Oculomasticatory Myorhythmia42-1

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

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.

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.

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