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
Subject Jerk Nystagmus; Gaze-Evoked Nystagmus; Abnormal Saccades
Creator Daniel R. Gold, DO, Departments of Neurology, Ophthalmology, Neurosurgery, Otolaryngology - Head & Neck Surgery, Emergency Medicine, and Medicine, The Johns Hopkins School of Medicine
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).
Publisher Spencer S. Eccles Health Sciences Library, University of Utah
Date 2020-04
Type Image/MovingImage
Format video/mp4
Relation is Part of NOVEL: Neuro-ophthalmology Virtual Education Library Examination Collection
Rights Management Copyright 2020. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright
Holding Institution Spencer S. Eccles Health Sciences Library, University of Utah, 10 N 1900 E, SLC, UT 84112-5890
Collection Neuro-ophthalmology Virtual Education Library: NOVEL http://NOVEL.utah.edu
Language eng
ARK ark:/87278/s6jh8vwp
Setname ehsl_novel_gold
Date Created 2020-04-13
Date Modified 2021-06-29
ID 1539416
Reference URL https://collections.lib.utah.edu/ark:/87278/s6jh8vwp
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