[Acute encephalopathy with symmetrical, widespread, edematous and necrotic lesions--an autopsy case report].
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)
Mizuguchi
M
M
Department of Neuropathology, University of Tokyo, Japan.
Tomonaga
M
M
D000208:Acute Disease; D000368:Aged; D001921:Brain / Q000473:pathology*; D001927:Brain Diseases / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000473:pathology*; D001929:Brain Edema / Q000473:pathology*; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007888:Leigh Disease / Q000175:diagnosis; D009336:Necrosis; D014057:Tomography, X-Ray Computed; D014899:Wernicke Encephalopathy / Q000175:diagnosis