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Neurological endemic cretinism,resulting from severe iodine deficiency,is characterized by mental deficiency,deaf-mutism,and spastic-rigid motor disorder.Its neuropathology and pathophysiology have been investigated very little.We report the clinical and magnetic resonance 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 hypointensity on T2-weighted images.The motor abnormality,characterized by truncal and proximal limb- girdle rigidity and spasticity,with relative sparing of the hands and feet,is analogous to another extrapyramidal disorders.Endemic cretinism is a unique form of cerebral palsy of potential interest for students of human neuromotor development. |
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