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Oculomasticatory Myorhythmia Shirley H. Wray, M.D., Ph.D. Professor of Neurology, Harvard Medical School Director, Unit for Neurovisual Disorders Massachusetts General Hospital
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Schwartz MA, Selhorst JB, Ochs AL, Beck RW, Campbell WW, Harris JK, Waters B, Velasco ME. Oculomasticatory myorhthmia: a unique movement disorder occurring in Whipple’s disease. Ann Neurol 20: 677-683, 1986.
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Oculomasticatory Myorythmia 1963 Van Bogaert et al. 1975 Knox et al. 1986 Jankovic J. 1986 Schwartz et al. 1987 Grotta et al. 1988 Hausser-Hauw et al. 1990 Adler and Galetta 1995 Simpson et al.
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Oculomasticatory Myorhythmia Unique pendular vergence oscillations Smooth rather than saccadic Peak velocities for various amplitudes typical of normal vergence movements Disjunctive, continuous, unaffected by saccadic effort, visual stimuli, or sleep PVOs are independently and uniquely generated within the vergence system Schwartz et al. Ann Neurol 20: 19, 677
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Pendular Vertical Oscillations PVOs are truly pendular and devoid of any rapid “jerk” phase. They differ from other forms of pendular nystagmus because Oscillations in z-axis (anteroposterior) rather than the x or y-axis Have greater amplitudes (5-25 degrees) Slower frequencies (0.5 – 1.5 v 2-4 Hz) Absence of palatal movement
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Pendular Vertical Oscillations Distinguished from the nystagmus of Parinaud’s syndrome, which is Episodic Provoked by vountary saccadic eye movements, especially attempted upgaze Has a high-velocity saccadic component
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Whipple’s Diagnosis Duodenal biopsy: PAS stain with diastase Non-intestinal tissues: electron microscopy Polymerase Chain Reaction: – Tissue, blood, and other bodily fluids In situ hybridization fluorescent rRNA probe Whipple’s bacillus “Tropheryma whippelii”
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Whipple’s Disease CNS Involvement 6-16% reported series Primary CNS < 5% –Progressive dementia –Myoclonus –Supranuclear ophthalmoplegia –Hypothalamic involvement –Obstruction of the aqueduct of Sylvius
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The causative organism Tropheryma whippelii is seen within macrophages in the parenchyma on PAS (peroidic acid-Schiff).
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The causative organism Tropheryma whippelii is seen within macrophages in the parenchyma on silver stains.
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References Whipple, GH (1907). A hitherto undescribed disease characterized anatomically by deposits of fat and fatty acids in the intestinal mesenteric lymphastic tissue. Bull. Johns Hopkins Hospital. 18:382. Sieracki, JC, et al. (1960). Central nervous system involvement in Whipple’s Disease. J. Neuropath. Exp. Neurol. 19:70.
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Lampert P, et al. (1962) Encephalopathy in Whipple’s Disease. Neurology 12:65. Badenoch, J, et al. (1963). Encephalopathy in acase of Whipple’s Disease. J. Neurol. Neurosurg. Psychiat. 26:203.
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Krucke, HW, Stochdorph, O. (1962). Uber veranderungen im Zentralnervensystem bei Whipple’ scher Krankheit. Verh. Dtsh. Ges. Pathol. 46:198. De Groodt-Lassell, M. and Martin, JJ. (1969). Etude ultra-structurale des lesions du systeme nerveus central dans la maladie de Whipple. Pathologie-Biologie. 17:121.
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Knox, D.I, et al. (1995). Cerebral ocular Whipple’s disease. Neurology. 45:617. See case 932-1
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www.library.med.utah.edu/NOVEL
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