J. Opp et al., RECURRENT EPISODES OF HYPERPYREXIA CAUSED BY HEREDITARY SENSORIC AND AUTONOMIC NEUROPATHY TYPE-IV, Monatsschrift fur Kinderheilkunde, 146(3), 1998, pp. 209-211
History: Hereditary sensoric and autonomic neuropathy type IV (HSAN IV
) is a rare autosomal recessiv disease. Clinical manifestations includ
e insensivity to pain, unpredictable rises of temperature, anhydrosis,
mental retardation with muscular hypotonia, skin blotching and absenc
e of histamine flare. Case report: In two twins presenting with high f
ever we could confirm this diagnosis. Discussion: In patients presenti
ng with recurrent episodes of otherwise unexplicable fever the lack of
sensivitiy to pain could give the decisive hint to this unusual neuro
pathy.