| A case of myositis ossificans progressiva. |
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Chai Sum Chang |
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Seoul Electric Company Hospital, Seoul |
| 進行性化骨性筋炎의 一例 |
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張 在 暹 |
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京電病院 小兒科長 |
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| Abstract |
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A nine years and two months old girl was seen on the 13. of Jan. with a chief complaint of firm swelling of the right sternocleidomastoid muscle. This swelling had been noticed for ten days without ony notable cause. She had had no fever nor any other attributable symptom. At about 15 months of age, her mother noted that the child showed scoliosis of the spinal column and limited movement of the neck. No history of similar diseases or hereditary disturbances were known in her sister, three brothers, nor other members of the family.
The patient appeared somewhat undernourished and shorter than normal for her age. There was stiffness of the neck with limited rotation and inability to flex the chin onto the chest. There were microdactily of the thumbs and great toes and marked scoliosis of the spine. The right sternal cleidomastoid muscle had swelled markedly with boardlike consistency. There were none of particular imflammtory changes on the overlying skin.
Laboratory examination revealed no abnormal findings except moderate positive result of Old Tuberculin test. Radiologic studies of skull, chest and long bones revealed normal formation except fusion of the I” II” III and IV. cervical spines. The swelling of the muscle has progressed downward, in the fallowing order of progression: front of the neck, left sternocleidomastoid muscle, right shoulder girdle, left shoulder girdle, right trapezius muscle, left trapezius muscle, right pectoral muscle groups, left pectoral muscle groups, both lumbal regions and
both arms, sparing only hands and lower extremities. Biopsy specimens were obtained from right trapezius muscle on Feb. 8. 1958. There were moderate fibrous proliferation, degenerative changes of muscle, round
cell infiltration, congestion and edema. On the 23. of July, bony masses were palpated in the left side of the neck, right axillar region extending from upper arm to the chest and left axillar region extending to the left flank, with limited movement of the upper extremities. The palpated bony masses were seen on the radiologic studies.
Treatment with cortisone and prednisolone was started on the 24. of Feb. 1958. The progress of the disease appearse to be unaffected by steroid therapy even when instituted early.
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