headache
HEADACHE
Headache is a very common problem presenting to primary care physicians and neurologists.
Most headaches are diagnosed based on the history & physical exam w/ no imaging or laboratory examination necessary.
Other types of headaches such as secondary headaches may require further evaluation & referral to a specialist.

Classification

Classification of Headache

  • Classified as primary, secondary, and other headaches

Primary Headaches

  • There is no underlying condition
  • No red flag signs and symptoms
  • Low risk of serious headache
  • Do not require neuroimaging

Migraine Headache

  • Includes migraine without aura, migraine with aura, chronic migraine, complications of migraine, probable migraine, or episodic syndromes that may be associated with migraine
  • Diagnostic criteria for migraine without aura
    • ≥5 headache episodes fulfilling the following:
      • Lasts 4-72 hours without treatment or unsuccessful treatment
      • ≥2 of the following: unilateral location, pulsating quality, moderate or severe pain intensity, and/or aggravation by or causing avoidance of routine physical activity such as walking or climbing stairs
    • During headache ≥1 of the following:
      • Nausea and/or vomiting
      • Photophobia and phonophobia
    • Headache is not accounted for by another disorder
  • Diagnostic criteria for migraine with aura
    • ≥2 headache episodes fulfilling the following:
      • ≥1 of the following fully reversible aura symptoms (visual, sensory, speech and/or language, brainstem, retinal)
    • ≥2 of the following characteristics:
      • ≥1 aura symptom spreads gradually over >5 minutes, and/or 2 or more symptoms occur in succession
      • Each individual aura symptom lasts 5-60 minutes
      • ≥1 aura symptom is unilateral
      • The aura is accompanied, or followed within 60 minutes, by headache
    • Headache is not accounted for by another disorder and transient ischemic attack has been excluded
  • Diagnostic criteria for chronic migraine
    • Headache (tension-type-like and/or migraine-like) occurring for >15 days per month for >3 months
    • ≥5 attacks fulfilling the criteria for migraine without aura and/or migraine with aura for >8 days per month for >3 months
    • Believed by the patient to be migraine at onset and relieved by a triptan or ergot derivative
    • Headache is not accounted for by another disorder and transient ischemic attack has been excluded

Tension-Type Headache

  • Most common form of headache with life-time prevalence in the general population of 30-78%
  • Includes infrequent episodic tension-type headache, frequent episodic tension-type headache, chronic tension-type headache or probable tension-type headache
  • Women are affected slightly more often than men
  • Nociceptors in the pericranial myofascial tissues are likely source of tension headaches
    • Individuals who experience chronic tension-type headaches have increased sensitivity to pressure, electrical stimuli, and thermal stimuli in the pericranial myofascial tissue, and may find normally harmless stimuli painful
  • Episodic tension-type headache occurs in attack-like episodes, with variable and often very low frequency and mostly short-lasting
  • Characterized by bilateral mild to moderate pressure without other associated symptoms
    • Headache can be unilateral but is more often generalized
    • Typically described as pressure or tightness, like a vice or tight band around the head, and commonly spreads into or arises from the neck
    • Increased pericranial tenderness is the most significant abnormal finding
  • Have normal neurologic examination and no neuroimaging nor laboratory tests needed
  • May be stress-related or associated with functional or structural cervical or cranial musculoskeletal abnormality
  • Previously used terms are tension headache, muscle contraction headache, psychomyogenic headache, stress headache, ordinary headache, essential headache, idiopathic headache, psychogenic headache
  • Diagnostic criteria for infrequent episodic tension-type headache
    • ≥10 episodes of headache which occurs on <1 day/month on average (<12 days/year) and fulfilling the following:
      • Lasts from 30 minutes to 7 days
      • ≥2 of the following characteristics: bilateral location, pressing or tightening (non-pulsating) quality, mild or moderate intensity, not aggravated by routine physical activity such as walking or climbing stairs, absence of nausea nor vomiting and photophobia or phonophobia
      • Headache is not accounted for by another disorder
  • Diagnostic criteria for frequent episodic tension-type headache
    • ≥10 episodes of headache which occurs on 1-14 days/month on average for >3 months (>12 and <180 days/year) and fulfilling the following:
      • Lasts from 30 minutes to 7 days
      • ≥2 of the following characteristics: bilateral location, pressing or tightening (non-pulsating) quality, mild or moderate intensity, not aggravated by routine physical activity such as walking or climbing stairs, absence of nausea nor vomiting and photophobia or phonophobia
    • Headache is not accounted for by another disorder
  • Diagnostic criteria for chronic tension-type headache
    • Headache which occurs on >15 days/month on average for >3 months (>180 days/year) and fulfilling the following:
      • Lasts hours to days, or unremitting
      • ≥2 of the following characteristics: bilateral location, pressing or tightening (non-pulsating) quality, mild or moderate intensity, not aggravated by routine physical activity such as walking or climbing stairs, absence of moderate or severe nausea nor vomiting and photophobia, or mild nausea, or phonophobia
    • Headache is not accounted for by another disorder
  • Diagnostic criteria for probable tension-type headache
    • Tension-type-like headache missing one of the features required to fulfill all criteria for a subtype of tension-type headache
    • Not fulfilling criteria for another headache disorder

Trigeminal Autonomic Cephalalgia

  • Includes cluster headache, paroxysmal hemicrania, short-lasting unilateral neuralgiform headache attacks, hemicrania continua, or probable trigeminal autonomic cephalalgia
  • Cluster headaches are the most common trigeminal autonomic cephalalgia
    • Characterized by daily occurrence of several brief episodes of severe headache with associated autonomic symptoms
    • Age of onset varies, with 70% of patients occurring before 30 years old
    • Affects mostly men with male to female ratio of 6:1
    • Very often in smokers
    • Typically occurs daily, at a similar time each day for about 6-12 weeks, once a year or two years, often at the same time each year
    • Relatively rare type of headache
  • Diagnostic criteria for cluster headache
    • ≥5 attacks fulfilling the following:
      • Severe or very severe unilateral orbital, supraorbital and/or temporal pain lasting 15-180 minutess without treatment
      • Either or both of the following: >1 of the following manifestations, ipsilateral to the headache (conjunctival injection and/or lacrimation, nasal congestion and/or rhinorrhea, eyelid edema, forehead and facial sweating, forehead and facial flushing, sensation of fullness in the ear, miosis and/or ptosis and/or sense of restlessness or agitation
      • Attacks occur between 1 every other day and 8 per day for more than half of the time when the headache is active
    • Headache is not accounted for by another disorder
  • Episodic cluster headache fulfills criteria for cluster headache, occurring in bouts (cluster periods), and >2 cluster periods lasting from 7 days to 1 year without treatment and separated by pain-free remission periods of >1 month
  • Chronic cluster headache fulfills criteria for cluster headache and occurs without remission period or with remissions lasting <1 month, for ≥1 year
  • Diagnostic criteria for paroxysmal hemicrania
    • ≥20 attacks fulfilling the following:
      • Severe unilateral orbital, supraorbital and/or temporal pain lasting for 2-30 minutes
      • ≥1 of the following manifestations, ipsilateral to the pain: conjunctival injection and/or lacrimation, nasal congestion and/or rhinorrhea, eyelid edema, forehead andfacial sweating, forehead and facial flushing, sensation of fullness in the ear, miosis and/ or ptosis
      • Attacks occur > 5 per day for more than half of the time
      • Attacks are prevented absolutely by therapeutic doses of indomethacin
    • Headache is not accounted for by another disorder
  • Episodic paroxysmal hemicrania fulfills criteria for paroxysmal hemicrania with occurring bouts, and with >2 bouts lasting from 7 days to 1 year without treatment and separated by pain-free remission periods of >1 month
  • Chronic paroxysmal hemicrania fulfills criteria for paroxysmal hemicrania and occurs without remission period or with remissions lasting <1 month, for at least 1 year
  • Diagnostic criteria for short-lasting unilateral neuralgiform headache attacks
    • >20 attacks fulfilling the following:
      • Moderate or severe unilateral headache, with orbital, supraorbital, temporal and/or trigeminal distribution, lasting for 1-60 seconds and occurring as single stabs, series of stabs or in a saw-tooth pattern
      • >1 of the following manifestations, ipsilateral to the headache: conjunctival injection and/or lacrimation, nasal congestion and/or rhinorrhea, eyelid edema, forehead and facial sweating, forehead and facial flushing, sensation of fullness in the ear, miosis and/ or ptosis
      • Attacks occur for >1 day for more than half of the time when the headache is active
    • Headache is not accounted for by another disorder
  • Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing fulfills criteria for short-lasting unilateral neuralgiform headache attacks but with both of conjunctival injection and lacrimation
  • Episodic short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing fulfills criteria for short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing and occurring in bouts
    • >2 bouts lasting from 7 days to 1 year and separated by pain-free remission periods of >1 month
  • Chronic short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing fulfills criteria for short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing and occurring in bouts but occurs without remission period or with remissions lasting <1 month, for >1 year
  • Episodic short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms fulfill the criteria for short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms and occurring in bouts; and with >2 bouts lasting from 7 days to 1 year and separated by pain-free remission periods of >1 month
  • Chronic short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms fulfill the criteria for short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms but occurs without remission period or with remissions lasting <1 month, for >1 year
  • Diagnostic criteria for hemicrania continua
    • Unilateral headache fulfilling the following:
      • Present for >3 month, with exacerbations of moderate or greater intensity
      • Either or both of the following: >1 of the following manifestations, ipsilateral to the headache (conjunctival injection and or lacrimation, nasal congestion and/or rhinorrhea, eyelid edema, forehead and facial sweating, forehead and facial flushing, sensation of fullness in the ear, miosis and/or ptosis and/or sense of restlessness or agitation
      • Attacks occur for at least 1 day for more than half of the time when the headache is active
    • Remitting subtype fulfills above criteria, and headache is not daily or continuous, but interrupted by remission periods of >1 day without treatment
    • Unremitting subtype fulfills above criteria, and headache is daily or continuous for >1 year, without remission periods of >1 day
  • Diagnostic criteria for probable trigeminal autonomic cephalalgia
    • Headache attacks fulfilling all except 1 criteria for cluster headache, paroxysmal hemicrania, short-lasting unilateral neuralgiform headache attacks and hemicrania continua
    • Not fulfilling criteria for any other headache disorder and not better accounted for by another diagnosis

Other Primary Headaches

  • Includes primary cough headache, primary exercise headache, primary headache associated with sexual activity, primary thunderclap headache, cold-stimulus headache, external-pressure headache, primary stabbing headache, nummular headache, hypnic headache, or new daily persistent headache
  • All criteria includes not being accounted for by another headache disorder
  • Primary cough headache is brought on by andccurring only in association with coughing, straining and/or other Valsalva maneuvers, with sudden onset, and/or lasting between 1 second to 2 hours
  • Primary exercise headache is characterized by headache brought on by and occurring only during or after strenuous physical exercise and lasting <48 hours
  • Primary headache associated with sexual activity fulfills >2 episodes of pain in the head and/or neck brought on by and occurring only during sexual activity, lasting from 1 minute to 24 hours with severe intensity and/or up to 72 hours with mild intensity, with pain increasing in intensity with increasing sexual excitement and/or abrupt explosive intensity just before or with orgasm
  • Primary thunderclap headache is characterized by severe headache with abrupt onset, reaching maximum intensity in <1 minute, lasting for >5 minutes
  • Headache attributed to external application of a cold stimulus fulfills >2 acute headache episodes brought on by and occurring only during application of an external cold stimulus to the head, and resolving within 30 minutes after removal of the cold stimulus
  • Headache attributed to ingestion or inhalation of a cold stimulus fulfills >2 episodes of acute frontal or temporal headache brought on by and occurring immediately after a cold stimulus to the palate and/or posterior pharyngeal wall from ingestion of cold food or drink or inhalation of cold air, resolving within 10 minutes after removal of the cold stimulus
  • Headache attributed to external-compression headache fulfills >2 headache episodes brought on by and occurring within 1 hour during sustained external compression of the forehead or scalp, maximal at the site of external compression, and/or resolving within 1 hour after external compression is removed
  • Headache attributed to external-traction headache fulfills >2 headache episodes brought on by and occurring only during sustained external traction on the scalp, maximal at the traction site, and resolves within 1 hour after traction is removed
  • Primary stabbing headache is characterized by pain occurring spontaneously as a single stab or series, with each stab lasting for up to a few seconds, recurring with irregular frequency, from one-many/day, and without cranial autonomic symptoms
  • Nummular headache is described as a continuous or intermittent headache felt exclusively in an area of the scalp, which is sharply contoured, fixed in size and shape, round or elliptical, and 1-6 cm in diameter
  • Hypnic headaches are recurrent headache attacks which develops only during sleep causing awakening, occurs for >10 days/month for >3 months, lasting for >15 minutes and for up to 4 hours after waking, and without cranial autonomic symptoms or restlessness
  • New daily persistent headache is characterized by a distinct and clearly remembered onset, with pain becoming continuous and unremitting within 24 hours, and present for 3 months

Secondary Headaches

  • Have another identifiable cause
  • When a new headache occurs for the first time in close temporal relation to another disorder that is known to cause headaches, or fulfills other criteria for causation by that disorder
    • This remains true even when the headache has the characteristics of a primary headache
  • When a pre-existing primary headache becomes chronic in close temporal relation to such a causative disorder, both the primary and the secondary diagnoses should be given
  • When a pre-existing primary headache is made significantly worse in close temporal relation to such a causative disorder, both the primary and the secondary headache diagnoses should be given, provided that there is good evidence that the disorder can cause headaches
  • Headache attributed to trauma or injury to the head and/or neck
    • Includes acute headache attributed to traumatic injury to the head, persistent headache attributed to traumatic injury to the head, acute headache attributed to whiplash, persistent headache attributed to whiplash, acute headache attributed to craniotomy, or persistent headache attributed to craniotomy
  • Headache attributed to cranial or cervical vascular disorder,
    • Includes headache attributed to ischemic stroke or transient ischemic attack, non-traumatic intracranial hemorrhage, unruptured vascular malformation, arteries, cervical carotid or vertebral artery disorder, cerebral venous thrombosis, other acute intracranial endovascular procedure, genetic vasculopathy, or pituitary apoplexy
  • Headache attributed to non-vascular intracranial disorder
    • Includes headache attributed to increased cerebrospinal fluid pressure, low cerebrospinal fluid pressure, non-infectious inflammatory disease, intracranial neoplasia, intrathecal injection, epileptic seizure, Chiari malformation type 1, or to other non-vascular intracranial disorder
  • Headache attributed to a substance or its withdrawal
    • Includes headache attributed to use of or exposure to a substance, medication-overuse headache, or headache attributed to substance withdrawal
  • Headache attributed to infection
    • Includes headache attributed to intracranial or systemic infection
  • Headache attributed to disorder of homeostasis
    • Includes headache attributed to hypoxia and/or hypercapnia, dialysis headache, arterial hypertension, hypothyroidism, fasting, cardiac cephalalgia, or other disorder of homeostasis
  • Headache or facial pain attributed to disorder of the cranium, neck, eyes, ears, nose, sinuses, teeth, mouth, or other facial or cervical structure
    • Also includes headache attributed to temporomandibular disorder, or inflammation of the stylohyoid ligament
  • Headache attributed to psychiatric disorder (somatization disorder or psychotic disorder)
  • Painful cranial neuropathies and other facial pains
    • Includes trigeminal neuralgia, glossopharygeal neuralgia, nervus intermedius (facial nerve) neuralgia, occipital neuralgia, optic neuritis, ischemic ocular motor nerve palsy, Tolosa-Hunt syndrome, paratrigeminal oculosympathetic (Raeder’s syndrome), recurrent painful ophthalmoplegic neuropathy, burning mouth syndrome, persistent idiopathic facial pain, or central neuropathic pain
  • Other headache disorders
    • Includes headache not elsewhere classified or headache unspecified

General Diagnostic Criteria for Secondary Headaches

  • Any headache with evidence of causation demonstrated by at least 2 of the following:
    • Headache has developed in temporal relation to the onset of the presumed causative disorder
    • One or both of the following:
      • Headache has significantly worsened in parallel with worsening of the presumed causative disorder
      • Headache has significantly improved in parallel with improvement of the presumed causative disorder
    • Headache has characteristics typical for the causative disorder
    • Other evidence exists of causation
  • Not better accounted for by another diagnosis

Diagnosis

  • Most headaches are diagnosed based on the history and physical exam with no imaging or laboratory examination necessary
    • Other types of headaches such as secondary headaches may require further evaluation and referral to a specialist

History

  • Temporal profile
    • Time from onset to peak
    • Usual time of onset (eg season, month, menstrual cycle, week, hour of day)
    • Frequency and duration
    • Stable or changing over past 6 months and lifetime
    • Previous attacks
  • Character of headache
    • Pulsatile, throbbing, pressing, sharp
  • Location
    • Unilateral, bilateral, frontal, peri-orbital, occipital, associated with neck pain, or changing sites
  • Severity and progression of symptoms
  • Presence of autonomic features
    • Nasal stuffiness, rhinorrhea, tearing, conjunctival injection, eyelid ptosis, or edema
  • Associated symptoms
    • Nausea, vomiting, photophobia, phonophobia
  • Precipitating factors
    • Stress, posture, cough, exertion, straining, neck movement, jaw pain
  • Aggravating factors
  • Relieving factors
  • Other medical conditions
    • Insomnia, depression, anxiety, hypertension, asthma, history of heart disease, or stroke
  • Pharmacological and non-pharmacological treatments
    • Response to therapy, side effects
  • Presence or absence of aura
  • Functional disabilities at work, school, household chore, or other activities
  • Family history of similar headache

Physical Examination

  • Vital signs
  • Extracranial structure evaluation such as carotid arteries, sinuses, scalp arteries, cervical paraspinal muscles
  • Neck examination such as neck posture, range of motion, palpation for muscle tender points, pain in neck flexion or lateral rotation for meningeal irritation
  • Examination for temporomandibular disorders
    • Includes jaw movements and palpation of the muscles of mastication for tender points

Neurological Examination

  • Assessment of mental status
  • Presence of confusion or memory impairment
  • Ophthalmological examination (eg fundoscopy, pupillary symmetry and reactivity, visual fields, and ocular motility)
  • Cranial nerve examination (eg corneal reflexes, facial sensation, and facial symmetry)
  • Muscle tone symmetry and strength
  • Deep tendon reflexes
  • Sensation
  • Plantar responses
  • Gait, arm, and leg coordination

Laboratory Tests

Diagnostic Tests

  • Indicated in patients with symptoms suggestive of secondary headache
  • Indicated in patients who present with signs or symptoms of headache with increased risk of intracranial pathology
  • Not indicated in patients with recurrent headaches with clinical features of migraine, normal neurological examination, and no red flags for potential causes of secondary headache

Electroencephalogram

  • Not routinely done in evaluating headache but may be useful in patients who have headache with periodic altered consciousness and have suspected seizures

Blood Exams

  • Consider in patients with focal neurologic signs, personality/mental status changes, changes in sensorium, and systemic illness with headache

Imaging

Cranial Magnetic Resonance Imaging (MRI)

  • Neuroimaging procedure of choice
  • Used to exclude a space-occupying lesion or Chiari malformation
  • May be considered in immunocompromised patients, patients >60 years with suspected temporal arteritis, patients with suspected meningitis or carotid artery dissection, pregnant patients with severe headache, patients presenting with severe onset or the worst headache experienced

Cranial Magnetic Resonance Angiogram (MRA)

  • May be considered in patients with severe onset of very painful headache

Non-contrast Cranial Computed Tomography (CT) Scan

  • Usually adequate to rule out a space-occupying lesion
  • Performed before lumbar puncture in all patients with suspected subarachnoid hemorrhage, regardless of findings on neurological examination
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