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Migraine
Review
Diagnostic and therapeutic aspects of hemiplegic migraine
  1. Vincenzo Di Stefano1,
  2. Marianna Gabriella Rispoli2,
  3. Noemi Pellegrino3,
  4. Alessandro Graziosi3,
  5. Eleonora Rotondo3,
  6. Christian Napoli4,
  7. Daniela Pietrobon5,6,
  8. Filippo Brighina1,
  9. Pasquale Parisi7
  1. 1 Department of Biomedicine, Neuroscience and Advanced Diagnostic (BIND), University of Palermo, Palermo, Sicilia, Italy
  2. 2 Department of Neuroscience Imaging and Clinical Sciences, ‘G. d'Annunzio’ University, Universita degli Studi Gabriele d'Annunzio Chieti e Pescara, Chieti Scalo, Chieti, Italy
  3. 3 Pediatrics, University Gabriele d'Annunzio of Chieti Pescara Department of Medicine and Aging Science, Chieti, Abruzzo, Italy
  4. 4 Department of Medical Surgical Sciences and Translational Medicine, Sapienza University of Rome, Roma, Lazio, Italy
  5. 5 Department of Biomedical Sciences & Padova Neuroscience Center, University of Padova, Padova, Italy
  6. 6 CNR Neuroscience Institute, Padova, Italy
  7. 7 Dipartimento di Neuroscienze Salute Mentale e Organi di Senso (NESMOS), University of Rome La Sapienza Faculty of Medicine and Psychology, Roma, Lazio, Italy
  1. Correspondence to Professor Pasquale Parisi, Dipartimento di Neuroscienze Salute Mentale e Organi di Senso (NESMOS), University of Rome La Sapienza Faculty of Medicine and Psychology, Roma 00189, Lazio, Italy; pasquale.parisi{at}uniroma1.it

Abstract

Hemiplegic migraine (HM) is a clinically and genetically heterogeneous condition with attacks of headache and motor weakness which may be associated with impaired consciousness, cerebellar ataxia and intellectual disability. Motor symptoms usually last <72 hours and are associated with visual or sensory manifestations, speech impairment or brainstem aura. HM can occur as a sporadic HM or familiar HM with an autosomal dominant mode of inheritance. Mutations in CACNA1A, ATP1A2 and SCN1A encoding proteins involved in ion transport are implicated. The pathophysiology of HM is close to the process of typical migraine with aura, but appearing with a lower threshold and more severity. We reviewed epidemiology, clinical presentation, diagnostic assessment, differential diagnosis and treatment of HM to offer the best evidence of this rare condition. The differential diagnosis of HM is broad, including other types of migraine and any condition that can cause transitory neurological signs and symptoms. Neuroimaging, cerebrospinal fluid analysis and electroencephalography are useful, but the diagnosis is clinical with a genetic confirmation. The management relies on the control of triggering factors and even hospitalisation in case of long-lasting auras. As HM is a rare condition, there are no randomised controlled trials, but the evidence for the treatment comes from small studies.

  • headache
  • EEG
  • ion transport
  • clinical neurology
http://creativecommons.org/licenses/by-nc/4.0/

This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited, appropriate credit is given, any changes made indicated, and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/.

https://doi.org/10.1136/jnnp-2020-322850

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    Footnotes

    • Contributors All authors contributed to the study conception and design. VDS, MGR, NP, AG and ER did the literature search, data analysis and wrote the first draft of the manuscript. All authors commented on previous version of the manuscript. CN, DP, FB and PP revised the work. All authors read and approved the final manuscript.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Patient consent for publication Not required.

    • Provenance and peer review Not commissioned; externally peer reviewed.