Understanding Different Types of Primary Headaches

B Y
K H A L E D O S A M A M O H A M E D
U N D E R S U P E R V I S I O N
PROF. NAGEH FOULLY EL GAMMAL
Headache other than
Migraine

Introduction
 Definition
 Pain sensitive structures,
 Referred pain
 History and examination
 Diagnosis steps

Definition of headache
 The term headache encompass aches
and pains located in the head,
 In practice its applications is restricted to
discomfort in the region of the cranial
vault.

Pain sensitive cranial structures
1. Scalp skin, SC tissue, muscle, extra cranial
arteries & periosteum of the skull (Inflammation,
spasm or trauma)
2. Parts of the dura of base of brain & falx cerebri
(Irritation, traction or displacement).
3. Intra-cranial venous sinuses & their large
tributaries (Traction or displacement).

4. Proximal parts of ACA & MCA, the intra-cranial
segments of ICA, middle meningeal & superficial
temporal arteries (Dilatation or traction).
5. V, IX, X cranial nerves & 1st 3 cervical nerves
(Compression, traction or inflammation)
6. Eye, ear, nasal cavities & sinuses.
Pain sensitive cranial structures (cont.)

Pain insensitive cranial structures
1. Bony skull,
2. Dura over the convexity of the brain,
3. Much of the pia-arachniod,
4. Parenchyma of the brain,
5. Ependyma
6. Choroids plexus

Referred Pain
 Naso-orbital region (sphenopalatine branch of VII)
 pain is referred to face.
 Anterior & middle fossae (V1 & V2) referred to
anterior two third of the head.
 Sphenoid & sella  vertex
 Posterior fossa & infra-tentorium (III, IX, X & C
1,2,3 ) back of the head & neck.

Headache History
 Age / Age at onset
 Prodroma / Aura
 Location / Radiation
 Character
 Building up
 Duration
 Severity
 Frequency / Periodicity
 Associated symptoms
 Postdrome
 Timing/Activity at onset
 Triggers
 Aggravating factors
 Reliving factors
 Recent change
 Other headaches
 Co-morbid conditions
 Medications used
 Family history

Headache Examination
 Vital signs: hypertension, bradycardia
 General appearance: restlessness, pallor
 Head and Neck:
 Scalp: tenderness, swelling, palpation of temporal
artery,
 TMJ: tenderness, crepitus
 Face: tenderness over cheeks/forehead
 Eyes: lacrimation, flushing, conjunctival injection,
 Nose: prurulent rhinorrhea
 Mouth: swelling; tenderness of teeth
 Neck: stiffness, tenderness, bruits

Headache Examination (cont.)
 Neurological exam
 Cranial nerves including fundus and visual field
 Symmetry on motor, sensory, reflexes and
cerebellar (coordination) tests,
 Getting up from seated position,
 Gait: walking on tiptoes and heels, tandem gait
and Romberg
 In kids
 Growth Parameters;

Diagnostic Steps
1. Exclude secondary headache
2. Which type of primary headache ?
3. Is there is another type of headache ?
4. What about co-morbidity ?

I- Exclude Secondary Headache?
Primary
Secondary
Joubert J. Diagnosing headache. Aust Fam Physician 2005; 34(8): 621-5

Red Flags Headaches
 Onset
 Course
 Timing & triggers
 Association
 Secondary headache risk factors

Red Flags Headaches
(Onset)
 New onset: > 50 years (mass lesion, temporal
arthritis) or < 5 years (mass)
 Sudden onset: SAH, bleed into a mass or AVM,
pituitary apoplexy.

Red Flags Headaches
(Course)
 Persistently progressive: mass lesion, subdural
hematoma.
 Change in the frequency, severity, or clinical
features of headache.

Red Flags Headaches
(Timing & Triggers)
 Awake the patient: Cerebrovascular disease,
mass, infection
 Specific trigger: cough or straining (mass or
SAH), changes in position (spontaneous CSF
leak).

Red Flags Headaches
(Association)
 Neurological signs: Focal neurologic signs other
than typical aura, ataxia, impaired consciousness,
personality or behavioral changes, neck stiffness,
diminution of vision, papilledema, pulsatile tinnitus,
cranial bruit.
 Systemic symptoms: fever, persistent vomiting
cutaneous rash, growth abnormalities.

 Pregnancy & postpartum (VST, carotid
dissection, pituitary apoplexy),
 Head trauma (Subdural or epidural hematoma,
intracranial hge, posttraumatic headache),
 Systemic cancer (metastases),
 HIV (opportunistic infection)
Red Flags Headaches
(Secondary headache risk factors)

Blue flag headaches
 Other neuropsychiatric disorders
 Other Head and neck structures
 Systemic disorder
 Iatrogenic

Blue flag headaches
(Related to other Neuropsychiatric disorders)
 Epileptic seizure (Coexistance, preictal, ictal or
postictal headache, hemicrania epileptica,
migralepsy, 1ry & 2nd epilepsy-migraine
syndromes)
 Cranial neuralgias (e.g. Trigeminal neuralgia)
 Psychiatric disorder (Somatization or Psychotic
disorder)

Blue flag headaches
(Related to Extracranial structures)
 Eyes, ears, nose and nasal sinuses, teeth,
jaws or related structures
 Cervical spine (e.g. cervical spondylosis)

Blue flag headaches
(Related to Systemic disorder)
 Disorder of homoeostasis (Hypertension,
Anemia, Fasting, Hypothyroidism, Hypoxia
and/or hyper-capnia, Dialysis)
 Systemic infection (e.g. influenza)
 Cold-stimulus (External application, Ingestion,
Inhalation)

Blue flag headaches
(Iatrogenic)
 Medication (e.g. vasodilators) or substance
ingestion or withdrawal
 Spinal puncture or intra-thecal injection
 Surgery in head and/or neck

Diagnostic Steps
1. Exclude secondary headache ?
2. Which type of primary headache ?
3. Is there is another type of headache ?
4. What about co-morbidity ?

II- Which type of Primary headache ?
 Key questions for the answer:
 What is the site, character & severity of pain ?
 How long it takes to build up & to ends ?
 Is there is associated autonomic manifestations;
horner’s syndrome, lacrimation, running nose,
ipsilaterlal flusching ?
 Is it has specific trigger; exercise, cough or
straining, sex, sleep ?
 Is episodic or chronic ?

Primary Headaches
Without Autonomic With Autonomic
Duration
< 4 hours
Duration
> 4 hours
Duration
< 4 hours
Duration
> 4 hours
Episodic
(<15/m)
-Migraine in chid
-1ry Stabbing H.
-1ry Thunder-
clap H.
-Cough H.
-Exertional H.
-Preorgasmic H.
-Orgasmic H
-Migraine
-Tension H.
-Cluster H.
-Parox.Hemic
Chronic
(≥15/m
for 3m)
-Hypnic H. -Ch. Migraine
-Ch. Tension H.
-NDPH
-Ch. Cluster H.
-Ch. Parox.
Hemic.
-SUNCT
-Hemicrania.
Continua

Location of common primary headaches

Tension-Type Headache (TTH)
(A) At least 10 episodes fulfilling the criteria B-D:
(B) Lasting 30 min - 7 days
(C) Has at least 2 of the following:
- Bilateral location
- Pressing/tightening (non-pulsating) quality
- Mild or moderate intensity
- Not aggravated by routine physical activity
(D) Both of the following:
- No nausea or vomiting (anorexia may occur)
- No > 1 episode of photophobia or phonophobia
(E) Not attributable to another disorder

Notes about tension headache
 Most common type of headache
 Higher prevalence in middle aged women
 Typically described as Band-like pressure,
although it may be numbness, tingling, burning,
boiling, dull aching, heaviness or fullness
 Build up over hours
 Worse towards end of day

Categories of Tension Headache
 Infrequent Episodic (IFETH): <1day a month
 Frequent Episodic (FETH): 1-15 days a month.
 Chronic (CTH): ≥15 days a month.
 Chronic daily headache: at least 6 days / week

Management of Tension Headache
Infrequent episodic TTH
 Reassurance
 Simple analgesic: Aspirin, paracetamol or ibuprofen
Chronic TTH
 Amitriptyline: is the prophylactic of choice
 Simple analgesics: may give short-term relief but is
inappropriate long-term

Management of Tension Headache
Non pharmacological Pharmacological
Prevention -Information, reassurance &
avoidance of trigger factors
-Psychological Treatments
(Relaxation Training,
Biofeedback, CBT)
-Physical Therapy
(Regular exercise)
-Acupuncture & Nerve block
-Antidepressants
-Topiramate ?
Treatment -Massage or hot packs on
the muscles of head & neck
-Simple Analgesics
-Muscle relaxants

Cluster headache
(migrainous neuralgia)
(A) At least 5 attacks fulfilling criteria B–D
(B) Severe or very severe unilateral orbital, supra-orbital and/or
temporal pain lasting 15-180 min.
(C) Accompanied by at least 1 of:
1. Ipsilat. conjunctival injection and/or lacrimation
2. Ipsilat. nasal congestion and/or rhinorrhoea
3. Ipsilat. eyelid oedema
4. Ipsilat. miosis and/or ptosis
5. Ipsilat. forehead & facial sweating
6. Sense of restlessness or agitation
(D) Frequency:1/2d - 8/d
(E) Not attributed to another disorder

Notes about Cluster Headache
 Male, 20-40 year, smokers
 Restless “banging head against wall”.
 The pain is explosive, deep boring, piercing, penetrating,
non throbbing.
 Often awaken patients after 1-2 hrs of sleep and it may
occur at the same time each day
 Maximal immediately if the patient awakens with the
headache in progress or it peaks within minutes if it begins
while awake

Notes about Cluster Headache
 Periodicity: daily in bouts for 6-12 weeks every 1 or 2 yrs
with headache free interval in between
 Side shifts: Some patients switch sides with different
cluster periods, and smaller number has side shifts within a
cluster period.
 Cluster headache face: leonine face, furrowed and
thickened skin with prominent folds, a broad chin, vertical
forehead creases, and nasal telangiectasias.
Typically tall and rugged-looking

Types of Cluster Headache
 Episodic cluster headache
 At least two cluster periods lasting 7-365 days and
 separated by pain-free remission periods of ≥1 month
 Frequency: every day
 Chronic cluster headache
 Attacks recur over >1 year without remission periods or
 with remission periods lasting <1 month
 Frequency: one every second day to eight day.
 Notes: Patients may switch from Chronic cluster to
Episodic cluster headache, and vice versa.

Abortive Treatment of Cluster headache
Non pharmacological Pharmacological
- Local application of
pressure over the
affected eye or
ipsilateral temporal artery
- local application of hot
or cold
- Intense physical activity,
rarely effective
-Oxygen: 100% at 10-12
L/min for 15 min
-Sumatriptan: 6 mg SC or IN
oral has no role
-Dihydroergotamine: IM
IN is less effective
-Lidocaine:
Nasal drops ?

Prophylactic Treatment of Cluster headache
Short Term Long Term
-Corticosteroids:
Prednisolone 60 mg daily,
taper over 18-24 weeks,
Methylprednisolone IV
-Ergotamine
-Greater occipital n block
-Surgical:
DBS & occipital n. stim.
-Verapamil:80-160mg/d
-Lithium:300 mg tid
-Topiramate
-Gabapentin
-Melatonin
-Methysergide
-Combination

Migraine Tension headache Cluster Headache
Age of onset Childhood to 20s Teens to 30s 20s
Gender Female 3 X male Female ≥ male Male 6 X female
Quality Pulsating Pressure, band-like Steady boring, piercing
Location Unilateral (60%)
Temporal
Generalized, occipital or
frontal
Unilateral (100%),Orbital,
supraorbital, temporal
Intensity Moderate to severe Mild to moderate Very severe
Building-Up Begin gradually Over hours Within mins (explosive)
Duration 4-72 hours
(Resolve slowly)
30 min – 7 days
(Wax-wane all day)
15–180 min
(Abruptly end)
Frequency 2-4/month Daily or near daily 1–8/day
Periodicity No No Daily for 6-12 weeks
every 1 or 2 yrs
Timing No diurnal pattern Worse late in the day Awaken after 1-2 hrs
Physical
activity
Aggravate headache
(Lie down)
Not worsen headache
(Keep going)
Restlessness
(Bang head on wall)
Associated
symptoms
Nausea, vomiting,
Photo., Phonophobia,
Pallor
Scalp tenderness,
anorexia,
Partial Horner
Lacrimation, Rhinorrhea
Unilateral facial Flushing
Triggers Stress, smoking,
insomnia, menses
Stress Smoking, nitrates,
alcohol

Trigeminal Autonomic Cephalalgias
(TAC)
 The TAC share the clinical features of headache
and prominent cranial parasympathetic autonomic
features.
 Human functional imaging suggests that these
syndromes activate a normal human trigeminal-
parasympathetic reflex with secondary cranial
sympathetic dysfunction.
 TAC include:
 Cluster headache
 Paroxismal Hemicrania
 SUNCT

Cluster Paroxysmal
hemicrania
SUNCT
Gender M:F Male (6:1) Female (1:2) Male (3:1)
Site Orbit, temple
Frequency 1-8 /d ( 2/d) 1-40 /d ( 8/d) 3-200 /d ( 30/d)
Duration 15-180 min ( 1h) 2-30 min ( 15 min) 5-240 sec ( 1min)
Severity Very severe Severe Moderate
Quality Boring, piercing Pulsating or boring Stabbing or pulsating
Autonomic Yes
Periodicity Yes Yes
Migranous Yes Yes rare
Alocohol trigger Yes Occasion No
Cutaneous trigger No No Yes
Abortive treatment O2, Sumatriptan Nil Nil
Prophylactic
treatment
Verapramil,
lithium
Indomethacin
(150 mg/d orally or
100 mg IM)
Lamotrigine,
Topiramate,
Gabapentin,
Surgical

Paroxismal Hemicrania
 As cluster headache but:
 Short lasting: 2-30 min
 More frequent: several times a day
 More in females
 Respond absolutely to Indomethacin (150 mg
daily orally and rectally or 100 mg by injection)
but for maintenance smaller doses are often
sufficient.

Paroxismal Hemicrania
A. At least 20 attacks fulfilling criteria B-D
B. Attacks of severe unilateral orbital, supraorbital or temporal pain lasting
2-30 min
C. Headache is accompanied by ≥1 of the following:
1. ipsilateral conjunctival injection and/or lacrimation
2. ipsilateral nasal congestion and/or rhinorrhoea
3. ipsilateral eyelid oedema
4. ipsilateral forehead and facial sweating
5. ipsilateral miosis and/or ptosis
D. Attacks have a frequency >5/d for > half of the time, although periods
with lower frequency may occur
E. Attacks are prevented completely by therapeutic doses of
indomethacin
F. Not attributed to another disorder
The International Classification of Headache Disorders 2nd Edition (2004); Cephalgia, Volume 24 Supplement 1

Types of Paroxismal Hemicrania
 Episodic paroxysmal hemicrania
 At least 2 attack periods lasting 7-365 d and
 separated by pain-free remission periods of ≥1 mo
 Chronic paroxysmal hemicrania
 Attacks recur over >1 y without remission periods or
 with remission periods lasting <1 mo

This syndrome is characterised by:
 Short-lasting attacks of unilateral moderately
severe sharp or stabbing pain in the 1st
division of trigeminal nerve that are
 Much briefer ( 1min) and
 More frequent ( 30/d) than other TAC
 Very often accompanied by prominent
lacrimation and redness of the ipsilateral
eye.
Short lasting Unilateral Neuraligiform headache
with Conjunctival injection and Tearing (SUNCT)

Short lasting Unilateral Neuraligiform headache
with Conjunctival injection and Tearing (SUNCT)
A. At least 20 attacks fulfilling criteria B-D
B. Attacks of unilateral orbital, supraorbital or temporal
stabbing or pulsating pain lasting 5-240 s
C. Pain is accompanied by ipsilateral conjunctival injection
and lacrimation
D. Attacks occur with frequency 3-200/d
E. Not attributed to another disorder

Notes about SUNCT
 Mimics :
 Lesions in posterior fossa or involving pituitary
gland
 Treatment:
 Lamotrigine (of choice), Gabapentin, Topiramate,
Carbamazepine, IV lidocaine
 Surgical: trigeminal ganglion compression,
retrogasserian glycerol rhizolysis

 Transient and localised stabs of pain in the head that
occur spontaneously in the absence of organic
disease of underlying structures or of the cranial
nerves.
 Stabs may move from one area to another in either the
same or the opposite hemicranium. When they are
strictly localised to one area, structural changes at this
site and in the distribution of the affected cranial nerve
must be excluded.
Primary Stabbing Headache
Ice-pick pains, jabs and jolts, ophthalmodynia periodica

(A) Head pain occurring as a single stab or a series
of stabs and fulfilling criteria B–D
(B) Exclusively or predominantly felt in the
distribution of the first division of the trigeminal
nerve (orbit, temple and parietal area)
(C) Stabs last up to a few sec and recur with
irregular frequency ranging from 1 to many/day
(D) No accompanying symptoms

 Stabbing pains are more commonly experienced
by people subject to migraine (about 40%) or
cluster headache (about 30%), in which cases they
are felt in the site habitually affected by these
headaches.
 A positive response to indomethacin has been
reported in some uncontrolled studies, whilst
others have observed partial or no responses.

 Headache precipitated by coughing or straining
in the absence of any intracranial disorder.
 Usually bilateral
 Predominantly affects patients >40 years
Primary cough headache
Benign cough headache, Valsalva-manoeuvre headache

Primary cough headache
Benign cough headache, Valsalva-manoeuvre headache
(A) Headache fulfilling criteria B and C
(B) Sudden onset, lasting from 1 sec to 30 min
(C) Brought on by and occurring only in association
with coughing, straining and/or Valsalva
manoeuvre
(D) Not attributed to another disorder

Notes about Primary cough headache
 40% of cough headache are secondary:
 Carotid or vertebrobasilar diseases
 Cerebral aneurysms
 Arnold-Chiari malformation type I
 Whilst indomethacin is usually effective in the treatment of
primary cough headache, a positive response to this medication
has also been reported in some symptomatic cases.
 Diagnostic neuroimaging plays an important role in differentiating
secondary cough headache from Primary cough headache.

Primary Exertional Headache
“weight-lifters’ headache
 Headache precipitated by any form of exercise.
 Particularly in hot weather or at high altitude
 On first occurrence of this headache type it is
mandatory to exclude subarachnoid haemorrhage
and arterial dissection.
 There are reports of prevention in some patients by
the ingestion of ergotamine tartrate.
 Indomethacin has been found effective in the
majority of the cases.

Primary Exertional Headache
“weight-lifters’ headache
(A) Pulsating headache fulfilling criteria B and C
(B) Lasting from 5 min to 48 hr
(C) Brought on by and occurring only during or after
physical exertion

 Headache precipitated by sexual activity,
usually starting as a dull bilateral ache as
sexual excitement increases and suddenly
becoming intense at orgasm, in the absence
of any intracranial disorder.
Primary headache associated with sexual activity
Coital cephalalgia, benign vascular sexual headache

Primary headache associated with sexual activity
Coital cephalalgia, benign vascular sexual headache
Preorgasmic headache
(A) Dull ache in the head and neck associated with
awareness of neck and/or jaw muscle contraction
and fulfilling criterion B
(B) Occurs during sexual activity and increases with
sexual excitement
(C) Not attributed to another disorder
Orgasmic headache
(A) Sudden severe (“explosive”) headache fulfilling
criterion B
(B) Occurs at orgasm
(C) Not attributed to another disorder

Notes about headache associated with
sexual activity
 Last from 1 min to 3 hrs
 50% associated with migraine
 Exclude : SAH and arterial dissection
 Treatment
 Reassurance; sympathy and understanding by partner
 Prevention : Propranolol, Diltiazem
 Ergotamine, indomethacin or methysergide before sexual
activity
 Ceasing sexual activity if milder warning develops

Hypnic Headache
Alarm clock” headache
 Attacks of dull headache that always awaken the
(A) Dull headache fulfilling criteria B-D
(B) Develops only during sleep, and awakens patient
(C) At least two of the following characteristics:
- Occurs >15 times/mo
- Lasts ≥15 min after waking
- First occurs after age of 50 y
(D) No autonomic symptoms and no >1 of: nausea,
photophobia or phonophobia

Notes about Hypnic Headache
 Bilateral
 Mild to moderate,
 Same time each night
 Last 15 - 180 minutes
 Exclude : intracranial causes
 Treatment: Using on of the following at bed time
 Caffeine 100mg
 Lithium 300-600mg
 Verapamil
 Methysegide

DD of headache the awake patients
Primary Secondary
Cluster headache: unilateral, poring,
max. immediately on awakening, with
autonomic, 15-180 min, 20-40 y
Hypnic headache: diffuse, regularly
awaken the patient at a particular time
of night, 15-180 min, after 50 y
Increased intracranial tension: recent
onset, allover the day and disrupt sleep
or worse on awaking.
Obstructive sleep apnea: Pancranial
headache on awaking and gradually
recedes over the course of the day,
patient is elderly, male, obese, smokes,
or has a history of COPD.
Medication over use: chronic daily
headache in the setting of regular
analgesic use, Dull, generalised, early
morning worsening

Hemicrania continua
 Persistent strictly unilateral headache responsive to indomethacin.
(A) Headache for >3 mo fulfilling criteria B-D
(B) All of the following:
- Unilateral pain without side-shift
- Daily & continuous, without pain-free periods
- Moderate intensity, with severe exacerbations
(C) At least 1 of the following autonomic features occurs during
exacerbations, ipsilateral to the pain:
- Conjunctival injection and/or lacrimation
- Nasal congestion and/or rhinorrhoea
- Ptosis and/or miosis
(D) Complete response to indomethacin

Notes about Hemicrania continua
Treatment
 Indomethacin 25-300 mg daily
 NSAIDs : Ibuprofen, piroxicam
 Paracetamol with codeine
 Corticosteroids

New daily-persistent headache
De novo chronic headache; chronic headache with acute onset
(A) Headache for >3 mo fulfilling criteria B-D
(B) Daily & unremitting from onset or from <3 d from onset
(C) At least 2 of the following:
- Bilateral location
- Pressing/tightening (non-pulsating) quality
- Mild or moderate intensity
- Not aggravated by routine physical activity
(D) Both of the following:
- Not >1 of photophobia, phonophobia or mild nausea
- Neither moderate or severe nausea nor vomiting

Notes about NDPH
 Onset clearly recalled in individuals without a prior
headache history
 It can have associated features suggestive of either
migraine or tension-type
 Two subforms :
 Self-limiting: typically resolves without therapy within
several months
 Refractory: resistant to aggressive treatment
programmes
 Exclude “red flag”
 Think about overuse headache

DD of Chronic Daily Headache
Primary Secondary
-Duration > 4 hours:
- Without Autonomic
Chronic Migraine
Transformed Migraine,
Chronic Tension H.
NDPH
- With Autonomic
Hemicrania Continua
-Duration < 4 hours
- Without Autonomic
Hypnic H.
- Without Autonomic
Chronic Cluster h.
Chronic Paroxysmal Hemicrania
SUNCT
- Vascular Disorders: AVM; arteritis,
dissection, subdural hematoma
- Non-Vascular disorders: tumor,
chronic CNS infections (EBV, HIV),
Intracranial Hypotension or HTN
-Cervical Spine Disorders
-TMJ, Sinus infections,
- Post-Traumatic Headache

Primary Thunderclap Headache
 High-intensity headache of abrupt onset
 Normal CSF and brain imaging
 Diagnosis only when all organic causes have been excluded
(A) Severe head pain fulfilling criteria B and C
(B) Both of the following:
- Sudden onset, reaching max in <1 min
- Lasting 1 h - 10 d
(C) Doesn’t recur regularly over subsequent weeks or months

DD of Thunderclap Headache
Primary Secondary
-Primary Thunderclap
Headache
- Subarachnoid hemorrhage
- Intracranial hemorrhage,
- Arterial dissection
- Acute hypertensive crisis
- Acute sinusitis (esp. with barotrauma)
- Unruptured vascular malformation
(mostly aneurysm),
- Cerebral venous thrombosis
- Colloid cyst of the third ventricle
- CNS angiitis,
- Reversible benign CNS angiopathy
- Pituitary aploplexy
- Spontaneous IC hypotension

D etailed H istory & E xam ination
S econdary headache R ed Flag
P rim ary headache
Yes
N o
W ith
A utonom ic
W ithout
A utonom ic
D uration
< 4 hours
D uration
> 4 hours
D uration
< 4 hours
D uration
> 4 hours
M igraine
Tension H .
C luster
P aroxism al
H em icrania
S U N C T
H em icrania
C ontinua
N D P H
P ulsating
4-72 h
P ressing
30m in-7d
B oring
about 1 hr
S udden severe
m ax in <1 m in
S harp stabbing
few sec
P ulsating
C ontinuous
U nilateral
P ressing
C ontinuous
B ilateral
P ulsating
1-72 h in child
M igraine
1ry S tabbing
H eadache
1ry Thunderclap
H eadache
P ulsating
about 15 m in
S tabbing
about 1 m in
C ough,
E xertional,
S exual & H ypnic
H eadache
T riggered by
cough, exertion,
sex and sleep

III- Is there is another type of headache ?
 It is common experience but under-appreciated that
headache patient attending clinic often have more than one
type of ICHD diagnosis.
 Patients can have up to five different ICHD-II diagnoses.
 Study show that about 90% of patients with chronic tension
headache had also migraine (transformed migraine).
 It is a common mistake to simplify the situation by boiling
down a complex clinic problem to just 1 diagnosis

IV- What about Co-morbidity ?
 Definition: any additional coexistent condition in a
patient with a particular index disease.
 Importance:
 Overlap of symptom profile,
 Overestimation of the disease burden,
 Therapeutic limitations or sometimes therapeutic
opportunities
 Epidemiological clues to the pathophysiological
mechanisms

Common co-morbidity with headache
 Psychiatric: depression, anxiety, panic disorder, bipolar
 Neurological: epilepsy, stroke
 Vascular risk factors: HTN, DM, hypercholesterolemia,
high homocysteine levels, over weight.
 Heart: coronary heart disease, patent foramen ovalea,
mitral valve prolapsea, atrial septal aneurysma
 Other: snoring/sleep apnea, asthma/allergy, SLE, non-
headache pain

Some types of Secondary Headches
 Medication Overuse Headache (MOH)
 Withdrawal or Rebound Headache
 Post-dural Puncture Headache
 Temporal arteritis
 Carotid and Vertebral Artery Dissection
 Sinusitis
 Acute Narrow Angle Glaucoma
 Hypertensive Headache
 Headache and Epilepsy
 Cranial Neuralgias

Medication Overuse Headache (MOU)
 Common in patients with chronic headache overusing
symptomatic medications
 Features:
 Original headaches become more frequent or continuous
 Medications no longer prevent headaches
 Early morning worsening
 Importance:
 Difficult diagnosis of the original headache
 Responsible for “transformation” of episodic into chronic
headache
 Psychiatric co-morbidity and dependency traits to be
considered
 MOU should be excluded or treated before diagnosing Primary
CDH disorders.

Medication Overuse Headache
 Common Culprits:
 Simple analgesics: > 15 days for > 3 months
 Opiods, Ergotamines, Triptans or Combination of
medications: > 10 days/month > 3 months
 May differ depending on drug being overused:
Triptans Analgesics
Daily migrainous
headache
Diffuse featureless
headache

Medication Overuse Headache (MOH)
Analgesic-overuse headache
A. Headache present on >15 days/month with at least 1 of the
following characteristics and fulfilling criteria C–D:
1. Bilateral
2. pressing/tightening (non-pulsating) quality
3. Mild or moderate intensity
B. Intake of simple analgesics on ≥15 days/month for ≥3 months
C. Headache has developed or markedly worsened during
analgesic overuse
D. Headache resolves or reverts to its previous pattern within 2
months after discontinuation of analgesics

Medication Overuse Headache (cont.)
Triptan-overuse headache
A. Headache present on >15 days/month with at least 1 of the following
characteristics and fulfilling criteria C–D:
1. Predominantly unilateral
2. Pulsating quality
3. Moderate or severe intensity
4. Aggravated by or causing avoidance of routine physical activity (eg,
walking or climbing stairs)
5. Associated with at least 1 of the following:
a) Nausea and/or vomiting
b) Photophobia and phonophobia
B. Triptan intake (any formulation) on ≥10 days/month on a regular basis
for ≥3 months
C. Headache frequency has markedly increased during triptan overuse
D. Headache reverts to its previous pattern within 2 months after
discontinuation of triptan

Medication Overuse Headache (cont.)
 Eliminate Overuse Headache
 Taper and stop offending agents
 Initiate preventive and nondrug therapy as taper
begins
 Explore and treat psychiatric related issues
 Supportive treatment: hydration, antiemetics, anti-
withdrawal agents if needed
 Add abortive therapy once withdrawal headache
passes
Ingredients: Succinic acid, fumaric acid,
dextrose and bioflavonoids

Withdrawal or Rebound Headache
 It is characterized by a dependency on acute medication
and refractoriness to preventive medication.
 If a patient stops overusing acute medications, the results
usually include withdrawal symptoms, a period of increased
headache, and then headache improvement.
 Continuous use of caffeine, nicotine, and other substances
can constrict blood vessels → blood vessels adapt a semi
constricted state. However, if this is withdrawn, blood
vessels dilate causing considerable headache.
 That is how stopping or delaying intake of coffee or tea can
cause headache

Post-dural Puncture Headache
 Bilateral throbbing headache that worsens with upright
position, usually cervical and occipital
 40% of cases after lumbar puncture
 Duration: It can last up to 5 days
 Cause: persistent leak of CSF that exceeds its production
 Prevention:
 Treatment: Rest, IV fluids, Caffeine, NSAIDS, theophylline,
Ergots, Narcotics, Blood patch
Small needle Hit it on the 1st attempt
Take less CSF Avoid lifting, bending,
squatting for 3 days

Temporal arteritis (TA)
 Suspect if
 New headache in patients over 50 years (Mean 71 yrs)
 Unilateral temporal headache that is
 Dull & boring with superimposed lancinating
 Appears gradually over few hrs before peak intensity
 Persistent but often worse at night or on cold exposure
 Accompanied by
 Marked scalp and temporal artery tenderness,
 Jaw claudication, visual disturbances,
 fever, sweats, weight loss
 fatigue, aches, proximal myalgias.

Temporal arteritis (cont.)
 It’s a medical emergency
 because long term sequelae is permanent visual
loss caused by ischemic optic neuropathy.
 Diagnosis:
 ESR, CRP, LFTs, platelet count, temporal artery
biopsy, neuroimaging
 Treatment
 Prednisone 60-120mg daily.

Carotid and Vertebral Artery Dissection
 Most common cause of stroke in persons younger
than 45 years.
 Cause: sudden neck movement or trauma
following neck torsion, chiropractic manipulation,
coughing, minor falls, MVA.
 Latent period: Patients can present with stroke
symptoms days to years after dissection.
 Pathology: intramural hemorrhage in the media of
the arterial wall that can be subtle in the early
phase leading to thrombus formation over time
with emboli or significant enough to occlude the
vessel.

A- Carotid artery Dissection
 Classic triad includes
 Unilateral headache,
 Ipsilateral partial Horner,
 Contralateral hemispheric findings like aphasia,
neglect, visual disturbance or hemiparesis.
 Older age, occlusive disease, stroke on initial
presentation has worse prognosis
 Diagnosis
 CT angiography,
 MRI/MRA

B- Vertebral Artery Dissection
 Clinically:
 Unilateral posterior headache, and
 Neurological findings like vertigo, ataxia,
diplopia, hemiparesis, and unilateral facial
weakness, tinnitus
 Diagnosis:
 Is same as in carotid dissection
 Treatment:
 Early anticoagulation followed by
 Antiplatelet therapy

Sinusitis
 Acute Sinusitis:
 Severe localized headache (behind brow bone
and/or cheek bones)
 With tenderness over sinus
 Chronic Sinusitis
 On awakening or in midmorning
 Worsened by stooping or bending or changes in
atmospheric pressure

Acute Narrow Angle Glaucoma
 Unilateral headache, vomiting, ↓ visual acuity,
 Halos around lights, scotomas
 Red eye
 Fixed mid dilated pupil
 Shallow anterior chamber (not in cluster headache)
 IOP: 60 to 90 mmHg (not in iritis)
 Treatment:
 topical miotics, b-blockers, carbonic anhydrase
inhibitors, ophthalmic consultation

Hypertensive Headache
 Elevated BP is not as important in headache as the
rate by which the BP increases
 Headache with severe HTN is well documented
especially in hypertensive encephalopathy
 Treatment
 Dehydrating measures
 Lowering BP slowly
 Headache may last for days until brain edema has
resolved

Epilepsy and Headache
 Coexisting epilepsy & migraine: Both disorders occur together at an
increased prevalence, but attacks occur independently
 Epilepsy-induced headache (preictal, ictal or postictal): Headache occurs
as part of seizure or postictal state
 Migraine-induced epilepsy (migralepsy): Seizures are triggered by migraine
aura
 Epilepsy-migraine syndromes: Syndromes with features of both migraine
and epilepsy
Primary
(without a specific underlying cause)
Secondary
(with a common underlying cause )
- Occipital epilepsies
(e.g., benign occipital epilepsy)
- Benign rolandic epilepsy
- Mitochondrial disorders (MELAS)
- Symptomatic
(e.g., AVM of occipital lobe)
- Neurofibromatosis
- Sturge-Weber

Epilepsy and Headache (cont.)
 Preictal and ictal headache:
 They are relatively rare and short-lived. The seizure itself
may limit the patient’s ability to observe or recall the
manifestations of these headaches.
 Hemicrania Epileptica: hemicranial attacks of pain
coincided with seizure activity and lasted for seconds to
minutes.
 Postictal headache
 It is common and can affect the patient's quality of life.
 It is most common with GTCC, is also common with
complex partial seizures, and is less common with simple
partial seizures

Cranial Neuralgias
 Neuralgia denotes a sharp, shooting (“lancinating”) pain,
that is momentary but characteristically recurs.
 It may be precipitated by touch to a sensitive area (“trigger
zone”), or may occur spontaneously.
 Unlike headache syndromes, which are probably mediated
centrally, neuralgias are more characteristic of peripheral
nerve localization.
 Neuralgias may follow nerve trauma, herpes zoster
infections, or may arise spontaneously.

Trigeminal Neuralgia
“Tic Doloureau”
 Definition: a neuropathic disorder characterized by sudden, unilateral,
severe brief paroxysms of shooting and stabbing pain in the area
innervated by the 2nd and 3rd branches of the trigeminal nerve, Lasting
from a few seconds to minutes, Precipitated from trigger zones or by
trigger factors.
 Pain :
 Severe Stereotypical
 Sharp Stabbing
 Superficial Shock-like
 Trigger factors:
 Talking Shaving
 Smiling Applying make-up
 Chewing Wind
 Teeth brushing

Notes about Trigeminal Neuralgia
Distribution of pain
Maxillary 35%, Mandibular 30%, both 20%
Trigger points
Cheek, lib, nose, buccal mucosa

Notes about Trigeminal Neuralgia
 Early attacks, appear most often in the 5th decade.
 Usually early attacks mild and brief
 Pain-free intervals may last minutes, hours, days, or longer
 With advancing years, the painful episodes tend to become
more frequent and agonizing
 Patient lives in constant fear of attacks

Types of Trigeminal Neuralgia
 Typical TN
 Atypical TN
 Pre-TN
 Multiple sclerosis-related TN
 Tumor-related TN
 Post-traumatic TN (trigeminal neuropathy)
 Failed TN

Classical Trigeminal Neuralgia
A. Paroxysmal attacks of pain lasting from a fraction of a
second to 2 minutes, affecting 1 or more divisions of
the trigeminal nerve, & fulfilling criteria B & C.
B. Pain has at least 1 of the following characteristics:
 Intense, sharp, superficial, or stabbing
 Precipitated from trigger zones or by trigger factors
C. Attacks are stereotyped in the individual patient
D. No clinically evident neurological deficit
E. Not attributed to another disorder.

Symptomatic Trigeminal Neuralgia
A. Paroxysmal attacks of pain lasting from a fraction of a second to 2
minutes, with or w/o persistence of pain between paroxysms, affecting
1 or more divisions of the trigeminal nerve, & fulfilling criteria B & C.
B. Pain has at least 1 of the following characteristics:
 Intense, sharp, superficial, or stabbing
 Precipitated from trigger zones or by trigger factors
C. Attacks are stereotyped in the individual patient
D. A causative lesion, other than vascular compression, has been
demonstrated by special investigations &/or posterior fossa exploration.
 Examples:
 Multiple sclerosis-related TN
 Tumor-related TN
 Post-traumatic TN (trigeminal neuropathy)
 Failed TN

Management of Trigeminal Neuralgia
 Medical: Carbamazepine, Gabapentin, Baclofen, Phenytoin,
Valproate, Chlorphenesin.
Adjuvant : TCAs ,NSAIDs
 Surgical
 After failure of Pharm agents
 Unusual
 Recurrences occur for many
 Both percutaneous & open techniques
 Glycerol injection Ballon Compression
 Radio Rhizotomy Gamma knife
 Partial Rhizotomy Microvascular decompression
 No Behavioral, unless it becomes a cause of Chronic Pain

Glossopharyngeal neuralgia
 Pain attacks similar to these in trigeminal neuralgia, but
located unilaterally in the distribution of the
glossopharyngeal nerve. (back of the throat, area near
tonsils, back of tongue, and part of the ear).
 Rare disorder, begins after age 40 and occurs more in men
 Its cause is unknown, rarely, caused by brain or neck tumor
 DIAGNOSE: For the test, a doctor touches the back of the throat
with a cotton-tipped applicator. If pain results, the doctor
applies a local anesthetic to the back of the throat.

Superior laryngeal neuralgia
Vagal neuralgia
 This syndrome is rare caused by compression of the upper fibers of the
vagal nerve as they leave the brain stem and traverse the
subarachnoid space to the jugular foramen
 C/P
 Paroxysms of shock-like pain in the side of the thyroid cartilage,
pyriform sinus, angle of the jaw, and, rarely, in the ear. Occasionally
the pain radiates into the upper thorax.
 When other portions of the vagus nerve are involved: hiccups,
inspiratory stridor, excessive salivation, or coughing.
 Trigger zone is usually in the larynx;
 Precipitated by talking, swallowing, yawning, or coughing.
 Diagnosis
 History and by identifying the site of the trigger zone
 Laryngeal topical anesthesia or blockade of the superior laryngeal
nerve stops the pain.

Nervus intermedius neuralgia
Ramsay Hunt Syndrome, Geniculate neuralgia
 Primary infection with VZV (HHV 3), latent in the geniculate
ganglion of CN VII
 Rare, self limiting, complete recovery rate <50%
 Morbidity: facial weakness
 C/P
 Facial paralysis
 Inner ear dysfunction (vertigo,
tinnitus, hyperacusis, hearing loss)
 Periauricular pain (otalgia)
 Herpetiform vesicles of the pinna,
ext auditory canal(herpes zoster
oticus), ant 2/3 of tongue, soft
palate

Nasociliary neuralgia
Charlin's neuralgia
 A rare condition
 Lancinating pain in one side of the nose radiating to the
medial frontal region, lasting seconds to hours.
 Precipitated by touching the lateral aspect of the ipsilateral
nostril
 Abolished by block or section of the nasociliary nerve, or by
the application of cocaine to the nostril on the affected side

Neck-tongue syndrome
 Pain in the neck and altered sensation in the
ipsilateral half of the tongue aggravated by neck
movement.
 Cause: damage to lingual afferent fibers
(proprioceptive) travelling in the hypoglossal nerve
to the C2 spinal roots.

Occipital Neuralgia
 Paroxysmal stabbing pain, with or without
persistent aching between paroxysms, in the
distribution(s) of the greater, lesser and/or third
occipital nerves (Tingling and numb sensation in
posterior scalp area, radiating to frontotemporal
region)
 Tenderness over the affected nerve
 Pain is eased temporarily by local anaesthetic
block of the nerve

Supraorbital neuralgia
jabs and jolts
 It is a severe headache syndrome, due to damage to the
supraorbital nerve just above the eye
 The neuralgia is locked to the affected side. This is unlike ice-
pick pains which can switch between different sites or sides of
the scalp.
 Rare
 Causes: blow to the head, black eye, swimming with ill-fitting
goggles "goggle headache" was applied for a while.
 The examination
 Tender supraorbital nerve .
 Reduced sensation or abnormally sensitive to pin-prick testing.

Cold-Stimulus Headache
Ice Cram Headache
 Short-lasting pain, acute frontal non-pulsatile , which may
be severe, develops immediately, and only, after cold
stimulus and resolves within 5 minutes after removal of
cold stimulus
 Induced in susceptible individuals by the passage of cold
material (solid, liquid or gaseous) over the palate and/or
posterior pharyngeal wall
 Due to ingestion of cold food (ice cream or ice), or drink
(cold water) or to inhalation of cold air (very cold weather).

Understanding Different Types of Primary Headaches

Understanding Different Types of Primary Headaches

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