So at some point around June I started noticing I was having consistent daily headaches from my right temple. Nothing severe, just a sort of pressure. It could have even started a bit before that, but that was the time when I started thinking to myself "Hmm this is happening pretty often." Around late July they are still happening, so I go to a doctor at my health plan, who says it may be cluster headaches. Of course I go off and read up on them and get incredibly depressed, as they sound like the worst hell imaginable to live through (seriously, look that shit up). Over a few days I sort of ease up and just say I'll probably be ok, as I haven't been hit with anything yet, and the doc was probably wrong since I haven't had any of the specific symptoms (such as reddening / dropping of the eye, tearing).
Time goes by and I'm still sort of having these temple headaches on and off. By Thanksgiving though, I'm noticing new sensations. These include lots of a sort of burning sensation in the cheek, as well as a burning line in the temple, burning line in the neck... hell sometimes just sort of a burning sensation all over my right face. It's not severe.... not yet at least. But after seeing a TMJ specialist for a while and seeing the night mouth guard wasn't working, I decided to see a doctor again. This time, she says Trigeminal Neuralgia (which is pretty fucking close to Cluster Headaches, as it's also called the Suicide disease). Once again I'm super depressed after reading up on it...
So that was probably two weeks ago, and still I'm having this burning sensation all over my right face at times. Sometimes I'm fine, other times I'm just in discomfort with burning / tingling. It hasn't been severe, but in general I've just been very depressed, sort of waiting for an attack of excrutiating pain to happen. Googling the ever living fuck out of neurological diseases hasn't helped (jesus christ there's some fucked up stuff out there that people go through). I'm also on anti-seizure meds for now since that's supposed to help the pain for this condition, if it is this condition, but they aren't helping much. They are turning me into a damn zombie though, and everyone can tell.
So that's where I am now... everyday I wake up and I'm literally praying that maybe this will all just wash over and not turn into a condition where I'll have to live in pain for the rest of my life. Other than sleep, I'm having a hard time even looking forward to anything in my current mental state. It's really opened up my eyes, and even if this all washes over, I'll never forget it. So is there anyone here who has to live through pain day in and day out through his or her life?
I'm sorry to hear about your diagnosis. Please ignore most of the advice you've received here (meditation, physical therapy referral, etc), as it's all wrong. I was thinking giant-cell (temporal) arteritis at first. I hope your doc at least checked some basic labwork (ESR, CRP) to rule out other possible conditions. Trigeminal neuralgia sucks. I'm going to copy-paste some info from UpToDate on the treatment of TN for you, so you can have an idea of what you can expect. (FYI, UpToDate is basically the be-all end-all of compilations of peer-reviewed medical data on the planet that most docs use on a daily basis to make sure we're managing things we aren't familiar with appropriately.)
CLINICAL FEATURES - TN is defined clinically by paroxysmal, stereotyped attacks of usually intense, sharp, superficial or stabbing pain in the distribution of one or more branches of the fifth cranial (trigeminal) nerve (figure 2) [1].
The pain of TN tends to occur in paroxysms and is maximal at or near onset. Facial muscle spasms can be seen with severe pain. This finding gave rise to the older term for this disorder, tic douloureux. The pain is often described as electric, shock-like or stabbing. It usually lasts from one to several seconds, but may occur repetitively. A refractory period of several minutes during which a paroxysm cannot be provoked is common. Some patients with longstanding TN may have continuous dull pain that is present between paroxysms of pain. Unlike some other facial pain syndromes, TN typically does not awaken patients at night.
TN is typically unilateral. Occasionally the pain is bilateral, but not on both sides simultaneously [4]. The distribution of pain most often involves the V2 and/or V3 subdivisions of the trigeminal nerve [1]. The V1 subdivision is involved in <5 percent of patients. Of note, V1 is most commonly affected by postherpetic neuralgia. (See "Postherpetic neuralgia".)
Trigger zones in the distribution of the affected nerve may be present and are often located near the midline. Lightly touching these zones often triggers an attack, leading patients to protect these areas. Trigger zones can sometimes be demonstrated on physical examination.
Other triggers of TN paroxysms include chewing, talking, brushing teeth, cold air, smiling, and/or grimacing.
Some patients have a history of "pretrigeminal neuralgia," which is said to be dull, continuous, aching pain in the jaw evolving eventually into TN. This brief, milder pain is sometimes suspected to have a dental origin and unnecessary dental procedures have been performed in many cases. On the other hand, TN can be precipitated by dental procedures (eg, dental extraction), resulting in increased confusion about the precise etiology of this problem [27].
The course of TN is variable. Episodes may last weeks or months, followed by pain-free intervals. Recurrence is common, and some patients have continuous pain. Most often, the condition tends to wax and wane in severity and frequency of pain exacerbations. However, there are no pure natural history studies of TN, most likely because the severity of the pain leads to intervention [28].
DIAGNOSIS - The diagnosis of TN is based upon the characteristic clinical features described above, primarily paroxysms of pain in the distribution of the trigeminal nerve.
Once the diagnosis of TN is suspected or confirmed on clinical grounds, a search for secondary causes should be undertaken. Patients with trigeminal sensory loss or bilateral involvement are probably at higher risk of secondary TN [29]. Younger age is also probably associated with a higher risk of secondary TN. However, age is not a clinically useful predictor for distinguishing classic from secondary TN because there is considerable age overlap. In addition, absence of any of these clinical features (sensory loss, bilateral involvement, younger age) does not rule out secondary TN.
Neuroimaging and trigeminal reflex testing are considered useful for distinguishing patients with classic TN (ie, idiopathic or caused by vascular compression) from those with secondary TN (ie, caused by structural brain lesion other than vascular compression) [29].
Diagnostic criteria - The International Headache Society (IHS) diagnostic criteria for classic TN are as follows [1]:
Paroxysmal attacks of pain lasting from a fraction of a second to two minutes, affecting one or more divisions of the trigeminal nerve
Pain has at least one of the following characteristics:
Intense, sharp, superficial, or stabbing
Precipitated from trigger areas or by trigger factors
Attacks are stereotyped in the individual patient
There is no clinically evident neurologic deficit
Not attributed to another disorder
Secondary (symptomatic) TN is characterized by pain indistinguishable from classic TN, but is caused by a demonstrable structural lesion other than vascular compression [1]. Unlike classic TN, there is no refractory period after a paroxysm of pain. Secondary TN may exhibit sensory impairment in the distribution of the appropriate trigeminal nerve [1].
Neuroimaging - Neuroimaging with head CT or MRI is useful for identifying the small proportion of patients who have a structural lesion (eg, tumor in the cerebellopontine angle, demyelinating lesions including multiple sclerosis) as the cause of secondary TN [29,30]. In addition, high resolution MRI and magnetic resonance angiography (MRA) may be useful for identifying vascular compression as the etiology of classic TN, but the utility of these studies has not been established.
While specific evidence-based recommendations regarding the indications for neuroimaging in patients with TN cannot be made, we suggest obtaining brain MRI for patients in the following groups to rule out a causative structural brain lesion:
Patients with trigeminal sensory loss
Patients with bilateral symptoms
Young patients (under the age of 40)
Some clinicians obtain an imaging study in all patients who present with TN.
An evidence-based systematic review and practice parameter published in 2008 from the American Academy of Neurology (AAN) and the European Federation of Neurological Societies (EFNS) identified four studies that evaluated consecutive patients with TN and normal neurologic examinations with head CT or MRI [29]. In the pooled data, routine brain imaging identified a secondary cause of TN (other than vascular compression) in 15 percent of patients (95% CI 11-20 percent).
The 2008 AAN/EFNS practice parameter identified seven studies that performed high-resolution brain MRI and/or magnetic resonance angiography (MRA) to identify neurovascular compression in patients with TN [29]. The following observations were made:
There was wide variation among the included studies for both sensitivity (range 52 to 100 percent) and specificity (29 to 93 percent)
In three of the five highest quality MRI studies (cohort surveys with prospective data collection), the difference in rate of neurovascular trigeminal nerve compression on the symptomatic side compared with asymptomatic side was statistically nonsignificant.
Given these inconsistent results, the AAN/EFNS concluded that there is insufficient evidence to support or refute the utility of MRI to identify neurovascular compression in classic TN, or to indicate the most reliable MRI technique [29].
Electrophysiologic tests - Electrophysiologic trigeminal reflex testing is probably useful for distinguishing classic TN from secondary TN [29]. In contrast, testing with trigeminal evoked potentials is not useful for making this distinction [29].
Trigeminal reflex tests include the blink reflex (obtained by recording from the orbicularis oculi muscles after electrical stimulation of the supraorbital nerve [V1]) and the masseter inhibitory reflex (obtained after electrical stimulation of the infraorbital [V2] and mental [V3] nerves). The responses are recorded by surface electrodes using standard electromyography equipment [31]. These tests are usually normal in patients with classic TN.
The 2008 AAN/EFNS practice parameter identified five studies that addressed the accuracy of trigeminal reflex testing in the evaluation of TN [29]. One was a prospective report of 120 consecutive patients with TN [32], while the remaining four studies were retrospective [33-36]. The pooled sensitivity and specificity of trigeminal reflex testing for distinguishing secondary and classic TN was 94 percent (95% CI 91-97) and 87 percent (95% CI 77-93). Based on high sensitivity and specificity, the AAN/EFNS concluded that abnormal trigeminal reflexes are associated with an increased risk of secondary TN.
The 2008 AAN/EFNS practice parameter identified four studies that used trigeminal evoked potentials to distinguish secondary from classic TN and found that the pooled sensitivity and specificity was 84 percent (95% CI 73-92) and 64 percent (95% CI 56-71) [29]. However, many patients with classic TN had abnormal evoked potentials and many with secondary TN had normal evoked potentials. Thus, trigeminal evoked potentials were not considered clinically useful for distinguishing classic from secondary TN.
Differential diagnosis - The differential diagnosis of TN includes short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT), cluster-tic syndrome, jabs and jolts syndrome, and other neuralgias. (See "SUNCT and SUNA headache syndromes: Clinical features and diagnosis".)
MEDICAL THERAPY - Pharmacologic therapy is the initial treatment of most patients with classic TN (ie, TN that is idiopathic or caused by neurovascular compression). Surgery is reserved for patients who are refractory to medical therapy.
Carbamazepine is the best studied treatment for classic TN and is established as effective [28,29,37]. Side effects can be a problem but are generally manageable, particular if low doses are prescribed initially with gradual titration.
A systematic review and practice parameter published in 2008 from the American Academy of Neurology (AAN) and the European Federation of Neurological Societies (EFNS) concluded that carbamazepine is effective for controlling pain in patients with classic TN, oxcarbazepine is probably effective, and baclofen, lamotrigine, and pimozide are possibly effective [29]. There is limited data and uncertain effectiveness regarding other drugs that have been used for TN, including clonazepam, gabapentin, phenytoin, tocainide, tizanidine, and valproate.
Periodic attempts to gradually withdraw these drugs are warranted in patients achieving relief of pain with oral medications.
No placebo-controlled trials have evaluated the treatment of secondary TN (ie, TN caused by a structural lesion other than vascular compression) and no medications have been established as effective for secondary TN. Treatment of the underlying condition (eg, multiple sclerosis) is recommended, if feasible. In addition, it is reasonable to treat the pain associated with secondary TN using the same medications that are employed in classic TN.
Carbamazepine - Four randomized, controlled trials with a total of 147 patients have established the effectiveness of carbamazepine (200 to 2400 mg daily) for TN [38-41].
A systematic review and practice parameter published in 2008 from the American Academy of Neurology (AAN) and the European Federation of Neurological Societies (EFNS) noted that the treatment response in these trials was robust, with complete or near complete pain control attained in 58 to 100 percent of patients on carbamazepine, compared with 0 to 40 percent of patients on placebo [29]. For the outcome of important pain relief, the number needed to treat was <2. However, carbamazepine was sometimes poorly tolerated, with numbers needed to harm for minor and severe adverse events of 3 and 24 respectively.
The usual starting dose of carbamazepine is 100 to 200 mg twice daily. The dose can be increased gradually in increments of 200 mg daily as tolerated until sufficient pain relief is attained. The typical total maintenance dose is 600 to 800 mg daily, given in two divided doses for tablets and extended release capsules, or four divided doses when for oral suspension. The maximum suggested total dose is 1200 mg daily.
Adverse effects of carbamazepine include drowsiness, dizziness, nausea and vomiting; slow titration may minimize these effects. Carbamazepine-induced leukopenia is not uncommon, but it is usually benign; aplastic anemia is a rare side effect. (See "Pharmacology of antiepileptic drugs", section on 'Carbamazepine'.)
The HLA-B*1502 allele is a genetic susceptibility marker in Asians that is associated with an increased risk of developing Stevens-Johnson syndrome and/or toxic epidermal necrolysis. For most patients of Asian ancestry, genetic testing for the presence of this marker is recommended by the manufacturer prior to initiation of carbamazepine.
Oxcarbazepine - Oxcarbazepine, an analogue of carbamazepine, was developed to retain the antineuralgic effect of carbamazepine while reducing side effects.
The AAN/EFNS practice parameter identified several randomized controlled trials that compared oxcarbazepine (600 to 1800 mg daily) with carbamazepine in 178 patients with classic TN [29]. In the pooled analysis, both medications were equally effective, with a >50 percent reduction of attacks achieved by 88 percent or more of patients in both treatment groups.
Oxcarbazepine can be started at a total dose of 600 mg daily, given in two divided doses. The dose can be increased as tolerated in 300 mg increments every third day to a total dose of 1200 to 1800 mg daily.
Baclofen - Limited evidence from a small double-blind crossover trial suggests that baclofen is beneficial for TN [42]. Treatment with baclofen 40 to 80 mg daily resulted in a reduction in paroxysms in seven of 10 patients with typical TN, compared with one of 10 who received placebo. [42].
The starting dose of baclofen is 15 mg daily given in three divided doses, with gradual titration to a maintenance dose of 50 to 60 mg per day. Sedation, dizziness, and dyspepsia can occur with treatment, and the drug should be discontinued slowly since seizures and hallucinations have been reported with upon withdrawal.
Lamotrigine - In a double-blind, placebo-controlled crossover study of 14 patients with TN that was refractory to carbamazepine or phenytoin, adjunct therapy with lamotrigine (400 mg daily) was beneficial for improvement on a composite outcome index [43]. Patients continued taking either carbamazepine or phenytoin for the duration of the trial.
Similarly, an open-label study found that lamotrigine was beneficial 11 of 15 patients with TN once the 400 mg dose was reached [44]. However, the clinical utility of lamotrigine for severe pain is limited by the need to titrate the dose over many weeks [28].
In patients who are not taking other anticonvulsants, lamotrigine is typically started at 25 mg daily for the first two weeks, and then increased to 50 mg daily for weeks three and four. The dose is then titrated to effect, increasing by 50 mg daily every one to two weeks. The suggested total dose of 400 mg daily is given in two divided doses.
For patients taking an anticonvulsant drug that induces hepatic enzymes (eg, carbamazepine, phenytoin, or primidone), the initial dose of lamotrigine is 50 mg once daily, titrating upward as needed to 100 mg once daily at week three, 200 mg once daily at week five, 300 mg once daily at week six, and 400 mg once daily at week seven.
For patients taking valproate, the initial dose of lamotrigine is 12.5 to 25 mg every other day, with increases of 25 mg every two weeks as needed to a maximum of 400 mg per day.
Pimozide - Pimozide, a dopamine receptor antagonist, was more effective than carbamazepine in a randomized, double-blind crossover trial of 48 patients with refractory TN [45]. There were no drop-outs among patients taking pimozide. However, pimozide is seldom used because it has many potentially serious side effects, including sedation, arrhythmias, anticholinergic effects, acute extrapyramidal symptoms and parkinsonism.
Other medications - Tizanidine appeared to be more effective than placebo in a small one-week trial, but patients who continued the drug in follow-up developed recurrent attacks of TN within one to three months [46].
Tocainide was as effective as carbamazepine at two weeks in a small cross-over trial [47,48].
Small open label studies have suggested benefit with a number of medications used for TN [49]:
Phenytoin [50] and intravenous phenytoin [51]
Fosphenytoin [52]
Valproic acid [53,54]
Gabapentin [55]
Pregabalin [56,57]
Clonazepam [58,59]
Topiramate [60]
Misoprostol, in patients with TN and multiple sclerosis [61]
However, these agents have not been studied in controlled trials, and their effectiveness in TN is not established.
Although there are no controlled data regarding the efficacy of opioids in TN specifically, we have used opiates in patients with acute exacerbations of pain lasting for days to weeks. Opiates may help make the pain bearable while other, more effective and long-term, treatments take effect. Our experience with opiates suggests partial analgesia with central side effects (particularly sedation) when these drugs are used alone, as high doses of morphine, hydromorphone or oxycodone are usually required. In combination with other neuropathic analgesics, opiates seem to be more effective at lower doses.
Refractory pain - There is little evidence to support treatment alternatives for patients who are refractory to first-line medical therapy. However, some patients who fail carbamazepine monotherapy may benefit from combination therapy with gabapentin, lamotrigine, topiramate, baclofen, or tizanidine. Intravenous infusion of phenytoin, fosphenytoin or lidocaine may provide analgesia while oral medications are titrated [51,62]. Phenytoin and fosphenytoin are dosed at 250 to 1000 mg intravenously [51] at no more than 50 mg/minute and lidocaine is given at 100 to 300 mg [62] over one-half hour while monitoring pulse and blood pressure.
Nevertheless, there are no randomized controlled trials comparing monotherapy with combination therapy for TN.
SURGICAL THERAPY - Patients with TN who are refractory to medical therapy are candidates for surgery. A variety of surgical methods have been employed to relieve the symptoms of TN. The major types of procedures are [28]:
Microvascular decompression
Ablative procedures, including:
Rhizotomy with either radiofrequency thermocoagulation, mechanical balloon compression, or chemical (glycerol) injection
Radiosurgery
Peripheral neurectomy and nerve block
However, few have been studied in controlled trials, and most of the evidence comes from observational studies [63].
A systematic review and practice parameter published in 2008 from the American Academy of Neurology (AAN) and the European Federation of Neurological Societies (EFNS) concluded that microvascular decompression, percutaneous procedures on the Gasserian ganglion (rhizotomy), and gamma knife radiosurgery are possibly effective in the treatment of TN [29]. Evidence for peripheral neurectomy was considered negative or inconclusive.
The AAN/EFNS noted that definitive conclusions regarding the relative effectiveness of surgical techniques for TN are precluded by the lack of studies directly comparing them [29]. Indirect comparisons of the findings from different surgical studies suggest that microvascular decompression has a longer duration of pain control than other surgical interventions for TN.
Microvascular decompression is invasive, although the overall mortality and complication rates are low. Ablative procedures are less invasive, but recurrence may be more common. The incidence of facial numbness is higher with rhizotomy procedures than with microvascular decompression or gamma knife radiosurgery.
Although surgical therapy for TN is generally well-tolerated, a feared complication is anesthesia dolorosa, a condition characterized by persistent, painful anesthesia or hypesthesia in the denervated region [1]. It can be more intolerable than the pain from TN itself [64]. This risk warrants careful decision making when considering surgical treatment for TN.
Anesthesia dolorosa most frequently occurs as a complication of rhizotomy or thermocoagulation for TN, but is rarely if ever a complication of gamma knife surgery.
Microvascular decompression - Microvascular decompression is a major neurosurgical procedure that involves craniotomy and the removal or separation of various vascular structures, often an ectatic superior cerebellar artery, away from the trigeminal nerve [65].
The AAN/EFNS practice parameter identified five studies of microvascular decompression for TN [66-70] that used independent outcome assessment [29]. The practice parameter concluded that initial pain relief is attained in 90 percent of patients, but that pain-free rates decline by one, three, and five years to 80, 75, and 73 percent.
The average mortality is approximately 0.2 percent. However, major adverse events, such as cerebrospinal fluid leaks, infarction or hematoma, occur in up to 4 percent of patients [29].
The most common complication is aseptic meningitis in 11 percent of patients [29]. Long term hearing loss occurs in up to 10 percent of patients, and sensory loss is found in 7 percent.
Rhizotomy - Rhizotomy encompasses a number of percutaneous surgical techniques that are performed by passing a cannula through the foramen ovale, followed by lesion of the trigeminal ganglion or root using one of several options [71]:
Radiofrequency thermocoagulation rhizotomy, which creates a lesion by application of heat
Mechanical balloon compression, which uses a Fogarty catheter to compress the gasserian ganglion
Chemical (glycerol) rhizolysis, which involves the injection of 0.1 to 0.4 mL of glycerol into the trigeminal cistern
The 2008 AAN/EFNS practice parameter identified four uncontrolled case series that used independent outcome assessment of these procedures [29], including two reports of radiofrequency thermocoagulation [72,73], one report of glycerol rhizolysis [74], and one of balloon compression [75]. The AAN/EFNS found that initial pain relief is achieved in 90 percent of patients, but that pain-free rates decline by one year to 68 to 85 percent, by three years to 54 to 64 percent, and by five years to approximately 50 percent [29].
The major perioperative complication after rhizotomy procedures is meningitis, mainly aseptic, seen in 0.2 percent [29]. Mortality is rare. Postoperative dysesthesia, described as a burning, heavy, aching, or tired feeling, occurs in 12 percent. Longer-term sequelae include trigeminal distribution sensory loss in nearly one-half of patients, anesthesia dolorosa in approximately 4 percent, and corneal numbness with risk of keratitis in 4 percent.
Radiosurgery - Gamma knife radiosurgery produces lesions with focused gamma radiation [76]. (See "Stereotactic cranial radiosurgery", section on 'Gamma Knife'.)
The therapy is aimed at the proximal trigeminal root since targeting the gasserian ganglion produced poor results [77]. The aiming of the beams is carried out with a stereotactic frame and MRI. The doses used are 70 to 90 Gy. The beams cause axonal degeneration and necrosis [77]. Pain relief with gamma knife surgery occurs after a lag time of about one month [77,78].
The 2008 AAN/EFNS practice parameter [29] identified one randomized controlled trial of gamma knife surgery for TN that compared two different treatment regimens [79] and found no important differences. In addition, the AAN/EFNS [29] identified three case series with independent outcome assessment [80-82]. Complete pain relief at one year was found in up to 69 percent of patients, and at three years in 52 percent [29]. An earlier systematic review found that approximately 75 percent of patients report complete relief within three months, but the proportion decreases to 50 percent by three years [83].
New or worsened facial sensory impairment occurred in 9 to 37 percent, with more bothersome sensory loss or paresthesia found in 6 to 13 percent of patients [29]. However, anesthesia dolorosa is rarely, if ever, a complication of gamma knife surgery.
Linear accelerator radiosurgery has been evaluated for the treatment of TN in retrospective case series [84,85], but no prospective studies or controlled trials are available. (See "Stereotactic cranial radiosurgery", section on 'Linac'.)
Peripheral neurectomy - Peripheral neurectomy can be performed on the branches of the trigeminal nerve, which are the supraorbital, infraorbital, alveolar, and lingual nerves. Neurectomy is accomplished by incision, alcohol injection, radiofrequency lesioning, or cryotherapy. Cryotherapy involves freezing of the nerve using special probes, in theory to selectively destroy the pain fibers.
The AAN/EFNS practice parameter noted that the evidence regarding peripheral techniques for the treatment of TN is either negative or inconclusive [29].
