Pattern difference of dissociated hand muscle atrophy in amyotrophic lateral sclerosis and variants

Jee Eun Kim, Yoon Ho Hong, Joon Hyung Lee, Suk Won Ahn, Sung Min Kim, Kyung Seok Park, Jung Joon Sung, Kwang Woo Lee, Seung Yong Seong

Research output: Contribution to journalArticlepeer-review

24 Scopus citations


Introduction: Split hand is considered to be a specific feature of amyotrophic lateral sclerosis (ALS). Methods: We evaluated the pattern difference of intrinsic hand muscles of upper limb-onset ALS (UL-ALS), upper limb-onset progressive muscular atrophy (UL-PMA), brachial amyotrophic diplegia (BAD), and Hirayama disease (HD) by measuring objective electrophysiological markers. Results: The abductor digiti minimi (ADM)/abductor pollicis brevis (APB) compound muscle action potential (CMAP) amplitude ratio was significantly higher in UL-ALS than other variants, but a considerable proportion of UL-ALS cases had an amplitude ratio in the range of other variants. Absent APB CMAP and abnormally high ADM/APB CMAP amplitude ratio (≥4) occurred only with UL-ALS. Conversely, an absent ADM CMAP was identified only in UL-PMA and BAD. Conclusions: The absolute ADM/APB CMAP amplitude ratio was not specific for ALS; however, several findings from simple electrophysiological measurements may help predict prognosis in patients with motor neuron diseases and may be early diagnostic markers for ALS.

Original languageEnglish
Pages (from-to)333-337
Number of pages5
JournalMuscle and Nerve
Issue number3
StatePublished - 1 Mar 2015

Bibliographical note

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© 2014 Wiley Periodicals, Inc.


  • Amyotrophic lateral sclerosis
  • Brachial amyotrophic diplegia, Hirayama disease
  • Progressive muscular atrophy
  • Split hand


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