Familial hemiplegic migraine (FHM)

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

Familial hemiplegic migraine (FHM). FHM1: CACNA1 gene, FHM2: ATP1A2 gene, FHM3: SCN1 gene, PRRT2 gene. Fully referenced neurology checklist.

Genetic types and mutations

  • FHM1: CACNA1 gene
  • FHM2: ATP1A2 gene
  • FHM3: SCN1 gene
  • PRRT2 gene

Triggers

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Clinical features

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The digiti quinti sign

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Elicited repetitive daily blindness (ERDB)

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Complications

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Differential diagnosis: sporadic hemiplegic migraine (SHM)

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Treatment

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References

  1. Russell MB, Ducros A. Sporadic and familial hemiplegic migraine: pathophysiological mechanisms, clinical characteristics, diagnosis, and management. Lancet Neurol 2011; 10:457–470.
  2. Vahedi K, Depienne C, Le Fort D, et al. Elicited repetitive daily blindness: a new phenotype associated with hemiplegic migraine and SCN1A mutations. Neurology 2009; 72: 1178-1183.
  3. Schubert V, Auffenberg E, Biskup S, Jurkat-Rott K, Freilinger T. Two novel families with hemiplegic migraine caused by recurrent SCN1A mutation p.F1499L. Cephalalgia 2018; 38:1503-1508.
  4. Riant F, Roos C, Roubertie A, Barbance C, et al. Hemiplegic migraine associated with PRRT2 variations: a clinical and genetic study. Neurology 2022; 98:e51-e61.
  5. Stam AH, Louter MA, Haan J, et al. A long term follow-up study of 18 patients with sporadic hemiplegic migraine. Cephalalgia 2011; 31:199-205.
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