[Pseudo-tumoral and ischemic encephalic Erdheim-Chester disease].
BACKGROUND
Erdheim-Chester disease (ECD) is a rare non-langerhans cell histiocytosis of unknown etiology. It is a multi-systematic xanthogranulomatous infiltration with almost constant bone involvement; the neurological manifestations are not specific and occur in 15-20% of cases.


METHODS
We report the case of a 59-year-old woman hospitalized for a frontal syndrome and right hemiparesis.


RESULTS
Imaging revealed a left caudate nucleus process with recent infarct. Cardiovascular involvement and bilateral osteosclerosis of long bones strongly suggested ECD, confirmed after biopsies of the pericardium and bone.


CONCLUSIONS
Pseudo-tumor encephalic ECD is very rare; the caudate nuclei is an unusual localization; ischemic stroke has been exceptionally described. Prognosis depends largely on the involvement of the central nervous and cardiovascular systems.
Amezyane
T
T
Service de médecine interne B, hôpital militaire d'instruction Mohammed-V, 10000 Hay Ryad, Rabat, Maroc. amez.tao@caramail.com
Abouzahir
A
A
Bassou
D
D
Zoubeir
Y
Y
Hammi
S
S
Mahassin
F
F
Ohayon
V
V
Archane
M-I
MI
D001842:Bone and Bones / Q000000981:diagnostic imaging / Q000473:pathology; D001921:Brain / Q000473:pathology; D002544:Cerebral Infarction / Q000473:pathology; D031249:Erdheim-Chester Disease / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D010496:Pericardium / Q000473:pathology; D011559:Pseudotumor Cerebri / Q000150:complications*; D011877:Radionuclide Imaging