Nigral involvement and nigrostriatal dysfunction in Huntington's disease: Evidences from an MRI and SPECT study

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• 作者：Lorenzo Kiferle ; Sonia Mazzucchi ; Elisa Unti ; Ilaria Pesaresi ; Serena Fabbri ; Valentina Nicoletti ; Duccio Volterrani ; Mirco Cosottini ; Ubaldo Bonuccelli ; Roberto Ceravolo
• 关键词：Huntington's disease ; SPECT ; MRI ; Substantia nigra ; Neuropsychiatry
• 刊名：Parkinsonism and Related Disorders
• 出版年：2013
• 出版时间：September, 2013
• 年：2013
• 卷：19
• 期：9
• 页码：800-805
• 全文大小：710 K

文摘

Background

Huntington disease (HD) is pathologically characterized by a selective neurodegeneration of vulnerable populations of neurons, with an early marked neuronal loss and atrophy in the neostriatum. Dopaminergic innervations of neostriatal neurons originate in the substantia nigra pars compacta. Few studies investigated the neuronal loss and the functional role of the substantia nigra in modulating clinical features in HD.

Methods

12 patients and 12 age-matched controls underwent SPECT scans with ¹²³I-FP-CIT and a 1.5?T MRI scan with inversion recovery technique. The association between both clinical and neuropsychological features and striatal uptake and volume of substantia nigra was explored.

Results

Striatal (p?<?0.05), caudate (p?<?0.05), and putaminal (p?<?0.01) uptake was significantly lower in patients with respect to controls. Further, the volume of substantia nigra was reduced in HD when compared to controls (p?<?0.01). No relationship between the volume of SN and tracer striatal uptake was found as well as between clinical and neuropsychological features with the SPECT and MRI results.

Conclusions

Our results confirm that the degeneration of nigrostriatal pathway may occur in symptomatic HD patients. If confirmed by larger studies, the lack of any kind of correlation between clinical and neuropsychological features with striatal uptake and volume of substantia nigra suggests that motor and cognitive aspects in HD are not directly related to nigrostriatal degeneration.