Department of Neurology, Kyung Hee University Hospital, Kyung Hee University College of Medicine, 23 Kyungheedae-ro, Dongdaemun-gu, Seoul, 02447, Republic of Korea.
Department of Pathology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Republic of Korea.
BMC Neurol. 2021 Apr 6;21(1):148. doi: 10.1186/s12883-021-02178-9.
Neurodegenerative disorders are characterized by insidious progression with poorly-delineated long latent period. Antecedent clinical insult could rarely unmask latent neurodegenerative disorders. Here, we report an autopsy-proven case of corticobasal degeneration which was preceded by a lacunar infarction.
A 58-year-old man presented with acute ataxia associated with a lacunar infarction in the right paramedian pons. His ataxia persisted with additional progressive gait difficulty and left arm clumsiness. Six months later, a follow-up neurological examination showed asymmetrical bradykinesia, apraxia, dystonic posturing, postural instability, and mild ataxia of the left limbs. Cognitive examination revealed frontal executive dysfunction and visuospatial difficulties. Dopamine transporter imaging scan demonstrated bilateral reduced uptakes in mid-to-posterior putamen, more prominent on the right side. Levodopa-unresponsive parkinsonism, asymmetric limb dystonia, and ideomotor apraxia became more conspicuous, while limb ataxia gradually vanished. The patient became unable to walk without assistance after 1 year, and died 4 years after the symptom onset. Autopsy findings showed frontoparietal cortical atrophy, ballooned neurons, and phosphorylated tau-positive astrocytic plaques and neuropil threads with gliosis and neuronal loss, confirming the corticobasal degeneration.
The case illustrates that precedent clinical events such as stroke might tip a patient with subclinical CBS into overt clinical manifestations.
神经退行性疾病的特点是隐匿性进展，潜伏期长且不明确。先前的临床损伤很少能揭示潜在的神经退行性疾病。在这里，我们报告了一例经尸检证实的皮质基底节变性病例，该病例之前曾发生腔隙性梗死。
一名 58 岁男性因右侧旁正中桥脑腔隙性梗死伴急性共济失调就诊。他的共济失调持续存在，并伴有进行性步态困难和左侧手臂笨拙。6 个月后，随访神经检查显示不对称性运动徐缓、失用症、扭曲姿势、姿势不稳和左侧肢体轻度共济失调。认知检查显示额叶执行功能和视空间困难。多巴胺转运蛋白成像扫描显示双侧中后壳核摄取减少，右侧更明显。左旋多巴反应性帕金森病、不对称性肢体肌张力障碍和意念运动性失用症变得更加明显，而肢体共济失调逐渐消失。1 年后，患者无法在没有帮助的情况下行走，并在症状出现 4 年后死亡。尸检结果显示额顶叶皮质萎缩、气球样神经元以及磷酸化 tau 阳性星形胶质斑块和神经丝缠结伴神经胶质增生和神经元丢失，证实为皮质基底节变性。
该病例表明，先前的临床事件，如中风，可能会使亚临床 CBS 患者出现明显的临床表现。
