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Central nervous system involvement in adults with epidemic hemolytic uremic syndrome.

ABSTRACT: 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 = 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.

SUBMITTER: Wengenroth M

PROVIDER: S-EPMC7964673 | biostudies-literature | 2013 May

REPOSITORIES: biostudies-literature

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Central nervous system involvement in adults with epidemic hemolytic uremic syndrome.

Wengenroth M M Hoeltje J J Repenthin J J Meyer T N TN Bonk F F Becker H H Faiss S S Stammel O O Urban P P PP Bruening R R

AJNR. American journal of neuroradiology 20130110 5

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 ...[more]

PMID: 23306013

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Project description:In typical hemolytic uremic syndrome (HUS) approximately 25% of patients show central nervous system (CNS) involvement often leading to serious long-term disabilities. We used the C5-complement inhibitor Eculizumab as rescue therapy. From 2011 to 2014, 11 children (median age 22 months, range 11-175) with enterohemorrhagic Escherichia coli-positive HUS requiring dialysis who had seizures (11/11) and/or were in a stupor or coma (10/11) were treated with Eculizumab. Two patients enrolled on the Safety and Efficacy Study of Eculizumab in Shiga-Toxin Producing E coli Hemolytic-Uremic Syndrome (STEC-HUS) each received 6 doses of Eculizumab, 3 patients 2 doses, and 6 patients 1 dose. Laboratory diagnostics of blood samples and magnetic resonance imaging (MRI) were performed as per center practice. Data were analyzed retrospectively. Cranial MRI was abnormal in 8 of 10 patients with findings in the basal ganglia and/or white matter. A 2-year-old boy with severe cardiac involvement and status epilepticus needed repeated cardio-pulmonary resuscitation and extracorporeal membrane oxygenation. He died 8 days after start of Eculizumab treatment. Two patients with hemorrhagic colitis and repeated seizures required artificial ventilation for 6 and 16 days, respectively. At the time of discharge, 1 patient showed severe neurological impairment and 1 mild neurological impairment. The 8 surviving patients experienced no further seizures after the first dose of Eculizumab. Three patients showed mild neurological impairment at discharge, whilst the remaining 5 showed no impairment. The platelets normalized 4 days (median) after the first dose of Eculizumab (range 0-20 days). The mean duration of dialysis after the first dose of Eculizumab was 14.1 ± 6.1 days. In children with typical HUS and CNS involvement early use of Eculizumab appears to improve neurological outcome. In severe HUS cases which progress rapidly with multiple organ involvement, late treatment with Eculizumab seems to show less benefit. We speculate that prophylactic Eculizumab therapy before development of neurological symptoms could be advantageous.

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Central nervous system involvement in adults with epidemic hemolytic uremic syndrome.

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Central nervous system involvement in adults with epidemic hemolytic uremic syndrome.

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