Trigeminal neuralgia

Last updated
Trigeminal neuralgia
Other namesTic douloureux, [1] prosopalgia, [2] Fothergill's disease, [3] suicide disease [4]
Gray778.png
The trigeminal nerve and its three major divisions (shown in yellow): the ophthalmic nerve (V1), the maxillary nerve (V2), and the mandibular nerve (V3)
Specialty Neurology
Symptoms Typical: episodes of severe, sudden, shock-like pain in one side of the face that lasts for seconds to minutes [1]
Atypical: constant burning pain [1]
Complications Depression [5]
Usual onset> 50 years old [1]
TypesTypical and atypical trigeminal neuralgia [1]
CausesBelieved to be due to problems with myelin of trigeminal nerve [1] [6]
Diagnostic method Based on symptoms [1]
Differential diagnosis Postherpetic neuralgia [1]
TreatmentMedication, surgery [1]
Medication Carbamazepine, oxcarbazepine [6]
Prognosis 80% improve with initial treatment [6]
Frequency1 in 8,000 people per year [1]

Trigeminal neuralgia (TN or TGN), also called Fothergill disease, tic douloureux, trifacial neuralgia, or suicide disease, is a long-term pain disorder that affects the trigeminal nerve, [7] [1] the nerve responsible for sensation in the face and motor functions such as biting and chewing. It is a form of neuropathic pain. [8] There are two main types: typical and atypical trigeminal neuralgia. [1] The typical form results in episodes of severe, sudden, shock-like pain in one side of the face that lasts for seconds to a few minutes. [1] Groups of these episodes can occur over a few hours. [1] The atypical form results in a constant burning pain that is less severe. [1] Episodes may be triggered by any touch to the face. [1] Both forms may occur in the same person. [1] It is regarded as one of the most painful disorders known to medicine, and often results in depression and suicide. [5]

Contents

The exact cause is unknown, but believed to involve loss of the myelin of the trigeminal nerve. [1] [6] This might occur due to compression from a blood vessel as the nerve exits the brain stem, multiple sclerosis, stroke, or trauma. [1] Less common causes include a tumor or arteriovenous malformation. [1] It is a type of nerve pain. [1] Diagnosis is typically based on the symptoms, after ruling out other possible causes such as postherpetic neuralgia. [8] [1]

Treatment includes medication or surgery. [1] The anticonvulsant carbamazepine or oxcarbazepine is usually the initial treatment, and is effective in about 90% of people. [8] Side effects are frequently experienced that necessitate drug withdrawal in as many as 23% of patients. [8] Other options include lamotrigine, baclofen, gabapentin, amitriptyline and pimozide. [6] [1] Opioids are not usually effective in the typical form. [1] In those who do not improve or become resistant to other measures, a number of types of surgery may be tried. [6]

It is estimated that trigeminal neuralgia affects around 0.03% to 0.3% of people around the world with a female over-representation around a 3:1 ratio between women and men. [9] It usually begins in people over 50 years old, but can occur at any age. [1] The condition was first described in detail in 1773 by John Fothergill. [10]

Signs and symptoms

Trigeminal neuralgia Trigeminal Neuralgia.png
Trigeminal neuralgia

This disorder is characterized by episodes of severe facial pain along the trigeminal nerve divisions. The trigeminal nerve is a paired cranial nerve that has three major branches: the ophthalmic nerve (V1), the maxillary nerve (V2), and the mandibular nerve (V3). One, two, or all three branches of the nerve may be affected. Trigeminal neuralgia most commonly involves the middle branch (the maxillary nerve or V2) and lower branch (mandibular nerve or V3) of the trigeminal nerve. [11]

An individual attack usually lasts from a few seconds to several minutes or hours, but these can repeat for hours with very short intervals between attacks. In other instances, only 4–10 attacks are experienced daily. The episodes of intense pain may occur paroxysmally. To describe the pain sensation, people often describe a trigger area on the face so sensitive that touching or even air currents can trigger an episode; however, in many people, the pain is generated spontaneously without any apparent stimulation. It affects lifestyle as it can be triggered by common activities such as eating, talking, shaving and brushing teeth. The wind, chewing, and talking can aggravate the condition in many patients. The attacks are said, by those affected, to feel like stabbing electric shocks, burning, sharp, pressing, crushing, exploding or shooting pain that becomes intractable. [8]

The pain also tends to occur in cycles with remissions lasting months or even years. Pain attacks are known to worsen in frequency or severity over time, in some people. Pain may migrate to other branches over time but in some people remains very stable. [12]

Bilateral (occurring on both sides) trigeminal neuralgia is very rare except for trigeminal neuralgia caused by multiple sclerosis (MS). This normally indicates problems with both trigeminal nerves, since one nerve serves the left side of the face and the other serves the right side. Occasional reports of bilateral trigeminal neuralgia reflect successive episodes of unilateral (only one side) pain switching the side of the face rather than pain occurring simultaneously on both sides. [13]

Rapid spreading of the pain, bilateral involvement or simultaneous participation with other major nerve trunks (such as Painful Tic Convulsif of nerves V & VII or occurrence of symptoms in the V and IX nerves) may suggest a systemic cause. Systemic causes could include multiple sclerosis or expanding cranial tumors. [14]

The severity of the pain makes it difficult to wash the face, shave, and perform good oral hygiene. The pain has a significant impact on activities of daily living especially as those affected live in fear of when they are going to get their next attack of pain and how severe it will be. It can lead to severe depression and anxiety. [15]

However, not all people will have the symptoms described above; there are variants of TN, one of which is atypical trigeminal neuralgia ("trigeminal neuralgia, type 2" or trigeminal neuralgia with concomitant pain), [16] based on a recent classification of facial pain. [17] In these instances there is also a more prolonged lower severity background pain that can be present for over 50% of the time and is described more as a burning or prickling, rather than a shock.

Trigeminal pain can also occur after an attack of herpes zoster. Post-herpetic neuralgia has the same manifestations as in other parts of the body. Herpes zoster oticus typically presents with inability to move many facial muscles, pain in the ear, taste loss on the front of the tongue, dry eyes and mouth, and a vesicular rash. Less than 1% of varicella zoster infections involve the facial nerve and result in this occurring. [18]

Trigeminal deafferentation pain (TDP), also termed anesthesia dolorosa, or colloquially as phantom face pain, is from unintentional damage to a trigeminal nerve following attempts to fix a nerve problem surgically. This pain is usually constant with a burning sensation and numbness. TDP is very difficult to treat as further surgeries are usually ineffective and possibly detrimental to the person. [19]

Causes

The trigeminal nerve is a mixed cranial nerve responsible for sensory data such as tactition (pressure), thermoception (temperature), and nociception (pain) originating from the face above the jawline; it is also responsible for the motor function of the muscles of mastication, the muscles involved in chewing but not facial expression. [20]

Several theories exist to explain the possible causes of this pain syndrome. It was once believed that the nerve was compressed in the opening from the inside to the outside of the skull; but leading research indicates that it is an enlarged or lengthened blood vessel – most commonly the superior cerebellar artery – compressing or throbbing against the microvasculature of the trigeminal nerve near its connection with the pons. [21] Such a compression can injure the nerve's protective myelin sheath and cause erratic and hyperactive functioning of the nerve. This can lead to pain attacks at the slightest stimulation of any area served by the nerve as well as hinder the nerve's ability to shut off the pain signals after the stimulation ends. This type of injury may rarely be caused by an aneurysm (an outpouching of a blood vessel); by an AVM (arteriovenous malformation); [22] by a tumor; such as an arachnoid cyst or meningioma in the cerebellopontine angle; [23] or by a traumatic event, such as a car accident. [24]

Short-term peripheral compression is often painless. [5] Persistent compression results in local demyelination with no loss of axon potential continuity. Chronic nerve entrapment results in demyelination primarily, with progressive axonal degeneration subsequently. [5] It is, "therefore widely accepted that trigeminal neuralgia is associated with demyelination of axons in the Gasserian ganglion, the dorsal root, or both." [25] It has been suggested that this compression may be related to an aberrant branch of the superior cerebellar artery that lies on the trigeminal nerve. [25] Further causes, besides an aneurysm, multiple sclerosis or cerebellopontine angle tumor, include: a posterior fossa tumor, any other expanding lesion or even brainstem diseases from strokes. [25]

Trigeminal neuralgia is found in 3–4% of people with multiple sclerosis, according to data from seven studies. [26] [27] It has been theorized that this is due to damage to the spinal trigeminal complex. [28] Trigeminal pain has a similar presentation in patients with and without MS. [29]

Postherpetic neuralgia, which occurs after shingles, may cause similar symptoms if the trigeminal nerve is damaged, called Ramsay Hunt syndrome type 2.

When there is no apparent structural cause, the syndrome is called idiopathic.

Diagnosis

Trigeminal neuralgia is diagnosed via the result of neurological and physical tests, as well as the individual's medical history. [1] Magnetic resonance angiography can be used to detect vascular compression of the trigeminal nerve and refer patients to surgery. [30]

As with many conditions without clear physical or laboratory diagnosis, TN is often misdiagnosed, and other conditions are also frequently misdiagnosed as TN. [31] [32] A person with TN may see three or four clinicians before a firm diagnosis is made. [31]

Temporomandibular disorder (TMD) can present similarly to TN, and differentiating between these conditions can be difficult. [33] Even suspected TN patients who experience brief attacks of sharp pain have had their symptoms resolve after being treated for TMD. [34] TMD pain can also be triggered by movements of the tongue or facial muscles, so TN must be differentiated from masticatory pain by differentiating between the clinical characteristics of deep somatic pain and neuropathic pain. Masticatory pain will not be arrested by a conventional mandibular local anesthetic block. [14] One quick test a dentist might perform is a conventional inferior dental local anesthetic block. If the pain is in the treated branch, the block will not arrest masticatory pain but will alleviate TN pain. [35]

Management

There is evidence that points towards the need to quickly treat and diagnose TN. It is thought that the longer a patient has TN, the harder it may be to reverse the neural pathways associated with the pain.[ citation needed ]

Medical

Surgical

Microvascular decompression provides freedom from pain in about 75% of patients presenting with drug-resistant trigeminal neuralgia. [41] [42] [43] While there may be pain relief after surgery, there is also a risk of adverse effects, such as facial numbness. Percutaneous radiofrequency thermorhizotomy may also be effective [44] as may stereotactic radiosurgery; however the effectiveness decreases with time. [45]

Surgical procedures can be separated into non-destructive and destructive:

Non-destructive

  • Microvascular decompression – this involves a small incision behind the ear and some bone removal from the area. An incision through the meninges is made to expose the nerve. Any vascular compressions of the nerve are carefully moved and a sponge-like pad is placed between the compression and nerve, stopping unwanted pulsation and allowing myelin sheath healing.[ citation needed ]

Destructive

All destructive procedures will cause facial numbness, post relief, as well as pain relief. [42]

  • Percutaneous techniques which all involve a needle or catheter entering the face up to the origin where the nerve splits into three divisions and then damaging this area, purposely, to produce numbness but also stop pain signals. These techniques are proven effective [44] especially in those where other interventions have failed or in those who are medically unfit for surgery such as the elderly.
  • Balloon compression – inflation of a balloon at this point causing damage and stopping pain signals.
  • Glycerol injection – deposition of a corrosive liquid called glycerol at this point causes damage to the nerve to hinder pain signals.
  • Radiofrequency thermocoagulation rhizotomy – application of a heated needle to damage the nerve at this point.
  • Stereotactic radiosurgery is a form of radiation therapy that focuses high-power energy on a small area of the body [46]

Support

Psychological and social support has found to play a key role in the management of chronic illnesses and chronic pain conditions, such as trigeminal neuralgia. Chronic pain can cause constant frustration to an individual as well as to those around them. [47]

History

Edvard Munch's The Scream has been used as a symbol of facial pain generally, and also specifically of trigeminal neuralgia. The Scream.jpg
Edvard Munch's The Scream has been used as a symbol of facial pain generally, and also specifically of trigeminal neuralgia.

Trigeminal neuralgia was first described by physician John Fothergill and treated surgically by John Murray Carnochan, both of whom were graduates of the University of Edinburgh Medical School. Historically TN has been called "suicide disease" due to studies by the pioneering forefather in neurosurgery Harvey Cushing involving 123 cases of TN during 1896 and 1912. In those studies it produced intense pain, higher rates of suicidal ideation in patients with severe migraines, and links to higher rates of depression, anxiety, and sleep disorders. [4] [50] [51]

Society and culture

Some individuals of note with TN include:

See also

Related Research Articles

<span class="mw-page-title-main">Ramsay Hunt syndrome type 2</span> Presentation of shingles in the geniculate ganglion

Ramsay Hunt syndrome type 2, commonly referred to simply as Ramsay Hunt syndrome (RHS) and also known as herpes zoster oticus, is inflammation of the geniculate ganglion of the facial nerve as a late consequence of varicella zoster virus (VZV). In regard to the frequency, less than 1% of varicella zoster infections involve the facial nerve and result in RHS. It is traditionally defined as a triad of ipsilateral facial paralysis, otalgia, and vesicles close to the ear and auditory canal. Due to its proximity to the vestibulocochlear nerve, the virus can spread and cause hearing loss, tinnitus, and vertigo. It is common for diagnoses to be overlooked or delayed, which can raise the likelihood of long-term consequences. It is more complicated than Bell's palsy. Therapy aims to shorten its overall length, while also providing pain relief and averting any consequences.

<span class="mw-page-title-main">Cluster headache</span> Neurological disorder

Cluster headache (CH) is a neurological disorder characterized by recurrent severe headaches on one side of the head, typically around the eye(s). There is often accompanying eye watering, nasal congestion, or swelling around the eye on the affected side. These symptoms typically last 15 minutes to 3 hours. Attacks often occur in clusters which typically last for weeks or months and occasionally more than a year.

Pudendal nerve entrapment (PNE), also known as Alcock canal syndrome, is an uncommon source of chronic pain in which the pudendal nerve is entrapped or compressed in Alcock's canal. There are several different types of PNE based on the site of entrapment anatomically. Pain is positional and is worsened by sitting. Other symptoms include genital numbness, fecal incontinence and urinary incontinence.

<span class="mw-page-title-main">Vestibular schwannoma</span> Medical condition

A vestibular schwannoma (VS), also called acoustic neuroma, is a benign tumor that develops on the vestibulocochlear nerve that passes from the inner ear to the brain. The tumor originates when Schwann cells that form the insulating myelin sheath on the nerve malfunction. Normally, Schwann cells function beneficially to protect the nerves which transmit balance and sound information to the brain. However, sometimes a mutation in the tumor suppressor gene, NF2, located on chromosome 22, results in abnormal production of the cell protein named Merlin, and Schwann cells multiply to form a tumor. The tumor originates mostly on the vestibular division of the nerve rather than the cochlear division, but hearing as well as balance will be affected as the tumor enlarges.

<span class="mw-page-title-main">Radiosurgery</span> Surgical Specialty

Radiosurgery is surgery using radiation, that is, the destruction of precisely selected areas of tissue using ionizing radiation rather than excision with a blade. Like other forms of radiation therapy, it is usually used to treat cancer. Radiosurgery was originally defined by the Swedish neurosurgeon Lars Leksell as "a single high dose fraction of radiation, stereotactically directed to an intracranial region of interest".

Neuropathic pain is pain caused by a lesion or disease of the somatosensory nervous system. Neuropathic pain may be associated with abnormal sensations called dysesthesia or pain from normally non-painful stimuli (allodynia). It may have continuous and/or episodic (paroxysmal) components. The latter resemble stabbings or electric shocks. Common qualities include burning or coldness, "pins and needles" sensations, numbness and itching.

<span class="mw-page-title-main">Stereotactic surgery</span> Medical procedure

Stereotactic surgery is a minimally invasive form of surgical intervention that makes use of a three-dimensional coordinate system to locate small targets inside the body and to perform on them some action such as ablation, biopsy, lesion, injection, stimulation, implantation, radiosurgery (SRS), etc.

Neuralgia is pain in the distribution of a nerve or nerves, as in intercostal neuralgia, trigeminal neuralgia, and glossopharyngeal neuralgia.

Occipital neuralgia (ON) is a painful condition affecting the posterior head in the distributions of the greater occipital nerve (GON), lesser occipital nerve (LON), third occipital nerve (TON), or a combination of the three. It is paroxysmal, lasting from seconds to minutes, and often consists of lancinating pain that directly results from the pathology of one of these nerves. It is paramount that physicians understand the differential diagnosis for this condition and specific diagnostic criteria. There are multiple treatment modalities, several of which have well-established efficacy in treating this condition.

Hemifacial spasm (HFS) is a rare neuromuscular disease characterized by irregular, involuntary muscle contractions (spasms) on one side (hemi-) of the face (-facial). The facial muscles are controlled by the facial nerve, which originates at the brainstem and exits the skull below the ear where it separates into five main branches.

Microvascular decompression (MVD), also known as the Jannetta procedure, is a neurosurgical procedure used to treat trigeminal neuralgia, a pain syndrome characterized by severe episodes of intense facial pain, and hemifacial spasm. The procedure is also used experimentally to treat tinnitus and vertigo caused by vascular compression on the vestibulocochlear nerve.

<span class="mw-page-title-main">Neuritis</span> Inflammation of a nerve or generally any part of the nervous system

Neuritis, from the Greek νεῦρον), is inflammation of a nerve or the general inflammation of the peripheral nervous system. Inflammation, and frequently concomitant demyelination, cause impaired transmission of neural signals and leads to aberrant nerve function. Neuritis is often conflated with neuropathy, a broad term describing any disease process which affects the peripheral nervous system. However, neuropathies may be due to either inflammatory or non-inflammatory causes, and the term encompasses any form of damage, degeneration, or dysfunction, while neuritis refers specifically to the inflammatory process.

<span class="mw-page-title-main">Superior cerebellar artery</span> Artery of the head

The superior cerebellar artery (SCA) is an artery of the head. It arises near the end of the basilar artery. It is a branch of the basilar artery. It supplies parts of the cerebellum, the midbrain, and other nearby structures. It is the cause of trigeminal neuralgia in some patients.

<span class="mw-page-title-main">Eagle syndrome</span> Medical condition

Eagle syndrome is an uncommon condition commonly characterized but not limited to sudden, sharp nerve-like pain in the jaw bone and joint, back of the throat, and base of the tongue, triggered by swallowing, moving the jaw, or turning the neck. First described by American otorhinolaryngologist Watt Weems Eagle in 1937, the condition is caused by an elongated or misshapen styloid process and/or calcification of the stylohyoid ligament, either of which interferes with the functioning of neighboring regions in the body, such as the glossopharyngeal nerve.

<span class="mw-page-title-main">Signs and symptoms of multiple sclerosis</span> Neurological signs and symptoms

Multiple sclerosis can cause a variety of symptoms: changes in sensation (hypoesthesia), muscle weakness, abnormal muscle spasms, or difficulty moving; difficulties with coordination and balance; problems in speech (dysarthria) or swallowing (dysphagia), visual problems, fatigue and acute or chronic pain syndromes, bladder and bowel difficulties, cognitive impairment, or emotional symptomatology. The main clinical measure in progression of the disability and severity of the symptoms is the Expanded Disability Status Scale or EDSS.

<span class="mw-page-title-main">Atypical trigeminal neuralgia</span> Medical condition

Atypical trigeminal neuralgia (ATN), or type 2 trigeminal neuralgia, is a form of trigeminal neuralgia, a disorder of the fifth cranial nerve. This form of nerve pain is difficult to diagnose, as it is rare and the symptoms overlap with several other disorders. The symptoms can occur in addition to having migraine headache, or can be mistaken for migraine alone, or dental problems such as temporomandibular joint disorder or musculoskeletal issues. ATN can have a wide range of symptoms and the pain can fluctuate in intensity from mild aching to a crushing or burning sensation, and also to the extreme pain experienced with the more common trigeminal neuralgia.

Atypical facial pain (AFP) is a type of chronic facial pain which does not fulfill any other diagnosis. There is no consensus as to a globally accepted definition, and there is even controversy as to whether the term should be continued to be used. Both the International Headache Society (IHS) and the International Association for the Study of Pain (IASP) have adopted the term persistent idiopathic facial pain (PIFP) to replace AFP. In the 2nd Edition of the International Classification of Headache Disorders (ICHD-2), PIFP is defined as "persistent facial pain that does not have the characteristics of the cranial neuralgias ... and is not attributed to another disorder." However, the term AFP continues to be used by the World Health Organization's 10th revision of the International Statistical Classification of Diseases and Related Health Problems and remains in general use by clinicians to refer to chronic facial pain that does not meet any diagnostic criteria and does not respond to most treatments.

<span class="mw-page-title-main">Orofacial pain</span> Medical condition

Orofacial pain is a general term covering any pain which is felt in the mouth, jaws and the face. Orofacial pain is a common symptom, and there are many causes.

<span class="mw-page-title-main">Cervicocranial syndrome</span> Medical condition

Cervicocranial syndrome or is a neurological illness. It is a combination of symptoms that are caused by an abnormality in the neck. The bones of the neck that are affected are cervical vertebrae (C1-C7). This syndrome can be identified by confirming cervical bone shifts, collapsed cervical bones or misalignment of the cervical bone leading to improper functioning of cervical spinal nerves. Cervicocranial syndrome is either congenital or acquired. Some examples of diseases that could result in cervicocranial syndrome are Chiari disease, Klippel-Feil malformation osteoarthritis, and trauma. Treatment options include neck braces, pain medication and surgery. The quality of life for individuals suffering from CCJ syndrome can improve through surgery.

<span class="mw-page-title-main">Konstantin Slavin</span>

Konstantin Slavin is a Professor and Head of the Department of Stereotactic and functional neurosurgery at the University of Illinois College of Medicine. He is a former president of the American Society for Stereotactic and functional neurosurgery and current vice-president of the World Society for Stereotactic and Functional Neurosurgery. His specialties include Aneurysm, Brain surgery, Brain Tumor, Cerebrovascular Disorders, Craniotomy, Dystonia, Essential Tremor, Facial Nerve Pain, Facial Pain, Glioblastoma, Headache disorders, Laminectomy, Lower back pain, Movement Disorders, Multiple Sclerosis, Neck Pain, Neurosurgery, Neurosurgical Procedures, Pain, Parkinson Disease, Spinal Cord Injuries, and Stroke.

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