cluster headache (suicide headache)

Classification

• most common of the trigeminal autonomic cephalgias

Etiology

• smoking is a risk factor

Epidemiology

• primarily affects men (72%)
  • females may be more severely affected than men
• onset generally in late 20s, but may occur at any age

Clinical manifestations

• main feature is periodicity
  • cluster period lasts 2-3 months; 6-8 weeks
  • recurs every 1-2 years; 2-6 months
  • average remission is about 2 years
  • may occur seasonally
  • 20% of patients do not have remission or remission does not last > 1 month
• attacks of 15 minutes to 3 hours duration
  • occur 1-8 times daily (mean duration 1 hour)
  • longest duration of the trigeminal autonomic cephalgias
  • headaches repeat for 6-8 weeks, then disapper for months to years
• nocturnal attacks occur in > 50%
• not associated with aura
• pain reaches a peak in about 10-15 min & lasts 45-60 min
• pain
  • generally unilateral, excruciating, penetrating, non-throbbing & maximally behind eye
  • retro-orbital pain (92%) or periorbital pain
  • temporal 70%, trigeminal
  • pain may also occur in teeth, forehead, jaw, cheek, neck
  • pain unilateral during a single episode, but may change side from episode to episode
• autonomic features are present
  • due to sympathetic block & parasympathetic overactivation
  • ipsilateral lacrimation (tearing)
  • injection of conjunctiva
  • nasal stuffiness or rhinorrhea
  • ptosis (may become permanent)
  • miosis
  • periorbital swelling
  • bradycardia
• case presenting with photophobia, nausea
• scalp, face & carotid artery may be tender
• restlessness, pacing

Diagnostic criteria

• at least 5 attacks fulfulling criteria
• severe unilateral orbital, supraorbital &/or temporal pain lasting 15 minutes to 3 hours untreated
• either
  • at least one sign/symptom ipsilateral to the headache
    • conjunctival injection &/or lacrimation
    • nasal congestion &/or rhinorrhea
    • eyelid edema
    • forehead & facial perspiration
    • forehad & facial flushing
    • sensation of fullness in the ear
    • miosis &/or ptosis
  • a sense of restlessness or agitation
• attach frequency of 1 every other day to 8/day when a cluster occurs
• not better explained by other diagnosis

Laboratory

• erythrocyte sedimentation rate & serum C-reactive protein for suspected temporal arteritis

Radiology

• MRI neuroimaging of patients with suspected cluster headache to exclude structural lesions
• head CT if intracranial hemorrhage is suspected

Differential diagnosis

• primary stabbing headache
  • duration seconds, but may be up to 1-2 minutes
  • no associated cranial autonomic features (lacrimation or tearing)

Management

• prophylaxis is mainstay of therapy
  • verapamil* or other calcium channel blocker
  • lithium carbonate 600-900 mg/day in divided doses
  • combination of verapamil & lithium best for chronic cluster headaches
  • methysergide
    • effective early in the course of disease
    • least effective in later years
  • ergotamine is effective for nocturnal attacks
  • 40 mg prednisone tapered over 3 weeks
  • anticonvulsants may be useful
    • gabapentin, valproate, topiramate
  • galcanezumab (weak recommenation)
  • melatonin 10 mg in the evening
  • no strong recommendations
  • continue prophylaxis for 4-6 weeks after remission
• abortive therapy
  • 100% oxygen inhalation (weak recommendation)
    • 5-8 L/min for 10 minutes;
    • 12 L/min by face mask for 15 minutes 80% effective
  • triptan for acute headadche (weak recommendation)
    • sumatriptan SC
    • zolimitriptan nasal
    • ergotamine suppository
  • glucocorticoids to break cycle & as bridge therapy:
    • dexamethasone 8 mg
    • prednisone 100 mg PO QD for 5 days, follow with taper of 20 mg every 3 days
  • intranasal lidocaine 4%
  • capsaicin in ipsilateral nostril
  • modified trigeminal cisternal injection of glycerol for chronic cluster headache
  • chlorpromazine may help some patients
  • olanzapine (Zyprexa) 5-10 mg PO QD PRN
  • opiates when other agents fail
  • hand-held vagus nerve stimulator
  • no strong recommendations
• surgical intervention
  • chronic cluster headache
  • not indicated for episodic cluster headache

* drug of choice

More general terms

• trigeminal autonomic cephalgia

More specific terms

• Horton's cephalgia