Central Nervous System Involvement in Adults with Epidemic Hemolytic Uremic Syndrome

Abstract

SUMMARY:Hemolytic uremic syndrome is a multisystem disorder that is caused by infection with Shiga-toxin-producing Escherichia coli. HUS affects mainly children and is rare among adults. This retrospective case series analyzes clinical signs and MR imaging findings of 11 adult patients with HUS associated nervous system involvement during the epidemic EHEC outbreak in northern Europe with its epicenter in Hamburg in May 2011. The most prevalent imaging finding was symmetric pointy vasogenic edema of the brain stem in the acute and subacute phases of the disease (n ϭ 5). One patient exhibited additional symmetric mesiotemporal signal changes mimicking limbic encephalitis. Two patients developed subcortical patchy lesions, and 4 subjects did not present with any signal changes. Remarkably, territorial ischemia, signs of hemorrhage, or blood-brain barrier disruption have not been detected. While brain stem lesions were transient and normalized with clinical recovery, supratentorial lesions did not resolve completely at 2-month follow-up examination.ABBREVIATIONS: EHEC ϭ enterohemorrhagic Escherichia coli; HUS ϭ hemolytic uremic syndrome; PRES ϭ posterior reversible encephalopathy syndrome;

Coma caused by Shigella toxin-producing Escherichia coli in the absence of haemorrhagic uraemic syndrome (HUS)

“…In 2011 an outbreak of STEC occurred in Western Europe, with around 3,000 cases of Shigella toxin-mediated gastro-enteritis reported in Germany alone. While HUS is a common complication of STEC, studies based on the 2011 outbreak reported neurological symptoms in unusually high numbers of patients (26-48%) [2][3][4] . Reported symptoms included tremors, seizures and neuropsychiatric disorders.…”

“…The diagnosis of HUS relies primarily on laboratory findings, but the diagnosis of neurological symptoms related to STEC is mainly based on the clinical presentation combined with imaging studies with a preference for MRI. Lesions on T2-weighted and diffusion-weighted MRI reported in the literature include symmetrical hyperintensities in the region of the sixth cranial nerve, the thalamus, and juxtacortical and periventricular white matter [2,4] . Generally, only supportive treatment is given for STEC-associated encephalopathy [2,4] .…”

“…Lesions on T2-weighted and diffusion-weighted MRI reported in the literature include symmetrical hyperintensities in the region of the sixth cranial nerve, the thalamus, and juxtacortical and periventricular white matter [2,4] . Generally, only supportive treatment is given for STEC-associated encephalopathy [2,4] . Several studies have suggested eculizumab as an experimental treatment [4,5] .…”

“…Recent studies on STEC epidemics report a high prevalence of neurological symptoms with a rapid degenerative course, often requiring intubation and admission to the intensive care unit [1][2][3][4][5] . While most of these cases occur with concurrent HUS, we describe a patient with severe neurological symptoms due to infection with STEC in the absence of HUS.…”

Recovery of thalamic microstructural damage after Shiga toxin 2-associated hemolytic–uremic syndrome

“…It comprises acute kidney injury, thrombocytopenia (platelet count b 150.000 cells/mm 3 ), and microangiopathic hemolytic anemia [2]. HUS is the most common cause of acute renal failure in childhood [3][4][5][6][7] and occurs predominantly in children younger than five years [2,[6][7][8][9][10][11]. Between May and July 2011, the world's largest reported HUS outbreak affected northern Germany [8,12,13].…”