[Anesthetic management of patients with neuronal ceroid lipofusucinosis].

Neuronal ceroid lipofuscinoses (NCL) are in a group of autosomal recessive inherited neurodegenerative diseases characterized by the accumulation of autofluorescent storage material in many cell types. Clinical manifestations of NCL are progressive mental and motor deterioration, seizures, and visual loss. We report anesthetic management for two siblings with NCL. Placement of percutaneous endoscopic gastrostomy tubes were scheduled for both 31-year-old woman and her 29-year-old sister with NCL. Although they were treated with several anticonvulsants, the grand mal and myoclonic seizures persisted. Anesthesia was maintained with propofol, nondepolarizing muscle relaxant, and narcotics. They showed no complications except for mild hypothermia during anesthesia. However, BIS index fluctuated widely during anesthesia in both cases. Their postoperative course was uneventful.