Oculomasticatory Myorhythmia

Q: What is Oculomasticatory Myorhythmia and signifies which disease?

 Answer: It is a continuous rhythmic movement of eye convergence with concurrent contractions of the masticatory or other muscles. This signifies the central nervous system (CNS) involvement in Whipple's disease. 

It is usually associated with cognitive abnormalities. If there is an involvement of skeletal muscles, it is called oculofacial-skeletal myorhythmia. The cause is not fully understood for this symptom. If present, eyes oscillate synchronously horizontally towards and away from each other. Other muscles involved can be any/some/all of masticatory, lids, tongue, palate, arms, and legs. Bilateral ptosis may be present. Interestingly, this is found to be associated with hypersomnolence. To make things complicated in isolated CNS involvement, PAS-positive cells may be negative on intestinal biopsy. 

Treatment for CNS Whipple's disease is not established though tetracycline, ceftriaxone, trimethoprim-sulfamethoxazole, pefloxacin, and valproate has been described.

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References:

1. Adler CH, Galetta SL. Oculo-facial-skeletal myorhythmia in Whipple disease: treatment with ceftriaxone. Ann Intern Med 1990; 112:467. 

2. Simpson DA, Wishnow R, Gargulinski RB, Pawlak AM. Oculofacial-skeletal myorhythmia in central nervous system Whipple's disease: additional case and review of the literature. Mov Disord 1995; 10:195. 

3. Hausser-Hauw C, Roullet E, Robert R, Marteau R. Oculo-facio-skeletal myorhythmia as a cerebral complication of systemic Whipple's disease. Mov Disord 1988; 3:179. 

4. Rajput AH, McHattie JD. Ophthalmoplegia and leg myorhythmia in Whipple's disease: report of a case. Mov Disord 1997; 12:111. 

5. Amarenco P, Roullet E, Hannoun L, Marteau R. Progressive supranuclear palsy as the sole manifestation of systemic Whipple's disease treated with pefloxacine. J Neurol Neurosurg Psychiatry 1991; 54:1121. 

6. Panegyres PK, Goh J. Sleep disorders of Whipple's disease of the brain. QJM 2015; 108:99. 

7. Calandra-Buonaura G, Provini F, Guaraldi P, et al. Oculomasticatory myorhythmia and agrypnia excitata guide the diagnosis of Whipple disease. Sleep Med 2013; 14:1428.

8.Adler CH, Galetta SL. Oculo-facial-skeletal myorhythmia in Whipple disease: treatment with ceftriaxone. Ann Intern Med 1990; 112:467.