Clinical study of neurological cretinism in Sikkim

Sikkim is a small hilly state in the eastern Himalayas. Iodine deficiency disorders constitute a major public health problem for the state. High prevalence of endemic goitre (54.7) and endemic cretinism (3.7) exist in the state. A total of 119 neurological cretins were studied to define the pattern and extent of neurological involvement in endimic cretinism. There were 65 men (54.6) and 54 women (45.4). Goitre was seen in 57 (47.9). Grade-I goitre was the most frequent type of goitre seen in 52.6 of the goitrous cretins. All the endemic cretins had moderate to severe neurological deficits. The most salient neurological feature was deaf-mutism which was seen in 91 cases (76.5). Examination of motor system revealed rigidity,more marked in lower limbs, patellar hyper-reflexia in 106 (89.2) and Babinnski's sign in 40 hypothyroidism. Audiometry done in a subset of 38 cretins revealed that only two cretins had normal hearing. The hearing loss was found to be bilateral and equal and was sever toprofound in 22 cretins; mild to moderate in 9 cretins and was bilateral but unequal in 5 cretins. Neurological cretinism is a distinct and easily identifiable clinical entity. It is an important indicator of severity of iodine deficiency in a community.