Primary cerebral non-Langerhans cell histiocytosis: MRI and differential diagnosis.
We report a young woman with primary cerebral non-Langerhans cell histiocytosis of the juvenile xanthogranuloma family. The clinical course was complicated by extensive infiltration of cranial nerves and meninges and epi- and intramedullary spinal dissemination. Whereas the cutaneous form of juvenile xanthogranuloma is usually benign and self-limited, central nervous system involvement is associated with high morbidity and mortality and might therefore be considered a separate clinical entity.
Ernemann
U
U
Department of Neuroradiology, University Hospital Tübingen, Hoppe-Seyler-Strasse 3, 72076 Tübingen, German. ulrike.ernemann@med.uni-tuebingen.de
Skalej
M
M
Hermisson
M
M
Platten
M
M
Jaffe
R
R
Voigt
K
K
D000328:Adult; D001927:Brain Diseases / Q000175:diagnosis* / Q000473:pathology; D003391:Cranial Nerves / Q000473:pathology; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008578:Meninges / Q000473:pathology; D014972:Xanthogranuloma, Juvenile / Q000175:diagnosis* / Q000473:pathology