title: The neurological syndrome in adults during the 2011 northern German E. coli serotype O104:H4 outbreak.
abstract: The aim of this study was to describe the neurological syndrome in the largest cohort of adult patients with a complicated Shiga toxin-producing Escherichia coli infection. The recent outbreak of Shiga toxin-producing E. coli serotype O104:H4 in northern Germany affected more than 3842 patients, 22% of whom developed haemolytic uraemic syndrome. The proportion of adult patients was unusually high, and neurological complications were frequent and severe. In three hospitals, population-based evaluation of 217 patients with complicated Shiga toxin-producing E. coli infection was carried out, including neurological, neuroradiological, neurophysiological, cerebrospinal fluid and neuropathological analyses. Of the 217 patients with complicated Shiga toxin-producing E. coli infection, 104 (48%) developed neurological symptoms. Neurological symptoms occurred 5.3 days (mean) after first diarrhoea and 4 days after onset of haemolytic uraemic syndrome. Of the infected patients with neurological symptoms, 67.3% presented with cognitive impairment or aphasia. During the course of the disease, 20% of the patients developed epileptic seizures. The onset of neurological symptoms was paralleled by increases in blood urea nitrogen and serum creatinine. In 70 patients with cerebral magnetic resonance imaging, the most common findings were symmetrical hyperintensities in the region of abducens nucleus and lateral thalamus. On follow-up scans, these abnormalities were resolved. Neuropathological analysis revealed regionally accentuated astrogliosis and microgliosis, more predominant in the thalamus and brainstem than in the cortex, and neuronal expression of globotriaosylceramide. There were no signs of microbleeds, thrombotic vessel occlusion or ischaemic infarction. The neurological syndrome in adult patients with complicated Shiga toxin-producing E. coli infection is a rapidly progressive and potentially life-threatening disease necessitating intensive care unit treatment and intubation in >30% of cases. The outcome of neurological patients in the 2011 northern German Shiga toxin-producing E. coli O104:H4 outbreak was surprisingly good. Magnetic resonance imaging and neuropathological findings point to a mixed toxic and inflammatory pathomechanism leading to largely reversible damage of neuronal function.
authors.affiliation: Department of Neurology, University Medical Centre Hamburg-Eppendorf, Martinistr. 52, 20246 Hamburg, Germany. t.magnus@uke.de
authors.forename: Tim
authors.initials: T
authors.lastname: Magnus
authors.forename: Joachim
authors.initials: J
authors.lastname: Röther
authors.forename: Olga
authors.initials: O
authors.lastname: Simova
authors.forename: Maria
authors.initials: M
authors.lastname: Meier-Cillien
authors.forename: Jonas
authors.initials: J
authors.lastname: Repenthin
authors.forename: Fabian
authors.initials: F
authors.lastname: Möller
authors.forename: Joystone
authors.initials: J
authors.lastname: Gbadamosi
authors.forename: Ulf
authors.initials: U
authors.lastname: Panzer
authors.forename: Martina
authors.initials: M
authors.lastname: Wengenroth
authors.forename: Christian
authors.initials: C
authors.lastname: Hagel
authors.forename: Stefan
authors.initials: S
authors.lastname: Kluge
authors.forename: Rolf K
authors.initials: RK
authors.lastname: Stahl
authors.forename: Karl
authors.initials: K
authors.lastname: Wegscheider
authors.forename: Peter
authors.initials: P
authors.lastname: Urban
authors.forename: Bernd
authors.initials: B
authors.lastname: Eckert
authors.forename: Markus
authors.initials: M
authors.lastname: Glatzel
authors.forename: Jens
authors.initials: J
authors.lastname: Fiehler
authors.forename: Christian
authors.initials: C
authors.lastname: Gerloff
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015703:Antigens, CD / Q000378:metabolism; D001806:Blood Urea Nitrogen; D002540:Cerebral Cortex / Q000473:pathology; D015331:Cohort Studies; D016001:Confidence Intervals; D003401:Creatine; D004196:Disease Outbreaks; D004569:Electroencephalography; D004927:Escherichia coli Infections / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology*; D005260:Female; D005858:Germany / Q000453:epidemiology; D006463:Hemolytic-Uremic Syndrome / Q000150:complications / Q000453:epidemiology / Q000209:etiology; D006801:Humans; D007770:L-Lactate Dehydrogenase; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009422:Nervous System Diseases / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology / Q000382:microbiology; D016017:Odds Ratio; D054323:Shiga-Toxigenic Escherichia coli / Q000472:pathogenicity*; D055815:Young Adult
chemical_list: D015703:Antigens, CD; D007770:L-Lactate Dehydrogenase; D003401:Creatine