48 year old male with sudden onset of right sided weakness

This 48 year-old male presented with sudden right sided weakness and hypertension, and on CT was found to have a deep left hemispheric intracerebral hematoma. There were accompanying bilateral hypointensities in the occipital lobes, unchanged 1 day later, when expansion of the hematoma and uncal herniation resulted in death. The past medical history included liver transplantation followed by long term cyclosporin, features of thrombotic thrombocytopenic purpura (TTP) attributed to cyclosporin, type 1 diabetes mellitus and acute renal failure attributed to diabetic nephropathy. There was a 2-month history of hypertension poorly responsive to therapy. The occipital lobes at autopsy showed focal cortical hemorrhages with bilateral discoloration of white matter sparing subcortical zones. Microscopy showed white matter pallor with parenchymal cysts, enlarged perivascular spaces and focal acute hemorrhages consistent with edema secondary to acute hypertension. Cortical hemorrhages were associated with intravascular thrombi and fibrin exudates, as well as an accompanying microinfarct, attributed to TTP. The occipital pathology reflects the posterior leukoencephalopathy syndrome (PLS) combined with cortical lesions of TTP. PLS is usually diagnosed radiologically by occipital hypointensities, and is reversible, so that autopsy pathology is rarely examined. TTP may predispose to the development of the PLS in certain cases.