Haemorrhagic shock and encephalopathy syndrome: neurological course and predictors of outcome

The haemorrhagic shock and encephalopathy syndrome (HSES) is a devastating disease. The aetiology of this syndrome is unknown, and, despite intensive treatment, the outcome is often fatal or associated with severe neurologica l sequelae. Objective: To assess the neurological features and potential prognostic mar kers of the disease. Design: Retrospective study. Setting: Division of Neuropaediatrics in a chi ldren's university hospital. Patients and methods: Fourteen patients f'ullfilling the HSES criteria out of 42 children admitted with fever and shock to the Paediatric Intensive Ca re Unit between 1986 and 1994, were analysed for clinical, biological, neur oradiological, EEG and neuropathological findings. Results: The patients (a ge range from 2 to 33 months) were found at night or in the morning either comatous (n = 3) or convulsing (n = 11). All but one were healthy before ad mission, although eight had had a brief prodromal infectious disease. All w ere febrile (mean body temperature 39.9 degrees C +/- 0.9 degrees). Seasona l clustering during the winter months was observed. Coma and seizures with frequent status epilepticus were the main neurological manifestations. All children recovered from their multiple organ failure within a few days. Sev en died (50 %); four survivors had neurological sequelae (29 %) with a deve lopmental quotient (DQ) of 50 % or less in three and a DQ of 75 % in one an d three infants (21%) had normal outcomes. Computed tomography (CT) display ed a diffuse area of low density mainly in the cerebral cortex and intraven tricular and parenchymal haemorrhages. Magnetic resonance imaging (MRI) sho wed haemorrhagic cortical lesions. Postmortem examination of the brain cond ucted in three patients showed necrotic and haemorrhagic lesions, mainly in cortical areas. Comparison of the children with adverse outcome (death or neurological sequelae) with those with normal outcome revealed that predict ors of poor outcome were status epilepticus (p = 0.003) and coma for more t han 24 h (p = 0.01). Infants without disseminated intravascular coagulation , without a biphasic course and without brain hypodensities or haemorrhages on CT scans performed at least 4 days after onset had a normal neurodevelo pmental outcome. Conclusion: The central nervous system appeared to be the main target of th e HSES lesions. The most common outcome was brain death or severe brain dam age. Further studies with a larger sample are necessary to determine whethe r the prognostic indicators we identified are reliable.