Neuropathic Facial Pain

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2 Neuropathic Facial Pain Kevin Weber, MD, MHA Assistant Professor of Neurology, Headache Division The Ohio State University Neurological Institute Department of Neurology

3 Disclosures I have no disclosures. I will be discussing the off-label use of treatments in this talk.

4 Neuropathic Facial Pain Trigeminal neuralgia and neuropathy Glossopharyngeal neuralgia Nervus intermedius neuralgia Other less common facial pain disorders Excluded from this talk: Trigeminal autonomic cephalgias (TACs) and occipital neuralgia.

5 Trigeminal Neuralgia ICHD-3 criteria: At least three attacks of unilateral facial pain fulfilling the following criteria: In one or more division of the trigeminal nerve but not beyond that distribution 3 of the follow 4 characteristics: Paroxysmal attacks lasting fraction of a second to 2 minutes Severe pain Electric shock-like, stabbing, shooting, or sharp Precipitated by innocuous stimuli to that side of the face No clinically evident neurologic deficits

6 Source: ufhealth.org Trigeminal Neuralgia

7 Classic TN vs. Neuropathy Classic: idiopathic cause or later discovered to be caused by neurovascular compression Trigeminal neuropathy: other secondary causes, such as herpes zoster, multiple sclerosis, tumor, etc. Source: ICHD-3

8 Clinical Features V3 or V2 > V1 Older patients > 50, women > men Pain provoked by triggers with refractory period after repeated triggers Can have constant dull pain in between, rarely occurs during sleep No autonomic features although tearing has been described Remissions are possible Source: Mays, M.M., Tepper, D.E., and Tepper, S.J. (2014) Diagnosis of Major Secondary Headaches, Nonvascular Disorders. In The Cleveland Clinic Manual of Headache Therapy (pp ).Cham, Switzerland: Springer.

9 TN vs. TACs There can be some diagnostic confusion with TN vs. TACs, particularly SUNCT/SUNA When TN is in V1 (rare), there can be diagnostic overlap. TN has refractory period, SUNCT/SUNA does not TN should have no autonomic features SUNCT/SUNA can have sawtooth pattern of attacks, although not always Some treatment overlap between the disorders, I am working on a systematic review on this currently. Source: Mays, M.M., Tepper, D.E., and Tepper, S.J. (2014) Diagnosis of Major Secondary Headaches, Nonvascular Disorders. In The Cleveland Clinic Manual of Headache Therapy (pp ).Cham, Switzerland: Springer.

10 Diagnostic Imaging Obtain a brain MRI with and without contrast when assessing a patient with TN. Add FIESTA or MP- RAGE (depending on software at your institution) and 3D CISS (constructive interference in steady state) sequence. If there is neurovascular compression and the patient is refractory to medication, he can be assessed for gamma knife treatment or microvascular decompression at a center with neurosurgeons who perform these procedures. Imaging also will help identify other secondary lesions causing trigeminal neuropathy.

11 Treatments Systematic Review for AAN in 2008: Effective: Carbamazepine Probably effective: oxcarbazepine Possibly effective: lamotrigine, pimozide, baclofen Screen for HLA allele B*1502 in patients of Asian decent before starting carbamazepine or oxcarbazepine Consider IV phenytoin or lidocaine for acute severe exacerbations, or SPG block Gronseth G, Cruccu G, Alksne J, et al. (2008). Practice parameter: the diagnostic evaluation and treatment of trigeminal neuralgia (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology and the European Federation of Neurological Societies. Neurology; 71: McCleane GJ. (1999). Intravenous infusion of phenytoin relieves neuropathic pain: a randomized, double-blinded, placebo-controlled, crossover study. Anesth Analg, 89: 985. Scrivani SJ, Chaudry A, Maciewicz RJ, Keith DA. (1999). Chronic neurogenic facial pain: lack of response to intravenous phentolamine. J Orofac Pain, 13: 89.

12 Other Treatments Clonazepam, gabapentin, pregabalin, topiramate, tizanidine, levetiracetam, lacosamide Ziconotide (intrathecal infusion), an N-type calcium channel blocker Benoliel, R., Heir, G.M., Eliav, E. (2015). Neuropathic orofacial pain. In Orofacial Pain & Headache (pp ). Philadelphia, PA: Elsevier Limited.

13 OnabotulinumtoxinA Several open label and RCTs supporting onabotulinumtoxina use in trigeminal neuralgia. Recent systematic review by Morra et. al shows that onabotulinumtoxina is safe and efficacious in trigeminal neuralgia Adverse events included facial edema and asymmetry Different studies used different amounts of onabotulinumtoxina and in different locations, further studies are needed to determine optimal amount and location

14 OnabotulinumtoxinA Overall, the main effect (patients with >50% reduction of pain score from baseline to endpoint favored botulinum toxin A: (RR = 2.87, 95 % CI [1.76, 4.69], P < ). Mean VAS score was significantly lower for the botulinum toxin A group at the end of month 1, 2, and 3. Mean paroxysm frequency per day was also significantly lower in the botulinum toxin A group. Followup ranged 8-12 weeks. Morra et al. (2016). Therapeutic efficacy and safety of Botulinum Toxin A Therapy in Trigeminal Neuralgia: a systematic review and metaanalysis of randomized controlled trials. The Journal of Headache and Pain. 17: 63.

15 Painful tic convulsif Not in ICHD-3, but described in literature as coexistent trigeminal neuralgia and hemifacial spasm Usually caused by neurovascular compression in posterior fossa Several case studies showing efficacy for botulinum toxin A in this disorder. Bosca-Blasco ME, Burguera Hernandez A, Roig-Morata S et al. (2006). Painful tic convulsif and botulinum toxin. Rev Neurol 42: Micheli F, Scorticati MC, Raina G. (2002). Beneficial effects of botulinum toxin type a for patients with painful tic convulsif. Clin Neuropharmacol 25:

16 Glossopharyngeal Neuralgia ICHD-3 criteria: At least three attacks of unilateral pain fulfilling the following criteria: Pain is located in the posterior part of the tongue, tonsillar fossa, pharynx, beneath the angle of the lower jaw and/or in the ear Pain has 3 of the following 4: Recurring in paroxysmal attacks lasting from a few seconds to 2 minutes Severe Shooting, stabbing, or sharp Triggered by swallowing, coughing, talking, or yawning No neurologic deficit

17 Glossopharyngeal Neuralgia Must rule out secondary cause, including Eagle syndrome: Elongation of the styloid process or calcification of the stylohyoid ligament. Pain in neck with head position change, pain with palpation of styloid process, foreign body sensation in throat, hypersalivation, otalgia, dysphagia, voice change Can even cause carotid dissection Diagnosis: lateral views of skull base and cervical spine Most cases acquired, trauma #1 Treatment usually surgical Kim E, Hansen K, Frizzi J. (2008). Eagle syndrome: case report and review of the literature. Ear Nose Throat J, 87:631.

18 Eagle Syndrome

19 Glossopharyngeal Neuralgia Glossopharyngeal neuralgia can even cause cardiac syncope in rare cases, due to nerve fibers connected to carotid sinus and dorsal motor nucleus of CN X affected. Treatment medically is similar to that of trigeminal neuralgia. Surgical treatment includes nerve sectioning and microvascular decompression. Check MRI brain with and without contrast with FIESTA or MP-RAGE (depending on software at your institution) and 3D CISS sequence like with trigeminal neuralgia. Patel A, Kassam A, Horowitz M, Chang YF. (2002). Microvascular decompression in the management of glossopharyngeal neuralgia: analysis of 217 cases. Neurosurgery, 50:705.

20 Nervus intermedius Neuralgia Small sensory branch of the facial nerve (cranial nerve VII) carrying general visceral efferent, special visceral afferent (taste), and general somatic afferent fibers ICHD-3 criteria: Three attacks of unilateral pain fulfilling the following criteria: Located in auditory canal, can radiate to parieto-occipital area 3 of the following 4 criteria: Recurring in paroxysmal attacks lasting from a few seconds to minutes Severe Shooting, stabbing, or sharp Precipitated by stimulation of a trigger area in the posterior wall of the auditory canal and/or periauricular region No neurologic deficit

21 Nervus intermedius Neuralgia

22 Ramsay-Hunt Nervus intermedius neuralgia can be secondary to mass, vascular compression, or reactivation of herpes zoster virus (Ramsay-Hunt syndrome). Check MRI IAC and MRA. Triad of ipsilateral facial paralysis, ear pain, and vesicles in the auditory canal and auricle. Taste, hearing, lacrimation, vestibular function can also be affected. Reactivation of virus in geniculate ganglion affecting multiple CNs. Treated with steroids like Bell s palsy, no evidence for efficacy of antivirals despite widespread use. Uscategui T, Dorée C, Chamberlain IJ, Burton MJ (2008). Antiviral therapy for Ramsay Hunt syndrome (herpes zoster oticus with facial palsy) in adults. Cochrane Database Syst Rev, CD

23 Nervus intermedius Neuralgia Again, treated similarly to trigeminal neuralgia when no major secondary cause is found. Surgical treatment includes nerve sectioning (with complications including partial facial palsy, ipsilateral xerophthalmia) or microvascular decompression. Pulec JL. (2008). Geniculate neuralgia: long-term results of surgical treatment. Ear Nose Throat J, 81:30. Lovely TJ, Jannetta PJ (1997). Surgical management of geniculate neuralgia. Am J Otol, 18:512.

24 Tolosa-Hunt Unilateral orbital pain associated with paresis of one or more of the IIIrd, IVth and/or VIth cranial nerves caused by a granulomatous inflammation in the cavernous sinus, superior orbital fissure or orbit. Unknown etiology Pain usually precedes ophthalmoparesis CN V1 and pericarotid sympathetic fibers can be affected as well, causing Horner s Hung CH, Chang KH, Chen YL, et al. (2015) Clinical and radiological findings suggesting disorders other than tolosa-hunt syndrome among ophthalmoplegic patients: a retrospective analysis. Headache, 55: 252.

25 Tolosa-Hunt Investigation: MRI with and without contrast, MRV CBC, CMP, inflammatory markers, autoimmune workup, lyme ab, SPEP, ACE. CSF studies: glucose, cell count with diff, protein, lyme, VDRL, cultures, ACE, cytology Prompt response to corticosteroids confirms diagnosis High dose IV solumedrol or high dose prednisone followed by taper of weeks to months. Radiographic improvement lags behind clinical improvement. Kline LB, Hoyt WF (2001). The Tolosa-Hunt syndrome. J Neurol Neurosurg Psychiatry, 71:577. Cakirer S (2003). MRI findings in Tolosa-Hunt syndrome before and after systemic corticosteroid therapy. Eur J Radiol, 45:83.

26 Raeder s syndrome Also known as Paratrigeminal oculosympathetic syndrome Constant, unilateral pain in the distribution of the ophthalmic division of the trigeminal nerve, sometimes extending to the maxillary division, accompanied by Horner s syndrome and caused by a disorder in the middle cranial fossa or of the carotid artery. Recommend immediate MRI /MRV w/wo contrast and MRA of brain and neck, or CTA/ CTV

27 Source: aao.org Raeder s syndrome

28 Horner s syndrome tip For help distinguishing 1st/2nd from 3rd order sympathetic pathology, use 4- hydroxyamphetamine drops If third order is intact, 4-hydroxyamphetamine will cause neurotransmitter release and pupil dilation If injured, there will be no dilation because the third order neuron releasing norepinephrine is damaged.

29 Burning mouth syndrome An intraoral burning or dysaesthetic sensation, recurring daily for more than 2 hours per day over more than 3 months, without clinically evident causative lesions. Rule out nutritional deficiency, xerostomia, HSV, allergic contact stomatitis. Otherwise considered idiopathic.?small fiber trigeminal neuropathy Usually start with TCAs, gabapentin. Systematic review also found alpha-lipoic acid (3 trials), klonopin (1 trial), CBT (1 trial) helped. Lauria G, Majorana A, Borgna M, et al (2005). Trigeminal small-fiber sensory neuropathy causes burning mouth syndrome. Pain, 115:332. Zakrzewska JM, Forssell H, Glenny AM (2005). Interventions for the treatment of burning mouth syndrome. Cochrane Database Syst Rev, CD

30 Atypical Facial Pain or Persistent Idiopathic Facial Pain Often used as a catch-all for facial pain not falling into previous criteria. Often lumped in with central-post stroke pain and central neuropathic pain attributed to MS. ICHD-3: A. Facial and/or oral pain fulfilling criteria B and C B. Recurring daily for >2 hours per day for >3 months C. Pain has both of the following characteristics: 1. poorly localized, and not following the distribution of a peripheral nerve 2. dull, aching or nagging quality D. Clinical neurological examination is normal E. A dental cause has been excluded by appropriate investigations

31 Atypical Facial Pain or Persistent Idiopathic Facial Pain Very little quality evidence for any particularly interventions. Four RCTs have been done in persistent idiopathic facial pain: venlafaxine, fluoxetine, sumatriptan subq, dothiepin (not available in U.S.) Harrison et. al found additional benefit with CBT in study with fluoxetine. Harrison, S. D., Glover, L., Feinmann, C., Pearce, S. A., & Harris, M. (1997). A comparison of antidepressant medication alone and in conjunction with cognitive behavioral therapy for chronic idiopathic facial pain. Proceedings of the 8th World Congress on Pain: Progress in Pain Research and Management, 8, Zakrzewska JM (2016). Chronic/Persistent Idiopathic Facial Pain. Neurosurg Clin N Am 27:

32 Atypical odontalgia Subform of persistent idiopathic facial pain Continuous toothache without any dental pathology Recent study cited 4 cases of efficacy of different periods, at different sites, of botulinum toxin A for atypical odontalgia. Cuadrado ML, Garcia-Moreno H, Arias JA, Pareja JA (2016). Botulinum Neurotoxin Type-A for the Treatment of Atypical Odontalgia. Pain Medicine 2016; 0: 1 5.

33 Central post-stroke pain Depressingly, a systematic review found no conclusive benefit for any interventions in 8 eligible RCTs. Anticonvulsants, TCAs, naloxone, acupuncture While often these therapies and also SNRIs and mexilitene, among others, are used in various types of atypical facial pain, there is very little evidence supporting their efficacy as of yet. Mulla SM et. al. (2015). Management of Central Poststroke Pain: Systematic Review of Randomized Controlled Trials. Stroke, 46:

34 Others Optic neuritis Headache attributed to ischemic ocular motor nerve palsy Recurrent painful ophthalmoplegic neuropathy (formerly ophthalmoplegic migraine) treat with steroids. MRI enhancement.

35 Thank You!