Headache

Dr. Ra’ed Ahmed

MBChB, FIBMS

Neurologist

Headache

A headache or cephalagia is pain anywhere in the region of head or neck

Headache is defined as diffuse pain in various parts of the head, with the pain not confined to the area of distribution of a nerve

Isolated involvement of the bony skull, most of the dura, or most regions of brain parenchyma does not produce pain.

Pain Sensitivity of Cranial Pain Sensitivity of Cranial StructuresStructures

Cranial venous sinuses with afferent veins

Arteries at base of brain and their major branches

Arteries of the dura Dura near base of brain and large

arteries Dural, Cranial and extracranial

nerves All extracranial structures

Brain parenchyma

Ependyma

Choroid

Pia

Arachnoid

Dura over convexity

Skull

Pain-Sensitive Pain-Insensitive

General Mechanisms of Headache

1.Traction, tension, or displacement of pain-sensitive structures

2. Distention or dilation of intracranial arteries

3. Inflammation of pain-sensitive structures

4. Obstruction of CSF pathways with consequent increased intraventricular pressure

5. Primary central pain: involvement of pain-modulating systems

6. Stimulation from disease of eye, ear, nose and sinuses (referred pain)

Primary headache syndromes

Migraine (with or without aura)

Tension type headache

Trigeminal autonomic cephalalgia (including cluster headache)

Primary coughing/exertional/sex- related headache

New daily persistent headache syndrome

Secondary causes of headache

Medication overuse headache( chronic daily headache)

Intracerebral bleeding (SDH, SAH or ICH)

Raised ICP ( Brain tumour , IIH )

Infetions (meningitis , encephalitis , brain abscess)

Inflammatory disease ( temporal arteritis , other vasculitis , arthritis)

Referred pain from other structures ( orbit , TM joint, neck).

Temporal distribution of different types of headache with time

Migraine

Tension headache

Migraine + Tension (combination)

Cluster headache

Raised intracranial pressure

MIGRAINE

Migraine, the second most common cause of headache,

Afflicts approximately 15% of women and 6% of men over a 1-year period.

Migraine is a benign and recurring syndrome of headache associated with other symptoms of neurologic dysfunction in varying admixtures.

Pathophysiology

The cause of migraine is unknown. Intracranial vasoconstriction and extracranial

vasodilatation More recent studies “Spreading depression” in cerebral

blood flow Later in the headache phase, blood flow increases to

parts of the cortex (cingulate, auditory, and visual association areas) and the contralateral brainstem

Aggregation of migraine within families

Clinical features of Migraine

Headache that is usually unilateral and frequently pulsatile in quality;

it is often associated with nausea, vomiting, photophobia, phonophobia, and lassitude.

Visual or other neurologic auras occur in about 20% of patients.

Two-thirds to three-fourths of cases of migraine occur in women;

Onset is early in life—more than 90% before age 40. A family history of migraine is present in most cases.

Clinical features of migraineClinical features of migraine

SleepySleepy

AnorexiaAnorexia nauseanausea Vomiting

Vomiting

yawningyawning

PhonophobiaPhonophobia

PhotophobiaPhotophobia

PhonophobiaPhonophobia

PhotophobiaPhotophobia

OsmophobiaOsmophobia

OsmophobiaOsmophobia

Vomiting

VomitingDeep sleepDeep sleep

HeadacheHeadache

IIIIII IV IVHeadache Resolution Headache Resolution

Blau (1992)Blau (1992)

II II II NormalNormal Prodromes Aura Prodromes Aura

NormalNormal

AppetiteAppetite

Awake/sleepAwake/sleep

Light toleranceLight tolerance

SmellSmell

NoiseNoise

Fluid balanceFluid balance

CravingCraving

TiredTired yawningyawning

HeightenedHeightened

perceptionperception

FluidFluid retentionretention

VVPostdromes NormalPostdromes Normal

Limited

Limited

Light toleranceLight tolerance

NoiseNoise

SmellSmell

Fluid balanceFluid balance

TiredTired

FeelingFeeling

high orhigh or

lowlow

DiuresisDiuresis

AppetiteAppetite

Awake/sleepAwake/sleep

food tolerance food tolerance

NormalNormal

Fortification Spectrum

DIAGNOSIS

The diagnosis of migraine is based on the history. If the headache is typical of migraine and the findings on neurologic examination are normal, no further studies

A headache diary can often be helpful in making the diagnosis;

The ddx includes tension-type headache,but migraine at its most basic level is headache with associated features, and tension-type headache is headache that is featureless.Most patients with disabling headache probably have migraine.

History suggestive of a secondary headache, further evaluation with MRI should be considered.

STRATEGIES FOR MIGRAINE STRATEGIES FOR MIGRAINE TREATMENTTREATMENT

Preemptive treatmentMigraine triggertime-limited and

predictable

Preemptive treatmentMigraine triggertime-limited and

predictable

PreventiveTreatment

Decrease inmigraine frequency

warranted

PreventiveTreatment

Decrease inmigraine frequency

warranted

Acutetreatment

To stop pain and prevent progression

Acutetreatment

To stop pain and prevent progression

Silberstein SD. Cephalalgia. 1997.

MIGRAINE TRIGGERSMIGRAINE TRIGGERS

Diet

Hormonal changes

Stress and anxiety

Sleep deprivation or excess

Environmental factors

Physical exertion

ACUTE ATTACK THERAPIES FOR MIGRAINE:

Severity of the attack Mild migraine attacks can usually be managed by

oral agents; Severe migraine attacks may require parenteral therapy

For mild attacks = Acetaminophen or Aspirin For moderate to severe attacks, options include -Dihydroergotamine ( intranasally); -Oral, intranasal, or SC Sumatriptan For very severe attacks = Dihydroergotamine SC or

IV is usually effective but generally requires an antiemetic(e.g., promethazine) before intravenous use.

Ergotamine and DHE are nonselective receptor agonists and they are C/I in pregnancy ,coronary ,peripheral vascular diseases and hypertension.

while the Triptans are selective 5-HT 1B/1D receptor agonists. They have the same contraindications as previous group and should never combined with them.

Recurrence of headache is another important limitation of triptan. Ergotamine appears to have a much higher incidence of nausea than triptans, but less headache recurrence.

Pevention

1-β-adrenergic blockers: Propranolol tab. 40–120 mg bid 2- Anticonvulsants : Topiramate tab.( 25–200 mg/d),

Valproate tab( 400–600 mg bid) 3-Tricyclic antidepressants : Amitriptyline, (10-25 mg

qhs) ; 4-Calcium-channel antagonists: Verapamil (120 to 480

mg/day) 5-Other alternatives include the serotonergic drug

Cyproheptadine(4 to 20 mg) 6-OnabotulinumtoxinA injection is also effective for

prophylaxis.

Tension-type headache

most common type of headache Experienced to some degree by the majority of the

population. It is a chronic disorder that begins after age 20. Women are more commonly affected than men. The headache may be episodic or chronic (present

>15 days per month). Pathophysiology of tension-type headache is less

well understood

Clinical features

‘dull’, ‘tight’ or like a ‘pressure’, and there may be a sensation of a band round the head or pressure at the vertex.

It is of constant character and generalised, but often radiates forwards from the occipital region.

In contrast to migraine, the pain can remain unabated for weeks or months without interruption

no associated vomiting or photophobia Activities are usually continued throughout, and the pain may

be less noticeable when the patient is occupied. The pain is usually less severe in the early part of the day,

becoming more troublesome as the day goes on. Tenderness may be present over the skull vault or in the

occiput.

Treatment

Episodic tension-type headaches are generally treated successfully with acetaminophen (650 to 1000 mg) or NSAIDs (aspirin, 900 to 1000 mg; naproxen, 250 to 500 mg; ibuprofen.

Chronic tension-type headaches may benefit from prophylactic treatment with amitriptyline (starting with 10 mg at bedtime and increased slowly up to 100 mg until the patient improves or intolerable side effects develop).

Muscle relaxants and physical therapy are also useful.

TRIGEMINAL AUTONOMIC CEPHALALGIAS (TACs)

1. Unifying features include pain in distribution of trigeminal nerve and autonomic signs reflecting activation of cranial parasympathetic system2. Includes cluster, paroxysmal hemicrania, short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) syndrome3. Because many of these syndromes are unilateral and associated with autonomic symptoms, neuroimaging is often necessary to rule out structural causes

Cluster headache

A rare form of primary headache much less common than migraine.

5 : 1 male predominance and onset is usually in the third decade

(migraine more common in female & has earlier onset typically start in teenage and rarely after 40).

Cluster headache lacks genetic predisposition (-ve family history). Cluster headache lack of provoking dietary factors , Alcohol

provokes the already ongoing attacks of cluster headache. Different drug effect: propranolol and amitriptyline used as

preventive treatment in migraine are ineffective in cluster headache.

Lithium is beneficial for cluster headache and ineffective in migraine.

Clinical features

Cluster headaches are almost always unilateral, and have characteristic ipsilateral autonomic features

Usually behind or above the eye (periorbital) Attacks occur 1-8 times a day and are usually described as

“boring” or “stabbing” pain, excruciating in intensity, constant nonthrobbing ,that is characteristically brief (30–90 minutes)

Highly agitated during the headache phase and are unable to sit or lie down

Patients are generally perfectly well between episodes. Onset is nocturnal in about 50% of patients and awaken the patient

from sleep.

Episodes are often precipitated by the use of alcohol or vasodilating drugs nitroglycerin , especially during a cluster siege

Striking periodicity of cluster attacks in at least 85% of patients (a daily periodicity and may also have a seasonal periodicity).

Cluster period is typically a few weeks (8 to 10 weeks a year), followed by remission for months to years, but a small proportion do not experience remission. (Chronic cluster headache may occur without a remission).

ACUTE ATTACK TREATMENT of Cluster headache

The attacks peak rapidly, and thus a treatment with quick onset is required Oxygen inhalation should be given as 100% oxygen at 10–12 L/min for 15–

20 min. It appears that high flow and high oxygen content are important. Sumatriptan: (4 to 6 mg) S.C injection can be helpful. Dihydroergotamine: 1-2mg I.M. , or S.C or even intravenously.

Prevention Preventive medications should be started at the beginning of a cluster

bout. Verapamil, 240 to 480 mg, is the drug of choice. Lithium (300 mg twice daily) is another alternative. • Corticosteroids (e.g., prednisone, 40 mg/day, or dexamethasone, 4 mg twice daily

for 2 weeks) act rapidly to prevent cluster headache and can be used acutely while other preventive medications are started

Exertion-Induced Headaches

More common in men than women Typically occurs in middle age Overall prevalence: about 1% Benign cough headache , Primary sex headache & Benign

exertional headache Diagnostic approach: exclusion of structural causes with

neuroimaging and/or lumbar puncture if subarachnoid hemorrhage is a consideration

Treatment Trial of prophylactic indomethacin if headaches are frequent; may require concurrent gastritis prophylaxis . Alternative: naproxen, other NSAIDS

New Daily Persistent Headache (NDPH)

Headache on most if not all days Headache typically develops within 1 to 3 days and persists

(evolution over 3 days) It is generally bilateral, nonpulsating, mild to moderate, and

associated with no more than one of the following: photophobia, phonophobia, or nausea ( headache characteristics similar to those of tension headache)

Diagnostically, it is first important to exclude secondary forms of headache (SAH, raised CSF pressure headache , post-traumatic headache & chronic meningitis)

Treatment: strategies similar to those for transformed migraine and chronic tension headache.

TRIGEMINAL NEURALGIA (TIC DOULOUREUX)

TN is a distinct, excruciatingly painful condition provoked by sensory stimuli in the distribution of the trigeminal nerve

TN occurs in 4 per 100,000 individuals, Between 50 and 70 years of age and in women slightly

more than in men (1.5:1). Associated with multiple sclerosis may result from a

plaque of demyelination in the brainstem (affect younger individuals, and bilateral involvement).

Trigeminal neuralgia pain is characteristically sharp, shooting, and electric shock–like in the distribution of the trigeminal nerve: cheek (V2), chin or lower teeth (V3) and a combination of V2 and V3 is the most common.

Involvement of the first division(V1) or bilateral disease occurs in less than 5% of cases.

The paroxysms are brief—seconds to up to 2 minutes and abate spontaneously. It may be triggered by touch, a cold wind ,eating or brushing the teeth.

Ocurrence during sleep is rare ( this suggest cluster headache rather than TN).

Pain free intervals may last from minutes to weeks but long-term spontaneous remission is rare.

Physical signs are usually absent.

Treatment Of Trigeminal neuralgia

A. Medications Carbamazepine (400 to 1200 mg) is considered the first-line agent for the neuralgia. Intravenous administration of phenytoin, 250 mg, will abort an acute attack.Phenytoin (200 to 300 mg), baclofen (40 to 80 mg), clonazepam (2 to 6 mg), lamotrigine (100 to 400 mg), , and oxcarbazepine (300 to 1800 mg)are also used.

B. Surgical treatments include Microvascular decompression of the vascular loop encroaching on the trigeminal root is said to have a 90% success rate and preserve sensory function. Otherwise, localised injection of alcohol or phenol into a peripheral branch of the nerve may be effective.

WORRISOME HEADACHE RED WORRISOME HEADACHE RED FLAGSFLAGS

“SNOOP”“SNOOP”

Older: new onset and progressive headache, especially in middle-age >50 (giant cell arteritis)

Systemic symptoms (fever, weight loss) or

Secondary risk factors (HIV, systemic cancer)

Neurologic symptoms or abnormal signs (confusion, impaired alertness, or consciousness)

Onset: sudden, abrupt, or split-second

Previous headache history: first headache or different (change in attack frequency, severity, or clinical features)

Medication Overuse Headache (rebound headache)

Overuse of analgesic medication for headache can aggravate headache frequency and induce a state of refractory daily or near-daily headache

Offending agents: many types, including narcotics, barbiturates or barbiturate-containing combination medications, general analgesics, triptans, ergotamine, caffeine

Headache is generally described as

a constant, diffuse, dull headache Anxiety and depression are common

Management of medication overuse

Out-patientsIt is essential to reduce or eliminate analgesic use. Regimes vary from reductions of 10% every week or two .A careful diary over a month or two is very helpful A small dose of an NSAID, as naproxen 500 mg twice daily if tolerated

In-patients (Some patients will require admission for detoxification)

•When such patients are admitted, acute medications are withdrawn completely on the first day, unless there is a contraindication.

•Antiemetics, such as domperidone orally or by suppository, and fluids are administered as required,

•If the patient does not settle over 3–5 days a course of intravenous (DHE)

Idiopathic intracranial hypertension(IIH)

IIH is a disorder of elevated ICP of unknown cause which may lead to visual loss due to papilloedema.

Occurs in obese women of childbearing age

The diagnosis depends on fulfilling the modified Dandy criteria:

1 Symptoms and signs of elevated ICP in an awake and alert patient.

2 No localizing symptoms or signs other than a VIth nerve palsy.

3 Normal neuroimaging (apart from changes due to the raised pressure itself).

4 Lumbar puncture showing elevated opening pressure (>250 mm/H2O) but normal fluid analysis.

Clinically

IIH presents with headache in >90% of patients, it is characteristically severe, daily and pulsatile; unilateral or bilateral , frontal or occipital may be worse in morning .

Associated symptoms may include Transient (seconds) visual obscurations: present in up to 70% of

patients (temporary graying of vision, especially with straining). Bilateral pulsatile tinnitus, and some patients develop hearing loss.

Diplopia (abducens palsy), usually horizontal is present in up to

40% of patients. Visual loss Nausea and vomiting.

Physical examination 1) Papilledema 2) Abducens palsy 3) Visual field abnormalities

Pathophysiology of IIH: unknown cause

Increased CSF formation Decreased CSF absorption Increased venous pressure

Disorders associated with IIH: Endocrine disorders thyroid disorders, Addison’s disease and

Cushing’s disease. Obstructive sleep apnoea, Medications including antibiotics (naladixic acid, ciprofloxacin,

tetracyclines, and nitrofurantoin), Hormonal medications including growth hormone,OCP &

corticosteroids. The strongest link of IIH is with hypervitaminosis A

Investigations of IIH

MRI and MR/CT venography brain to rule out secondary causes of increased ICP (to exclude hydrocephalus, mass lesions, meningeal infiltration, and venous thrombosis).

Radiographic signs of raised intracranial pressure in IIH include flattening of the posterior globe (80%) and an empty sella (70%).

CSF examination to measure opening pressure and rule out secondary causes (meningitis). Also to do therapeutic (high-volume) lumbar puncture.

Treatment of IIH

A.Nonpharmacologic: weight loss with restriction of calorie, salt and fluid intake

B.Pharmacologic Diuretics:Acetazolomide is a strong carbonic anhydrase inhibitor "drug of choice" as that reduces CSF production and is highly effective in IIH. The dose is gradually increased to 1-2 g/day. Common side effects: weight loss, diarrhea, nausea and/or vomiting, altered taste sensation, paresthesias, confusion, polyuria. Other diuretics such as frusemide (40-60 mg twice daily) can be used if acetazolomide not tolerated. Close follow-up is required with assessment of visual acuity, visual fields initially at one month and then 3-monthly.

C.Procedures (serial lumbar punctures).

D. Surgery includes:Lumboperitoneal shunt or ventriculoperitoneal shunt (used to reduce severe headaches or progressive visual loss despite conservative treatment) Optic nerve fenestration: incision of dural covering of intraorbital optic nerve, used to prevent progressive visual loss.

Outcome of IIH : Early treatment prevents visual loss Blindness may occur in up to 10% of patients

Temporal (Giant Cell) Arteritis

Temporal arteritis is an inflammatory process seen mainly in elderly individuals

Headache is one of the most common features

Epidemiology It affects women more often than men (3:1), is more common in

white individuals & almost all older than 50

Pathology Inflammatory vasculitis affecting medium and large extracranial

branches of aortic arch . Other intracranial vessels (vertebral and carotid arteries) can be involved

Clinical presentation

Constitutional symptoms (low-grade fever, fatigue, night sweats, anorexia)

Jaw claudication Temporal headaches with scalp tenderness The headache has no specific feature, but the pain is usually

continuous, generalized, and occasionally throbbing. Ischemic stroke is uncommon but may occur in anterior or

posterior circulation Transient monocular blindness and diplopia can occur. Half of the patients have polymyalgia rheumatica

GCA

Diagnosis Elevation of ESR and CRP occurs almost invariably. ESR more than 50 mm/hIrregularities may be found on angiography of extracranial blood vessels Temporal artery biopsy

TreatmentHigh-dose prednisoneImmediate treatment with corticosteroids, Oral prednisone (doses between 40 and 80 mg daily) should be started without awaiting biopsy results because of risk of blindness Treatment usually continued for 2 year