Oculomasticatory Myorhythmia 42-1. Schwartz MA, Selhorst JB, Ochs AL, Beck RW, Campbell WW, Harris JK, Waters B, Velasco ME. Oculomasticatory myorhythmia:

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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: , 1986.

Oculomasticatory Myorythmia 1963 Van Bogaert et al Knox et al Jankovic J Schwartz et al Grotta et al Hausser-Hauw et al 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 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”

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.