Department of Neurology and Clinical Pharmacology, Ehime University Graduate School of Medicine, Tohon Ehime, Japan.
Department of Neurology and Geriatrics, Kagoshima University Graduate School of Medicine and Dental Sciences, Kagoshima, Japan.
Parkinsonism Relat Disord. 2017 Nov;44:137-141. doi: 10.1016/j.parkreldis.2017.08.012. Epub 2017 Aug 14.
Although spinocerebellar ataxia type 2 (SCA2) is classified as hereditary spinocerebellar degeneration, some patients present with parkinsonism before developing cerebellar ataxia.
I-metaiodobenzyl guanidine (I-MIBG) myocardial scintigraphy and/or dopamine transporter single photon emission computed tomography (DAT SPECT) using I-ioflupane (I-FP-CIT) were performed for the six patients from three SCA2 families.
I-MIBG myocardial scintigraphy showed reduced cardiac uptake in four of five patients and an association with Lewy body disease was suggested. DAT SPECT showed decreased uptake in the striatum in all four patients who were scanned, including one patient without parkinsonism. When patterns of uptake were compared to those with Parkinson's disease, most of the patients had minimal reduction of uptake in the putamen.
DAT SPECT is expected to be useful in differentiating SCA2 from Parkinson's disease, making an early diagnosis, and allowing early therapeutic intervention.
虽然脊髓小脑性共济失调 2 型（SCA2）被归类为遗传性脊髓小脑变性，但一些患者在出现小脑共济失调之前会出现帕金森病。
对来自三个 SCA2 家族的六名患者进行碘代间位苄胍（I-MIBG）心肌闪烁显像和/或使用碘-123 碘-β-单环乙基托烷（I-FP-CIT）的多巴胺转运体单光子发射计算机断层扫描（DAT SPECT）。
I-MIBG 心肌闪烁显像显示五名患者中的四名心脏摄取减少，提示与路易体病有关。DAT SPECT 显示在所有四名接受扫描的患者的纹状体中摄取减少，包括一名无帕金森病的患者。当比较摄取模式与帕金森病时，大多数患者的壳核摄取减少很少。
DAT SPECT 有望有助于将 SCA2 与帕金森病区分开来，以便进行早期诊断并允许早期治疗干预。
