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J Neurol Neurosurg Psychiatry 1998;65:597-599 ( October )

Short report

Cerebral Erdheim-Chester disease: report of two cases with progressive cerebellar syndrome with dentate abnormalities on magnetic resonance imaging Eric Pautas,a Patrick Chérin,a Sophie Pelletier,a Marie Vidailhet,b Serge Hersona

a Department of Internal Medecine, b Department of Neurology, Hôpital de la Pitié-Salpétrière, Paris, France

Correspondence to: Dr Chérin, Service de Médecine Interne, Hôpital de la Salpétrière, 47 bd de l'Hôpital, 75856 Paris Cedex, France. Fax 0033 1 42 16 10 58.

Received 4 July 1998 and in revised form 11 February 1998; Accepted 9 March 1998

Two patients with Erdheim-Chester disease with progressive cerebellar dysfunction and pyramidal signs are reported on. Cerebral MRI showed bilateral increased signal intensity in peridentatal regions on T2 weighted sequences. Both patients had kidney and bone involvement, established on bone biopsy for one. One patient improved with steroid therapy. This contrasts with previous reports, which describe rare neurological manifestations and the failure of different therapeutic approaches.

Keywords: Erdheim-Chester disease; cerebellar syndrome; pyramidal signs; magnetic resonance imaging

© 1998 by Journal of Neurology, Neurosurgery, and Psychiatry


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