Trigeminal neuropathy is a condition characterized by a specific pattern and location of facial pain. Clinical features involve unilateral and paroxysmal attacks of very intense pain in one or more areas innervated by the trigeminal nerve. The pathogenesis is frequently unclear and the vast majority of cases remain idiopathic. A thorough patient history and a physical examination are crucial steps of the workup, whereas imaging studies and neurophysiological testing are often necessary to solidify the diagnosis.
Presentation
Trigeminal neuropathy is an important neuropathic disorder that can have a considerable impact on the quality of life [1] [2] [3]. This condition is often regarded as idiopathic, although vascular compression of the trigeminal nerve seems to be the most probable cause in the majority of patients [3] [4]. Tumors and demyelinating diseases (for example multiple sclerosis) have been described as potential etiologies as well [3] [4]. The main symptom of this condition, namely facial pain, stems from the irritation of the trigeminal nerve and its branches - ophthalmic (V1), maxillary (V2), and mandibular (V3) nerves [1] [4] [5] [6]. The pain is characterized as stabbing, superficial, electric shock-like, sharp, or intense pain, and regarded as a very debilitating condition [1] [4] [6]. It is virtually always unilateral, but isolated reports show that bilateral occurrence has been encountered in about 30% of multiple sclerosis patients affected by trigeminal neuropathy [2]. The onset is paroxysmal, lasting seconds to minutes, and is often precipitated by a seemingly benign event, such as a light touch, on the affected side of the face [1] [4] [5]. One or more divisions of the trigeminal nerve are involved while the pain never spreads to parts of the head and neck which are not innervated by the trigeminal nerve [1] [2] [4] [5].
Entire Body System
- Pain
pain (painful temporomandibular disorder). [ncbi.nlm.nih.gov]
- Amyloidosis
There was no evidence of systemic amyloidosis or underlying inflammatory or neoplastic disorders. Her blink reflex and thin-slice magnetic resonance imaging demonstrated that the right trigeminal nerve was involved. [ncbi.nlm.nih.gov]
Respiratoric
- Rhinorrhea
Short-lasting, unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) present with pain of longer duration (2-3 min) and associated prominent autonomic symptoms (eg, lacrimation, rhinorrhea). [20] Temporomandibular joint [emedicine.medscape.com]
Gastrointestinal
- Nausea
Adverse effects include nausea, drowsiness, and confusion. Alcohol injection This numbs the affected areas of the face and provides temporary pain relief. The doctor injects alcohol into the painful part of the face. [medicalnewstoday.com]
If medication does not relieve your symptoms, or if it causes undesirable side effects such as nausea, dizziness, confusion, or severe drowsiness, we may recommend surgery. [mountsinai.org]
Unfortunately, due to the progressive nature of TN, medications may lose their effectiveness over time and people may find the side effects (memory loss, confusion, drowsiness, dizziness or nausea) of higher dosages intolerable. [tuftsmedicalcenter.org]
[…] pain: Short, sporadic, and extreme pain that may only last a few seconds or a few minutes, and that may also include a burning feeling T2 or TN2 pain: Constant pain that is similar to T1 pain but is ongoing with no relief Sensitivity to light and sound Nausea [arizonapain.com]
[…] does not control the pain and you need immediate relief When your pain prevents eating and drinking and places you at risk for malnutrition or dehydration When you experience profound side effects of your medication such as severe drowsiness, sedation, nausea [emedicinehealth.com]
- Vomiting
[…] sporadic, and extreme pain that may only last a few seconds or a few minutes, and that may also include a burning feeling T2 or TN2 pain: Constant pain that is similar to T1 pain but is ongoing with no relief Sensitivity to light and sound Nausea and vomiting [arizonapain.com]
[…] control the pain and you need immediate relief When your pain prevents eating and drinking and places you at risk for malnutrition or dehydration When you experience profound side effects of your medication such as severe drowsiness, sedation, nausea, or vomiting [emedicinehealth.com]
These include: tiredness and sleepiness dizziness (lightheadedness) difficulty concentrating and memory problems confusion feeling unsteady on your feet feeling sick and vomiting double vision a reduced number of infection-fighting white blood cells [nhs.uk]
(vi) Xerostomia Percutaneous trigeminal rhizotomy (i) Recurrence of pain (ii) Dysesthesia (troublesome numbness) (iii) Loss of corneal reflex Oxcarbazepine (i) Dizziness (ii) Fatigue (iii) Nausea (iv) Vomiting (v) Headache (vi) Acne (vii) Dry mouth [hindawi.com]
Eyes
- Conjunctival Injection
injection and tearing (SUNCT) # Symptomatic (secondary) trigeminal neuralgia* (trigeminal neuralgia) Tic Douloureux* (trigeminal neuralgia) Trigeminal neuralgia* * another SOP applies - the SOP has the same name unless otherwise specified Clinical onset [clik.dva.gov.au]
Short-lasting, unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) present with pain of longer duration (2-3 min) and associated prominent autonomic symptoms (eg, lacrimation, rhinorrhea). [20] Temporomandibular joint [emedicine.medscape.com]
Face, Head & Neck
- Facial Spasm
In some cases, it is accompanied by a brief facial spasm or tic. [1] Clinically Relevant Anatomy The Trigeminal nerve, the fifth cranial nerve, is the nerve responsible for sensation in the face, and control of motor functions such as biting and chewing [physio-pedia.com]
This should include: What your pain feels like (for example, is it sharp, shooting, aching, burning, or other), where exactly is your pain is located (lower jaw, cheek, eye/forehead), is it accompanied by other symptoms (headache, numbness, facial spasms [aans.org]
Trigeminal neuralgia was first described at the end of the first century and was later given the name “tic douloureux” because of the distinctive facial spasms that often accompany the attacks. [aafp.org]
Neurologic
- Neuralgia
Trigeminal neuropathy and neuralgia are most commonly caused by vascular compression. Only 9 cases of trigeminal neuropathy and neuralgia due to pontine infarction have been reported in medical literature. [ncbi.nlm.nih.gov]
Treating trigeminal neuralgia and atypical trigeminal neuralgia Atypical trigeminal neuralgia shares several symptoms of trigeminal neuralgia. [upmc.com]
Conditions not covered by these SOPs Classical trigeminal neuralgia* (trigeminal neuralgia) Cluster headache* Glossopharyngeal neuralgia # Herpetic neuralgia # - That is pain at the time of the infection (Herpes Zoster) Migraine* Neuropathy in other cranial [clik.dva.gov.au]
This procedure may be done to treat trigeminal neuralgia, hemifacial spasms, or glossopharyngeal neuralgia. If the trigeminal nerve is compressed, an area on the back of the head is shaved, and an incision is made. [msdmanuals.com]
- Trigeminal Neuralgia
Treating trigeminal neuralgia and atypical trigeminal neuralgia Atypical trigeminal neuralgia shares several symptoms of trigeminal neuralgia. [upmc.com]
(SUNCT) # Symptomatic (secondary) trigeminal neuralgia* (trigeminal neuralgia) Tic Douloureux* (trigeminal neuralgia) Trigeminal neuralgia* * another SOP applies - the SOP has the same name unless otherwise specified Clinical onset The clinical onset [clik.dva.gov.au]
A common cause of trigeminal neuralgia is An abnormally positioned artery that compresses the trigeminal nerve near where it exits the brain Occasionally in younger people, trigeminal neuralgia results from nerve damage due to multiple sclerosis. [msdmanuals.com]
neuralgia but is caused by a space-occupying lesion. [ncbi.nlm.nih.gov]
- Confusion
Adverse effects include nausea, drowsiness, and confusion. Alcohol injection This numbs the affected areas of the face and provides temporary pain relief. The doctor injects alcohol into the painful part of the face. [medicalnewstoday.com]
If medication does not relieve your symptoms, or if it causes undesirable side effects such as nausea, dizziness, confusion, or severe drowsiness, we may recommend surgery. [mountsinai.org]
Atypical Trigeminal Neuralgia Symptoms Symptoms of atypical trigeminal neuralgia can be confusing. They may mimic other conditions such as sinus infections or dental problems. [neurosurgeonsofnewjersey.com]
Unfortunately, due to the progressive nature of TN, medications may lose their effectiveness over time and people may find the side effects (memory loss, confusion, drowsiness, dizziness or nausea) of higher dosages intolerable. [tuftsmedicalcenter.org]
Stimulating a tooth or teeth worsens the pain so it is often confused with a toothache. Teeth appear normal, however. The cause is unknown, most commonly affects women in their mid-40's and is often associated with depression. [tnnme.com]
- Cranial Nerve Involvement
Group 1 involved patients such as those described above with oculosympathetic paresis and trigeminal and potentially other cranial nerve involvement. [medlink.com]
Lesions of the facial and trigeminal nerve in leprosy. An evaluation of 43 cases. Int J Oral Surg 1982; 11: 14–20. Kumar S, Alexander M and Gnanamuthu C. Cranial nerve involvement in patients with leprous neuropathy. Neurol India 2006; 54: 283–285. [oasisdiscussions.ca]
Frequency of involvement of the different oculomotor nerves – The third and sixth cranial nerves are the most commonly affected. [scielo.br]
Severe dysphagia in lower cranial nerve involvement as the initial symptom of Wegener's granulomatosis. Journal of the Neurological Sciences. 2007; 263 (1-2):187–190. [ PubMed ] [ Google Scholar ] 112. Pamuk ON, Doğutan H, Pamuk GE, Cakir N. [ncbi.nlm.nih.gov]
- Seizure
When one anti-seizure drug loses effectiveness, another anti-seizure medication may be tried. More than one anti-seizure medication may be necessary to control pain in an affected individual. [rarediseases.org]
Other diagnostic tools include a short course of anti-seizure medication or tricyclic antidepressants, a positive response to which may indicate trigeminal neuralgia. [arizonapain.com]
This medication is commonly used for seizures but can often be effective in reducing pain from trigeminal neuralgia. Other medications include baclofen, phenytoin, oxacarbazepine, and gabapentin. [texasspineandneurosurgerycenter.com]
Medications used to treat trigeminal neuralgia are those used for many other nerve pain syndromes-drugs originally designed to treat seizures. [emedicinehealth.com]
These are normally used to prevent seizures, but they can also reduce or block the pain signals sent to the brain. They do this by calming the nerve impulses. [medicalnewstoday.com]
Workup
The diagnostic workup of patients in whom trigeminal neuropathy is suspected should start with a comprehensive patient history during which the physician must cover the duration of symptoms and the characteristics of pain, including its pattern of occurrence as well as severity and location [4] [6] [7]. Together with the obtained information, the physical examination can provide important clues to the etiology of the disease. Reduced sensitivity of the trigeminal branches, either partial or total (hypoesthesia and anesthesia, respectively), during the neurological examination is a crucial finding in this patient population and should prompt a more detailed investigation [5]. Imaging studies, mainly magnetic resonance imaging (MRI), are highly useful in recognizing an ongoing process in the brain that could induce trigeminal neuropathy [1] [2] [4] [6]. MRI is particularly useful for detecting vascular malformations that compress the trigeminal nerve and currently serves as a gold-standard for the examination of craniofacial pain [1]. Some authors recommend X-rays and computed tomography (CT) scans to exclude other, more common, pathologies [4] [5]. In addition, neurophysiological trigeminal reflex testing has shown to be equally effective compared to MRI in identifying trigeminal neuropathy [2] [7].
Treatment
Worsening of primary headache disorders by interferon treatment has been previously reported. Our case suggests that treatment with interferon beta may also exacerbate symptomatic trigeminal neuralgia in multiple sclerosis. [ncbi.nlm.nih.gov]
Dade Lunsford discusses the benefits of glycerol rhizotomy treatment. [upmc.com]
Prognosis
The lesions underlying neoplastic trigeminal neuropathy can be located at any point along the trajectory of the trigeminal nerve, and their prognosis is very poor. [Indexed for MEDLINE] Free full text [ncbi.nlm.nih.gov]
Article / Publication Details First-Page Preview Abstract The electroneuronographic registration of the summating potentials of the face muscles has been used to assess the prognosis in 72 traumatic facial nerve lesions. [karger.com]
Etiology
Tumors and demyelinating diseases (for example multiple sclerosis) have been described as potential etiologies as well. [symptoma.com]
Complementary studies are needed to establish the etiologic diagnosis, with laboratory tests to discard the possible causative diseases underlying the trigeminal neuropathy, and the opportune radiographic examinations in the form of plain X-rays or a [ncbi.nlm.nih.gov]
Epidemiology
Epidemiology of typical and atypical craniofacial neuralgias. Neurological Sciences. 2005 May 1;26(2):s65-7. ↑ Rushton JG, Olafson RA. Trigeminal neuralgia associated with multiple sclerosis: report of 35 cases. [physio-pedia.com]
PROXIMAL DIABETIC NEUROPATHY (PDN) OF THE LOWER LIMBS Clinical and epidemiological aspects – Diabetic patients, usually over the age of 50, may present proximal neuropathy of the lower limbs characterized by a variable degree of pain and sensory loss [scielo.br]
Epidemiology of primary Sjögren's syndrome in north-west Greece, 1982–2003. Rheumatology. 2006; 45 (2):187–191. [ PubMed ] [ Google Scholar ] 103. Westhoff G, Zink A. Epidemiology of primary Sjörgren's syndrome. [ncbi.nlm.nih.gov]
Epidemiology of typical and atypical craniofacial neuralgias. Neurological Sciences 2005;26 Suppl 2:s65-67. Summary Putzki N et al. Prevalence of migraine, tension-type headache and trigeminal neuralgia in multiple sclerosis. [mstrust.org.uk]
Epidemiology The prevalence of TN has been estimated at 107.5 males and 200.2 females per 1 million populations [23]. [hindawi.com]
Pathophysiology
Abstract Painful posttraumatic trigeminal neuropathy (PPTTN) is a chronic condition that is difficult to endure and has a poorly understood pathophysiology. [ncbi.nlm.nih.gov]
The pain may manifest in any of many distinct forms that are believed to reflect differences in neural pathophysiologies, even if the details are not well known. [ichd-3.org]
Nor is it clear whether the two diseases are distinct entities or share common pathophysiological mechanisms. [bmcneurol.biomedcentral.com]
Diagnosis Although the diagnosis remains based exclusively on history and symptomatology, modern diagnostic techniques, particularly high-resolution magnetic resonance imaging, provides valuable new insight into the pathophysiology of these cases with [columbianeurosurgery.org]
Prevention
Doctors do not know how to prevent trigeminal neuralgia, to predict who will get it, or determine who will respond to a particular treatment until it is tried. [emedicinehealth.com]
Prevention There are no guidelines for preventing the development of trigeminal neuralgia. [medicalnewstoday.com]
There are several different methods that can be used: Radiofrequency Ablation (RFA) – Radio waves are applied to a nerve or plexus, preventing the transmission of pain. [ainsworthinstitute.com]
The surgeon locates the blood vessel that is pushing against the trigeminal nerve, causing the pain, and inserts a pad between the nerve and the blood vessel to prevent any further contact. [pabrainspine.com]
References
- Cruccu G, Finnerup NB, Jensen TS, et al. Trigeminal neuralgia: New classification and diagnostic grading for practice and research. Neurology. 2016;87(2):220-228.
- Montano N, Conforti G, Di Bonaventura R, Meglio M, Fernandez E, Papacci F. Advances in diagnosis and treatment of trigeminal neuralgia. Ther Clin Risk Manag. 2015;11:289-299.
- McMillan R. Trigeminal Neuralgia — A Debilitating Facial Pain. Rev Pain. 2011;5(1):26-34.
- Bennetto L, Patel NK, Fuller G. Trigeminal neuralgia and its management. BMJ. 2007;334(7586):201-205.
- Peñarrocha M, Cervelló MA, Martí E, Bagán JV. Trigeminal neuropathy. Oral Dis. 2007;13(2):141-150.
- Al-Quliti KW. Update on neuropathic pain treatment for trigeminal neuralgia: The pharmacological and surgical options. Neurosciences (Riyadh). 2015;20(2):107-114.
- Obermann M. Treatment options in trigeminal neuralgia. Ther Adv Neurol Disord. 2010;3(2):107-115.