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 Citations (Scopus)

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
StatePublished - Sep 22 1999

Fingerprint

Motor Neurons
Arm
Upper Extremity
Lower Extremity
Phenotype
Motor Neuron Disease
Aptitude
Atrophy
Motor Disorders

Keywords

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

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Katz, J. S., Wolfe, G. I., Andersson, P. B., Saperstein, D. S., Elliott, J. L., Nations, S. P., ... Barohn, R. J. (1999). Brachial amyotrophic diplegia: A slowly progressive motor neuron disorder. Neurology, 53(5), 1071-1076.

Brachial amyotrophic diplegia : A slowly progressive motor neuron disorder. / Katz, Jonathan S.; Wolfe, G. I.; Andersson, P. B.; Saperstein, D. S.; Elliott, J. L.; Nations, S. P.; Bryan, W. W.; Barohn, R. J.

In: Neurology, Vol. 53, No. 5, 22.09.1999, p. 1071-1076.

Research output: Contribution to journalArticle

Katz, JS, Wolfe, GI, Andersson, PB, Saperstein, DS, Elliott, JL, Nations, SP, Bryan, WW & Barohn, RJ 1999, 'Brachial amyotrophic diplegia: A slowly progressive motor neuron disorder', Neurology, vol. 53, no. 5, pp. 1071-1076.
Katz JS, Wolfe GI, Andersson PB, Saperstein DS, Elliott JL, Nations SP et al. Brachial amyotrophic diplegia: A slowly progressive motor neuron disorder. Neurology. 1999 Sep 22;53(5):1071-1076.
Katz, Jonathan S. ; Wolfe, G. I. ; Andersson, P. B. ; Saperstein, D. S. ; Elliott, J. L. ; Nations, S. P. ; Bryan, W. W. ; Barohn, R. J. / Brachial amyotrophic diplegia : A slowly progressive motor neuron disorder. In: Neurology. 1999 ; Vol. 53, No. 5. pp. 1071-1076.
@article{a16b856523f04bb6ab8d51fafdb8fd05,
title = "Brachial amyotrophic diplegia: A slowly progressive motor neuron disorder",
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.",
keywords = "Amyotrophic lateral sclerosis, Brachial amyotrophic diplegia, Focal amyotrophy, Man-in-the-barrel syndrome, Motor neuron disease",
author = "Katz, {Jonathan S.} and Wolfe, {G. I.} and Andersson, {P. B.} and Saperstein, {D. S.} and Elliott, {J. L.} and Nations, {S. P.} and Bryan, {W. W.} and Barohn, {R. J.}",
year = "1999",
month = "9",
day = "22",
language = "English (US)",
volume = "53",
pages = "1071--1076",
journal = "Neurology",
issn = "0028-3878",
publisher = "Lippincott Williams and Wilkins",
number = "5",

}

TY - JOUR

T1 - Brachial amyotrophic diplegia

T2 - A slowly progressive motor neuron disorder

AU - Katz, Jonathan S.

AU - Wolfe, G. I.

AU - Andersson, P. B.

AU - Saperstein, D. S.

AU - Elliott, J. L.

AU - Nations, S. P.

AU - Bryan, W. W.

AU - Barohn, R. J.

PY - 1999/9/22

Y1 - 1999/9/22

N2 - 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.

AB - 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.

KW - Amyotrophic lateral sclerosis

KW - Brachial amyotrophic diplegia

KW - Focal amyotrophy

KW - Man-in-the-barrel syndrome

KW - Motor neuron disease

UR - http://www.scopus.com/inward/record.url?scp=0033595565&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0033595565&partnerID=8YFLogxK

M3 - Article

C2 - 10496268

AN - SCOPUS:0033595565

VL - 53

SP - 1071

EP - 1076

JO - Neurology

JF - Neurology

SN - 0028-3878

IS - 5

ER -