Visual loss due to optic neuropathy is a rare manifestation of thiamine deficiency. We report a case of a 39-year-old woman with a body mass index (BMI) of 29 kg/m2 who developed visual loss and bilateral optic nerve head swelling after a short, self-limited gastrointestinal illness. She was disoriented and inattentive and had absent ankle jerk reflexes, diminished sensation in both legs below the knees, and marked truncal ataxia. Magnetic resonance imaging (MRI) showed increased T2-signal in the medial thalami and mammillary bodies. The serum thiamine level was 8 nmol/L (normal 8–30). The diagnosis of thiamine deficiency was made, and the patient’s vision and neurologic symptoms improved significantly with intramuscular thiamine treatment. Thiamine deficiency can occur in the absence of an obvious predisposing factor such as alcoholism or low body weight. The clinician must be aware of the factors that govern vitamin availability and maintain a high index of suspicion to make the diagnosis in such cases.
Background: In recent years, CTLA-4 and PD-1/PD-L1 checkpoint inhibitors have proven to be effective and have become increasingly popular treatment options for metastatic melanoma and other cancers. These agents work by enhancing autologous antitumor immune responses. Immune-related ophthalmologic complications have been reported in association with checkpoint inhibitor use but remain incompletely characterized. This study seeks to investigate and further characterize the neuro-ophthalmic and ocular complications of immune checkpoint blockade treatment. Methods: A survey was distributed through the secure electronic data collection tool REDCap to neuroophthalmology specialists in the North American Neuro-Ophthalmology Society listserv. The study received human subjects approval through the University of California at Los Angeles Institutional Review Board. The survey identified patients sent for neuro-ophthalmic consultation while receiving one or more of a PD-1 inhibitor (pembrolizumab, nivolumab, or cemiplimab); PD-L1 inhibitor (atezolizumab, avelumab, or durvalumab); or the CTLA-4 inhibitor ipilimumab. Thirty-one patients from 14 institutions were identified. Patient demographics, neuro-ophthalmic diagnosis, diagnostic testing, severity, treatment, clinical response, checkpoint inhibitor drug used, and cancer diagnosis was obtained. Results: The checkpoint inhibitors used in these patients included pembrolizumab (12/31), nivolumab (6/31), combined ipilimumab with nivolumab (7/31, one of whom also received pembrolizumab during their course of treatment), durvalumab (3/31), ipilimumab (2/31), and cemiplimab (1/ 31). Malignant melanoma (16/31) or nonsmall cell lung carcinoma (6/31) were the most common malignancies. The median time between first drug administration and the time of ophthalmological symptom onset was 14.5 weeks. Eleven
Background: Idiopathic intracranial hypertension is a rare neurologic condition characterized by elevated intracranial pressure with normal cerebrospinal fluid analysis and neuroimaging. A subset of pediatric idiopathic intracranial hypertension patients are coincidentally found to have papilledema and elevated intracranial pressure without symptoms (eg, headache, visual blurring, tinnitus). This study aims to investigate the features of asymptomatic pediatric idiopathic intracranial hypertension. Methods: Retrospective case-control study of patients aged 0 to 18 years who received idiopathic intracranial hypertension diagnosis from 2005 to 2016. Subjects were included if they met established diagnostic criteria for idiopathic intracranial hypertension diagnosis. Subjects were classified as symptomatic if they presented with 1 symptom related to elevated intracranial pressure, and asymptomatic if no symptoms were present. Statistical analysis was performed to compare the 2 groups. Results: 12 (22.6%) of 53 pediatric idiopathic intracranial hypertension subjects were asymptomatic. Compared to symptomatic idiopathic intracranial hypertension, asymptomatic idiopathic intracranial hypertension had younger age of onset, lower initial opening pressure on lumbar puncture, lower optic nerve edema grades bilaterally, lower likelihood of globe flattening on magnetic resonance imaging (MRI), and smaller required dose of acetazolamide for resolution of papilledema (all P < .05). Conclusion: Asymptomatic idiopathic intracranial hypertension is common among pediatric patients with papilledema and is an important disease entity that requires special clinical management. It may exist as a milder version of idiopathic intracranial hypertension that occurs in younger children, or as a precursor state that later evolves into symptomatic disease.
Several oral β-blockers are US Food and Drug Administrationapproved for migraine prophylaxis, but their gradual absorption and modification by first-pass metabolism delays effective plasma levels for hours to days, limiting their use in acute migraines. 1 Timolol eyedrops provide a rapid route of delivery with the maximum plasma concentration achieved within 15 minutes of administration. 2,3 This pharmacokinetic advantage supports a potential role for timolol eyedrops in managing acute migraine. 4,5 Methods | We conducted a randomized, crossover, placebocontrolled pilot study of timolol eyedrops as an abortive migraine treatment in adults. The study was approved by the institutional review board of the University of Missouri-Kansas City (Trial Protocol in the Supplement). Participants were recruited via our neurology and ophthalmology clinics, a listing on clinicaltrials.gov (NCT02630719), and flyers. Participants provided written consent.Men and women who were at least age 18 years and met the International Headache Society criteria for migraine with or without aura 6 were eligible for the study. All patients underwent a screening, which included complete neurological and ophthalmological examinations and a pregnancy test for Conclusions | Timolol is an effective abortive treatment for some patients with migraines. Future research should focus on identifying which patients will respond and at what dosage.
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