Trigeminal Cephalgias and Facial Pain Syndromes Associated with Autonomic Dysfunction

Abstract

Trigeminal autonomic cephalgias (TACs) include a spectrum of primary headache syndromes associated with cranial autonomic dysfunction. Other types of headache and facial pain syndromes can be associated with marked localized facial or ear autonomic changes. We report on a group of patients suffering from episodic migraine with cranial autonomic features, patients with different presentations of the 'red ear syndrome' (RES), cluster headache with prominent lower facial involvement and crossover cases. In our experience crossover between TACs and migraine, RES and cluster headache is not uncommon. We propose that all these conditions belong to the same group and a unifying causative mechanism is proposed.

Pathology of the Trigeminal Nerve

“…10 Less commonly, autonomic symptoms, such as conjunctival injection, lacrimation, eyelid edema, and nasal congestion as features of trigeminal autonomic cephalgias may be seen in association with TN. 12,13 Although the clinical diagnosis of TN is straightforward in most cases, other causes of facial pain, such as dental pain, temporomandibular joint pain, migraine, temporal arteritis, trigeminal deafferentation pain, and atypical facial pain should be eliminated in patients in whom a paroxysmal and a steady pain component are noted. As opposed to TN, trigeminal deafferentation pain is pain that results from intentional injury to the trigeminal nerve system in an attempt to treat TN, particularly after destructive neurosurgical intervention.…”

“…TN, or tic douloureux, is the most frequently occurring condition of all nerve pain disorders. [10][11][12][13] The pain, which comes and goes, feels like bursts of sharp, stabbing, electric shocks in the V1 or V2 distribution of the trigeminal nerve, and can last from a few seconds to a few minutes. Although the onset of TN is most often seen in middle or old age, young adults and children can also be affected.…”

“…A light touch or vibration is the most effective trigger. [10][11][12][13][14][15][16][17] Because of this, common daily activities, such as brushing the teeth, shaving, eating, drinking, or talking, trigger the attacks. Typically, attacks are similar in individual patients and no neurologic deficits are clinically evident.…”

The red ear syndrome

“…It can radiate towards the cheek and mandible [16,20], as well as towards the occiput [4] and the involvement of the V1 area or whole hemicranium has also been described [1,10,12]. …”

Unusual Headaches

“…The redness can occur without pain. The syndrome can be idiopathic or occur in association with migraine, 27,28 thalamic syndrome, atypical glossopharyngeal and trigeminal neuralgia, upper cervical spine pathology (cervical arachnoiditis, cervical spondylosis, traction injury, Chiari malformation, or herpes zoster of the upper cervical roots), and dysfunction of the temporomandibular joint. 29–31 …”