興味ある筋萎縮症の2例—I．脊髄性進行性筋萎縮症の片麻痺型 II．末梢型を示した進行性筋ジストロフィー症

TWO CASES OF ATYPICAL MUSCULAR ATROPHY

I．脊髄性進行性筋萎縮症の片麻痺型症例

第1例：喜○公○　44歳　男　警察官

主訴：左上下肢筋萎縮

Case 1: 44-year-old man. At the age of 31, he noted muscular atrophy in the left hand, which slowly progressed to the left fore and upper arm. At the age of 44 the left leg became atrophic. Neurologic examination revealed nor-mal mental state, muscular atrophy in the left arm and leg. Deep reflexes were dimin-ished on the left. The right limbs were al-most normal. EMG revealed fasciculation and giant motor nuit potential in the left limbs.

He died of postoperative complication of sto-mach ulcer after the course of 13 years. Autopsy revealed unilateral atrophy and loss of ventral horn cells of the spinal cord on the left. The long course with left muscular atro-phy due to spinal origin was reported.

Case 2: 34-year-old man. At the age of 21, he noticed gait disturbance and two years later slight muscular wasting of both legs and feet. He was admitted first time at age 25 in our clinic, when moderate atrophy was seen in both small hand muscles, extensor muscle of the forearms and legs, and sternomastoids. But there was no definite sensory loss. Hisc co-urse was slowly progressive. At aged of 31 muscular wastings developed in both proximal portion of arms and shoulder girdles, but there are no myotonic reacton, frontal baldness, cataracta, fasciculation, and sensory dystur-bances.

Both muscle biopsy and repeated electro-myographies revealed definite myopathic feat-ures. A rare case, which was diagnosed as an atypical distal type of progressive muscular dystrophies, was reported.