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Headaches need not be a headache for optometristsC-19309 O/D

Tina Kipioti, MD, FRCSEdOf all the painful states that afflict humans, headache (cephalalgia) is the most common. According to a large study,1 95% of all young women and 91% of men experienced headache during a 12-month period and 18% of the women and 15% of the men found their headache significant enough to consult a doctor. More recent figures in the UK corroborate the significance of headache as a problem.2 Patients see an ophthalmologist or optometrist because they experience pain in, or around, the eyes, or other ophthalmic symptoms and signs such as obscuration or visual phenomena. Widespread knowledge of associations between the eyes and headache means that more patients seek an eye specialist’s opinion, so optometrists may examine patients with headaches often before a GP, due to accessibility. This article discusses the most common causes of headaches and offers advice about optometric investigation and diagnosis.

ClassificationIn the broad sense, headache is any

pain or ache located in the head, but in

practice, only the ones located in the

cranial vault are referred to as headaches.

Headaches have such diverse aetiology

that it is has been a significant challenge

to classify the different types and their

diagnostic criteria. In 1988, after three

years of congresses and combined effort,

the International Headache Society with

a headache classification sub-committee

produced the first edition of The

International Classification of Headache

Disorders with the second, most recent

edition, in 2004.3 In the second edition,

45 primary and 120 secondary headache

types and subtypes are identified, as well

as a further 29 causes of cranial neuralgias

and central causes of facial pain.

When optometrists are faced with a

patient complaining of headaches, an

attempt at classifying the disorder as a

primary headache (eg migraine, tension

headache) or secondary headache (eg

tumour, stroke) should be made. In

general, primary headaches are far

more common and are not related

to significant underlying pathology,

whereas secondary headaches are rarer,

but may be a warning sign of a sinister

underlying cause. The key to aiding

this differentiation is in the history

and symptoms reported by the patient.

The primary headachesMigrainesThese are ranked by the World Health

Organization (WHO) as number 19

among all diseases worldwide causing

disability. They affect all ages, including

children, and there is frequently a

positive family history. They can

be unilateral or bilateral, pulsating,

moderate or severe and can last from a

few hours to three days. The pain is often

localised to the periocular region, or there

may be associated visual aura in the form

of zigzag lines (fortification spectrum).

Occasionally, patients report diplopia.

Migraine without associated aura often

has a strict menstrual relationship.

The aura is fully reversible and consists

of positive features (eg flickering lights,

spots or lines) and/or negative features

(eg loss of vision, scotoma). It may

be accompanied by fully reversible

sensory symptoms, including positive

features (pins and needles) and/or

negative features (numbness) and fully

reversible dysphasic speech. Apart

from the visual aura, other premonitory

symptoms include photophobia and

phonophobia (aversion to noise),

fatigue, neck stiffness, blurred vision

and difficulty in concentrating.

Tension-type headache (TTH)With or without peri-cranial tenderness,

TTH is the least studied of the primary

headache disorders and yet it is, by far,

the commonest. Lifetime prevalence in

the general population is estimated to

be 30-78%4 and is believed to have the

highest socio-economic impact. It was

previously considered to be primarily

psychogenic. The pain is typically

bilateral, pressing or tightening in

quality and of mild to moderate intensity.

Cluster headache and trigeminal autonomic cephalalgias (TAC)Cluster headache is of particular interest

to ophthalmologists and optometrists

because of their frequent localisation

around the eyes. One of the commonest

examples is the ‘short-lasting unilateral

neuralgiform headache attacks with

conjunctival injection and tearing

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(SUNCT)’. Cluster headaches are

attacks of severe, strictly unilateral

pain, which can be orbital, supraorbital

or temporal, lasting 15-180 minutes

and with a typical regular recurrence,

from once every other day to eight

times a day. It is often associated with

conjunctival injection, lacrimation,

nasal congestion, rhinorrhoea, forehead

and facial sweating, miosis, ptosis and

eyelid oedema. The patients are typically

restless or agitated during an attack (in

contrast to the migraine patient, who

wants to lie down in a quiet room).

The secondary headachesThe classification of secondary

headaches includes those listed in Table

1. Those secondary headaches that are

of ocular relevance and consequence

are described in the following sections.

Giant cell arteritis (GCA)Of the secondary headaches, one of

the most important to recognise is

GCA, often referred to as temporal

arteritis. Pathologically, it is a patchy

granulomatous inflammation of medium

to large arteries and is not confined to

the temporal region. One should always

consider GCA if a patient over 50

years of age presents with a headache,

especially if it associated with visual

symptoms or even visual loss. A blood

test (erythrocyte sedimentation rate

– ESR and C-reactive protein – CRP)

can be diagnostic, although it can

also provide a false negative result.

Patients often describe their headache

as a new type or unusually severe.

Other classic symptoms of GCA

include scalp tenderness, pain on

jaw claudication, proximal myalgia,

weight loss, malaise, and more rarely,

eye or orbital pain (indicating ocular

ischaemic syndrome). The headache may

worsen on standing up and be associated

with transient blurred vision (amaurosis

Headache attributed to head and neck trauma

Headache attributed to cranial or cervical vascular disorder

Headache attributed to non-vascular intracranial disorder

Headache attributed to a substance or its withdrawal

Headache attributed to infection

Headache attributed to disturbance of homoeostasis

Headache or facial pain attributed to disorder of cranium, neck, eyes, ears, nose,

sinuses, teeth, mouth or other facial or cranial structures

Headache attributed to psychiatric disorders

Table 1 Types of secondary headaches

Figure 1 Papilloedema

fugax), transient diplopia or even cranial

nerve palsies. AION (anterior ischaemic

optic neuropathy) with optic nerve

swelling and visual loss is a common

first presentation of GCA and, again, the

diagnosis of AION in a patient over 50

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visual obscurations (often postural),

photopsias and transient or persistent

diplopia (there may be third, fourth or

sixth cranial nerve palsy due to raised

ICP). The headache is typically diffuse

and constant, aggravated by coughing,

straining, bending over or lying down

and worse in the morning than in the

afternoon. Disc swelling is usually

bilateral (papilloedema) and necessitates

urgent neuroimaging (magnetic

resonance imaging – MRI – or magnetic

resonance angiogram – MRA) to exclude

a space-occupying lesion or venous sinus

thrombosis. Monitoring of papilloedema

clinically and with Goldmann visual

fields and colour vision testing is

essential, as it can result in visual loss.

Idiopathic intracranial hypertension (IIH)This is sometimes referred to

as pseudotumour cerebri. The

previous name of benign intracranial

hypertension is now abandoned as it

can be very aggressive and refractory to

treatment and many patients lose their

vision (complete bilateral blindness

is possible) or have severe disabling

headaches. It is associated with obesity

(except in children, who may have

normal body weight) and patients are

usually overweight women, who present

with swollen discs, headaches and often

visual obscurations. Diagnosis is based on

the clinical image, a normal appearance

of the brain on neuroimaging and high

opening pressure on lumbar puncture

with normal consistency of the CSF.

Carotid artery dissectionIntracranial vascular disorders causing

headaches are less common, but

important to recognise as they are life

threatening. Previous studies have

suggested that more than 5% of stroke in

young adults is due to dissection (split)

of the carotid artery.5 The split in the

vessel wall leads to stenosis or complete

occlusion of the lumen, resulting in

reduced or absent blood flow, which may

lead to a cerebrovascular accident (CVA)

or stroke. More commonly, clots form on

the ragged vessel wall and embolise to the

head where they lodge in distal arteries,

again resulting in a CVA. Due to the close

proximity of the internal carotid artery to

the sympathetic plexus, 50% of patients

will get an ipsilateral Horner’s syndrome

(Figure 2) and reduced blood flow to

other parts of the brain may result in focal

neurological signs (ie limb weakness on

the opposite side, speech disturbance and

visual field loss) if not recognised early.

Most cases of carotid artery dissection

occur spontaneously, although it can

result from direct head or neck trauma

(eg whiplash) or triggered by a prolonged

bout of coughing. The accompanying

headache is usually gradual in onset

(occasionally sudden) and deteriorates

in severity, often accompanied by scalp

tenderness and pain in the area of the

arm and neck. There may be associated

visual loss from ischaemic optic

years of age with a headache should raise

a strong suspicion of temporal arteritis.

Acute visual loss in one or both eyes

may ensue if not urgently treated with

high dose steroids, and it can be fatal.

The commonest clinical scenario of GCA

is that of an elderly patient with loss of

vision in one eye and pain (headache).

The temporal arteries may be prominent,

inflamed and non-pulsatile, and upon

examination the clinician confirms

an optic nerve swelling and a visual

field defect, usually altitudinal. Such a

patient needs urgent admission to A&E

and intravenous steroids followed by

systemic steroid treatment for a year.

Raised intracranial pressure (ICP)ICP can cause papilloedema. The optic

nerve sheaths are an extension of dura

around the brain and the sub-arachnoid

space of the sheath contains CSF

(cerebrospinal fluid), which is in direct

communication with the CSF flowing

around the brain. When there is high

pressure of the CSF, the pressure extends

around the optic nerve and results in

obstruction of the axoplasmic flow in the

optic nerve axons. A build-up of blocked

axoplasm in the optic nerve head

becomes visible as a swelling, causing the

appearance of papilloedema (Figure 1). If

pressure is unrelieved, the consequences

are optic nerve axon dysfunction

and eventually death (optic atrophy).

Raised ICP may be caused by a number

of reasons, the commonest being an

intracranial space-occupying lesion (eg

a brain tumour or abscess), intracranial

haemorrhage (stroke, trauma),

hydrocephalus, meningitis, dural

venous sinus thrombosis or idiopathic

(pseudotumour cerebri). Symptoms that

patients may report include blurred

vision from optic nerve dysfunction

or from induced hypermetropia (the

eyeball is shortened by pressure from

the dilated optic nerve sheath), transient

Figure 2 Ipsilateral Horner’s Syndrome

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neuropathy or retinal artery occlusion

and diplopia from cranial nerve palsies.

Subarachnoid haemorrhageThis is a medical emergency and requires

an urgent referral to neurology as it is fatal

for over 50% of patients within 24 hours

of onset. The great majority of cases are

due to leakage of blood from an arterial

wall defect of the middle cerebral artery,

a terminal branch of the internal carotid

artery. The blood then spreads between

two of the meninges (the membranes that

cover the brain), the pia and arachnoid

mater, causing headache and raised ICP.

Other causes include venous bleeds,

clotting disorders and haemorrhages due

to anticoagulation (warfarin). Typically, it

presents with a ‘thunderclap’ headache,

which has an onset within a split second

and is frequently described as the ‘worst

ever’ that the patient has experienced.

Often, it is occipital (back of the head)

in site and may be associated with neck

stiffness, loss of consciousness, agitation,

nausea and vomiting (blood is a very good

irritant of the meninges, so it resembles an

acute onset of meningitis). Confusion and

altered consciousness are poor prognostic

indicators, as are focal neurological signs

(eg limb weakness). Ocular manifestations

include the features of raised intracranial

pressure such as papilloedema and sixth

nerve palsies. Infrequently, sub-hyaloid

(pre-retinal) haemorrhage with or without

vitreous haemorrhage may occur, which

is referred to as Terson’s syndrome.

Dural venous sinus thrombosisThrombosis of cerebral veins (or venous

sinuses) is an uncommon condition

(although a lot more prevalent than

previously thought), which often

presents a diagnostic challenge, with a

non-specific and, occasionally, dramatic

presentation which the optometrist may

be the first to see. In this condition, one

of the cerebral veins (usually the superior

sagittal or one of the transverse sinuses)

Headaches

Aura

Examination

Primary headaches(migraine - TTH)

Family history of migraines

Daily recurrence

Chronic headache

Conjunctival signs Cluster headaches/ TAC

Giant Cell Arteritis

Patient over 50 years of age

Thickened, non-pulsatile temporal arteries

Scalp tenderness

Jaw claudication

Symptoms Consider

Optic Neuropathy

Myalgia

Malaise

Visual Obscurations

Headache change with

posture

Transient diplopia

Worse in the morning Swollen Optic nerves

Raised Intracranial Pressure (ICP)

Enlarged blind spot

Deteriorating headache

Focal Neurological SignsArm and Neck pain

Diplopia Cranial nerve palsies

Horner’s syndrome

Visual loss

Carotid Artery Dissection

Thunderclap headache

Nausea & Vomiting

Neck stiffness

Confusion & Altered Consciousness

6th Nerve Palsy

Subhyaloid Haemorrhage

PapilloedemaSubarachnoid Haemorrhage

Electric shock-like quality Anisocoria

Reduced corneal

sensation

Unpleasant sensations Trigeminal Neuralgia

Figure 3 Differential diagnosis of headaches

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with presbyopia). Confirmation of the

diagnosis is based on the rapid response

to appropriate glasses. Similarly, a

heterophoria or heterotopia may also

cause recurrent, non-pulsatile, mild to

moderate frontal headaches, usually

absent upon awakening, but worsening

throughout the day. Headache-inducing

heterophoria tends to be either significant

(close to or at limit of the fusion range)

or intermittent (controlling a large-angle

divergent squint). Other symptoms

include intermittent blurred vision or

diplopia and difficulty adjusting visual

focus from distance to near and vice versa.

Diagnostic approachWhen faced with a patient complaining

of headaches, one has to remember that the

vast majority of headaches are primary or

innocuous, but it is important not to miss

the few that are caused by a more sinister

underlying cause. To this effect, the eye

care practitioner should pay attention

to some important symptoms and signs

that may point to a secondary cause:

Symptoms•History–where,when,triggersofthe

headache, any change in the pattern of

pain

•Otherneurologicalsymptoms(nausea,

vomiting, tinnitus) or migraineous

aura

•Headacheuponwakingor

deteriorating with postural changes

•Neckorarmpain

•Feverorseizuresorchangein

personality and mental status

•Diplopia,blurredvisionorvisual

obscuration

•Rednessorswellingoftheeye(s)

•Ifthepatientisover50yearsofage,

it is important to specifically enquire

about other GCA symptoms such as

scalp tenderness

Signs•Reducedvisualacuity(withbest

refractive correction)

•Ocularmotilityandcovertest

revealing the presence of heterotropia

and muscle under-actions following

IIIrd, IVth and VIth nerve palsies

•Visualfielddefects,egthoserelated

to papilloedema (enlarged blind spot)

or AION (altitudinal)

•Anisocoria,and/orfixeddilated

pupils

•Eyelidptosis(asseeninHorner’s

syndrome)

•Slitlampexaminationofocular

redness and the anterior chamber angle

•Binocularindirectfundoscopy

(looking for the presence of

papilloedema)

•Palpatetemporalarteries

Figure 3 provides a quick reference

guide practitioners can use to aid

differential diagnosis.

ConclusionThere is no doubt that, in the

course of their career, optometrists

are highly likely to be presented with

headache cases, most of which will

be benign but others which may be

life-threatening. Their skill lies in

identifying these few sinister cases and

making a difference to the patient’s

life or vision. In case of uncertainty,

a telephone call to emergency eye

services for advice may avoid a referral

or indeed expedite an admission

and appropriate management.

About the authorTina Kipioti is a consultant ophthalmic

surgeon with an interest in paediatrics

and strabismus. She trained in the UK,

Switzerland and Greece. She was clinical

director in ophthalmology, and honorary

senior lecturer at Aston University.

ReferencesSee www.optometry.co.uk/clinical.

Click on the article title and then

on ‘references’ to download.

becomes obstructed by a clot and ceases

to drain CSF from the sub-arachnoid

space, thus resulting in raised ICP,

headaches and papilloedema, with or

without visual symptoms. The patient

may be otherwise well initially, but as

the drainage of cerebral veins remains

obstructed, the slow flow and back-

pressure may eventually lead to stroke,

with focal neurological signs, seizures

and coma. Imaging is paramount for

the diagnosis of this condition, but

the findings may be subtle and the

clinician must have a high index of

clinical suspicion to order the correct

examination (MRI with venography is

the investigation of choice; CT scan alone

will miss a significant number of cases)

and instigate appropriate treatment.

Cranial neuralgias, facial pain and other headachesThe important cranial neuralgias and

facial pains to remember include

trigeminal neuralgia, optic neuritis,

ophthalmolplegic ‘migraine’, head

or facial pain attributed to herpes

zoster and Tolosa-Hunt syndrome.

Trigeminal neuralgias may be idiopathic

or secondary due to compression of

the nerve by a tumour or aneurysm, or

secondary to multiple sclerosis. It may

be persistent or recurrent, unilateral

or periocular and can occasionally

have an electric shock-like quality,

or unpleasant sensations of ‘pins and

needles’ or ‘ants crawling under the

skin’. Associated decreased corneal

or facial sensation or the presence of

anisocoria, increases the risk of a tumour.

Ophthalmic causes of headache include

angle-closure glaucoma, herpes zoster

ophthalmicus, uncorrected refractive

error and heterophoria or heterotropia.

Headaches due to refractive error tend to

be recurrent, mild, frontal and/or ocular,

are normally absent on awakening and

are typically precipitated or aggravated

by prolonged visual tasks (eg reading

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PEER REVIEWED


PLEASE NOTE There is only one correct answer. All CET is now FREE. Enter online. Please complete online by midnight on September 7, 2012 – you will be unable to submit exams after this date. Answers to the module will be published on www.optometry.co.uk/cet/exam-archive. CET points for these exams will be uploaded to Vantage on September 17, 2012. Find out when CET points will be uploaded to Vantage at www.optometry.co.uk/cet/vantage-dates

1. Which of the following is a common feature of cluster headaches?a) Bilateral eye painb) Generalised headachec) Diplopiad) Red and watery eye

2. What should you do if a 75-year-old man develops an inferior visual field defect and complains of headaches?a) Enquire about scalp tenderness, jaw pain and loss of weight or malaiseb) Perform fixation disparity testing and prescribe the full amount of prismc) Refer him routinely to ophthalmology for further testing (including blood tests)d) Reassure the patient that the headaches are likely to be migraines

3. Which of the following is NOT a common feature of carotid artery dissection?a) Unilateral limb weaknessb) Visual field loss c) Colour vision defectsd) Horner’s syndrome

4. Which of the following is most likely to be TRUE for a 42-year-old overweight woman who complains of recent onset diplopia and severe head pain?

a) She is likely to have a sixth nerve palsy which warrants correction with prismsb) She is likely to have papilloedema and should be referred as an emergencyc)There will be no other signs or symptom associated with this conditiond) The underlying condition is likely to be benign and no further action is required

5. Which of the following is TRUE for a 35-year-old man who develops amaurosis fugax and neck pain on the left side, one week after a whiplash injury?a) He is likely to develop sudden onset occipital headachesb) He should be referred routinely to ophthalmologyc) There could be a left Horner’s syndromed) A visual field defect is unlikely to be present

6. Which of the following is MOST consistent with a headache due to refractive error or heterotropia?a) Thunderclap headache, which changes with different postureb) Headache worse in the morning, often waking up the patientc) Unilateral headache or pain around the eye with conjunctival redness and lacrimationd) Mild to moderate chronic / recurrent headache, worse in the evening, relieved by painkillers

Module questions Course code: C-19309 O/D

1. Which of the following is NOT likely to cause dry eyes?a) Systemic antihistaminesb) Increasing agec) Previous laser refractive surgeryd) Frequent preservative free lubrication

2. Which of the following signs is NOT associated with dry eyes?a) Congestion of conjunctival vesselsb) Filamentary keratitisc) Superficial punctate corneal erosionsd) Cells in the anterior chamber

3. Which of the following tests may be used in the diagnosis of dry eyes?a) Schirmers Type 1 and 2 b) Tear osmolarityc) Fluorescein and lissamine dye stainingd) All of the above

4. What is the MOST appropriate first line treatment for dry eyes?a) Artificial tear substitutesb) Autologous serum eye dropsc) Vitamin A therapyd) Tarsorrhaphy

5. Which of the following statements regarding treatment of dry eyes is TRUE?a) Tetracyclines may be effective in treating meibomian gland dysfunctionb) Preservative-free medication may exacerbate symptoms of dry eyesc) Dietary modification is not effective for treating dry eyesd) Autologous serum carries no risk as it is derived from the patient’s own blood

6. Which of the following statements about punctal plugs is TRUE?a) They are used as a last resort in the treatment of dry eyesb) They are only placed in the lower eyelid punctaec)They can cause irritation of the ocular surface if not fitted correctlyd) They are a first choice treatment for dry eyes caused by blepharitis

Module questions Course code: C-19306 O/D (P44-48)

cet 27 july 2012 kipioti

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