Trigeminal neuralgia overview

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Trigeminal neuralgia (TN) is a neuropathic disorder of the trigeminal nerve that causes episodes of intense pain in the eyes, lips, nose, scalp, forehead, and jaw.It is also known as Tic Douloureux or Fothergill's disease (named after John Fothergill). Trigeminal nerve is one of the largest and widely distributed cranial nerve with three branches ophthalmic nerve (V1), maxillary nerve (V2) and mandibular nerve (V3). Ophthalmic and maxillary are purely sensory, while mandibular nerve has both sensory and motor functions. Pain of trigeminal neuralgia affects sensory distributions and typically radiates to maxillary and mandibular divisions in 35% of affected individuals. International Association of Study of Pain (IASP) defined TN as "sudden usually unilateral severe brief stabbing recurrent pain in the distribution of one or more branches of 5th cranial nerve". Similarly, International Headache society (IHS) defined it as "painful unilateral affliction of face characterized by brief electric shock like pain limited to the distribution of one or more divisions of trigeminal nerve". TN is usually characterized by paroxysmic episodes of sudden, severe, shock like pain that lasts for seconds to a few minutes. It is almost always unilateral, but is bilateral in rare cases, and right side is affected more often than the left side. These episodes can occur spontaneously or can be triggered by a mild cutaneous stimuli like chewing, shaving, touching, brushing teeth or even air currents can trigger an episode. The pain tends to occur in cycles with remissions lasting months or even years. These episodic attacks are known to worsen in frequency or severity overtime and can have a significant impact on a person's quality of life, resulting in problems such as weightloss, depression and, in severe cases, suicide. Attacks may provoke patients to grimace, wince or make an aversive head movement, known as tic, hence the term 'tic douloureux". Data is not consistent, but some studies have shown that migraine and hypertension can act as a risk factor for trigeminal neuralgia. According to etiology, IHS has classified TN into two main types classical and symptomatic. Symptomatic TN is further divided into two types typical TN and atypical TN. Although majority of the cases of TN are idiopathic but other causes may include systemic diseases like multiple sclerosis, diabetes mellitus, infectious conditions caused by Mycobacterium leprae, Secondary syphilis, Leptospirosis etc, injuries to trigeminal nerve both centrally and peripherally caused by trauma, ENT pathologies, intracranial tumors, cysts, arteriovenous malformations, tuberculomas and allergic reactions. Pathology of TN is related to demyelination and dystrophy of nerve caused by above mentioned etiological factors. Allergic reaction and its frequent manifestations such as cold, chronic rhinitis, maxillary sinusitis, tonsillitis and chronic inflammation can trigger immune response which in turn results in accumulation of immunoglobulins and histamine in trigeminal nerve region and play role in TN pathogenesis. Diagnosis of TN is mainly clinical, based primarily on history, physical examination and neurological examination, but other causes of facial pain should be ruled out. Common differentials include glossopharyngeal neuralgia, cracked tooth syndrome, cluster headaches and postherpetic neuralgia. Treatment of TN depends upon severity and underlying cause of disease and include medicines mainly anti epileptics, surgery and complimentary approaches. Carbamazepine is the first line drug used for initial management. However, as it is a long term condition, some people overtime may stop responding to medications, or they may experience unpleasant side effects. For those people surgical options can be considered. Psychosocial support also plays an important role in improving quality of life.

Historical Perspective

Historically trigeminal neuralgia has been called "suicide disease" by Harvey Cushing, who did a series of studies involving 123 cases of TN during 1896 and 1912.

• First full and accurate description of trigeminal neuralgia was given by John Fothergill in 1773, but early descriptions of TN can be inferred from the writings of Galen, Aretaeus of Cappadocia and in the 11th century by Avicenna("tortura Orgs").
• The most convincing early description was of a German physician, Johannes Laurentius Bausch. In 1671, he suffered from a lightening like pain in the right face and became unable to speak or eat properly and apparently succumbed to malnutrition.
• In 1677, John Locke, a physician and well known philosopher described the condition in a series of letters to Dr John Mapletoft. His account tells of his unfortunate patient, the Countess of Northumberland, wife of Ambassador to France.
• In 1756, Nicolas Andre conceptualized TN in terms of convulsive behavior and invented the term tic Douloureux to reflect both the pain his patients described as well as the facial spasms he documented.

• In 1773, John Fothergill's description of "a painful affection of the face" was presented to Medical Society to London.

== Famous cases: ==

• Four-time British Prime Minister William Gladstone is believed to have had the disease.
• In 2009, the entrepreneur and author Melissa Seymour was diagnosed with TN and underwent microvascular decompression surgery. Her case was covered by magazines and newspapers which helped to raise public awareness of the illness in Australia. Seymour was subsequently made a Patron of the Trigeminal Neuralgia Association of Australia.
• In 2011, Salman Khan, one of India's most successful film stars, was diagnosed with TN, resulting in tremendous media coverage in the country and abroad. He underwent surgery in the US.
• In 2013 All-Ireland winning Gaelic footballer Christy Toye was diagnosed with the condition.
• Jim Fitzpatrick - Member of Parliament for Poplar and Limehouse - disclosed he suffered from trigeminal neuralgia before undergoing neurosurgery. He has openly discussed his condition at parliamentary meetings and is a prominent figure in the TNA UK charity.
• Andrea Jenkyns - Member of Parliament for Morley and Outwood - diagnosis with TN came to light during her television debate on Prime Minister’s Questions where she struggled to get her words out.
• Jefferson Davis - President of the Confederate States of America.
• Charles Sanders Peirce - American philosopher, scientist and father of pragmatism.
• Gloria Steinem - American feminist, journalist, and social and political activist.
• Anneli van Rooyen, Afrikaans singer-songwriter popular during the 1980s and 1990s, was diagnosed with atypical trigeminal neuralgia in 2004.
• H.R., singer of hardcore punk band Bad Brains.

Epidemiology and Demographics

An estimated one in 15,000 people suffers from trigeminal neuralgia, although numbers may be significantly higher due to frequent misdiagnosis. It usually develops after the age of 40, although there have been cases with patients being as young as three years of age.

Diagnosis

Physical Examination

Signs of trigeminal neuralgia can be seen in males who may deliberately miss an area of their face when shaving, in order to avoid triggering an episode. Although trigeminal neuralgia is not fatal, successive recurrences may be incapacitating, and the fear of provoking an attack may make sufferers reluctant to engage in normal activities.

Treatment

Medical Therapy

There is no cure for trigeminal neuralgia, but most people find relief from medication or sometimes from one of the many so-called complementary or alternative therapies. Atypical trigeminal neuralgia, which involves a more constant and burning pain, is more difficult to treat, both with medications and surgery. During a TN attack, some patients may get quick relief by applying an ice pack or a readily available source of cold temperature to the area of pain.

Surgery

There is no cure for trigeminal neuralgia but most people find relief from one of the five surgical options. Surgery may result in varying degrees of numbness to the patient and lead occasionally to "anesthesia dolorosa," which is numbness with intense pain. However, many people do find dramatic relief with minimal side effects from the various surgeries that are now available.