NORD gratefully acknowledges Jeffrey Cohen, MD, PhD, Director, Epilepsy Program & Clinical Neurophysiology, Beth Israel Medical, New York NY, for assistance in the preparation of this report.
Trigeminal neuralgia (TN), also known as tic douloureux, is a disorder of the fifth cranial nerve (trigeminal nerve). The disorder is sometimes broken down into type 1 and type 2. TN type 1 (TN1) is characterized by attacks of intense, stabbing pain affecting the mouth, cheek, nose, and/or other areas on one side of the face. TN type 2 (TN2) is characterized by less intense pain, but a constant dull aching or burning pain. Both types of pain can occur in the same individual, even at the same time. In some cases, the pain can be excruciating and incapacitating. If untreated, TN can have a profound effect on a person’s quality of life. In most cases, TN1 develops due to a blood vessel pressing against the trigeminal nerve, but sometimes no underlying cause can be identified (idiopathic). TN2 can be idiopathic, due to compression of the trigeminal nerve, or can occur due to a known underlying cause such as a tumor or multiple sclerosis. It is not known why one person gets symptoms of TN1 versus TN2; it may be due to the number of vessels (e.g. arteries, veins) or the degree of compression. TN can usually be managed through medications, surgery or injections.
There is no consensus or agreed upon classification system for TN. TN1 is also known as classical trigeminal neuralgia. TN2 was once known as atypical or symptomatic TN. However the term "atypical" trigeminal neuralgia has been inconsistently used for individuals who do not have TN1 and remains a vague, undefined term. Consequently, many researchers and patients have advocated eliminating the term "atypical TN", which remains a "wastebasket" diagnosis that serves no useful purpose and is often a disservice to patients. Symptomatic TN is often reserved for cases that develop because of multiple sclerosis. The term trigeminal neuropathic facial pain may be used for pain that results from unintentional injury to the trigeminal nerve, which can result from a variety of conditions including facial trauma, oral surgery, ear, nose and throat surgery, or stroke.
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