Acquired hepatocerebral degeneration (AHD): a peculiar neurological impairment in advanced chronic liver disease
| Autor: | F. Iuculano, Lorena Airaghi, Anna Ludovica Fracanzani, C. Saetti, Silvia Fargion, Luca Valenti, Alessandro Pigoni, M. Curioni, S. Spreafico, Rosa Lombardi, L. Burdick |
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| Rok vydání: | 2017 |
| Předmět: |
Adult
Liver Cirrhosis medicine.medical_specialty Pathology Neurology Cirrhosis Chronic liver disease Biochemistry 03 medical and health sciences Cellular and Molecular Neuroscience Liver disease 0302 clinical medicine Hepatolenticular Degeneration Humans Medicine Stroke Hepatic encephalopathy Autoimmune encephalitis business.industry Brain medicine.disease Magnetic Resonance Imaging Chronic Disease Female 030211 gastroenterology & hepatology Neurology (clinical) Liver function business 030217 neurology & neurosurgery Follow-Up Studies |
| Zdroj: | Metabolic Brain Disease. 33:347-352 |
| ISSN: | 1573-7365 0885-7490 |
| Popis: | We discuss the case of a rare and often unrecognized neurologic syndrome, called Acquired Hepatocerebral Degeneration (AHD), observed in patients with advanced liver disease and portosystemic shunts. The clinical manifestations can be very heterogeneous and in our case included a combination of cerebellar and extrapyramidal signs, arisen in a period of few days. Brain Magnetic Resonance Imaging (MRI) showed, in T1-weighted images, diffuse bilateral hyper intensities in basal ganglia and biemispheric brain and cerebellar cortices, resembling paramagnetic deposits. No other neurological impairments, like stroke, infection or neoplasia, were found. It was excluded an episode of acute hepatic encephalopathy. We also ruled out Wilsonian degeneration, iron overload and autoimmune encephalitis and we lastly found high manganese levels as the possible cause of the brain paramagnetic deposits. Even though either serum Mn determination or its accumulation in the brain are not specific for AHD, however the chronic and progressively worsening of the neurological manifestations advocated a degenerative condition, possibly AHD. We finally opted for the early restoration of liver function by OLT, and we observed complete clinical symptoms' resolution and partial MRI reversal after a follow up of 6 months. |
| Databáze: | OpenAIRE |
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