The face is richly supplied with pain-sensitive structures the teeth, gums, sinuses, temporomandibular joints, jaw and eyes-disease of which causes facial pain. Facial pain is also caused by some specific neurological conditions; these are mentioned below.
Migrainous neuralgia (cluster headache)
This condition, which is distinct from migraine despite its name, causes recurrent bouts of excruciating pain that wake the patient at night and are centred around one eye. It affects adults in the third and fourth decades and is more common in men. Alcohol sometimes precipitates an attack. The pain lasts for several hours. Vomiting occurs. The face and nostril feel congested. A transient ipsilateral Horner’s syndrome is common during the attack. Despite the pain there are no serious sequelae. Treatment of the attack with analgesics is unhelpful. Prophylactic drugs for migraine are of little value. Lithium carbonate sometimes has a dramatic effect in preventing attacks: the drug level should be monitored carefully. Atypical facial pain
Facial pain for which no cause can be found is seen in the elderly, mainly in women. It is believed to be a somatic equivalent of depression. Tricyclic antidepressants are sometimes helpful.
Facial pain occurs in usual variants of migraine and in giant-cell arteritis (see below). Giant-cell arteritis (cranial arteritis,temporal arteritis) (see p. 406) This important syndrome is a granulomatous arteritis of unknown aetiology occurring chiefly in those over the age of 0 years and affecting in particular the extradural arteries. Other forms of arteritis, e.g. SLE (see p. 400) and polyarteritis nodosa (see p. 406) can present with similar features but with a more generalized arteritis affecting intracranial arteries and vasa nervorum of the cranialnerves. Giant-cell arteritis is closely related to polymyalgia and these can occur in the same patient.
Headache is almost invariable. It is felt over the inflamed superficial, temporal or occipital arteries. Touching the kin over the inflamed vessel (e.g. combing the hair) causes pain. The arterial pulsation is soon lost and the artery becomes hard, tortuous and thickened. The skinover the vessels may be red. Rarely, gangrenous patches appear in the scalp.
Pain in the face, jaw and mouth occurs and is caused by inflammation of the facial, maxillary and lingual branches of the external carotid artery. Pain is characteristically worse on eating (jaw claudication). Opening the mouth and protruding the tongue is difficult. A painful, ischaemic tongue occasionally occurs.
Visual loss due to inflammation and occlusion of the ciliary and/or central retinal artery occurs in 25% of untreated cases. The patient complains of sudden uniocular visual loss, either partial or complete, which is painless. Amaurosis fugax (see p.906) may precede total visual loss, which is usually permanent.
When the ciliary vessels are affected, the optic disc becomes swollen and pale (see p. 882). The retinal branch vessels are usually normal. If the central retinal artery is occluded, there is sudden unilateral blindness, pallor of the disc and retinal ischaemia.Systemic features
Generalized limb pains, proximal limb girdle pain and tenderness, without joint effusion, i.e. polymyalgia rheumatica (see p. 406), occurs in half the cases. Weight loss, sweating and malaise also occur.
Brain stem ischaemia, cortical blindness, ischaemic neuropathy of peripheral nerves, cranial nerve lesions, and involvement of the aorta, coronary, renal and mesenteric arteries are sometimes seen.
The ESR is greatly elevated, 60-100 rom hour-I being common, although very rarely the ESR is normal. Plasma u2-globulins are raised and the albumin is occasionally reduced. Normochromic normocytic anaemia occurs. The diagnosis is usually confirmed by biopsy of a superficial temporal artery. A 1 em (or greater) segment should be excised because the characteristic granulomatous changes within the walls (lymphocytes, plasma cells, multinucleate giant cells, destruction of the internal elastic lamina) may be patchy.
The diagnosis should be established without delaybecause of the risk of blindness. High doses of steroids (prednisolone, initially 60-100 mg daily) should be started immediately in a patient with typical features even before the biopsy. The dose is reduced as the ESR falls. A characteristic of the condition is that the headache subsides within hours of the first large dose of steroid. It is usually possible to stop steroid treatment after some months to several years.