Portal‐systemic shunt encephalopathy presenting with diffuse cerebral white matter lesion: An autopsy case

Abstract

We report herein an autopsy case of portal-systemic encephalopathy (PSE) presenting with diffuse tissue rarefaction in the cerebral deep white matter. Clinically, the patient showed recurrent episodes of unconsciousness, abnormal behavior and urinary incontinence, as well as flapping tremor. Cognitive impairment and peripheral neuropathy developed following recurrent episodes. Although conventional arterial portography revealed a small portal-systemic collateral vessel of a left gastro-renal venous shunt, abdominal CT and liver biopsy showed no evidence of liver cirrhosis and serum ammonia level showed a mild increase. T2-weighted MRI demonstrated symmetrical signal hyperintensities in the deep white matter. Neuropathological findings showed Alzheimer type II astrocytes in the deep layers of the cerebral cortices and severe tissue rarefaction with no or slight reactive astrocytosis in the subcortical and deep white matter. These white matter changes have been reported infrequently in patients with PSE. The present case suggests that chronic PSE without liver cirrhosis may develop diffuse white matter lesions.

Acute Liver Failure Induces Glial Reactivity, Oxidative Stress and Impairs Brain Energy Metabolism in Rats

“…In parallel, astrocytic reactivity may be a defense mechanism to modulate brain homeostasis by increasing astrocytic workload, and it may contribute to the elevation of brain pressure due to increased cellular volume. This process has been described in several cerebral diseases, including experimental and human HE (Pilbeam et al, 1983;Kimura et al, 2008). In this manuscript, we describe severe astrocytic morphological changes that characterize a state of diffuse astrocytic reactivity.…”

Systematic review of congenital and acquired portal-systemic shunts in otherwise normal livers

“…Several studies used Doppler ultrasound imaging and scintigraphy to measure shunt rate and size, with many reporting the PSS to be ‘large’ or with a diameter ranging from 0·2 to 5 cm. It has been suggested that encephalopathy is not associated with PSSs, even in patients with cirrhotic livers, if the shunted fraction is less than 24–30 per cent.…”

Νευροχημικά Και Απεικονιστικά Χαρακτηριστικά Ασθενών Με Επαυξημένα Παρκινσονικά Σύνδρομα

“…Πλην της αρχετυπικής εκδήλωσης της φλοιοβασικής εκφύλισης με φαινότυπο φλοιοβασικού συνδρόμου, ήδη από πολλών ετών είχαν αναγνωρισθεί και άλλες εκδηλώσεις του νοσήματος, όπως με προεξάρχουσα άνοια ή αφασία 119,120 , με προοδευτική μείωση του παραγόμενου λόγου και στοματοπροσωπική δυσπραξία 121 , ως πρωτοπαθής προϊούσα αφασία, συνηθέστερα με τη μορφή μη ρέουσας αγραμματικής μορφής 122,123 , αλλά και ως αφασία αγωγιμότητας, με προεξάρχουσα δυσκολία στην επανάληψη 124 . Επίσης έχει αναφερθεί εκδήλωση ως οπίσθια φλοιώδης ατροφία, με οφθαλμοκινητική απραξία, οπτική αταξία και ταυτοχροναγνωσία 125 .…”