H. Wiethölter and H. Wilhelm
Headache is the most frequent cause for cooperative interaction between neurologists and ophthalmologists. Frequently, patients are seen first by their primary care physicians, and are then referred to ophthalmologists for further testing and management, chiefly to rule out ocular sources of pain. From there, they are commonly referred to neurologists, to be sure that something serious will not be missed. The diagnostic classification of headache is at first glance extraordinarily confusing or overwhelming, considering that the International Headache Society (IHS) has defined 176 different types of headache. This large number can be reduced to a more manageable level, since 90% of all primary headache syndromes are attributable to the two most common types, migraine and tension headache. The fact that there is no clearly defined system that allows for rapid classification of headache, especially cases of primary headache, underscores the importance of a carefully taken history and clinical examination.
A properly structured history will allow proper classification of 25% of all cases of headache. The most important elements of the history include the frequency, location, duration of episodes, intensity of pain, the course taken during episodes, instigating factors, and a family history of headache (■ Table 16.1).
Table 16.1. Checklist of history taking for a work up of headache
Duration of episodes Intensity of pain Usual course Triggering factors Family history
Throbbing headaches that occur once or twice a year or several times a month and which are accompanied by auto-nomic symptoms or signs are, generally, attributable to migraine. The episodic headaches of a cluster syndrome are usually confined to specific periods and often come in groups of up to several episodes each day. More frequent attacks of head and facial pain (up to 20 times daily) are caused by chronic paroxysmal hemicrania. The daily appearance of chronic headache is often the presenting manifestation of a tension headache or an analgesic-induced pain syndrome.
Headaches can be strictly unilateral (as in cluster syndromes), predominantly unilateral (as in migraine), retro-bulbar or retro-orbital (as in cluster syndromes), cervical with pain radiating into the occiput and parietal region (as in cervical neuralgia), or holocephalic (as in tension or analgesic-induced headache).
The episodic pain of SUNCT syndrome (short-lasting unilateral neuralgiform headache with conjunctival injection and tearing) lasts for seconds, the paroxysmal hemicrania from 5 to 30 min, whereas the pain of cluster headaches lasts from 20 min to 2 h. Typical attacks of migraine headache can last from 4 to 72 h. Chronically persistent headache is found in those with tension headaches, analgesic-induced headaches, temporal arteritis, and pseudotumor cerebri.
Intensity of Pain
The intensity of pain in headache syndromes is naturally highly subjective and differs substantially from one person to the next. The most intense pain is usually found in cluster headaches, migraine headaches, and paroxysmal hemi-crania. Temporal arteritis causes a pain of intermediate intensity, while mild to intermediate levels of discomfort are associated with chronic tension headache. Venous sinus thrombosis commonly causes alternating phases of mild to intermediate pain.
An intracranial hemorrhage can produce pain that starts precipitously and with full intensity in a stroke like fashion. Cluster headaches and chronic paroxysmal hemicrania develop within a few minutes. The pain of migraine attains its greatest intensity between 15 min and 2 h of duration. The pain of venous sinus thrombosis has a diurnal course with nocturnal and morning pain, aggravated by the horizontal position during sleep, and partially relieved by erect posture after arising.
A steadily progressive intensity of head pain in a patient with no prior history of headache suggests a serious cause, such as obstruction of the flow of cerebrospinal fluid (e.g., aqueductal compression), or elevated intracranial pressure (pseudotumor cerebri).
Some headaches have specific triggering factors. For migraine, these include alcohol, changes in the sleep-wake cycle (e.g., jet lag), hunger, hormonal changes (e.g., the menstrual cycle), states of stress or agitation, abrupt climate change, and withdrawal from the use of caffeine. Attacks of cluster headache can be triggered by alcohol or by vasodilating medications. There are no specific trigger factors for chronic paroxysmal hemicrania or for tension headaches.
In those with migraine, there is frequently a strongly positive family history, especially among primary relatives - parents and siblings.
The most important disease that produces headache is migraine. The pain can arise with or without an antecedent aura. About 7% of men and 14% of women suffer from this malady.
Migraine is a unilateral, frontotemporally located, pulsating, or boring headache. It frequently appears on awakening and worsens with activity after arising. Due to the accompanying light and sound hypersensitivity (photophobia and phonophobia), patients are compelled to seek out rest, darkness, and silence. Associated autonomic phenomena include nausea and vomiting. Initiating trigger factors can be identified by 90% of sufferers. The pain lasts for 4 to 72 h and is commonly relieved by the following night of sleep. Attacks that last more than a day are uncommon.
The source of pain is thought to be a sterile inflammatory reaction within the perivascular tissues of meningeal vessels, induced by changes in the neuronal activity of a generator in the region of the brain stem nuclei. It is accompanied by the release of vasoactive preinflammatory substances (neuropeptides).
Initially, an aura is produced by a spreading wave of neuronal activity ("cortical spreading depression") that moves across the cerebral cortex. This in turn induces a change in cerebral perfusion. Experimental evidence has found that the volume flow of blood decreases in the occipital cortex, and the hypoperfusion then spreads like a wave at a rate of 2 to 3 mm/min in the direction of the frontal cortex. The release of proinflammatory substances that stimulate pain receptors is responsible for the associated pain.
By nomenclature, migraine with aura (previously known as classic migraine, ophthalmic migraine, hemiparetic migraine, hemiplegic migraine, or complicated migraine) is distinguished from migraine without aura (previously known as simple migraine or hemicrania). The aura corresponds to a disturbance in the occipital cortex, or less commonly to disturbances within the brain stem. Affected patients describe the perception of lightning flashes, and fortification scotomas. The latter are shimmering, zigzag
Fig. 16.1. The most common visual aura of migraine appears as a unilateral (binocular) geometric zigzag figure that usually begins in the center of the visual field and gradually extends to the periphery over a period of 15 to 30 min. The aural images can be very bright and colorful. The term fortification scotoma is sometimes used to describe the figure, due to the similarity in appearance to the ground plan of a seventeenth century fortification
Fig. 16.1. The most common visual aura of migraine appears as a unilateral (binocular) geometric zigzag figure that usually begins in the center of the visual field and gradually extends to the periphery over a period of 15 to 30 min. The aural images can be very bright and colorful. The term fortification scotoma is sometimes used to describe the figure, due to the similarity in appearance to the ground plan of a seventeenth century fortification lines that start at the center of the visual field and gradually expand peripherally with a temporal convexity, leaving behind a central zone of transient blindness: a so-called scintillating scotoma (■ Fig. 16.1). Other symptoms of aura include slowly ascending, from distal to proximal, disturbances of sensation in the extremities, dysarthria (disturbances of articulation), or aphasia (disturbances of language expression or recognition), hemiparesis, and complex neuropsychological deficits, especially in the visual system. These include, for example, changes in color perception, changes in the perceived sizes of objects, loss of movement perception, and visual hallucinations (see Chap. 13). Aura symptoms develop slowly over a period of 5 to 10 min, and as a rule, last at most for an hour before subsiding. In some cases, aura symptoms occur without a subsequent headache.
There are several unusual types of migraine. In basilar artery migraine, the auras reflect disturbances in the blood supply to the brainstem and cerebellum. Symptoms include diplopia, vertigo, tinnitus, paresthesias in the face, ataxia, and quadriparesis.
Ophthalmoplegic migraine takes the form of an oculomotor disturbance with ptosis and diplopia.
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