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KMID : 1130620080040010045
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Journal of Clinical Neurology
2008 Volume.4 No. 1 p.45 ~ p.50
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A Case of Cerebral Erdheim-Chester Disease With Progressive Cerebellar Syndrome
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Na Sang-Jun
Kim Jung-Eun
Lee Kee-Ook
Kim Young-Duk
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Abstract
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Erdheim-Chester disease (ECD) is a rare non-Langerhans form of histiocytosis. Cerebellar involvement is rare
in this syndrome. We report a 37-year-old woman with slowly progressive cerebellar ataxia, dysmetria of limbs,
nystagmus, and dysarthria, bilateral painful axillary masses, and generalized arthralgia. Brain MRI revealed cerebellar
atrophy with focal lesions in the pons, middle cerebellar peduncle, and the cerebellum. She underwent incisional
biopsy of her axillary masses which showed findings consistent with ECD. An MRI of her lower extremities revealed
lesions in the diaphyses, metaphyses, and epiphyses of the proximal tibia and distal femur bilaterally. This is a rare
case of cerebral ECD with progressive cerebellar syndrome associated with cerebellar atrophy.
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KEYWORD
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Cerebral Erdheim-Chester disease, Cerebellar syndrome, Brain MRI
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