Atypical trigeminal neuralgia

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Atypical trigeminal neuralgia
Other namesType 2 trigeminal neuralgia
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Detailed view of trigeminal nerve, shown in yellow.
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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. [1] 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.

Contents

Signs and symptoms

ATN pain can be described as heavy, aching, stabbing, and burning. Some patients have a constant migraine-like headache. Others may experience intense pain in one or in all three trigeminal nerve branches, affecting teeth, ears, sinuses, cheeks, forehead, upper and lower jaws, behind the eyes, and scalp. In addition, those with ATN may also experience the shocks or stabs found in type 1 TN.[ citation needed ]

Many TN and ATN patients have pain that is "triggered" by light touch on shifting trigger zones. ATN pain tends to worsen with talking, smiling, chewing, or in response to sensations such as a cool breeze. The pain from ATN is often continuous, and periods of remission are rare. Both TN and ATN can be bilateral, though the character of pain is usually different on the two sides at any one time. [2]

Causes

The trigeminal nerve. Gray784.png
The trigeminal nerve.

ATN is usually attributed to inflammation or demyelination, with increased sensitivity of the trigeminal nerve. These effects are believed to be caused by infection, demyelinating diseases, or compression of the trigeminal nerve (by an impinging vein or artery, a tumor, dental trauma, accidents, or arteriovenous malformation) and are often confused with dental problems. Though TN and ATN most often present in the fifth decade, cases have been documented as early as infancy.[ citation needed ]

Risk factors

Both forms of facial neuralgia are relatively rare, with an incidence recently estimated between 12 and 24 new cases per hundred thousand population per year. [3] [4]

ATN often goes undiagnosed or misdiagnosed for extended periods, leading to a great deal of unexplained pain and anxiety. A National Patient Survey conducted by the US Trigeminal Neuralgia Association in the late 1990s indicated that the average facial neuralgia patient may see six different physicians before receiving a first definitive diagnosis. The first practitioner to see facial neuralgia patients is often a dentist who may lack deep training in facial neurology. Thus ATN may be misdiagnosed as Temporomandibular Joint Disorder. [5]

This disorder is regarded by many medical professionals to comprise the most severe form of chronic pain known in medical practice. In some patients, pain may be unresponsive even to opioid drugs at any dose level that leaves the patient conscious. The disorder has thus acquired the unfortunate and possibly inflammatory nickname, "the suicide disease". [6]

Symptoms of ATN may overlap with a pain disorder occurring in teeth called atypical odontalgia (literal meaning "unusual tooth pain"), with aching, burning, or stabs of pain localized to one or more teeth and adjacent jaw. The pain may seem to shift from one tooth to the next, after root canals or extractions. In desperate efforts to alleviate pain, some patients undergo multiple (but unneeded) root canals or extractions, even in the absence of suggestive X-ray evidence of dental abscess.[ citation needed ]

ATN symptoms may also be similar to those of post-herpetic neuralgia, which causes nerve inflammation when the latent herpes zoster virus of a previous case of chicken pox re-emerges in shingles. Fortunately, post-herpetic neuralgia is generally treated with medications that are also the first medications tried for ATN, which reduces the negative impact of misdiagnosis.[ citation needed ]

The subject of atypical trigeminal neuralgia is considered problematic even among experts. The term atypical TN is broad and due to the complexity of the condition, there are considerable issues with defining the condition further. Some medical practitioners no longer make a distinction between facial neuralgia (a nominal condition of inflammation) versus facial neuropathy (direct physical damage to a nerve).[ citation needed ]

Due to the variability and imprecision of their pain symptoms, ATN or atypical odontalgia patients may be misdiagnosed with atypical facial pain (AFP) or "hypochondriasis", both of which are considered problematic by many practitioners. [7] The term "atypical facial pain" is sometimes assigned to pain which crosses the mid-line of the face or otherwise does not conform to expected boundaries of nerve distributions or characteristics of validated medical entities. As such, AFP is seen to comprise a diagnosis by reduction.[ citation needed ]

As noted in material published by the [US] National Pain Foundation: "atypical facial pain is a confusing term and should never be used to describe patients with trigeminal neuralgia or trigeminal neuropathic pain. Strictly speaking, AFP is classified as a "somatiform pain disorder"; this is a psychological diagnosis that should be confirmed by a skilled pain psychologist. Patients with the diagnosis of AFP have no identifiable underlying physical cause for the pain. The pain is usually constant, described as aching or burning, and often affects both sides of the face (this is almost never the case in patients with trigeminal neuralgia). The pain frequently involves areas of the head, face, and neck that are outside the sensory territories that are supplied by the trigeminal nerve. It is important to correctly identify patients with AFP since the treatment for this is strictly medical. Surgical procedures are not indicated for atypical facial pain." [8] [ citation needed ]

The term "hypochondriasis" is closely related to "somatoform pain disorder" and "conversion disorder" in the Diagnostic and Statistical Manual (DSM-IV) of the American Psychiatric Association. As of July 2011, this axis of the DSM-IV is undergoing major revision for the DSM-V, with introduction of a new designation "Complex Somatic Symptom Disorder". However, it remains to be demonstrated that any of these "disorders" can reliably be diagnosed as a medical entity with a discrete and reliable course of therapy. [9] [10] [11] [12]

It is possible that there are triggers or aggravating factors that patients need to learn to recognize to help manage their health. Bright lights, sounds, stress, and poor diet are examples of additional stimuli that can contribute to the condition. The pain can cause nausea, so beyond the obvious need to treat the pain, it is important to be sure to try to get adequate rest and nutrition.

Depression is frequently co-morbid with neuralgia and neuropathic pain of all sorts, as a result of the negative effects that pain has on one's life. Depression and chronic pain may interact, with chronic pain often predisposing patients to depression, and depression operating to sap energy, disrupt sleep and heighten sensitivity and the sense of suffering. Dealing with depression should thus be considered equally important as finding direct relief from the pain. [13]

Diagnosis

Diagnosis for ATN is more difficult and complex than typical TN. However, the diagnosis for ATN can be supported by a positive response to a low dose of tricyclic antidepressant medications (such as amitriptyline or nortriptyline), similar to neuropathic pain diagnoses.[ citation needed ] [14]

Treatment

Medication

Treatment of people believed to have ATN or TN is usually begun with medication. The long-time first drug of choice for facial neuralgia has been carbamazepine, an anti-seizure agent. Due to the significant side-effects and hazards of this drug, others have recently come into common use as alternatives. These include oxcarbazepine, lamotrigine, and gabapentin. A positive patient response to one of these medications might be considered as supporting evidence for the diagnosis, which is otherwise made from medical history and pain presentation. There are no present medical tests to conclusively confirm TN or ATN.[ citation needed ]

If the anti-seizure drugs are found ineffective, one of the tricyclic antidepressant medications, such as amitriptyline or nortriptyline, may be used. The tricyclic antidepressants are known to have dual action against both depression and neuropathic pain. Other drugs which may also be tried, either individually or in combination with an anti-seizure agent, include baclofen, pregabalin, and opioid drugs such as oxycodone or an oxycodone/paracetamol combination.[ citation needed ]

For some people with ATN opioids may represent the only viable medical option which preserves quality of life and personal functioning. Although there is considerable controversy in public policy and practice in this branch of medicine, practice guidelines have long been available and published. [15] [16] [17]

Surgery

If drug treatment is found to be ineffective or causes disabling side effects, one of several neurosurgical procedures may be considered. The available procedures are believed to be less effective with type II (atypical) trigeminal neuralgia than with type I (typical or "classic") TN. Among present procedures, the most effective and long lasting has been found to be microvascular decompression (MVD), which seeks to relieve direct compression of the trigeminal nerve by separating and padding blood vessels in the vicinity of the emergence of this nerve from the brain stem, below the cranium. [18]

Choice of a surgical procedure is made by the doctor and patient in consultation, based on the patient's pain presentation and health and the doctor's medical experience. Some neurosurgeons resist the application of MVD or other surgeries to atypical trigeminal neuralgia, in light of a widespread perception that ATN pain is less responsive to these procedures. However, recent papers suggest that in cases where pain initially presents as type I TN, surgery may be effective even after the pain has evolved into type II. [19]

Related Research Articles

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

Inflammation of the geniculate ganglion of the facial nerve is a late consequence of varicella zoster virus (VZV) known as Ramsay Hunt syndrome (RHS), commonly known as herpes zoster oticus. 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 (hearing noises that are not caused by outside sounds), 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.

<span class="mw-page-title-main">Trigeminal neuralgia</span> Neurological pain disorder

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

<span class="mw-page-title-main">Peripheral neuropathy</span> Nervous system disease affecting nerves beyond the brain and spinal cord

Peripheral neuropathy, often shortened to neuropathy, is a general term describing damage or disease affecting the nerves. Damage to nerves may impair sensation, movement, gland function, and/or organ function depending on which nerves are affected. Neuropathy affecting motor, sensory, or autonomic nerves result in different symptoms. More than one type of nerve may be affected simultaneously. Peripheral neuropathy may be acute or chronic, and may be reversible or permanent.

Postherpetic neuralgia (PHN) is neuropathic pain that occurs due to damage to a peripheral nerve caused by the reactivation of the varicella zoster virus. PHN is defined as pain in a dermatomal distribution that lasts for at least 90 days after an outbreak of herpes zoster. Several types of pain may occur with PHN including continuous burning pain, episodes of severe shooting or electric-like pain, and a heightened sensitivity to gentle touch which would not otherwise cause pain or to painful stimuli. Abnormal sensations and itching may also occur.

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.

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.

Pain disorder is chronic pain experienced by a patient in one or more areas, and is thought to be caused by psychological stress. The pain is often so severe that it disables the patient from proper functioning. Duration may be as short as a few days or as long as many years. The disorder may begin at any age, and occurs more frequently in girls than boys. This disorder often occurs after an accident, during an illness that has caused pain, or after withdrawing from use during drug addiction, which then takes on a 'life' of its own.

A neurectomy, or nerve resection is a neurosurgical procedure in which a peripheral nerve is cut or removed to alleviate neuropathic pain or permanently disable some function of a nerve. The nerve is not intended to grow back. For chronic pain it may be an alternative to a failed nerve decompression when the target nerve has no motor function and numbness is acceptable. Neurectomies have also been used to permanently block autonomic function, and special sensory function not related to pain.

Hypoesthesia or numbness is a common side effect of various medical conditions that manifests as a reduced sense of touch or sensation, or a partial loss of sensitivity to sensory stimuli. In everyday speech this is generally referred to as numbness.

Geniculate ganglionitis or geniculate neuralgia (GN), also called nervus intermedius neuralgia, Ramsay Hunt syndrome, or Hunt's neuralgia, is a rare disorder characterized by severe paroxysmal neuralgic pain deep in the ear, that may spread to the ear canal, outer ear, mastoid or eye regions. GN may also occur in combination with trigeminal or glossopharyngeal neuralgia.

<span class="mw-page-title-main">Multiple sclerosis signs and symptoms</span>

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.

Neuralgia-inducing cavitational osteonecrosis (NICO) is a diagnosis whereby a putative jawbone cavitation causes chronic facial neuralgia; this is different from osteonecrosis of the jaw. In NICO the pain is said to result from the degenerating nerve ("neuralagia"). The condition is probably rare, if it does exist.

<span class="mw-page-title-main">Nerve compression syndrome</span> Human disease

Nerve compression syndrome, or compression neuropathy, or nerve entrapment syndrome, is a medical condition caused by chronic, direct pressure on a peripheral nerve. It is known colloquially as a trapped nerve, though this may also refer to nerve root compression. Its symptoms include pain, tingling, numbness and muscle weakness. The symptoms affect just one particular part of the body, depending on which nerve is affected. The diagnosis is largely clinical and can be confirmed with diagnostic nerve blocks. Occasionally imaging and electrophysiology studies aid in the diagnosis. Timely diagnosis is important as untreated chronic nerve compression may cause permanent damage. A surgical nerve decompression can relieve pressure on the nerve but cannot always reverse the physiological changes that occurred before treatment. Nerve injury by a single episode of physical trauma is in one sense an acute compression neuropathy but is not usually included under this heading, as chronic compression takes a unique pathophysiological course.

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

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<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.

References

  1. ↑ Quail G (August 2005). "Atypical facial pain--a diagnostic challenge". Aust Fam Physician. 34 (8): 641–5. PMID   16113700.
  2. ↑ R.A. Lawhern, Ph.D., "Classification and Treatment of Chronic Face Pain"
  3. ↑ Koopman JS, Dieleman JP, Huygen FJ, de Mos M, Martin CG, Sturkenboom MC (December 2009). "Incidence of facial pain in the general population". Pain. 147 (1–3): 122–7. doi:10.1016/j.pain.2009.08.023. PMID   19783099. S2CID   35327709.
  4. ↑ Hall GC, Carroll D, Parry D, McQuay HJ (May 2006). "Epidemiology and treatment of neuropathic pain: the UK primary care perspective". Pain. 122 (1–2): 156–62. doi:10.1016/j.pain.2006.01.030. PMID   16545908. S2CID   6844949.
  5. ↑ Drangsholt M, Truelove EL (July 2001). "Trigeminal Neuralgia Mistaken as Temporomandibular Disorder". J Evid Base Dent Pract. 1 (1): 41–50. doi:10.1067/med.2001.116846.
  6. ↑ TN "Trigeminal Neuralgia Description / Definition", [US] Facial Pain Association, "TN (Trigeminal Neuralgia) Description / Definition | TNA the Facial Pain Association". Archived from the original on 2011-10-04. Retrieved 2011-08-01.
  7. ↑ Graff-Radford SB, Solberg WK (May 1993). "Is atypical odontalgia a psychological problem?". Oral Surg. Oral Med. Oral Pathol. 75 (5): 579–82. doi:10.1016/0030-4220(93)90228-v. PMID   8155097.
  8. ↑ National Pain Foundation, "Trigeminal Neuralgia — Definitions" Archived 2011-10-03 at the Wayback Machine
  9. ↑ Rief W, Isaac M (March 2007). "Are somatoform disorders 'mental disorders'? A contribution to the current debate". Curr Opin Psychiatry. 20 (2): 143–6. doi:10.1097/YCO.0b013e3280346999. PMID   17278912. S2CID   23584862.
  10. ↑ Voigt K, Nagel A, Meyer B, Langs G, Braukhaus C, Löwe B (May 2010). "Towards positive diagnostic criteria: a systematic review of somatoform disorder diagnoses and suggestions for future classification". J Psychosom Res. 68 (5): 403–14. doi:10.1016/j.jpsychores.2010.01.015. PMID   20403499.
  11. ↑ Kroenke K, Sharpe M, Sykes R (2007). "Revising the classification of somatoform disorders: key questions and preliminary recommendations". Psychosomatics. 48 (4): 277–85. CiteSeerX   10.1.1.631.4736 . doi:10.1176/appi.psy.48.4.277. PMID   17600162. Archived from the original on 2012-07-30.
  12. ↑ Dimsdale J, Sharma N, Sharpe M (2011). "What do physicians think of somatoform disorders?". Psychosomatics. 52 (2): 154–9. doi: 10.1016/j.psym.2010.12.011 . PMID   21397108.
  13. ↑ Daniel K. Hall-Flavin, MD, "Depression (Major Depression)", Mayo Clinic Expert Answers
  14. ↑ "Trigeminal Neuralgia Fact Sheet". ninds.nih.gov. Retrieved 20 June 2022.
  15. ↑ O'Connor AB, Dworkin RH (October 2009). "Treatment of neuropathic pain: an overview of recent guidelines". Am. J. Med. 122 (10 Suppl): S22–32. doi:10.1016/j.amjmed.2009.04.007. PMID   19801049.
  16. ↑ Dworkin RH, O'Connor AB, Audette J, et al. (March 2010). "Recommendations for the pharmacological management of neuropathic pain: an overview and literature update". Mayo Clin. Proc. 85 (3 Suppl): S3–14. doi:10.4065/mcp.2009.0649. PMC   2844007 . PMID   20194146. Archived from the original on 2013-01-28.
  17. ↑ Vadalouca A, Siafaka I, Argyra E, Vrachnou E, Moka E (November 2006). "Therapeutic management of chronic neuropathic pain: an examination of pharmacologic treatment". Ann. N. Y. Acad. Sci. 1088 (1): 164–86. Bibcode:2006NYASA1088..164V. doi:10.1196/annals.1366.016. PMID   17192564. S2CID   21295855.
  18. ↑ Sindou M, Leston J, Decullier E, Chapuis F (December 2007). "Microvascular decompression for primary trigeminal neuralgia: long-term effectiveness and prognostic factors in a series of 362 consecutive patients with clear-cut neurovascular conflicts who underwent pure decompression". J. Neurosurg. 107 (6): 1144–53. doi:10.3171/JNS-07/12/1144. PMID   18077952.
  19. ↑ Tiril Sandell, M.D, Per Kristian Eide, M.D., Ph.D. "Effect of Microvascular Decompression in Trigeminal Neuralgia Patients with or without Constant Pain". Archived from the original on 2011-07-13. Retrieved 2011-08-04.{{cite web}}: CS1 maint: multiple names: authors list (link) Note: registration with the Facial Pain Association is required for access to this article.
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