Hemiplegic migraine (HM) is a rare form of migraine with an aura characterized by transient motor weakness or hemiparesis (motor aura). HM is also associated with other non-motor aura manifestations (visual, sensory, aphasic, or basilar-type/brainstem symptoms) and with other symptoms typically accompanying migraine, such as nausea, vomiting, photophobia, or phonophobia. A motor aura represents the peculiarity of HM compared to other forms of migraine with aura (MA), and its diagnostic criteria have been updated in the latest classification of headache disorders. HM can be sporadic (SHM) or familial (FHM), with autosomal dominant inheritance.
HM Diagnostic Criteria |
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A. Attacks fulfilling criteria for 1 MA and criterion B below. B. Aura consisting of both of the following: 1. Fully reversible motor weakness; 2. Fully reversible visual, sensory, and/or speech/language symptoms. |
HM Diagnostic Criteria Minor Revisions |
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Aura (B2 in Table 1) could consist of at least 2 symptoms instead of 3 A gradual development of aura (in at least 60 min) Presence of headache is not mandatory 1 BM could be included as a symptom |
FHM 1 | FHM 2 | FHM 3 |
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A. Attacks fulfilling criteria for FHM | A. Attacks fulfilling criteria for FHM | A. Attacks fulfilling criteria for FHM |
B. A mutation on the CACNA1A gene has been demonstrated. | B. A mutation on the ATP1A2 gene has been demonstrated. | B. A mutation on the SCN1A gene has been demonstrated. |
Gene | Phenotypic Spectrum |
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CACNA1A | 1 HM, transient focal neurologic deficit without headache, coma after minor brain injury, progressive cerebella ataxia [10,22] |
ATP1A2 | HM, epilepsy, intellectual disability, prolonged HM attacks, confusion, and coma, 2 BM [12,23] |
SCN1A | HM, epilepsy [24] |
PRRT2 | benign familial childhood epilepsy, episodic kinesigenic dyskinesia, familial childhood seizures with paroxysmal choreoathetosis [17] |
SLC4A4 | HM, renal tubular acidosis, glaucoma [19] |
SLC1A3 | HM [20] |
SLC1A2 | epilepsy—Glut1 deficiency syndrome, HM-like attacks [21] |
This entry is adapted from the peer-reviewed paper 10.3390/jcm12113783