J Korean Neurol Assoc.  2006 Feb;24(1):47-50.

Clinical Features of Brachial Amyotrophic Diplegia

• Lee SS
• Lee SH
• Kwon JH

Abstract

BACKGROUND: The purpose of this study was to describe a pure lower motor neuron disease in adults that is isolated to the upper limbs over time and to emphasize a differential diagnosis from other motor neuron diseases or motor neuropathies.
METHODS
We reviewed retrospectively five male patients who had a bilateral upper limb weakness without any sensory changes confirmed by clinical and electrophysiological examinations at least 2 years after onset.
RESULTS
Initially weakness remained largely confined to the arms. It then progressed slowly during the follow-up periods ranging from about 5 to 7 years since onset. However, weakness and muscle atrophy spared the lower limbs, respiratory and bulbar musculatures. The patients did not develop any bulbar dysfunctions or pyramidal tract signs. They were still able to ambulate at the end of follow-up.
CONCLUSIONS
Brachial amyotrophic diplegia presenting with severe weakness that is completely confined to the upper limbs over time, without upper motor neuron signs, might be a stable and relatively benign variant of motor neuron disease. It should be differentiated from other motor neuropathies.

Keyword

Brachial amyotrophic diplegia; Amyotrophic lateral sclerosis; Motor neuron disease; Monomelic amyotrophy

MeSH Terms

Adult
Amyotrophic Lateral Sclerosis
Arm
Diagnosis, Differential
Follow-Up Studies
Humans
Lower Extremity
Male
Motor Neuron Disease
Motor Neurons
Muscular Atrophy
Pyramidal Tracts
Retrospective Studies
Upper Extremity