Diagnostic and therapeutic aspects of hemiplegic migraine

Area of Science:

• Neurology
• Genetics
• Rare Diseases

Background:

• Hemiplegic migraine (HM) is a rare, heterogeneous neurological disorder characterized by migraine attacks with transient motor weakness.
• Symptoms can include impaired consciousness, ataxia, and intellectual disability, with motor deficits typically lasting under 72 hours.
• HM presents sporadically or familiarly with autosomal dominant inheritance, linked to mutations in ion transport genes (CACNA1A, ATP1A2, SCN1A).

Purpose of the Study:

• To review the epidemiology, clinical presentation, diagnostic assessment, differential diagnosis, and treatment of hemiplegic migraine.
• To consolidate current evidence for managing this rare neurological condition.

Main Methods:

• Literature review focusing on epidemiology, clinical features, diagnosis, and treatment of hemiplegic migraine.
• Analysis of diagnostic tools including neuroimaging, CSF analysis, EEG, and genetic confirmation.
• Evaluation of management strategies based on available evidence from small studies.

Main Results:

• The differential diagnosis for HM is extensive, encompassing various migraine types and conditions causing transient neurological symptoms.
• Diagnosis is primarily clinical, often confirmed by genetic testing, with neuroimaging and other tests aiding the assessment.
• Management involves identifying and controlling triggers, with hospitalization considered for prolonged auras.

Conclusions:

• Hemiplegic migraine requires a broad differential diagnosis and a clinical diagnosis supported by genetic confirmation.
• Evidence for treatment is limited due to the rarity of HM, with management strategies focused on trigger avoidance and supportive care.
• Further research is needed to establish evidence-based treatment guidelines for hemiplegic migraine.