Acute neurology and neurophysiology of haemolytic-uraemic syndrome
Acute neurology and neurophysiology of haemolytic-uraemic syndrome. Eriksson KJ, Boyd SG, Tasker RC. Archives of Disease in Childhood 2001;84:434-435.
Involvement of the central nervous system (CNS) is found in about 30% of children with hemolytic uremic syndrome (HUS). The most common acute problem is generalized or partial seizure activity in more than half of those with neurological signs. Early CNS involvement is also a major contributor to chronic neurological morbidity. This study reviewed the clinical and neurophysiological features of 22 children treated at a London children’s hospital. All children had initial gastrointestinal symptoms, with positive stool cultures in five patients (E. coli O157:H7 in three). Five (22%) died in the acute phase of HUS, with a mean of eight days between onset of symptoms and death. On follow-up of the surviving patients, seven had neurological sequelae and 10 had no long-term neurological problems. Seizures, alone or as part of neurological involvement, were associated with mortality or long-term neurological sequelae. In terms of early neurophysiology, survivors with early generalized slowing in electroencephalographic (EEG) activity alone did not have any long-term neurological sequelae. Periodic EEG findings were associated with either death (three cases) or multiple neurological impairments (two cases). Slowing of EEG activity lateralized to one hemisphere of the brain led to moderately severe cognitive deficit in one patient and hemiparesis and dysphasia in another. Focal discharges in the temporal region were associated with epilepsy with complex partial seizures. Two other patients developed visual impairments. Certain neurophysiological involvement in children with HUS, then, may indicate long-term neurological outcomes, particularly when these are localized to the occipital or temporal regions.