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.

Figure 1.

Patient 49: MRI findings at day 3 in a 3-year-old girl with severe coma, pyramidal and extrapyramidal symptoms, and cortical blindness suspected on day 15 of evolution. (a and b) Fluid-attenuated inversion recovery axial view on the third day after admission shows bilateral and symmetrical high-intensity signal in the lateral geniculate bodies (1), posterior limbs of internal capsule (3), thalamus (4), and medial occipital cortex (5) as well as temporal insulae (2), including external capsule, claustrum, and extreme capsule. (c) Diffusion-weighted imaging shows hypersignal in lateral the geniculate bodies (1) and insulae (2), only areas demonstrating a restricted diffusion as a result of cytotoxic edema. (d) Noncontrast coronal T1-weighted view shows a spontaneous hyperintense signal in the lateral geniculate bodies (1), probably as a result of hemorrhage.

Figure 2.

Patient 49: MRI findings at day 15 (a) and 6 months later (b). (a and b) Fluid-attenuated inversion recovery axial view. At day 15 (a), hypersignal persists only in the previous restricted diffusion areas. Six months later (b), slight hypersignal in the left insula and the geniculate bodies is still visible (the child had at that time disturbed color vision).