History
Trigeminal neuralgia (TN) presents as a stabbing unilateral facial pain that is triggered by chewing or similar activities or by touching affected areas on the face. The disorder affects the right side of the face 5 times more frequently than the left.
According to Fromm et al, some patients may present with pretrigeminal neuralgia syndrome for a period of weeks or even years before developing the customary symptoms of trigeminal neuralgia. [15] They complain of an unrelenting sinus pain or toothache lasting for hours, triggered by moving the jaw or drinking fluids. Not surprisingly, they first seek dental care. Some find benefit from baclofen or carbamazepine.
Pain localization
Patients can localize their pain precisely. The pain is not confined exclusively to 1 of the 3 divisions of the trigeminal nerve but more commonly runs along the line dividing either the mandibular and maxillary nerves or the mandibular and ophthalmic portions of the nerve. Of affected patients, 60% complain of lancinating pain shooting from the corner of the mouth to the angle of the jaw; 30% experience jolts of pain from the upper lip or canine teeth to the eye and eyebrow, sparing the orbit itself—this distribution falls between the division of the first and second portions of the nerve. According to Patten, less than 5% of patients experience ophthalmic branch involvement. [16]
Pain quality
The pain quality is characteristically severe, paroxysmal, and lancinating. It commences with a sensation of electrical shocks in an affected area, then quickly crescendos in less than 20 seconds to an excruciating discomfort felt deep in the face, often contorting the patient's expression. The pain then begins to fade within seconds, only to give way to a burning ache lasting seconds to minutes. During attacks, patients may grimace, wince, or make an aversive head movement, as if trying to escape the pain, thus producing an obvious movement, or tic; hence the term "tic douloureux."
Pain chronicity and frequency
This condition is an exception to the rule that nerve injuries typically produce symptoms of constant pain and allodynia. If the pain is particularly frequent, patients may be difficult to examine during the height of an attack. The number of attacks may vary from less than 1 per day, to a 12 or more per hour, up to hundreds per day. Outbursts fully abate between attacks, even when they are severe and frequent.
Pain triggers and zones
A valuable clue to the diagnosis is the triggering of the pain with certain activities. Patients carefully avoid rubbing the face or shaving a trigger area, in contrast to other facial pain syndromes, in which they massage the face or apply heat or ice. Also, many patients try to hold their face still while talking to avoid precipitating an attack. According to Sands, trigger zones, or areas of increased sensitivity, are present in one half of patients and often lie near the nose or mouth. [17] Chewing, talking, smiling, or drinking cold or hot fluids may initiate the pain of trigeminal neuralgia. Touching, shaving, brushing teeth, blowing the nose, or encountering cold air from an open automobile window may also elicit pain.
In contrast to migrainous pain, persons with this condition rarely suffer attacks during sleep, which is another key point in the history.
Concomitant multiple sclerosis
Patients with multiple sclerosis (MS) and trigeminal neuralgia have similar complaints to those with the idiopathic variety, except that these individuals present at a much younger age (often < 40 y). Some present with atypical facial pain, without trigger zones, and without the lancinating brief paroxysms of discomfort. Atypical facial pain is characterized by persistent pain in the facial region and can be further divided into pain with demonstrable organic disease and conditions in which no pathology can be found. As previously noted, trigeminal neuralgia is not unusual in multiple sclerosis, but it is rarely the first manifestation. Typically, it occurs in the advanced stages of multiple sclerosis.
Physical Examination
Physical examination will usually eliminate alternative diagnoses. However, remember that patients report pain following stimulation of a trigger point; thus, some patients may limit their examination for fear of stimulating these points. For example, male patients may present with an area of the face, the trigger zone, that is unshaven and unkempt.
The diagnosis of idiopathic trigeminal neuralgia (TN) is tenable only if no physical findings of fifth nerve dysfunction or are present. Neurologic examination findings are normal, and facial sensation, masseter bulk and strength, and corneal reflexes should be intact. Thus, no sensory loss is found unless checked immediately after a burst of pain; any permanent area of numbness excludes the diagnosis. Loss of the corneal reflex also excludes the diagnosis of idiopathic trigeminal neuralgia, unless a previous trigeminal nerve section procedure has been performed. Any jaw or facial weakness or swallowing difficulties suggests another etiology.
In patients with multiple sclerosis (MS) or a structural lesion and trigeminal neuralgia, sensory loss often is found on examination.
Although hypesthesia or dysesthesia in the face may be observed transitorily in classic trigeminal neuralgia, these symptoms should be considered part of the symptomatic forms. Paramount, however, is that the absence of these findings does not preclude the presence of an underlying cause; that is, it does not exclude a symptomatic form of trigeminal neuralgia.
TN Criteria and Classification
A lack of clear definitions for facial pain has hampered the understanding of trigeminal neuralgia (TN), as the condition has no clear natural history and no long-term follow-up study of the progression of the disorder has been published. Below, 2 classifications for classic trigeminal neuralgia are presented.
International Headache Society criteria
Strict criteria for trigeminal neuralgia as defined by the International Headache Society (IHS) (International Classification of Headache Disorders, 2nd ed) in 2004 are as follows [1] :
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A - Paroxysmal attacks of pain lasting from a fraction of a second to 2 minutes, affecting 1 or more divisions of the trigeminal nerve and fulfilling criteria B and C
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B - Pain has at least 1 of the following characteristics: (1) intense, sharp, superficial or stabbing; or (2) precipitated from trigger areas or by trigger factors
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C - Attacks stereotyped in the individual patient
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D - No clinically evident neurologic deficit
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E - Not attributed to another disorder
The criteria for symptomatic trigeminal neuralgia vary slightly from the strict criteria above and include the following [1] :
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A - Paroxysmal attacks of pain lasting from a fraction of a second to 2 minutes, with or without persistence of aching between paroxysms, affecting 1 or more divisions of the trigeminal nerve and fulfilling criteria B and C
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B - Pain has at least 1 of the following characteristics: (1) intense, sharp, superficial or stabbing; or (2) precipitated from trigger areas or by trigger factors
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C - Attacks stereotyped in the individual patient
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D - A causative lesion, other than vascular compression, demonstrated by special investigations and/or posterior fossa exploration
Proposed classification scheme by Eller et al
In an attempt to rationalize the language of facial pain, in 2005, Eller et al introduced a new classification scheme that divides facial pain into several distinct categories [18] :
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Trigeminal neuralgia type 1 (TN1): The classic form of trigeminal neuralgia in which episodic lancinating pain predominates
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Trigeminal neuralgia type 2 (TN2): The atypical form of trigeminal neuralgia in which more constant pains (aching, throbbing, burning) predominate
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Trigeminal neuropathic pain (TNP): Pain that results from incidental or accidental injury to the trigeminal nerve or the brain pathways of the trigeminal system
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Trigeminal deafferentation pain (TDP): Pain that results from intentional injury to the system in an attempt to treat trigeminal neuralgia (Numbness of the face is a constant part of this syndrome, which has also been referred to as anesthesia dolorosa or one of its variants.)
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Symptomatic trigeminal neuralgia (STN): Trigeminal neuralgia associated with multiple sclerosis (MS)
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Postherpetic neuralgia (PHN): Chronic facial pain that results from an outbreak of herpes zoster (shingles), usually in the ophthalmic division (V1) of the trigeminal nerve on the face and usually in elderly patients
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Geniculate neuralgia (GeN): Pain typified as episodic and lancinating, felt deep in the ear
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Glossopharyngeal neuralgia (GPN): Pain typified in the tonsillar area or throat, usually triggered by talking or swallowing
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Illustration depicting the trigeminal nerve with its 3 main branches
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Microscopic demonstration of demyelination in primary trigeminal neuralgia. A tortuous axon is surrounded by abnormally discontinuous myelin. (Electron microscope; 3300×).
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Magnetic resonance image (MRI) with high resolution on the pons demonstrating the trigeminal nerve root. In this case, the patient with trigeminal neuralgia has undergone gamma-knife therapy, and the left-sided treated nerve (arrow) is enhanced by gadolinium.
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Microvascular decompression (Jannetta procedure) used to treat trigeminal neuralgia. The anteroinferior cerebellar artery and the trigeminal nerve are in direct contact. Courtesy of PT Dang, CH Luxembourg
Tables
Condition |
Male:Female Ratio |
Age of onset, y |
Localization |
Accompanying Symptoms |
Attack Duration |
Cycles |
Provocation |
Trigeminal neuralgia |
1:2 |
>50 |
Unilateral |
None |
Seconds |
Month intervals |
Trigger zones |
Cluster headache |
1:6-9 |
30-40 |
Always unilateral |
Horner syndrome, conjunctival injection, epiphora |
15-180 minutes |
Clusters with weeks to months intervals |
Nocturnal attacks |
Migraine |
1:1 |
10-20 |
Variable |
Photophobia, phonophobia, gastrointestinal symptoms |
4-72 hours |
Days to weeks intervals |
Variable |
Feature |
Trigeminal Neuralgia |
Atypical Facial Pain |
Prevalence |
Rare |
Common |
Main location |
Trigeminal area |
Face, neck, ear |
Pain duration |
Seconds to 2 minutes |
Hours to days |
Character |
Electric jerks, stabbing |
Throbbing, dull |
Pain intensity |
Severe |
Mild to moderate |
Provoking factors |
Light touch, washing, shaving, eating, talking |
Stress, cold |
Associated symptoms |
None |
Sensory abnormalities |
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