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  • Cerebral Erdheim-Chester disease: report of two cases with progressive cerebellar syndrome with dentate abnormalities on magnetic resonance imaging

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Cerebral Erdheim-Chester disease: report of two cases with progressive cerebellar syndrome with dentate abnormalities on magnetic resonance imaging
  1. Eric Pautasa,
  2. Patrick Chérina,
  3. Sophie Pelletiera,
  4. Marie Vidailhetb,
  5. Serge Hersona
  1. aDepartment of Internal Medecine, bDepartment of Neurology, Hôpital de la Pitié-Salpétrière, Paris, France
  1. 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.

Abstract

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.

  • Erdheim-Chester disease
  • cerebellar syndrome
  • pyramidal signs
  • magnetic resonance imaging

https://doi.org/10.1136/jnnp.65.4.597

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