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Oculo-Facial-Skeletal Myorhythmia in Whipple Disease: Treatment with Ceftriaxone

Charles H. Adler, MD, PhD; and Steven L. Galetta, MD
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Requests for Reprints: Steven L. Galetta, MD, Department of Neurology, Hospital of the University of Pennsylvania, 3400 Spruce Street, Philadelphia, PA 19104-4283.

Current Author Addresses: Drs. Adler and Galetta: Department of Neurology, Hospital of the University of Pennsylvania, 3400 Spruce Street, Philadelphia, PA 19104-4283.

Ann Intern Med. 1990;112(6):467-469. doi:10.7326/0003-4819-76-3-112-6-467
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Whipple disease is a chronic, systemic disorder resulting in steatorrhea, weight loss, arthralgias, fever, and neurologic symptoms (1). Central nervous system manifestations may occur without systemic involvement (2-4), and include headache, seizures, meningitis, myoclonus, dementia, and supranuclear ophthalmoplegia (1-4). A unique, possibly pathognomonic, movement disorder, involving a slow (1 Hz) convergent-divergent pendular nystagmus, associated with synchronous, rhythmic movements of the mouth and other body parts, has been called oculomasticatory or oculo-facial-skeletal myorhythmia. It has previously been reported in six patients with biopsy-proven Whipple disease (5-9). We report a patient with Whipple disease with amnesia, meningitis, supranuclear ophthalmoparesis, and oculo-facial-skeletal myorhythmia


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