Table 1

Diagnostic criteria of hemiplegic migraine

Diagnostic criteria ICHD-3
Hemiplegic migraineA. At least two attacks fulfilling criteria
B. One or more of the following fully reversible aurasymptoms:
1. visual
2. sensory
3. speech and/or language
4. motor
5. brainstem
6. retinal
C. At least three of the following six characteristics:
1. at least one aura symptom spreads graduallyover 5 minutes
2. two or more aura symptoms occur in succession
3. each individual aura symptom lasts 5–60 minutes
4. at least one aura symptom is unilateral
5. at least one aura symptom is positive
6. the aura is accompanied, or followed within 60 min, by headache
D. Aura consisting of both of the following:
1. fully reversible motor weakness
2. fully reversible visual, sensory and/or speech/ language symptoms.
Familial hemiplegic migraine (FHM)A. Attacks fulfilling criteria for Hemiplegic migraine
B. At least one first- or second-degree relative has had attacks fulfilling criteria for Hemiplegic migraine.
Familial hemiplegic migraine type 1 (FHM1)A. Attacks fulfilling criteria for Familial hemiplegic migraine
B. A mutation on the CACNA1A gene has been demonstrated.
Familial hemiplegic migraine type 2 (FHM2)A. Attacks fulfilling criteria for Familial hemiplegic migraine
B. A mutation on the ATP1A2 gene has been demonstrated.
Familial hemiplegic migraine type 3 (FHM3)A. Attacks fulfilling criteria for Familial hemiplegic migraine
B. A mutation on the SCN1A gene has been demonstrated.
Familial hemiplegic migraine, other lociA. Attacks fulfilling criteria for Familial hemiplegic migraine
B. Genetic testing has demonstrated no mutation onthe CACNA1A, ATP1A2 or SCN1A genes.
Sporadic hemiplegic migraine (SHM)A. Attacks fulfilling criteria for Hemiplegic migraine
B. No first- or second-degree relative fulfils criteriafor Hemiplegic migraine.
  • ICHD-3, third edition of International Classification of Headache Disorders.