Parents' knowledge about the management of fever in children has hardly changed over the past few years in spite of a host of educational interventions aimed at improving awareness of the dangers relating to high fever. Unexpected death in children's febrile diseases is considered to be relatively rare and may mostly occur due to meningitis, bronchiolitis, malaria, acute leukaemia and Kawasaki syndrome. Two rare autosomal recessively inherited diseases can present high fever bouts in the neonatal period, which might represent a serious threat due to their intrinsic risk of unpredictable sudden death: Sohar–Crisponi syndrome (SCS, OMIM 601378) and Stüve–Wiedemann syndrome (SWS, OMIM 601559).

SCS is already evident at birth with paroxysmal muscular contractions, resembling neonatal …