Fig. 13.6 Atrophic optic disc in a 79-year-old woman with temporal arteritis. Note the abnormal disc pallor; the patient is blind in this eye.

Table 13.8 Diagnostic criteria for spondylogenic headache

Characteristics of pain

radiating from occipital to frontal

usually unilateral

coming in attacks, or

permanent pain of variable intensity

not throbbing

moderately severe

Cervical spine

prior history of trauma to the head or cervical spine

or prior whiplash injury

or episodes of torticollis (wry neck),

perhaps accompanied by arm pain

clinical or radiological evidence of cervical spine pathology

Precipitating and alleviating factors

pain induced by movement (or manipulation) of the cervical spine

or by keeping the head in a particular position for a longer than usual time

or by local pressure on the nape of the neck or the occiput

pain temporarily alleviated by infiltration of the greater occipital n. or the C2 nerve root with local anaesthetic

Accompanying symptoms

dizziness

nausea

blurred vision

phonoand photophobia

dysphagia

Diagnostic evaluation. The erythrocyte sedimentation rate is nearly always markedly elevated. The diagnosis is confirmed by temporal artery biopsy: histopathological examination reveals giant cell arteritis. A biopsy should be performed in all suspected cases, because there is no other way to establish the diagnosis with the certainty required before starting long-term treatment with corticosteroids. These often need to be given continuously for a year or more.

Spondylogenic Headache (“Migraine Cervicale”)

Our experience suggests that this condition is overdiagnosed. The appropriate diagnostic criteria to be used are summarized in Table 13.8.

Dangerous Types of Headache

All patients who consult a physician because of headache are in pain and therefore deserve our full attention and respect. More than 90 % of them, however, will turn out not to have a serious medical problem. One of the important tasks facing the physician is to be on guard for those few, unusual cases of headache that are, in fact, due to a dangerous underlying condition. The main alarm signals are the following:

headache of an unusual nature in a patient who never had headaches before;

headache arising at an advanced age;

headache of sudden (lightning-like) onset;

headache that always occurs in precisely the same location (except cluster headache or trigeminal neuralgia, both of which, by definition, always occur in the same place);

progressively severe headache (crescendo headache);

continuous headache;

headache accompanied by:

personality change,

impairment of consciousness,

epileptic seizures;

neurological deficits revealed on physical examination.

If any of the above applies, further investigation is needed, usually with an imaging study.

Painful Syndromes of the Face

Facial pain is often due to a lesion of a sensory nerve in the face, most commonly the trigeminal n. It typically presents with very brief, but very intense attacks of pain (“classic” or “genuine” neuralgia in the face). There are also a variety of other kinds of facial pain with other pathogenetic mechanisms, e. g., a structural anomaly of the jaw. The pain may resemble neuralgia in these other conditions as well; thus, patients with any kind of facial pain always require careful evaluation to establish the differential diagnosis.

“Genuine” Neuralgias in the Face

Typical manifestations of “genuine” neuralgia in the face are the following:

pain is located in the face or the mucous membranes of the head,

usually comes in brief attacks lasting a few seconds to a few minutes at most,

is usually very intense;

is described as electrical, knifelike, cutting, stabbing, or lightninglike;

arises either spontaneously or on provocation by touch or other mechanical or thermal stimuli;

Fig. 13.8 Trigeminal neuralgia: schematic diagram.

duration of pain: 5–10 seconds

maximum in 1 second

up to 100 times per day

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Treatment. About 80 % of patients initially respond to correctly dosed treatment with carbamazepine or gabapentin. The medication must be taken every day and the dose must be steadily increased until the pain is relieved.

!The pharmacological treatment of trigeminal neuralgia often fails because of underdosing or irregular consumption of the prescribed drug.

If pharmacological treatment does not eliminate the pain despite a high dose of medication (in some patients, just below the threshold for intolerable side effects, which varies greatly from one patient to the next), neurosurgical treatment is indicated. The available, effective procedures include open microvascular decompression of the trigeminal n. (requires craniotomy) and percutaneous techniques such as selective radiofrequency coagulation of the Gasserian ganglion, balloon compression of the ganglion, and glycerol injection into Meckel’s cave.

Auriculotemporal Neuralgia

In this disorder, the pain is located in the temple and in front of the ear. It is usually a sequela of disease of the ipsilateral parotid gland, appearing a few days or months after the parotid condition resolves.

The attacks of pain can be provoked by chewing or by chemical stimuli, particularly sour (acidic) food. The pain is of a burning quality. It is often accompanied by erythema and increased sweating in the preauricular area.

The differential diagnosis of this condition includes temporomandibular joint syndrome.

Nasociliary Neuralgia

The attacks of pain are located in the nose and on the inner canthus of the eye. There may be a continuous, background pain in addition to the typical, lightninglike, shooting pain. In this condition, as in the other neuralgias of the face, the pain is provoked by chewing or by touch—here, by touching the eye. The attacks are often accompanied by redness of the eye, swelling of the nasal mucosa, and lacrimation. They can often be aborted by the application of a 5 % cocaine solution in-

Glossopharyngeal Neuralgia

This condition is usually seen in the elderly. Its typical manifestations are lightninglike pain in the base of the tongue, the hypopharynx, and the tonsillar fossa, radiating toward the ear. The pain can be provoked by swallowing (especially of cold liquids), speaking, or sticking out the tongue. The painful attacks are, on rare occasions, accompanied by syncope. The pharmacological treatment of this disorder is like that of trigeminal neuralgia. If surgical treatment is required, resection of the glossopharyngeal n. and the upper root of the vagus n. has a good chance of success.

Rarer Types of Neuralgia

The neuralgias of the geniculate ganglion, the superior laryngeal n., and the auricular branch of the vagus n. are all very rare. The existence of occipital neuralgia is still debated.

Other Diseases Causing

Facial Pain

Temporomandibular Joint Syndrome

This condition has many other names, including myofacial syndrome and Costen syndrome. Our experience suggests that it is overdiagnosed.

Pathogenesis. This condition is thought to be due to abnormal mechanical stress on the jaw joint caused by malocclusion, e. g., after tooth extraction, or by local changes in the joint itself. This, in turn, causes the muscles of mastication to be activated in nonphysiological ways, which leads to pain in the muscles, so that their pattern of activation is distorted even further. A vicious circle arises in which pain produces more pain.

Clinical manifestations. Patients typically suffer from more or less continuous preauricular pain, which is described as dull or neuralgia-like. The pain can be brought on, or made worse, by chewing.

Diagnostic evaluation. The jaw joint is tender to pressure on one or both sides. CT or MRI occasionally demonstrates an abnormality of the joint.

Table 13.9 Differential diagnosis of headache and facial pain

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