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Headache, Cluster
OVERVIEW: Attacks of severe, unilateral headache typically localized in periorbital area and temple associated with ipsilateral lacrimation, rhinorrhea, ptosis, miosis, and nasal congestion. Individual attacks last 30-180 minutes and occur 1-6 times per day. Two forms exist: episodic with attack phases lasting 4-16 weeks, followed by a cluster-free interval of generally 6 months to years duration; and chronic, with a cluster-free interval of less than 1 week in a 12 month period of time.
System(s) affected: Nervous
Genetics: Unknown
Incidence/Prevalence in USA: 0.5-1% of adult population
Predominant age: Mean age of onset: 30 years in men, later in women
Predominant sex: Male > Female (6:1)
SIGNS AND SYMPTOMS: • Sudden onset of severe headache
• Headache reaches crescendo within 15 minutes, lasts < 3 hours
• Pain is unilateral, oculotemporal or oculofrontal; rare in other locations
• Severe, piercing, boring, exploding, penetrating (occasionally throbbing) pain
• Ipsilateral partial Horner's syndrome (ptosis and miosis)
• Lacrimation (84%)
• Injected conjunctiva (58%)
• Ptosis (57%)
• Nasal stuffiness (48%)
• Rhinorrhea (43%)
• Bradycardia (43%)
• Nausea (40%)
• Perspiration (26%)
• Restlessness and agitation during attacks
• Attacks may occur at same time for consecutive days; frequently an attack occurs within 90 minutes of falling to sleep (corresponding to first REM sleep)
CAUSES: Unknown, perhaps:
• Disruption of circadian rhythm based on hypothalamus
• Disturbed autoregulation of cerebral arteries
• Disorder of serotonin metabolism or transmission in CNS
• Disorder of histamine concentrations or receptors
RISK FACTORS: • Male gender
• Age > 30 years
• Small amounts of vasodilators, such as, alcohol or nitroglycerin
• Occasional relationship to previous head trauma or surgery
DIAGNOSIS DIFFERENTIAL DIAGNOSIS: Diagnosis generally made through careful history. Differential includes other head and neck pathology, migraine, trigeminal and other facial neuralgias, chronic paroxysmal hemicrania (probably a cluster variant), temporal arteritis, pheochromocytoma.
LABORATORY: Not useful except to rule out differential diagnosis
Drugs that may alter lab results: N/A
Disorders that may alter lab results: N/A
PATHOLOGICAL FINDINGS: N/A
SPECIAL TESTS: N/A
IMAGING: Generally of little value except in atypical presentations or those unresponsive to therapy
DIAGNOSTIC PROCEDURES: N/A
TREATMENT APPROPRIATE HEALTH CARE: Outpatient except in patient at suicidal risk
GENERAL MEASURES: • During cluster periods, avoid alcohol, bright lights and glare, excessive emotion and stress as these may precipitate attacks
• Avoid narcotic analgesics, especially oral preparations
• Tobacco (high predilection for tobacco abuse in this population) may make patients more refractory to therapy
SURGICAL MEASURES: Radiofrequency trigeminal gangliolysis in carefully selected refractory patients with strictly unilateral attacks
ACTIVITY: • Avoid self-injury during bouts of excruciating pain
• Vigorous physical activity at first symptom may abort attack in some
• Compression of ipsilateral carotid or temporal artery may reduce pain in some. Caution exercised in recommending carotid massage in patient at risk for occult carotid disease.
DIET: • During cluster phase, alcohol even in small amounts frequently precipitates attacks
• Rarely, specific foods may trigger attacks
PATIENT EDUCATION: • Focus on the validity, natural history, and pathology of the condition
• Advise patient to avoid known precipitants
• Assist patient with learning self-treatment methods
• Provide supportive relationship and follow-up
• Avoid high altitudes
MEDICATIONS DRUG(S) OF CHOICE: • General information
• Prophylactic therapy is paramount
• Avoid pain therapy for acute attacks, especially narcotic analgesics
• Assess cardiovascular risk before instituting vasoactive drugs, such as, ergotamine or sumatriptan
• Acute attacks
• Oxygen 100% at 7-10 liters for 10-15 minutes administered through a tight-fitting face mask with patient in sitting position and breathing at normal respiratory rate
• Sumatriptan (Imitrex) 6 mg subcutaneous, maximum of 12 mg per 24 hours with at least 1 hour between injections
• Dihydroergotamine mesylate (DHE 45) 1 mg IM or IV. May teach self-administration with SC.
• Prophylaxis (to shorten cluster period or prevent expected attacks):
• Verapamil 80 mg PO qid spaced evenly through waking hours
• Lithium carbonate (Eskalith) 300 mg 2-4 times a day
• Methysergide (Sansert) 4-10 mg daily divided doses tid or qid. More useful in younger patient in early stage of disease.
• Ergotamine timed to be at peak serum level during anticipated attack, e.g., 2 mg rectal or 1-2 mg oral 2 hours before. This is especially useful to prevent nocturnal attacks.
• Prednisone, various schedules, e.g., 60-80 mg PO for 7 days followed by rapid tapering over 6 days or 40 mg/day for 5 days tapered over 3 weeks. This therapy is initiated while other long-term agent is being employed, such as, verapamil or lithium.
Contraindications: Refer to manufacturer's literature
Precautions: Refer to manufacturer's literature
Significant possible interactions: Refer to manufacturer's literature
ALTERNATIVE DRUGS: • Acute attack:
• Lidocaine intranasal instillation of 1 mL of 4% topical solution slowly on same side as symptoms. Position patient supine with head extended 45 degrees and rotated 40 degrees to the side of pain. May need to premedicate with 1-2 drops of intranasal 0.5% phenylephrine for nasal stuffiness.
• Prophylaxis
• Indomethacin up to 150 mg per day in divided doses. Absolute responsiveness in chronic paroxysmal hemicrania (CPH) and useful in female cluster patients.
• Nifedipine 40-120 mg/day
• Nimodipine up to 240 mg/day
• Combinations of verapamil and lithium with or without ergotamine may be useful when single drug therapy is ineffective
• Histamine desensitization done at certain major headache centers
FOLLOW UP PATIENT MONITORING: • To anticipate cluster bouts and initiate early prophylaxis
• Monitor for adverse medication response and side-effects
• Monitor for unmasking of underlying cardiovascular disorder
• Education for patient and family
PREVENTION/AVOIDANCE: • Alcohol, nitroglycerine, and some foods can induce cluster attack
• Disturbances in sleep cycle can induce attacks (sleep cycle disruption common due to anticipation and occurrence of nocturnal attacks)
• Strong emotions, anger, excessive physical activity may induce attacks
• Tobacco may slow responsiveness to medication
• Narcotics may expedite transformation of episodic cluster to chronic cluster
POSSIBLE COMPLICATIONS: • Self-injury during attack
• Side-effects of medication including unmasking of coronary heart disease
• Potential for drug abuse
EXPECTED COURSE AND PROGNOSIS: • Recurrent attacks
• Prolonged remissions
• Possibility of transformation of episodic cluster to chronic cluster and occasionally chronic cluster to episodic cluster
MISCELLANEOUS ASSOCIATED CONDITIONS:
• Significantly higher incidence of peptic ulcer and coronary heart disease (males)
• Prior history of migraine frequently in female patients
• Increased risk of suicide
AGE-RELATED FACTORS:
Pediatric: Very rare cases reported
Geriatric: N/A
Others:
• With age - more likely to begin in women, often in peri- or post-menopausal years
• Characteristic appearance - leonine face, thickened skin, above average height, more likely to have hazel eye color, and be heavy smokers. No evidence of specific psychologic type.
PREGNANCY: Very rare in pregnancy
SYNONYMS: • Migrainous neuralgia
• Sphenopalatine neuralgia
• Histamine cephalalgia
ICD-9-CM: 346.2 Variants of migraine (including cluster and histamine)
784.0 Headache
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