Isolated CNS Whipple disease with a variant of oculofacial-skeletal myorhythmia (OFSM).

A 62-year-old woman presented with insidious onset of depressive mood and progressive difficulties with daily activities. She had no diarrhea, abdominal cramps, arthralgia, weight loss, or palpable lymphadenopathy. Physical examination revealed fever, delirium, bilateral upper motor neuron signs, and myorhythmic movements1 (see the video on the Neurology Web site [www. neurology.org]). Supranuclear ophthalmoplegia and cranial nerve involvement were absent. CSF showed a predominantly lymphocytic pleocytosis (85 cells/mm). MRI disclosed subcortical lesions (figure 1). Stereotactic biopsy (figure 2) confirmed CNSWhipple disease. Ceftriaxone (4 g/day for 28 days), sulfamethoxazole-trimethoprim (320/1,600 mg/day for 1 year),2 and monthly gamma-globulin (2 g/kg) were started. After 3 months the oculofacial–skeletal myorhythmia (OFSM) variant disappeared, the CSF normalized, and she was afebrile and responded to verbal commands.