INVOLVEMENT OF THE CENTRAL-NERVOUS-SYSTEM IN HEMOLYTIC-UREMIC SYNDROME AND THROMBOTIC-THROMBOPENIC PURPURA

Cerebral involvement is typical for thrombotic microangiopathies like haemolytic uremic syndrome (HUS) and thrombotic-thrombopenic purpura ( Moschcowitz disease or TTP). Symptoms are irritation, restless behavio ur, disorientation, disturbance of consciousness, seizures, and focal neurological deficits. The lack of typical imaging changes or patholog ical observations may explain the unknown pathophysiological cascade l eading to the neurological symptoms. We describe the development of HU S/TTP in a 52-year-old woman after acute pneumonia caused by Diplococc us pneumoniae. The patient showed an increasing psyche-organic syndrom e with disorientation, followed by severe loss of consciousness and co ma, Initially, computed tomography showed slight diffuse brain oedema, which was not found in later follow-up images, Magnetic resonance ima ging was normal. The TCD examination revealed general velocity increas es and vasospasms (especially MCA, ACA and PCA bilateral and BA), The reduction in blood flow velocities in the basal arteries was accompani ed by a marked clinical improvement, The development of vasospasms may be an explanation for the neurological deficits in HUS/TTP, The origi n of the vasospasms may be found in disturbed prostacyclin production, increased serotonin or platelet factor IV release, and leucocyte acti vation with consecutive endothelial damage.