Cerebral manifestation of Erdheim-Chester disease: clinical and radiologic findings.
A 33-year-old woman presented with a 3-year history of progressive numbness in the hand, cerebellar ataxia, limb weakness, nystagmus, and dysarthria. T2-weighted MRI revealed abnormal foci of increased signal intensity mimicking demyelinating plaques in the periventricular white matter, and brain 18FDG-PET scan showed increased uptake in the pons. Biopsy from a tibial lesion showed aggregates of foamy histiocytes in the intertrabecular spaces replacing the bone marrow, characteristic of Erdheim-Chester disease. The patient was treated with craniospinal radiation. After 6 months, the clinical picture was stable and the MRI was unchanged.
Bohlega
S
S
Department of Neurosciences, King Faisal Specialist Hospital & Research Centre, Riyadh, Saudi Arabia.
Alwatban
J
J
Tulbah
A
A
Bakheet
S M
SM
Powe
J
J
D000328:Adult; D001175:Arthrography; D001921:Brain / Q000473:pathology; D001927:Brain Diseases / Q000175:diagnosis* / Q000209:etiology*; D003937:Diagnosis, Differential; D005260:Female; D019788:Fluorodeoxyglucose F18; D015614:Histiocytosis / Q000150:complications* / Q000175:diagnosis*; D006801:Humans; D007719:Knee Joint / Q000000981:diagnostic imaging; D008279:Magnetic Resonance Imaging; D009103:Multiple Sclerosis / Q000175:diagnosis; D019275:Radiopharmaceuticals; D014055:Tomography, Emission-Computed
D019275:Radiopharmaceuticals; D019788:Fluorodeoxyglucose F18