Diagnostic and therapeutic aspects of hemiplegic migraine

Abstract

Hemiplegic migraine (HM) is a clinically and genetically heterogeneous condition with attacks of headache and motor weakness which may be associated with impaired consciousness, cerebellar ataxia and intellectual disability. Motor symptoms usually last <72 hours and are associated with visual or sensory manifestations, speech impairment or brainstem aura. HM can occur as a sporadic HM or familiar HM with an autosomal dominant mode of inheritance. Mutations in CACNA1A, ATP1A2 and SCN1A encoding proteins involved in ion transport are implicated. The pathophysiology of HM is close to the process of typical migraine with aura, but appearing with a lower threshold and more severity. We reviewed epidemiology, clinical presentation, diagnostic assessment, differential diagnosis and treatment of HM to offer the best evidence of this rare condition. The differential diagnosis of HM is broad, including other types of migraine and any condition that can cause transitory neurological signs and symptoms. Neuroimaging, cerebrospinal fluid analysis and electroencephalography are useful, but the diagnosis is clinical with a genetic confirmation. The management relies on the control of triggering factors and even hospitalisation in case of long-lasting auras. As HM is a rare condition, there are no randomised controlled trials, but the evidence for the treatment comes from small studies.

Migraine as a Cortical Brain Disorder

“…According to the hypothesis linking lack of habituation to reduced cortical activation, this would mean that cortical excitability is further reduced in the preictal period, triggering the “brainstorm” of a migraine attack during which the high activation and increased cortical excitability normalize the habituation phenomenon. Interestingly, normal habituation has been proven to occur in familial hemiplegic migraine and CM, headache disorders characterized by a relevant increase in cortical excitability 93,94,97 …”

CACNA1A Gene Variants in Eight Chinese Patients With a Wide Range of Phenotypes

“…The migraine may be followed by the aura or may occur before the aura, its duration is uncertain, and occasionally absent. The aura lasts from several minutes to hours, and may last up to 4 weeks when hemiplegia acts as the aura (16,17). In rare cases, diplegia has been reported as a motor aura symptom (18).…”

“…Compared with FHM, SHM patients tends to have less non-motor auras, longer and more severe attacks, manifesting as acute encephalopathy, characterized by hemiplegia, coma, fever, convulsions, and so on (19). It is thought to be associated with increased glutamic acid release, decreased cortical spreading depression (CSD) threshold, and prolonged and repetitive CSD, all of which lead to cytotoxic and vasogenic edema (17,20). In our cohort, three patients were diagnosed with SHM1, with the age of onset between 2 to 4 years, which was much earlier than that of FHM1 as reported before (17).…”

“…It is thought to be associated with increased glutamic acid release, decreased cortical spreading depression (CSD) threshold, and prolonged and repetitive CSD, all of which lead to cytotoxic and vasogenic edema (17,20). In our cohort, three patients were diagnosed with SHM1, with the age of onset between 2 to 4 years, which was much earlier than that of FHM1 as reported before (17). All of them suffered severe acute encephalopathy, which was usually triggered by minor head trauma.…”

Migraine and Sport in a Physically Active Population of Students: Results of a Cross‐Sectional Study

“…1,2 In recent decades much progress has been made in the understanding of the pathophysiology of migraine, especially on genetics, biology, and neurophysiology, thus creating new opportunities for effective management through targeted therapies. [3][4][5][6][7][8] Different factors have been identified as being able to provoke, induce or, in contrast, to protect against the precipitation of migraine attacks. 3 The identification of protective and inducing conditions is important for an effective therapeutic approach, and in some cases can be effective in reducing the need for drug treatment.…”