Diffusion-weighted imaging in Huntington's disease

• Published in: Movement disorders Vol. 21; no. 7; pp. 1043 - 1047
• Main Authors: Seppi, Klaus, Schocke, Michael F.H., Mair, Katherina J., Esterhammer, Regina, Weirich-Schwaiger, Helga, Utermann, Barbara, Egger, Karl, Brenneis, Christian, Granata, Roberta, Boesch, Sylvia, Poewe, Werner, Wenning, Gregor K.
• Format: Journal Article
• Language: English
• Published: Hoboken Wiley Subscription Services, Inc., A Wiley Company 01-07-2006; Wiley
• Subjects: Activities of Daily Living - classification; Adult; Aged; Basal Ganglia - pathology; Biological and medical sciences; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Diffusion Magnetic Resonance Imaging; diffusion-weighted imaging; Disease Progression; Female; Humans; Huntington Disease - diagnosis; Huntington's disease; Image Processing, Computer-Assisted; Investigative techniques, diagnostic techniques (general aspects); Magnetic Resonance Imaging; Male; Medical sciences; Middle Aged; Nervous system; Neurology; Radiodiagnosis. Nmr imagery. Nmr spectrometry; Reference Values; Severity of Illness Index; striatum; Thalamus - pathology; Nervous system diseases; magnetic resonance imaging; Huntington disease; Diffusion imaging; Huntington's disease; Nuclear magnetic resonance imaging; Cerebral disorder; Genetic disease; striatum; diffusion-weighted imaging; Central nervous system disease; Degenerative disease; Extrapyramidal syndrome
• ISSN: 0885-3185; 1531-8257
• DOI: 10.1002/mds.20868

Huntington's disease (HD) is an autosomal dominant progressive neurodegenerative disorder that results from an expanded trinucleotide (CAG) repeat on the huntingtin gene. Neurodegeneration in HD affects most prominently the basal ganglia. Therefore, diffusivity was obtained in the basal ganglia and thalamus of 29 patients with early HD and 27 healthy volunteers by means of the trace of the diffusion tensor (Trace(D)). Putaminal, caudate, pallidal, and thalamic Trace(D) values were increased in patients with HD compared with controls. Increased diffusivity in the putamen and caudate nucleus correlated with global functional impairment, CAG repeat length, as well as bicaudate ratio. Diffusion‐weighted imaging appears to be a promising surrogate marker for disease severity in HD. Sensitivity to change remains to be established longitudinally. © 2006 Movement Disorder Society