CEREBRAL ERDHEIM-CHESTER DISEASE - REPORT OF 2 CASES WITH PROGRESSIVECEREBELLAR SYNDROME WITH DENTATE ABNORMALITIES ON MAGNETIC-RESONANCE-IMAGING

Authors
PAUTAS E CHERIN P PELLETIER S VIDAILHET M HERSON S
Citation
E. Pautas et al., CEREBRAL ERDHEIM-CHESTER DISEASE - REPORT OF 2 CASES WITH PROGRESSIVECEREBELLAR SYNDROME WITH DENTATE ABNORMALITIES ON MAGNETIC-RESONANCE-IMAGING, Journal of Neurology, Neurosurgery and Psychiatry, 65(4), 1998, pp. 597-599
Citations number
10
Categorie Soggetti
Psychiatry,"Clinical Neurology",Surgery
Journal title
Journal of Neurology, Neurosurgery and Psychiatry → ACNP
ISSN journal
00223050
Volume
65
Issue
4
Year of publication
1998
Pages
597 - 599
Database
ISI
SICI code
0022-3050(1998)65:4<597:CED-RO>2.0.ZU;2-2
Abstract
Two patients with Erdheim-Chester disease with progressive cerebellar dysfunction and pyramidal signs are reported on. Cerebral MRI showed b ilateral increased signal intensity in peridentatal regions on T2 weig hted sequences. Both patients had kidney and bone involvement, establi shed on bone biopsy for one. One patient improved with steroid therapy . This contrasts with previous reports, which describe rare neurologic al manifestations and the failure of different therapeutic approaches.