Cerebral Erdheim-Chester disease: report of two cases with progressive cerebellar syndrome with dentate abnormalities on magnetic resonance imaging.
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.
Department of Internal Medicine, Hôpital de la Pitié-Salpétrière, Paris, France.
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E
Pautas
P
P
Chérin
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S
Pelletier
M
M
Vidailhet
S
S
Herson
D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001706:Biopsy; D002524:Cerebellar Ataxia / Q000150:complications* / Q000188:drug therapy / Q000473:pathology*; D018891:Dentate Gyrus / Q000473:pathology*; D018450:Disease Progression; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications* / Q000188:drug therapy / Q000473:pathology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D011712:Pyramidal Tracts / Q000473:pathology; D012598:Sclerosis / Q000473:pathology; D013256:Steroids; D013977:Tibia / Q000473:pathology
D000893:Anti-Inflammatory Agents; D013256:Steroids