trigeminal neuralgia and other cranial neuralgias · incidence and clinical features of trigeminal...

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Trigeminal Neuralgia and Other Cranial Neuralgias

Paul G. Mathew, MD, DNBPAS, FAAN, FAHSDirector of Visiting Scholars ProgramJohn R. Graham Headache Center

Brigham & Women’s HospitalStaff Neurologist

Harvard Vanguard/Cambridge Health AllianceAssistant Professor of Neurology

Harvard Medical School

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FINANCIAL DISCLOSURES

• Consulting

– Allergan– Amgen

– Amag

– Analgesic Solutions

– Avanir

– Biomobie

– Promius

– Teva

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OBJECTIVES:

• Participants will be able to

accurately diagnose trigeminal neuralgia and other cranial

neuralgias

• Participants will be able to describe the pathophysiology

and natural history of trigeminal

neuralgia and other cranial neuralgias

• Participants will be able to select

the optimal medication and interventional techniques used for

the treatment of trigeminal

neuralgia and other cranial neuralgias

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CLASSICAL TRIGEMINAL NEURALGIA DIAGNOSTIC CRITERIA

• A) At least three attacks of unilateral facial pain fulfilling criteria B and C

• B) Occurring in one or more divisions of the trigeminal nerve, with no radiation beyond the trigeminal distribution

• C) Pain has at least three of the following four characteristics:– Recurring in paroxysmal attacks lasting from a fraction of a second to two

minutes

– Severe intensity– Electric shock-like, shooting, stabbing or sharp in quality– At least three attacks precipitated by innocuous stimuli to the affected side of

the face (some attacks may be, or appear to be, spontaneous)

• ●D) No clinically evident neurologic deficit

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

Headache Classification Committee of the International Headache Society (IHS). The International Classification of Headache Disorders, 3rd edition (beta version). Cephalalgia. 2013 Jul;33(9):629-808.

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CLASSICAL TRIGEMINAL NEURALGIA

• Previously known as Tic douloureux– When very severe, the pain often evokes ipsilateral facial muscle

contraction

• Trigeminal neuralgia developing without apparent cause other than neurovascular compression.

– Most frequently by the superior cerebellar artery


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CLASSICAL TRIGEMINAL NEURALGIA

• Following a painful paroxysm there is usually a refractory period during which pain cannot be triggered.

• If there is prolonged background pain in the affected area • Subform 13.1.1.2 Classical trigeminal neuralgia with

concomitant persistent facial pain


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TRIGEMINAL NEURALGIA FEATURES

• Triggered by trivial stimuli including washing, shaving, smoking, talking and/or brushing the teeth (trigger factors) and frequently occurs spontaneously.

• Usually involves the second or third divisions with first division involvement in <5% of patients


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TRIGEMINAL NEURALGIA FEATURES• In some cases a paroxysm can be triggered from

somatosensory stimuli outside the trigeminal area, such as a limb, or by other sensory stimulation such as bright lights, loud noises or tastes.

• Attack periods can last for weeks to months followed by remissions, but the pain usually returns

• Usually responsive, at least initially, to pharmacotherapy


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TRIGEMINAL NEURALGIA FEATURES

• TN does not typically involve unilateral autonomic features that can be seen with Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT), short-lasting unilateral neuralgiform headache attacks with autonomic symptoms (SUNA)

• Based on this image President Vladimir Putin is more likely to have SUNCT than TN


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TRIGEMINAL NEURALGIA EPIDEMIOLOGY

• Annual incidence of TN is 4-13 per 100,000 people

• Incidence increases with age, and most idiopathic cases occur to those over the age of 50

• Male to female ratio of TN is about 1:1.7

• Hypertension may be a risk factor

• Some estimates as high as 91% have vascular compression

Katusic S, Williams DB, Beard CM, Bergstralh EJ, Kurland LT. Epidemiology and clinical features of idiopathic trigeminal neuralgia and glossopharyngeal neuralgia: similarities and differences, Rochester, Minnesota, 1945-1984. Neuroepidemiology. 1991;10(5-6):276.

Katusic S, Beard CM, Bergstralh E, Kurland LT. Incidence and clinical features of trigeminal neuralgia, Rochester, Minnesota, 1945-1984. Ann Neurol. 1990 Jan;27(1):89-95.

Hamlyn PJ. Neurovascular relationships in the posterior cranial fossa, with special reference to trigeminal neuralgia.

Neurovascular compression of the trigeminal nerve in cadaveric controls and patients with trigeminal neuralgia: quantification and influence of method. Clin Anat. 1997;10(6):380.

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TRIGEMINAL NEURALGIA RED FLAGS

• According to the AAN and EFNS

– Structural causes in up to 15% of patients

– Features that increase risk of underlying lesion

• Trigeminal sensory deficits (Trigeminal Neuropathy)

• Bilateral involvement of the trigeminal nerve

• Younger age

Gronseth G1, Cruccu G, Alksne J, Argoff C, Brainin M, Burchiel K, Nurmikko T, Zakrzewska JM. 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 Oct 7;71(15):1183-90.

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TRIGEMINAL NEURALGIA

• TN due to secondary causes = Painful Trigeminal Neuropathy – Acute herpes zoster/Postherpetic neuralgia

• Most commonly affects V1

– Post-traumatic trigeminal neuropathy

– Multiple Sclerosis

– Vestibular schwannoma/acoustic neuroma

– Cerebellopontine Meningioma

– Epidermoid or other cyst

– Saccular aneurysm

– Arteriovenous malformation


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TRIGEMINAL NEURALGIA PHARMACOTHERAPY

• According to the AAN and EFNS– Carbamazepine (Level A, Established as effective)

• 100mg daily � 600mg BID• Test for HLA-B*1502 allele in patients of Asian ancestry

• Stevens-Johnson syndrome and/or toxic epidermal necrolysis

– Oxcarbazepine (Level B, Probably effective)• 300mg daily � 900mg BID

– Baclofen (Level C, Possibly Effective)• 5 mg PO q8hr � 80 mg/day

– Lamotrigine (Level C, Possibly Effective)• 50mg daily � 400mg daily

– Phenytoin, Valproic acid, Gabapentin, Pregabalin, and Topiramate have small study support

Gronseth G, Cruccu G, Alksne J, Argoff C, Brainin M, Burchiel K, Nurmikko T, Zakrzewska JM. 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 Oct 7;71(15):1183-90.

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TRIGEMINAL NEURALGIA PHARMACOTHERAPY

• Medications with IV formulations may be useful in intractable cases and/or the ED

– Levetiracetam

– Phenytoin

– Valproic Acid

1. Tate R, Rubin LM, Krajewski KC. Treatment of refractory trigeminal neuralgia with intravenous phenytoin. Am J Health Syst Pharm. 2011 Nov 1;68(21):2059-61.

2. Zakrzewska JM1, Linskey ME. Trigeminal neuralgia. Clin Evid (Online). 2009 Mar 12;2009. pii: 1207.

3. Mitsikostas DD1, Pantes GV, Avramidis TG, Karageorgiou KE, Gatzonis SD, Stathis PG, Fili VA, Siatouni AD, Vikelis M. An observational trial to investigate the efficacy and tolerability of levetiracetam in trigeminal neuralgia. Headache. 2010 Sep;50(8):1371-7.

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TRIGEMINAL NEURALGIA BOTULINUM TOXIN INJECTIONS

• Randomized controlled trial with 42 subjects with TN– 22 subjects received 75 units of BTX

– 20 subjects received saline injections

– Significant reduction in pain frequency at week 1 and intensity at week 2

– More responders in BTX group (68.18%) than in the placebo group (15.00%).

– BTX was well tolerated, with few treatment related adverse events at the

end of 12 weeks

Wu CJ1, Lian YJ, Zheng YK, Zhang HF, Chen Y, Xie NC, Wang LJ. Botulinum toxin type A for the treatment of trigeminal neuralgia: results from a randomized, double-blind, placebo-controlled trial. Cephalalgia. 2012 Apr;32(6):443-50.

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TRIGEMINAL NEURALGIA INTERVENTIONAL PROCEDURES

• In cases resistant to pharmacotherapy, there are multiple procedures that

can be used for the treatment of TN

– Microvascular Decompression

– Denervating/Destructive Procedures

• Percutaneous Trigeminal Rhizotomy

• Radiofrequency, Glycerol, or Balloon

• Stereotactic Radiosurgery

• Gamma Knife

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MICROVASCULAR DECOMPRESSION

• 2 inch craniotomy exposes area posterior to ear

• Under microscope, the superior cerebellar artery is decompressed from nerve, and teflon felt is placed in between

• More invasive than other procedures, but no nerve destruction

• Faster results and longer lasting

• If no compression found, open denervation (microsurgical rhizotomy) could be performed

• Destructive procedures could be considered in MVD failure

1. Pollock BE. Surgical management of medically refractory trigeminal neuralgia. Curr Neurol Neurosci Rep. 2012 Apr;12(2):125-31.

RISKS OF NEUROSURGERY

• Highest rates of permanent cranial nerve deficit

• Meningitis/Encephalitis

• Intracranial Hemorrhage/Stroke

• Cranial Nerve Deficits/Neuralgias

• CSF Leaks

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• First denervating/destructive procedures were peripheral trigeminal

neurectomies

– Caused dense numbness

– Earlier recurrence of pain

– Treated focused, small, superficial branch of TN

• Proximal treatment (rhizotomy, root exit zone) has better results

– Longer lasting

– Less or no facial numbness

• Worst case is anesthesia dolorosa

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• Percutaneous Trigeminal Rhizotomy

– Needle inserted through cheek one inch from angle of the mouth

– Needle advanced through foramen ovale using fluoroscopy

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• Percutaneous Trigeminal Rhizotomy Via– Radiofrequency Ablation (heat) � 6205 patients

• Stimulation is performed prior to ablation to ensure correct target

• Only selective technique

• If V1 involved, caution to not over-numb corneal sensation, which risks

keratophathy

• Highest rates of initial pain relief and the lowest rates of pain recurrence

– Glycerol (chemical) � 1217 patients

• CSF coming from needle is a good finding before bathing nerve

• Highest recurrence rate

– Balloon (mechanical) � 759 patients

• More likely to affect mastication

1. Taha JM, Tew JM Jr. Comparison of surgical treatments for trigeminal neuralgia: reevaluation of radiofrequency rhizotomy. Neurosurgery. 1996 May;38(5):865-71.

2. Scrivani SJ, Mathews ES, Maciewicz R: Trigeminal Neuralgia. In Mehta N, Maloney GE, Bana D, Scrivani SJ (eds): Head, Face and Neck Pain: Science, Evaluation and Management. 1st ed., John Wiley & Sons, Inc., Hoboken, NJ, 465-510, 2009.

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• Stereotactic Radiosurgery

– Used for treatment of tumors, vascular lesions, and functional disorders like TN

– Highly focused beams of ionizing radiation with high precision

– Useful for targets that are inaccessible for open surgery

– Immediately outside of target there is a steep drop in radiation so surrounding tissues are relatively spared

• Not useful for large targets

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• Stereotactic Radiosurgery– 497 patients presenting with TN underwent GKS

• No clear vascular compression or history of multiple sclerosis• Results

• 169 patients became pain free within the first 48 hour � Pain recurrence in 66 patients (39%)

� Postoperative hypesthesia in 18 patients (13.7%)

• 194 patients became pain free within post treatment Day 3-30 � Pain recurrence in 71 patients (36.6%)

� Postoperative hypesthesia in 30 patients (19%)

• 91 patients became pain free 30 days post-GKS � Pain recurrence in 27 patients (29.7%)

� Postoperative hypesthesia in 22 patients (30.6%)

1. Tuleasca C, Carron R, et al. Patterns of pain-free response in 497 cases of classic trigeminal neuralgia treated with Gamma Knife surgery and followed up for least 1 year. J Neurosurg. 2012 Dec;117 Suppl:181-8.

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GLOSSOPHARYNGEAL NEURALGIA DIAGNOSTIC CRITERIA

• A. At least three attacks of unilateral pain fulfilling criteria B and C

• B. 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

• C. Pain has at least three of the following four characteristics:1. recurring in paroxysmal attacks lasting from a few seconds to 2 min2. severe intensity3. shooting, stabbing or sharp in quality

4. precipitated by swallowing, coughing, talking or yawning

• D) No clinically evident neurologic deficit

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


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GLOSSOPHARYNGEAL NEURALGIA

• Previously used term: Vagoglossopharyngeal neuralgia.

• May remit and relapse in the fashion of classical trigeminal neuralgia.

• Less severe than classical trigeminal neuralgia but can be bad enough for

patients to lose weight. These two disorders can occur together.

• Rare cases associated with vagal symptoms

– Cough, hoarseness, syncope and/or bradycardia.

• Imaging may show neurovascular compression of the glossopharyngeal

nerve.

• Usually responsive, at least initially, to antiepileptics

• Application of local anaesthetic to the tonsil and pharyngeal wall can prevent

attacks for a few hours.


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CLASSICAL NERVUS INTERMEDIUS NEURALGIA DIAGNOSTIC CRITERIA

• A. At least three attacks of unilateral pain fulfilling criteria B and C

• B. Pain is located in the auditory canal, sometimes radiating to the parieto-occipital region

• C. Pain has at least three of the following four characteristics1. recurring in paroxysmal attacks lasting from a few seconds to minutes2. severe intensity3. shooting, stabbing or sharp in quality

4. precipitated by stimulation of a trigger area in the posterior wall of the auditory canal and/or periauricular region

• D) No clinically evident neurologic deficit

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


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CLASSICAL NERVUS INTERMEDIUS NEURALGIA

• Can involve lacrimation, salivation and/or taste alteration

• Neurovascular compression can be a cause

• Nervus intermedius neuropathy attributed to Herpes zoster

– Ramsay Hunt syndrome

– Herpetic eruption has occurred in the ear and/or oral mucosa, in the territory of the nervus intermedius

– Peripheral facial paresis

• Sensory innervation of the ear is complicated


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OCCIPITAL NEURALGIA DIAGNOSTIC CRITERIA

• A. Unilateral or bilateral pain fulfilling criteria B-E

• B. Pain is located in the distribution of the greater, lesser and/or third occipital nerves

• C. Pain has two of the following three characteristics:

– 1. recurring in paroxysmal attacks lasting from a few seconds to minutes

– 2. severe intensity

– 3. shooting, stabbing or sharp in quality

• D. Pain is associated with both of the following:

– 1. dysaesthesia and/or allodynia apparent during innocuous stimulation of the scalp and/or hair

– 2. either or both of the following:

• a) tenderness over the affected nerve branchesb) trigger points at the emergence of the greater occipital nerve or in the area of

distribution of C2

• E. Pain is eased temporarily by local anaesthetic block of the affected nerveF. Not better accounted for by another ICHD-3 diagnosis.


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OCCIPITAL NEURALGIA EXAM

• Exam maneuvers to perform…

– Cranial tinel’s sign demonstrating pain/paresthesias along nerve distribution

– Neck passive range of motion elicits pain

– Best results: Lancinating pain occurs with tinel’s and PROM when patient denies any significant headache otherwise

NERVE BLOCKSDIAGNOSTIC AND THERAPEUTIC

• Generally safe, well tolerated office based procedures

• Can be performed for the acute treatment of numerous headache disorders.

• Can have prolonged effects beyond the duration of the injected anesthetic at times

lasting weeks to months

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Afridi SK, Shields KG, Bhola R, Goadsby PJ. Greater occipital nerve injection in primary headache syndromes--prolonged effects from a single injection. Pain. 2006 May;122(1-2):126-9

NERVE BLOCK COMPOSITION• Nerve blocks are performed with an anesthetic with or

without a steroid

• Anesthetic is usually lidocaine, bupiviaine, or a combination.– 0.75% bupivicain is my preference

• Steroids added can include methylprednisolone and

triamcinolone

• Steroid alone proven to be useful, but lack of immediate relief makes this less successful;

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Ambrosini A, Vandenheede M, Rossi P, Aloj F, Sauli E, Pierelli F, Schoenen J. Suboccipital injection with a mixture of rapid- and long-acting steroids in cluster headache: a double-blind placebo-controlled study. Pain. 2005 Nov;118(1-2):92-6.

NERVE BLOCK CENTRAL EFFECTS • Peripheral nerve blocks may modulate central pain

structures

• In one study, occipital nerve blocks were performed in

the setting of an acute migraine with improvement of – Migraine pain

– Brush allodynia in the trigeminal nerve distribution

– Photophobia

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Young W, Cook B, Malik S, Shaw J, Oshinsky M. The first 5 minutes after greater occipital nerve block. Headache. 2008;48:1126-1128.

MIG_01161_Migraine Pathophysiology_DT3 11/10/2017 12:08 PM

Nerve Block

Needle Point

of Entry

Greater Occipital

Nerve

Lesser Occipital

Nerve

Areas of

InfiltrationPaul G. Mathew, MD, DNBPAS, FAAN, FAHS

Occipital Nerve BlockProne, 6cc per side

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TRIGEMINAL NERVE BRANCHES…

1. Tuleasca C, Carron R, et al. Patterns of pain-free response in 497 cases of classic trigeminal neuralgia treated with Gamma Knife surgery and followed up for least 1 year. J Neurosurg. 2012 Dec;117 Suppl:181-8.

• Auriculotemporal Neuraliga• Supraorbital Neuralgia• Supratrochlear Neuralgia


Nerve Block

Needle Point

of Entry

Auriculotemporal

Nerve

Areas of Infiltration

Paul G. Mathew, MD, DNBPAS, FAAN, FAHS

Auriculotemporal Nerve BlockSupine, 2cc per side


Nerve Block

Needle Points

of Entry

Supraorbital

Nerve

Supratrochlear Nerve

Areas of

Infiltration

Paul G. Mathew, MD, DNBPAS, FAAN, FAHS

Supraorbital/Supratrochlear Nerve BlockSupine, 0.5-1cc per foramen

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OCCIPITAL NEURALGIA AND MIGRAINE

Mathew PG, Robbins L. Cranial neuralgia vs entrapment neuropathy decompression … better names than migraine trigger site deactivation surgery. Headache. 2015 May;55(5):706-10.

Sahai-Srivastava S, Zheng L. Occipital neuralgia with and without migraine: Difference in pain characteristics and risk factors. Headache. 2011;51:124-128.

– 35 consecutive occipital neuralgia cases, 15 had both occipital neuralgia and

migraines

– Chances are good that many patients with migraines and focal neuralgias are only being diagnosed with migraines

– Patients being treated with decompression procedures for migraines may be

responding because they actually have a cranial neuralgias

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MIGRAINE SURGERY

• Surgical Deactivation of Potential Trigger Sites

– Frontal Trigger Site

• Supraorbital and supratrochlear nerves

• Resection of corrugator supercilii, depressor supercilii muscles, lateral

procerus

Mathew PG. A critical evaluation of migraine trigger site deactivation surgery. Headache. 2014 Jan;54(1):142-52. .

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MIGRAINE SURGERY

• Surgical Deactivation of Potential Trigger Sites

– Temporal Trigger Site

• Zygomatictemporal branch of Trigeminal Nerve through the temporalis

muscle

• Avulsion of the nerve

Mathew PG. A critical evaluation of migraine trigger site deactivation surgery. Headache. 2014 Jan;54(1):142-52.

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MIGRAINE SURGERY

• Surgical Deactivation of Potential

Trigger Sites

– Occipital Trigger Site

• Greater occipital nerve

• Resection of small portion of semispinalis capitis muscle and

shielding of the nerve with a subcutaneous flap (fat pad)

• If there is contact between the occipital artery and occipital nerves,

the artery is at times also resected

Mathew PG. A critical evaluation of migraine trigger site deactivation surgery. Headache. 2014 Jan;54(1):142-52.

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CRITIC OF THE CRITIC

Guyuron B. A discussion of "critical evaluation of migraine trigger site decompression surgery". Headache. 2014 Jun;54(6):1065-72.

• Insult to study neurologists

• Two separate diagnoses cannot co-exist at the same

time in the same patient. – That would be like having carpal tunnel syndrome and

cervical radiculopathy at the same time

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MIGRAINE PRE-SURGERY EVALUATION

• Doppler Evaluation– Headache point of origin identified with 1 finger by patient

– Site is explored with Doppler.

– If an arterial Doppler signal is identified at the site, it is considered an active arterial trigger site.

Guyuron B, Nahabet E, Khansa I, Reed D, Janis JE. The Current Means for Detection of Migraine Headache Trigger Sites. Plast Reconstr Surg. 2015 Oct;136(4):860-7.

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FRONTAL, TEMPORAL, OCCIPITAL TRIGGER SITES

Oturai AB, Jensen K, Eriksen J, Madsen F. Neurosurgery for trigeminal neuralgia: Comparison of alcohol block, neurectomy, and radiofrequency coagulation. Clin J Pain. 1996;12:311-315.

Taha JM, Tew JM Jr. Comparison of surgical treatments for trigeminal neuralgia: Reevaluation of radiofrequency rhizotomy. Neurosurgery. 1996;38: 865-871.

• If nerve compression is serving as a trigger for migraines, why are branches

of the trigeminal nerve being resected rather than decompressed in the temporal region.

– Based on the trigeminal neuralgia literature, damaging or destroying a peripheral nerve can lead to numbness, paresthesias, dysesthesias, and even

worsening of preoperative pain

• If nerve compression is thought to be occuring, why do these patients not have numbness, paresthesias, or neuralgiaform pain in the distribution of the

suspected nerve compression

– Suprorbital, Supratrochlear, Auriculotemporal, and Greater/Lesser Occipital

Neuralgia may have existed in these patients in addition to migraine

– Decompression of the nerve improved/resolved the neuralgia, which has a tendency to improve, but not CURE migraine

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CRANIAL NEURALGIA OR PRIMARY STABBING HEADACHE?

• DIAGNOSTIC CRITERIA

– A. Head pain occurring spontaneously as a single

stab or series of stabs and fulfilling criteria B-D

– B. Each stab lasts for up to a few seconds

– C. Stabs recur with irregular frequency, from one to many per day

– D. No cranial autonomic symptoms

– E. Not better accounted for by another ICHD-3 diagnosis.


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• Transient and localized stabs of pain in the head that occur spontaneously in

the absence of organic disease

• When stabs are strictly localized to one area, structural changes at this site

and in the distribution of the affected cranial nerve must be excluded.

• Involves extratrigeminal regions in 70% of cases.

• If cranial autonomic symptoms are present, think . Short-lasting unilateral

neuralgiform headache attacks (SUNCT)

• Primary stabbing headache is more common in migraineurs

– Stabs tend to be localized around areas of frequent migraine headache

pain.


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• My take…– Reproducible physical exam findings + single location = neuralgia

– No significant exam findings + multiple locations = primary stabbing

headache

– Little risk and high potential yield with nerve blocks


CONCLUSIONS REGARDING CRANIAL NEURALGIAS

• There are many treatments for these conditions

• Medication trials should start at a low dose, and titrations should be

fast/slow based on patient preference and side effects

• Combination therapies should be considered

• Do not hesitate to refer patients to another provider for treatments

that you may not provide

• AHS has issued a position statement on migraine surgery for a

reason

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If you enjoyed the clinical content…

Dr. Mathew (4 chapters), Dr. Scrivani

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If you enjoyed the humor…

http://www.health.harvard.edu/blog/author/pgmathew

Sinus headache or sign-us up for a migraine consultation

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THANK YOU!!!

JOHN R. GRAHAM HEADACHE CENTER

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2017-2018

trigeminal neuralgia and other cranial neuralgias · incidence and clinical features of trigeminal...

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