Isolated CNS Whipple disease with a variant of oculofacial—skeletal myorhythmia (OFSM)
D. Ciampi de Andrade, MD,
R. C. Nogueira, MD,
L. T. Lucato, MD, PhD,
P. E. Marchiori, MD, PhD,
L. R. Machado, MD, PhD,
M. J. Teixeira, MD, PhD and
M. Scaff, MD, PhD
From the Departments of Neurology (D.C.d.A., R.C.N., P.E.M., L.R.M., M.J.T., M.S.) and Radiology (L.T.L.), University of São Paulo, Brazil.
Address correspondence and reprint requests to Dr. D. Ciampi de Andrade, R Dr James Ferraz Alvim 93 ap. 71, 05641-020 São Paulo SP Brazil ciampi{at}terra.com.br
A 62-year-old woman presented with insidious onset of depressivemood and progressive difficulties with daily activities. Shehad no diarrhea, abdominal cramps, arthralgia, weight loss,or palpable lymphadenopathy. Physical examination revealed fever,delirium, bilateral upper motor neuron signs, and myorhythmicmovements1 (see the video on the Neurology Web site [www.neurology.org]).Supranuclear ophthalmoplegia and cranial nerve involvement wereabsent. CSF showed a predominantly lymphocytic pleocytosis (85cells/mm3). MRI disclosed subcortical lesions (figure 1). Stereotacticbiopsy (figure 2) confirmed CNS Whipple disease. Ceftriaxone(4 g/day for 28 days), sulfamethoxazole-trimethoprim (320/1,600mg/day for 1 year),2 and monthly gamma-globulin (2 g/kg) werestarted. After 3 months the oculofacial-skeletal myorhythmia(OFSM) variant disappeared, the CSF normalized, and she wasafebrile and responded to verbal commands.
Axial fluid-attenuated inversion recovery image obtained at the level of the third ventricle discloses hyperintense lesions, bilateral and relatively symmetric, in thalami, lentiform nuclei, and caudate nuclei, without appreciable mass effect.
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