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Identifier Wray_Case042-1_PPT
Title Oculomasticatory_Myorhythmia
Subject Somnolence; Supranuclear Paralysis of Up and Downgaze; Pendular Vergence Oscillations; Oculomasticatory Myorhythmia; Tropheryma Whippelii - Infection; CNS Whipple's Disease; Supranuclear Paralysis of Up and Downgaze Infection-Whipple's Disease; Oculomasticatory Myorhythmia (Whipple's)
Creator Shirley H. Wray, M.D., Ph.D., FRCP, Professor of Neurology Harvard Medical School, Director, Unit for Neurovisual Disorders, Massachusetts General Hospital
Description This case, previously reported in 1986, is published courtesy of John Selhorst, M.D., Saint Louis University School of Medicine, St. Louis, MO. (4) The patient is a 46 year old man who, over a period of six months, lost the ability to read and complained of excessive somnolence, occasional urinary incontinence, and recent 6-kg weight loss. A disabling, seronegative, non-deforming migratory arthritis had developed three years earlier. Gastrointestinal symptoms and fever had not occurred. Family History: Negative for neurological or arthritic disease. Neuro-ophthalmological examination: Visual acuity 20/50 right eye, 20/100 left eye Mild exposure keratitis, No uveitis by biomicroscopy Normal pupil reflexes Normal fundus exam Eye movements: 1.Mild bilateral ptosis 2.1 Hz smooth rhythmic convergence and divergence oscillations in primary gaze 3.Slow hypometric horizontal volitional saccades slightly restricted. 4.Rotation-induced horizontal eye movements full, compensatory saccades absent. 5.Supranuclear paralysis of vertical gaze 6.Voluntary vergence movements were disrupted by the continuous oscillations. Additional signs: Rhythmic elevation and depression movements of the mandible due to concurrent contractions of the masticatory muscles. The rhythmic jaw movements were synchronous with the ocular oscillations. Both the eye and jaw movements were continuous during the day, persistent in sleep, and unaltered by environmental stimuli. Palatal myoclonus was never observed. Hematological Studies: Sedimentation rate 87 mm/per hour Circulating immune complexes Raji cell, C1-Q binding markedly elevated. Serum immunoelectrophoresis mildly increased IgA and IgG, no monoclonal peaks Lumbar Puncture: Cerebrospinal fluid 1 WBC, Protein 41mg/dl, Glucose 49 mg/dl, IgG/albumin ratio 0.40 (normal <0.25) EEG: Normal. Visual, auditory and somatosensory evoked potentials: normal. Brain CT: Normal MRI and Angiograpy: normal. Diagnosis: Dr. Selhorst made a diagnosis of oculomasticatory myorhythmia (OMM) and suspected Whipple's disease. Intestinal Biopsy: showed normal duodenum, a site often not involved in Whipple's disease. A search of the literature, however, brought to Dr. Selhorst's attention DeJonghe's report in 1979 of a man with Whipple's disease and cerebral symptoms. DeJonghe gave little attention to the movement disorder, but close review of the original report of DeJonghe's patient by Van Bogaert some 16 years earlier revealed an eye movement disorder consistent with OMM. Pathology Slides: Dr. Selhorst prevailed upon the GI attending to obtain a fluoroscopically guided biopsy of the duodenal-jejunal junction that proved positive for Tropheryma whippelii (T. whippelii) Whipple's disease. Figures 1 and 2 are sections from a biopsy of the duodenal jejunal junction stained with periodic acid Schiff (PAS) that show well-preserved intestinal villi with dilated lymphatics (large empty areas) and PAS positive staining of fat globules in the mucosa and more importantly bacterium digested within submucosal macrophages and the Tropheryma whippelii bacterium digested within the macrophage. Figure 3A shows a PAS-stained multinucleated giant cell (open arrow) and macrophages (dark arrow). Figure 3B is an electron microscopic image of intracellular bacilli in longitudinal section (closed arrows) and in cross-section (open arrows)). Treatment: The patient was treated intravenous penicillin (2 million units every 4 hours) for 14 days and simultaneously with oral trimetho- prim/sulfamethoxazole (TMP/SMX) (160 mg/800 mg., 3 x daily) for seven weeks. Trials of oral trihexyphenidyl (ranging to 16 mg daily), oral clonazepam (4 mg daily), and oral prednisone (60 mg daily) were ineffective in reducing the constant movements and deteriorating status. Prednisone had no effect on the persistent arthralgias. TMP/SMX was discontinued after two months, and oral tetracycline was begun at a dose of 250 mg. 4x daily. The patient's mentation began to improve several days after beginning tetracycline but the movement disorder persisted. He was discharged after 125 days of hospitalization on tetracycline and followed up over the next six months. Over this time there was a decrease in the amplitude of the eye and jaw movements and recovery of 10 degrees of vertical gaze. His previous lethargy and insomnia left him and mental function improved further. The arthralgias remitted completely. Follow-up ten years later found the man enjoying good health with a minimal amount of OMM. See also: http://content.lib.utah.edu/cdm/ref/collection/ehsl-shw/id/283
Publisher Spencer S. Eccles Health Sciences Library, University of Utah
Type Text; Image
Format application/vnd.ms-powerpoint
Format Creation Created in Microsoft PowerPoint
Relation is Part of 042-1
Rights Management Copyright 2002. 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/s6j13brz
Setname ehsl_novel_novel
Date Created 2012-07-13
Date Modified 2015-09-02
ID 186800
Reference URL https://collections.lib.utah.edu/ark:/87278/s6j13brz
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