NEUROLOGY 2007;69:E12
© 2007 American Academy of Neurology
Resident and Fellow Section
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 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/mm3). MRI disclosed subcortical lesions (figure 1). Stereotactic biopsy (figure 2) confirmed CNS Whipple 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.
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Figure 1 Axial fluid-attenuated inversion recovery MRI
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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Figure 2 Biopsy specimen
Right thalamus biopsy specimen stained by periodic acid-Schiff (PAS) showing PAS-positive bacilli inside histiocytes in the space of Virchow (arrows).
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Disclosure: The authors report no conflicts of interest.
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REFERENCES
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- Lynch T, Fahn S, Louis ED, Odel JG. Oculofacial-skeletal myorhythmia in Whipple's disease. Mov disord 1997;12:625–626.[Medline]
- Durand DV, Lecomte C, Cathebras P, et al. Whipple disease: clinical review of 52 cases. Medicine 1997;76:170–184.[Medline]
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