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). Function: It supplies sensations to the face, mucous membranes, corneal reflex and other structures of the head. It is the motor nerve for the muscles of mastication and contains proprioceptive fibers. 1 2 3 INTRODUCTION Trigeminal neuralgia is a condition/neuropathic disorder of the fifth cranial nerve that is characterized by paroxysms of pain in the area innervated by any of the three branches of the trigeminal nerve. In Trigeminal neuralgia the sensory or afferent branches, primarily the maxillary and mandibular branches are involved. The pain ends as abruptly as it starts and is describes as unilateral shooting or stabbing sensation. 4 Pain usually occurs at eyes, forehead, lips, scalp and jaws. It has been labelled as suicide disease due to insignificant number of people taking their own life because they are have their pain controlled by medication or surgery. The condition was first described in detail in 1773 by John Fothergill 5 DEFINITION Sudden usually unilateral, severe brief stabbing recurrent pain in the distribution of one or more branches of 5TH cranial nerve. - International association for study of pain Painful, unilateral affection of the face, characterized by brief electric shock like pain limited to the distribution of one or more divisions of trigeminal nerve. Pain is commonly evoked by trivial stimuli including washing, shaving, smoking, talking, brushing but may also occur spontaneously. The pain is abrupt in onset and terminations may remit for varying periods. – International Headache Society 6 SYNONYMS Tic Douloureux Trifacial Neuralgia Fothergill’s Disease Prosopalgia 7 EPIDEMIOLOGY Incidence: 5 in every 100,000 Peak incidence: 50 to 60 years 90% of cases occur after age 40. Gender Ratio: Female : Male 2:1 Right sided 56% of the time. Only 3% of people experience pain on both sides of face. Maxillary V2 > Mandibular V3 > Ophthalmic V1 Roughly 15,000 new cases annually in The United States. Zakrzewska JM, Hamlyn PJ In Epidemiology of Pain, 1999 8 Katusic Neuroepidemiology 1991 Contd.. Between September 2007 and April 2015, 20 patients underwent micro vascular decompression (MVD) of Trigeminal Neuralgia at Department of Neurosurgery, Bir Hospital. 9 males and 11 females and age ranged from 30-70 years. The neuralgic pain was localized on right side in 13 patients and left on 7 patients. Pain distribution was on V3 in 11, V2 in 4, V2-3 in 2 and V1- 2-3 in 3 patients respectively. 20 patients felt pain relief immediately after procedure and 1 patients came after 3 years with recurrent pain requiring second surgery. Nepal Journal of Neuroscience, 2017. 9 CLASSIFICATION International Headache Society (IHS) classified TN in two types: Classical/ Idiopathic/Typical : • Unilateral, severe, stabbing, shock like pain in one side of the face. • They are abrupt in onset and termination. Symptomatic/ Atypical : • Constant dull aching, burning pain that is less severe. • Caused by demonstrable structural lesion other than vascular compression. 10 RISK FACTORS Advanced Age : High among old people; especially between 50 to 60 years. Female Sex Multiple Sclerosis While the presence of these risk factors increases the likelihood of developing TN, it is also possible for younger people or children to have TN. In rare cases, trigeminal neuralgia occurring in people below 50 years of age typically involves the ophthalmic division of the trigeminal nerve (the branch that is least involved in TN) and may cause loss of vision 11 ETIOLOGY Usually Idiopathic Compression from near-by blood vessels: Majority of TN cases occur due to compression of the trigeminal nerve by one or more arteries and/or veins. Large or small blood vessels can grow over, wrap themselves around, or combine together to squeeze the nerve. A constant irritation to the nerve can also occur from the pulsating action of blood vessels. 12 13 Multiple sclerosis (MS): MS is a neurodegenerative and inflammatory condition that causes breakdown of the myelin sheath around the nerves. This loss of protective coating around the trigeminal nerve can cause irritability to the nerve, resulting in TN as potentially an early symptom of MS. 14 Tumor and Cysts Blood vessel abnormalities like Aneurysms and Ateriovenous malformations Viral etiology: Herpes virus infection, Syphilis 15 Dental Etiology: It is possible that certain dental procedures or fillings may trigger an already developing TN to suddenly become fully noticeable. Abnormally thickened arachnoid tissue layer of the brain Trauma 16 Inflammatory conditions such as sarcoidosis and Lyme disease or vascular diseases such as scleroderma and systemic lupus erythematous. Atypical TN is presumed to be caused by tumors or cysts. Typical TN with symptoms on both sides of the face is also believed to commonly have a cancerous origin. 17 PATHOPHYSIOLOGY Compression of the Trigeminal nerve Injure the nerve’s protective myelin sheath Cause erratic and hyperactive functioning of the nerve Pain attacks at the slightest stimulation Hinder the nerve’s ability to shut off the pain signals 18 SIGNS AND SYMPTOMS Severe pain: Sharp, stabbing, or shooting pain, on one side of the face that feels like a series of electric shocks and lasts for seconds to minutes in typical TN A persistent dull ache or burning sensation with occasionally sharp come-andgo pains are common in atypical TN. Severe, sudden, short-duration (30–60 seconds to 2-3 min),excruciating pain, unilateral facial pain in the distribution of one or two branches of the trigeminal nerve (commonly V2 and V3). 19 • The pain intensity is highest at the start of an episode and lessens rapidly toward the end. It starts and stops suddenly. Dominant right side pain. • Pain in the same location for each episode. 20 Periods of relief. In typical TN ,pain episodes come-and-go with periods of relief between attacks that may last for hours or days. Sometimes a steady dull ache may be present between episodes with no complete relief. In Atypical TN, The sharp pain attacks are less severe compared to typical TN and there are also fewer or no periods of relief between pain cycles. 21 CONTD… Specific Areas Affected: Commonly the maxillary and mandibular branches are affected, causing symptoms in the lips, cheek, jaw, and nose of the affected side. Less often, when the ophthalmic branch is involved, the eyes, forehead, and temples may be affected. 22 TRIGGER ZONES V1 V2 V3 • Supra orbital ridge • Skin of upper lip, Cheek, Upper gum, ala • Lower lip, teeth, gum and jaw 23 CONTD… Easily triggered 24 During a TN attack, the pain may cause wincing on the painful side or even a sudden head jerk. The affected side of the face may also have skin turn red, along with excessive eye tearing and/or salivation. The repetitive cycles of pain with breaks in between can last for weeks or months and may be followed by a long pain-free period that can last up to a year or more. Extreme cases “ Frozen face” OR “ Mask like face”. 25 Impaired function of the affected body part due to pain or muscle weakness due to motor nerve damage. Loss of Deep tendon reflexes and muscle mass Increased sensitivity of the skin or numbness of affected skin Depression and weight loss and occur Patient may frequently have unwashed or unshaven face. 26 PROGRESSION OF TRIGEMINAL NEURALGIA OVERTIME LATER IN THE COURSE OF DISEASE EARLY IN THE COURSE OF DISEASE Periods of Exacerbations Periods of Remission 27 HISTORY TAKING: • • • • • • General History: triggering stimuli and site of the pain Chief complain History of present illness Ask about nature of pain: Brief, paroxysmal, severe, stabbing Onset Location Quality Intensity Frequency Duration 28 CONTD….. Aggravating factors Alleviating factors Ask about other neurological symptoms particularly those common like ataxia, dizziness, focal weakness, unilateral vision changes. Medical History 29 PHYSICAL EXAMINATION Neurological examination Trigeminal Nerve Examination • SENSORY ASSESSMENT eg: light touch, pin prick test • MOTOR ASSESSMENT eg : Jaw jerk reflex • CORNEAL REFLEX 30 • Checking for signs of redness in the face or eye of the affected side • Examining parts of the face and jaw affected during the pain attacks—this also allows the doctor to understand the branches of the trigeminal nerve that may be affected. • Examination of Trigger zones 31 MAGNETIC RESONANCE IMAGING(MRI) • An MRI shows a clear picture of soft tissues, such as the brain, spinal cord, and nerves. It can help assess nerve structure and is especially useful in determining whether TN is caused as a result of multiple sclerosis or tumors. CT SCAN SKULL X-RAY 32 MAGNETIC RESONANCE ANGIOGRAPHY ( MRA) • MRA is a sensitive and specific method to diagnose TN caused by blood vessel compression. In this technique, a dye is injected into the blood vessel to highlight the blood flow. The presence and severity of compressions caused by blood vessels on nerves are well defined in MRAs. 33 DIFFERENTIAL DIAGNOSIS • • • • • • Dental pathology Migraine Cluster headaches Multiple sclerosis Overlying aneurysm of blood vessels Acoustic Neuroma • Meningiomas 34 PHARMACOLOGICAL MANAGEMENT Medications are mainly used to control the pain in TN, and do not treat the cause of TN. For this reason, medications are generally taken long-term, as long as the underlying causes are at work. 35 • Certain medicines sometimes help reduce pain and the rate of attacks. These medicines include: – Anti-seizure drugs (carbamazepine, gabapentin, lamotrigine, phenytoin, valproate, and pregabalin) – Muscle relaxants (baclofen, clonazepam) – Tricyclic antidepressants (amitriptyline, nortriptyline, or carbamazepine) 36 FIRST LINE THERAPY Anti convulsant drugs Carbamazepine: 400- 800 mg/day Oxcarbazepine: 900-1200 mg/day 37 SECOND LINE THERAPY Lamotrigine : 150-400 mg/day Baclofen : 40-80 mg/day Phenytoin : 300-500 mg/day 38 THIRD LINE OF APPROACH Clonazepam Valporic acid : 500-1500 mg/day 39 CONSERVATIVE THERAPY Nerve Blocking with Local Anesthesia Relief of pain is temporary, lasting from 6-18 months Complications include bruising, swelling at the site of injection 40 SURGICAL MANAGEMENT Surgical and injection procedures are recommended in trigeminal neuralgia (TN) patients with intolerable pain, after adequate treatment with medications in various combinations and dosages have been tried and failed. 41 MICROVASCULAR DECOMPRESSION Cranial surgery performed to find and fix an offending blood vessel that is injuring or compressing the trigeminal nerve. This procedure involves making a small opening in the lower back portion of the skull, locating the blood vessel, and inserting a small pad (Teflon sponge or shredded Teflon) to keep the blood vessel and nerve apart. In most cases, the causative blood vessel is an artery. 42 SUCCESS RATE 73% TO 80 % WITH PAIN FREE PERIOD UPTO 5 YEARS 43 GAMMA KNIFE SURGERY Gamma knife surgery is a radiosurgery and no actual incision is made, making it the least invasive surgical option. Gamma knife surgery is a common procedure for patients who cannot tolerate surgery and/or those who have unsuccessful treatments with medications. Focused beams of cobalt-60 radiation are directed on a particular area of the brain to cut off the trigeminal nerve’s blood supply, causing scarring and death of the nerve tissue. 44 SUCCESS RATE ESTIMATED 52%-69% WITH PAIN FREE LIVES UPTO 3 YEARS 45 GLYCEROL RHIZOTOMY This is a procedure in which pure anhydrous glycerol injection is administered in the trigeminal ganglion. Glycerol causes nerve damage by disintegration of the nerve’s myelin sheath. This nerve injury in turn prevents the nerve from sending pain signals to the brain. The goal is to damage the nerve selectively in order to interfere with the transmission of the pain signals to the brain. 46 BALLOON COMPRESSION A large needle is inserted into the trigeminal ganglion, and a tiny balloon is inflated at its tip with a small amount of liquid. The goal is to squeeze the nerve against the bony tissue and cause enough damage to disrupt the pain signals. The balloon is then deflated, and the needle is removed. 47 Success rate: 92% Pain free upto 35 months 48 RADIOFREQUENCY THERMAL LESIONING An electrode inserted through the cheek is used to heat the nerve and cause selective damage to stop pain signals from traveling to the brain. The treatment provides immediate pain relief in up to 90% of patients, but can cause more facial numbness than the other procedures and has a pain recurrence rate of 40% at 2 to 3 years post-surgery. If necessary, the procedure can be repeated. 49 ASSESSMENT • Take complete history of the patient, of pain, including duration, severity, and aggravating factors. • Perform physical examination including neurological examination. • Assess for nutritional status and hydration. • Assess for anxiety and depression, including problems with sleep, social interaction, coping ability/skills. 50 NURSING DIAGNOSIS Chronic pain r/t trigeminal nerve compression as evidenced by pain scale rating. Imbalanced body nutrition r/t pain during chewing as evidenced by weight loss. Ineffective individual coping r/t severe pain as evidenced by patient’s verbalization 51 NURSING DIAGNOSIS Deficient knowledge r/t the disease condition as evidenced by frequently asked questions. Anxiety r/t the prognosis of disease and change in health as evidenced by increased BP, insomnia and fear of consequences. Ineffective management of therapeutic regimen r/t less knowledge about prevention of stimulus triggers as evidenced by pt’s verbalization 52 NURSING DIAGNOSIS Fear r/t treatment or invasive procedures, sensory impairment as evidenced by avoidance behaviour. Self care deficit r/t pain, discomfort as evidenced by poor personal hygiene, disorderly appearance. Powerlessness related to lack of control over painful episodes. Risk for injury to the eyes r/t possible reduction in corneal sensation 53 RELIEVING PAIN • To minimize the pain episodes, review with patient potential triggering factors and develop individual coping methods. • Encourage patient to take medicine regularly. • Instruct patient to avoid exposure of affected area to cold. • Help in communication methods without pain while talking. • Improve the quality of sleep. • Encourage patient to keep a pain diary noting the severity and frequency of pain. • Serum levels must be monitored to avoid toxicity in patients who require high doses to control the pain. 54 MONITOR ADEQUATE NUTRITION • Monitor daily intake and output. • Instruct the patient to take food and fluids at room temperature. Avoid foods that are too cold or hot. • Encourage to chew with the help of unaffected side. • Have the patient consult with dietician for appropriate meal, texture and composition. • Encourage small frequent meals to avoid fatigue and pain. • Advice about use of nutritional supplements as needed. • In severe cases NG Tube feeding can be done. 55 PATIENT EDUCATION • Teach relaxation exercises such as breathing, progressive muscle relaxation and guided imagery to relief muscle tension • Instruct patient to share his/her fears with family or with nurses for relief and assurance. • Teach patient about the disease process and it’s treatment methods. • Instruct patients the methods to prevent environment stimulation of pain. • Instruct patient to inspect eye for redness and foreign body if corneal sensation is impaired and use of eye drops as prescribed. 56 HEALTH MAINTAINENCE • Teach them about follow up visit, regular medication, and consult if any changes in sensation on face like numb. • Refer the patient to physiotherapy and speech therapy for facial exercise and to improve communication. 57 MAINTAIN HYGIENE • Provide cotton pads and room temperature water for washing the face. • Instruct the patient to rinse mouth with mouthwash after eating if toothbrush causes pain. • Instruct to perform personal hygiene during pain free intervals. • Schedule routine dental care to prevent extensive dental treatment. • Warm normal saline irrigation of the affected eye 2/3 times a day is helpful in preventing corneal infection. 58 INCREASING CONTROL • Support patient through treatment trials. • Teach relaxation exercises, such as guided imagery to relieve tension. • Encourage participation in support groups, and facilitate a therapeutic relationship with the health care provider 59 POST OPERATIVE MANAGEMENT Postoperative neurologic assessments are conducted to evaluate the patient for facial motor and sensory deficits in each of the three branches of the trigeminal nerve. If the surgery results in sensory deficits to the affected side of the face, the patient is instructed not to rub the eye, because pain will not be felt if there is injury. The eye is assessed for irritation or redness. Artificial tears may be prescribed to prevent dryness in the affected eye. 60 Contd…. The patient is cautioned not to chew on the affected side until numbness has diminished. The patient is observed carefully for any difficulty in eating and swallowing foods of different consistency. 61 COMPLICATIONS • Morbidity associated with trigeminal nerve decompression stems from hemorrhage, infection, and possible damage to the brainstem around the area of decompression. • Adverse effects of surgery include corneal anesthesia, facial numbness outside of the trigger zone, new facial pain, facial dysesthesias, and intracranial hemorrhage (rare). • Anesthesia dolorosa • Facial dysesthesia , facial numbness • Blurred vision or chewing problems are usually temporary 62 PROGNOSIS After the initial attack, the disorder may become inactive for months or even years. Over time, the attacks may become more frequent, more easily triggered, disabling, and may eventually require long-term medication. Overall, the prognosis depends on the cause of the problem. If there is no underlying disease, some people find that treatment provides at least partial relief. In some patients, however, the pain may become constant and severe. 63 REFERENCES • Cheever. Brunner & Suddarth's Textbook of Medical-surgical Nursing. Edition 10th • https://www.pain-health.com/conditions/facial-pain • https://www.ninds.nih.gov/disorders/patient-caregivereducation/fact-sheets/trigeminal-neuralgia-fact-sheet • https://www.aans.org/Patients/Neurosurgical-Conditions-andTreatments/Trigeminal-Neuralgia 64