| Journal of Neurology Research, ISSN 1923-2845 print, 1923-2853 online, Open Access |
| Article copyright, the authors; Journal compilation copyright, J Neurol Res and Elmer Press Inc |
| Journal website https://www.neurores.org |
Review
Volume 11, Number 5, October 2021, pages 77-86
Vestibular Migraine With Brainstem Auras: A Review of Pathogenesis, Clinical Varieties, Abortive and Prophylactic Treatment
Table
| FHM1 | FHM2 | FHM3 | |
|---|---|---|---|
| FHM: familial hemiplegic migraine. | |||
| Chromosome location | 19p13 | 1q23 | 2q24 |
| Gene | CACNA1A | ATP1A2 | SCN1A |
| Protein | Pore-forming α1 subunit of neuronal Cav2.1 (P/Q type) voltage-gated calcium channels | Catalytic α2 subunit of a glial and neuronal sodium-potassium pump | Pore-forming α1 subunit of neuronal Nav1.1 voltage-gated sodium channels |
| Location | Both at central synapses and at neuromuscular junction, densely expressed in cerebellar Purkinje cell | During development, predominantly expressed in neurons, but expression shifts to glial cells by adulthood | Expressed primarily on inhibitory neurons |
| Main function | To trigger the release of neurotransmitters | Utilizes ATP hydrolysis to transport Na+ ions out of the cell in exchange for K+ ions into the cell, providing the steep Na+ gradient for the transport of glutamate and Ca2+. In adults, to modulate the removal of potassium and glutamate from the synaptic cleft into the glial cell | Voltage-gated sodium channels are primarily responsible for the generation and propagation of action potentials in excitable cells |
| Penetrance | 67-89% | 63-87% | 100% |
| Type of mutation | Over 50 missense mutations | Over 60 missense mutations | 5 missense mutations |