Acquired hepatocerebral degeneration

Abstract

Cirrhosis and its co-morbidities may cause a variety of neurological complications, the most common being bouts of toxic metabolic encephalopathy. A proportion of patients with chronic liver disease develop acquired hepatocerebral degeneration (AHD), a chronic progressive neurological syndrome characterized by parkinsonism, ataxia and other movement disorders. This article reviews the clinical spectrum, pathophysiology, neuroimaging features and differential diagnosis of AHD along with emerging treatment options.

Acquired hepatocerebral degeneration and hepatic encephalopathy: correlations and variety of clinical presentations in overt and subclinical liver disease

“…Even so, there are many other reports in which chorea emerged as the prominent manifestation. Choreoathetosis is almost invariably present in the face, affecting the tongue, eyes and lips, with the appearance of so called grimacing 3,16,20 . Nonetheless, even patients with different clinical presentations have an invariable association with direct or indirect signs of portocaval shunts 9,11,28,29 .…”

“…The neuroimaging and pathological distinctions between chronic acquired hepatocerebral degeneration (AHD) and genetic hepatolenticular degeneration (Wilson's disease) are well elucidated and applied worldwide [1][2][3][4][5] . In the inherited form there is a metabolic dysfunction which leads to copper deposition in basal ganglia.…”

Challenges in Diagnosing Hepatic Encephalopathy

“…In Parkinson's disease extrapyramidal symptoms will develop more slowly than in patients with cirrhosis-related parkinsonism, will not be accompanied by cerebellar or pyramidal signs and will more often be asymmetric than in cirrhosis-related parkinsonism [9,12]. In addition patients with Parkinson's disease will respond very well to dopaminergic drugs.…”

“…If a patient, however, develops chronic progressive extrapyramidal symptoms a proper analysis of the clinical findings, the patient's medical history and neuroimaging findings must be performed aiming at the differentiation between liver cirrhosis-related parkinsonism and Parkinson's disease or multiple system atrophy (MSA) as a concomitant disorder. The experienced clinician will consider the clinical course, accompanying cerebellar or pyramidal signs, the results of brain imaging, and to some extent the response to treatment [9][10][11][12].…”

Acquired Hepato Cerebral Degeneration – An Atypical Finding in MRI of Brain mimicking Bilateral Ischaemic Stroke

“…His biochemical parameters were altered with WBC count 3.34k/ μL, Platelet count 78k/ μL, Serum bilirubin-2.4 mg/dL, ALT-48 U, AST-63 U, AFP-4.73U, Ammonia-56, PT -19.5 sec, INR 1.66, Albumin -2.1 g/dl, Globulin -4.3g/dl, A:G ratio 0. [2] . The aim of this case report is to highlight the MRI finding of Acute Hepatocerebral Degeneration which often can be underappreciated owing to the possibility of diagnosing a relatively common condition that is Bilateral Ischemic stroke.…”