Cerebral manifestation of Erdheim-Chester disease : Clinical and radiologic findings

• Published in: Neurology Vol. 49; no. 6; pp. 1702 - 1705
• Main Authors: BOHLEGA, S, ALWATBAN, J, TULBAH, A, BAKHEET, S. M, POWE, J
• Format: Journal Article
• Language: English
• Published: Hagerstown, MD Lippincott Williams & Wilkins 01-12-1997
• Subjects: Adult; Arthrography; Biological and medical sciences; Brain - pathology; Brain Diseases - diagnosis; Brain Diseases - etiology; Diagnosis, Differential; Diseases of the osteoarticular system; Female; Fluorodeoxyglucose F18; Histiocytosis - complications; Histiocytosis - diagnosis; Humans; Knee Joint - diagnostic imaging; Magnetic Resonance Imaging; Medical sciences; Miscellaneous. Osteoarticular involvement in other diseases; Multiple Sclerosis - diagnosis; Radiopharmaceuticals; Tomography, Emission-Computed; Tropical medicine; Cerebellum; Human; Nervous system diseases; Diseases of the osteoarticular system; Central nervous system; Exploration; Nuclear magnetic resonance imaging; Language disorder; Cerebral disorder; Case study; Symptomatology; Eye disease; Cerebellar disease; Central nervous system disease; Nystagmus; Medical imagery; Ataxia; Complication; Female; Oculomotor syndrome; Neurological disorder; Dysarthria; Brain (vertebrata); Chester-Erdheim disease
• ISSN: 0028-3878; 1526-632X
• DOI: 10.1212/wnl.49.6.1702

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.