Gaze-Evoked Nystagmus & Slow Saccades Due to Anti-GAD Antibodies in a Patient with Stiff Person Syndrome

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Identifier Gaze-evoked_nystagmus_and_slow_saccades_due_to_anti-GAD_antibodies_in_a_patient_with_stiff_person_syndrome
Title Gaze-Evoked Nystagmus & Slow Saccades Due to Anti-GAD Antibodies in a Patient with Stiff Person Syndrome
Creator Daniel R. Gold, DO
Affiliation (DRG) Departments of Neurology, Ophthalmology, Neurosurgery, Otolaryngology - Head & Neck Surgery, Emergency Medicine, and Medicine, The Johns Hopkins School of Medicine, Baltimore, Maryland
Subject Jerk Nystagmus; Gaze-Evoked Nystagmus; Abnormal Saccades
Description This is a 70-year-old woman with a several year long history of imbalance and stiffness. Exam demonstrated axial and lower extremity stiffness, and ocular motor exam demonstrated gaze-evoked nystagmus (e.g., right-beating in right gaze, left-beating in left gaze, up-beating in up gaze), and mild to moderate slowing of saccades vertically and horizontally. Vestibular function was normal. Evaluation for a posterior fossa syndrome given gaze-evoked nystagmus (GEN, implies dysfunction of cerebellum or its connections) and slow saccades (implies impairment of brainstem burst neurons) demonstrated normal neuroimaging, although she had a very high level of anti-glutamic acid decarboxylase (GAD-65) serum IgG (hers was >300,000 IU/ml where the upper limit of normal is 5 IU/ml). The patient was diagnosed with probable stiff person syndrome, and ocular motor abnormalities were also attributed to the elevated anti-GAD-65 antibody levels. She has not yet received intravenous immunoglobulin. An anti-GAD syndrome with posterior fossa involvement should be considered in any patient with dizziness/vertigo and/or imbalance with central ocular motor signs including GEN and/or slow saccades (both have been reported). It is an important consideration, especially since it can be treated with immunotherapy and benzodiazepines (for stiffness and spasm).
Date 2020-04
Language eng
Format video/mp4
Type Image/MovingImage
Collection Neuro-Ophthalmology Virtual Education Library: Dan Gold Neuro-Ophthalmology Collection: https://novel.utah.edu/Gold/
Publisher North American Neuro-Ophthalmology Society
Holding Institution Spencer S. Eccles Health Sciences Library, University of Utah, 10 N 1900 E SLC, UT 84112-5890
Rights Management Copyright 2016. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright
ARK ark:/87278/s6jh8vwp
Setname ehsl_novel_gold
ID 1539416
Reference URL https://collections.lib.utah.edu/ark:/87278/s6jh8vwp
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