Headaches need not be a headache for optometrists

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1 Headaches need not be a headache for optometrists C 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 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. 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 The International Classification of Headache Disorders with the second, most recent edition, in 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 headaches Migraines These 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 49 13/07/12 CET For the latest CET visit

2 50 (SUNCT). Cluster headaches are attacks of severe, strictly unilateral pain, which can be orbital, supraorbital or temporal, lasting 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). 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 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. Table 1 Types of secondary headaches 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 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 Find out when CET points will be uploaded to Vantage at

3 CET 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 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 dissection Intracranial 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 51 For the latest CET visit

4 neuropathy or retinal artery occlusion Headaches and diplopia from cranial nerve palsies. Subarachnoid haemorrhage 52 Aura Family history of migraines Daily recurrence Patient over 50 years of age Jaw claudication Transient diplopia Visual Obscurations Deteriorating headache Visual loss Thunderclap headache Neck stiffness Electric shocklike quality Symptoms Chronic headache Scalp tenderness Myalgia Malaise Worse in the morning Headache change with posture Diplopia Arm and Neck pain Nausea & Vomiting Confusion & Altered Consciousness Unpleasant sensations Figure 3 Differential diagnosis of headaches Examination Conjunctival signs Thickened, non-pulsatile temporal arteries Optic Neuropathy Swollen Optic nerves Enlarged blind spot Horner s syndrome Cranial nerve palsies Focal Neurological Signs Papilloedema 6th Nerve Palsy Subhyaloid Haemorrhage Reduced corneal sensation Anisocoria Consider Primary headaches (migraine - TTH) Cluster headaches/ TAC Giant Cell Arteritis Raised Intracranial Pressure (ICP) Carotid Artery Dissection Subarachnoid Haemorrhage Trigeminal Neuralgia 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 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 thrombosis Thrombosis 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) Find out when CET points will be uploaded to Vantage at

5 becomes obstructed by a clot and ceases with presbyopia). Confirmation of the refractive correction) to drain CSF from the sub-arachnoid diagnosis is based on the rapid response Ocular motility and cover test space, thus resulting in raised ICP, to appropriate glasses. Similarly, a revealing the presence of heterotropia headaches and papilloedema, with or heterophoria or heterotopia may also and muscle under-actions following without visual symptoms. The patient cause recurrent, non-pulsatile, mild to IIIrd, IVth and VIth nerve palsies may be otherwise well initially, but as moderate frontal headaches, usually Visual field defects, eg those related the drainage of cerebral veins remains absent upon awakening, but worsening to papilloedema (enlarged blind spot) obstructed, the slow flow and backpressure may eventually lead to stroke, throughout the day. Headache-inducing heterophoria tends to be either significant or AION (altitudinal) Anisocoria, and/or fixed dilated 53 with focal neurological signs, seizures (close to or at limit of the fusion range) pupils and coma. Imaging is paramount for or intermittent (controlling a large-angle Eyelid ptosis (as seen in Horner s the diagnosis of this condition, but divergent squint). Other symptoms syndrome) the findings may be subtle and the include intermittent blurred vision or Slit lamp examination of ocular clinician must have a high index of diplopia and difficulty adjusting visual redness and the anterior chamber angle clinical suspicion to order the correct focus from distance to near and vice versa. Binocular indirect fundoscopy examination (MRI with venography is the investigation of choice; CT scan alone will miss a significant number of cases) and instigate appropriate treatment. Diagnostic approach When faced with a patient complaining of headaches, one has to remember that the (looking for the presence of papilloedema) Palpate temporal arteries Figure 3 provides a quick reference Cranial neuralgias, facial pain and other headaches The 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, 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 guide practitioners can use to aid differential diagnosis. 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 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 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 are normally absent on awakening and See are typically precipitated or aggravated Signs Click on the article title and then by prolonged visual tasks (eg reading Reduced visual acuity (with best on references to download. For the latest CET visit

6 PEER REVIEWED 54 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 CET points for these exams will be uploaded to Vantage on September 17, Find out when CET points will be uploaded to Vantage at Module questions Course code: C 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 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 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 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 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 Module questions Course code: C 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) 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 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 Horner s 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 Find out when CET points will be uploaded to Vantage at