Clinical Features of Brachial Amyotrophic Diplegia

J Korean Neurol Assoc > Volume 24(1); 2006 > Article

Journal of the Korean Neurological Association 2006;24(1): 47-50.

위팔근육위축양측마비의 임상적 특성

이상수, 이성현 권지현

충북대학교 의과대학 신경과학교실

Clinical Features of Brachial Amyotrophic Diplegia

Sang-Soo Lee

Department of Neurology, College of Medicine, Chungbuk National University, Cheongju, Korea

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.