Migraine in mitochondrial disorders: Prevalence and characteristics

C. Vollono, G. Primiano, G. Della Marca, A. Losurdo, S. Servidei

Research output: Contribution to journalArticlepeer-review


Background: Migraine is a well-known feature of mitochondrial disorders (MDs). However, no systematic epidemiological data are available in large populations of patients. Aims: The aim of this cross-sectional cohort study was to describe the prevalence and migraine characteristics in a large cohort of patients with mitochondrial encephalomyopathies. Methods: We studied 93 consecutive patients with characterised MDs referred to our Neuromuscular Unit during a 12-month period. All patients (age range = 16–78 years; 31 men; 58 progressive external ophthalmoplegia [PEO], 12 myoclonic epilepsy with ragged red fibres [MERRF], eight mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes [MELAS], two mitochondrial neurogastrointestinal encephalomyopathy [MNGIE] and 13 other MDs) underwent a structured diagnostic headache interview using an operational diagnostic tool following the IHS criteria. If they met the criteria for migraine, they were included in the ‘Migraine Group’. The other patients were counted in the ‘No Migraine Group’. Patient demographic and migraine characteristics were examined. Clinical, neuroradiological and neurophysiological data were compared between groups. Results: Migraine was reported in 35.5% of patients. Migraine without aura was the most common headache (81.8%). The migraine group showed younger age (P <0.01), increased prevalence of epilepsy (P = 0.01), myoclonus (P = 0.03), stroke-like episodes (P = 0.03) and decreased prevalence of muscle weakness (P <0.01). Multivariate analysis showed that migraine was positively associated with absence of muscle weakness (P = 0.04) and presence of EEG abnormalities (P = 0.02). Conclusion: Migraine has a higher prevalence in MDs compared with general population-based data, independently from genotype or phenotype. Migraine is not merely a phenotypic aspect of specific MDs but is rather the expression of vulnerability of the central nervous system, probably directly related with defects of the respiratory chain. © 2017, © International Headache Society 2017.
Original languageEnglish
Pages (from-to)1093-1106
Number of pages14
Issue number6
Publication statusPublished - 2018


  • adolescent
  • adult
  • age
  • aged
  • Article
  • cerebrovascular accident
  • chronic progressive external ophthalmoplegia
  • clinical feature
  • cohort analysis
  • cross-sectional study
  • demography
  • diagnostic test
  • EEG abnormality
  • epilepsy
  • female
  • genotype
  • human
  • major clinical study
  • male
  • MELAS syndrome
  • MERRF syndrome
  • middle aged
  • migraine
  • migraine with aura
  • mitochondrial encephalopathy
  • MNGIE syndrome
  • muscle weakness
  • myoclonus
  • neurophysiology
  • neuroradiology
  • patient referral
  • phenotype
  • population research
  • prevalence
  • young adult


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