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Migraine and Other Primary Headache Disorders

Chapter 441 | Part 13: Neurologic Disorders

KEY CLINICAL POINTS

  • Migraine is the second most common cause of headache and the most common neurologic cause of disability, affecting ~15% of women and 6% of men.
  • Pathophysiology involves dysfunction of monoaminergic systems, trigeminovascular activation, and CGRP-mediated mechanisms.
  • Treatment includes acute therapies (triptans, NSAIDs, CGRP antagonists) and preventive strategies (beta-blockers, anticonvulsants, neuromodulation).

1. DEFINITION & OVERVIEW

Migraine is a primary headache disorder characterized by recurrent attacks of headache with associated neurologic features. Tension-type headache (TTH) and trigeminal autonomic cephalalgias (TACs) are other primary headache disorders. The International Classification of Headache Disorders (ICHD-3) provides diagnostic criteria for these conditions.

Table 441-1: Primary Headache Disorders

Category Subtypes
Migraine 1.1 Migraine without aura, 1.2 Migraine with aura, 1.3 Chronic migraine
Tension-Type Headache 2.1 Infrequent, 2.2 Frequent, 2.3 Chronic
Trigeminal Autonomic Cephalalgias 3.1 Cluster headache, 3.2 Paroxysmal hemicrania, 3.3 SUNCT/SUNA

1.1 Primary Headache Disorders

Migraine, TTH, and TACs (e.g., cluster headache) are classified as primary headaches. Migraine has subtypes including migraine with/without aura, chronic migraine, and hemiplegic migraine.

1.2 Diagnostic Criteria

Migraine diagnosis requires recurrent attacks with unilateral, pulsating headache, moderate/severe intensity, aggravation by activity, and associated symptoms (nausea, photophobia, phonophobia).

2. EPIDEMIOLOGY

Migraine affects ~15% of women and 6% of men globally. It is more common in women, with prevalence peaking in reproductive years. Chronic migraine is defined as ≥ 15 headache days/month for ≥ 3 months.

2.1 Risk Factors

Genetic predisposition, hormonal fluctuations, environmental triggers (stress, sleep changes, dietary factors), and family history of migraine.

2.2 Demographics

Women are 2–3 times more likely to have migraine than men. Age of onset varies, with episodic migraine common in adolescents and chronic migraine increasing with age.

3. ETIOLOGY & PATHOPHYSIOLOGY

Migraine involves complex interactions between genetic, environmental, and neurobiological factors. Key mechanisms include cortical spreading depression, trigeminovascular activation, and dysregulation of 5-HT and CGRP pathways.

Table 441-2: Migraine Symptoms by Attack Phase

Phase Symptoms
Premonitory (Prodromal) Neck discomfort, fatigue, yawning, polyuria, food cravings
Aura Scintillating scotoma, visual disturbances
Headache Phase Pain, nausea, photophobia, phonophobia, allodynia
Postdrome Tiredness, concentration impairment

3.1 Neurotransmitter Involvement

5-HT (serotonin) and dopamine play critical roles. Dopamine receptor antagonists (e.g., metoclopramide) are effective in acute treatment. CGRP (calcitonin gene-related peptide) is a key mediator of pain.

3.2 Genetic Mutations

Familial hemiplegic migraine (FHM) is linked to mutations in CACNA1A (FHM1), ATP1A2 (FHM2), and SCN1A (FHM3).

4. CLINICAL FEATURES

Migraine attacks are characterized by unilateral, pulsating pain with associated neurologic symptoms. Tension-type headache is typically bilateral, non-pulsating, and without associated features.

4.1 Migraine Features

Unilateral, throbbing pain; nausea/vomiting; photophobia/phonophobia; aura (visual/brainstem symptoms).

4,2 Tension-Type Headache

Bilateral, pressing/pressure-like pain without associated neurologic features. Often featureless compared to migraine.

5. DIFFERENTIAL DIAGNOSIS

Distinguish migraine from TTH, cluster headache, and secondary headaches (e.g., sinusitis, brain tumors). Key differentiators include aura, duration, and associated autonomic symptoms.

5.1 Red Flags for Secondary Headache

Sudden severe headache (thunderclap), neurological deficits, fever, or history of trauma.

6. INVESTIGATIONS & DIAGNOSIS

Diagnosis is based on clinical criteria (ICHD-3). Imaging (MRI) is used to exclude secondary causes, especially in atypical presentations or chronic cases.

Table 441-3: Simplified Diagnostic Criteria for Migraine

Criteria Details
Attack Duration 4–72 hours
Features Unilateral pain, nausea/vomiting, photophobia, phonophobia
Exclusion No other cause for headache

6.1 Diagnostic Criteria

Table 441-3 outlines simplified diagnostic criteria for migraine, including attack duration, features, and associated symptoms.

7. MANAGEMENT & TREATMENT

Acute treatment includes triptans, NSAIDs, and CGRP antagonists. Preventive therapy targets underlying mechanisms with beta-blockers, anticonvulsants, and neuromodulation.

Table 441-4: Acute Migraine Treatment

Drug Dosage Route
Sumatriptan 50–100 mg PO Oral
Zolmitriptan 2.5 mg PO Oral
Rimegepant 75 mg PO Oral
Dihydroergotamine 1 mg IV/SC Parenteral

7.1 Acute Attack Therapies

Triptans (e.g., sumatriptan, rizatriptan), NSAIDs (ibuprofen, naproxen), and gepants (rimegepant, ubrogepant) are first-line options. Oxygen and non-invasive vagus nerve stimulation (nVNS) are also used.

7.2 Preventive Therapy

Beta-blockers (propranolol), anticonvulsants (topiramate), CGRP inhibitors (erenumab), and neuromodulation (sTMS, nVNS) are recommended for chronic migraine.

8. PROGNOSIS & COMPLICATIONS

Migraine is generally benign but can significantly impact quality of life. Complications include status migrainosus, persistent aura without infarction, and migrainous infarction.

8.1 Chronic Migraine

Defined as ≥ 15 headache days/month for ≥ 3 months. Risk factors include medication overuse and frequent acute attacks.

9. SPECIAL CONSIDERATIONS

Pregnancy, pediatric, and elderly populations require tailored management. Medication overuse headache is a common complication of frequent acute treatment.

9.1 Pregnancy

Avoid NSAIDs in late pregnancy. Paracetamol is preferred. Triptans are generally contraindicated.

9.2 Medication Overuse

Chronic use of analgesics (e.g., NSAIDs, triptans) can lead to medication-overuse headache. Discontinuation is often required for prevention.

10. KEY POINTS & CLINICAL PEARLS

Use the MIDAS score to assess migraine disability. Prioritize triptans for acute attacks and CGRP inhibitors for prevention. Avoid NSAIDs in patients with cardiovascular risk. Neuromodulation (sTMS, nVNS) is effective for acute and preventive treatment.