Introduction. Hemorrhagic shock and encephalopathy syndrome (HSES) is a rare
disorder with prevalence at the early age. The main features of HSES are:
acute diarrhea, shock, disseminated intravascular coagulation, multisystem
impairment, and encephalopathy. The prognosis is very poor, with high
mortality, especially in the cases with status epilepticus. Case outline. The
presented infant had typical features of HSES associated with superrefractory
status epilepticus as de novo epileptic event, followed by parmacoresistant
epilepsy. Clinical course of disease was very severe and required urgent
circulatory and respiratory support, and at the same time, managing of
super-refractory status epilepticus by continuous intravenous infusion of
midazolam, barbiturate and levetiracetam. The outcome was very poor with
serious neurological consequence and resistant epileptic seizures.
Conclusion. The treatment of the presented patient with HSES was very
challengeable due to life threatening condition associated with
superrefractory status epilepticus, and further pharmacoresistant epilepsy.
Additionally, the choice of antiepileptic drugs is limited due to multisystem
impairment and adverse effects, which might worsen, anyhow, severe course of
disease.