Morphology of the cerebral cortex in preclinical Huntington's disease

OBJECTIVE: Cortical morphology was evaluated in subjects with known gene expansion for Huntington's disease and no manifest disease. METHOD: Magnetic resonance imaging scans were obtained for 24 subjects with preclinical Huntington's disease and were compared to those for 24 matched healthy subjects by means of novel imaging methods to quantify aspects of cortical structure. RESULTS: In relation to the comparison subjects, those with preclinical Huntington's disease showed altered cortex morphology with enlargement of gyral crowns and abnormally thin sulci. These changes were manifested in global alterations of gyral and sulcal shape. CONCLUSION: These findings lend support to the notion that, in addition to the degenerative process, abnormal neural development may also be an important process in the pathoetiology of Huntington's disease.