Headache Attributed to Nonvascular, Noninfectious Intracranial Disorders Headache Attributed to Trauma or Injury to the Head and/or Neck Headache Attributed to Infection Headache Attributed to Cranial or Cervical Vascular Disorders Headache Associated with Disorders of Homeostasis Headache Caused by Disorders of the Cranium, Neck, Eyes, Ears, Nose, Sinuses, Teeth, Mouth, or Other Facial or Cranial Structures Headaches and the Cervical Spine Migraine Chronic Daily Headache Cluster Headache Other Trigeminal Autonomic Cephalalgias Other Primary Headaches

1. HEADACHE :
PRIMARY HEADACHE
DR VAROON
FIRST YEAR DNB RESIDENT
NEUROLOGY KMC

2. Classification of Headache Disorders
From Headache Classification Committee of the International
Headache Society, 2013.
International Classification of Headache Disorders, 3e

4. APPROACH : HISTORY
• Onset of Headaches
• Frequency and Periodicity Of Episodic Headaches
• Temporal Profile : Onset, Peak, End
• Time of Day
• Location
• Quality and Severity
• Precipitating Factors, Aggravating and Mitigating Factors.
• Premonitory Symptoms, Aura and Accompanying Symptoms

5. RED FLAGS FOR WORRISOME HEADACHES
• Head or neck injury
• New onset or new type or worsening pattern of existing headache
• New level of pain (e.g., worst ever)
• Abrupt or split-second onset
• Triggered by Valsalva maneuver or cough
• Triggered by exertion
• Triggered by sexual activity
• Headache during pregnancy or puerperium
• Age > 50 years
• Neurological signs or symptoms: Seizure, Confusion, Impaired alertness,
Weakness, Papilledema
• Systemic Illness : Fever, Nuchal Rigidity, Wt Loss, Scalp Artery tenderness
• Recent Travel

6. CHRONIC DAILY OR
NEAR-DAILY
HEADACHE : Headache
on 15 days or more per month.
CDH is not a single entity; it
encompasses a number of
different
headache syndromes, both
primary and secondary

7. Medication Overuse Headache
previously rebound or medication-induced headache
• Overuse of acute medications by patients with frequent headache
Opioids, butalbital-containing compounds, and some combination analgesics : highest risk
Triptans : moderate risk
NSAIDs : lowest risk.
• Facilitation of central trigeminal sensitization caused by a medication-induced impairment
of descending inhibition of nociceptive trafficking
• Outpatients : Reduce the medication dose by 10% every 1–2 weeks OR Immediate
cessation of analgesic use (if no contraindication)
• Use of a medication diary maintained during the month or two before cessation.
• NSAID such as naproxen, 500 mg bid, if tolerated, will help relieve residual pain as
analgesic use is reduced.

8. • Inpatients :
• For acute intolerable pain during the waking
hours, aspirin, 1 g IV OR IM
chlorpromazine can be helpful at night;
hydrated.
• Three to five days into the admission, as the
effect of the withdrawn substance wears off,
a course of IV dihydroergotamine (DHE) :
administered every 8 h for 5 consecutive
days, can induce a significant remission that
allows a preventive treatment to be
established.
• Serotonin 5-HT3 receptor antagonists, such
as ondansetron or granisetron, or the
neurokinin receptor antagonist, aprepitant,
may be required with DHE to prevent
significant nausea, and domperidone (not
approved in the United States) orally or by
suppository can be very helpful.
• Avoiding sedating or otherwise side effect–
prone antiemetics is helpful

9. • Migraine and its subtypes
• Tension-type headache and its
subtypes
• Trigeminal autonomic cephalalgias
• Cluster headache
• Paroxysmal hemicrania
• Short-lasting unilateral neuralgiform
headache attacks
• Hemicrania continua
• Other primary headache disorders
• 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
• New daily persistent headache
PRIMARY HEADACHE : CLASSIFICATION

10. Tension-Type or Muscle-Contraction Headache
• Bilateral tight, band-like discomfort
• Types :
• Infrequent episodic tension-type headache
• Frequent episodic tension-type headache
• Chronic tension-type headache
• Probable tension-type headache
• Acetaminophen, Aspirin, or NSAIDs & Behavioral approaches
• Chronic TTH : Amitrptyline

11. TRIGEMINAL AUTONOMIC CEPHALALGIAS
• Cluster headache
• Paroxysmal hemicrania
• Short-lasting unilateral neuralgiform headache attacks
• Hemicrania continua

12. Cluster headache
• Episodic : attacks of pain occur in periods lasting 7 days to 1 year,
separated by pain-free periods lasting 1 month or longer.
• Chronic : attacks of pain occur for more than 1 year without remission or
with remissions lasting less than 1 month
• Begins : 3rd decade. Periodicity is a cardinal feature ; M > F
• Cluster Period : 6 to 12 weeks f/b remission lasting for months/yrs.
Consistent for given Pt ; 1-2/year ; Nocturnal
• Unilateral Same type
• Retro-orbital and temporal regions (upper syndrome) but may be maximal
in the cheek or jaw (lower syndrome).
• Steady or boring and of terrible intensity (so-called “suicide headache”).

13. • Feeling the eye being pushed out or an auger or hot poker going
through the eye.
• Abrupt Onset ; Peak : 5 to 10 minutes ; Persist: 45 minutes to 2 hours
• Tend to move about during attacks, pacing, rocking, or rubbing their
head for relief; occ. aggressive during attacks
• Ipsilateral symptoms of cranial parasympathetic autonomic activation:
conjunctival injection or lacrimation, aural fullness, rhinorrhea or
nasal congestion, or cranial sympathetic dysfunction such as ptosis
• Migrainous Features
• Alcohol Triggers

14. Treatment
• ACUTE : 100% oxygen at 10–12 L/min for 15–20 min ; Sumatriptan 6
mg SC ; nVNS three 2-min stimulation cycles
• PREVENTIVE : Transitional & Maintenance Prophylaxis

16. PAROXYSMAL HEMICRANIA
• 3rd Decade . M=F
• Chronic and Episodic
• Unilateral; very severe pain : sharp, stabbing, boring, or throbbing
• Short-lasting attacks (2–45 min); very frequent attacks (usually >5 a
day); marked autonomic features ipsilateral to the pain; rapid course
(<72 h)
• Excellent response to indomethacin(25-300mg/d).
• Therapeutic response to indomethacin is the most reliable differential
diagnostic criterion for cluster headache and PH..

17. Short-Lasting Unilateral Neuralgiform Headache Attacks
• Severe, unilateral orbital or temporal
pain that is stabbing or throbbing in
quality
• Diagnosis requires at least 20 attacks,
lasting for 5–240 s; ipsilateral
conjunctival injection and lacrimation.
• Single stabs last on average 58
seconds
• Groups of stabs usually last 396
seconds
• Sawtooth attack (many stabs
between which the pain does not
totally resolve) typically lasts 1160
seconds
SUNCT (short-lasting unilateral neuralgiform headache with
conjunctival injection and tearing)
SUNA (Short lasting unilateral neuralgiform headache attacks
with cranial autonomic symptoms)

18. TREATMENT
ABORTIVE THERAPY
• Therapy of acute attacks is not a useful concept in SUNCT/SUNA because the
attacks are of such short duration.
• However, IV lidocaine, which arrests the symptoms, can be used in hospitalized
patients : 1 to 3.5 mg/kg/h for up to 1 week
PREVENTIVE THERAPY
• Lamotrigine, 200–400 mg/d.
• Topiramate and gabapentin may also be effective. Carbamazepine, 400–500
mg/d, has been reported by patients to offer modest benefit.
• Indomethacin Not effective
• Occipital nerve block, opioid blockade of the superior cervical ganglion,
hypothalamic stimulation, and surgical removal of a pituitary microadenoma.

19. Hemicrania Continua
• F:M= 2 : 1,
• Average age of onset is 28 years (range, 5–67 years).
• Moderate and continuous unilateral pain associated with fluctuations of severe
pain
• 2 versions of hemicrania continua are unremitting and remitting (periods of pain-
freedom lasting more than 24 hours).
• Complete resolution of pain with indomethacin
• Exacerbations that may be associated with autonomic features, including
conjunctival injection, lacrimation, and photophobia on the affected side.

21. Other Primary Headches

22. Primary Cough Headache
• Preventable by avoiding coughing or other precipitating events, which can include sneezing,
straining, laughing, or stooping
• Indomethacin 25–50 mg two to three times daily is the treatment of choice.
• Some patients with cough headache obtain complete cessation of their attacks with lumbar
puncture

23. • Young ; Bilateral and often throbbing at onset
• Migrainous features may develop in patients susceptible to migraine.
• Acute venous distension : Cerebral Vasodilation
• Warm Up, Hydration
• Indomethacin at daily doses from 25 to 150 mg is generally effective in benign exertional
headache.
• Indomethacin (50 mg), ergotamine (1 mg orally), and dihydroergotamine (2 mg by nasal
spray) are useful prophylactic measures
• Exercise-induced cardiac ischemic pain can refer to the head and neck, and is referred to
as cardiac cephalalgia.
Primary Exercise Headache

24. • M > F
• A postural headache developing after coitus arising from vigorous sexual activity : low CSF pressure headache
Not a primary headache disorder
• Education and reassurance
• Indomethacin (25–50 mg) given 30 to 60 minutes prior to sexual activity may prevent the headache.
• For those intolerant of, or unresponsive to indomethacin, an oral triptan can be tried 30 to 45 minutes before
sexual activity.
• For patients with frequent attacks, daily propranolol, metoprolol, or diltiazem may be effective.
sexual headache,
benign vascular sexual
headache, coital cephalalgia,
coital headache, intercourse
headache, (pre)orgasmic
cephalalgia, and
(pre)orgasmic headache

26. Primary Thunderclap Headache
• Sudden onset of severe headache may occur in the absence of any known provocation
• 20 and 50 years of age, with a female predominance
• Medical emergency that requires urgent evaluation
• D/D : subarachnoid hemorrhage, reversible cerebral vasoconstriction syndromes, cervical artery
dissection, cerebral venous sinus stenosis, and hypertensive crisis
• No established treatment.
• A short course of steroids can be given.
• Nimodipine has been demonstrated to prevent further attacks of thunderclap headaches in most
patients and should be recommended for 2 to 3 months.
• It is also important to avoid vasoconstrictors, such as triptans, ergot derivatives, cocaine, or similar
drugs

27. Cold-Stimulus Headache :
ice-cream headache or brain-freeze headache
• Triggered by passing solid, liquid, or gaseous cold materials
over the palate and posterior pharynx
• It is bought on quickly and typically resolves within 10–30 min
of the stimulus being removed.
• The transient receptor potential cation subfamily M member 8
(TRPM8) channel, a known cold temperature sensor, may be a
mediator of this syndrome
• Short-lived, cold-stimulus headache does not require a specific
treatment aside from trigger avoidance

29. Primary Stabbing Headache
“ice-pick pains” or “jabs and jolts.” or ophthalmodynia periodica
• Indomethacin (25–50 mg two to three times daily) is usually
excellent.

30. Nummular Headache
• Local sensory disturbance, such as allodynia or hypesthesia.
• Difficult to treat when present in isolation
• Gabapentin (300–1800 mg daily), tricyclic antidepressants, and onabotulinumtoxinA may
be useful

31. Hypnic Headache (“alarm clock headache”
• Bilateral : F>M
• Daytime naps can also precipitate head pain; and the onset is usually after age 60 years.
• Bedtime dose of lithium carbonate (200–600 mg).
• For those intolerant of lithium, verapamil (160 mg) is an alternative strategy. One to two cups
of coffee or caffeine, 60 mg orally, at bedtime may be effective in approximately one-third of
patients.
• Case reports also suggest that flunarizine, 5 mg nightly, or indomethacin, 25–75 mg nightly,
can be effective.

32. New Daily Persistent Headache
• The vast majority of patients can pinpoint the exact date their headache started.
• M=F : Adolescents
• Migrainous type and Featureless, appearing as new-onset TTH.
• Those with migrainous features are the most common form, and include unilateral
headache and throbbing pain; each feature is present in about one-third of patients.
Nausea, photophobia, and/or phonophobia occur in about half of patients.

33. Treatment
• Migrainous-type primary NDPH
consists of using the
preventive therapies effective
in migraine
• Featureless NDPH is one of
the primary headache forms
most refractory to treatment.
Standard preventive therapies
can be offered but are often
ineffective.