[MIM 540 000]

Acronym for Mitochondrial Encephalomyopathy with Lactic Acidosis and Stroke-like episodes.

Incidence: 16-18/100.000. Mitochondrial cytopathy usually due to a mutation in the mitochondrial DNA. Approximately 80 % of patients are carriers of the mutation 3243A>G in leucine transfer RNA gene (tRNA Leu) .

Clinical presentation of progressive degenerative

neuropathy characterized by:

-        episodes of repeated non-ischemic stroke (focal MRI or CT abnormalities) resulting in a transient neurological deficit (aphasia, paresis, hemianopia) the recovery of which is complete at the onset of the affection but leaving more serious sequelae as the disease progresses,

-        lactic acidosis,

-        the presence of red ragged fibers at muscle biopsy,

-        epilepsy, dementia, chronic migraine type headache

-        cardiac conduction disturbances (AVB, Wolf-Parkinson-White) hypertrophic cardiomyopathy that progresses slowly to dilated cardiomyopathy.

Quite frequently: diabetes mellitus, gastrointestinal disorders, malnutrition.

Anesthetic implications:

anesthetic management  of a mitochondrial cytopathy (see this topic), which implies, in principle: preoperative ECG and echocardiography, checking the patient’s basal blood lactate levels, monitor blood glucose and blood lactate, avoiding the use of lactate-containing IV solutions (ideal: Plasmalyte&®), monitor the curarization, avoiding succinylcholine in case of muscle injury, avoiding the use of a continuous propofol infusion (increased risk of PRIS ?) , avoid hypo- and hyperthermia. There is no increased risk of malignant hyperthermia.

In a retrospective series of MELAS cases, all anesthetics including total propofol based IV anesthesia as well as Ringer's lactate solution have been used without causing evident complications. The authors found a high incidence of hyponatremia associated with hyperkalemia that may be due to adrenal insufficiency. It is therefore recommended to check pre- and post-operative electrolytes. Several cases of gastroparesis have been described in this series: full stomach situation ! Remimazolam could be an interesting option.

A case of major degradation with convulsions and metabolic acidosis has been reported in a young girl who had received a dose of propofol (1.25 mg/kg) in the absence of  glucose intake for an MRI done in the context of a recent neurological problem: it is possible that this would have happened without any administration of propofol but this requires caution in case of anesthesia in a context of neurological degradation.

References : 

-        Bolton P, Peutrell J, Zuberi S, Robinson P. 
Anaesthesia for an adolescent with mitochondrial encephalomyopathy-lactic acidosis-stroke-like episodes syndrome. 
Paediatr Anaesth 2003 ; 13 : 453-6.

-         Maurtua M, Torres A, Ibarra V, DeBoer G, Dolak J. 
Anesthetic management of an obstetric patient with MELAS syndrome: case report and literature review. 
Int J Obstetr Anesth 2009; 17: 370-3.

-         Blair MT
Neuraxial anaesthesia in MELAS syndrome. Anaesth Intensive Care 2011; 39: 1152-3

-            Gurrieri C, Kivela JE, Bojanic K, Gravilova RH, Flick RP, Sprung J, Weingarten TN. 
Anesthetic considerations in mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes syndrome : a case series. 
Can J Anesth 2011; 58: 751-63.

-         Gentilli M-E, Raud C, Enel D, Hénot M, Bothereau H. 
Combinaison d’une anesthésie générale et d’une analgésie péridurale chez une patiente porteuse d’une myopathie mitochondriale. 
Ann Fr Anesth Réanim 2013 ; 32 : e149.

-         Mtaweh H, Bayir H, Kochanek PM, Bell MJ. 
Effect of a single dose of propofol and lack of dextrose administration in a child with mitochondrial disease : a case report. 
J Child Neurol 2014; 29: NP 40-6.  

-        Yamadori Y, Yamagami Y, MatsumotoY, Koizumi M, Nakamura A, Mizuta D, Yasuda K, Shirakami G.
General anesthesia with remimazolam for a pediatric patient with MELAS and recurrent epilepsy: a case report.
JA Clinical Reports 2022 ; 8:75 doi.org/10.1186/s40981-022-00564-x

Updated: June 2023