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Images in Clinical Practices

Indian Pediatrics 1998; 35:1115-1116

Kocher- Debre-SemeIaigne Syndrome


A 31h-year-old girl was brought with the complaint that she refused to run and walked very slowly. There was mild global delay in milestones. Examination showed a dull facies (Fig. 1); height of 75.5 cm (normal 91.7-104.5 cm); pseudohypertrophy of calf muscles (Fig. 2), and a very prominent delay in contraction and relaxation of the ankle reflex. TSH was> 50 U /ml (Normal 2-10 U/ml). She was started on Thyroxine 0.1 mg tablet, 1;2 daily. After 4 months the muscles were soft, ankle reflex was normal, and she had gained 7 cm in height. After 2 years her height had increased by 24 cm and she was reported as being above aver- age in studies.


 

Fig. 1. Note the dull facies. Fig. 2. Pseudohypertrophy of calf muscles.



Kocher-Debre-Semelaigne syndrome is characterized by pseudohypertrophy of muscles secondary to longstanding hypothyroidism. Unlike the present case, it is usually more common in mates and in severe cases. Management of the hypothyroidism is associated with regression of the pseudohypertrophy. The main differential diagnosis is Duchenne Muscular Dystrophy, in which the ankle reflex is usually brisk, the TSH is normal, and there is no response to thyroxine. Confusion is common because both are more common in males, and can be associated with mental retardation.

 

Newton Luiz,
Consultant Pediatrician,
Ohanya Mission Hospital,
Potta
P.O., Thrissur Dt.,
Kerala 680 722,
India.


 

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