상완근위축양측마비의 경과 관찰 2례 - 증례보고 -
Follow Up of Brachial Amyotrophic Diplegia - Report of Two Cases -
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Brachial amyotrophic diplegia (BAD) is a rare subtype of motor neuron disease (MND). It presents severe weakness confined to upper extremities. Because lower extremities and bulbar musculatures are spared for several years, BAD has better prognosis than other MND. Therefore, we should distinguish BAD to other MND. Here we report 2 cases of BAD eventually progressed to the other body part. They showed predominantly proximal muscle weakness in both upper extremities. There were no evidence of weakness in lower extremities and bulbar musculatures for 3-4 years. We diagnosed them as BAD with clinical features and electrodiagnostic examinations. Nerve conduction studies were normal except the low amplitude of median and ulnar CMAP. Needle electromyography showed denervation potential and neuropathic MUAP throughout upper extremities, however lower extremities and facial musculatures were normal. After 1 year, on follow up, they presented disease progression in lower limbs and bulbar muscles. Electrodiagnostic findings are consistent with disease progression.
Abstract
Brachial amyotrophic diplegia (BAD) is a rare subtype of motor neuron disease (MND). It presents severe weakness confined to upper extremities. Because lower extremities and bulbar musculatures are spared for several years, BAD has better prognosis than other MND. Therefore, we should distinguish BAD to other MND. Here we report 2 cases of BAD eventually progressed to the other body part. They showed predominantly proximal muscle weakness in both upper extremities. There were no evidence of weakness in lower extremities and bulbar musculatures for 3-4 years. We diagnosed them as BAD with clinical features and electrodiagnostic examinations. Nerve conduction studies were normal except the low amplitude of median and ulnar CMAP. Needle electromyography showed denervation potential and neuropathic MUAP throughout upper extremities, however lower extremities and facial musculatures were normal. After 1 year, on follow up, they presented disease progression in lower limbs and bulbar muscles. Electrodiagnostic findings are consistent with disease progression.
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