describe the methods used to develop and validate a computerized system to analyze Humphrey visual fields obtained from patients with non-arteritic anterior ischemic optic neuropathy (NAION) and enrolled in the Ischemic Optic Neuropathy Decompression Trial (IONDT). The IONDT was a multicenter study that included randomized and non-randomized patients with newly diagnosed NAION in the study eye. At baseline, randomized eyes had visual acuity of 20/64 or worse and non-randomized eyes had visual acuity of better than 20/64 or were associated with patients refusing randomization. Visual fields were measured before treatment using the Humphrey Field Analyzer with the 24-2 program, foveal threshold, and size III stimulus. METHODS: We used visual fields from 189 non-IONDT eyes with NAION to develop the computerized classification system. Six neuro-ophthalmologists ("expert panel") described definitions for visual field patterns defects using 19 visual fields representing a range of pattern defect types. The expert panel then used 120 visual fields, classified using these definitions, to refine the rules, generating revised definitions for 13 visual field pattern defects and 3 levels of severity. These definitions were incorporated into a rule-based computerized classification system run on Excel(R) software. The computerized classification system was used to categorize visual field defects for an additional 95 NAION visual fields, and the expert panel was asked to independently classify the new fields and subsequently whether they agreed with the computer classification. To account for test variability over time, we derived an adjustment factor from the pooled short term fluctuation. We examined change in defects with and without adjustment in visual fields of study participants who demonstrated a visual acuity decrease within 30 days of NAION onset (progressive NAION). RESULTS: Despite an agreed upon set of rules, there was not good agreement among the expert panel when their independent visual classifications were compared. A majority did concur with the computer classification for 91 of 95 visual fields. Remaining classification discrepancies could not be resolved without modifying existing definitions. Without using the adjustment factor, visual fields of 63.6% (14/22) patients with progressive NAION and no central defect, and all (7/7) patients with a paracentral defect, worsened within 30 days of NAION onset. After applying the adjustment factor, the visual fields of the same patients with no initial central defect and 5/7 of the patients with a paracentral defect were seen to worsen. CONCLUSION: The IONDT developed a rule-based computerized system that consistently defines pattern and severity of visual fields of NAION patients for use in a research setting.

Wilbrand's knee: does it exist?

OBJECTIVE: In 1904, Hermann Wilbrand reported that nasal ON fibers form a loop into the contralateral ON, subsequently referred to as Wilbrand's knee. He had further theorized that a lesion affecting Wilbrand's knee would develop a distinct visual field defect characterized by an ipsilateral central scotoma with a contralateral superotemporal visual field defect. We present clinical evidence that disputes the existence of Wilbrand's knee. METHODS: A retrospective analysis of 3 patients whose ONs were divided at the ON-chiasm junction is presented. Two patients had an ONS meningioma and the other patient had sarcoidosis of the ON. Resection of the lesion and the ON up to the nerve-chiasm junction was done to prevent the disease from extending into the OC and the contralateral ON. The patients had detailed neuro-ophthalmologic evaluations preoperatively and postoperatively. RESULTS: After the resection of the ON at the ON-chiasm junction in the 3 patients, junctional scotoma could not be detected by visual perimetry. CONCLUSION: No clinical perimetric evidence was found to support the existence of Wilbrand's knee in the anterior visual pathway.

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.