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Acquired Hepatocerebral Degeneration

Acquired Hepatocerebral Degeneration. Sian Chess-Williams 2012 Manorlands Hospice. Objectives . To gain knowledge about a not widely known illness. Introduction. Case Background to disease PMH Problems on admission Examination Actions during admission Patient & family Readmission

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Acquired Hepatocerebral Degeneration

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  1. Acquired Hepatocerebral Degeneration Sian Chess-Williams 2012 Manorlands Hospice

  2. Objectives • To gain knowledge about a not widely known illness.

  3. Introduction • Case • Background to disease • PMH • Problems on admission • Examination • Actions during admission • Patient & family • Readmission • Signs present • AHD • Conclusion • References

  4. Mr AHD • Elderly man with liver cancer & AHD. • Originally admitted to Manorlands in March 2012 for respite for 2/52 due to carer strain. • Readmission April due to deterioration.

  5. Mr AHD Background to disease: • Hepatocellular carcinoma diagnosed July 2008 (on a background of previously undiagnosed chronic liver disease, from likely non-alcoholic steatohepatitis). • Percutaneous ablation of the tumour 2008. • MRI Dec 2011 showed 3 new liver tumours – not for treatment and to rescan in 6 months. • Consultant neurologist diagnosed acquired hepatocerebral degeneration and hepatic myelopathy (at first incorrectly diagnosed with parkinsons and lewy body dementia)

  6. Mr AHD PMH: • Type 2 diabetes (on insulin) • Previous rheumatic fever • Oesophageal varices Problems on admission: • Carer strain • Difficulty speaking • Difficulty swallowing • Mobility problems • Constipation • Left loin/back pain • Recent likely TIA’s

  7. Mr AHD Examination: • No overt ascites. • Continuous writhing movements of his tongue, face and eyes. • Increased tone all 4 limbs. • Reflexes all brisk & plantars downgoing. • Poor coordination. • Power 5/5, sensation intact.

  8. Mr AHD Actions during admission: • SALT assessment – uvula deviated to left, therefore not sealing nasal cavity, looked into a palatal lift appliance. • No obvious cause found for pain: oxynorm • Over sensitive to medications iemidazolam, haloperidol. Therefore try and avoid. • 3/3/12 likely TIA lasting 20 mins – speech worse & weakness left arm. D/W stroke team & clopidogrel started. • Furosemide stopped as deteriorating renal function. • Insulin decreased & later stopped (D/W Diabetes consultant AGH). • Ferrous sulphate stopped as Hb stable 12.6 • D/w consultant neurologist: OPD 1 year ago, difficult to give prognosis, risk of confusion at any point, hallucinations can be part of disease, stop Rivastigmine (help with drooling symptom). • Started oral buscopan for drooling.

  9. Mr AHD Patient & family: • Wife main carer needed to take to toilet, ect. Plus carers BD to wash & dress. Accepted hospital bed on 2nd admission. • Wanted to know prognosis and more about disease. • Jehovah’s witnesses. • Day therapy FU.

  10. Mr AHD Readmission: • Deterioration at home over past week, decreasing mobility, bed bound, not eating, not rousable on admission. • Overall decline, still fluctuating during admission. • Advance care planning – hospital bed, PPD here.

  11. Mr AHD Signs present: • Fluctuating cognition – sometimes AMTS 0/10 sometimes 7/10. • Tremor • Chorecoid movements – mainly tongue, mouth, eyes. • Increased tone • Ataxia. • Hallucinations

  12. Acquired hepatocerebral degeneration • A chronic progressive neurological syndrome. • Characterised by parkinsonism, ataxia & other movement disorders. • First reported by W. Van Woerken in 1914. • Lacks proven medical therapy & very little research done (despite greater prevalence than Wilsons disesase). • Remains under-diagnosed.

  13. Acquired hepatocerebral degeneration Pathogenesis: • May accompany any form of chronic liver disease associated with portosystemic shunting. • Metal intoxication may contribute, particularly manganese. Prevalence: • 2% in those with cirrhosis • 20-90% in those awaiting liver transplant have a broad spectrum movement disorder. Age of onset: • Usually fifth to sixth decade • Occasionally affects children

  14. Acquired hepatocerebral degeneration Pattern of disease: • Insidious onset or subacute after several weeks to decades of hepatic dysfunction. • Generally progressive but variable – gradual deterioration to years of clinical stability. Survival: • Several weeks to excess of 30 years. • Though usually die from cirrhosis complications of cirrhotic liver failure (infection, coagulopathy, hepatorenal syndrome, cancer, hepatic coma).

  15. Acquired hepatocerebral degeneration Clinical presentation: • Cognitive & behavioural changes (apathy, bradyphrenia, poor attention). • Cognitive dysfunction frequently • Visuo-spatial attention impairments. • Generally not accompanied with a reduced level of consciousness • Language function, memory & praxis usually well preserved.

  16. Acquired hepatocerebral degeneration Clinical presentation: • Abnormal movements :– • parkinsonism (tremor, rigidity, bradykinesia, postural instability, shuffling gait)(bilateral & symmetrical at onset, peak severity at 7 months)(1/5 awaiting transplant) • ataxia • dystonia • chorea • action tremor • orobuccolingualsteretypy similar to tardivedyskinesia.

  17. Acquired hepatocerebral degeneration Clinical presentation: • Myelopathy • Cranial dyskinesia • Dystonic spasms (forced grimacing, jaw opening, bleparospasm & occular deviations) • Dysarthria & hypophonia • No sensory loss or pyramidal signs

  18. Acquired hepatocerebral degeneration Investigations: • MRI head: increased T1 signal within the pallidal nuclei, sometimes extending into adjacent basal ganglia structures & the thalami. T2 weighted images normal. • MRI spine usually normal, however motor evoked potentials demonstrate thoracic spinal cord dysfunction. • EEG: generalised slowing of the background rhythm Management: • None. • Levodopa may benefit a few with parkinsonism. • Dopamine receptor antagonists can help with chorea & orobuccolingualdyskinesias. Tetrabenazine is sometimes preferred due to less risk of irreversible tardive symptoms. • Lactulose, antibiotics and short term dietary protein restriction may minimise co-morbid toxic-metabolic encephalopathy. • EDTA is a chelator of manganese – efficacy incertain. • Symptoms may resolve following liver transplant. • Iron deficiency anaemia may contribute to the progression of AHD.

  19. Conclusion • Frequently confused with other diseases (hepatic encephalopathy, Wilsons disease, Parkinsons, Lewy body dementia) • A disease that can fluctuate with spontaneous remissions & sudden relapses. • Prognosis difficult to predict. • More research is needed.

  20. References • Acquired hepatocerebral degeneration. Journal of Neurology (2009). 256: 320 -332. J Ferrara et al. • ACD: MR & pathologic findings. AGNR Am J Neuroradiology 19:485-487. March 1998. Jonwon Lee et al. • Victor MD, Adams RD, Cole M. The Acquired (Non-Wilsonian) Type of Chronic Hepatocerebral Degeneration. Medicine (Baltimore) 1965; 44:345–96. • Lewis MB, MacQuillan G, Bamford JM, Howdle PD. Delayed myelopathic presentation of the acquired hepatocerebral degeneration syndrome. Neurology2000; 54:1011. • Soffer D, Sherman Y, Tur-Kaspa R, Eid A. Acquired hepatocerebral degeneration in a liver transplant recipient. ActaNeuropathol (Berl) 1995; 90:107–11

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