Hemorrhagic Shock and Encephalopathy Syndrome: Clinical Course and Neurological Outcome

Abstract

The syndrome of hemorrhagic shock and encephalopathy is associated with an acute onset of diarrhea, followed by shock, disseminated intravascular coagulopathy, multiorgan failure, and encephalopathy. The etiology of this syndrome is unknown, and despite intensive treatment, the outcome is often fatal or associated with severe neurological sequelae. Two infants aged 6 and 9 months were admitted with this syndrome within a 24-hour time interval. The authors hereby present the clinical course and a prospective evaluation of the neurological outcome. A review of the literature regarding this infrequent syndrome is presented.

Successful Outcome in an Adult Patient with Influenza-associated Hemorrhagic Shock and Encephalopathy Syndrome

“…Most patients with HSES have a very poor clinical course. Up to 60% of patients with HSES reportedly have died, and 30% of survivors had neurological sequelae ( 2 ).…”

“…The abovementioned mechanism of a BBB transporter disorder may be involved in the pathogenesis. Hyperpyrexia has often been also suspected as an important factor that induces HSES; however, some reports have contradicted this hypothesis because, in some cases, there was no high fever upon admission ( 2 ). Indeed, the patient in our case did not present with a high fever on admission.…”

“…Some reports have attributed the cause and severity of HSES to the individual's susceptibility, not the thermal environment. Heat-shock proteins, which protect organisms from a variety of stresses, including hyperthermia, may be constitutionally or transitionally defective in patients with HSES ( 2 ).…”

“…In 1983, hemorrhagic shock and encephalopathy syndrome (HSES) was proposed by Levin et al as a severe subtype of infection-associated acute encephalopathy, where patients present with neurological disorder accompanied by gastrointestinal bleeding ( 1 ). In most cases, HSES occurs in children; the mortality rate has been reported to be approximately 60%, and most of the surviving patients suffered from sequelae ( 2 ). Few patients have experienced HSES triggered by influenza infection, and an adult onset of HSES is especially rare.…”

Hemorrhagic shock and encephalopathy syndrome – the markers for an early HSES diagnosis

“…In these cases, the first problem the physician is faced with is the difficulty of the differential diagnosis. Some reports have indicated in the differential diagnosis the diseases of the toxic shock syndrome, the hemolytic-uremic syndrome, and Reye's syndrome, among others [ 6 - 8 ]. In our patients, those diseases were excluded because of the lack of skin or mucosal manifestations, hemolysis, and/or blood ammonia levels.…”