| A Case of Kugelberg-Welander Disease. |
|
H S Han1, K Shim1, Y S Kang1, Y S Yoon1, Y K Kim1, J G Chi2 |
1Department of pediatrics, Han-il Hospital, Korea.
2Department of pathology, college of medicine, Seoul, National University,Korea. |
| Werdnig-Hoffmann 병 (Kugelberg-Welander 형)의 1例
|
|
한창수1, 심 광1, 강용섭1, 윤용수1, 김연기1, 지제근2 |
1한일병원 소아과
2서울대학교 의과대학 병리학교실 |
|
|
|
|
| Abstract |
|
A case of Kugelberg-Welander Disease, a rare neuromuscular disease in childhood, in a 28 month old Korean male is presented. This patient was fairly good until 24 months of age, when she was found to have waddling gait, weakness of both lower extremities along with tremor of hands. At the time of admission he showed obvious proximal muscle weakness, more proximal groups and showed Gower's sign. Deep tendon reflexes were abolished. Electromyogrtaphy showed patterns compatable with anterior horn cell disease. And Vastus muscle biopsy showed small round fibers without definite grouping. No degeneration or inflammation was present. Maternal cousin of this patient was known to have muscle disease probably related to this disease. |
|
PDF Links
Download Citation
