Magnetization Transfer MR Imaging Demonstrates Degeneration of the Subcortical and Cortical Gray Matter in Huntington Disease

• Published in: American journal of neuroradiology : AJNR Vol. 31; no. 10; pp. 1807 - 1812
• Main Authors: GINESTRONI, A, BATTAGLINI, M, DICIOTTI, S, NAVE, R. Della, MAZZONI, L. N, TESSA, C, GIANNELLI, M, PIACENTINI, S, DE STEFANO, N, MASCALCHI, M
• Format: Journal Article
• Language: English
• Published: Oak Brook, IL American Society of Neuroradiology 01.11.2010
• Subjects: Adult; Aged; Biological and medical sciences; Brain - pathology; Cerebral Cortex - pathology; Corpus Striatum - pathology; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Disability Evaluation; Female; Functional; Humans; Huntington Disease - genetics; Huntington Disease - pathology; Investigative techniques, diagnostic techniques (general aspects); Magnetic Resonance Imaging - methods; Male; Medical sciences; Middle Aged; Nerve Degeneration - genetics; Nerve Degeneration - pathology; Nervous system; Neurology; Radiodiagnosis. Nmr imagery. Nmr spectrometry; Severity of Illness Index; Thalamus - pathology; Nervous system diseases; Magnetization; Radiodiagnosis; Central nervous system disease; Huntington disease; Degenerative disease; Degeneration; Subcortex; Nuclear magnetic resonance imaging; Genetic disease; Cerebral disorder; Extrapyramidal syndrome
• ISSN: 0195-6108; 1936-959X
• DOI: 10.3174/ajnr.A2225

GM is typically affected in HD since the presymptomatic stage. Our aim was to investigate with MT MR imaging the microstructural changes of the residual brain subcortical and cortical GM in carriers of the HD gene and to preliminarily assess their correlation with the clinical features. Fifteen HD gene carriers with a range of clinical severity and 15 age- and sex-matched healthy controls underwent MT MR imaging on a 1.5T scanner. The MT ratio was measured automatically in several subcortical and cortical GM regions (striatal nuclei; thalami; and the neocortex of the frontal, temporal, parietal, and occipital lobes) by using FLS tools. The MT ratio was significantly (P < .05 with Bonferroni correction for multiple comparison) decreased in all subcortical structures except the putamen and decreased diffusely in the cerebral cortex of HD carriers compared with controls. Close correlation was observed between the subcortical and cortical regional MT ratios and several clinical variables, including disease duration, motor disability, and scores in timed neuropsychological tests. MT imaging demonstrates degeneration of the subcortical and cortical GM in HD carriers and might serve, along with volumetric assessment, as a surrogate marker in future clinical trials of HD.