Comparing and Contrasting Trigeminal Neuralgia and Giant Cell Arteritis
Comparing and Contrasting Trigeminal Neuralgia and Giant Cell Arteritis
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Comparing and Contrasting Trigeminal Neuralgia and Giant Cell Arteritis
Presentation
Trigeminal neuralgia is an abrupt onset, orofacial pain disorder that affects roughly 15 people per 100,000 in the United States every year (Araya, et.al, 2020). The prevalence of trigeminal neuralgia is at a 3:1 proportion between women and men (Araya, et.al, 2020). About 90% of cases occur in individuals over 40 years old (Araya, et.al, 2020). This chronic disorder is characterized by abrupt, spasm or stabbing-like pain along branches of the trigeminal nerve that can last from two seconds to two minutes (Di and Truini, 2020). The pain is typically associated with the maxillary or mandibular section of the trigeminal nerve and is unilateral (Di and Truini, 2020). It is important to note that the pain is typically on the right side, however, it can occur on the left (Di and Truini, 2020).
Similar to trigeminal neuralgia, giant cell arteritis affects adults 50 years and older (Buttaro, et.al, 2021). It also has a higher female to male ratio of 3:1 to 2:1 (Buttaro, et.al, 2021). This disorder is characterized as vasculitis in the large and medium vessels, most commonly the carotid artery and the thoracic aorta (Watanabe, et.al, 2023). Like trigeminal neuralgia, the clinical presentation of this disorder can involve complaints of the head and face (Watanabe, et.al, 2023). Some key differences in the patient complaints of giant cell arteritis are visual impairment, scalp tenderness, tenderness in the temporal artery, and stroke-like symptoms (Buttaro, et.al, 2021). The complaint of a new, severe headache in the temporal region is a common complaint (Watanabe, et.al, 2023). So the location of the pain differs from trigeminal neuralgia, as does the description of the pain. It is important to note that stroke-like symptoms and visual complaints are considered a medical emergency and warrant a referral to the emergency department. Another key difference in the presentation of this disorder is that there may be tenderness, swelling, and nodular appearance of the temporal artery (Buttaro, et.al, 2021).
Pathophysiology
Most cases of primary trigeminal neuralgia are caused by vascular compression of the cerebral arteries, which in turn can lead to demyelination (Di and Truini, 2020). This demyelination has been found to cause neuralgia pain, nerve atrophy, and altered transmission of impulses resulting in this stabbing-like pain (Di and Truini, 2020). Secondary trigeminal neuralgia is known to be caused by an underlying disease or cause, such as trauma, a tumor, or multiple sclerosis (Di and Truini, 2020). The third type of trigeminal neuralgia makes up roughly 10% of cases and is idiopathic in that no found cause for nerve damage (Di and Truini, 2020).
The cause of giant cell arteritis is unknown (Buttaro, et.al, 2021). It is believed that it is an autoimmune disease (Watanabe, et.al, 2023). Unlike trigeminal neuralgia, where vascular compression is occurring, arterial wall inflammation is happening with this disorder (Watanabe, et.al, 2023). The body’s immune system is attacking the arterial walls leading to inflammation and occlusion (Watanabe, et.al, 2023). Where the compression in trigeminal neuralgia leads to demyelination, the occlusion in giant cell arteritis can actually lead to ischemia which is why some patients present with stroke-like symptoms and visual disturbances (Watanabe, et.al, 2023).
Assessment
The key complaints of this disorder are the recurrent episodes of pain along the trigeminal nerve that are described as spasm and or stabbing-like (Araya, et.al, 2020). This pain is unilateral, tends to be on the right side, and lasts usually a short period of time (Araya, et.al, 2020). Talking, facial movements, touching the face, and chewing can all cause the episodes of pain (Araya, et.al, 2020). Patients are usually able to pinpoint the area of pain upon examination (Araya, et.al, 2020). During the interview, it is important to ensure the patient has had no recent facial trauma (Araya, et.al, 2020). It is also important to examine all cranial nerves and perform a corneal light reflex test because it may be abnormal in secondary trigeminal neuralgia (Di and Truini, 2020). Appropriate diagnostic testing for this disorder relies greatly on the history and physical (Di and Truini, 2020). Bilateral facial pain, prolonged pain, facial sensory loss should lead to further diagnostic testing such as MRI or trigeminal reflex testing (Di and Truini, 2020).
As discussed previously, patients tend to present with new onset headaches, visual impairment, scalp tenderness, and tender temporal arteries (Buttaro, et.al, 2021). Similarly to trigeminal neuralgia, jaw pain with movement and eating can be a common complaint, however, it is not relieved with rest (Buttaro, et.al, 2021). The physical exam is extremely important in diagnosing this disorder. Some important assessments include palpation and examination of the temporal arteries (they may be tender, swollen or nodular), examination of the scalp, auscultation of the neck arteries, ocular fundoscopy, and blood pressure measurements in both arms (Buttaro, et.al, 2021). Fever, weight loss, and loss of appetite are also common complaints of giant cell arteritis (Buttaro, et.al, 2021). Unlike trigeminal neuralgia, there are key diagnostic exams for giant cell arteritis. Blood work can reveal elevated ESR and CRP, normochromic anemia, leukocytosis, and thrombocytosis (Buttaro, et.al, 2021). Color doppler ultrasound of the temporal arteries is used as a diagnostic aid (Buttaro, et.al, 2021). The gold standard for diagnosis is a temporal artery biopsy (Buttaro, et.al, 2021).
Diagnosis
Diagnoses to consider with trigeminal neuralgia include multiple sclerosis, migraines, dental abscess, tumors, and sinusitis. Multiple sclerosis can present with symptoms of spasms, pain, and tingling on the face, body, or extremities (National Multiple Sclerosis Society, n.d.). It is important to note that multiple sclerosis can cause secondary trigeminal neuralgia (Di and Truini, 2020). One of the key differences to distinguish secondary trigeminal neuralgia due to multiple sclerosis is the patient would present with bilateral facial pain, instead of the typical, unilateral pain (Di and Truini, 2020). Migraines can also present as unilateral facial pain, however, the pain typically is throbbing and other symptoms like nausea, light sensitivity, and vomiting occur (NIH, 2023). Both tumors and dental abscess can cause unilateral neuralgia pain, like trigeminal neuralgia, but to distinguish these, further imaging like a MRI is needed.
A major differential diagnosis of giant cell arteritis is polymyalgia rheumatica which presents as pain and/or stiffness in the neck, shoulders, and hips (Buttaro, et.al, 2021). Like trigeminal neuralgia, migraine and headaches of unknown origin may be a differential diagnosis due to the throbbing pain and blurred vision (Watanabe, et.al, 2023). Infections, cancer, and rheumatic diseases should also be ruled out (Watanabe, et.al, 2023). It is important to note that this disorder is the leading cause of fever of unknown origin in patients 65 and up (Buttaro, et.al, 2021).
Management
Anticonvulsants are considered the first line treatment for trigeminal neuralgia (Di and Truini, 2020). Carbamazepine is given anywhere in doses of 200 to 1200 mg per day, or oxcarbazepine is given in doses of 300 to 1800 mg per day (Di and Truini, 2020). In patients who take carbamazepine, CBC, serum sodium levels, and liver function tests must be checked regularly (Buttaro, et.al, 2021). Patient education on this medication is not only the need for labs if taking carbamazepine, but als that the patient should not abruptly stop this medication (Buttaro, et.al, 2021). If treatment with anticonvulsants alone does not relieve the symptoms, baclofen, lamotrigine, and phenytoin may be started (Buttaro, et.al, 2021). For cute attacks, botox and intranasal lidocaine injections have been shown to relieve pain (Buttaro, et.al, 2021).
On the other hand, the first line pharmacological treatment for giant cell arteritis is prednisone daily at 1 mg/kg with a max of 60 mg daily (Buttaro, et.al, 2021). Typically, patients taper to 10 to 15 mg daily (Buttaro, et.al, 2021). Methotrexate is recommended as adjunctive therapy to help lower the dose of prednisone (Buttaro, et.al, 2021). Lastly, aspirin 81 mg daily is recommended to prevent ischemia (Buttaro, et.al, 2021). Monitoring of glucose, symptoms, ESR and CRP for these patients should start at week 1, 3, and 6, then monthly (Buttaro, et.al, 2021).
For both trigeminal neuralgia and giant cell arteritis, consultations are warranted to other specialties. For trigeminal neuralgia, a neurosurgeon should be consulted if medication therapy does not relieve symptoms (Buttaro, et.al, 2021). Surgical interventions can include radiofrequency ablation, microvascular decompression, and stereotactic radiosurgery (Buttaro, et.al, 2021). For giant cell arteritis, a rheumatologist should be consulted for patients who do not respond to steroids (Buttaro, et.al, 2021). A ophthalmologist, general or plastic surgeon should be consulted to perform the temporal artery biopsy (Buttaro, et.al, 2021).
Education for both disorders include information about the disease processes. In addition to this, it is important to discuss the pharmacologic options and their side effects. It is important to educate the patient on their plan of care, especially in this situation where patients are in pain. Discussing other specialties that are involved and how they play into the patient’s care is also important.
References
Araya, C., Piovesan, E., & Chichorro, J. (2020). Trigeminal neuralgia: basic and clinical aspects. Current Neuropharmacol, 18(2), 109-119. doi: 10.2174/1570159X17666191010094350.
Buttaro, T. M., Polgar-Bailey, P., Sandberg-Cook, J., Trybulski, J., & Distler, J. (Eds.). (2021). Primary care: Interprofessional collaborative practice (6th ed.). Elsevier.
Di, S. & Truini, A. (2020). Trigeminal neuralgia. The New England Journal of Medicine, 383(8), 754-762. https://doi.org/10.1056/NEJMra1914484
National Institute of Health. (2023). Migraine. NIH. https://www.ninds.nih.gov/health-information/disor…
National Multiple Sclerosis Society. MS signs and symptoms. MS. https://www.nationalmssociety.org/Symptoms-Diagnos…
Watanabe, R., Berry, G., Liang, D., Goronzy, J., & Weyand, C. (2020). Pathogenesis of giant cell arteritis and takayasu arteritis- similarities and differences. Current rheumatology reports, 22(68), 1-11. https://doi.org/10.1007/s11926-020-00948-x
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