One of the questions of concern to many people with Migraine disease is whether the disease or severe Migraine attacks cause any permanent damage to the brain.
A new case study report indicates that hemiplegic Migraine (HM) attacks may result in brain atrophy (decrease in size or wasting away). Sporadic (SHM) and familial (FHM) hemiplegic Migraine are rare forms of Migraine characterized by transient motor weakness and/or hemiplegia during the aura phase. They’re caused by genetic mutations in CACNA1A, ATP1A2, or SCN1A. In some cases, neurological symptoms such as the motor weakness and hemiplegia may continue for days, weeks, or even months after the headache phase and the rest of the Migraine attack have passed.
“Patients with hemiplegic migraine (HM) may sometimes develop progressive neurological deterioration of which the pathophysiology is unknown.” 1
The report is about a “16-year clinical and neuroradiological follow-up of a patient carrying a de novo p.Ser218Leu CACNA1A HM mutation who had nine severe HM attacks associated with seizures and decreased consciousness between the ages of three and 12 years.” 1
Repeated neuroimaging, performed both ictally (during a Migraine attack) and postictally (after a Migraine attack), revealed cytotoxic (toxic to cells) edema in the symptomatic hemisphere during severe HM attacks, which later showed atrophic changes.
The cytoxic edema is thought to be caused by enhanced susceptibility to cortical spreading depression.
Progressive cerebellar atrophy was observed.
Brain atrophy halted after severe attacks stopped, possibly due to preventive treatment with flunarizine (Sibelium) and sodium valproate (Depacon).
“This unique case illustrates development of secondary cortical atrophy in previously affected brain areas during severe HM attacks and suggests that adequate prophylaxis may be critical to prevent permanent brain damage in HM patients.” 1
“Severe hemiplegic migraine attacks may result in brain atrophy.” 1
“Adequate prophylaxis may be critical to prevent permanent brain damage in HM patients.” 1
Comments and implications for patients:
This case report reveals valuable information for patients with hemiplegic Migraine and the doctors who treat them. It may be disconcerting to learn that hemiplegic Migraine attacks could result in brain atrophy, but it’s important to remember the second clinical implication noted by the authors — that effective preventive treatment is critical.
This case report is also demonstrative of the need for more research into the pathophysiology and epidemiology of Migraine disease.
David Watson, MD, director of the headache center at West Virginia University, commented:
“This case report is a fascinating case report, and should be seen as such. The HM diagnosed in this case is based primarily on genetic testing, and is not the more typical HM diagnosed. It is difficult to know exactly the mechanism of the brain atrophy and the effect of the prophylactic medications in the case, but it does point to the need for a much better understanding of HM and its long-term effects.” 2
1 Pelzer N, Hoogeveen E, Ferrari M, et. al.Brain atrophy following hemiplegic migraine attacks. Cephalalgia. Online First. First published. July 27, 2017.
2 Email interview with David B. Watson, MD. October 24, 2017.
©Teri Robert, 2017.
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Teri Robert is a leading patient educator and advocate and the author of Living Well with Migraine Disease and Headaches. A co-founder of the Alliance for Headache Disorders Advocacy and the American Headache and Migraine Association, she received the National Headache Foundation’s Patient Partners Award and a Distinguished Service Award from the American Headache Society. Teri can be found on her website, and blog, Facebook, Twitter, StumbleUpon, Pinterest, LinkedIn, and Google+.