Anesthesia management in a child with Neuronal Ceroid Lipofuscinoses (batten syndrome) Nöral seroi̇d li̇pofusi̇nozi̇sli̇ (batten sendromu) hastada anestezi̇

UZUN Ş., Altinel S., Erden I. A., AYPAR Ü.

Anestezi Dergisi, vol.21, no.3, pp.187-189, 2013 (Scopus) identifier

  • Publication Type: Article / Article
  • Volume: 21 Issue: 3
  • Publication Date: 2013
  • Journal Name: Anestezi Dergisi
  • Journal Indexes: Scopus, TR DİZİN (ULAKBİM)
  • Page Numbers: pp.187-189
  • Keywords: Anesthesia general, Arrythmias cardiac, Bradycardia, Infantile neuronal lipofuscinoses
  • Lokman Hekim University Affiliated: Yes


Neuronal Ceroid Lipofuscinoses (NCL) is a group of rare autosomal recessive neurodegenarative disorder seen in children, which is characterized by lisosomsal accumulation of autofluorescent materials. During anesthesia, hypothermia and bradycardia are often seen in patients with this diagnosis. Generally, in the patients who are diagnosed as NCL, hypothermia and bradycardia are frequently observed during anesthesia. Malign hyperthermia and difficult intubation are not specific for anesthesia of this syndrome. Succinylcholine should be avoided in the myotonic cases. For muscle relaxation, non depolarizing agents could be used. In this case, the administration of anesthesia in a child with Neuronal Ceroid Lipofuscinoses during tracheotomy was reported.