[Cerebral Erdheim-Chester disease].
We report the case of a 26-old-year man hospitalized for first partial complex epileptic seizure. Brain MRI showed an asymptomatic pseudo-tumor lesion in the brainstem. Diabetes insipidus, hypophyseal gonadotropic deficiency and osteosclerosis of long bones strongly suggested Erdheim-Chester disease, a rare histiocytosis, confirmed after tibial biopsy. Six months later, the patient remained stable. A persistent, and even increased, enhancement with Gd-DTPA on brain MR images was noted as previously described. The review of the literature collected 64 cases, and only 7 cases of cerebral "tumor".
Service de Neurologie, Hôpital d'Instruction des Armées du Val-de-Grâce, Paris.
H
H
Taillia
T
T
de Greslan
C
C
Adem
F
F
Talarmin
J L
JL
Renard
F
F
Flocard
D000328:Adult; D001847:Bone Diseases / Q000473:pathology; D001842:Bone and Bones / Q000473:pathology; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology; D001933:Brain Stem / Q000473:pathology; D003287:Contrast Media; D017029:Epilepsy, Complex Partial / Q000000981:diagnostic imaging / Q000209:etiology; D031249:Erdheim-Chester Disease / Q000000981:diagnostic imaging / Q000473:pathology*; D019786:Gadolinium DTPA; D006624:Hippocampus / Q000000981:diagnostic imaging / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D011877:Radionuclide Imaging; D012598:Sclerosis
D003287:Contrast Media; D019786:Gadolinium DTPA