비전형적인 운동신경세포병: 위팔근육위축양측마비 = Atypical Form of Motor Neuron Disease: Brachial Amyotrophic Diplegia

다국어 초록 (Multilingual Abstract)

Brachial amyotrophic diplegia is a rare disease presenting with severe weakness that is completely confined to the upper limbs over time without upper motor neuron sign. It might be a stable and relatively benign variant
of motor neuron disease and should be differentiated from other motor neuropathies. A 60-year-old man presented with a two year history of symmetrical muscle wasting and weakness in his arm and shoulder girdle. Bulbar and leg muscle were not affected and he was independent in ambulation. Nerve conduction studies revealed normal except the low amplitude of median and ulnar compound muscle action potential with no evidence of conduction block. Needle electromyography showed denervation potential and neuropathic motor unit action potential throughout the upper limb. We report this case with a brief review of related literatures.

참고문헌 (Reference)

1 이상수, "위팔근육위축양측마비의 임상적 특성" 대한신경과학회 24 (24): 47-50, 2006

2 Hudson AJ, "The motor neuron disease and related disorders" JB Lippincott 4 : 1-35, 1996

3 Neudert C, "The course of the terminal phase in patients with amyotrophic lateral sclerosis" 248 : 612-616, 2001

4 Mulder DW, "The clinical syndrome of amyotrophic lateral sclerosis" 32 : 427-436, 1957

5 Williams DB, "Motor neuron disease (amyotrophic lateral sclerosis)" 66 : 54-82, 1991

6 Hu MT, "Flail arm syndrome: A distinctive variant of amyotrophic lateral sclerosis" 65 : 950-951, 1998

7 Gamez J, "Flail arm syndrome or Vulpian-Bernhart’s form of amyotrophic lateral sclerosis" 67 : 258-, 1999

8 Katz JS, "Brachial amyotrophic diplegia: A slowly progressive motor neuron disorder" 53 : 1071-1076, 1999

9 Sasaki S, "Atypical form of amyotrophic lateral sclerosis" 66 : 581-585, 1999

10 Mitsumoto H, "Amyotrophic lateral sclerosis" F. A. Davis Company 3-163, 1998

11 Kang SH, "A case report of X-linked Recessive bulbospinal muscular atrophy (Kennedy’s syndrome)" 11 : 174-179, 2007