Acute neurological involvement in diarrhea-associated hemolytic uremic syndrome.
OBJECTIVE
Neurologic involvement is the most threatening complication of diarrhea-associated hemolytic uremic syndrome (D+HUS).


METHODS
We report a retrospective multicenter series of 52 patients with severe initial neurologic involvement that occurred in the course of D+HUS.


RESULTS
Verotoxigenic Escherichia coli infection was documented in 24. All except two patients had acute renal failure that required peritoneal dialysis, hemodialysis, or both techniques. A first group of eight patients remained with normal consciousness; five of them had protracted seizures. A second group of 23 patients had stuporous coma; five of these had protracted severe seizures, and 18 had a neurologic defect including pyramidal syndrome, hemiplegia or hemiparesia, and extrapyramidal syndrome. A third group of 21 patients had severe coma. Plasma exchanges were undertaken in 25 patients, 11 of whom were treated within 24 hours after the first neurologic sign; four died, two survived with severe sequelae, and five were alive without neurologic defect. Magnetic resonance imaging (MRI) for 29 patients showed that (1) every structure of the central nervous system was susceptible to involvement; (2) no correlation seemed to exist between special profile of localization on early MRI and the final prognosis; and (3) MRI did not exhibit any focal lesions in three patients. The overall prognosis of the series was marked by the death of nine patients and severe sequelae in 13.


CONCLUSIONS
Neurologic involvement is associated with a severe renal disease but does not lead systematically to death or severe disability.
Nathanson
Sylvie
S
Pediatric Unit, Hôpital Mignot, 177 rue de Versailles, F-78150 le Chesnay, France. sylvie.nathanson@gmail.com
Kwon
Thérésa
T
Elmaleh
Monique
M
Charbit
Marina
M
Launay
Emma Allain
EA
Harambat
Jérôme
J
Brun
Muriel
M
Ranchin
Bruno
B
Bandin
Flavio
F
Cloarec
Sylvie
S
Bourdat-Michel
Guylhene
G
Piètrement
Christine
C
Champion
Gérard
G
Ulinski
Tim
T
Deschênes
Georges
G
D058186:Acute Kidney Injury / Q000382:microbiology* / Q000401:mortality / Q000628:therapy; D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003128:Coma / Q000382:microbiology; D003967:Diarrhea / Q000382:microbiology* / Q000401:mortality / Q000628:therapy; D004185:Disability Evaluation; D004421:Dystonia / Q000382:microbiology; D004927:Escherichia coli Infections / Q000150:complications / Q000382:microbiology* / Q000401:mortality / Q000628:therapy; D005260:Female; D005602:France; D006463:Hemolytic-Uremic Syndrome / Q000382:microbiology* / Q000401:mortality / Q000628:therapy; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D009422:Nervous System Diseases / Q000175:diagnosis / Q000382:microbiology* / Q000401:mortality / Q000628:therapy; D010291:Paresis / Q000382:microbiology; D010530:Peritoneal Dialysis; D010951:Plasma Exchange; D006435:Renal Dialysis; D012189:Retrospective Studies; D012640:Seizures / Q000382:microbiology; D012720:Severity of Illness Index; D054323:Shiga-Toxigenic Escherichia coli / Q000472:pathogenicity*; D013997:Time Factors; D016896:Treatment Outcome