Neuroacanthocytosis: A rare movement disorder with magnetic resonance imaging

In betalipoprotein deficiencies, lack of microsomal triglyceride transfer protein (MTP) or a direct mutation in the gene for betapolipoprotein leads to a decreased absorption of vitamin E as well as other vitamins and possibly other cofactors. The case discussed here gives us an important message, i.e., typical MRI imaging finding "eye-of-the-tiger" sign appearance with associated peripheral blood smear study showing acanthocytes is useful in detecting this rare neurological disease.