Acute Neurological Involvement in Diarrhea-Associated Hemolytic Uremic Syndrome

Abstract

Background and objectives: Neurologic involvement is the most threatening complication of diarrhea-associated hemolytic uremic syndrome (D؉HUS).Design, setting, participants, & measurements: 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.

Central Nervous System Involvement in Adults with Epidemic Hemolytic Uremic Syndrome

“…Some authors report complete clinical and neuroimaging recovery, but so far no reliable predictive parameter for clinical outcome could be established. 15 Hemorrhagic components within acute lesions might be indicative of residual brain lesions at later stages and poorer clinical outcome. 2,28 • Marked areas of increased DWI signal (mostly vasogenic edema) are the most common neuroimaging finding.…”

“…[2][3][4][5][6][7][8][9][10][11][12] Only a very few retrospective studies on larger pediatric patient groups with HUSassociated encephalopathy exist. [13][14][15] The pathophysiology of neurologic complications still remains enigmatic; therefore, the treatment is essentially empiric. 16 Neuroimaging studies in children revealed mainly bilateral symmetric lesions of the basal ganglia 4,6,15 or extensive cortical and/or subcortical lesions, 14 which may or may not be reversible.…”

Lack of the Lectin-like Domain of Thrombomodulin Worsens Shiga Toxin-Associated Hemolytic Uremic Syndrome in Mice

“…Extrarenal manifestations of diarrhea-associated HUS (D+HUS) are increasingly being recognized, with the most common organ affected being the brain (31)(32)(33). Indeed, neurologic involvement in HUS may be an important determinant of morbidity and mortality.…”

Compromiso cerebrovascular agudo en síndrome hemolítico urémico: descripción de dos casos pediátricos

“…Si bien, en estos niños el deterioro neurológico fue evidente, es relevante realizar un estudio neuropsicológico de forma estandarizada a todos los pacientes con manifestaciones neurológicas en el curso de un SHU+D para definir necesidad de terapias específicas. Los estudios retrospectivos de series clínicas con un elevado número de pacientes pediátricos, han constatado compromiso severo del SNC durante el período agudo del SHU+D en el 25,6% de los casos, con déficits persistentes en la quinta parte de ellos y mortalidad del 17%, lo que estaría asociado a lesiones desmielinizantes e isquemia del parénquima encefálico 4,14 .…”

“…En el paciente 2 se confirmó la estenosis de las ramas M1 y M2 de ambas arterias cerebrales medias, probablemente en el contexto de daño endotelial por Stx. Un estudio retrospectivo que incluyó 52 niños con SHU+D y compromiso neurológico severo 14 , demostró una elevada frecuencia de lesiones focales en territorios de circulación terminal. En dicho estudio no hubo correlación estadísticamente significativa entre la localización de la lesión y el pronóstico, a diferencia de nuestros casos donde, las lesiones neuroradiológicas tuvieron una alta concordancia con las secuelas a largo plazo.…”