B. Thebaud et al., Haemorrhagic shock and encephalopathy syndrome: neurological course and predictors of outcome, INTEN CAR M, 25(3), 1999, pp. 293-299
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.