September 4, 2013 E-Pearl: Watching for Whipple’s: Oculomasticatory Myorhythmia
September 4, 2013
Central nervous system involvement in Whipple's disease occurs in approximately 6-43% of patients (1). Symptoms include progressive dementia, hypothalamic dysfunction, myoclonus, and ocular abnormalities, classically a supranuclear vertical opthalmoplegia. Oculomasticatory myorhythmia is a unique movement disorder that is pathognomonic of Whipple's disease. Patients exhibit continuous smooth pendular convergent and divergent oscillations of the eyes at primary position, with saccades, with fixation, and even during sleep. Concurrent synchronous contraction of masticatory muscles (e.g. masseter, genioglossus) but not palatal muscle characterizes this condition (2). Diagnosis is confirmed by duodenal biopsy or polymerase chain reaction demonstrating the presence of tropheryma whippelii, the causative organism.
- Louis ED, Lynch T, Kaufmann P, et al. Diagnostic guidelines in central nervous system Whipple's disease. Ann Neurol 1996; 40: 561-568.
- Revilla FJ, de la Cruz R, Khardori N, Espay AJ. Teaching NeuroImage: Oculomasticatory myorhythmia: pathognomonic phenomenology of Whipple disease. Neurology 2008; 70: e25.
Submitted by Roy Strowd, M.D. Resident Physician, Wake Forest School of Medicine, Winston Salem, NC.
Dr. Strowd is a member of the Resident and Fellow Section of Neurology.