Department of Neurology, Shengjing Hospital of China Medical University, 36 Sanhao Street, Heping District, Shenyang, 110004, Liaoning, People's Republic of China.
Neurol Sci. 2020 Jan;41(1):175-181. doi: 10.1007/s10072-019-04068-y. Epub 2019 Sep 7.
Acquired hepatocerebral degeneration (AHD) is now widely recognized by physicians. Although hyperintensity in the bilateral globus pallidus in T1-weighted magnetic resonance images (MRIs) are characteristic neuroimaging findings, accumulating reports indicate that atypical neuroimaging findings are not rare. This study aimed to describe the spectrum of atypical neuroimaging findings and related factors in patients with AHD.
From February 2017 to January 2019, a retrospective study was conducted of 28 patients with AHD in the Shengjing Hospital of China Medical University. The neurological manifestations, clinical parameters, and biochemical and neuroimaging findings were analyzed.
Among 28 patients, 14 patients were diagnosed with viral hepatitis-caused hepatocirrhosis, which was the most common cause of AHD. Resting tremor, cognitive impairment, and parkinsonian gait were the most common neurologic symptoms. Bilateral globus pallidus T1-weighted hyperintensity was detected in 26 patients (26/28, 92.9%). Ten patients (10/28, 35.7%) were determined to have an atypical neuroimaging finding. Binary logistic regression analysis indicated that age at onset of neurologic symptoms (odds ratio = 1.29, 95% confidence interval [CI] 1.03-1.61; p = 0.030) and Child-Pugh scores (odds ratio = 2.52, 95% CI, 1.01-6.31; p = 0.048) were independently associated with atypical neuroimaging findings in AHD.
The clinical manifestations of AHD are diverse; resting tremor, cognitive impairment, and parkinsonian gait were the most common. More than one third of patients had atypical neuroimaging findings. Age at onset of neurologic symptoms and Child-Pugh scores may be important predictors of atypical neuroimaging findings in patients with AHD.
获得性肝性脑变性(AHD)现在已被医生广泛认识。虽然 T1 加权磁共振成像(MRI)双侧苍白球高信号是其特征性神经影像学表现,但越来越多的报道表明,不典型的神经影像学表现并不少见。本研究旨在描述 AHD 患者的非典型神经影像学表现谱及其相关因素。
回顾性分析 2017 年 2 月至 2019 年 1 月在中国医科大学盛京医院就诊的 28 例 AHD 患者的临床资料,分析其神经症状、临床参数、生化及神经影像学表现。
28 例患者中,14 例病毒性肝炎导致肝硬化,为 AHD 最常见的病因。静止性震颤、认知障碍和帕金森步态是最常见的神经症状。26 例(26/28,92.9%)患者双侧苍白球 T1 加权高信号。10 例(10/28,35.7%)患者存在不典型神经影像学表现。二元逻辑回归分析表明,神经症状发病年龄(比值比=1.29,95%置信区间[CI]:1.031.61;p=0.030)和 Child-Pugh 评分(比值比=2.52,95%CI:1.016.31;p=0.048)与 AHD 的不典型神经影像学表现独立相关。
AHD 的临床表现多样,静止性震颤、认知障碍和帕金森步态是最常见的。超过三分之一的患者存在不典型神经影像学表现。神经症状发病年龄和 Child-Pugh 评分可能是 AHD 患者不典型神经影像学表现的重要预测因素。