MS Signs & Symptoms: What is Trigeminal Neuralgia?

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A sharp pain explodes through your face and jaw. You think that perhaps an invisible bolt of lightning just struck without any warning. You’re not sure what happened, but you do know that you don’t want it ever to happen again. You may have experienced your first acute attack of trigeminal neuralgia.

What is trigeminal neuralgia?

Trigeminal neuralgia (TN), also known as the tic douloureux, prosopalgia, the “suicide disease,” and Fothergill’s disease, is a facial pain disorder characterized by brief electric shock-like pains that can occur abruptly, typically on only one side of the face and along one or more of the three branches of the trigeminal nerve. The trigeminal nerve, which is the fifth cranial nerve, is responsible for sensation in the face and motor function controlling the jaw.

The stabbing pain of TN most often affects the right side of the face. Frequency of attacks can range from one per day to 12 or more per hour. Common triggers of TN include chewing, talking, or smiling; drinking cold or hot fluids; touching, shaving, brushing your teeth, or blowing your nose; or sudden contact with cold air.

An individual can often point out exactly where the pain of TN is felt. In the majority of cases, the pain shoots from the corner of the mouth to the back of the jaw. In fewer cases, the pain spreads from the upper lip or teeth up to and around the eye and eyebrow.

There are two patterns of pain in trigeminal neuralgia. The first pattern is episodic and affects more than 50 percent of patients. The second pattern features constant pain, the mechanisms and development of which are not well understood. In episodic cases, the intensity of the pain typically increases from simply being present to an excruciating pain felt deep in the face in less than 20 seconds. Muscle spasms may accompany the pain.

During severe attacks patients may be unable to speak or eat. Between attacks, the fear of reoccurrence may be overwhelming. TN can severely affect quality of life, daily functioning, sleep, mood, well-being, and even employment. Depression is common in people living with TN.

How is trigeminal neuralgia diagnosed?

The diagnosis of TN is based primarily on clinical history. The challenge of diagnosing TN is distinguishing it from other causes of intense facial pain, such as cluster headache.

There are two types of trigeminal neuralgia: classic TN (CTN), also called essential or idiopathic TN, and symptomatic TN (STN), which is secondary to another condition. The pain for each is similar, but the underlying cause is different. The most common causes of STN include intercranial tumors and multiple sclerosis (MS) lesions. Electrophysiological and imaging studies are useful in distinguishing between CTN and STN.

Strict diagnostic criteria for classic TN as defined by the International Headache Society (IHS) are as follows:

  • A – Paroxysmal attacks of pain lasting from a fraction of a second to two minutes, affecting one or more branches of the trigeminal nerve and fulfilling criteria B and C
  • B – Pain has at least one of the following characteristics: (1) intense, sharp, superficial or stabbing; or (2) precipitated from trigger areas or by trigger factors
  • C – Attacks stereotyped in the individual patient
  • D – No clinically evident neurologic deficit
  • E – Not attributed to another disorder

IHS criteria for symptomatic TN vary slightly from the strict criteria and include the following:

  • A – Paroxysmal attacks of pain lasting from a fraction of a second to two minutes, with or without persistence of aching between paroxysms, affecting one or more branches of the trigeminal nerve and fulfilling criteria B and C
  • B – Pain has at least one of the following characteristics: (1) intense, sharp, superficial or stabbing; or (2) precipitated from trigger areas or by trigger factors
  • C – Attacks stereotyped in the individual patient
  • D – A causative lesion, other than vascular compression, demonstrated by special investigations and/or posterior fossa exploration

How is trigeminal neuralgia treated?

Treatment options for TN range from oral medication to surgery. First-line oral therapies are carbamazepine and oxycarbazepine, while second-line therapies include lamotrigine, baclofen, or pimozide. Alternative pharmacologic therapies include gabapentin, pregabalin, topiramate, tocainide, valproate, and misoprostol.

Surgical interventions include Gasserian ganglion procedures, gamma knife surgery, and microvascular decompression. Surgery for TN is either destructive (ablative), where the trigeminal nerve sensory function is intentionally destroyed, or non-destructive, where the trigeminal nerve is decompressed, preserving its normal function.

Gasserian ganglion percutaneous techniques are all destructive and include radiofrequency thermocoagulation (RFT), balloon compression (BC), and percutaneous glycerol rhizolysis (PGR). Ninety percent of patients report pain relief following these procedures. After one year, 68-85 percent of patients are still pain free; after three years, this number is reduced to 54-64 percent; and after five years, only 50 percent of patients are still pain free following RFT. The most common side effects are sensory loss, dysesthesias, anaesthesia dolorosa, and corneal numbness with risk of keratitis.

In gamma knife surgery, a focused beam of radiation is aimed at the trigeminal root in the posterior fossa. The procedure is ablative and the only non-invasive technique used to treat TN. Pain relief may take up to one month to take effect. One year after gamma knife surgery, 69 percent of patients are pain free without additional medication. At three years, 52 percent are still pain free. Side effects include sensory complications that may develop with a delay of up to six months, facial numbness that improves over time, and paresthesias.

Microvascular decompression achieves the most sustained pain relief with 90 percent of patients reporting initial pain relief. Over 80 percent, 75 percent, and 73 percent of patients are still pain free after one year, three years, and five years, respectively. The procedure, however, is highly invasive and involves craniotomy to reach the trigeminal nerve in the posterior fossa. The average mortality rate ranges from 0.2 to 0.5 percent, and up to 4 percent of patients suffer from major problems, such as cerebrospinal fluid leakage, infarct, or hematoma. The most common complications are aseptic meningitis, sensory loss, and hearing loss.

Trigeminal neuralgia and multiple sclerosis

In a survey of 8,590 people diagnosed with MS, 830 respondents (9.7 percent) said they experienced TN. Of the 588 people who reported the year in which TN was diagnosed, TN preceded MS in 15 percent of the cases, and the mean age at diagnosis of TN was 45.3 years. The odds of reporting TN were higher among females and among those with greater disability and longer disease duration. Pharmacologic treatments were used by 85 percent of respondents, while 9.7 percent underwent surgical interventions.

Researchers suggest that further epidemiologic studies, as well as clinical trials to identify effective pharmacologic and nonpharmacologic therapies for TN in MS, are warranted.

See More Helpful Articles:

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Coping With the Fluctuating Face of Pain When Living With MS

MS Signs vs Symptoms: What Is L’Hermittes Sign?

Sources:

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