What are the signs and symptoms of hepatic encephalopathy in a patient?

Signs and Symptoms of Hepatic Encephalopathy

Hepatic encephalopathy presents with a wide spectrum of neuropsychiatric and motor manifestations ranging from subtle cognitive deficits detectable only on psychometric testing to coma, with the key clinical markers being personality changes, sleep disturbances, disorientation, asterixis, and altered consciousness. 1

Cognitive and Behavioral Manifestations

Minimal/Covert Hepatic Encephalopathy

• Psychometric abnormalities affecting attention, working memory, psychomotor speed, and visuospatial ability without obvious clinical signs 1
• Occurs in 20-80% of cirrhotic patients but requires specialized testing for detection 1

Progressive Mental Status Changes

• Personality alterations including apathy, irritability, and disinhibition reported by family members 1
• Sleep-wake cycle disturbances with excessive daytime sleepiness (complete reversal is less common than traditionally thought) 1
• Progressive disorientation to time and space 1
• Inappropriate behavior and acute confusional states with either agitation or somnolence 1
• Stupor and coma in advanced stages 1

The ISHEN consensus specifically defines overt hepatic encephalopathy as beginning when disorientation or asterixis appears. 1, 2

Motor System Abnormalities

Asterixis (Flapping Tremor)

• Present in early to middle stages (West Haven Grades 1-2) before stupor develops 1, 2
• Actually a negative myoclonus (loss of postural tone), not a true tremor 1
• Elicited by hyperextending wrists with fingers separated or rhythmically squeezing examiner's fingers 2
• Can be observed in feet, legs, arms, tongue, and eyelids 1, 2
• Disappears in advanced stages (Grades 3-4) as consciousness deteriorates toward coma 2
• Not pathognomonic—also occurs in uremia and other metabolic encephalopathies 1, 3

Pyramidal Signs (Upper Motor Neuron)

• Hypertonia 1, 4
• Hyperreflexia 1, 4
• Positive Babinski sign 1, 4
• Deep tendon reflexes diminish and disappear in coma, though pyramidal signs may persist 1
• Transient focal neurological deficits rarely occur 1
• Seizures are very rare in hepatic encephalopathy 1

Extrapyramidal Signs (Basal Ganglia)

• Hypomimia (reduced facial expression) 1, 4
• Muscular rigidity (lead-pipe or cogwheel quality, distinct from pyramidal spasticity) 1, 4
• Bradykinesia and hypokinesia 1, 4
• Monotonous and slow speech 1, 4
• Parkinsonian-like tremor 1, 4
• Dyskinesia with diminished voluntary movements 1, 4
• Involuntary movements resembling tics or chorea occur rarely 1

Important Clinical Caveats

Staging Challenges

• Mental and motor signs do not necessarily progress in parallel in individual patients, creating difficulties in severity staging 1
• Some patients may have prominent motor abnormalities with relatively mild cognitive dysfunction 1

Special Presentations

Hepatic Myelopathy:

• Related to marked, long-standing portocaval shunting 1
• Paraplegia with progressive spasticity and weakness of lower limbs with hyperreflexia 1
• Relatively mild mental alterations 1
• Does not respond to standard ammonia-lowering therapy but may reverse with liver transplantation 1

Cirrhosis-Associated Parkinsonism:

• Presents in approximately 4% of advanced liver disease cases 1
• Prominent extrapyramidal signs with persistent symptoms 1
• Unresponsive to ammonia-lowering therapy 1

Reversibility Concerns

• While traditionally considered completely reversible, episodes of overt hepatic encephalopathy may be associated with persistent cumulative deficits in working memory and learning 1
• Some mental deficits may persist even after liver transplantation 1

Differential Diagnosis Warning

Patients with localized neurological signs or those not improving with lactulose and rifaximin should be investigated for other causes of altered sensorium, as hepatic encephalopathy typically produces symmetrical findings. 5