6.8.2 Headache attributed to Mitochondrial Encephalopathy, Lactic Acidosis and Stroke-like episodes (MELAS)

Description:

Headache, which is either recurrent in migraine-like attacks or a presenting symptom of stroke-like episodes, caused by and associated with the other clinical features of Mitochondrial Encephalopathy, Lactic Acidosis and Stroke-like episodes (MELAS).

Diagnostic criteria:
  1. Recurrent attacks of headache fulfilling criterion C
  2. A mitochondrial genetic abnormality associated with Mitochondrial Encephalopathy, Lactic Acidosis and Stroke-like episodes (MELAS) has been demonstrated
  3. Either or both of the following:
    1. recurrent migraine attacks with or without aura
    2. acute headache preceding or associated with focal neurological deficits and/or seizures
  4. Not better accounted for by another ICHD-3 diagnosis.
Comments:

Mitochondrial Encephalopathy, Lactic Acidosis and Stroke-like episodes (MELAS) is a genetically heterogeneous mitochondrial disorder with a variable clinical phenotype, including features of central nervous system involvement (seizures, hemiparesis, hemianopia, cortical blindness, sensorineural deafness and/or episodic vomiting). Headache is common in MELAS, either as recurrent migraine-like attacks or as the presenting symptom of stroke-like episodes.

The high frequency of migraine-like attacks as part of MELAS has led to the hypothesis that mitochondrial mutations play a role in migraine with aura, but the 3243 mutation was not detected in two groups of subjects with 1.2 Migraine with aura. Other yet-undetected mutations may play a role in both migraine and ischaemic stroke, since migraine attacks, mostly with aura, also occur in other mitochondrial disorders.