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Trigeminal Neuralgia and Orofacial Pain

5868 words (23 pages) Essay in Medical

08/02/20 Medical Reference this

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OVERVIEW

THIS CHAPTER CONCENTRATES ON OROFACIAL PAIN, WITH SPECIFIC INSIGHT INTO DIAGNOSIS AND TREATMENT OF TRIGEMINAL NEURALGIA. THE CHALLENGES FACED BY BOTH CLINICIANS AND PATIENTS IS EVIDENT, AND EMPHASIS IS PLACED ON THE IMPORTANCE OF ACCURATE HISTORY TAKING AND CAREFUL TREATMENT PLANNING IN ORDER TO SUPPORT PATIENTS WHO MAY PRESENT WITH THIS DISTRESSING CONDITION.

Introduction to Orofacial pain

Orofacial pain in the clinical setting can be one of the most challenging conditions to treat, even for the more experienced clinicians. Initially, many patients may present within a primary care setting such as with their General Dental Practitioner (GDP) or General Medical Practitioner (GMP). This initial presentation may be for advice and a first attempt at diagnosis of their pain and desperation for treatment, but when local causes cannot be identified and treated, clinicians may refer patients to a secondary care setting for further investigation.

Neuralgia can be defined as paroxysmal, intense intermittent pain that is usually confined to specific nerve branches to the head and neck.1 Historically, one of the early descriptions of possible Trigeminal Neuralgia was described by Avicenna as ‘Tortura Oris’2, however in 1773, it was John Fothergill who was seen to have given the first full and accurate description of Trigeminal Neuralgia which was presented to the Medical Society in London.3 Investigation of the cause of the neuralgia present and treatment planning of these symptoms can pose as a challenge for any clinician, and the importance of detailed assessment and history taking of orofacial pain can be highlighted. Extreme care must be taken in order to identify the underlying cause of the symptoms experienced by the patient, who may often present in distress with the condition, as suffering from head and neck neuralgia can truly affect a patient’s quality of life. In this chapter, we review the common neuralgias occurring within the oral and maxillofacial region with special emphasis on Trigeminal Neuralgia. We will discuss the historical evolution of treatment including the medical and surgical modalities with the use of current literature and newer developments. This highlights the need for further studies and investigation into the phenomenon of neuralgia in order to improve patient management and treatment outcomes.

Neuralgia Categories

Within the maxillofacial region neuralgias can present in different severities and can affect patients from any race, gender and age. Certain conditions may be distinctive to certain groups of people but there is no current classification followed for the diagnosis and management of neuralgic pain, however groups do exist in order to distinguish the categories that they may be separated into.

Trigeminal Neuralgia

The trigeminal nerve is responsible for sensory innervation of the scalp, face and mouth and damage or disease to this nerve may result in sensory loss, pain or both. Trigeminal Neuralgia, also referred to as ‘Tic Doulureux’ is sought to be the most intense and well-known neuralgias that displays classical features of intense sharp, stabbing sensations with or without burning pain throughout the face. It is considered in being one of the most chronic painful conditions known within the body. This severe medical condition affects one or more branches of the fifth cranial nerve known as the trigeminal nerve, which is the largest cranial nerve and has both sensory and motor functions.  >85% of cases of Trigeminal Neuralgia are of the classic type known as Classical Trigeminal Neuralgia (CTN) while the remaining cases can be separated to secondary Trigeminal Neuralgia (STN). STN is thought to be initiated by multiple sclerosis or a space-occupying lesion affecting the trigeminal nerve, whereas the leading cause of CTN is known to be compression of the trigeminal nerve in the region of the dorsal root entry zone by a blood vessel.4,5

Clinical Presentation

These pain episodes experienced may last from seconds up to several minutes and can be described by the patient as an ‘electric shock’ feeling. This sensation may occur frequently per day (up to hundreds of times) over weeks and months and then suddenly stop with pain free periods in between. It may also present infrequently with periods of remission which may possibly last for years6. The pain often occurs unilaterally and does not usually cross the midline of the face and is often unbearable for the patient. It has been shown that only 3% of cases are known to be bilateral in nature.6

Risk Factors

Sex:

It has been highlighted that Trigeminal Neuralgia affects females more than males.6

Age:

In patients over 80 years old, males tend to have a higher incidence (45/100,000)7-11It can be prominent within all age ranges but most frequently Trigeminal Neuralgia affects individuals over the age of 504 with about 70% of patients present older than 60 years at onset.13,14  It is known that the incidence of Trigeminal Neuralgia increases with age and has been emphasised that this condition is rare affecting people younger than 40 years old.6 This is therefore highly important in suggesting that multiple sclerosis may be a present in younger patients who suffer from Trigeminal Neuralgia.4

Initiating factors

The pain felt can be precipitated by trigger areas or factors of light touch on specific areas of the face, and patients often avoid these actions which they may feel causes the attacks. These activities may include:

                      Shaving

                      Brushing the teeth

                      Speaking

                      Vibration

                      Cold wind

                      Chewing

                      Touching or washing certain areas of the face.

Prevalence

NICE guidelines data and studies6,13 indicate that a survey carried out within general practice in the United Kingdom highlighted that the annual incidence of Trigeminal Neuralgia ‘was 8 per 10 000 and a lifetime prevalence of 0.7 per 100000 people per year.’ The prevalence of this condition is unclear as there is little data to support the evidence of how common this condition is.6,13,45

Causes & Diagnosis

Trigeminal Neuralgia can occur as a result of several causes such as trauma, tumours, infectious or demyelinating diseases, connective tissue diseases and can also be idiopathic in nature. This poses a challenge to the clinician when trying to investigate the cause of the pain. The importance of the causative factors may highlight the possibility that Trigeminal Neuralgia can present as a first manifestation of an underlying systemic disease. This emphasises that careful and in depth investigations with detailed history taking is required in order to appropriately treat this life affecting condition. A referral to a specialist in pain management or neurologist should be considered in severe cases affecting the patient.4,6,13

As Trigeminal Neuralgia is indicated when a patient may present with severe and intense pain in the orofacial region, other avenues may need to be explored for patients who present with physical signs of motor or sensory problems as they may result from other conditions. Neoplasms, Infective conditions such as HIV, Multiple sclerosis and even Cerebrovascular disease may cause neuralgic pain, and so it is important to be aware of the differential diagnosis that could be derived from neuralgia within the head and neck.

Accurate diagnosis relies greatly on a detailed history of symptoms from the patient with pattern and nature of the pain highlighting the condition, as there is no definitive diagnostic test yet available. SOCRATES is a useful assessment tool (TABLE 1) which can be used to help clinicians in achieving an accurate history.

Site/Localisation

Can the pain be localised to a specific area?

Onset

Sudden or gradual? When- Day/Night/Spontaneous?

Characteristic of pain

Sharp, stabbing, dull ache

Radiation

Does the pain radiate elsewhere?

Associated signs and symptoms

Any associated?

Timing/Duration

Seconds/Minutes/Hours? (constant, paroxysmal-recurrent, slowly/rapidly progressive)

Exacerbating or relieving factors

Anything make it better/worse?

Severity

How intense is the pain? Scale 1-10

TABLE 1- SOCRATES ASSESMENT TOOL

Trigeminal Neuralgia may be misdiagnosed for dental pathology and so it is important that unnecessary dental treatment is not carried out without full investigation of the source of the pain.  When patients suffer from the condition it often becomes apparent that their quality of life decreases as they may be unable to carry out their normal daily activities, have weight loss due to problems eating and the condition may in turn lead to depression and isolation.

On clinical examination, trigeminal reflex testing may be used to test all three divisions of the nerve and may reveal loss of sensitivity in the cutaneous region which may relate to the affected nerve. This may present as partial numbness (hypoesthesia) or complete numbness (anaesthesia) and occasionally may present as hyperaesthesia causing considerable discomfort.14

In idiopathic forms of Trigeminal Neuralgia, it is typical for no cause to be detected with the patient having both normal neurological and MRI examinations which can cause difficulty with treatment planning. In about 60-90% of cases described in neurosurgical and neuroradiological series, compression of the trigeminal nerve root by an aberrant loop of a blood vessel, usually within a few millimetres of the entry into the pons is the most common cause.15-18

Special Investigations

In order to establish a more definitive diagnosis, special investigations must be carried out as an aid to clinical examination and detailed history. In order to rule out possible diseases, specific laboratory tests may be carried out in adjunct with radiographic examinations such as plain radiographs including intra oral (periapicals) and orthopantograms. These may be carried out in first instance to rule out dental pathology and orthopantograms may also be able to detect temperomandibular joint pathology. Cranial computed tomography scan (CT) may also be used in order to identify any changes of the maxillary sinus.19

Magnetic resonance imaging (MRI) is an important investigation to follow and a valid method to investigate and differentiate between patients suffering from secondary trigeminal neuralgia related to tumours and that related to multiple sclerosis.20 It may demonstrate the close and potentially causative relationship between the trigeminal root and adjacent blood vessel and can be of specific value to exclude posterior cranial fossa lesions.4 Trigeminal Neuralgia caused by multiple sclerosis should be ruled out, specifically in the younger patient and this may be aided by magnetic resonance imaging. Even although MRI is commonly used, from previous studies carried out, suggestion of its sensitivity and specificity seems variable 21-28 As a result, emphasis is placed on the challenge in identifying the cause of Trigeminal Neuralgia as a relationship between the clinical symptoms and radiological findings as this may not be clear.

Additional Neuralgias

Direct or Indirect trauma: this category may include neuralgic pain caused by extraction of third molars, injection of anaesthesia from alveolar nerve blocks, agents used within endodontic treatment, orthognathic surgery and implantology.

Temporomandibular joint disorders (TMJD):  TMJD may also present with facial pain symptoms with the involvement of tension-type headaches and persistent idiopathic facial pain. This can result in diagnostic difficulties in identifying pain from the facial muscles and muscles of mastication or from the temporomandibular joint itself, whether this is unilateral or bilateral. Temporomandibular joint disorders may occur as a result from trauma, inflammation, jaw joint dysfunction, tumours and can also be referred pain. The severity of this pain suffered varies between patients and can last from minutes to hours and present as a dull ache or a throbbing sensation. The pain is mostly initiated from the masticatory muscles and palpation of the TMJ and jaw movements. Conservative management is the first choice of treatment if disease has been excluded.29,30

Benign or Malignant tumours: can also be involved in causing facial pain with spread into the cranial nerves resulting in neuralgia like symptoms to be known.

Infectious Aetiology as a result of Herpes zoster-Shingles (post-herpetic trigeminal neuralgia) causing neuralgia of herpetic origin has been documented 6 and can present as a burning pain which is continuous and severe for the patient. The reactivation of latent varicella zoster virus is known as acute herpes zoster, which is a disease of the dorsal root ganglion and can present itself decades after the primary infection as occurred with the ophthalmic branch being affected in over 80% of the trigeminal nerve cases.31 Pain control is the most important aspect of treatment as well as minimising the risk of complications and it is evident that when antivirals are administered within 72 hours from the onset of the rash, they are known to reduce the rash duration, pain severity and incidence of post herpetic trigeminal neuralgia.32-38There has not been any classifications in place that can describe when acute herpes zoster may be defined as post herpetic neuralgia and so classifying this may also pose as a challenge for physicians.

Periodic migrainous neuralgia, also known as ‘cluster headaches’ is a rare form of retro-orbital pain which tends to affect young men mostly at night time and may cause the eye of the affected side to water or may even cause nasal congestion. It tends to affect patients unilaterally with each attack lasting less than an hour with it being related to vascular changes and are often precipitated by the consumption of alcohol. The condition is often managed by a physician with non-steroidal anti-inflammatory medications such as ibuprofen, or agents such as sumatriptine/ergotamine and oxygen inhalations. Drugs such as verapamil (calcium channel blockers) may be taken as prophylaxis.4 In contrast to this, Tension Headaches is another form of neuralgic headache pain which is not uncommon. These headaches are often stress induced and initiated by anxiety/stress causing pain within the temple, forehead and/or neck region. They specifically affect the frontal, temporal muscles and occipital muscles within the neck. They don’t cause pain within the mouth but often the patient suffers from a constant ache which tends to worsen in the evening. The management of these patients often relies on reassurance and relaxation techniques with the aid of lifestyle changes but sometimes anxiolytics or benzodiazepines may be used.31

Historical evolution of treatment for Trigeminal Neuralgia (medical/surgical modalities)

Emphasis must be placed on the importance of ensuring that patients are assessed for signs of depression as it is evident that patients who suffer from Trigeminal Neuralgia can be affected greatly within their quality of life. This may be in regards to their general daily activities, self-care, mobility, sleeping, eating and general social interactions, and so it is very important that this is understood within the patients symptoms and complaints.6 Patients may also suffer from suicidal thoughts due to the intensity of the pain and so great care must be taken to ensure that patients are thoroughly listened to and assessed. This has been emphasised from a 2015 study39 that highlighted that Trigeminal Neuralgia is associated with and increased risk of both depression and suicide. It is also been made aware from guidelines6,45 that if ‘red flag’ symptoms or signs are evident, then this must be investigated, as it may advocate an underlying serious or systemic cause and so the appropriate measures must be taken to ensure that it is not overlooked.

Trigeminal Neuralgia Treatment

Initial First Line Treatment

It has been highlighted that first line of treatment for Trigeminal Neuralgia is pharmacological treatment. The desired outcome of these patients is to treat the pain experienced, manage the symptoms, and with time preferably eradicate these symptoms in order to improve the patient’s quality of life. Where appropriate, referral to a specialist pain service and/or neurologist may be necessary with clear information and advice given to the patient.

Medical Intervention

There are several drugs that have been delivered systemically or topically in the use of treating Trigeminal Neuralgia which include:

  • Baclofen
  • Dextramethorphan
  • Lamotrigine
  • Pimozide
  • Proparcaine
  • Tizanidine
  • Tocainide
  • Topiramate

However, the most common therapy of choice is Carbamazepine.

All the above drugs have been evaluated using RCT’s whereas other drugs such as capsaicin cream, phenytoin, clonazepam, gabapentin, oxcarbazepine, mexilitine, and tramadol have been assessed from case reports and series. Studies involving many of these drugs in regards to the full benefit and effect on the treatment of Trigeminal Neuralgia are limited, and so further evaluation is required.29,40 The response to the drugs mentioned are unique to each patient and it is evident that the doses also vary between patients in order to achieve a beneficial effect to counteract the pain experienced. It has been highlighted that it may be helpful for the patient to keep a pain diaryin order to record episodes and help the patient and clinician identify possible trigger factors and timing of pain. This may even aid with treatment planning and may give patients back a sense of control which has been lost due to their condition.

Carbamazepine

Anticonvulsant drugs have been commonly used to treat Trigeminal Neuralgia and have been the drug of choice for many years. Based on existing evidence, carbamazepine also known with the trade name ‘Tegretol’ is an anticonvulsant drug used primarily in the treatment of epilepsy29,41 and remains the drug of choice for standard first line treatment of Trigeminal Neuralgia in patients over 18 years of age.6 It is considered to be of diagnostic help if complete resolution or reduction of symptoms occur after its use4, however carbamazepine must be used prophylactically and continuously for long periods, with tiered dosages prescribed to suit individual patients in regards to their response. Carbamazepine should be used with caution and as it not an analgesic, it is not appropriate to use this medication during a pain episode for relief as it will not have an analgesic effect on symptoms. Patients can often misinterpret what the purpose of the medication is and so this is turn highlights the importance of patient communication. The mechanism of the medication, the instructions in terms of dosage titration, timing of effects and the possible adverse side effects associated with its use should be highlighted. From current guidelines such as –NICE 2013,20176,45 it has been advised that if no sinister or red flag symptoms are evident and carbamazepine is not contraindicated for the patient then the following dosage guideline can be offered:

  • 100mg up to twice daily, titrated in increments of 100-200mg every two weeks until pain has been relieved
  • 200mg three of four times daily (600-800mg daily) is seen in majority of people to be the dosage of choice sufficient to manage pain
  • 1600mg maximum dose daily
  • Once pain is in remission, the dosage should be gradually reduced to the lowest possible maintenance level or even discontinued until a further episode occurs6

When patients are treated with Carbamazepine, it is strongly advised that a full blood count and liver function tests are carried out prior to starting treatment and then reviewed periodically in order to monitor the possible effects of the drug. Hyponatraemia which refers to low sodium levels is thought to occur in 20% of patients and NICE 2013 guidelines suggest that carbamazepine in concurrent use with sertraline can also increase this risk. Serum levels of the drug are not routinely monitored unless carbamazepine toxicity is suspected but within the BNF all information is available in regards to drug interactions, adverse effects and contraindications and cautions. Within primary care settings this is the standard first choice of treatment if the physician is confident with the diagnosis, otherwise it is advised to refer the patient to a secondary care specialist for further investigations and treatment.

 

Surgical Intervention

When medical treatment of Trigeminal Neuralgia fails, diagnosis of the condition should be re visited prior to consideration of surgical intervention. It has been viewed that up to 10% of people with Trigeminal Neuralgia will not have a beneficial response to pharmacological therapy in order to treat the pain6,29

Surgical interventions in the treatment of Trigeminal Neuralgia for patients who had a limited response to drug treatment may include microvascular decompression with a variety of methods such as neurectomy, radio frequency thermal ablation, balloon compression, glycerol injections and radiosurgery. The evidence base for surgical modalities is weak and the surgical treatment interventions are explored only when pharmacological intervention does not have a positive outcome.

Radiofrequency Denervation

A paper from 201342 indicated that for elderly patients who may suffer from multiple sclerosis and are not fit for surgical treatment such as microvascular decompression, then radiofrequency denervation may be a suitable alternative. This paper highlights a possible treatment option that mat be effective in treating a selected group of patients, however further studies and evidence is needed in order to rely on this surgical intervention.

Microvascular decompression

This surgical technique involves the separation of the trigeminal nerve from adjacent blood vessels involving the above methods mentioned. In some large sequential case series carried out within specialist centres, it has been reported that over two thirds of patients who have been treated using microvascular decompression, are pain free at 10 years, with 1% experiencing facial numbness.43

Other studies revealed less optimistic results with newer techniques in magnetic resonance imaging, highlighting that this may identify microvascular compression more readily and result in the improvement of patient case selection. The treatment of microvascular decompression is a surgical modality which has been shown to have beneficial results when carried out in expert hands. As with all surgical procedures, it is surgeon dependant and even although the procedure is designed to improve symptoms and not damage the trigeminal nerve, there is a small but certain risk of serious and even fatal complications.

Newer surgical procedures

Newer surgical interventions for diagnosis and treatment include the possibility of Sphenopalatine Blocks for symptomatic relief of acute exacerbations which was revealed in a study in 201544. This study highlighted this intervention carried out on 15 patients  to treat or diagnose the maxillary division of the nerve affected in Trigeminal Neuralgia and emphasised a safe, simple and possibility effective technique for a sphenopalatine ganglion block.

Conclusion

It is evident that Trigeminal Neuralgia is a disorder which needs further investigation and research in order to identify additional treatment modalities to offer patients who suffer from this condition. The importance of accurate history and detailed assessment is crucial, with the aid of a multi-disciplinary approach and to patient care in order to aim towards pain management, reduction and in some case eradication of this life limiting condition.

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42-    Radiofrequency denervation (RFD) in the management of Trigeminal Neuralgia (TGN) in the elderly and patients with Multiple Sclerosis (MS) when the drugs don’t work Jonathan Hayter ‘Correspondence information about the author Jonathan Hayter, Margaret Bone University Hospitals of Leicester, United Kingdom PlumX Metrics DOI: https://doi.org/10.1016/j.bjoms.2013.05.128

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44-    February 2016Volume 54, Issue 2, Pages 226–227 Sphenopalatine block in the management of trigeminal neuralgia Grant Isherwood’Correspondence information about the author Grant Isherwood DOI: https://doi.org/10.1016/j.bjoms.2015.08.274

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