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The Journal of Headache and Pain

Official Journal of the "European Headache Federation" and of "Lifting The Burden - The Global Campaign against Headache"

The Journal of Headache and Pain Cover Image

Volume 16 Supplement 1

1st Joint ANIRCEF-SISC Congress

Open Access

O035. Headaches in Mitochondrial Disorders

  • Catello Vollono1Email author,
  • Giudo Primiano1,
  • Anna Losurdo2,
  • Serenella Servidei1 and
  • Giacomo Della Marca1
The Journal of Headache and Pain201516(Suppl 1):A68

Published: 28 September 2015


MigraineMuscle WeaknessBinary Logistic RegressionIdentify Risk FactorBinary Logistic Regression Analysis


Headaches are a well known feature of Mitochondrial Disorders (MCDs). However, no systematic epidemiological data are available in large populations of patients.

We aimed to describe the prevalence and the characteristics of headache in a large group of patients with mitochondrial encephalomyopathies.


We studied all consecutive patients referred to our Neuromuscular Unit, during a 6-month period. Ninety-three patients (aged 15 to 78 years, 31 males) with a typical phenotype of MCDs, underwent a structured diagnostic headache interview, using an operational diagnostic tool following the IHS criteria. If they met the criteria for primary headache, they were included in the “Headache Group” (HEAD+). The other patients were collected in the “No Headache Group” (HEAD-). Clinical, neuroradiological, and neurophysiological data were compared between groups. Mann-Whitney U-test was used to analyze numeric variables; Fisher's exact test was used to analyze nominal variables. Binary logistic regression analysis was performed to identify risk factors of headache.


Headaches were reported in 35.48% of patients. Migraine was the most common headache. The patients of the Headache Group were younger (HEAD+ = 45.5±17.2 years; HEAD- = 54.5±14.8 years; U-test = 7.393; p = 0.007), increased prevalence of epilepsy (p = 0.0103), myoclonus (p = 0.0309), stroke (p = 0.0290), EEG focal slow abnormalities (p = 0.0359), EEG epileptic focal abnormalities (p = 0.0425), and decreased prevalence of muscle weakness (p < 0.0001) and EEG normal pattern (p = 0.0136). Multivariate analysis showed that HEAD+ was significantly associated with absence of muscle weakness (CI = 1.007-14.894; p = 0.049) and EEG abnormalities (CI = 1.347-78.085; p = 0.025).


Migraine has a higher prevalence in MCDs compared to population-based data. Our findings are consistent with the widely hypothesized role of mitochondria in the migraine pathophysiology.

Written informed consent to publication was obtained from the patient(s).

Authors’ Affiliations

Unit of Neurophysiopathology and Sleep Medicine, Department of Geriatrics, Neurosciences and Orthopedics, Catholic University, Rome, Italy
Istituto Auxologico Italiano, Department Neurology, S. Luca Hospital, Milan, Italy


© Vollono et al. 2015

This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver ( applies to the data made available in this article, unless otherwise stated.


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