One and a Half Syndrome

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Identifier Wray_Case937-8_PPT
Title One and a Half Syndrome
Creator Shirley H. Wray, MD, PhD, FRCP
Affiliation Professor of Neurology Harvard Medical School, Director, Unit for Neurovisual Disorders, Massachusetts General Hospital
Subject Unilateral Internuclear Ophthalmoplegia; Unilateral Horizontal Gaze Palsy; Upbeat Nystagmus on Upgaze; Convergence Normal; Fisher's One-and-a-Half Syndrome; Uhthoff's Symptom; Multiple Sclerosis; Unilateral Horizontal Gaze Palsy Demyelination; Gaze Evoked Upbeat Nystagmus; Abducting Nystagmus
Description This 44 year old woman presented in 1973 with an acute attack of optic neuritis in the right eye that fully recovered after a course of ACTH therapy. In 1991, 18 years later, she developed unsteadiness of gait, "walking like a chicken", stiff legs that jerked spontaneously in bed at night, and numbness in the legs distal to the knees. The left leg was more affected than the right. She had urinary frequency, urgency and occasional incontinence. She denied any symptoms in the upper extremities. She had a negative L'Hermitte symptom. In hot weather her legs became weak and fatigue increased (Uhthoff's symptom). Past History: Negative for additional neurological symptoms Family History: Negative for CNS disease. Neuro-ophthalmological examination: Visual acuity OD: 20/25, OS: 20/20 Right afferent pupil defect Mild dyschromatopsia OD Fundus exam showed optic atrophy OD. Normal optic disc OS Ocular Motility A left horizontal gaze palsy with gaze evoked nystagmus A left internuclear ophthalmoplegia on gaze right with adduction weakness OS Abducting nystagmus OD Full vertical gaze Upbeat nystagmus on upgaze (ill sustained) Convergence normal Neurological examination: Titubation Spastic paraparesis, with spastic left foot drop Marked hyperreflexia, bilateral ankle clonus and extensor plantar responses. Ataxia on finger-nose and heel-knee-shin Broad base spastic ataxic gait. Spinal tap: Cerebrospinal fluid protein 51 mg/dl, sugar 66 mg/dl, 4 white blood cells, elevated IgG and positive oligoclonal bands. VDRL negative. Brain MRI: MRI of the brain with gadolinium showed multiple white matter lesions in the central and periventricular white matter. The largest of these lesions was approximately 9 mm in diameter and ring enhancing, reflecting the presence of both acute and chronic demyelinating lesions consistent with a diagnosis of MS. MRI study of the cervical spine showed cervical spondylosis with disc herniation at C5-6 and C6-7 lateralizing to the right and compressing the spinal cord. A neurosurgeon recommended conservative management. Diagnosis: Multiple Sclerosis (MS) Treatment: The patient received high dose intravenous methylprednisolone 1,000 mg in 1,000 cc of dextrose saline daily for 5 days, then tapered to a dose of 500 mg. in 500 cc of dextrose saline daily for 2 days. Only the patient's gait improved on treatment. See also: http://content.lib.utah.edu/cdm/ref/collection/ehsl-shw/id/346
Date 2002
Language eng
Format application/pdf
Format Creation Microsoft PowerPoint
Type Text
Relation is Part of 937-8
Collection Neuro-Ophthalmology Virtual Education Library: NOVEL https://NOVEL.utah.edu
Publisher North American Neuro-Ophthalmology Society
Holding Institution Spencer S. Eccles Health Sciences Library, University of Utah
Rights Management Copyright 2002. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright
ARK ark:/87278/s6ms72b0
Setname ehsl_novel_novel
ID 186807
Reference URL https://collections.lib.utah.edu/ark:/87278/s6ms72b0
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