Peripheral nervous system manifestations of Shiga toxin-producing E. coli-induced haemolytic uremic syndrome in children

• Main Authors: Luisa Santangelo (Author), Giuseppe Stefano Netti (Author), Diletta Domenica Torres (Author), Giovanni Piscopo (Author), Vincenza Carbone (Author), Luciana Losito (Author), Leonardo Milella (Author), Maria Luigia Lasorella (Author), Pasquale Conti (Author), Delio Gagliardi (Author), Maria Chironna (Author), Federica Spadaccino (Author), Elena Bresin (Author), Antonio Trabacca (Author), Elena Ranieri (Author), Mario Giordano (Author)
• Format: Book
• Published: BMC, 2021-09-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.
• Notes: 10.1186/s13052-021-01133-1; 1824-7288
• Keywords: Hemolytic uremic syndrome; Enterohemorrhagic Escherichia Coli; Peripheral nervous system; Eculizumab; Plasma exchange; Neurorehabilitation; Pediatrics; RJ1-570
• Source: Italian Journal of Pediatrics, Vol 47, Iss 1, Pp 1-8 (2021)

Abstract Background The Neurological involvement is the most common extra-renal complication of Shiga toxin-producing E. coli-hemolytic uremic syndrome (HUS) or typical HUS. On brain magnetic resonance examination, main neurological signs encompass acute lesions of the basal ganglia and the white matter, which could usually regress after Eculizumab infusion. In contrast, peripheral nervous system (PNS) manifestations in typical HUS are very rare and, when occurring, they require a careful management of neurological sequelae and an intensive multidisciplinary neuro-rehabilitation program. Case presentation Here, we present two pediatric cases of severe and complicated typical HUS with PNS manifestations who required therapeutic treatment and an intensive multidisciplinary neuro-rehabilitation program. In both cases, PNS manifestations were followed by the recovery from typical HUS-related severe central neurological damage and manifested mainly with marked bilateral motor deficit and hyporeflexia/areflexia in the lower limbs. The peripheral polyneuropathy was treated with immunosuppressive therapy (methylprednisolone boluses, i.v. immunoglobulins, plasma exchange), followed by a prolonged intensive neuro-rehabilitation program. After 8 months of rehabilitation, both patients gained complete functional recovery. Conclusions PNS manifestations during typical HUS are a rare event and potentially leading to severe disability. A timely clinical assessment is mandatory to set up a prompt therapeutic and rehabilitation program and to obtain a complete clinical and functional recovery.