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- Title
Clinical manifestation and neurosurgical intervention of encephalocraniocutaneous lipomatosis-a case report and review of the literature.
- Authors
Chiang, Chia-Chun; Lin, Shih-Chieh; Wu, Hsiu-Mei; Wang, Jia-Chi; Yang, Tsui-Fen; Chen, Hsin-Hung; Ho, Donald; Wong, Tai-Tong
- Abstract
Introduction: Currently, there are only a few reported cases of symptomatic or asymptomatic subpial (intramedullary) spinal lipoma, and therefore no guidelines are available to indicate surgery. These lesions are infrequently associated with spina bifida. Case report: Herein, we provide our experience in the neurosurgical intervention of compressive myeloradiculopathy for encephalocraniocutaneous lipomatosis (ECCL). The patient initially presented with bilateral upper hand paralysis, then regained muscle power after surgery and during 1 year of follow-up. We discuss the neurosurgical indications and intervention, imaging studies, other associated symptoms, and the pathogenesis of ECCL in an infant.
- Subjects
LIPOMATOSIS; SURGICAL decompression; RADICULOPATHY; PARALYSIS; NEUROSURGERY
- Publication
Child's Nervous System, 2014, Vol 30, Issue 1, p13
- ISSN
0256-7040
- Publication type
Article
- DOI
10.1007/s00381-013-2252-z