2019
DOI: 10.5334/tohm.475
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Uncommon Movement Disorders in Chronic Hepatic Disease with Response to Rifaximin

Abstract: BackgroundChronic hepatic disease can present with extrapyramidal symptoms. We describe two cases that presented with highly unusual movement disorders: ballism and gait freezing.Case reportPatient 1 is a 42-year-old man with previous episodes of hepatic encephalopathy (HE) who presented with upper limb dystonia and generalized chorea that progressed to ballism. Patient 2 is a 55-year-old woman who presented with pronounced gait freezing. In both patients, features of HE and acquired hepatocerebral degeneratio… Show more

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Cited by 4 publications
(5 citation statements)
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“…We were unable to establish a definite cause of chorea in 8 cases (cases [31][32][33][34][35][36][37][38], despite an extensive work-up. We suspected frontotemporal dementia in 4 cases (cases 30, 33,34 and 37) and hereditary ataxia in 2 patient (case 32 and 36) although the findings of genetic analyses were negative for hexanucleotide expansion (C9ORF72), progranulin mutation and/or common dominant hereditary/recessive ataxias (SCA 1,2-3-6,7,8, 17,36, DRPLA and Friedreich).…”
Section: Unknown Etiologymentioning
confidence: 89%
See 1 more Smart Citation
“…We were unable to establish a definite cause of chorea in 8 cases (cases [31][32][33][34][35][36][37][38], despite an extensive work-up. We suspected frontotemporal dementia in 4 cases (cases 30, 33,34 and 37) and hereditary ataxia in 2 patient (case 32 and 36) although the findings of genetic analyses were negative for hexanucleotide expansion (C9ORF72), progranulin mutation and/or common dominant hereditary/recessive ataxias (SCA 1,2-3-6,7,8, 17,36, DRPLA and Friedreich).…”
Section: Unknown Etiologymentioning
confidence: 89%
“…Chorea may be observed in many systemic and metabolic conditions such as uncontrolled diabetes, liver and renal disease, hyperthyroidism, electrolyte disturbances and vitamin B12 deficiency [8,10,[32][33][34][35][36][37][38]. Suspicion of metabolic disorders can be especially high when movement disorders, including chorea, are observed in emergency settings [8,10].…”
Section: Discussionmentioning
confidence: 99%
“…The increased levels of natural agonist of the 5-HT 2A receptor in the cortex might be related to the HTR in WD-fed mice. It is worth mentioning that HE may manifest extrapyramidal dysfunctions, such as involuntary body movements or dyskinesia [ 61 , 62 ], which can appear as head bobbing, twitching, or body swaying. Psychotic symptoms like hallucinations may appear in patients with HE [ 63 ].…”
Section: Discussionmentioning
confidence: 99%
“…Choreic movements mostly appear to be independent from the hepatic encephalopathy episodes. Hepatic transplantation is, therefore, the only effective treatment in most cases, but ammonia-reducing therapy such as lactulose may improve movement disorders in some patients ( 89 ).…”
Section: Acute Metabolic Disordersmentioning
confidence: 99%