Clinical features

Although on the face of it, classic trigeminal neuralgia is easily diagnosed, it has long been recognized that there are other forms of trigeminal neuralgia which most frequently have been called atypical trigeminal neuralgia. Neurosurgeons have suggested that these two forms should be called type 1 and type 2 [50]. The atypical forms are more difficult to differentiate from other forms of unilateral pain and may also be associated with some form of trigeminal neuropathy, as suggested by Nurmikko & Eldridge [51].

The major differentiator between classic and atypical trigeminal neuralgia is in the character and its timing. Patients with classic trigeminal neuralgia report just a sharp shooting electric shock-like pain that lasts for a few seconds and may be repeated many times a day. After weeks or months, a period of complete pain remission may result which may last weeks or months. The atypical trigeminal neuralgia patients also have a sharp shooting electric shock but they have a burning, dull, aching after-pain. In some patients this just lasts for several minutes to a few hours and then gradually disappears, leaving a completely pain-free period. Other patients, however, report that this after-pain is persistent and there is no completely pain-free interval. It is this latter type of pain that may not respond as effectively to surgical management. It is postulated that this pain may be a continuation of the original condition. However, it could have a different etiology and therefore represent another disease form. There is currently no evidence of sufficient quality to support these theories. It has been suggested that many patients with trigeminal neuralgia will report a memorable onset and neurosurgeons have gone so far as to suggest that this could be a prognostic factor for improved outcomes.

The following clinical features are consistent with those published by the IHS [11] and IASP [46].

• Site: along one or more divisions of the trigeminal nerve, unilateral only, 3% bilateral

• Radiation: within trigeminal nerve

• Character: shooting, sharp, stabbing, electric shocklike, may be some burning

• Severity: usually severe but can be mild

• Duration: each attack lasts for seconds to maximum 2 minutes but attacks can follow in rapid succession

• Periodicity: paroxysmal with periods of complete pain remission which gradually get shorter

• Provoking factors: daily activities such as eating, talking, washing the face or cleaning the teeth but can be spontaneous

• Relieving factors: avoiding trigger factors, drugs

• Associated factors: stereotype attacks in the individual patient

Trigeminal neuralgia is relatively rare in only the first division and if it is reported as being present only in the first division then other causes should be carefully ascertained such as paroxysmal hemicrania, SUNCT (short-lasting unilateral neuralgiform headaches with conjunctival tearing) or SUNA (short-lasting neuralgiform pain with autonomic symptoms). It is important to note whether the pain is evoked by light touch activities and/or whether it occurs spontaneously as drugs may be more effective in reducing the number of spontaneous attacks. This is of considerable significance, as it is these spontaneous attacks that are more likely to reduce quality of life and make patients live in fear of having an attack at a time when they are away from their usual sources of support. Although the pain can be mild, there are reports of patients committing suicide due to the severity of this pain. Many patients with trigeminal neuralgia report the severity of their pain being worse during the day and only a third of patients will report experiencing pain at night resulting in awakening. There is evidence to suggest that trigem-inal neuralgia patients will develop depression which will lift once the pain is successfully managed [52].

On examination, many patients will exhibit no neurologic deficit. However, this may be very subtle and may change with time. Sensory testing is essential as this will potentially differentiate between symptomatic and idiopathic trigeminal neuralgia. Patients will exhibit trigger areas from which pain is initiated and this could be classified as a form of allodynia.

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