What is Familial Hemiplegic Migraine?
Familial hemiplegic migraine (FHM) is an autosomal dominant disease characterized by migraine with aura that causes motor impairment on one side of the body (hemiplegia) in addition to an aura before the migraine begins. There are four subtypes to this disease. This website will focus on type III (FHM3), which is characterized by a mutation in the SCN1A gene [1].
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Auras and hemiplegia from FHM3 are believed to be caused by cortical spreading depression (CSD). This is a wave of neuronal depolarization that slowly propagates through the brain [1]. Part of this is an influx of sodium into neurons, which causes water to enter cells. This causes cells to swell and extracellular space to be reduced [2].
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Symptoms of FHM
FHM is a type of migraine with aura. This aura can be things such as visual disturbances, speech disturbances, or sensory disturbances such as numbness or tingling. FHM also causes hemiplegia, which is fully reversible motor impairment on one side of the body. Hemiplegia typically starts in the hand and spreads to the arm and face. Most patients with FHM also have a headache associated with the aura symptoms.
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References
[1] Kazemi, H., Speckmann, E.-J., & Gorji, A. (2014). Familial Hemiplegic Migraine and Spreading Depression. Iranian Journal of Child Neurology, 8(3). https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4135274/
[2] James, M. F., Smith, J. M., Boniface, S. J., Huang, C. L.-H., & Leslie, R. A. (2001). Cortical spreading depression and migraine: New insights from imaging? Trends in Neurosciences, 24(5), 266–271. https://doi.org/10.1016/s0166-2236(00)01793-8
[3] Kahlig, K. M., Rhodes, T. H., Pusch, M., Freilinger, T., Pereira-Monteiro, J. M., Ferrari, M. D., van den Maagdenberg, A. M., Dichgans, M., & George, A. L. (2008). Divergent sodium channel defects in familial hemiplegic migraine. Proceedings of the National Academy of Sciences, 105(28), 9799–9804. https://doi.org/10.1073/pnas.0711717105
[4] Kumar, A. (2023, July 4). Hemiplegic migraine. StatPearls [Internet]. https://www.ncbi.nlm.nih.gov/books/NBK513302/#:~:text=Nonrandomized%20studies%20have%20suggested%20acetazolamide,for%20patients%20with%20hemiplegic%20migraine.
Images created in BioRender.
[2] James, M. F., Smith, J. M., Boniface, S. J., Huang, C. L.-H., & Leslie, R. A. (2001). Cortical spreading depression and migraine: New insights from imaging? Trends in Neurosciences, 24(5), 266–271. https://doi.org/10.1016/s0166-2236(00)01793-8
[3] Kahlig, K. M., Rhodes, T. H., Pusch, M., Freilinger, T., Pereira-Monteiro, J. M., Ferrari, M. D., van den Maagdenberg, A. M., Dichgans, M., & George, A. L. (2008). Divergent sodium channel defects in familial hemiplegic migraine. Proceedings of the National Academy of Sciences, 105(28), 9799–9804. https://doi.org/10.1073/pnas.0711717105
[4] Kumar, A. (2023, July 4). Hemiplegic migraine. StatPearls [Internet]. https://www.ncbi.nlm.nih.gov/books/NBK513302/#:~:text=Nonrandomized%20studies%20have%20suggested%20acetazolamide,for%20patients%20with%20hemiplegic%20migraine.
Images created in BioRender.
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About the website
This web page was produced as an assignment for Genetics 564, an undergraduate capstone course at UW-Madison
Joely Swanson, [email protected]
Last updated April 9th, 2024
Genetics 564 website
Joely Swanson, [email protected]
Last updated April 9th, 2024
Genetics 564 website