Hemiplegic Headache: Diagnosis and Management
Diagnosis
Hemiplegic migraine is diagnosed clinically based on ICHD-3 criteria requiring at least two attacks with fully reversible motor weakness as the aura symptom, lasting 5-72 hours, accompanied by or followed by headache within 60 minutes. 1
Clinical Diagnostic Criteria
The diagnosis requires 1, 2, 3:
- At least two attacks with motor weakness as aura
- Motor aura spreads gradually over ≥5 minutes
- Duration of 5-72 hours (typically <72 hours)
- At least one additional aura symptom: visual, sensory, speech/language, or brainstem symptoms
- Headache accompanies or follows aura within 60 minutes
- Not better explained by another diagnosis
Essential History Elements
Document the following 1:
- Age at onset (often at or around puberty)
- Duration and frequency of attacks
- Pain characteristics: location, quality, severity, aggravating factors
- Accompanying symptoms: photophobia, phonophobia, nausea, vomiting
- Specific aura features: gradual spread, succession of symptoms, unilateral presentation
- Family history of migraine (strongly positive in familial hemiplegic migraine)
Familial vs. Sporadic Classification
- Familial hemiplegic migraine (FHM): At least one first- or second-degree relative with hemiplegic migraine 4, 2
- Sporadic hemiplegic migraine (SHM): No affected relatives 4, 2
Mandatory Diagnostic Workup
Neuroimaging (MRI preferred) is essential to exclude stroke, TIA, or structural lesions, which present with sudden simultaneous onset rather than gradual spread over ≥5 minutes. 5
Additional testing to exclude mimics 5, 4, 2:
- EEG to rule out epilepsy
- CSF analysis to exclude encephalitis
- Genetic testing for CACNA1A, ATP1A2, and SCN1A mutations (confirms but does not rule out diagnosis) 4, 3
Critical pitfall: Conventional cerebral angiography is contraindicated as it may provoke an attack 4
Acute Treatment
Acetaminophen and NSAIDs are first-line acute treatments for hemiplegic migraine attacks. 4
First-Line Acute Therapy
- NSAIDs (ibuprofen, diclofenac potassium, acetylsalicylic acid) 1, 6, 4
- Acetaminophen 4
- Administer early in the headache phase for maximum effectiveness 1
Adjunctive Medications
- Prokinetic antiemetics (domperidone or metoclopramide) for nausea/vomiting 1
Controversial: Triptans
Triptans can be prescribed when headaches are not relieved sufficiently with common analgesics, though their use remains controversial in hemiplegic migraine. 4
The controversy stems from theoretical concerns about vasoconstriction during motor aura, but insufficient evidence exists to contraindicate them 4
Medications to Avoid
- Ergot alkaloids: poorly effective and potentially toxic 1
- Opioids and barbiturates: questionable efficacy, risk of dependency 1
Management of Severe/Prolonged Attacks
- Consider hospitalization for long-lasting auras 2
- No effective acute treatment currently exists specifically for severe aura symptoms 4
- Monitor for complications: seizures, decreased consciousness, cytotoxic edema 7
Preventive Treatment
Preventive therapy should be initiated when attack frequency exceeds 2 attacks per month or when severe attacks pose significant burden. 6, 4
First-Line Preventive Options
In no strictly preferred order 4:
- Flunarizine
- Sodium valproate
- Lamotrigine
- Verapamil
- Acetazolamide
Second-Line Preventive Options
- Topiramate (less evidence but can be considered) 4
- Candesartan 6, 4
- Pizotifen 4
- Amitriptyline (particularly with comorbid sleep disturbances) 6
Controversial: Beta-Blockers
Propranolol use in hemiplegic migraine is controversial, but insufficient evidence exists to contraindicate beta-blockers. 4
Novel Therapies
CGRP monoclonal antibodies (galcanezumab) have shown promising results in case series, reducing both headache days and days with motor weakness. 8
- Reduced monthly headache days in 3/6 patients 8
- Reduced monthly days with weakness in 4/6 patients 8
- Improved disability scores in 5/6 patients 8
- Well tolerated with no adverse events 8
Duration of Preventive Therapy
- Assess efficacy after 2-3 months for oral medications 1
- Consider pausing after 6-12 months of successful treatment 1
- Early prophylaxis may prevent brain atrophy from severe attacks 7
Critical Monitoring and Follow-Up
Warning Signs Requiring Urgent Attention
Severe hemiplegic migraine attacks with seizures and decreased consciousness can cause cytotoxic edema and progressive brain atrophy. 7
Monitor for 7:
- Prolonged attacks (>72 hours)
- Seizures during attacks
- Altered consciousness
- Progressive neurological deterioration
Medication Overuse Prevention
Educate patients that frequent use of acute medications (≥15 days/month for NSAIDs, ≥10 days/month for other acute medications) risks medication-overuse headache. 1, 6
Long-Term Monitoring
- Maintain headache diary to track triggers and treatment response 6
- Regular follow-up to assess frequency, severity, and disability 6
- Consider genetic counseling for familial cases 4, 3
Non-Pharmacological Management
Implement lifestyle modifications 6:
- Regular meals and consistent sleep patterns
- Stress management techniques
- Adequate hydration
- Regular exercise (40 minutes three times weekly)
- Cognitive behavioral therapy or biofeedback
Key Clinical Pitfalls
- Misdiagnosis as stroke: Remember gradual spread over ≥5 minutes distinguishes hemiplegic migraine from stroke 5
- Performing conventional angiography: This is contraindicated and may trigger attacks 4
- Withholding preventive therapy: Early prophylaxis may prevent permanent brain damage in severe cases 7
- Abandoning preventive treatment prematurely: Benefits may take several weeks to months 6
- Ignoring genetic implications: Familial cases have autosomal dominant inheritance requiring family counseling 4, 3