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KMID : 0360919700130090759
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Journal of the Korean Medical Association
1970 Volume.13 No. 9 p.759 ~ p.766
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REPORT OF TWO CASES OF WERDNIG-HOFFMANN PARALYSIS IN ONE SIBLING
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安裕哲/Ahn, Yoo Chul
李昇薰/柳聖熙/金泳祚/Lee, Seung Hoon/Rhu, Sung Hi/Kim, Young Jo
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Abstract
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In many reports of clinical manifestation of neuromuscular disorders, it has been recognized that repetition of the same syndrome in several members of a family group is, a common phenomenon.
Werdnig-Hoffmann disease in childhood is also called progressive familial spinal muscular atrophy, because it has been described as occuring in siblings between the age of six months and one year, and is inherited in an autosomal recessive pattern.
we report here that Werdnig-Hoffmann disease appears to have affected seven out of eight children in one sibling group. Five of the children expired before the age of five, and two children have been brought into our out-patient clinic as they are showing the same syndrome are following the same pathological course as the previous five children. One child has shown no signs of the disease. The seven affected children were said to have been born in a normal healthy condition, but atrophy and weakness of the shoulder, back muscle, hip and extremities were noted between the age of six months and one year. The infants lost power in one limb after another, ultimately developed complete flaccid paralysis of entire limb muscles and spinal erectal muscles.
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