Risk of Acute Ischemic Stroke in Patients With Monocular Vision Loss of Vascular Etiology.

BACKGROUND: To evaluate the risk of concurrent acute ischemic stroke and monocular vision loss (MVL) of vascular etiology. DESIGN: Retrospective, cross-sectional study. SUBJECTS: Patients aged 18 or older diagnosed with MVL of suspected or confirmed vascular etiology who had no other neurologic deficits and who received brain MRI within 7 days of onset of visual symptoms were included. METHODS: A medical record review was performed from 2013 to 2016 at Yale New Haven Hospital. Patients were included if vision loss was unilateral and due to transient monocular vision loss (TMVL), central retinal artery occlusion (CRAO), or branch retinal artery occlusion (BRAO). Any patients with neurologic deficits other than vision loss were excluded. Other exclusion criteria were positive visual phenomena, nonvascular intraocular pathology, and intracranial pathology other than ischemic stroke. MAIN OUTCOME MEASURES: The presence or absence of acute stroke on diffusion-weighted imaging (DWI) on brain MRI. RESULTS: A total of 641 records were reviewed, with 293 patients found to have MVL. After excluding those with focal neurologic deficits, there were 41 patients who met the inclusion criteria and received a brain MRI. Eight of the 41 subjects (19.5%) were found to have findings on brain MRI positive for acute cortical strokes. The proportion of lesion positive MRI was 1/23 (4.3%) in TMVL subjects, 4/12 (33.3%) in CRAO subjects, and 2/5 (40%) in BRAO subjects. Brain computed tomography (CT) scans were not able to identify the majority of acute stroke lesions in this study. CONCLUSIONS: Patients with MVL of vascular etiology such as TMVL, CRAO, or BRAO may have up to 19.5% risk of concurrent ischemic stroke, even when there are no other neurologic deficits. These strokes were detected acutely with brain MRI using DWI but were missed on CT.

Visual prognosis in compressive optic neuropathy secondary to sphenoid sinus mucocele: A systematic review.

Sphenoid sinus mucoceles (SSMs) are rare, benign lesions that can expand, often presenting with ocular symptoms-decreased vision, diplopia, visual field defects, proptosis, and external ophthalmoplegia. Reported cases are few, visual compromise varies, and factors affecting visual prognosis are poorly characterized. We investigate whether prompt surgical intervention (within 2 weeks of visual symptom onset) affects best-corrected visual acuity (BCVA) regained in patients with vision loss secondary to compressive SSM. We present a retrospective review of three cases and published literature to date. Our primary outcome was BCVA regained after surgical intervention; secondary outcomes included change in visual field defect and ophthalmological symptoms other than vision loss. Our three cases of SSM varied in onset, ranging from several hours to several months with patients aged from 13 to 80 years. All patients had severe vision loss to light perception (LP) or worse. Rapid neuro-imaging and urgent surgical intervention improved vision to count fingers at best. Of the two patients who underwent prompt decompression, one improved from no LP to LP and the other did not recover any vision. The patient who had visual loss for 3 months before intervention improved from LP to 20/400. Findings from our literature search, which yielded 12 cases of urgent intervention, supported the variability in visual prognosis despite prompt surgical intervention. SSMs are rare, pathologically benign lesions which can expand to cause ocular involvement. Prompt diagnosis and surgical decompression are recommended, but visual recovery may be limited even with urgent intervention.

Topiramate-induced palinopsia: a case series and review of the literature.

BACKGROUND: To report palinopsia as a possible side effect of topiramate. METHODS: Case series and review of the literature. RESULTS: Nine patients in our series, and 4 previously reported patients, who developed palinopsia while on topiramate, are reviewed. All patients were women, and comorbidities included migraine, idiopathic intracranial hypertension, and bulimia nervosa. Palinopsia resolved in 8 patients after stopping or decreasing the dose of topiramate. The lowest dose of topiramate causing palinopsia was 25 mg twice a day. More than half of our patients reported exacerbation of visual disturbance in early morning or late evening. CONCLUSIONS: Topiramate-induced palinopsia may be underdiagnosed because physicians do not inquire about such visual symptoms.

Antiphospholipid Antibody Syndrome: Raised Intracranial Pressure Without Cerebral Venous Sinus Thrombosis.

Antiphospholipid antibody syndrome (APS) has been reported to cause elevated intracranial pressure, but usually this is due to cerebral venous sinus thrombosis (CVST). We present a 36-year old man with APS with elevated intracranial pressure with neuro-ophthalmic, renal and hematological involvement without identifiable CVST.

Genetic Anticipation in Familial Neuromyelitis Optica: Case and Literature Review.

OBJECTIVES: To describe genetic anticipation in a mother and daughter with antiaquaporin 4 (AQP4) antibody-positive neuromyelitisoptica (NMO). METHODS: Retrospective case review. RESULTS: A woman with onset of transverse myelitis at age 38 was found to have a positive AQP4 antibody during work-up of recurrent symptoms. Subsequently, she developed intermittent episodes of monocular vision loss with optic nerve involvement that were treated with intravenous methylprednisolone and chronic rituximab. Eighteen years after initial presentation, her 78-year-old mother, with a history of recurrent urinary tract infections, also developed monocular vision loss and her anti-AQP4 antibody was positive. Previous reports of genetic anticipation in familial NMO are identified and discussed. CONCLUSIONS: These cases highlight genetic anticipation in familial NMO. Disease onset can occur with a chronological age difference of as much as 40 years between parent and child. Patients with NMO should be counseled regarding the possibility of subsequent disease onset in family members, particularly parents, with significant differences in calendar or chronological year of onset.

Ocular myasthenia gravis.

PURPOSE OF REVIEW: To review ocular myasthenia gravis (OMG), a localized form of myasthenia gravis clinically involving only the extraocular, levator palpebrae superioris, and orbicularis oculi muscles. RECENT FINDINGS: Ocular manifestations can masquerade as a variety of ocular motility disorders, including central nervous system disorders and peripheral cranial nerve palsies. While sparing the pupils, the diagnosis and management can be challenging. SUMMARY: Because several diagnostic and treatment options are available for OMG, clinicians must decide the sequence and combination based on the level of disease activity and patient disability.

Is intravitreal bevacizumab an effective treatment option for nonarteritic anterior ischemic optic neuropathy?

Nonarteritic anterior ischemic optic neuropathy (NAION) causes sudden profound loss of vision with no known cause or cure. Various treatment modalities, both surgical and pharmacologic, have been tried without success. The purpose of our retrospective study was to evaluate the effect of intravitreal bevacizumab (Avastin) as a treatment option for NAION. We evaluated demographics of 5 patients and compared visual acuity and automated visual fields prior to and following intravitreal bevacizumab injection. Visual acuity at presentation was 20/20 in 4 of 5 patients and 20/150 in 1. Visual acuity improved to 20/40 in the patient who presented with decreased acuity and decreased slowly in 3 patients and rapidly in 1. All patients presented with variable visual field defects: 1 improved slightly, 3 progressed, and 1 remained stable. One patient subsequently developed NAION in the fellow eye. These results are consistent with the natural course of the disease, and bevacizumab did not appear to have a dramatic effect on the clinical outcome in this small series of patients with NAION.

COVID-19 in Patients With Inflammatory Arthritis: A Prospective Study on the Effects of Comorbidities and Disease-Modifying Antirheumatic Drugs on Clinical Outcomes.

OBJECTIVE: To characterize the hospitalization and death rates among patients with inflammatory arthritis (IA) affected by coronavirus disease 2019 (COVID-19) and to analyze the associations of comorbidities and immunomodulatory medications with infection outcomes. METHODS: Data on clinical and demographic features, maintenance treatment, disease course, and outcomes in individuals with IA (rheumatoid arthritis and spondyloarthritis) with symptomatic COVID-19 infection were prospectively assessed via web-based questionnaire followed by individual phone calls and electronic medical record review. Baseline characteristics and medication use were summarized for hospitalized and ambulatory patients, and outcomes with the different medication classes were compared using multivariable logistic regression. RESULTS: A total of 103 patients with IA were included in the study (80 with confirmed COVID-19 and 23 with high suspicion of COVID-19). Hospitalization was required in 26% of the participants, and 4% died. Patients who were hospitalized were significantly more likely to be older (P < 0.001) and have comorbid hypertension (P = 0.001) and chronic obstructive pulmonary disease (P = 0.02). IA patients taking oral glucocorticoids had an increased likelihood of being admitted for COVID-19 (P < 0.001), while those receiving maintenance anticytokine biologic therapies did not. CONCLUSION: Among patients with underlying IA, COVID-19 outcomes were worse in those receiving glucocorticoids but not in patients receiving maintenance anticytokine therapy. Further work is needed to understand whether immunomodulatory therapies affect COVID-19 incidence.

Long-term efficacy of fractionated conformal radiotherapy for the management of primary optic nerve sheath meningioma.

BACKGROUND/AIM: Fractionated conformal radiotherapy (FCRT) is now used to treat vision-threatening optic nerve sheath meningioma (ONSM), but long-term efficacy and safety data are lacking; the purpose of this study was to assess these key data. METHODS: This is a retrospective chart review with prospective follow-up of adult patients treated with FCRT for primary ONSM at four academic medical centres between 1995 and 2007 with ≥10 years of follow-up after treatment. RESULTS: 16 patients were identified with a mean post-treatment follow-up of 14.6 years (range: 10.5-20.7 years). The mean age at symptom onset was 47.6 years (range: 36-60 years). FCRT was performed at a mean of 2.3 years after symptom onset (range: 0.2-14.0 years). At last follow-up, visual acuity had improved or stabilised in 14 of the 16 (88%) patients, and 11 (69%) had retained or achieved ≥20/40. The mean deviation on automated perimetry remained stable (-14.5 dB pretreatment vs -12.2 dB at last follow-up; p=0.68, n=10). Two (11%) patients had persistent pain, proptosis or diplopia, compared with six (38%) pretreatment (p=0.11). Two (13%) patients developed radiation retinopathy more than 6 months after completion of therapy, one (50%) of whom had worse visual acuity compared with pretreatment. No patient developed tumour involvement or radiation damage in the fellow eye. CONCLUSION: FCRT stabilises or improves visual function in patients with primary ONSM and is associated with a low risk of significant ocular sequelae. This treatment should be considered instead of surgery in patients with primary ONSM who require intervention due to loss of visual sensory and/or ocular motor function.

Neuro-ophthalmologic presentations of hemicrania continua.

PURPOSE: We describe a series of patients with hemicrania continua, a rare indomethacin-responsive primary headache syndrome, who presented for evaluation with neuro-ophthalmologic signs and symptoms. DESIGN: Observational case series. METHODS: Nine patients between the ages of 29 and 58 years were seen with various neuro-ophthalmologic findings and a unilateral continuous headache. A detailed history was taken from each patient, followed by a focused ophthalmologic and neurologic examination. The risks and benefits of treatment with indomethacin were discussed. Patients were instructed to call after several days of treatment to report any change in their headache and neuro-ophthalmologic symptoms, in addition to any adverse side effects. RESULTS: All patients responded favorably to indomethacin, with rapid near-complete or complete resolution of headache and autonomic symptoms, and treatment was initiated as early as possible. CONCLUSIONS: Because of its absolute response to indomethacin, recognizing the neuro-ophthalmologic symptoms of hemicrania continua as a component of the headache syndrome is critical for prompt initiation of treatment.

Progression of visual field defects in leber hereditary optic neuropathy: experience of the LHON treatment trial.

PURPOSE: To describe the visual fields of patients with Leber hereditary optic neuropathy (LHON), a maternally inherited disorder characterized by bilateral, often sequential vision loss, before and during progressive visual deterioration. DESIGN: Prospective longitudinal follow-up of serial visual fields in patients enrolled onto an open-label, nonrandomized pilot study of topical brimonidine purite as prophylactic treatment after first eye involvement in LHON. METHODS: Nine molecularly confirmed primary mutation patients with LHON with monocular vision loss for less than six months and normal visual function in the other eye were followed prospectively for up to two years. Visual fields were performed on automated perimetry at baseline and on many follow-up visits. RESULTS: Despite normal visual acuity at baseline in all patients, seven patients had some minimal changes in the central visual field of the second eye. All patients had subsequent deterioration of visual acuity, mean deviation, and foveal sensitivity in their second eye. The earliest pattern of abnormality was typically a cecocentral defect enlarging to become a central defect, often with a superior or inferior predilection. The visual field defects in the two eyes of any given patient were remarkably similar. CONCLUSIONS: LHON may be a bilateral condition at onset more frequently than appreciated. Automated static perimetry of the "normal" eye may reveal subclinical findings that typically worsen rapidly over weeks to months to similar central scotomatous damage. Quantitative automated static perimetry is helpful in elucidating the natural history of LHON and in understanding the underlying pathology and pathophysiology of this disease.

Prophylaxis for second eye involvement in leber hereditary optic neuropathy: an open-labeled, nonrandomized multicenter trial of topical brimonidine purite.

PURPOSE: To test a topical agent with purported antiapoptotic properties as prophylactic treatment after first eye involvement in Leber hereditary optic neuropathy (LHON), a maternally-inherited disorder characterized by bilateral, often sequential, visual loss. DESIGN: Open labeled, nonrandomized prospective pilot study. METHODS: Nine primary mutation molecularly confirmed LHON patients with one eye vision loss for less than 6 months and normal visual function in the fellow eye were treated with brimonidine purite 0.15% (Alphagan P) 4 times daily in the unaffected eye for up to 2 years. Visual acuity was the primary efficacy outcome. Secondary measures included changes on automated perimetry and quantification of the relative afferent pupillary defect. RESULTS: There were 8 men and 1 woman enrolled, aged 13 to 54 years (mean 32 years), eight with the 11778 mitochondrial DNA (mtDNA) mutation, and one with the 3460 mutation. Despite normal visual acuity at baseline in all patients, 7 patients had some minimal changes in the central visual field of the study eye. All patients had deterioration of vision in their second eye. In 1 of the 2 patients who had treatment initiated within 16 days after first eye involvement, good visual acuity was maintained in the study eye at 15 month followup, despite a mildly abnormal study eye baseline visual field. CONCLUSIONS: LHON may be a bilateral condition at onset more frequently than appreciated, with asymmetric severity at presentation. Topical brimonidine purite in this dosage was unsuccessful in preventing second eye involvement in recently monocularly-symptomatic LHON.

Leopard-spot pattern of yellowish subretinal deposits in central serous chorioretinopathy.

OBJECTIVE: To describe clinical and angiographic features of patients with central serous chorioretinopathy (CSC) who had yellowish subretinal deposits forming a reticulated leopard-spot pattern during fluorescein angiography. METHODS: We conducted case studies using the clinical and photographic records of 5 patients. RESULTS: All 5 patients were older men between the ages of 68 and 81 years who had been treated with corticosteroids and had bilateral CSC. Nine eyes of the 5 patients developed yellowish deposits in a reticulated pattern in the macular region under the chronic detached neurosensory retina. The pattern of leopard-spot deposits was well demonstrated on the fluorescein angiogram, with hypofluorescence in most of the deposits and hyperfluorescence from atrophy of the retinal pigment epithelium. Later phases of the fluorescein angiographic study showed leaks from the retinal pigment epithelium. During the indocyanine green angiography evaluation of 4 patients, all had bilateral multifocal patches of hyperfluorescence in the midphase, findings typical of CSC. CONCLUSIONS: Yellowish deposits forming a reticulated leopard-spot pattern may occur under the neurosensory retina and are associated with chronic neurosensory detachment caused by CSC. All patients were older men being treated with corticosteroids. This report described a newly recognized finding: the subretinal deposition of a yellowish material in a leopard-spot pattern in eyes with CSC.

The effect of prednisone on the progression from ocular to generalized myasthenia gravis.

Fifty percent of ocular myasthenia gravis (OMG) patients will progress to generalized myasthenia, 90% within 3 years from the onset of ocular symptoms. This study was performed to determine whether treatment with oral prednisone initiated and completed within 2 years from the onset of ocular symptoms would affect the progression of ocular myasthenia to generalized myasthenia gravis (GMG). Fifty-six patients were included in this review, with 27 patients in the prednisone-treated group and 29 patients in the untreated group. The treated group was initiated on 60 mg of prednisone daily with a slow taper over 3-6 months. At 2 years, significantly fewer patients in the treated group (3 of 27) progressed to generalized myasthenia when compared to the untreated group (10 of 29) (chi(2), p=0.04). Our results suggest that the early use of steroids may decrease progression of ocular to generalized myasthenia gravis. The decision to use steroids should be considered early in the course of patients diagnosed with ocular myasthenia gravis. This study should be considered preliminary and a prospective trial is warranted to confirm our observations.

Periocular necrotizing fasciitis causing blindness.

IMPORTANCE: Periocular necrotizing fasciitis is a rare but potentially devastating disease, accompanied by high rates of morbidity and mortality. OBSERVATIONS: We report 5 cases of periocular necrotizing fasciitis resulting in severe vision loss, 3 of which required exenteration to contain the disease and only 1 of which recovered vision. Three cases were caused by group A streptococcus; 1, by methicillin-resistant Staphylococcus aureus; and 1, by Streptococcus anginosus constellatus. CONCLUSIONS AND RELEVANCE: Providers should maintain a high clinical suspicion for necrotizing fasciitis and distinguish it from more common forms of cellulitis. As seen in these 5 cases, periocular necrotizing fasciitis may cause severe visual loss more often than previously recognized. To our knowledge, this is also the first report of Streptococcus anginosus constellatus causing necrotizing fasciitis.