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Cluster Headache

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Cluster Headache
Kyle D. Parish
Basics
Description
  • A primary headache disorder characterized by recurrent attacks of excruciating, short-lasting unilateral headache typically localized to the periorbital and temporal area. Disorder is associated with signs of ipsilateral autonomic dysfunction such as conjunctival injection, lacrimation, rhinorrhea or nasal congestion, facial diaphoresis, miosis, ptosis, and eyelid edema.
  • Attacks last 15–180 min if left untreated.
  • 2 types exist:
    • Episodic: 1 or 2 episodes per year separated by a pain-free interval of at least 1 mo; account for 80% of all cases
    • Chronic: Ongoing attacks of headaches for 1 yr or more without more than 1 mo of remission; represent the remaining 20% of cases
  • System(s) affected: Nervous
  • Synonym(s): Horton's headache; Erythromelalgia of the head; Histaminic cephalalgia; Ciliary or migrainous neuralgia; Erythroprosopalgia of Bing; Petrosal neuralgia of Gardner; Hemicrania periodica neuralgiformis; Sphenopalatine, Vidian, or Sluder neuralgia
Epidemiology
Incidence
  • Predominant age: Most often affects young adults; average age of onset occurs after 30 yrs.
    • A significant diagnostic delay has been reported in most cases (median of 3-yr delay).
    • Symptoms are rare in children.
    • Decrease is often noted after age 70.
  • Predominant gender: Male > Female (reported at just higher than 2:1)
Prevalence
Affects ∼0.05–0.1% of adults
Risk Factors
  • Male gender
  • Age between 20 and 40 yrs
  • Alcohol use
  • Tobacco use, particularly cigarette smoking
  • History of head injury
  • Family history of headaches
  • Shift work
  • Nitroglycerin use
Genetics
Genetic aspects have been noted in twin studies, but no clear locus or transmission mode has been established.
General Prevention
  • Abstinence from alcohol and tobacco, especially during headache bouts
  • Regular sleep cycle
Etiology
  • Exact cause is currently unknown.
  • PET scan and functional MRI studies have established a fundamental role of the hypothalamus in the pathophysiology of cluster headaches.
Commonly Associated Conditions
  • Tobacco use
  • Sleep apnea
  • Seasonal allergic rhinitis
  • Suicidal ideation
Diagnosis
At least 5 attacks fulfilling the following criteria (1,2)[C]:
  • Severe or very severe unilateral periorbital and/or temporal pain lasting 15–180 min if untreated
  • Headache accompanied by at least one of the following (ipsilateral) symptoms:
    • Conjunctival injection and/or lacrimation
    • Nasal congestion and/or rhinorrhea
    • Eyelid edema
    • Facial sweating
    • Miosis and/or ptosis
    • A general sense of restlessness or agitation
  • Attacks from 1 every other day to 8 per day
  • Not attributed to another disorder
History
Diagnosis generally is made through history alone.
Physical Exam
  • Few distinguishing features outside active bout
  • Signs or trigeminal autonomic dysfunction ipsilateral to side of headache during attack
  • Restless and agitated behavior (pacing and rocking while holding head in hands)
Diagnostic Tests & Interpretation
Lab
Useful only to rule out diagnosis included in differential
Imaging
In most cases normal, but used to exclude other diagnosis and/or for individuals not responding to appropriate therapy
Differential Diagnosis
  • Migraine headache
  • Paroxysmal hemicrania
  • Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) syndrome
  • Hemicrania continua
  • Trigeminal neuralgia
  • Intracranial tumor or bleed
  • Orbital tumor or infection
  • Sinusitis
  • Carotid dissection
  • Temporal arteritis
  • Herpes zoster

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Treatment
Pre-Hospital
Treatment in most cases is outpatient.
Medication
  • Avoid treatment with analgesics, particularly narcotics, for acute attacks because they are generally ineffective and risk side effects such as GI bleeding, hepatic injury, and/or opioid dependence.
  • For contraindications, warnings, cautions, and possible drug interactions for each medication listed, please refer to manufacturers' literature.
First Line
  • Acute attacks:
    • Oxygen 100% at a rate of 7 L via face mask for at least 15 min (3)[A]
    • Sumatriptan 20 mg via nasal spray or 6 mg via SC injection, with repeat dose if needed once in a 24-hr period (1)[A]
    • Treatments may be used in combination.
  • Prophylaxis: Verapamil 120–160 mg PO t.i.d. (3)[A]
Second Line
  • Acute attacks:
    • Lidocaine 1 mL of 10% solution placed intranasally with a cotton swab (3)[B]
    • Dihydroergotamine 0.5 mg via nasal spray placed in each nostril (3)[B]; must not be combined with any of the triptans
    • Capsaicin intranasal, ipsilateral nostril t.i.d. ×7 days (3)[B]
  • Prophylaxis:
    • Prednisone 50–80 mg PO daily tapered over 10–12 days (3)[B]
    • Topiramate 100–200 mg PO daily (3)[B]; dose must be tapered up from starting dose of 25 mg.
    • Ergotamine 2–4 mg PO daily in divided doses (3)[B]; should be avoided if current active treatment with a triptan.
    • Divalproex 600–2,000 mg daily (3)[B]
    • Methylergonovine maleate 0.2 mg PO t.i.d. to q.i.d. (3)[B]
    • Lithium 600–1,200 mg PO in divided doses; blood levels must be checked periodically to avoid intoxication.
Complementary and Alternative Medicine
  • Melatonin 10 mg taken at night (3)[B]
  • Massotherapy, physiotherapy, and acupuncture all have been described but not adequately studied to determine effect.
Surgery/Other Procedures
  • Destructive approaches have variable results and irreversible complications (1)[B].
    • Trigeminal section
    • Thermocoagulation of gasserian ganglion
    • Glycerol rhizotomy
    • Radiosurgery of the trigeminal nerve
  • Hypothalamic deep brain stimulation (1)[B]
  • Neurostimulation of the greater occipital nerve
  • Greater occipital nerve blocks (1)[B]
In-Patient Considerations
Admission Criteria
  • Concern that patient is a high risk for suicide
  • Intercranial pathology is considered, requiring inpatient workup.
Ongoing Care
Diet
Avoidance of alcohol is highly recommended during bouts because it is the only known dietary trigger.
Patient Education
  • Little research is available to assess the effectiveness of lifestyle modifications, but exercise, relaxation techniques, biofeedback, and smoking cessation have been suggested to improve quality of life.
  • There is no evidence that exercise or athletic participation is contraindicated during an acute bout of cluster headache, but severity of headache may limit ability of individual participation during episode.
Prognosis
  • Very unpredictable course:
    • Recurrent attacks
    • Prolonged remission
  • Episodic type can evolve to chronic type.
  • Total remission has been described.
Complications
  • Medication side effects
  • Self-injury or suicide during attacks
  • Potential for prescription drug abuse
References
1. Leroux E, Ducros A. Cluster headache. Orphanet J Rare Dis. 2008;3:20
2. The International Classification of Headache Disorders 2nd Edition. Cephalalgia. 2004;24: 1–160.
3. Beck E, Sieber WJ, Trejo R. Management of cluster headache. Am Fam Physician. 2005;71:717–724.
Additional Reading
Bussone G. Cluster headache: from treatment to pathophysiology. Neurol Sci. 2008;29 (Suppl 1):S1–S6.
Codes
ICD9
  • 339.00 Cluster headache syndrome, unspecified
  • 339.01 Episodic cluster headache
  • 339.02 Chronic cluster headache
Clinical Pearls
  • Patients are often misdiagnosed with secondary cause of headache, delaying definitive diagnosis.
  • Once diagnosis is made, effort should be directed toward avoidance of narcotic analgesic treatment, which may prolong bouts and risk patient dependence.
  • There is currently no known curative treatment.


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