Brooks D J, Ibanez V, Playford E D, Sawle G V, Leigh P N, Kocen R S, Harding A E, Marsden C D
MRC Cyclotron Unit, Hammersmith Hospital, London, England.
Ann Neurol. 1991 Aug;30(2):166-71. doi: 10.1002/ana.410300207.
Using [18F]dopa, [11C]raclopride, C15O2, and positron emission tomography, we have assessed striatal dopamine storage capacity, dopamine D2-receptor integrity, and regional cerebral blood flow, respectively, of 6 patients with neuroacanthocytosis. The patients with neurocanthocytosis all had chorea and variable combinations of seizures, dementia, axonal neuropathy, and orolingual self-multiation. [18F]dopa positron emmission tomographic findings were compared with 30 normal controls and 16 patients with sporadic, L-dopa-responsive, Parkinson's disease. Caudate and anterior putamen [18F]dopa uptake were normal in patients with neuroacanthocytosis, but mean posterior putamen [18F]dopa uptake was reduced to 42% of normal, similar to that in patients with Parkinson's disease. In patients with neuroacanthocytosis, mean equilibrium caudate: cerebellum and putamen: cerebellum [11C]raclopride uptake ratios were reduced to 54% and 62% of normal, compatible with a 65% and 53% loss of caudate and putamen D2-receptor-binding sites, respectively. Striatal and frontal blood flow was also depressed. The severe loss of D2-receptor-bearing striatal neuron, with concomitant loss of dopaminergic projections from the nigra to the posterior putamen, is consistent with both chorea and extrapyramidal rigidity being features of patients with neuroacanthocytosis.
我们使用[18F]多巴、[11C]雷氯必利、C15O2和正电子发射断层扫描技术，分别评估了6例神经棘红细胞增多症患者的纹状体多巴胺储存能力、多巴胺D2受体完整性和局部脑血流。神经棘红细胞增多症患者均有舞蹈症，伴有癫痫、痴呆、轴索性神经病和口面部自残行为的不同组合。将[18F]多巴正电子发射断层扫描结果与30名正常对照者以及16例散发性、对左旋多巴有反应的帕金森病患者进行了比较。神经棘红细胞增多症患者的尾状核和壳核前部[18F]多巴摄取正常，但壳核后部的平均[18F]多巴摄取降至正常水平的42%，与帕金森病患者相似。在神经棘红细胞增多症患者中，尾状核：小脑和壳核：小脑的平均平衡期[11C]雷氯必利摄取率分别降至正常水平的54%和62%，分别相当于尾状核和壳核D2受体结合位点丧失65%和53%。纹状体和额叶血流也降低。含有D2受体的纹状体神经元严重丧失，同时黑质到壳核后部的多巴胺能投射丧失，这与神经棘红细胞增多症患者出现舞蹈症和锥体外系僵硬症状是一致的。
