MAGNETIC-RESONANCE-IMAGING OF BRAIN AND THE NEUROMOTOR DISORDER IN ENDEMIC CRETINISM

Neurological endemic cretinism, resulting from severe iodine deficienc y, is characterized by mental deficiency, deaf-mutism, and a spastic-r igid motor disorder. Its neuropathology and pathophysiology have been investigated very little. We report the clinical and magnetic resonanc e imaging brain scan findings of 3 adult Chinese cretins. All show an apparent magnetic resonance imaging abnormality in the globus pallidus and substantia nigra, with hyperintensity on T1-weighted images and h ypointensity on T2-weighted images. The motor abnormality, characteriz ed by truncal and proximal limb-girdle rigidity and spasticity, with r elative sparing of the hands and feet, is analogous to other extrapyra midal disorders. Endemic cretinism is a unique form of cerebral palsy of potential interest for students of human neuromotor development.