Recurrent Hemiplegia with Migraine in a Teenager: ATP1A2-linked Familial Hemiplegic Migraine Type 2

Background: Hemiplegic migraine (HM), a rare subtype of migraine featuring transient hemiparesis during attacks, presents diagnostic challenges due to its dramatic and variable manifestations. Clinical Description: We present a case of a 17-year-old boy with recurrent hospitalizations for severe headache preceded by visual aura, followed by acute-onset hemiplegia and transient neurological deficits, all resolving completely between episodes. Management and Outcome: Baseline blood and cerebrospinal investigations were normal. Contrast-enhanced magnetic resonance (MR) imaging showed sulcal effacement in the right temporoparietal area with no meningeal enhancement; the electroencephalogram, MR venography, and MR spectroscopy were normal. Whole exome sequencing revealed a pathogenic heterozygous missense mutation (c.2723G>A) in exon 20 of ATP1A2 gene on chromosome 1q23 in both the patient and his mother, thus diagnosing familial HM type 2. He was treated with flunarizine and improved thereafter. Conclusion: Recurrent transient hemiplegia associated with headache, every time preceded by visual or speech abnormalities, can be due to HM. A thorough clinical evaluation and family history are essential to raise suspicion of this disorder, and genetic analysis can confirm the diagnosis. Treatment is simple and rewarding.

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Publication Details

Journal
Indian Pediatrics Case Reports
Published
2025-10-01
DOI
https://doi.org/10.4103/ipcares.ipcares_113_25
Primary Topic
Migraine and Headache Studies
Type
article
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article

Recurrent Hemiplegia with Migraine in a Teenager: ATP1A2-linked Familial Hemiplegic Migraine Type 2

Indian Pediatrics Case Reports
Migraine and Headache Studies
article

Recurrent Hemiplegia with Migraine in a Teenager: ATP1A2-linked Familial Hemiplegic Migraine Type 2

article en

Abstract

Background: Hemiplegic migraine (HM), a rare subtype of migraine featuring transient hemiparesis during attacks, presents diagnostic challenges due to its dramatic and variable manifestations. Clinical Description: We present a case of a 17-year-old boy with recurrent hospitalizations for severe headache preceded by visual aura, followed by acute-onset hemiplegia and transient neurological deficits, all resolving completely between episodes. Management and Outcome: Baseline blood and cerebrospinal investigations were normal. Contrast-enhanced magnetic resonance (MR) imaging showed sulcal effacement in the right temporoparietal area with no meningeal enhancement; the electroencephalogram, MR venography, and MR spectroscopy were normal. Whole exome sequencing revealed a pathogenic heterozygous missense mutation (c.2723G>A) in exon 20 of ATP1A2 gene on chromosome 1q23 in both the patient and his mother, thus diagnosing familial HM type 2. He was treated with flunarizine and improved thereafter. Conclusion: Recurrent transient hemiplegia associated with headache, every time preceded by visual or speech abnormalities, can be due to HM. A thorough clinical evaluation and family history are essential to raise suspicion of this disorder, and genetic analysis can confirm the diagnosis. Treatment is simple and rewarding.

Indian Pediatrics Case ReportsVol. 5(4)
Defence Research and Development Organisation (IN)
Openalex Percentile: Top 43%
Migraine and Headache Studies
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Recurrent Hemiplegia with Migraine in a Teenager: ATP1A2-linked Familial Hemiplegic Migraine Type 2 · Indian Pediatrics Case Reports (2025) | TGRS Research Map | TGRS