Portosystemic Encephalopathy Clinical Presentation

Updated: Sep 17, 2019
  • Author: Gagan K Sood, MD; Chief Editor: BS Anand, MD  more...
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Patients with portosystemic encephalopathy may present with vague complaints, such as fatigue, or they may be brought for evaluation by relatives. Indeed, the family members of patients often are the most useful historians. They may describe restlessness (especially at night), somnolence (typically during the day), and instances reflecting episodic confusion.

No neurologic features are entirely specific for this disorder.

Clinical history or physical examination findings suggestive of liver disease, without evidence of another etiology for neurologic dysfunction, form the basis for the diagnosis.

A precipitating factor identified from the clinical history strongly suggests the diagnosis. Therefore, directed questioning in this regard is essential.

Several predisposing conditions also may contribute to the development of chronic portosystemic encephalopathy.


Physical Examination

As with many toxic and metabolic encephalopathies, mental status and, in particular, the level of consciousness may fluctuate dramatically. In addition to impairment of the level of consciousness, patients with portosystemic encephalopathy (PSE) may demonstrate a variety of neurologic signs together or in isolation.

PSE grades are as follows:

  • Subclinical PSE: Early in the course of the condition, patients may appear normal clinically but perform poorly on psychometric testing. This is termed subclinical PSE. It may be particularly important to diagnose subclinical PSE in patients performing tasks that require rapid reaction times because these are prolonged in affected patients. Judgment may also be erratic or questionable. Recognition of subtle impairment, therefore, may lead to a recommendation to avoid driving a motor vehicle or operating machinery.

  • Grade 1 PSE: With deterioration to grade 1 PSE, patients have difficulty with memory, mild confusion, agitation, and irritability. Other complaints may include restlessness or sleepiness during the day and remaining awake at night. Tremor, a rhythmic or regular oscillation, and incoordination may be seen. Handwriting skills are typically impaired. Constructional apraxia may be easily demonstrated at the bedside by requesting the patient to draw a five-pointed star.

  • Grade 2 PSE: Progression to grade 2 PSE involves a slowing of mentation and speech, with the appearance of lethargy. The patient's confusion progresses to difficulty with orientation to time, and loss of inhibition ultimately may result in inappropriate behavior. Neurologic signs include asterixis and an irregular flapping tremor that is observed best with the wrists dorsiflexed and fingers spread. Dysarthria, ataxia, and hypoactive deep-tendon reflexes are characteristic.

  • Grade 3 PSE: This grade portends the significant possibility of coma; therefore, plans to perform endotracheal intubation should be made upon recognition of this state. Patients are drowsy but can be woken up; however, they remain markedly confused. They may exhibit frankly aggressive behavior. Asterixis persists, but the deep-tendon reflexes become hyperactive as they are disinhibited, a process that culminates in the development of the decerebration seen in grade 4 PSE. Babinski signs may be seen.

  • Grade 4 PSE: This also is known as hepatic coma and represents a medical emergency. Patients are unable to protect their airway reliably. Prophylactic endotracheal intubation is mandatory. Patients may remain unresponsive for several days.

  • Stigmata of chronic liver disease and portal hypertension may be evident upon examination. These include the presence of gynecomastia and testicular atrophy in males, palmar erythema, spider nevi, splenomegaly, caput medusae, ascites, peripheral edema, and a shrunken liver.

More specific signs resulting from the underlying liver diseases also may be present, as follows:

  • The Kayser-Fleischer rings of Wilson disease are typically identified only with a slit-lamp examination.

  • Bronzed skin and arthropathy are seen in hereditary hemochromatosis.

  • Cholesterol deposition may be seen as xanthelasma in patients with chronic cholestasis of any cause, but it often is most striking in primary biliary cirrhosis.

  • Alcoholic liver disease is frequently accompanied by advanced malnutrition; however, any long-standing liver disease also may lead to temporal, shoulder girdle, and hip girdle muscle wasting.

  • Cutaneous features of hepatitis C virus infection include a characteristic rash due to leukocytoclastic vasculitis resulting from mixed cryoglobulinemia, porphyria cutanea tarda, and lichen planus.