A Case of Cerebral Erdheim-Chester Disease With Progressive Cerebellar Syndrome
0
조회
0
다운로드
1
인용
0
참고문헌
키워드
초록
Erdheim-Chester disease (ECD) is a rare non-Langerhans form of histiocytosis. Cerebellar involvement is rare in this syndrome. We report a 37-year-old woman with slowly progressive cerebellar ataxia, dysmetria of limbs, nystagmus, and dysarthria, bilateral painful axillary masses, and generalized arthralgia. Brain MRI revealed cerebellar atrophy with focal lesions in the pons, middle cerebellar peduncle, and the cerebellum. She underwent incisional biopsy of her axillary masses which showed findings consistent with ECD. An MRI of her lower extremities revealed lesions in the diaphyses, metaphyses, and epiphyses of the proximal tibia and distal femur bilaterally. This is a rare case of cerebral ECD with progressive cerebellar syndrome associated with cerebellar atrophy.
Abstract
Erdheim-Chester disease (ECD) is a rare non-Langerhans form of histiocytosis. Cerebellar involvement is rare in this syndrome. We report a 37-year-old woman with slowly progressive cerebellar ataxia, dysmetria of limbs, nystagmus, and dysarthria, bilateral painful axillary masses, and generalized arthralgia. Brain MRI revealed cerebellar atrophy with focal lesions in the pons, middle cerebellar peduncle, and the cerebellum. She underwent incisional biopsy of her axillary masses which showed findings consistent with ECD. An MRI of her lower extremities revealed lesions in the diaphyses, metaphyses, and epiphyses of the proximal tibia and distal femur bilaterally. This is a rare case of cerebral ECD with progressive cerebellar syndrome associated with cerebellar atrophy.
참고문헌 (15)
- Veyssier-Belot C, "Erdheim-Chester disease: Clinical and radiologic characteristics of 59 cases", Medicine, 72, pp.157~169 (1996)
- Chester W, "Uber lipoidgranulomatose", Virchows Arch patholol Anat, 279, pp.561~602 (1930)
- Clerico A, "Erdheim-Chester disease in a child", Med Pediatr Oncol, 41, pp.575~577 (2003)
- Globerman H, "A xanthogranulomatous histiocytosis in a child presenting with short stature", Am J Pediatr Hematol Oncol, 13, pp.42~46 (1991)
- Tan AP, "Erdheim-Chester disease involving breast and muscle: imaging findings", AJR Am J Roentgenol, 164, pp.1115~1117 (1995)
- Lachenal F, "Neurological manifestations and neuroradiological presentation of Erdheim-Chester disease: report of 6 cases and systematic review of the literature", J Neurol, 253, pp.1267~1277 (2006)
- Wright RA, "Neurological manifestations of Erdheim-Chester disease", J Neurol Neurosurg Psychiatry, 66, pp.72~75 (1999)
- Weidauer S, "Cerebral Erdheim-Chester disease: case report and review of the literature", Neuroradiology, 45, pp.241~245 (2003)
- Grothe C, "Cerebellar syndrome, exophthalmus and secondary hypogonadism in Erdheim-Chester disease", Nervenarzt, 72, pp.449~452 (2001)
- Pautas E, "Cerebral Erdheim-Chester disease: report of two cases with progressive cerebellar syndrome with dentate abnormalities on magnetic resonance imaging", J Neurol Neurosurg Psychiatry, 65, pp.597~599 (1998)
- Fukazawa T, "Erdheim-Chester disease and slowly progressive cerebellar dysfunction", J Neurol Neurosurg Psychiatry, 58, pp.238~240 (1995)
- . Kujat C, "Cerebral manifestations of Erdheim-Chester disease", Radiologe, 31, pp.307~309 (1991)
- Adle-Biassette H, "Pathology of the central nervous system in Chester-Erdheim disease: report of three cases", J Neuropathol Exp Neurol, 56, pp.1207~1216 (1997)
- Bohlega S, "Cerebral manifestation of Erdheim-Chester disease: clinical and radiologic findings", Neurology, 49, pp.1702~1705 (1997)
- Vital C, "Brain stem infiltration by mixed Langerhans cell histiocytosis and Erdheim-Chester disease: more than just an isolated case", Clin Exp Pathol, 47, pp.71~76 (1999)