Focal Neurological Syndromes in Hepatic Failure.

THE neuropsychiatric disturbances occurring in patients with liver disease have been recognized since the time of the Babylonians (Sherlock, I958). Only in the last ten years, however, have their clinical and biochemical significances been appreciated (Walshe, I95 I) (Adams and Foley, 1953). The clinical and EEG findings have been described in detail by Sherlock (I955), Davidson and Summerskill (I956) and Parsons Smith, Summerskill, Dawson and Sherlock (I957). These are characterized by:i. Mental symptoms: lack of awareness, euphoria and apathy, associated with inversion of sleeprhythm and drowsiness which may lead to delirium and coma. In the precoma stage, childish and inappropriate behaviour, as well as defects of recent memory, are common. 2. Motor signs. the most characteristic is a coarse irregular flapping tremor with frequency of 6-9/sec. Other signs include a rigidity of 'cog wheel' or 'lead-piping' type, masking of the facies, dysarthria and increased tendon jerks. Agnosia, usually spatial or visual, is seen less commonly, and constructional apraxia is another manifestation commonly seen. In the coma stage, bilateral clonus and extensor plantar responses may occur. 3. Electroencephalographic (EEG) findings. indicate diffuse cerebral damage and take the form of bilaterally synchronous and symmetrical high voltage 2-per-second slow waves. (Foley, Watson and Adams, I950; Read, Laidlaw and Sherlock, 1961, Parsons Smith, and others, I957. Laidlaw 1959, Laidlaw and Read, I96I). These changes are associated with a high blood and C.S.F. ammonia level and less consistently with an increase in glutamic acid glutamine in the C.S.F. (White, Phear, Summerskill and Sherlock, 1955; Whitehead and Whittaker, I955; Booth, Swadey, Fiol, Klein and Hall, I963). The neurological findings are usually of a generalized nature, with a notable absence of focal signs, as indeed would be expected in a diffuse metabolic disorder. The following cases are reported because they show that occasionally focal signs may occur in patients with hepatic precoma or coma, and although their mechanism is obscure, their recognition at the bedside is important.