ABSTRACT
Introduction: Chronic acquired hepatocerebral degenerationis a rare disorder typically accompanied by cognitivedisturbances and parkinsonian features secondary to liverdisease. It is the deposition of manganese in the basal ganglialeading to T1 hyperintensity on MRI of these patients and theresulting clinical features. Familial association is found insome cases.Case report: Here, we report the case of a 32 year old postpartum female who presented with tremors and historyof liver disease. MRI of the brain showed symmetrichyperintensities in globus pallidi, substantiae nigrae, andsuperior cerebral peduncles in T1-weighted images. Althoughliver transplantation is the best modality of treatment atpresent, the patient showed improved on treatment withLevodopa+carbidopa.Conclusion: It is important to recognize this disease entity forsymptomatic relief of the patient. However further research isrequired for better management.
ABSTRACT
Abstract Acquired hepatocerebral degeneration is a neurological syndrome with typical clinical (extrapyramidal and neuropsychiatric) symptoms and brain magnetic resonance imaging findings (high T1 signal in the globus pallidus). It occurs mainly in patients with advanced liver disease, such as in patients co-infected with hepatitis B virus (HBV) and hepatitis delta virus (HDV). However, there are no reports relating HBV/HDV coinfection and acquired hepatocerebral degeneration. This report presents the case of a 49-year-old woman with characteristics of acquired hepatocerebral degeneration and liver cirrhosis due to HBV/HDV coinfection, and presents the main theories of the physiopathology of this condition.
Subject(s)
Humans , Female , Hepatitis D/complications , Hepatitis B/complications , Hepatolenticular Degeneration/virology , Liver Cirrhosis/virology , Coinfection/virology , Middle AgedABSTRACT
Acquired hepatocerebral degeneration is an underdiagnosed neurologic syndrome characterized by parkinsonism,ataxia or other movement disorders and by neuropsychiatric and cognitive symptoms. It occurs in patients with chronic liverdisease, especially those who develop portosystemic shunting and is often unrecognized as a cause of cognitive decline.Recently, its pathogenesis has been associated with manganese accumulation in basal ganglia and some treatmentsproposed. The aim of this article was to report a case and discuss some discoveries in connection with the disease.
Degeneração hepatocerebral adquirida é uma síndrome neurológica subdiagnosticado caracterizada porparkinsonismo, ataxia ou outros distúrbios do movimento e por sintomas neuropsiquiátricos e cognitivos. Ocorre empacientes com doença hepática crônica, especialmente aqueles que desenvolvem shunts porto-sistêmicos e frequentementenão é reconhecido como uma causa de declínio cognitivo. Recentemente, sua patogênese tem sido associada ao acúmulode manganês nos gânglios da base e alguns tratamentos foram propostos. O objetivo do artigo foi relatar um caso e discutiralgumas descobertas nesta doença.
Subject(s)
Humans , Hepatic Encephalopathy , Hepatolenticular Degeneration , Liver DiseasesABSTRACT
Nystagmus or ataxia is a rare manifestation of acquired hepatocerebral degeneration (AHCD). A 49-year-old woman presented with downbeat nystagmus and limb and gait ataxia. She was diagnosed as primary biliary cirrhosis with a gastric varix. Brain MRI showed cerebellar vermian atrophy and characteristic T1 high-signal intensities in bilateral globus pallidi and ventral midbrain. We report a rare case of AHCD manifesting prominent cerebellar symptoms. This has not yet been reported in Korea.
Subject(s)
Female , Humans , Middle Aged , Ataxia , Atrophy , Brain , Esophageal and Gastric Varices , Extremities , Gait Ataxia , Hepatolenticular Degeneration , Korea , Liver Cirrhosis, Biliary , Magnetic Resonance Imaging , MesencephalonABSTRACT
BACKGROUND: Chronic acquired hepatocerebral degeneration (CAHD), a slowly progressive neurologic disease characterized by chronic intermittent hepatic encephalopathy, is seen sporadically in patients with chronic hepatic disease. The spectrum of clinical presentations could include neuropsychiatric (apathy, lethargy, excessive somnolence), a movement disorder (ataxia, tremor, chorea, Parkinsonism, myoclonus, dystonia) or both. Unfortunately only a few reports are available concerning the neurological manifestations as well as the findings of brain Magnetic resonance image (MRI) in patients with CAHD in spite of high prevalence of chronic liver disease in Korea. METHODS: We reviewed clinical or laboratory data of 3 patients with CAHD. All patients had evaluations such as neurological examination, mini-mental status examination, liver function test and brain MRI. From sagittal image of their brain MRI, we calculated pallidal signal intensity. RESULTS: The most common neurological findings were cognitive dysfunction, dysarthria, and parkinsonism including tremor, rigidity and bradykinesia. The brain MRI of all three patients showed hyperintensity signal in globus pallidus on T1 weighted image. Some patients also showed similar signal intensity on their brainstem. CONCLUSIONS: We describe three cases of CAHD with clinical, radiological feature.
Subject(s)
Humans , Brain , Brain Stem , Chorea , Dysarthria , Globus Pallidus , Hepatic Encephalopathy , Hepatolenticular Degeneration , Hypokinesia , Korea , Lethargy , Liver Diseases , Liver Function Tests , Magnetic Resonance Imaging , Movement Disorders , Myoclonus , Neurologic Examination , Neurologic Manifestations , Parkinsonian Disorders , Prevalence , TremorABSTRACT
Chronic acquired hepatocerebral degeneration (CAHD) is a progressive or episodic neurologic syndrome that occurs occasionally in patients who have chronic liver disease or portocaval shunt. The clinical features of CAHD include action and postural tremors, generalized chorea, asterixis, myoclonus, dystonia, resting tremor, gait ataxia, and variable impairments of intellectual function. We experienced 2 cases of CAHD with unusual neurologic manifestations, which were hypokinetic parkinsonian symptoms. Both cases had a history of liver cirrhosis and the same symptoms. They had masked faces, cogwheel rigidiy in both wrists, slurred speech, tongue tremor, bradykinesia, and gait ataxia. They had normal mental status and no pathologic reflex. Brain MRI showed abnormal, increased signal on T1-weighted images in the globus pallidus and mesencephalon bilaterally. The hypokinetic parkinsonian symptoms disappeared when given doses levodopa.
Subject(s)
Humans , Brain , Chorea , Dyskinesias , Dystonia , Gait Ataxia , Globus Pallidus , Hepatolenticular Degeneration , Hypokinesia , Levodopa , Liver Cirrhosis , Liver Diseases , Magnetic Resonance Imaging , Masks , Mesencephalon , Myoclonus , Neurologic Manifestations , Reflex , Tongue , Tremor , WristABSTRACT
BACKGROUND AND SIGNIFICANCE: Chronic acquired hepatocerebral degeneration(CAHD) is a heterogenous disorder that can occur with a primary neurologic, hepatic, or combined presentation. Characteristic radiologic finding is high signal in globus pallidus on T1WI MRI, and which is associated with increased level of Manganese. We experienced a patient with CAHD presenting various involuntary movements. Our patient had multiple anomalous vessels with a porto-systemic shunt in abdomen in absence of liver cirrhosis. CASE: A 74-years-old female was admitted because of gradually progressive buccal and lingual choreiform movements with moderate generalized chorea. In our patient, T1 weighted MRI of the brain showed symmetric high signal intensity in both globus pallidus and subthalamus. Increased ammonia level(165umol/L) and Manganese level(7.75ug/dl) in whole blood, pancytopopenia in peripheral blood smear and a multiple vessel anomaly with a porto-systemic shunt on abdominal ultrasonography and CT were found. These involuntary movements had a dramatic response to neuroleptics and nearly disappeared within 5 days. CONCLUSION: We report one patient with chronic acquired hepatocerebral degeneration which had a porto-systemic shunt by anomalous vessels and various involuntary movements.