THE NATURAL-HISTORY OF TYPE-I (SEVERE) SPINAL MUSCULAR-ATROPHY

被引：82

作者：

THOMAS, NH ^{[1
]}

DUBOWITZ, V ^{[1
]}

机构：

[1] HAMMERSMITH HOSP,DEPT PAEDIAT & NEONATAL MED,LONDON W12 0HS,ENGLAND

来源：

NEUROMUSCULAR DISORDERS | 1994年 / 4卷 / 5-6期

关键词：

SPINAL MUSCULAR ATROPHY; WERDNIG-HOFFMANN DISEASE;

D O I：

10.1016/0960-8966(94)90090-6

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

The clinical features of 36 patients who satisfied the diagnostic criteria for type I (severe) spinal muscular atrophy (Werdnig-Hoffmann disease) are reported. Survival data for both the whole cohort and for groups within the cohort subdivided on the age of onset are presented. These data suggest that the patients with onset at birth or within the first 2 months of life have a more uniformly poor prognosis with earlier death. This is of potential importance in any therapeutic trials in the future whose outcome may be based on length of survival.

引用

页码：497 / 502

页数：6

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