Dash Santosh Kumar, Kamble Nitish, Stezin Albert, Yadav Ravi, Netravathi M, Saini Jitender, Pal Pramod
Neurology, Kalinga Institute of Medical Sciences, Bhubaneswar, IND.
Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, IND.
Cureus. 2024 Aug 27;16(8):e67896. doi: 10.7759/cureus.67896. eCollection 2024 Aug.
Background Multiple system atrophy (MSA) is a rare, adult-onset neurodegenerative disorder marked by autonomic failure, parkinsonism, and cerebellar ataxia, with subtypes classified as parkinsonian (MSA-P), cerebellar (MSA-C), and autonomic (MSA-A). This study aims to identify MRI biomarkers for MSA and their correlation with disease severity. Methodology A total of 30 patients with probable MSA (20 MSA-C, 10 MSA-P) aged 45-65 years were studied. Motor and non-motor symptoms were assessed using the Unified Multiple System Atrophy Rating Scale (UMSARS), and all patients underwent 3T MRI brain imaging. Data analysis was performed using SPSS version 22 (IBM Corp., Armonk, NY, USA) with Spearman's correlation for clinical-imaging correlations. Results The mean age of the study population was 54.43 years, with a male predominance (56.7%). The most common symptoms included gait ataxia (43.3%) and urinary dysfunction (96.7%), with orthostatic symptoms in 33.3%. Moderate disease severity was observed, with mean UMSARS scores of 15.9 (Part 1) and 16 (Part 2), showing no significant subtype differences. MRI revealed abnormalities in all patients, predominantly cerebellar atrophy (90%). The "hot cross bun" (HCB) sign was seen in 75% of MSA-C patients, but none of MSA-P patients showed the same. The HCB sign was significantly correlated with severity in MSA-C (USMSARS-4). Putaminal signs were less frequent and slightly more prevalent in MSA-P, without significant clinical-imaging correlation. Conclusions This study reinforces the critical role of MRI biomarkers in the diagnosis of MSA patients. Notably, the HCB sign exhibited a significant association with clinical severity in MSA-C patients, while such correlation was absent in MSA-P cases.
多系统萎缩(MSA)是一种罕见的成人起病的神经退行性疾病,其特征为自主神经功能衰竭、帕金森综合征和小脑共济失调,亚型分为帕金森型(MSA-P)、小脑型(MSA-C)和自主神经型(MSA-A)。本研究旨在识别MSA的MRI生物标志物及其与疾病严重程度的相关性。
共研究了30例年龄在45至65岁之间的可能患有MSA的患者(20例MSA-C,10例MSA-P)。使用统一多系统萎缩评定量表(UMSARS)评估运动和非运动症状,所有患者均接受3T MRI脑部成像。使用SPSS 22版(美国纽约州阿蒙克市IBM公司)进行数据分析,采用Spearman相关性分析临床与影像的相关性。
研究人群的平均年龄为54.43岁,男性占优势(56.7%)。最常见的症状包括步态共济失调(43.3%)和排尿功能障碍(96.7%),33.3%的患者有体位性症状。观察到疾病严重程度为中度,UMSARS平均评分为15.9(第1部分)和16(第2部分),各亚型之间无显著差异。MRI显示所有患者均有异常,主要为小脑萎缩(90%)。75%的MSA-C患者出现“热交叉面包”(HCB)征,但MSA-P患者均未出现相同表现。HCB征与MSA-C的严重程度(USMSARS-4)显著相关。壳核体征较少见,在MSA-P中略为普遍,临床与影像无显著相关性。
本研究强化了MRI生物标志物在MSA患者诊断中的关键作用。值得注意的是,HCB征在MSA-C患者中与临床严重程度显著相关,而在MSA-P病例中则不存在这种相关性。