2004Archives of NeurologyRequires access

Prominent Cerebellar Symptoms With Unusual Magnetic Resonance Imaging Findings in Acquired Hepatocerebral Degeneration

Sun Ah Park, Kyoung Heo

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Abstract

BACKGROUND: Cerebellar lesions revealed by abnormal signals on magnetic resonance images are extremely rare in acquired hepatocerebral degeneration (AHCD). OBJECTIVE: To report a case of AHCD with prominent cerebellar findings both clinically and radiologically. DESIGN AND SETTING: Case report and tertiary-care hospital. PATIENT: 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. RESULTS: 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. CONCLUSIONS: 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.

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What this paper is about

BACKGROUND: Cerebellar lesions revealed by abnormal signals on magnetic resonance images are extremely rare in acquired hepatocerebral degeneration (AHCD). OBJECTIVE: To report a case of AHCD with prominent cerebellar findings both clinically and radiologically. DESIGN AND SETTING: Case report and tertiary-care hospital. PATIENT: 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. RESULTS: 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. CONCLUSIONS: 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.

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Available abstract

BACKGROUND: Cerebellar lesions revealed by abnormal signals on magnetic resonance images are extremely rare in acquired hepatocerebral degeneration (AHCD). OBJECTIVE: To report a case of AHCD with prominent cerebellar findings both clinically and radiologically. DESIGN AND SETTING: Case report and tertiary-care hospital. PATIENT: 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. RESULTS: 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. CONCLUSIONS: 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.

Key concepts: Cerebellum, Magnetic resonance imaging, Dysarthria, Ataxia, Ataxic Gait, Cerebellar ataxia, Medicine, Pathology

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Prominent Cerebellar Symptoms With Unusual Magnetic Resonance Imaging Findings in Acquired Hepatocerebral Degeneration — Research Paper | ScholarLens