Oculomasticatory Myorhythmia 42-1. Schwartz MA, Selhorst JB, Ochs AL, Beck RW, Campbell WW, Harris...
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Transcript of Oculomasticatory Myorhythmia 42-1. Schwartz MA, Selhorst JB, Ochs AL, Beck RW, Campbell WW, Harris...
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Oculomasticatory Myorhythmia42-1
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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.
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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 Galetta1995 Simpson et al.
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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 voluntary saccadic eye movements, especially attempted
upgaze
Has a high-velocity saccadic component
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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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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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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
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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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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
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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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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.
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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.
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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.
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www.library.med.utah.edu/NOVEL