Brachial amyotrophic diplegia: A slowly progressive motor neuron disorder

Jonathan S. Katz, G. I. Wolfe, P. B. Andersson, D. S. Saperstein, J. L. Elliott, S. P. Nations, W. W. Bryan, R. J. Barohn

Research output: Contribution to journalArticle

110 Scopus citations

Abstract

Objective: To describe a sporadic motor neuron disorder that remains largely restricted to the upper limbs over time. Background: Progressive amyotrophy that is isolated to the upper limbs in an adult often suggests ALS. The fact that weakness can remain largely confined to the arms for long periods of time in individuals presenting with this phenotype has not been emphasized. Methods: We reviewed the records of patients who had a neurogenic 'man-in-the-barrel' phenotype documented by examination at least 18 months after onset. These patients had severe bilateral upper-extremity neurogenic atrophy that spared lower-extremity, respiratory, and bulbar musculature. Results: Nine of 10 patients meeting these criteria had a purely lower motor neuron disorder. During follow-up periods ranging from 3 to 11 years from onset, only three patients developed lower-extremity weakness, and none developed respiratory or bulbar dysfunction or lost the ability to ambulate. Conclusion: Patients presenting with severe weakness that is fully isolated to the upper limbs, without pyramidal signs, may have a relatively stable variant of motor neuron disease.

Original languageEnglish (US)
Pages (from-to)1071-1076
Number of pages6
JournalNeurology
Volume53
Issue number5
DOIs
StatePublished - Sep 22 1999

Keywords

  • Amyotrophic lateral sclerosis
  • Brachial amyotrophic diplegia
  • Focal amyotrophy
  • Man-in-the-barrel syndrome
  • Motor neuron disease

ASJC Scopus subject areas

  • Clinical Neurology

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    Katz, J. S., Wolfe, G. I., Andersson, P. B., Saperstein, D. S., Elliott, J. L., Nations, S. P., Bryan, W. W., & Barohn, R. J. (1999). Brachial amyotrophic diplegia: A slowly progressive motor neuron disorder. Neurology, 53(5), 1071-1076. https://doi.org/10.1212/wnl.53.5.1071