Journal of Pediatric Neurology 2022; 20(06): 437-439
DOI: 10.1055/s-0042-1747936
Case Report

Migraine with Brainstem Aura Associated with SCN2A Mutation: A Case Report

1   Neurology Department, University Hospital La Paz, Madrid, Spain
,
María Sastre-Real
1   Neurology Department, University Hospital La Paz, Madrid, Spain
,
Pilar Tirado
2   Pediatric Neurology Service, University Hospital La Paz, Madrid, Spain
,
Javier Díaz-de-Terán
1   Neurology Department, University Hospital La Paz, Madrid, Spain
3   La Paz Institute for Health Research (IdiPAZ), Madrid, Spain
4   Physiotherapy Department, CranioSPain Research Group, La Salle Higher Center for University Studies, Autonomous University of Madrid, Madrid, Spain
› Author Affiliations
Funding None.

Abstract

Background Migraine with brainstem aura (MBA) is a rare subtype of migraine with aura that associates headache with paroxysmal episodes of ataxia, dysarthria, and diplopia among other symptoms attributed to brainstem involvement. The manifestation of this disease in the absence of typical aura symptoms (i.e., fully reversible visual, sensory and/or speech/language symptoms) is even more infrequent.

Case Report In this article, we reported a case of a 16-year-old male patient with MBA associated with a sodium voltage-gated channel alpha subunit 2 (SCN2A) de novo mutation. The patient did not have typical aura symptoms. Ataxia was the most prominent clinical feature, leading to initial diagnosis of episodic ataxia. Patient responded partially to treatment with onabotulinum toxin A.

Conclusion MBA needs to be taken into consideration in the differential diagnosis of recurrent ataxia, even in the absence of typical aura symptoms. Finding a de novo SCN2A mutation in our patient helps in further understanding of MBA genetics.

Authors' Contributions

J.D.d.T. conceptualized and designed the manuscript.


J.A.M. drafted the manuscript.


M.S.-R., P.T., and J.D.-d.T. revised it for intellectual content.


J.D.-d.T. gave final approval of the completed manuscript.




Publication History

Received: 17 November 2021

Accepted: 07 March 2022

Article published online:
11 July 2022

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  • References

  • 1 Headache Classification Committee of the International Headache Society (IHS) The International Classification of Headache Disorders, 3rd edition. Cephalalgia 2018; 38 (01) 1-211
  • 2 Yamani N, Chalmer MA, Olesen J. Migraine with brainstem aura: defining the core syndrome. Brain 2019; 142 (12) 3868-3875
  • 3 Salman MS, Klassen SF, Johnston JL. Recurrent ataxia in children and adolescents. Can J Neurol Sci 2017; 44 (04) 375-383
  • 4 Blumenfeld AE, Victorio MC, Berenson FR. Complicated migraines. Semin Pediatr Neurol 2016; 23 (01) 18-22
  • 5 Demarquay G, Ducros A, Montavont A, Mauguiere F. Migraine with brainstem aura: Why not a cortical origin?. Cephalalgia 2018; 38 (10) 1687-1695
  • 6 Passi GR, Mohammad SS. Dominant SCN2A mutation with variable phenotype in two generations. Brain Dev 2021; 43 (01) 166-169
  • 7 Kowalska M, Prendecki M, Kapelusiak-Pielok M. et al. Analysis of genetic variants in SCN1A, SCN2A, KCNK18, TRPA1 and STX1A as a possible marker of migraine. Curr Genomics 2020; 21 (03) 224-236
  • 8 Papetti L, Ursitti F, Moavero R. et al. Prophylactic treatment of pediatric migraine: is there anything new in the last decade?. Front Neurol 2019; 10: 771 DOI: 10.3389/fneur.2019.00771.
  • 9 Winner PK, Kabbouche M, Yonker M, Wangsadipura V, Lum A, Brin MF. A randomized trial to evaluate onabotulinumtoxinA for prevention of headaches in adolescents with chronic migraine. Headache 2020; 60 (03) 564-575
  • 10 Shah S, Calderon M-D, Crain N, Pham J, Rinehart J. Effectiveness of onabotulinumtoxinA (BOTOX) in pediatric patients experiencing migraines: a randomized, double-blinded, placebo-controlled crossover study in the pediatric pain population. Reg Anesth Pain Med 2021; 46 (01) 41-48