Park Sun Ah, Heo Kyoung
Department of Neurology, Soonchunhyang University Bucheon Hospital, 1174 Jung-dong, Wonmi-gu, Bucheon, Gyeonggido 420-767, South Korea.
Arch Neurol. 2004 Sep;61(9):1458-60. doi: 10.1001/archneur.61.9.1458.
Cerebellar lesions revealed by abnormal signals on magnetic resonance images are extremely rare in acquired hepatocerebral degeneration (AHCD).
To report a case of AHCD with prominent cerebellar findings both clinically and radiologically.
Case report and tertiary-care hospital.
A 46-year-old man complained of progressive speech difficulties of 5 months' duration. Two years earlier, he had been diagnosed as having cirrhosis of the liver caused by alcoholism and hepatitis B virus infection.
The patient had progressive ataxic dysarthria and limb and gait ataxia as manifestations of AHCD. Magnetic resonance imaging of the brain revealed distinctive symmetrical T2 high-signal intensities in the bilateral cerebellar hemispheres and brachium pontis, which were consistent with his neurologic deficits. Simultaneously, high T1 signals in the bilateral pallidum and ventral midbrain were noted, which are typical manifestations of AHCD. Follow-up magnetic resonance imaging 3 months later showed the same cerebellar signs and abnormal signals.
The cerebellar cortex and middle cerebellar peduncle are considered highly vulnerable structures to metabolic insults in liver disease. Findings from our patient suggest that dominant cerebellar deficits with compatible T2 high-signal lesions are another type of clinical manifestation in AHCD.
在获得性肝脑变性(AHCD)中,磁共振成像显示异常信号的小脑病变极为罕见。
报告1例在临床和放射学上均有明显小脑表现的AHCD病例。
病例报告及三级护理医院。
一名46岁男性,主诉持续5个月的进行性言语困难。两年前,他被诊断为酒精性肝硬化合并乙型肝炎病毒感染。
该患者有进行性共济失调性构音障碍、肢体和步态共济失调,为AHCD的表现。脑部磁共振成像显示双侧小脑半球和脑桥臂有明显的对称性T2高信号强度,这与他的神经功能缺损一致。同时,双侧苍白球和中脑腹侧可见T1高信号,这是AHCD的典型表现。3个月后的随访磁共振成像显示相同的小脑体征和异常信号。
小脑皮质和小脑中脚被认为是肝病代谢损伤的高度易损结构。我们患者的发现表明,伴有T2高信号病变的显性小脑功能缺损是AHCD的另一种临床表现类型。