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Headaches need not be a headache for optometrists

C-19309 O/D
Tina Kipioti, MD, FRCSEd Of 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 specialists 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.

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, (eg loss of vision, scotoma). It may be accompanied by fully reversible sensory symptoms, including positive features reversible (pins and needles) speech. and/or Apart negative features (numbness) and fully dysphasic from the visual aura, other premonitory symptoms include photophobia and phonophobia and (aversion in to noise), fatigue, neck stiffness, blurred vision difficulty concentrating.

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Tension-type headache (TTH)

Classification
In 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

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.

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. bilateral, The pain or is typically in pressing tightening

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 conjunctival headache injection attacks and with tearing

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.

The primary headaches

Migraines These are ranked by the World Health Organization (WHO) as number 19 among all diseases worldwide causing

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spots or lines) and/or negative features

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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

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

50

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).

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The secondary headaches

The 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 include classic scalp symptoms tenderness, of pain GCA on

Table 1 Types of secondary headaches

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

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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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.

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Figure 2 Ipsilateral Horners Syndrome

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 as is sometimes referred cerebri. to The pseudotumour with normal consistency of the CSF. Carotid artery dissection Intracranial vascular disorders causing headaches threatening. are less common, studies but have important to recognise as they are life Previous 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 Horners 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

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 hydrocephalus, (stroke, meningitis, trauma), dural

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

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

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neuropathy or retinal artery occlusion

Headaches
Symptoms Examination Consider

and diplopia from cranial nerve palsies. Subarachnoid haemorrhage This 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
Primary headaches (migraine - TTH)

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Aura

Chronic headache

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

Family history of migraines

Daily recurrence

Conjunctival signs

Cluster headaches/ TAC

cover the brain), the pia and arachnoid mater, causing headache and raised ICP. Other causes include venous bleeds, clotting disorders and haemorrhages due

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Patient over 50 years of age

Scalp tenderness Myalgia

Thickened, non-pulsatile temporal arteries Optic Neuropathy

to anticoagulation (warfarin). Typically, it

Giant Cell Arteritis

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

Jaw claudication

Malaise

Transient

diplopia

Worse in the morning Headache change with posture

Swollen Optic nerves

stiffness, loss of consciousness, agitation,

Raised Intracranial Pressure (ICP)

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

Visual Obscurations

Enlarged blind spot

Deteriorating headache Visual loss

Diplopia Arm and Neck pain

Horners syndrome

Cranial nerve palsies

(eg limb weakness). Ocular manifestations

Carotid Artery Dissection

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

Focal Neurological Signs

Thunderclap headache Neck stiffness

Nausea & Vomiting Confusion & Altered Consciousness

Papilloedema

6th Nerve Palsy

is referred to as Tersons syndrome.

Subarachnoid Haemorrhage

Subhyaloid Haemorrhage

Dural venous sinus thrombosis Thrombosis of cerebral veins (or venous sinuses) is an uncommon condition (although a lot more prevalent than

Electric shocklike quality

Unpleasant sensations

Reduced corneal sensation

Anisocoria

Trigeminal Neuralgia

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)

Figure 3 Differential diagnosis of headaches

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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 backpressure 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.

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.

refractive correction) Ocular motility and cover test revealing the presence of heterotropia and muscle under-actions following IIIrd, IVth and VIth nerve palsies Visual field defects, eg those related to papilloedema (enlarged blind spot) or AION (altitudinal) Anisocoria, and/or fixed dilated pupils Eyelid ptosis (as seen in Horners syndrome) Slit lamp examination of ocular redness and the anterior chamber angle (looking for the presence of papilloedema) Palpate temporal arteries Figure 3 provides a quick reference guide practitioners can use to aid differential diagnosis. Binocular indirect fundoscopy

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Diagnostic approach
When 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, triggers of the headache, any change in the pattern of pain  Other neurological symptoms (nausea, vomiting, tinnitus) or migraineous aura  Headache upon waking or deteriorating with postural changes Neck or arm pain  Fever or seizures or change in personality and mental status  Diplopia, blurred vision or visual obscuration Redness or swelling of the eye(s)  If the patient is over 50 years of age, it is important to specifically enquire about other GCA symptoms such as scalp tenderness Signs Reduced visual acuity (with best

Cranial neuralgias, facial pain and other headaches

The important cranial neuralgias and facial pains to remember optic migraine, include neuritis, head trigeminal neuralgia,

Conclusion
There 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 patients life or vision. In case of uncertainty, a telephone call to emergency eye services for advice may avoid a referral or and indeed expedite an admission appropriate management.

ophthalmolplegic zoster and

or facial pain attributed to herpes 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

About the author

Tina 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.

References
See on www.optometry.co.uk/clinical. references to download. Click on the article title and then

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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

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

1. Which of the following is NOT likely to cause dry eyes? a) Systemic antihistamines b) Increasing age c) Previous laser refractive surgery d) Frequent preservative free lubrication 4. What is the MOST appropriate first line treatment for dry eyes? a) Artificial tear substitutes b) Autologous serum eye drops c) Vitamin A therapy d) Tarsorrhaphy 5. Which of the following statements regarding treatment of dry eyes is TRUE? a) Tetracyclines may be effective in treating meibomian gland dysfunction b) Preservative-free medication may exacerbate symptoms of dry eyes c) Dietary modification is not effective for treating dry eyes d) Autologous serum carries no risk as it is derived from the patients 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 eyes b) They are only placed in the lower eyelid punctae c)They can cause irritation of the ocular surface if not fitted correctly d) They are a first choice treatment for dry eyes caused by blepharitis

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2. Which of the following signs is NOT associated with dry eyes? a) Congestion of conjunctival vessels b) Filamentary keratitis c) Superficial punctate corneal erosions d) 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 osmolarity c) Fluorescein and lissamine dye staining d) All of the above

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Module questions Course code: C-19309 O/D

1. Which of the following is a common feature of cluster headaches? a) Bilateral eye pain b) Generalised headache c) Diplopia d) 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 malaise b) Perform fixation disparity testing and prescribe the full amount of prism c) 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 weakness b) Visual field loss c) Colour vision defects d) Horners 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 prisms b) She is likely to have papilloedema and should be referred as an emergency c)There will be no other signs or symptom associated with this condition d) 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 headaches b) He should be referred routinely to ophthalmology c) There could be a left Horners syndrome d) 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 posture b) Headache worse in the morning, often waking up the patient c) Unilateral headache or pain around the eye with conjunctival redness and lacrimation d) Mild to moderate chronic / recurrent headache, worse in the evening, relieved by painkillers

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