trigeminal neuralgia & other algies of the...

Trigeminal Neuralgia & Other Algies of the Face

Abdullah M Kaki, MD, FRCPC

Professor & Consultant of Anesthesiology & Pain Medicine

Faculty of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia

Dubai Pain Diploma, May 2017

Disclosure

I have nothing to disclose

Case I

´  53 year old female patient, c/o R facial pain for the last 2 yrs. Sudden onset, severe, exaggerated by chewing, swallowing and teeth brushing. Seen by dentist who extracted all her wisdom teeth, and referred her to you

´  What is your management approach?

Trigeminal neuralgia




History

•  John Fothergill was first to describe TN, in 1773 (Medical Society of London entitled “Of a Painful Affliction of the Face.” • 14 pts, most elderly women. • Brief and sudden bouts of sharp, excruciating

pain, triggered by light touch or eating.

1. Fothergill J. Of a painful affection of the face. Medical Observations and Inquiries. 1773;5:129–142. 2. Rose FC. Trigeminal Neuralgia. Arch Neurol. 1999;56:1163–1164.


´  Recurrent brief episodes of unilateral electric shock-like pains,

´  Abrupt in onset and termination,

´  Distribution of one or more divisions of the fifth cranial

´  Triggered by innocuous stimuli.

Headache Classification Committee of the International Headache Society (IHS). The International Classification of Headache Disorders, 3rd edition (beta version). Cephalalgia 2013; 33:629.

ANATOMY

´  TN is the sensory supply to face, and sensory and motor supply to the muscles of mastication. It has three major divisions:

●Ophthalmic (V1)

●Maxillary (V2)

●Mandibular (V3)

´  The nerve starts at the midlateral surface of the pons, and its sensory ganglion (gasserian ganglion) resides in Meckel's cave in the floor of the middle cranial fossa

EPIDEMIOLOGY

´  TN is a rare condition that affects women > men.

´  The annual incidence of TN is 4 -13 /100,000 people.

´  The incidence increases gradually with age; most idiopathic cases begin after age 50, although onset may occur in the second and third decades or, rarely, in children.

´  Male to female prevalence ratio: 1:1.5 - 1:1.7 (increased longevity of women).

´  Rare familial cases have been reported.

´  HTN may be a risk factor for TN, migraine is a risk factor for TN.

See references in next slide

References

´  Katusic S, Williams DB, Beard CM, et al. Epidemiology and clinical features of idiopathic trigeminal neuralgia and glossopharyngeal neuralgia: similarities and differences, Rochester, Minnesota, 1945-1984. Neuroepidemiology 1991; 10:276.

´  MacDonald BK, Cockerell OC, Sander JW, Shorvon SD. The incidence and lifetime prevalence of neurological disorders in a prospective community-based study in the UK. Brain 2000; 123 ( Pt 4):665.

´  Childs AM, Meaney JF, Ferrie CD, Holland PC. Neurovascular compression of the trigeminal and glossopharyngeal nerve: three case reports. Arch Dis Child 2000; 82:311.

´  Katusic S, Beard CM, Bergstralh E, Kurland LT. Incidence and clinical features of trigeminal neuralgia, Rochester, Minnesota, 1945-1984. Ann Neurol 1990; 27:89.

´  Maarbjerg S, Gozalov A, Olesen J, Bendtsen L. Trigeminal neuralgia--a prospective systematic study of clinical characteristics in 158 patients. Headache 2014; 54:1574.

´  Fleetwood IG, Innes AM, Hansen SR, Steinberg GK. Familial trigeminal neuralgia. Case report and review of the literature. J Neurosurg 2001; 95:513.

´  Pan SL, Yen MF, Chiu YH, et al. Increased risk of trigeminal neuralgia after hypertension: a population-based study. Neurology 2011; 77:1605.

´  Siqueira SR, Teixeira MJ, Siqueira JT. Clinical characteristics of patients with trigeminal neuralgia referred to neurosurgery. Eur J Dent 2009; 3:207.

´  Teruel A, Ram S, Kumar SK, et al. Prevalence of hypertension in patients with trigeminal neuralgia. J Headache Pain 2009; 10:199.

´  Lin KH, Chen YT, Fuh JL, Wang SJ. Increased risk of trigeminal neuralgia in patients with migraine: A nationwide population-based study. Cephalalgia 2015.

ETIOLOGY AND PATHOGENESIS

´  Mainly compression of nerve root, usually within a few mm of entry into the pons (root entry zone).

´  Compression by an aberrant loop of artery or vein in 80 - 90 %. Idiopathic TN (classic TN).

´  Other causes of TN via nerve compression include vestibular schwannoma (acoustic neuroma), meningioma, epidermoid or other cyst, or, rarely, a saccular aneurysm or a-v malformation.

´  Compression of the nerve => demyelination => ectopic impulse generation (ephaptic transmission =Ephaptic cross-talk between fibers mediating light touch.

´  Evidence for central pain mechanisms: presence of refractory periods after a triggered episode, trains of painful sensations after a single stimulus, and latency from the time of stimulation to the onset of pain.

´  Electrophysiologic evidence of central sensitization of trigeminal nociceptive processing has been observed in patients with atypical TN who have concomitant chronic facial pain.

´  Demyelination of one or more of TN nuclei may be caused by MS or other lesions of the brainstem. In MS, a plaque of demyelination typically occurs in the root entry zone of TN, although vascular compression has been noted.


References

´  Love S, Coakham HB. Trigeminal neuralgia: pathology and pathogenesis. Brain 2001; 124:2347.

´  Bowsher D. Trigeminal neuralgia: an anatomically oriented review. Clin Anat 1997; 10:409.

´  Hamlyn PJ. Neurovascular relationships in the posterior cranial fossa, with special reference to trigeminal neuralgia. 2. Neurovascular compression of the trigeminal nerve in cadaveric controls and patients with trigeminal neuralgia: quantification and influence of method. Clin Anat 1997; 10:380.

´  Headache Classification Committee of the International Headache Society (IHS). The International Classification of Headache Disorders, 3rd edition (beta version). Cephalalgia 2013; 33:629.

´  Cheng TM, Cascino TL, Onofrio BM. Comprehensive study of diagnosis and treatment of trigeminal neuralgia secondary to tumors. Neurology 1993; 43:2298.

´  Linskey ME, Jho HD, Jannetta PJ. Microvascular decompression for trigeminal neuralgia caused by vertebrobasilar compression. J Neurosurg 1994; 81:1.

´  Ildan F, Göçer AI, Bağdatoğlu H, et al. Isolated trigeminal neuralgia secondary to distal anterior inferior cerebellar artery aneurysm. Neurosurg Rev 1996; 19:43.

´  Figueiredo PC, Brock M, De Oliveira AM Júnior, Prill A. Arteriovenous malformation in the cerebellopontine angle presenting as trigeminal neuralgia. Arq Neuropsiquiatr 1989; 47:61.

´  Matthies C, Samii M. Management of 1000 vestibular schwannomas (acoustic neuromas): clinical presentation. Neurosurgery 1997; 40:1.

´  Haddad FS, Taha JM. An unusual cause for trigeminal neuralgia: contralateral meningioma of the posterior fossa. Neurosurgery 1990; 26:1033.

´  Mohanty A, Venkatrama SK, Rao BR, et al. Experience with cerebellopontine angle epidermoids. Neurosurgery 1997; 40:24

´  Love S, Hilton DA, Coakham HB. Central demyelination of the Vth nerve root in trigeminal neuralgia associated with vascular compression. Brain Pathol 1998; 8:1.

´  Hilton DA, Love S, Gradidge T, Coakham HB. Pathological findings associated with trigeminal neuralgia caused by vascular compression. Neurosurgery 1994; 35:299.

´  Fromm GH, Terrence CF, Maroon JC. Trigeminal neuralgia. Current concepts regarding etiology and pathogenesis. Arch Neurol 1984; 41:1204.

´  Obermann M, Yoon MS, Ese D, et al. Impaired trigeminal nociceptive processing in patients with trigeminal neuralgia. Neurology 2007; 69:835.

´  Gass A, Kitchen N, MacManus DG, et al. Trigeminal neuralgia in patients with multiple sclerosis: lesion localization with magnetic resonance imaging. Neurology 1997; 49:1142.

´  Truini A, Prosperini L, Calistri V, et al. A dual concurrent mechanism explains trigeminal neuralgia in patients with multiple sclerosis. Neurology 2016; 86:2094.

´  Meaney JF, Watt JW, Eldridge PR, et al. Association between trigeminal neuralgia and multiple sclerosis: role of magnetic resonance imaging. J Neurol Neurosurg Psychiatry 1995; 59:253.

Classification

´  Classic TN: idiopathic TN & TN 2ry to vascular compression.

´  Painful trigeminal neuropathy caused by lesions other than vascular compression:

ü  Painful trigeminal neuropathy attributed to acute herpes zoster

ü  Postherpetic trigeminal neuropathy

ü  Painful posttraumatic trigeminal neuropathy

ü  Painful trigeminal neuropathy attributed to multiple sclerosis plaque

ü  Painful trigeminal neuropathy attributed to space-occupying lesion

ü  Painful trigeminal neuropathy attributed to other disorder


´  ICHD-3 classification scheme for painful trigeminal neuropathy is problematic.

´  In practice good number of pts with symptoms identical to classic TN and with normal neurologic examinations turn out to have MS or a tumor.

´  Furthermore, such pts often respond well to the same drugs used for classic TN.

Cruccu G, Finnerup NB, Jensen TS, et al. Trigeminal neuralgia: New classification and diagnostic grading for practice and research. Neurology 2016; 87:220.

CLINICAL FEATURES

´  Paroxysmal, stereotyped attacks of usually intense, sharp, superficial, or stabbing pain in distribution of one or more branches of V cranial nerve, maximal at or near onset, might be associated with muscle spasms (tic douloureux).

´  It is electric, shock-like, or stabbing. 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 pts with longstanding TN may have continuous dull pain that is present between paroxysms of pain.

´  Typically does not awaken patients at night.

´  Typically unilateral. Occasionally bilateral.

´  Involves V2 and/or V3 subdivisions.

´  Autonomic symptoms, usually mild or moderate, V1, including lacrimation, conjunctival injection, and rhinorrhea (<5 % of pts).

´  When prominent or severe: ? syndromes of short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache attacks with autonomic symptoms (SUNA).

´  Trigger zones in distribution of affected nerve may present and are often located near midline. Light touch often triggers an attack. Other triggers: chewing, talking, brushing teeth, cold air, smiling, and/or grimacing.

´  “Pretrigeminal neuralgia," a dull, continuous, aching pain in the jaw that evolves over time into TN (dental origin, and unnecessary dental procedures have been performed), TN can be precipitated by dental procedures (eg, dental extraction).

References


´  Maarbjerg S, Gozalov A, Olesen J, Bendtsen L. Trigeminal neuralgia--a prospective systematic study of clinical characteristics in 158 patients. Headache 2014; 54:1574.

´  Zakrzewska JM, Linskey ME. Trigeminal neuralgia. BMJ 2014; 348:g474.

´  Sjaastad O, Pareja JA, Zukerman E, et al. Trigeminal neuralgia. Clinical manifestations of first division involvement. Headache 1997; 37:346.

´  Pareja JA, Barón M, Gili P, et al. Objective assessment of autonomic signs during triggered first division trigeminal neuralgia. Cephalalgia 2002; 22:251.

´  Fromm GH, Graff-Radford SB, Terrence CF, Sweet WH. Pre-trigeminal neuralgia. Neurology 1990; 40:1493.

´  Evans RW, Graff-Radford SB, Bassiur JP. Pretrigeminal neuralgia. Headache 2005; 45:242.

´  Wright E, Evans J. Oral pre-trigeminal neuralgia pain: clinical differential diagnosis and descriptive study results. Cranio 2014; 32:193.

´  von Eckardstein KL, Keil M, Rohde V. Unnecessary dental procedures as a consequence of trigeminal neuralgia. Neurosurg Rev 2015; 38:355.

DIAGNOSIS

´  Clinical features

´  Search for secondary causes

´  Neuroimaging of the brain (CT, MRI with and without contrast).

´  Pts with trigeminal sensory loss or bilateral involvement, younger age pts are probably at higher risk of 2ry TN.

´  Electrophysiologic testing does not distinguish classic TN from 2ry TN and has no role in diagnostic evaluation of TN.

´  In 2008 guideline suggested that trigeminal reflex testing is probably useful for distinguishing classic TN from 2ry TN while trigeminal evoked potentials were not useful for making this distinction.

Gronseth G, Cruccu G, Alksne J, et al. 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 2008; 71:1183.

Diagnostic criteria

●A) At least 3 attacks of unilateral facial pain fulfilling criteria B and C

●B) Occurring in one or more divisions of TN, with no radiation beyond trigeminal distribution

●C) Pain has at least 3 of the following four characteristics:

´  Recurring in paroxysmal attacks lasting from a fraction of a second to two min

´  Severe intensity

´  Electric shock-like, shooting, stabbing, or sharp in quality

´  At least 3 attacks precipitated by innocuous stimuli to affected side of the face (some attacks, spontaneous)

●D) No clinically evident neurologic deficit

●E) Not better accounted for by another ICHD-3 diagnosis

´  Occasional pts have persistent facial pain of moderate intensity in affected area, atypical TN or TN type 2.

´  Hypoesthesia or hypoalgesia in the affected trigeminal region always indicates axonal damage => a trigeminal neuropathy.

´  In contrast, hyperalgesia in painful region should not necessarily lead to a diagnosis of 2ry TN or trigeminal neuropathy because it may reflect increased attention to the painful side

•  Headache Classification Committee of the International Headache Society (IHS). The International Classification of Headache Disorders, 3rd edition (beta version). Cephalalgia 2013; 33:629.

•  Maarbjerg S, Gozalov A, Olesen J, Bendtsen L. Trigeminal neuralgia--a prospective systematic study of clinical characteristics in 158 patients. Headache 2014; 54:1574.

Neuroimaging

´  Neuroimaging with head MRI (or CT) is useful to identify pts with structural lesion (tumor in cerebellopontine angle, demyelinating lesions of MS).

´  MRA may identify vascular compression as etiology of C-TN.

´  In 2014 meta-analysis of 9 blinded case-control studies:

ü  Neurovascular contact of TN on MRI/MRA was significantly more frequent with symptomatic nerves compared with asymptomatic nerves (89 vs 36%).

ü  Accuracy of neurovascular compression on MRI: sensitivity (75 – 95 %) and a specificity (26 - 86 %).

ü  Anatomic change (ie, atrophy, distortion, or flattening) of the trigeminal nerve on MRI at the site of vascular contact was significantly more frequent with symptomatic nerves (53 vs 9 %). Sensitivity (20 -74 %) & specificity (79 -100 %).

•  Gronseth G, Cruccu G, Alksne J, et al. 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 2008; 71:1183.

•  Goh BT, Poon CY, Peck RH. The importance of routine magnetic resonance imaging in trigeminal neuralgia diagnosis. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2001; 92:424.

•  Antonini G, Di Pasquale A, Cruccu G, et al. Magnetic resonance imaging contribution for diagnosing symptomatic neurovascular contact in classical trigeminal neuralgia: a blinded case-control study and meta-analysis. Pain 2014; 155:1464.

Differential Diagnosis

DIFFERENTIAL DIAGNOSIS

´  Acute herpes zoster, postherpetic neuralgia, trauma to the trigeminal nerve, demyelination from MS, and compression of TN by a nonvascular space-occupying lesion.

´  V1 is most commonly affected PHN.

´  Dental causes of pain: pain is usually continuous, intraoral pain that is dull or throbbing, some pts have a phase of "pretrigeminal neuralgia" ( jaw or tooth pain) that might mimic dental pain.

´  Several uncommon causes of headache and craniofacial pain: short-lasting unilateral neuralgiform headache attacks, cluster-tic syndrome, and primary stabbing headache.


•  Maarbjerg S, Gozalov A, Olesen J, Bendtsen L. Trigeminal neuralgia--a prospective systematic study of clinical characteristics in 158 patients. Headache 2014; 54:1574.

●Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache attacks with autonomic symptoms (SUNA):

Sudden brief attacks of severe unilateral head pain in orbital, peri-orbital, or temporal regions, accompanied by ipsilateral cranial autonomic symptoms, triggered by skin contact, stabbing nature of the attacks

●Primary stabbing headache:

transient, sharp jabbing pains that occur at variable locations within trigeminal and cervical dermatomes, last only a few sec and occur at irregular intervals from one to many times each day.

Lambru G, Matharu MS. SUNCT, SUNA and trigeminal neuralgia: different disorders or variants of the same disorder? Curr Opin Neurol 2014; 27:325.

MEDICAL THERAPY

´  Pharmacologic therapy is the initial treatment of most pts with classic TN.

´  Interventional therapy is reserved for pts who are refractory to medical therapy.

´  Carbamazepine is the best studied treatment for classic TN.

´  A systematic review and practice parameter published in 2008 from AAN and EFNS:

´  carbamazepine is effective for controlling pain in pts with C-TN (A), oxcarbazepine is probably effective (B), and baclofen, lamotrigine, and pimozide are possibly effective (C).

´  Limited data and uncertain effectiveness: botulinum toxin injections, clonazepam, gabapentin, phenytoin, tocainide, tizanidine, and valproate.


•  Lambru G, Matharu MS. SUNCT, SUNA and trigeminal neuralgia: different disorders or variants of the same disorder? Curr Opin Neurol 2014; 27:325. •  Bennetto L, Patel NK, Fuller G. Trigeminal neuralgia and its management. BMJ 2007; 334:201.

Carbamazepine

´  4 RCT with a total of 147 pts: effectiveness of carbamazepine (200 - 2400 mg daily).

´  A systematic review and practice parameter published in 2008- AAN and EFNS: treatment response was robust, with complete or near complete pain control attained in 58 -100 % of pts on carbamazepine, compared with 0- 40 % on placebo. NNT <2. However, carbamazepine was sometimes poorly tolerated, with numbers needed to harm for minor and severe adverse events of 3 and 24, respectively.

´  Starting dose is 100- 200 mg BID, increased gradually in increments of 200 mg daily as tolerated until sufficient pain relief is attained (maintenance dose 600 -800 mg daily), maximum dose is 1200 mg daily.

´  A/E: drowsiness, dizziness, N & V. Leukopenia is not uncommon, but it is usually benign; aplastic anemia is a rare side effect. The HLA-B*15:02 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.

•  Campbell FG, Graham JG, Zilkha KJ. Clinical trial of carbazepine (tegretol) in trigeminal neuralgia. J Neurol Neurosurg Psychiatry 1966; 29:265. •  Rockliff BW, Davis EH. Controlled sequential trials of carbamazepine in trigeminal neuralgia. Arch Neurol 1966; 15:129. •  Killian JM, Fromm GH. Carbamazepine in the treatment of neuralgia. Use of side effects. Arch Neurol 1968; 19:129. •  Nicol CF. A four year double-blind study of tegretol in facial pain. Headache 1969; 9:54. •  Gronseth G, Cruccu G, Alksne J, et al. 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 2008; 71:1183

´  Oxcarbazepine: 2008 AAN/EFNS practice parameter: several RCT compared oxcarbazepine (600 - 1800 mg daily) with carbamazepine in 178 C-TN pts => both equally effective, with a >50 % reduction of attacks achieved by ≥ 88 % of pts in both groups

´  Oxcarbazepine dose: 600 mg daily, given in two 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. Testing for the HLA-B*15:02 allele in genetically at-risk populations (Asian ancestry) before initiating treatment with oxcarbazepine.

´  Baclofen: Limited evidence from a small double-blind crossover trial. 40-80 mg daily resulted in a reduction in paroxysms in 7 /10 pts, compared to 1/10 on placebo.

´  Starting dose: 15 mg daily given tid, with gradual titration to a maintenance dose of 50 to 60 mg/day.

´  Sedation, dizziness, and dyspepsia can occur, slow discontinuation to avoid seizures and hallucinations.


•  Fromm GH, Terrence CF, Chattha AS. Baclofen in the treatment of trigeminal neuralgia: double-blind study and long-term follow-up. Ann Neurol 1984; 15:240.

´  Lamotrigine: Double-blind, placebo-controlled crossover study of 14 TN pts refractory to carbamazepine or phenytoin, adjunct therapy with lamotrigine (400 mg daily) was beneficial.

´  Similarly, an open-label study found beneficial effect in 11/ 15 pts on 400 mg dose. Draw back: titrating the dose over many weeks.

´  In pts who are not taking other anticonvulsants, starting dose 25 mg daily for 2 wks, then increased to 50 mg daily for another 2 wks. Then dose titrated to effect, increasing by 50 mg daily every 1-2 wks.

•  Zakrzewska JM, Chaudhry Z, Nurmikko TJ, et al. Lamotrigine (lamictal) in refractory trigeminal neuralgia: results from a double-blind placebo controlled crossover trial. Pain 1997; 73:223. •  Lunardi G, Leandri M, Albano C, et al. Clinical effectiveness of lamotrigine and plasma levels in essential and symptomatic trigeminal neuralgia. Neurology 1997; 48:1714. •  Bennetto L, Patel NK, Fuller G. Trigeminal neuralgia and its management. BMJ 2007; 334:201.

´  Pimozide: a dopamine receptor antagonist, was > carbamazepine in a randomized, double-blind crossover trial of 48 pts with refractory TN. S/E: sedation, arrhythmias, anticholinergic effects, acute extrapyramidal symptoms, and parkinsonism.

´  Topical Lidocaine given by intra-oral application was more effective than placebo for pain reduction in 2 wk, randomized cross-over trial of 24pts.

´  Tizanidine: appeared to be more effective than placebo in a small 1 wk trial, but pts who continued the drug, developed recurrent attacks 1-3 months.

´  Tocainide was as effective as carbamazepine at 2 wks in a cross-over trial of 12 pts.

•  Lechin F, van der Dijs B, Lechin ME, et al. Pimozide therapy for trigeminal neuralgia. Arch Neurol 1989; 46:960. •  Zhang J, Yang M, Zhou M, et al. Non-antiepileptic drugs for trigeminal neuralgia. Cochrane Database Syst Rev 2013; :CD004029. •  Niki Y, Kanai A, Hoshi K, Okamoto H. Immediate analgesic effect of 8% lidocaine applied to the oral mucosa in patients with trigeminal neuralgia. Pain Med 2014; 15:826. •  Fromm GH, Aumentado D, Terrence CF. A clinical and experimental investigation of the effects of tizanidine in trigeminal neuralgia. Pain 1993; 53:265. •  Lindström P, Lindblom U. The analgesic effect of tocainide in trigeminal neuralgia. Pain 1987; 28:45.

´  Small open-label studies: benefit with some medications:

´  Phenytoin and intravenous phenytoin

´  Fosphenytoin

´  Valproic acid

´  Gabapentin

´  Pregabalin

´  Clonazepam

´  Topiramate

´  Misoprostol, in patients with TN and MS

´  Morphine, hydromorphone, or oxycodone

Sindrup SH, Jensen TS. Pharmacotherapy of trigeminal neuralgia. Clin J Pain 2002; 18:22.

Refractory Pain

´  Weak data to support botulinum toxin injections.

´  Combination therapy with gabapentin, lamotrigine, topiramate, baclofen, or tizanidine. Intravenous infusion of phenytoin, fosphenytoin, or lidocaine may provide analgesia while oral medications are titrated.

´  Phenytoin and fosphenytoin (250-1000 mg IV slowly), and lidocaine 100- 300 mg/ 30 min, while monitoring pulse and BP.

•  McCleane GJ. Intravenous infusion of phenytoin relieves neuropathic pain: a randomized, double-blinded, placebo-controlled, crossover study. Anesth Analg 1999; 89:985. •  Scrivani SJ, Chaudry A, Maciewicz RJ, Keith DA. Chronic neurogenic facial pain: lack of response to intravenous phentolamine. J Orofac Pain 1999; 13:89.

Botulinum toxin injections

´  A 2014 literature review: 2 small RCT. One assigned 42 pts received either botulinum toxin A injections or placebo (saline) into skin or mucosa in the regions of pain.

´  At 12 wks, botulinum toxin group showed significant reductions in mean pain scores and attack frequency compared with the placebo.

´  However, small pt numbers and other concerns about the quality of trial limit the strength of these findings.

•  Guardiani E, Sadoughi B, Blitzer A, Sirois D. A new treatment paradigm for trigeminal neuralgia using Botulinum toxin type A. Laryngoscope 2014; 124:413. •  Wu CJ, Lian YJ, Zheng YK, et al. Botulinum toxin type A for the treatment of trigeminal neuralgia: results from a randomized, double-blind, placebo-controlled trial. Cephalalgia 2012;

32:443. •  Zakrzewska JM. Botulinum toxin for trigeminal neuralgia--do we have the evidence? Cephalalgia 2012; 32:1154.

SURGICAL THERAPY

´  The major types of procedures are:

´  Microvascular decompression

´  Ablative procedures, including: Rhizotomy with RF thermocoagulation, mechanical balloon compression, or chemical (glycerol) injection

´  Radiosurgery

´  Peripheral neurectomy and nerve block

Bennetto L, Patel NK, Fuller G. Trigeminal neuralgia and its management. BMJ 2007; 334:201.

´  Observational studies.

´  A systematic review and practice parameter (2008) AAN and EFNS: microvascular decompression, percutaneous procedures on the gasserian ganglion (rhizotomy), and gamma knife radiosurgery are possibly effective treatment of TN.

´  Microvascular decompression has a longer duration of pain control than other surgical interventions.

´  Microvascular decompression is invasive, 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.

´  A feared complication is painful posttraumatic trigeminal neuropathy (anesthesia dolorosa), a condition characterized by persistent, painful anesthesia or hypesthesia in the denervated region.

´  Anesthesia dolorosa most frequently occurs as a complication of rhizotomy > thermocoagulation, but is rarely, if ever, a complication of gamma knife surgery

•  Zakrzewska JM, Akram H. Neurosurgical interventions for the treatment of classical trigeminal neuralgia. Cochrane Database Syst Rev 2011; :CD007312. •  Gronseth G, Cruccu G, Alksne J, et al. 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 2008; 71:1183. •  Headache Classification Committee of the International Headache Society (IHS). The International Classification of Headache Disorders, 3rd edition (beta version).

Cephalalgia 2013; 33:629.

´  Microvascular decompression: is a major neurosurgical procedure that involves craniotomy and the removal of ectatic superior cerebellar artery, away from TN.

´  2008 AAN/EFNS practice parameter: 5 studies concluded that initial pain relief was attained in 90 % of pts, but pain-free rates declined by 1, 3, and 5 yrs to 80, 75, and 73 %, respectively.

´  Mortality 0.2 %. However, major adverse events: CSF leaks, infarction, or hematoma, occurred in up to 4% of pts.

´  The most common complication was aseptic meningitis in 11 % of pts. Long term hearing loss occurred in up to 10 %, and sensory loss 7 %.

•  Jannetta PJ. Microsurgical management of trigeminal neuralgia. Arch Neurol 1985; 42:800. •  Barker FG 2nd, et al. The long-term outcome of microvascular decompression for trigeminal neuralgia. N Engl J Med 1996; 334:1077. •  Zakrzewska JM, et al. Patient reports of satisfaction after microvascular decompression and partial sensory rhizotomy for trigeminal neuralgia. Neurosurgery

2005; 56:1304. •  Broggi G, et al. Microvascular decompression for trigeminal neuralgia: comments on a series of 250 cases, including 10 patients with multiple sclerosis. J

Neurol Neurosurg Psychiatry 2000; 68:59. •  Piatt JH Jr, Wilkins RH. Microvascular decompression for tic douloureux. Neurosurgery 1984; 15:456. •  Barker FG 2nd, Jannetta PJ, Bissonette DJ, Jho HD. Trigeminal numbness and tic relief after microvascular decompression for typical trigeminal neuralgia.

Neurosurgery 1997; 40:39. •  Gronseth G, Cruccu G, Alksne J, et al. 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 2008; 71:1183.

´  Radiofrequency thermocoagulation rhizotomy: lesion by application of heat

´  Mechanical balloon compression: uses a Fogarty catheter to compress the gasserian ganglion

´  Chemical (glycerol) rhizolysis: injection of 0.1 to 0.4 mL of glycerol into the trigeminal cistern

´  2008 AAN/EFNS practice parameter identified: 4 uncontrolled case series; 2 reports RF thermocoagulation, 1 report glycerol rhizolysis, and 1 balloon compression. Initial pain relief was 90 % of pts, but declined by 1 yr to 68 -85 %, by 3 yrs to 54 - 64 %, and by 5 yrs to approximately 50 %.

´  Complications: meningitis, mainly aseptic (0.2 %), Mortality is rare. Postop dysesthesia, described as a burning, heavy, or aching feeling, occurs 12 %. Longer-term sequelae include trigeminal distribution sensory loss in nearly 50% pts, anesthesia dolorosa in approximately 4 %, and corneal numbness with risk of keratitis in 4 %.

•  Lopez BC, et al. Systematic review of ablative neurosurgical techniques for the treatment of trigeminal neuralgia. Neurosurgery 2004; 54:973. •  Mittal B, Thomas DG. Controlled thermocoagulation in trigeminal neuralgia. J Neurol Neurosurg Psychiatry 1986; 49:932. •  Zakrzewska JM, Jassim S, Bulman JS. A prospective, longitudinal study on patients with trigeminal neuralgia who underwent radiofrequency thermocoagulation of the

Gasserian ganglion. Pain 1999; 79:51. •  North RB, et al. Percutaneous retrogasserian glycerol rhizotomy. Predictors of success and failure in treatment of trigeminal neuralgia. J Neurosurg 1990; 72:851. •  de Siqueira SR, et al, Teixeira MJ. Frequency of postoperative complications after balloon compression for idiopathic trigeminal neuralgia: prospective study. Oral Surg Oral

Med Oral Pathol Oral Radiol Endod 2006; 102:e39. •  Gronseth G, et al. 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 2008; 71:1183.

´  Gamma knife radiosurgery: lesions with focused gamma radiation.

´  Therapy is aimed at proximal trigeminal root not gasserian ganglion.

´  The aiming of the beams is carried out with a stereotactic frame and MRI. Doses 70 - 90 grays (Gy). The beams cause axonal degeneration and necrosis.

´  Pain relief occurs after a lag time ≥ 1 month.

´  The 2008 AAN/EFNS practice parameter: 1 RCT and found no important differences. In addition, 3 case series. Complete pain relief at 1 yr was found in up to 69 % of pts, and at 3 yrs in 52 %.

´  An earlier systematic review found that approximately 75 % of pts reported complete relief within 3 months, but the proportion decreased to 50 % by 3 yrs.

´  New or worsened facial sensory impairment occurred in 9 -37 %, with more bothersome sensory loss or paresthesia found in 6-13 % of pts.


References

´  de Siqueira SR, da Nóbrega JC, de Siqueira JT, Teixeira MJ. Frequency of postoperative complications after balloon compression for idiopathic trigeminal neuralgia: prospective study. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2006; 102:e39.

´  Young RF, Vermeulen SS, Grimm P, et al. Gamma Knife radiosurgery for treatment of trigeminal neuralgia: idiopathic and tumor related. Neurology 1997; 48:608.

´  Nurmikko TJ, Eldridge PR. Trigeminal neuralgia--pathophysiology, diagnosis and current treatment. Br J Anaesth 2001; 87:117.

´  Sheehan J, Pan HC, Stroila M, Steiner L. Gamma knife surgery for trigeminal neuralgia: outcomes and prognostic factors. J Neurosurg 2005; 102:434.

´  Flickinger JC, Pollock BE, Kondziolka D, et al. Does increased nerve length within the treatment volume improve trigeminal neuralgia radiosurgery? A prospective double-blind, randomized study. Int J Radiat Oncol Biol Phys 2001; 51:449.

´  Maesawa S, Salame C, Flickinger JC, et al. Clinical outcomes after stereotactic radiosurgery for idiopathic trigeminal neuralgia. J Neurosurg 2001; 94:14.

´  Petit JH, Herman JM, Nagda S, et al. Radiosurgical treatment of trigeminal neuralgia: evaluating quality of life and treatment outcomes. Int J Radiat Oncol Biol Phys 2003; 56:1147.

´  Régis J, Metellus P, Hayashi M, et al. Prospective controlled trial of gamma knife surgery for essential trigeminal neuralgia. J Neurosurg 2006; 104:913.

´  Lopez BC, Hamlyn PJ, Zakrzewska JM. Stereotactic radiosurgery for primary trigeminal neuralgia: state of the evidence and recommendations for future reports. J Neurol Neurosurg Psychiatry 2004; 75:1019.

´  Smith ZA, Gorgulho AA, Bezrukiy N, et al. Dedicated linear accelerator radiosurgery for trigeminal neuralgia: a single-center experience in 179 patients with varied dose prescriptions and treatment plans. Int J Radiat Oncol Biol Phys 2011; 81:225.

´  Chen JC, Girvigian M, Greathouse H, et al. Treatment of trigeminal neuralgia with linear accelerator radiosurgery: initial results. J Neurosurg 2004; 101 Suppl 3:346.

´  Gronseth G, Cruccu G, Alksne J, et al. 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 2008; 71:1183.

´  Peripheral neurectomy can be performed on the branches of the trigeminal nerve (supraorbital, infraorbital, alveolar, and lingual nerves).

´  Neurectomy is accomplished by incision, alcohol injection, RF lesioning, or cryotherapy.

´  The AAN/EFNS practice parameter noted that the evidence regarding peripheral techniques for the treatment of TN is either negative or inconclusive.

Gronseth G, Cruccu G, Alksne J, et al. 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 2008; 71:1183.

General Complications

´  Numbness, hypesthesia, or dysesthesia in the entire trigeminal nerve or in one of its branches (V1, V2, V3)

´  Reactivation of a dominant herpes simplex

´  Corneal abnormalities such as absence of corneal reflex, ulceration, and keratitis

´  Bleeding at the injection site or localized pain

´  Intracranial hemorrhage

´  Infection

PROGNOSIS

´  The course of TN is variable.

´  Episodes may last wks or months, followed by pain-free intervals.

´  Recurrence is common, and some patients have concomitant persistent background facial pain.

´  Most often, TN tends to wax and wane in severity and frequency of pain exacerbations. However, there are no pure natural history studies of TN.

Bennetto L, Patel NK, Fuller G. Trigeminal neuralgia and its management. BMJ 2007; 334:201.

Trigeminal Neuralgia & Other Algies of the Face




Dubai Pain Diploma, May 2017

NEURALGIAS AND PAINFUL CRANIAL NEUROPATHIES ´  Neuralgia: occurs in distribution of a particular nerve(s) that otherwise are

normal in function,

´  Neuropathy: disturbance of function or pathologic change in nerve(s) Neuropathic pain can be caused by a lesion of the central or peripheral somatosensory nervous system.

ü  Trigeminal

ü  Nervus intermedius

ü  Glossopharyngeal

ü  Vagus

ü  Upper cervical spinal cord roots


Neuralgic pain

´  Paroxysmal quality,

´  Maximum at onset,

´  Described as lancinating, electrical shock-like, or jabbing.

´  Might be a single sharp pain or repetitive pains in succession.

´  Duration: last a fraction of a sec or endure for several sec.

´  Might be followed by a refractory period (pain will not occur).

´  Trigger zones (areas that when stimulated provoke an attack).

Rowbotham MC. Mechanisms of neuropathic pain and their implications for the design of clinical trials. Neurology 2005; 65:S66

Detailed History

´  Non-paroxysmal types of pain: continuous aching, burning, or throbbing pain.

´  Patients only mention severe exacerbations.

´  Response to treatment may provide a clue to diagnosis but can also prove misleading;

´  “Diagnostic blocks" in the setting of facial pain do not necessarily define the site from which the pain arises because of the overlap of cranial nerves V, VII, IX, X, which converge on the spinal trigeminal nucleus and the tractus solitarius.

Rowbotham MC. Mechanisms of neuropathic pain and their implications for the design of clinical trials. Neurology 2005; 65:S66

Neuropathic pain

´  The mechanisms are complex.

´  A nerve injury can induce peripheral and central changes that contribute to persistent pain and abnormal sensation (inflamm, nociceptor activation, tissue injury), primary afferent fibers and central structures become sensitized.

´  Permanent loss or injury of primary afferent fibers (deafferentation) results in peripheral neuropathic pain, direct damage to central nervous system structures results in central pain.

Rowbotham MC. Mechanisms of neuropathic pain and their implications for the design of clinical trials. Neurology 2005; 65:S66.


´  Trigeminal neuralgia is one of the most well-defined and common causes of facial pain. The pain of trigeminal neuralgia tends to occur in paroxysms and is maximal at or near onset. The pain has been described as electric shock-like or stabbing.


Case II

´  63 yr old female patient with Hx of pain in L jaw for the last 6 months after the disappearance of skin rash.

´  What is the diagnosis? The management?

Painful trigeminal neuropathy

´  Defined by head & /or facial pain in the distribution of one or more branches of the trigeminal nerve caused by another disorder and indicative of neural damage.

´  Potential causes include acute herpes zoster, postherpetic neuralgia (PHN), trauma, trigeminal trophic syndrome, multiple sclerosis plaque, and space-occupying lesions


Postherpetic neuralgia

´  Defined as pain that persists anywhere from 1 – 6 months after the rash of acute herpes zoster has healed.

´  In rare cases, PHN can occur months to yrs after resolution of the initial event.

Painful post-traumatic trigeminal neuropathy (anesthesia dolorosa)

´  Is characterized by unilateral facial or oral pain that occurs after traumatic injury of the trigeminal nerve, accompanied by additional symptoms or signs of trigeminal nerve dysfunction.

´  In anesthesia dolorosa, pain is superimposed in an area of the face that either lacks or has impaired sensation


Diagnostic criteria, require the following:

●Unilateral facial and/or oral pain

●History of an identifiable traumatic event to the trigeminal nerve, with clinically evident positive (hyperalgesia, allodynia) and/or negative (hypoesthesia, hypoalgesia) signs of trigeminal nerve dysfunction

●Evidence of causation demonstrated by both of the following:

•Pain is located in the distribution of the same trigeminal nerve

•Pain has developed within 3-6 months of the traumatic event

●Not better accounted for by another ICHD-3 diagnosis


´  Traumatic event: mechanical, chemical, thermal, or radiation-induced.

´  Most frequently occurs as a complication of rhizotomy or thermocoagulation done to treat TN.

´  In case series of pts treated for TN, developed after glycerol rhizotomy in 0 -1.6 % of cases, after RF rhizotomy in 0.8 -2 %, and following percutaneous controlled thermocoagulation in 3 %.

´  PPTTN more intolerable than the pain from TN.

´  Treatment:

´  Tricyclic antidepressants (amitriptyline) as initial line. When TCA are contraindicated or poorly tolerated, gabapentin or pregabalin are preferred alternative choices. For superimposed neuralgiform pain, carbamazepine is often used in clinical practice.

See references, next slide

References

´  Blomstedt PC, Bergenheim AT. Technical difficulties and perioperative complications of retrogasserian glycerol rhizotomy for trigeminal neuralgia. Stereotact Funct Neurosurg 2002; 79:168.

´  Ischia S, Luzzani A, Polati E. Retrogasserian glycerol injection: a retrospective study of 112 patients. Clin J Pain 1990; 6:291.

´  Fujimaki T, Fukushima T, Miyazaki S. Percutaneous retrogasserian glycerol injection in the management of trigeminal neuralgia: long-term follow-up results. J Neurosurg 1990; 73:212.

´  Sahni KS, Pieper DR, Anderson R, Baldwin NG. Relation of hypesthesia to the outcome of glycerol rhizolysis for trigeminal neuralgia. J Neurosurg 1990; 72:55.

´  Young RF. Glycerol rhizolysis for treatment of trigeminal neuralgia. J Neurosurg 1988; 69:39.

´  Mathews ES, Scrivani SJ. Percutaneous stereotactic radiofrequency thermal rhizotomy for the treatment of trigeminal neuralgia. Mt Sinai J Med 2000; 67:288.

´  Kanpolat Y, Savas A, Bekar A, Berk C. Percutaneous controlled radiofrequency trigeminal rhizotomy for the treatment of idiopathic trigeminal neuralgia: 25-year experience with 1,600 patients. Neurosurgery 2001; 48:524.

´  Ischia S, Luzzani A, Polati E, Ischia A. Percutaneous controlled thermocoagulation in the treatment of trigeminal neuralgia. Clin J Pain 1990; 6:96.

´  Heros RC, Heros DA, Schumacher, et al. Principles of neurosurgery. In: Neurology in Clinical Practice, Bradley WG, Daroff RB, Fenichel GM (Eds), Butterworth Heinemann, Philadelphia 2004. p.963.

´  Rozen TD. Relief of anesthesia dolorosa with gabapentin. Headache 1999; 39:761.

Case III

´  48 yr old male patient with facial skin ulcer on R side of face for last 4 months.

´  He c/o of itching, and sensation of foreign body in the eye and nasal obstruction.

´  O/E: facial hypoesthesia. Skin ulcer below the eye.

´  What is the diagnosis?

Trigeminal trophic syndrome

´  Facial skin ulceration and dysesthesia related to damage in TN pathway affecting either peripheral components or its central sensory nuclei.

´  The most frequent causes: therapeutic TN ablation and ischemic medullary or pontine stroke; craniofacial surgery, trauma, and herpes zoster infection.

´  Most common symptoms: dysesthesia, including itching, tickling, crawling, burning, as well as ocular foreign body and nasal airflow obstruction sensations.

´  Facial pain in 50 % of pts.

´  O/E: facial hypoesthesia or anesthesia. Skin ulceration tends to occur in the distribution of the infraorbital nerve, although it can occur in other trigeminal distributions (self manipulation with rubbing and scratching of the dysesthetic regions).

´  No good treatment, gabapentin and carbamazepine are often employed in efforts to control neuropathic symptoms .

´  Measures to protect the injured area and promote healing may be beneficial: protective facial prosthetics and dressings, finger nail trimming, use of nocturnal scratch mittens, and behavioral modification.


References

´  Elloumi-Jellouli A, Ben Ammar S, Fenniche S, et al. Trigeminal trophic syndrome: a report of two cases with review of literature. Dermatol Online J 2003; 9:26.

´  Sadeghi P, Papay FA, Vidimos AT. Trigeminal trophic syndrome--report of four cases and review of the literature. Dermatol Surg 2004; 30:807.

´  Garza I. The trigeminal trophic syndrome: an unusual cause of face pain, dysaesthesias, anaesthesia and skin/soft tissue lesions. Cephalalgia 2008; 28:980.

´  Sawada T, Asai J, Nomiyama T, et al. Trigeminal trophic syndrome: report of a case and review of the published work. J Dermatol 2014; 41:525.

´  Golden E, Robertson CE, Moossy JJ, et al. Trigeminal trophic syndrome: A rare cause of chronic facial pain and skin ulcers. Cephalalgia 2015; 35:636.

´  Collyer S, Fuller G. Trigeminal trophic syndrome. Pract Neurol 2012; 12:341.

´  Willis M, Shockley WW, Mobley SR. Treatment options in trigeminal trophic syndrome: a multi-institutional case series. Laryngoscope 2011; 121:712.

Cluster-tic syndrome

´  A combination of cluster headache with TN. It is characterized by 3 types of pain:

´  One component resembles TN pain (paroxysmal, short lasting, and severe).

´  2nd component is more like a cluster headache, although of variable length, with autonomic phenomena (lacrimation, tearing).

´  3rd pain is a mixture of the first two and may be provoked by trigger points or moving the neck.

´  Affects pts between 20-70 yrs of age. It may exist in chronic or episodic (remissions and recurrences) forms.

´  Treatment of both: cluster headache and TN, is often unsuccessful.

´  Microvascular decompression or trigeminal rhizotomy relieves the neuralgia, the cluster-like pain may be lessened and become more responsive to medical therapy.


•  Watson P, Evans R. Cluster-tic syndrome. Headache 1985; 25:123. •  Alberca R, Ochoa JJ. Cluster tic syndrome. Neurology 1994; 44:996. •  Mulleners WM, Verhagen WI. Cluster-tic syndrome. Neurology 1996; 47:302. •  Pascual J, Berciano J. Relief of cluster-tic syndrome by the combination of lithium and carbamazepine. Cephalalgia 1993; 13:205. •  Solomon S, Apfelbaum RI, Guglielmo KM. The cluster-tic syndrome and its surgical therapy. Cephalalgia 1985; 5:83.

Case IV

´  63 year old female patient c/o: severe, dozen attacks, stabbing pain involving tonsillar fossa, base of the tongue, and beneath the angle of the jaw.

´  Pain initiated by chewing, and touching external auditory canal.

´  What is the problem?

Glossopharyngeal neuralgia

´  Paroxysmal pain in areas innervated by cranial nerves IX and X.

´  Paroxysmal, severe, stabbing pain involving ear, tonsillar fossa, base of the tongue, or beneath the angle of the jaw, in distributions of auricular and pharyngeal branches of vagus nerve as well as of glossopharyngeal nerve.

´  Bilateral in 12 % of pts.

´  Typical triggers: chewing, swallowing, coughing, speaking, yawning, certain tastes, or touching the neck or external auditory canal (rarely the pre- or postauricular areas).

´  Pain typically radiates upward from oropharynx toward ear.

´  Duration of the severe paroxysms is sec - min, but there may also be a low grade, constant dull background pain.

´  Several dozen attacks can occur per day, sometimes awakening the patient from sleep.

´  Severe attacks have rarely been associated with bradycardia/asystole resulting in syncope.


•  Rozen TD. Trigeminal neuralgia and glossopharyngeal neuralgia. Neurol Clin 2004; 22:185. •  Rushton JG, Stevens JC, Miller RH. Glossopharyngeal (vagoglossopharyngeal) neuralgia: a study of 217 cases. Arch Neurol 1981; 38:201. •  Elias J, Kuniyoshi R, Carloni WV, et al. Glossopharyngeal neuralgia associated with cardiac syncope. Arq Bras Cardiol 2002; 78:510.

Diagnostic criteria (ICHD-3): ´  At least three attacks of unilateral pain

´  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 at least three of the following four characteristics:

•Recurring in paroxysmal attacks lasting from a few sec to two min

•Severe intensity

•Shooting, stabbing, or sharp in quality

•Precipitated by swallowing, coughing, talking, or yawning

´  No clinically evident neurological deficit

´  Not better accounted for by another ICHD-3 diagnosis


´  idiopathic and 2ry forms of GN {2ry to demyelinating lesions, cerebellopontine angle tumor, peritonsillar abscess, carotid aneurysm, and Eagle syndrome (in which cranial nerve IX is compressed laterally against an ossified stylohyoid ligament)}.

´  Vascular compression of cranial nerves IX and X can occur at the nerve root entry zone by the vertebral artery or posterior inferior cerebellar artery.

´  DDx: nervus intermedius neuralgia.

´  Evaluation: through history, especially inquiring about the presence of trigger factors and nocturnal awakening. ENT examination to exclude local disease.

´  MRI/MRA: to detect mass lesion or vascular pathology; plain skull films may reveal an ossified stylohyoid ligament (Eagle syndrome).

´  Medical therapy of glossopharyngeal neuralgia is essentially the same as for trigeminal neuralgia.

´  LA to oropharynx may prove both diagnostic and therapeutic.

´  Surgical treatment is considered for pts who fail medical therapy (intracranial sectioning of cranial nerve IX plus the upper three to four rootlets of cranial nerve X at the jugular foramen, or vascular decompression).


References

´  Bruyn GW. Glossopharyngeal neuralgia. Cephalalgia 1983; 3:143.

´  Soh KB. The glossopharyngeal nerve, glossopharyngeal neuralgia and the Eagle's syndrome--current concepts and management. Singapore Med J 1999; 40:659.

´  Kim E, Hansen K, Frizzi J. Eagle syndrome: case report and review of the literature. Ear Nose Throat J 2008; 87:631.

´  Hiwatashi A, Matsushima T, Yoshiura T, et al. MRI of glossopharyngeal neuralgia caused by neurovascular compression. AJR Am J Roentgenol 2008; 191:578.

´  Laha RK, Jannetta PJ. Glossopharyngeal neuralgia. J Neurosurg 1977; 47:316.

´  Resnick DK, Jannetta PJ, Bissonnette D, et al. Microvascular decompression for glossopharyngeal neuralgia. Neurosurgery 1995; 36:64.

´  Patel A, Kassam A, Horowitz M, Chang YF. Microvascular decompression in the management of glossopharyngeal neuralgia: analysis of 217 cases. Neurosurgery 2002; 50:705.

Nervus intermedius neuralgia

´  Nervus intermedius (facial nerve) neuralgia (geniculate neuralgia or Hunt neuralgia) is a rare disorder characterized by brief paroxysms of pain felt deeply in the auditory canal.

Occipital neuralgia

´  Occipital neuralgia: cause of occipital headache. It is described as a paroxysmal jabbing pain in the greater, lesser, and/or third occipital nerve distribution, ± diminished sensation or dysesthesia in the affected area. Tenderness overlying the nerve may be present.

´  Third occipital headache is similar to greater occipital neuralgia, but much less common

Optic neuritis

´  Headache due to optic neuritis (retrobulbar neuritis) is characterized by pain behind the eye that is secondary to demyelination of the optic nerve with associated central vision impairment.


Headache attributed to ischemic ocular nerve palsy

´  Headache caused by ischemic injury of the ipsilateral cranial nerves III, IV, or VI (ie, headache attributed to ischemic ocular motor nerve palsy), unilateral frontal and/or periorbital pain caused by and associated with other symptoms and signs of ischemic injury of the ocular nerves that control eye movements.

´  Most prevalent with ischemic injury to cranial nerve III, regardless of the presence or absence of underlying diabetes.


Tolosa-Hunt syndrome

´  Headache due to Tolosa-Hunt syndrome: unilateral orbital pain associated with paresis of one or more of cranial nerves III, IV, or VI caused by a granulomatous inflammation in the cavernous sinus, superior orbital fissure, or orbit.

Paratrigeminal oculosympathetic (Raeder) syndrome

´  Constant unilateral burning facial pain with hypesthesia and/or dysesthesia in the distribution of the ophthalmic division of the trigeminal nerve, along with ptosis and miosis.

´  Causes: trauma, middle cranial fossa mass lesion, syphilis, and sinusitis, e.g. lesion in the middle cranial fossa can directly compromise trigeminal nerve fibers and cause neuralgic pain or sensory change with ptosis and/or miosis, but no anhidrosis.

•  Mokri B. Raeder's paratrigeminal syndrome. Original concept and subsequent deviations. Arch Neurol 1982; 39:395. •  Goadsby PJ. Raeder's syndrome [corrected]: paratrigeminal paralysis of the oculopupillary sympathetic system. J Neurol Neurosurg Psychiatry 2002; 72:297.

Diagnostic criteria (ICHD-3):

´  Constant, unilateral headache

´  Imaging evidence of underlying disease of either the middle cranial fossa or of the ipsilateral carotid artery

´  Evidence of causation demonstrated by both of the following:

´  Headache has developed in temporal relation to the onset of the underlying disorder

´  Headache has either or both of the following features:

-Localized to the distribution of the ophthalmic division of the trigeminal nerve,

with or without spread to the maxillary division

-Aggravated by eye movement

´  Ipsilateral Horner syndrome



Recurrent painful ophthalmoplegic neuropathy

´  Or Ophthalmoplegic migraine, a rare condition, seen in children and young adults.

´  Repeated attacks of paralysis of one or more ocular cranial nerves, typically cranial nerve III, with ipsilateral headache. Headache can develop up to two wks before onset of eye muscle weakness.

´  Brain MRI reveals gadolinium enhancement of the cisternal segment of the affected cranial nerve in approximately 75 % of pts with a typical clinical presentation, ?? recurrent demyelinating neuropathy.

´  In rare cases, oculomotor nerve tumors may mimic recurrent painful ophthalmoplegic neuropathy (no recovery of ophthalmoplegia between attacks).


References


´  Choi JY, Jang SH, Park MH, et al. Ophthalmoplegic migraine with alternating unilateral and bilateral internal ophthalmoplegia. Headache 2007; 47:726.

´  Mark AS, Casselman J, Brown D, et al. Ophthalmoplegic migraine: reversible enhancement and thickening of the cisternal segment of the oculomotor nerve on contrast-enhanced MR images. AJNR Am J Neuroradiol 1998; 19:1887.

´  Gelfand AA, Gelfand JM, Prabakhar P, Goadsby PJ. Ophthalmoplegic "migraine" or recurrent ophthalmoplegic cranial neuropathy: new cases and a systematic review. J Child Neurol 2012; 27:759.

´  Lance JW, Zagami AS. Ophthalmoplegic migraine: a recurrent demyelinating neuropathy? Cephalalgia 2001; 21:84.

´  Kawasaki A. Oculomotor nerve schwannoma associated with ophthalmoplegic migraine. Am J Ophthalmol 1999; 128:658.

´  Murakami T, Funatsuka M, Komine M, et al. Oculomotor nerve schwannoma mimicking ophthalmoplegic migraine. Neuropediatrics 2005; 36:395.

´  Bisdorff AR, Wildanger G. Oculomotor nerve schwannoma mimicking ophthalmoplegic migraine. Cephalalgia 2006; 26:1157.

´  Akimoto J, Fukami S, Hashimoto R, Haraoka J. Neuromuscular hamartoma is a possible primary pathology of oculomotor ophthalmoplegic migraine. Cephalalgia 2012; 32:171.

´  Kim R, Kim JH, Kim E, et al. Oculomotor nerve tumors masquerading as recurrent painful ophthalmoplegic neuropathy: report of two cases and review of the literature. Cephalalgia 2015; 35:825.


´  At least 2 attacks

´  Unilateral headache accompanied by ipsilateral paresis of one, two or all three ocular motor nerves

´  Orbital, parasellar or posterior fossa lesion has been excluded by appropriate investigation


´  Limited observational data suggest that treatment with glucocorticoids is beneficial for some patients.


•  Gelfand AA, Gelfand JM, Prabakhar P, Goadsby PJ. Ophthalmoplegic "migraine" or recurrent ophthalmoplegic cranial neuropathy: new cases and a systematic review. J Child Neurol 2012; 27:759.

Burning mouth syndrome

´  Intraoral burning sensation without medical or dental cause, may be restricted to the tongue, or just tip of tongue, and may be associated with dysesthesia, altered taste, ± a sensation of having a dry mouth.

´  Predominantly affects postmenopausal women, and 30-50 % of pts improve spontaneously.

´  Cause: trigeminal small-fiber sensory neuropathy, higher number of unoccupied D2 dopamine receptors in the putamen associated with painful clinical conditions.

´  Pramipexole, a nonergot dopamine agonist with a high selectivity for dopaminergic D2 receptors.


References


´  Kohorst JJ, Bruce AJ, Torgerson RR, et al. A population-based study of the incidence of burning mouth syndrome. Mayo Clin Proc 2014; 89:1545.

´  Cranial neuralgias and central causes of facial pain. In: Mechanism and management of headache, Lance JW, Goadsby PJ (Eds), Elsevier Butterworth Heinemann, Philadelphia 2005. p.333.

´  Lauria G, Majorana A, Borgna M, et al. Trigeminal small-fiber sensory neuropathy causes burning mouth syndrome. Pain 2005; 115:332.

´  Wood PB. Role of central dopamine in pain and analgesia. Expert Rev Neurother 2008; 8:781.

´  Stuginski-Barbosa J, Rodrigues GG, Bigal ME, Speciali JG. Burning mouth syndrome responsive to pramipexol. J Headache Pain 2008; 9:43.


´  Oral pain

´  Recurring daily for more than two hours per day for greater than three months

´  Pain has both of the following characteristics:

´  Burning quality

´  Felt superficially in the oral mucosa

´  Oral mucosa is of normal appearance and clinical examination including sensory testing is normal



´  Rule out oral mucosal diseases, e.g. herpes simplex and aphthous stomatitis, psychiatric disorders, xerostomia (from drugs, connective tissue disease, or age), nutritional deficiencies (vitamin B12, iron, folate, zinc, vitamin B6), and allergic contact stomatitis, candidiasis, diabetes, denture-related pain, thyroid abnormalities, and menopause.

´  Treating the underlying cause.

´  TCA, pregablin or gabapentin, clonazepam may be beneficial.

´  A systematic review of 9 clinical trials identified 3 interventions that demonstrated a reduction in symptoms compared with placebo. These were alpha-lipoic acid (3 trials), clonazepam (1 trial), and cognitive behavioral therapy (1 trial).


References

´  Cranial neuralgias and central causes of facial pain. In: Mechanism and management of headache, Lance JW, Goadsby PJ (Eds), Elsevier Butterworth Heinemann, Philadelphia 2005. p.333.

´  Evans RW, Drage LA. Burning mouth syndrome. Headache 2005; 45:1079.

´  Maltsman-Tseikhin A, Moricca P, Niv D. Burning mouth syndrome: will better understanding yield better management? Pain Pract 2007; 7:151.

´  Zakrzewska JM, Forssell H, Glenny AM. Interventions for the treatment of burning mouth syndrome. Cochrane Database Syst Rev 2005; :CD002779.

Persistent idiopathic facial pain

´  Or (Atypical facial pain): persistent facial ± oral pain in the absence of a neurologic deficit.

´  In a study of Dutch primary care pts, the incidence was 39.5/ 100,000 person-yrs.

´  Most cases were women.

´  Minor surgery or mild injury to the face, teeth, or gums may initiate the symptoms, these persist after healing without a clear local cause. The pain is commonly felt in the nasolabial fold or one side of the chin, but can spread to wider areas of the face and neck.


•  Dieleman JP, Kerklaan J, Huygen FJ, et al. Incidence rates and treatment of neuropathic pain conditions in the general population. Pain 2008; 137:681. •  Manzoni GC, Torelli P. Epidemiology of typical and atypical craniofacial neuralgias. Neurol Sci 2005; 26 Suppl 2:s65.

Diagnostic criteria(ICHD-3):

´  Facial and/or oral pain

´  Recurring daily for more than two hours per day for more than three months

´  Pain has both of the following characteristics:

Poorly localized, and not following the distribution of a peripheral

nerve

Dull, aching, or nagging quality

Clinical neurologic examination is normal

A dental cause has been excluded by appropriate investigations


´  Diagnosed by exclusion; potential structural lesions (craniofacial neoplasms or abscesses), sometimes attributed to a psychogenic etiology (depression).

´  Treatment: TCA (amitriptyline), gabapentin or pregabalin are preferred alternative choices. In one case report, topiramate (titrated to 125 mg two times a day) was beneficial.

•  Boes CJ, Copobianco DJ, Cutrer FM, et al. Headache and other craniofacial pain. In: Neurology in clinical practice, Bradley WG, Daroff RB, Fenichel GM, et al (Eds), Butterworth Heinemann, Philadelphia 2004. p.2055.

•  Agostoni E, Frigerio R, Santoro P. Atypical facial pain: clinical considerations and differential diagnosis. Neurol Sci 2005; 26 Suppl 2:s71. •  Volcy M, Rapoport AM, Tepper SJ, et al. Persistent idiopathic facial pain responsive to topiramate. Cephalalgia 2006; 26:489.

Central neuropathic facial pain

´  Caused by a lesion or dysfunction in CNS.

´  ICHD recognizes two entities:

´  Central neuropathic pain attributed to multiple sclerosis

´  Central post-stroke pain


OTHER CAUSES

´  Secondary causes of craniofacial pain include the following conditions:

´  Cancer pain – Cancer is a rare cause of facial pain. Extracranial bony or soft tissue metastases may impinge upon cranial and upper cervical nerves causing headache or facial pain. In addition, occult lung neoplasms may cause referred pain in the periauricular region.

´  Dental pain – Dental pathology is a common cause of facial pain. The presence of provocative factors such as chewing or heat or cold sensitivity may provide useful clues. TN has been associated with ipsilateral dental pathology.

´  Temporomandibular joint syndrome (TMJ): chr or acute musculoskeletal pain with dysfunction of the masticatory system. The typical headache associated with TMJ syndrome presents as unilateral ear or preauricular pain that radiates to the jaw, temple, or neck. The pain is deep, dull, continuous, and usually worse in the morning.

Graff-Radford SB. Facial pain. Neurologist 2009; 15:171.

´  Giant cell arteritis (GCA): is a chr vasculitis of large and medium sized vessels. The most feared complication of GCA, visual loss. The head pain tends to be located over temporal areas but can be frontal or occipital, mild or severe. Nearly one-half of GCA pts suffer from jaw claudication. In some cases, a trismus-like symptom occurs rather than fatigue of the chewing muscles. Tender temporal or occipital arteries are found in 30% of pts.

´  Carotidynia: attributed to noninflammatory lesions affecting the cervical carotid or vertebral arteries. 2ry to arterial dissection, postendarterectomy headache, and headache attributed to carotid or vertebral angioplasty.


•  Stanbro M, Gray BH, Kellicut DC. Carotidynia: revisiting an unfamiliar entity. Ann Vasc Surg 2011; 25:1144.

´  Post-traumatic and postoperative pain: occur after trauma (bullet wounds or other head injuries) and maxillofacial surgery, orbital enucleations, sinus, and dental procedures. Manifest constant burning pain, occasionally with tingling and stabbing but without trophic changes, edema, and redness. Blockade of stellate ganglion may be effective in patients who complain of significant burning pain. Amitriptyline, and agents useful for treating migraine headaches.

Jaeger B, Singer E, Kroening R. Reflex sympathetic dystrophy of the face. Report of two cases and a review of the literature. Arch Neurol 1986; 43:693.

trigeminal neuralgia & other algies of the...

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