David W S, Jones H R
Department of Neurology, Children's Hospital, Boston, Massachusetts.
Muscle Nerve. 1994 Apr;17(4):424-30. doi: 10.1002/mus.880170410.
Eighty infants with nonarthrogrypotic floppy infant syndrome (FIS) were evaluated between 1979 and 1990. Electromyographic data were correlated with results of muscle and nerve biopsies in 41 of 80 who had concomitant biopsies (38) or other diagnostic analyses (3). A diagnosis was made of Werdnig-Hoffmann disease (WHD) in 15, a congenital infantile polyneuropathy (IPN) in 3, neuromuscular transmission defect (NMTD) in 2, myopathy in 12, and presumed "central" hypotonia in 9. A very positive correlation rate between nerve conduction studies with electromyography and biopsy results was found in 93% (14 of 15) with WHD and 100% in IPN (3 of 3). However, only 4 of 10 infants (40%) with biopsy-proven myopathy had an abnormal EMG. Only once did the results of electromyography and biopsy conflict.
1979年至1990年间，对80例非关节挛缩型松软婴儿综合征（FIS）患儿进行了评估。在80例中有41例同时进行了活检（38例）或其他诊断分析（3例），其肌电图数据与肌肉和神经活检结果相关。诊断为Werdnig-Hoffmann病（WHD）15例、先天性婴儿多发性神经病（IPN）3例、神经肌肉传递缺陷（NMTD）2例、肌病12例、推测为“中枢性”肌张力低下9例。在WHD患者中，神经传导研究与肌电图及活检结果的相关性非常高，WHD患者中有93%（15例中的14例），IPN患者中为100%（3例中的3例）。然而，在10例经活检证实为肌病的婴儿中，只有4例（40%）肌电图异常。肌电图和活检结果仅出现过一次冲突。
