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Central nervous system involvement in hemolytic uremic syndrome (HUS)

Central nervous system involvement in hemolytic uremic syndrome (HUS)—a retrospective analysis of cerebral CT and MRI studies. Theobald I, Kuwertz-Broking E, Schiborr M, Heindel W. Clinical Nephrolology 2001;56(6):S3-8.

ABSTRACT:

Cerebral involvement is one of the major complications of hemolytic uremic syndrome (HUS). According to the literature, basal ganglia involvement in HUS is common and quite often associated with other cerebral pathologies. To evaluate the morphological changes of cerebral involvement in children with HUS utilizing CT and MRI studies, this study retrospectively analyzed 13 cranial CTs (CCT) and three cranial MRI studies of five of 93 patients with clinically proven HUS and severe central nervous system (CNS) involvement (seizures and coma and dysregulation of breathing) referred to the department of pediatric nephrology between 1987-2000. Three of five patients had CT and MRI studies; two patients had CT scans only. One of two patients with isolated basal ganglia ischemia and a normal first CCT developed a secondary hemorrhagic infarction. Another patient with an initially normal MRI developed an infarction of the right cerebral arteries with mass effects. One of two patients with basal ganglia involvement showed additional infarction of thalami and external and internal capsules whereas the other had only minimal involvement of adjacent white matter, but consecutive hemorrhagic infarction. Four of five children died (three of them with varying extents of basal ganglia and adjacent white matter involvement, one with right cerebral artery infarction). Basal ganglia involvement was found in the majority of cases as well as in all lethal cases. The surviving patient with isolated basal ganglia involvement now suffers from tetraspastic disorder and convulsions. The authors conclude that first imaging findings may not show pathologies. Contradictory to previous reports, even children with isolated basal ganglia pathology and/or less involvement of white matter and coma may either die from the underlying disease or their clinical outcome may be poor.