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Diagnostic and therapeutic aspects of hemiplegic migraine
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  • Published on:
    Response to Commentary by Prof. Gupta: Hemiplegic migraine, genetic mutations, and cortical spreading depression: a presumed pathophysiologic nexus that defies scientific logic
    • Di Stefano Vincenzo, Neurologist Department of Biomedicine, Neuroscience and advanced Diagnostic, University of Palermo
    • Other Contributors:
      • Marianna Gabriella Rispoli, Neurologist
      • Noemi Pellegrino, Pediatrician
      • Alessandro Graziosi, Peditrician
      • Eleonora Rotondo, Pediatrician
      • Christian Napoli, Epidemiologist
      • Daniela Pietrobon, Neurologist
      • Filippo Brighina, Neurologist
      • PASQUALE PARISI, Pediatrician

    We read with interest the commentary from Prof Gupta (1). Migraine is a complex and heterogeneous disorder with multifactorial pathogenesis (2). In fact, it is a well-known fact that both genetic and environmental factors are involved in the etiopathogenesis of migraine (2). Conversely, hemiplegic migraine (HM) is a complex monogenic disorder related to a mutation in genes encoding for ion transporters (3). Even if many consider HM as a subtype of migraine, this condition offers insight in migraine pathophysiology, especially in the case of migraine with aura, as well as in other conditions overlapping between headache and epilepsy, such as the so called “Ictal Epileptic Headache”, a new concept defined in the last decade (4–6).
    Our knowledge on the pathophysiology of both migraine and HM is evolving with new insights coming from the last years (3). However, we partially agree that ….“No systemic influence can explain the characteristic lateralizing headache of migraine, unilateral, bilateral, side-shifting or side-locked” (7,8). Interestingly, new data have come from neurophysiology: hyperexcitability/dysexcitability (5) in migraine has been clearly demonstrated in migraine sufferers with more prominent results especially in migraine with aura (5,9,10). These data could make a reasonable link between the genesis of hyperexcitability/dysexcitability of multisensory cortices, cortical spreading depression (CSD) and the “headache” phase of migraine, mediated by the tri...

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    Conflict of Interest:
    None declared.
  • Published on:
    Hemiplegic migraine, genetic mutations, and cortical spreading depression: a presumed pathophysiologic nexus that defies scientific logic
    • Vinod K. Gupta, Physician (Internist) Migraine-Headache Institute, GUPTA MEDICAL CENTRE, New Delhi, India

    I read the article by Stefano et al with interest.1 Genetic predisposition per se cannot explain discrete self-limited recurrent attacks of headache and aura manifestations of hemiplegic migraine, with the state/clinical predisposition appearing and disappearing seemingly inexplicably over several decades or the life-time of a sufferer.2

    More than two decades ago, I elucidated a fundamental clinico-theoretical principle for the understanding of migraine-linked physiologic mechanisms that has well-stood the test of time, technology and biologic commonsense: “No systemic influence can explain the characteristic lateralizing headache of migraine, unilateral, bilateral, side-shifting or side-locked”.3 Over the last fifty years, some of the "systemic" influences believed to play key pathogenetic roles in migraine include serotonin, platelets, catecholamines, calcitonin-gene related peptide, magnesium depletion, stress, post-stress state, and recurrent micro thrombo-embolisms across the patent foramen ovale at the level of the cardiac inter-atrial septum. 4,5,6 Little vertical and biologically-plausible robust generalizable progress, however, has been made in gestalt understanding of the disorder well into the 21st century.7 Trait-linked genetic association is also a “systemic” influence. Ion transporters – as well as the three main causative genes—CACNA1A, ATP1A2 and SCN1A—which encode for ion transporters1 do not offer clues to the mechanistic physiological b...

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    Conflict of Interest:
    None declared.