Headache is a prominent feature in
mitochondrial disorders (MIDs) but no comprehensive overview is currently available. This review aims at summarising and discussing findings concerning type, frequency, pathogenesis, and treatment of
headache in MIDs. The most frequent
headache types in MIDs are
migraine and
migraine-like
headache (MLH). MLH is classified as secondary
headache. More rarely,
tension-type headache, trigemino-autonomic
headache, or different secondary
headaches can be found.
Migraine or MLH may manifest with or without
aura. MLH is frequently associated with an ongoing or previous
stroke-like episode (SLE) or a seizure but may also occur independently of other neurological features. MLH may be associated with prolonged
aura or visual phenomena after
headache. Except for MLH, treatment of
headache in MIDs is not at variance from other causes of
headache. Beyond the broadly accepted subtype-related
headache treatment, diet, cofactors,
vitamins, and
antioxidants may provide a supplementary benefit.
Midazolam,
l-arginine, or l-
citrulline may be beneficial for MLH. The pathogenesis of
headache in MIDs largely remains unsolved. However, since
migraine and MLH respond both to triptanes, a shared pathomechanism is likely. In conclusion,
migraine and MLH are the prominent
headache types in MIDs. MLH may or may not be associated with current or previous SLEs. MLH is pathophysiologically different from
migraine and requires treatment at variance from that of
migraine with aura.