Related Experiment Video

Updated: May 10, 2026

3D-Neuronavigation In Vivo Through a Patient's Brain During a Spontaneous Migraine Headache
10:39

3D-Neuronavigation In Vivo Through a Patient's Brain During a Spontaneous Migraine Headache

Published on: June 2, 2014

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Familial hemiplegic migraine in Indian children-a tertiary center experience

Lokesh Saini1, Pradeep Kumar Gunasekaran1, Sarbesh Tiwari2

  • 1Department of Pediatrics, All India Institute of Medical Sciences, Jodhpur 342005, Rajasthan, India.

Journal of Tropical Pediatrics
|April 5, 2024
PubMed

Insights

Familial hemiplegic migraine (FHM) in children presents with hemiparesis and headache. Genetic testing confirmed mutations, and topiramate showed favorable treatment responses in this study.

Area of Science:

  • Neurology
  • Genetics
  • Pediatrics

Background:

  • Familial hemiplegic migraine (FHM) is a rare, autosomal dominant subtype of migraine with aura.
  • It is a neurological channelopathy characterized by severe headache, hemiplegia, and other neurological deficits.
  • FHM presents significant diagnostic challenges, particularly in pediatric populations.

Purpose of the Study:

  • To investigate the clinical presentation, genetic basis, and treatment response of familial hemiplegic migraine in children.
  • To highlight the diagnostic delay and neuroimaging findings in pediatric FHM cases.
  • To evaluate the efficacy of topiramate in managing FHM symptoms in children.

Main Methods:

  • A cross-sectional study involving children aged 1-18 years suspected of FHM.
  • Clinical data collection, including neurological symptoms and episode duration.
  • Genetic testing for FHM-associated genes (ATP1A2, SCN1A) and neuroimaging (MRI T2/FLAIR).
  • Treatment with oral topiramate and follow-up assessment.

Main Results:

  • Five children with genetically confirmed FHM were included, presenting with hemiparesis, headache, and focal seizures.
  • Mean age at onset was 6.8 years, with a significant diagnostic delay of 6.1 years.
  • Neuroimaging showed accentuated gray-white differentiation; genetic testing revealed ATP1A2 and SCN1A mutations.
  • All patients responded favorably to oral topiramate treatment.

Conclusions:

  • Familial hemiplegic migraine diagnosis in children is primarily clinical, supported by genetic analysis.
  • MRI, especially perfusion and diffusion-weighted sequences, can aid diagnosis during acute episodes.
  • Topiramate is an effective treatment option for pediatric FHM, demonstrating favorable outcomes.

Keywords:
ATP1A2 mutationsSCN1A mutationschannelopathyfamilial hemiplegic migraineparoxysmal hemiplegia

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