neurology chapter 11 Cluster Headaches Flashcards
Cluster Headaches
Description
- Cluster headaches are classified as a trigeminal autonomic cephalgia and may be classified episodic or chronic.
- These headaches may be considered primary, secondary, or both.
Cluster headaches
Incidence
- 1% of population
- men affected 2-3 times more often than women
- cluster headache incidence is equivalent to only 2-9% of migraine incidence.
Cluster headache
Etiology
- Pathophysiology incompletely understood
- periodicity suggests possible biological clock within hypothalamus, with central disinhibition of the trigeminal nociceptive pathways.
- Possible causative factor is release of histamines or serotonin.
- Dilation of blood vessels and resulting application of pressure to the trigeminal nerve thought to be a secondary response.
Cluster headache
Risk factors
- Heritable tendency in first-degree relatives, fourteenfold to forty-eightgold increased risk.
- male sex
- age of onset 20-40 years
- previous head trauma or surgery
- tobacco and alcohol use
Cluster headaches
Triggers
- tobacco use
- alcohol intake
- nitroglycerin use
- volatile/strong smells
- body temp elevation
- hypoxic conditions (sleep apnea, altitude disorders
- link between hypothalamus and sleep regulation
- closure glaucoma
- brain stem gliomas
- cavernous sinus syndromes
- craniopharyngioma
- herpes zoster
- changing sleep patterns
- daytime napping
- stress, allergens, seasonal changes
Cluster headaches
Assessment Findings
Symptoms
- Onset: sudden, peaking in 10-15 minutes
- Duration: 5 minutes to 3 hours
- pain intensity: severe to very severe
- pain quality: boring, burning, lancinating, sharp
- location: unilateral orbital, supraorbital, temporal
- less common: jaw, cheek, teeth, ear, nose, or neck.
- most commonly occurs at night (with onset of REM sleep)
- rocking and pacing occur in 90% of patients
- common migraine features (photophobia, phonophobia, nausea and vomiting) may be present, but alone are not key diagnostic features.
Cluster headaches
Characteristic assessment findings
- Leonine facies, multi furrowed and thickened skin with prominent folds; a broad chin, vertical forehead creases, and nasal telangiectasis
- parasympathetic over-activity: ipsilateral lacrimation, conjunctival injection, rhinorrhea or congestion
- ocular sympathetic paralysis: mild Horner syndrome (ptosis, myosis, anihidrosis) that may persist between attacks.
- eyelid and facial swelling
- aural fullness
- bradycardia
- facial blushing or pallor
- scalp and facial tenderness
- ipsilateral carotid tenderness
Cluster headaches
Differential Diagnosis
- eye disorders: acute optic neuritis, angle-closure glaucoma
- intracranial hemorrhage
- migraine variants
- persistent idiopathic facial pain
- pituitary tumors
- postherpetic neuralgia
- subarachnoid hemorrhage
- trigeminal neuralgia
- giant cell arteritis
- paroxysmal hemicrania
Cluster headaches
Diagnostic criteria
Diagnosis is based on clinical history. Symptoms should fulfill diagnostic criteria set by the International Headache Society.
Cluster Headache Diagnostic Criteria
A. At least five attacks fulfilling criteria B-D below.
B. Severe or very severe unilateral pain lasting 15-180 minutes.
C. Headaches accompanied by at least one of the following:
1. Ipsilateral conjunctival injection and/or lacrimation.
2. Ipsilateral nasal congestion and/or rhinorrhea.
3. Ipsilateral eyelid edema.
4. Ipsilateral forehead and facial sweating.
5. Ipsilateral miosis and/or ptosis.
6. Sense of restlessness or agitation.
D. Frequency of one every other day to eight per day (Also cyclical, occurring around the same time each day)
E. Not attributed to another disorder.
Diagnostic Criteria
Cyclical
Last 1-3 months and occurring around the same month each year; may have prolonged symptom-free periods lasting months to years.
- episodic form: disctinct circannual periodicity with at least two cluster phases lasting 7 days to 1 year; seperated by a cluster-free interval of 1 month or longer.
- chronic form; occur regularly without periods of remission, or cluster-free interval is shorter than 1 month.
Diagnostic criteria
Continued
- Pain may shift sides between attacks, but not during the attack itself.
-Laboratory testing and imaging to rule out other possible causes, consider:
~ ESR, pituitary function, thyroid function, LH, FSH, insulin-like growth factor 1, cortisol, prolactin, testosterone, estradiol, progesterone, glucose, growth hormone
~ CT or MRI of the brain
~ Polysomnography
~ ECG (to exclude conduction abnormalities resulting from medications)
Cluster headache
Prevention
- Avoid triggering substance (alcohol, nicotine)
- temper, strong emotions
- maintain usual sleep/wake hours
- pharmacologic interventions for prophylaxis
- avoidance of vasodilators
Cluster headache
Nonpharmacologic management
- deep brain stimulation
- greater occipital nerve blocks
- relaxation training
- thermal biofeedback
- cognitive behavioral therapy
Cluster headache
Pharmacologic therapy
- Compressed Air
- Abortive agents, Serotonin 5HT1 receptor agonists
- Corticosteroids
- Calcium channel blocker
- Antipsychotic Agent
- Antiepileptics
Compressed Air
- generally safe. caution in lung disease.
- 10-15L/min using non-rebreather mask with resevoir.
- must order home O2
- Not contraindicated in hypertension or vascular disease.
- relief obtained in 15 minutes.
Serotonin 5HT1 receptor agonists (Triptans)
general comments
- monitor for angina, cerebrovascular events, GI ischemic events
- Do not use within 24 hours of ergotamine medications or other triptans.
- Take as soon as possible after onset of headache.
5HT1 receptor agonists
Sumatriptan (Imitrex)
- subQ or nasal spray
- evidence of human fetal risk
- consider ECG monitoring in patients with likelihood of unrecognized coronary disease.
- watch for drug interactions, especially with SSRIs
- unknown safety in treating more than 4 headaches in 30 days.
5HT1 receptor agonists
Zolmitriptan nasal spray (Zoming AMT)
- Evidence of human fetal risk
- ZMT tabs: disolve under tongue and swallow without water
- do not remove tablet from blister until immediately before using
- do not break ZMT in half
dosage adjustment for hepatic dysfunction
Corticosteroids
general comments
- fastest-acting agents for inducing remission
- treatment course of 18 days is most effective, providing time to increase prophylactic treatment to therapeutic levels.
- monitor for Cushing syndrome, sodium and fluid retention, mood swings
Corticosteroids
Prednisone
- Pregnancy: contraindicated
- cautious use in CV disease, diabetes
- without prophylactic treatment, headaches likely to recur when taper is complete
- steroid-related adverse effects are likely if taper is prolonged
- monitor electrolytes
Calcium channel blocker
general comments
- off-label use
Calcium channel blocker
Verapamil (Calan)
- Evidence of human fetal risk
- monitor LFTs, BP, HR, ECG
- slow titration due to potential ECG changes
- avoid grapefruit
Antipsychotic agent
general comments
- off-label use
- baseline thyroid and renal function should be performed prior to therapy
- tremor, confusion, lethargy and ataxia at high doses
Antipsychotic agent
Lithium carbonate
- pregnancy: contraindicated
- pregnancy test before initiation of therapy
- monitoring of lithium levels
- do not cut/crush/chew ER form
Antiepileptics
Topiramate (Topamax)
- evidence of human fetal risk
- contraindicated in untreated elevated intraocular pressure
- baseline and periodic measurement of serum bicarbonate recommended.
- depression and mood problems may occur.
- increased risk of kidney stones
- monitor for drug interactions, especially with oral contraceptives, metformin, lithium, carbonic anhydrase inhibitors
- dosage adjustment required for renal and hepatic dysfunction, older adults
- dosing for sprinkle caps exactly as for tablets
Cluster headaches
Special considerations
- avoid triptans in patients with CAD, poorly controlled HTN
- Triptans most effective if given during early headache phase. Due to short duration, oral preparations are not considered abortive.
- Avoid concomitant use of triptans and ergotamine within a 24-hour period
- Melatonin may be helpful in cluster headache as a preventive; one controlled trial demonstrated superiority to placebo. Doses up to 9mg daily are typically used.
- Less well-studied alternatives for acute treatment include intranasal dihydroergotamine, intranasal lidocaine, and intranasal capsaicin.
- reserve narcotics for infrequent use due to addiction potential
- advise smoking cessation
Cluster headache
Consultation / Referral
- refer to neurologist for severe headaches or unresponsive to drug therapy.
- Atypical features requiring further investigation:
~ Absence of periodic pattern
~ residual headache between exacerbations
~ bilateral headache
~ incomplete or minimal response to standard therapy
~ presence of lateralizing findings on examination
Cluster headache
Follow-up
- return to clinic or emergency department if headache unresolved after treatment, becomes more severe, or varies from the usual pattern
- periodically monitor ECG, thyroid function, renal function and electrolytes
- follow-up visits to monitor headache response to therapy and potential for side effects
- taper of preventive medications
~ episodic: completely headache-free for 2 weeks and/or moved beyond typical cluster period duration
~ chronic: after 6-12 months of therapy, can consider taper, as long as headaches do not recur.
Cluster headaches
Expected course
- recurrent attacks are usual until cycle can be interrupted
- symptoms typically resolve with increasing age
Cluster headaches
Possible complications
- no real long-term complications, but may be excruciating and incapacitating
- depression and aggression with SI common in up to 55% of patients
- possibility of self-injury during an attack
- risk of addiction to narcotic analgesics.