A 67-year-old, non-alcoholic Japanese female case with
liver cirrhosis, in the course of admission due to
ascites and
rupture of the rectal
varix, was affected by an unusual type of acute progressive
encephalopathy, presenting inattentiveness and slurred speech as initial symptoms. Her consciousness was increasingly clouded. Variable symptoms such as saccadic eye movement, nystagmus, weakness,
hyperreflexia,
dysmetria,
adiadochokinesis and painful
dysesthesia were also noted. Laboratory examination disclosed abnormal liver functions,
hyponatremia,
respiratory alkalosis and normal blood
ammonia. Cerebrospinal fluid was xanthochromic and contained slightly increased
protein. On CT scan, bilateral symmetrical low density areas were demonstrated in the diencephalon, brainstem and cerebellum. A week after the onset, she was
comatose with rigidity of the extremities.
Hyperbilirubinemia and severe
hyponatremia developed. On the second CT, low density areas extended to the cerebral deep white matter. Her respiration became irregular, and she expired 16 days after the onset. Autopsy disclosed edematous lesions with dark brown discoloration in the medial basal ganglia, ventral diencephalon and mesencephalic tegmentum. Less severely affected lesions with pale yellow discoloration extended into the cerebral white matter, pontine and medullar tegmentum and cerebellar dentate nuclei. In the central lesions, diapedesis of erythrocytes and serum-plasma was marked, with
necrosis of the neurons. In the peripheral lesions, diapedesis of less proteinaceous fluid was noted, with less severe neuronal damages. Neither capillary prominence nor
gliosis was remarkable. The clinical and pathological features of the present case bore some similarity to those of Wernicke's and Leigh's
encephalopathies. However, the patient's age, habitus or
clinical course was atypical for the latter.(ABSTRACT TRUNCATED AT 250 WORDS)