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Trigeminal Neuralgia Clinical Presentation

  • Author: Manish K Singh, MD; Chief Editor: Robert A Egan, MD  more...
 
Updated: Oct 22, 2015
 

History

Trigeminal neuralgia (TN) presents as a stabbing unilateral facial pain that is triggered by chewing or similar activities or by touching affected areas on the face. The disorder affects the right side of the face 5 times more frequently than the left.

According to Fromm et al, some patients may present with pretrigeminal neuralgia syndrome for a period of weeks or even years before developing the customary symptoms of trigeminal neuralgia.[15] They complain of an unrelenting sinus pain or toothache lasting for hours, triggered by moving the jaw or drinking fluids. Not surprisingly, they first seek dental care. Some find benefit from baclofen or carbamazepine.

Pain localization

Patients can localize their pain precisely. The pain is not confined exclusively to 1 of the 3 divisions of the trigeminal nerve but more commonly runs along the line dividing either the mandibular and maxillary nerves or the mandibular and ophthalmic portions of the nerve. Of affected patients, 60% complain of lancinating pain shooting from the corner of the mouth to the angle of the jaw; 30% experience jolts of pain from the upper lip or canine teeth to the eye and eyebrow, sparing the orbit itself—this distribution falls between the division of the first and second portions of the nerve. According to Patten, less than 5% of patients experience ophthalmic branch involvement.[16]

Pain quality

The pain quality is characteristically severe, paroxysmal, and lancinating. It commences with a sensation of electrical shocks in an affected area, then quickly crescendos in less than 20 seconds to an excruciating discomfort felt deep in the face, often contorting the patient's expression. The pain then begins to fade within seconds, only to give way to a burning ache lasting seconds to minutes. During attacks, patients may grimace, wince, or make an aversive head movement, as if trying to escape the pain, thus producing an obvious movement, or tic; hence the term "tic douloureux."

Pain chronicity and frequency

This condition is an exception to the rule that nerve injuries typically produce symptoms of constant pain and allodynia. If the pain is particularly frequent, patients may be difficult to examine during the height of an attack. The number of attacks may vary from less than 1 per day, to a 12 or more per hour, up to hundreds per day. Outbursts fully abate between attacks, even when they are severe and frequent.

Pain triggers and zones

A valuable clue to the diagnosis is the triggering of the pain with certain activities. Patients carefully avoid rubbing the face or shaving a trigger area, in contrast to other facial pain syndromes, in which they massage the face or apply heat or ice. Also, many patients try to hold their face still while talking to avoid precipitating an attack. According to Sands, trigger zones, or areas of increased sensitivity, are present in one half of patients and often lie near the nose or mouth.[17] Chewing, talking, smiling, or drinking cold or hot fluids may initiate the pain of trigeminal neuralgia. Touching, shaving, brushing teeth, blowing the nose, or encountering cold air from an open automobile window may also elicit pain.

In contrast to migrainous pain, persons with this condition rarely suffer attacks during sleep, which is another key point in the history.

Concomitant multiple sclerosis

Patients with multiple sclerosis (MS) and trigeminal neuralgia have similar complaints to those with the idiopathic variety, except that these individuals present at a much younger age (often < 40 y). Some present with atypical facial pain, without trigger zones, and without the lancinating brief paroxysms of discomfort. Atypical facial pain is characterized by persistent pain in the facial region and can be further divided into pain with demonstrable organic disease and conditions in which no pathology can be found. As previously noted, trigeminal neuralgia is not unusual in multiple sclerosis, but it is rarely the first manifestation. Typically, it occurs in the advanced stages of multiple sclerosis.

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Physical Examination

Physical examination will usually eliminate alternative diagnoses. However, remember that patients report pain following stimulation of a trigger point; thus, some patients may limit their examination for fear of stimulating these points. For example, male patients may present with an area of the face, the trigger zone, that is unshaven and unkempt.

The diagnosis of idiopathic trigeminal neuralgia (TN) is tenable only if no physical findings of fifth nerve dysfunction or are present. Neurologic examination findings are normal, and facial sensation, masseter bulk and strength, and corneal reflexes should be intact. Thus, no sensory loss is found unless checked immediately after a burst of pain; any permanent area of numbness excludes the diagnosis. Loss of the corneal reflex also excludes the diagnosis of idiopathic trigeminal neuralgia, unless a previous trigeminal nerve section procedure has been performed. Any jaw or facial weakness or swallowing difficulties suggests another etiology.

In patients with multiple sclerosis (MS) or a structural lesion and trigeminal neuralgia, sensory loss often is found on examination.

Although hypesthesia or dysesthesia in the face may be observed transitorily in classic trigeminal neuralgia, these symptoms should be considered part of the symptomatic forms. Paramount, however, is that the absence of these findings does not preclude the presence of an underlying cause; that is, it does not exclude a symptomatic form of trigeminal neuralgia.

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TN Criteria and Classification

A lack of clear definitions for facial pain has hampered the understanding of trigeminal neuralgia (TN), as the condition has no clear natural history and no long-term follow-up study of the progression of the disorder has been published. Below, 2 classifications for classic trigeminal neuralgia are presented.

International Headache Society criteria

Strict criteria for trigeminal neuralgia as defined by the International Headache Society (IHS) (International Classification of Headache Disorders, 2nd ed) in 2004 are as follows[1] :

  • A - Paroxysmal attacks of pain lasting from a fraction of a second to 2 minutes, affecting 1 or more divisions of the trigeminal nerve and fulfilling criteria B and C
  • B - Pain has at least 1 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

The criteria for symptomatic trigeminal neuralgia vary slightly from the strict criteria above and include the following[1] :

  • A - Paroxysmal attacks of pain lasting from a fraction of a second to 2 minutes, with or without persistence of aching between paroxysms, affecting 1 or more divisions of the trigeminal nerve and fulfilling criteria B and C
  • B - Pain has at least 1 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

Proposed classification scheme by Eller et al

In an attempt to rationalize the language of facial pain, in 2005, Eller et al introduced a new classification scheme that divides facial pain into several distinct categories[18] :

  • Trigeminal neuralgia type 1 (TN1): The classic form of trigeminal neuralgia in which episodic lancinating pain predominates
  • Trigeminal neuralgia type 2 (TN2): The atypical form of trigeminal neuralgia in which more constant pains (aching, throbbing, burning) predominate
  • Trigeminal neuropathic pain (TNP): Pain that results from incidental or accidental injury to the trigeminal nerve or the brain pathways of the trigeminal system
  • Trigeminal deafferentation pain (TDP): Pain that results from intentional injury to the system in an attempt to treat trigeminal neuralgia (Numbness of the face is a constant part of this syndrome, which has also been referred to as anesthesia dolorosa or one of its variants.)
  • Symptomatic trigeminal neuralgia (STN): Trigeminal neuralgia associated with multiple sclerosis (MS)
  • Postherpetic neuralgia (PHN): Chronic facial pain that results from an outbreak of herpes zoster (shingles), usually in the ophthalmic division (V1) of the trigeminal nerve on the face and usually in elderly patients
  • Geniculate neuralgia (GeN): Pain typified as episodic and lancinating, felt deep in the ear
  • Glossopharyngeal neuralgia (GPN): Pain typified in the tonsillar area or throat, usually triggered by talking or swallowing
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Contributor Information and Disclosures
Author

Manish K Singh, MD Assistant Professor, Department of Neurology, Teaching Faculty for Pain Management and Neurology Residency Program, Hahnemann University Hospital, Drexel College of Medicine; Medical Director, Neurology and Pain Management, Jersey Institute of Neuroscience

Manish K Singh, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pain Medicine, American Headache Society, American Association of Physicians of Indian Origin, American Medical Association, American Society of Regional Anesthesia and Pain Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Gordon H Campbell, MSN FNP-BC, Neuroscience Nurse Practitioner, Neurology Service, Portland Veterans Affairs Medical Center; Primary Faculty, Clinical Instructor, and Guest Lecturer, Family Nursing Department, Oregon Health Sciences University School of Nursing

Gordon H Campbell, MSN is a member of the following medical societies: American Academy of Neurology

Disclosure: Nothing to disclose.

Helmi L Lutsep, MD Professor and Vice Chair, Department of Neurology, Oregon Health and Science University School of Medicine; Associate Director, OHSU Stroke Center

Helmi L Lutsep, MD is a member of the following medical societies: American Academy of Neurology, American Stroke Association

Disclosure: Medscape Neurology Editorial Advisory Board for: Stroke Adjudication Committee, CREST2.

Siddharth Gautam, MBBS Resident Physician, Jersey Neuroscience Institute

Disclosure: Nothing to disclose.

Chief Editor

Robert A Egan, MD Director of Neuro-Ophthalmology and Stroke Service, St Helena Hospital

Robert A Egan, MD is a member of the following medical societies: American Academy of Neurology, American Heart Association, North American Neuro-Ophthalmology Society, Oregon Medical Association

Disclosure: Received honoraria from Biogen Idec for speaking and teaching; Received honoraria from Teva for speaking and teaching.

Acknowledgements

Jane W Chan, MD Professor of Neurology/Neuro-ophthalmology, Department of Medicine, Division of Neurology, University of Nevada School of Medicine

Jane W Chan, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Ophthalmology, American Medical Association, North American Neuro-Ophthalmology Society, and Phi Beta Kappa

Disclosure: Nothing to disclose.

James R Couch, MD, PhD, FACP Professor of Neurology, University of Oklahoma Health Sciences Center

Disclosure: Nothing to disclose.

Theodore J Gaeta, DO, MPH, FACEP Clinical Associate Professor, Department of Emergency Medicine, Weill Cornell Medical College; Vice Chairman and Program Director of Emergency Medicine Residency Program, Department of Emergency Medicine, New York Methodist Hospital; Academic Chair, Adjunct Professor, Department of Emergency Medicine, St George's University School of Medicine

Theodore J Gaeta, DO, MPH, FACEP is a member of the following medical societies: Alliance for Clinical Education, American College of Emergency Physicians, Clerkship Directors in Emergency Medicine, Council of Emergency Medicine Residency Directors, New York Academy of Medicine, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

J Stephen Huff, MD Associate Professor of Emergency Medicine and Neurology, Department of Emergency Medicine, University of Virginia School of Medicine

J Stephen Huff, MD is a member of the following medical societies: American Academy of Emergency Medicine, American Academy of Neurology, American College of Emergency Physicians, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Simon K Law, MD, PharmD Associate Professor of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine

Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology

Disclosure: Nothing to disclose.

Andrew W Lawton, MD Medical Director of Neuro-Ophthalmology Service, Section of Ophthalmology, Baptist Eye Center, Baptist Health Medical Center

Andrew W Lawton, MD is a member of the following medical societies: American Academy of Ophthalmology, Arkansas Medical Society, and Southern Medical Association

Disclosure: Nothing to disclose.

Marc E Lenaerts, MD, FAHS Staff Neurologist, Mercy Medical Group; Associate Clinical Professor of Neurology, Department of Neurology, University of California, Davis, School of Medicine

Marc E Lenaerts, MD, FAHS is a member of the following medical societies: American Academy of Neurology, American Headache Society, and International Headache Society

Disclosure: Nothing to disclose.

Jorge E Mendizabal, MD Consulting Staff, Corpus Christi Neurology

Jorge E Mendizabal, MD is a member of the following medical societies: American Academy of Neurology, American Headache Society, National Stroke Association, and Stroke Council of the American Heart Association

Disclosure: Nothing to disclose.

Hampton Roy Sr, MD Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Tom Scaletta, MD Chair, Department of Emergency Medicine, Edward Hospital; Past-President, American Academy of Emergency Medicine

Tom Scaletta, MD is a member of the following medical societies: American Academy of Emergency Medicine

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Brian R Younge, MD Professor of Ophthalmology, Mayo Clinic School of Medicine

Brian R Younge, MD is a member of the following medical societies: American Medical Association, American Ophthalmological Society, and North American Neuro-Ophthalmology Society

Disclosure: Nothing to disclose.

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Illustration depicting the trigeminal nerve with its 3 main branches
Microscopic demonstration of demyelination in primary trigeminal neuralgia. A tortuous axon is surrounded by abnormally discontinuous myelin. (Electron microscope; 3300×).
Magnetic resonance image (MRI) with high resolution on the pons demonstrating the trigeminal nerve root. In this case, the patient with trigeminal neuralgia has undergone gamma-knife therapy, and the left-sided treated nerve (arrow) is enhanced by gadolinium.
Microvascular decompression (Jannetta procedure) used to treat trigeminal neuralgia. The anteroinferior cerebellar artery and the trigeminal nerve are in direct contact. Courtesy of PT Dang, CH Luxembourg
Table 1. Characteristic Features of 3 Common Craniofacial Pains
Condition Male:Female Ratio Age of onset, y Localization Accompanying Symptoms Attack Duration Cycles Provocation
Trigeminal neuralgia 1:2 >50 Unilateral None Seconds Month intervals Trigger zones
Cluster headache 31:1 30-40 Always unilateral Horner syndrome, conjunctival injection, epiphora 15-180 minutes Clusters with weeks to months intervals Nocturnal attacks
Migraine 1:1 10-20 Variable Photophobia, phonophobia, gastrointestinal symptoms 4-72 hours Days to weeks intervals Variable
Table 2. Distinguishing Features Between Trigeminal Neuralgia and Atypical Facial Pain
Feature Trigeminal Neuralgia Atypical Facial Pain
Prevalence Rare Common
Main location Trigeminal area Face, neck, ear
Pain duration Seconds to 2 minutes Hours to days
Character Electric jerks, stabbing Throbbing, dull
Pain intensity Severe Mild to moderate
Provoking factors Light touch, washing, shaving, eating, talking Stress, cold
Associated symptoms None Sensory abnormalities
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