248 13 Painful Syndromes

Fig. 13.2 Scintillating scotoma during an attack of ophthalmic migraine: typical fortification figures.

Table 13.5 Manifestations of basilar migraine (according to frequency), after Sturzenegger and Meienberg

Bilateral visual disturbance

scintillating scotoma or elementary hallucinations

diffuse loss of visual acuity

transient amaurosis

visual field defects

dysmorphopsias

Nausea

Impairments of consciousness

syncope

confusion

stupor

amnesia

coma

Paresthesiae (bilateral)

Vomiting

Dizziness

Gait ataxia

Dysarthria

Limb weakness

overshadowing the headache to such an extent that the patient’s illness is not immediately recognizable as migraine. We will now describe the major types of complicated migraine individually.

Ophthalmic migraine , the most common and probably best-known type of migraine, is often called “classic migraine” in the English-speaking world. (Note: clinicians in other parts of the world call simple migraine “classic migraine,” so we will avoid confusion here by not using the term any further.) A scintillating scotoma appears 10 to 20 minutes before the headache. It begins in the center of the visual field, impairing the patient’s ability to read (for example). A bright, zigzag line then travels from the center of the visual field outward in one-half of the visual field, until it “falls off the visual field” at the periphery, leaving behind a transient blurriness of vision in the corresponding hemifield. A typical scintillating scotoma or “fortification specter” (think of a crenellated medieval fortress) is shown in the diagram in Fig. 13.2. The scintillating scotoma is usually followed by

an attack of hemicranial headache. Occasionally, a scintillating scotoma may occur without subsequent headache; such events are called “migraine sans migraine.” So-called retinal migraine is a very rare variant involving a vertically demarcated scintillating scotoma or a monocular visual disturbance lasting a few minutes.

Migraine accompagnée is characterized by the combination of headache with hemiparesis, a hemisensory deficit, or some other type of focal neurological or neuropsychological deficit. This variant of migraine usually has its onset in childhood or adolescence. In each attack, the affected individual progressively develops, for example, weakness or numbness and paresthesia on one half of the body, over the course of a few minutes. When the migrainous process is located in the left hemisphere, the patient can often become aphasic as well. The hemiparesis is never complete (i. e., never a hemiplegia) and the patient remains conscious. The headache usually comes after the neurological disturbances, but may also accompany or precede them, and it may be on the same or the opposite side. All manifestations resolve within a few hours, or in one or two days at most. Low-grade CSF pleocytosis and a focus of δ-activity in the EEG can often be demonstrated during an attack. SPECT, too, reveals focal changes. If there is no headache, one speaks of “migraine sans migraine” here as well (just as in ophthalmic migraine without headache). With regard to differential diagnosis, an attack of migraine accompagnée can usually be distinguished from an acute ischemic stroke by the slow development of the neurological deficit and by the accompanying headache. In patients without headache, however, this distinction is not always easy to make, particularly in a first attack, and a thorough diagnostic evaluation is required.

Basilar migraine. In this type of migraine, the pathological process takes place in the structures of the posterior fossa. Basilar migraine mainly affects girls and young women. Its main manifestations are listed in Table 13.5: visual disturbances, symmetrical paresthesiae in the perioral region and the limbs, and impairment of consciousness. The accompanying headache is commonly felt at the back of the head.

Other types. Two special types of migraine deserve mention. Familial hemiplegic migraine, caused by a hereditary disease of ion channels, generally appears in childhood and may be accompanied by cerebellar ataxia. Rarely, an attack of this type of migraine can be followed by a permanent neurological deficit. The rare alternating migraine of childhood begins in the first year of life and is associated with progressive psychomotor retardation. Attacks of hemiparesis on alternating sides of the body last from a quarter of an hour to several hours or even days. There may also be dystonic movements, nystagmus, or tonic crises. Naloxone and flunarizine are effective in treating this disorder.

Cluster Headache

Alternative names for this disorder include “Bing−Horton neuralgia” and “erythroprosopalgia.”

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Painful Syndromes of the Head And Neck 249

Fig. 13.3 Cluster headache: schematic diagram of an attack.

maximum 10–20 minutes after onset

duration of pain: 1–2 hours

1–3 times per day

often at night, often at the same time

Etiology and pathogenesis. The appearance of cluster headache attacks in a circadian rhythm seems to imply that this disorder is due to a functional disturbance of the diencephalon. It shares a number of pathophysiological and biochemical features with migraine. In addition to idiopathic cluster headache, there are also symptomatic forms, caused, e. g., by mass lesions.

Clinical manifestations. These are highly typical and are illustrated in Fig. 13.3.

The headache attacks always occur on the same side of the head. The pain is mainly felt in the temple, eye, and forehead.

The pain reaches its maximum in 10−20 minutes. Each attack lasts from half an hour to two hours; attacks may occur “on schedule” at the same time of day every day (during a cluster), particularly at night.

A number of attacks can occur “in series” in a 24-hour period.

The pain is very intense, often pulsating and throbbing. The patient is agitated and paces around restlessly.

Attacks are usually accompanied by the following objective findings:

Horner syndrome of the ipsilateral eye.

A red, teary eye and periorbital erythema.

A stuffed nose and/or increased nasal secretion.

The attacks appear during periods called “clusters,” lasting weeks or months; the clusters alternate with attackfree intervals lasting months or years.

Besides the episodic form just described, there is also a chronic form of cluster headache, in which the headache attacks occur every day and there are no attack-free intervals. It is not uncommon for typical migraine to be replaced by typical cluster headache (or vice versa) at some point in a patient’s life. Many patients, too, have headaches bearing some of the features of both migraine and cluster headache.

Fig. 13.4 Cluster headache, left-sided attack in a 45-year-old man. The left palpebral fissure is narrowed, and the conjunctiva and periorbital area are erythematous.

Diagnostic evaluation. The acute attacks, because they are brief, are only rarely observed by the physician. The diagnosis thus depends on a precise clinical history (as it does in nearly all types of headache). A physician who has the good fortune to observe the patient during an attack will likely see the characteristic appearance shown in Fig. 13.4.

Treatment. Treatment of an acute attack is difficult.

Triptanes can be given by subcutaneous injection, or the patient can be given pure oxygen to breathe (7 liters per minute for 15 minutes). Medications for the reduction of attack frequency include verapamil and indomethacin, sometimes in combination with a tricyclic antidepressant. A brief course of cortisone treatment is often effective. The chronic form responds to lithium.

Tension-type Headache

This is probably the most common type of headache. It was previously known as “vasomotor headache.” Its clinical features resemble those of postconcussive headache.

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250	13 Painful Syndromes
		Table 13.6 Some rare types of primary headache
		Type	Clinical features	Remarks
		Hemicrania continua	persistent unilateral headache	responds to indomethacin and perhaps to ASA
		“Ice-cream	acute headache, usually temporal, lasting 20−30 seconds,
		headache”	precipitated by a cold stimulus on the palate (such as ice
			cream)

Cough headache precipitated by coughing, abdominal straining, or bending over; an intense, diffuse headache lasting a few seconds

Coital headache sudden onset, lasts minutes or hours, sometimes accompanied by vomiting

usually innocuous, but sometimes due to a pathological process in the posterior cranial fossa

no meningismus (differential diagnosis: subarachnoid hemorrhage!)

Etiology. The cause of tension-type headache is unknown. It is no longer thought to be due to muscle tension. A current hypothesis attributes it to abnormal sensitivity to pain in the trigeminal nuclear complex. This complex, in turn, receives input from other structures in the brain, including the limbic system.

Clinical manifestations. Patients complain of a pressing, aching pain in the head, which is usually diffuse, i. e., there is no particular location at which it is most intense. It is of no more than intermediate severity and does not worsen with physical activity. It is not associated with nausea, photophobia, or phonophobia, and usually does not keep patients from going about their everyday activities.

There are episodic and chronic forms of tension-type headache. Patients with the episodic form suffer from headache on fewer than 15 days per month (180 days per year). The individual episodes of headache may last from 30 minutes to several days. Patients with the chronic form suffer from headache on more than 15 days per month (180 days per year).

Diagnostic evaluation. The history is crucial, as the neurological examination and ancillary tests do not reveal any abnormalities.

Treatment. Life-style readjustment is the recommended first line of therapy: removal of headache-producing substances such as alcohol and nicotine, regular physical exercise, regular sleep, stress reduction, and, if necessary, other changes in the patient’s living situation and mode of living. If medications are needed, tricyclic antidepressants are the agents of choice, followed by beta-blockers or tizanidine.

Rare Varieties of Primary headache

Table 13.6 provides an overview of rarer types of primary headache other than migraine, cluster headache, and tension-type headache.

Symptomatic Headache

Symptomatic headache is due to a structural lesion, infection, or inflammation of intraand/or extracranial tissue. Its direct cause is often a pathological alteration of intracranial pressure, which excites nociceptive nerve endings in the meninges. The ICP may be either too high (particularly because of space-occupying lesions such as

hematomas, tumors, and hydrocephalus) or too low (e. g., in the intracranial hypotension syndrome after a lumbar puncture).

Symptomatic headache, however, is not necessarily due to neurological disease. Pathological conditions of the ears, nose, throat, eyes, teeth, or jaw can also cause head and/or facial pain, which may be quite severe. Even diseases of the cervical spine can, rarely, produce headache (spondylogenic headache).

Table 13.7 provides an overview of the major causes of symptomatic headache. We will describe a few of the causative neurological illnesses and spondylogenic headache, in detail in the following paragraphs.

Occlusions and Dissections of Cranial Vessels

Arterial occlusion only rarely causes headache, but, if it does, the site of the headache may be a clue to the identity of the occluded vessel: occlusion of the internal carotid a. produces temporal headache, while occlusion of the basilar a. produces headache in a ringlike distribution around the head. Very intense pain on one side of the neck and face accompanies acute dissection of the internal carotid a. Vertebral artery dissection produces pain in the ipsilateral occiput and nuchal region. Dissections can arise spontaneously or after trauma to the head or neck.

Intracranial Hemorrhage

Subarachnoid hemorrhage (SAH) due to a ruptured saccular aneurysm at the base of the brain produces an extremely intense, diffuse headache of lightning-like onset (“the worst headache of my life”), possibly accompanied by meningismus and an impairment of consciousness. If the patient is comatose, of course, the headache and meningismus may be masked or absent.

Intracerebral hemorrhage (e. g., spontaneous hemorrhage due to hydrocephalus, into a tumor, or from a ruptured arteriovenous malformation) causes rapidly progressive headache in addition to hemiparesis and progressive impairment of consciousness. A more slowly progressive headache, perhaps accompanied by a fluctuating level of consciousness, is typical of chronic subdural hematoma. Neurological deficits in these patients are surprisingly rare (p. 91).

Cranial Arteritis

Cranial (also called temporal) arteritis is an autoimmune disease of blood vessels that almost exclusively

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		Painful Syndromes of the Head And Neck			251
Table 13.7 Important types of symptomatic headache
Type	Cause	Features	Remarks
Headache of	usually rupture of a saccular	sudden, extremely severe headache, usually dif-	see p. 108
subarachnoid	aneurysm at the base of the brain	fuse, accompanied by vomiting, drowsiness,
hemorrhage		and meningism
Headache due to	brain tumor, chronic subdural hema-	permanent headache of increasing severity;	neuroimaging is
intracranial mass	toma, brain abscess	nausea, bradycardia, papilledema, focal neuro-	essential; see
		logical deficits	p. 94
Headache due to	aqueductal stenosis, intraventricular	manifestations like those of a brain tumor	neuroimaging is
occlusive hydro-	mass, mass in posterior cranial fossa		essential; see
cephalus			p. 84
Headache due to	prior subarachnoid hemorrhage or	diffuse, increasingly severe headache, gait	isotope cisterno-
malresorptive	meningitis, venous sinus thrombosis	ataxia, incontinence	graphy or MRI
hydrocephalus			may be useful
Intracranial	prior lumbar puncture, (rarely) spon-	orthostatic headache that improves or resolves
hypotension	taneous	when the patient lies down; normal neurologi-
		cal examination, CSF not obtainable by lumbar
		puncture (or only with aspiration); elevated CSF
		protein concentration

Pseudotumor cerebri often seen in overweight young women; may be secondary to prior head trauma, anovulatory drugs, steroid withdrawal, tetracycline, etc.

chronic headache without any other detectable cause; often papilledema; CT or MRI reveals slit ventricles; elevated pressure on LP

Headache in	bacterial or viral meningitis	hyperacute in purulent meningitis; very severe
meningitis		headache, meningism, drowsiness, vomiting
Headache in carcino-	primary tumors of various types, e. g.,	chronic, diffuse headache, cranial nerve deficits	can often be di-
matous or leukemic	breast carcinoma	or spinal radicular deficits; LP and CSF cytology	agnosed by MRI
meningitis		are essential
Postinfectious	after recovery from a (viral) infection	diffuse, often intractable headache without
headache		other neurological abnormalities, resembling
		tension headache; mild elevation of CSF cell
		count

Headache in ENT	chronic sinusitis, neoplasia in the
disease	pharyngeal cavity
Headache in eye	e. g., heterophorias, acute glaucoma,
disease	iritis, infectious/inflammatory
	processes in the orbit
Headache due	pulpitis, periodontitis, retained teeth,
to dental conditions	and myofascial pain syndrome due to
	malocclusion

headache or facial pain depending on the site of the disease process; no neurological deficit

usually frontal and temporal headache

severe, acute facial pain or chronic facial pain, depending on cause

affects persons over age 60. Its most prominent, though by no means only, clinical manifestations are in the large and middle-sized extracranial arteries.

Clinical manifestations. The leading symptom is an atypical headache that rapidly worsens over a few days or weeks and then becomes constant, often in the temporal region. The affected arteries (particularly the superficial temporal a.) are tender, tortuous, and swollen (Fig. 13.5). They may occlude by thrombosis, in which case they cease to pulsate. The headache is often accompanied by further manifestations: pain in the shoulder and pelvic girdle muscles, fatigue, subfebrile temperature, weight loss, and nocturnal diaphoresis—a systemic syndrome known as polymyalgia rheumatica. The complication to be most feared is involvement of the ophthalmic a. causing occlusion of the central retinal a. and sudden blindness (Fig. 13.6).

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Fig. 13.5 Temporal arteritis in a 65-year-old man. Note the thickened, painful, no longer pulsating superficial temporal a.

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