Background: Glutaric aciduria type 1 (GA1) is a rare hereditary metabolic disorder caused by a deficiency of glutaryl-CoA dehydrogenase; it has an estimated overall prevalence of 1 in 100 000 newborns. 
Case report: The authors describe a clinical case of a 20-year-old male patient, with a dystonic-dyskinetic syndrome secondary to GA1, who was submitted to elective laparoscopic cholecystectomy under general anaesthesia with sevoflurane. 
Discussion: In these patients it is important to prevent acute encephalopathic crises while undergoing surgery. To the best of our knowledge, there are no definite reports on the superiority of one anaesthetic drug over another. 
The main question is whether to use volatile anaesthetic agents or not. Some authors argue against the use of volatile anesthetics because of the possible association between mitochondrial dysfunction and malignant hyperthermia.  
On the other hand, more recent literature rules out episodes of malignant hyperthermia and other intraoperative events attributable to the general anesthesia with sevoflurane in paediatric patients with mitochondrial disease and there are also concerns about the possibility of occurrence of propofol infusion syndrome and severe metabolic acidosis. We chose to use sevoflurane as maintenance agent and the anaesthetic course was uneventful. 
References:
1.	K. Stefan et al; Diagnosis and management of glutaric aciduria type I – revised recommendations; J Inherit Metab Dis (2011) 34:677–694
2.	H.P. Joaquin et al; Anesthetic management in two siblings with glutaric aciduria type 1; Pediatric Anesthesia (2006) 16: 188–191
3.	W.N. Teng et al; Anesthetic management of comprehensive dental restoration in a child with glutaric aciduria type 1 using volatile sevoflurane; Journal of the Chinese Medical Association 77 (2014) 548-551
Learning points: Although the described case was successful, the anaesthetic management of patients with metabolic disorders like GA1 remains challenging and more clinical data is necessary.

Glutaric aciduria type 1: anaesthetic management of an adult patient for elective laparoscopic cholecystectomy