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Oculomasticatory Myorhythmia. 42-1. Schwartz MA, Selhorst JB, Ochs AL, Beck RW, Campbell WW, Harris JK, Waters B, Velasco ME. Oculomasticatory myorhythmia: a unique movement disorder occurring in Whipple’s disease. Ann Neurol 20: 677-683, 1986. Oculomasticatory Myorythmia.
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Schwartz MA, Selhorst JB, Ochs AL, Beck RW, Campbell WW, Harris JK, Waters B, Velasco ME. Oculomasticatory myorhythmia: a unique movement disorder occurring in Whipple’s disease. Ann Neurol 20: 677-683, 1986.
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.
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
Pendular Vertical Oscillations Distinguished from the nystagmus of Parinaud’s syndrome, which is Episodic Provoked by voluntary saccadic eye movements, especially attempted upgaze Has a high-velocity saccadic component
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
Whipple’s Diagnosis Whipple’s bacillus “Tropheryma whippelii” 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
Brain Biopsy #2 Open biopsy: wall of third ventricle “Perivascular and parenchymal infiltration with foamy macrophages with stained +ve for PAS.” Brain tissue and small bowel biopsy insufficient for PCR studies
The causative organism Tropheryma whippelii is seen within macrophages in the parenchyma on PAS (peroidic acid-Schiff).
The causative organism Tropheryma whippelii is seen within macrophages in the parenchyma on silver stains.
Treatment Ceftriaxone 2g IV bid or PCN G procaine 1.2 mU IM qd + Streptomycin 1g IM qd for 2 weeks then Trimethoprim-sulfamethoxazole 160/800 mg po bid 1x year
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
References Whipple, GH (1907). A hitherto undescribed disease characterized anatomically by deposits of fat and fatty acids in the intestinal mesenteric lymphatic 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.
Lampert P, et al. (1962) Encephalopathy in Whipple’s Disease. Neurology 12:65. Badenoch, J, et al. (1963). Encephalopathy in a case of Whipple’s Disease. J. Neurol. Neurosurg. Psychiat. 26:203.
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.
Knox, D.I, et al. (1995). Cerebral ocular Whipple’s disease. Neurology. 45:617.
