Clinical manifestation and neurosurgical intervention of encephalocraniocutaneous lipomatosis-a case report and review of the literature

被引：6

作者：

Chiang, Chia-Chun ^{[1
,5
]}

Lin, Shih-Chieh ^{[2
,5
]}

Wu, Hsiu-Mei ^{[3
,5
]}

Wang, Jia-Chi ^{[4
,5
]}

Yang, Tsui-Fen ^{[4
,5
]}

Chen, Hsin-Hung ^{[1
,5
]}

Ho, Donald M. ^{[3
,5
]}

Wong, Tai-Tong ^{[1
,5
]}

机构：

[1] Taipei Vet Gen Hosp, Neurol Inst, Div Pediat Neurosurg, Taipei, Taiwan

[2] Taipei Vet Gen Hosp, Dept Pathol, Taipei, Taiwan

[3] Taipei Vet Gen Hosp, Dept Radiol, Taipei, Taiwan

[4] Taipei Vet Gen Hosp, Dept Phys Med & Rehabil, Taipei, Taiwan

[5] Natl Yang Ming Univ, Sch Med, Taipei 11217, Taiwan

来源：

CHILDS NERVOUS SYSTEM | 2014年 / 30卷 / 01期

关键词：

Encephalocraniocutaneous lipomatosis; Surgical decompression; Compressive cervical myeloradiculopathy; Spine; INVOLVEMENT; ANOMALIES; PATIENT;

D O I：

10.1007/s00381-013-2252-z

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

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. 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.

引用

页码：13 / 17

页数：5

共 16 条

[11] ENCEPHALOCRANIOCUTANEOUS LIPOMATOSIS WITH A MUTATION IN THE NF1 GENE [J].