Trigeminal neuralgia, facial pain in MS

Prof Joanna Zakrzewska

Head of facial pain unit

Eastman Dental Hospital

Aims and Objectives

Trigeminal neuralgia Diagnosis

Investigations

Medical management

Surgical management

Differential diagnosis

Epidemiology

Patho-

physiology

Epidemiology TN

Incidence : 4.5 / 100,000

Prevalence : 0.001% - 0.3%

Peak incidence : 50-60 years

Multiple sclerosis (2- 4 % of TN patients)

Hypertension

Stroke

Zakrzewska JM, Hamlyn PJ. In Epidemiology of

Pain. IASP, 1999

Mueller D et al Cephalagia 2011

Pan et al Cephalagia 2011

Incidence

per 100,000

PY

Hall et al

2006

Dieleman

2008

Koopman et

al 2009

Number 8,268

322 118

Incidence 26.8

28.9 12.6

Changing epidemiology of TN?

What are other causes of

unilateral episodic facial pain?

Drangsholt et al 2001

Dental causes of pain

Dental Pain – acute/ chronic

Chronic continuous pain

Unilateral continuous orofacial pain

Yes No

Yes No

• Post herpetic

neuralgia

• Post traumatic

trigeminal pain

• Anesthesia

dolorosa

• Persistent

dentoalveolar

pain

• Referred pain

• Temporomandibular

disorders**

• Persistent orofacial

muscle pain **

Unilateral episodic orofacial pain

Yes No

• Trigeminal

neuralgia

classical (type 1)

• Trigeminal

neuralgia

symptomatic

• Trigeminal

neuralgia +

concomitant pain

( type 2)

• Glossopharyngeal

neuralgia

• Tension type

headache

• Medication

overuse

headache

• Post stroke pain

• Giant cell arteritis *

Cancer pain

Burning mouth

syndrome

Chronic migraine • Trigeminal autonomic

cephalagias

• Episodic migraine *

Persistent idiopathic facial

pain

Zakrzewska BJA 2013

Temporomandibular Disorders

musculoskeletal

© Zakrzewska

TMD

© Zakrzewska

Education- explanation

© Zakrzewska

Education vs splint

Michelotti JADA 2012;143:47-53 © Zakrzewska

TMD management

Reassurance, education, relaxation, behaviour changes, intermittent

NSAID

Physiotherapy/ acupuncture, pain coping strategies, reinforce self

care, address psychological distress

Tricyclic antidepressants, cognitive behaviour

therapy, reinforce self care

© Zakrzewska

© Zakrzewska

Why is the right diagnosis important?

• Epidemiological studies

• Genetic studies

• Recruiting into clinical trials

• Appropriate managements and referrals

• Variants may involve different mechanisms

Who makes the diagnosis?

HCP making

diagnosis

TN

5287

ATN

189

OFP

857

General medical

practitioner % 36 36 18

Neurologist % 29 30 17

Dentist % 20 15 10

Other specialists % 15 19 55

Listen to your patient he is

telling you the diagnosis

The Story of Pain

curiosity to ask “tell me about yourself”

patience to wait for the answer Platt et al 2001

Stories

• patients tell stories to become who they are

• patient stories are addressed to someone

• the clinician must listen

• the quality of attention is important and

you " have to be caught up” – it is an act of

surrender

Misery by Rosa Sepple

What are the features of trigeminal neuralgia?

Character of TN pain

The character of trigeminal neuralgia is very

distinctive and pts will use words such electric

shock, lightening – implies speed as well as severity

© Zakrzewska

Duration

paroxysmal

pain of abrupt onset and termination

refractory intervals

no background pain

memorable first attack

© Zakrzewska

Temporal features

Refractory period

Classical TN TN – Type 2, concomitant pain

Periodicity of TN

Site and Radiation

Rare

© Zakrzewska

Provoking factors

R Conelly

Evoked vs spontaneous

Trigeminal Neuralgia –IASP

“ a sudden, usually unilateral,

severe, brief, stabbing, recurrent

pain in the distribution of one or

more branches of the fifth cranial

nerve”.

How does TN affect quality of life ?

© Zakrzewska

© R. Sepple

Associated features Loneliness – isolation – how can

you go to social activities when

you cannot eat,

how can you be intimate when

you cannot bear your face to be

touched

How do you get across the

message that despite looking

normal you have one of the most

suicide pain

Depression is common

Fear of a returning attack is

always with the patients and

psychologist will tell us that this

drives further pain

Fear

©Eastman

Tolle et al Pain Practice 2006

The Patient’s Journey

Through Trigeminal Neuralgia Zakrzewska Padfield May 2014

Trigeminal Neuralgia

Limonadi et al 2006, Obermann et al 2007

What is the diagnosis of this

patient ?

Symptomatic TN

Trigeminal nerve

Tumour

© Zakrzewska

TG

MS plaque

vessel

nerve

Neurovascular compression

Aetiology / Pathophysiology

© Zakrzewska

Nerve Structure

Root entry zone, change in nerve

structure

Love and Coakham, Brain 2001: 124, 2347 – 2360

Management

Outcomes

Therapy % pain

relief

Decreased

intensity

frequency

Character of pain

Provoking factors

Side

effects

Beliefs

concerns

Objective

measures

© Zakrzewska

Health utilisation

Medical management

Systematic reviews guidelines

www.aan.com

Which have been evaluated in

Randomised controlled trials - RCTs?

baclofen carbamazepine

valproate lamotrigine epanutin

clonazepam

Which have been evaluated in RCTs?

gabapentin

pregabalin oxcarbazepine

sumatriptan

leviteracetam

Dextromethorphan

Topiramate

Botox

Carbamazepine

3 RCT studies (total 160 patients)

NNT 1.7 for >50% pain relief

Maximum 2.4 g / day

Usual dose 200-1,200 mg

NNH 2.6

Multiple drug interactions

Carbamazepine adverse events

osteoporosis

© Zakrzewska

Oxcarbazepine

Starting 300 mg bd

Dose range 600-1,200 mg /day

Maximum 2,400 mg/day

Hyponatraemia higher doses

Few drug interactions

Side effects to anticonvulsants

Acute management

Fosphenytoin infusion

Sumatriptan 3mg sc

24 patients in RCT

12/24 improved for mean 8 hours

Lidocaine

Injection

Intranasal

IV

Cheshire Fosphenytoin J Pain Sympt 2001

Kania Sumatriptan Head 2006

Han Lidocaine Clin Prac 2008, Kania Br J Aneasth 2006

Results of RCTs in TN

carbamazepine

oxcarbazepine

lamotrigine

baclofen

gabapentin + LA

pimozide =

tizanidine ?

tocainide

dextromethorphan ?

topiramate ?

AAN/ EFNS Guideline

New drug for TN

Novel Sodium Channel Blocker

CNV1014802 June 2014

• Treatment failure rate

33% CNV1014802 vs 65% placebo

• Decrease in pain severity

55% CNV1014802 vs 18% placebo

• Average reduction in number of paroxysms

60% CNV1014802 vs 12% placebo

Medications

There is a medicine

you can take

But it’s not the cure

It may make your

drowsy

It may make you sick

There are more pills

to take

And there’s no

miracle fix

Response to medication

Insufficient evidence to say

when surgery should be offered

Patients should be given details of all surgical procedures

Systematic review surgery

3 studies

2 radiofrequency

thermocoagulation

comparing techniques

1 stereotactic surgery

comparing dosages

Cochrane Database of Systematic Reviews 2011, Issue 9

Grading

Gasserian Ganglion procedures

Radiofrequency rhizotomy

Glycerol rhizotomy

Balloon compression

Microvascular decompression

Partial sensory rhizotomy

Posterior fossa

Zakrzewska Coakham Current Neurol 2012

Posterior fossa

Trigeminal nerve

compressed by

superior cerebellar

artery

© Prof H Coakham

Gamma knife

Radiation doses of 90- 70Gy

Non invasive ablative

procedure

Complete pain relief after surgery

0

10

20

30

40

50

60

70

80

90

100

0 1 2 3 4 5years

% p

rob

ab

ilit

y

radiofrequency thermocoagulation percutaneous glycerol rhizotomy

microvascular decompression gamma knife

© Zakrzewska

data from 14 studies in 2785 patients

Complications

Mortality

0.2 - 0.5 % for MVD

© Zakrzewska

Complications

Peripheral ganglion lesions

© Zakrzewska

Complications

© Zakrzewska

How 302 patients with MS and TN

compare with 7982 TN only patients ?

Same age and demographics

More constant, bilateral pain

Use wider range of drugs

Undergo more ablative surgery

Why are there few TN Drug Trials ? Diagnosis strictly clinical

Condition relatively rare

Medications may interfere with other drugs

Medications may take time to work

Spontaneous remission common

Side-effects may take time to appear

Pain very severe: justify using placebo

AAN/EFNS guidelines

Patient Information

© Zakrzewska

Support groups US , UK, Australia , Canada

Web sites : http://www.tna-support.org/

http://www.tna.org.uk/

© Zakrzewska

Microvascular

decompression

MS

Drug

therapy

Neurovascular compression

Drug therapy

Tumours etc

Idiopathic TN

MRI

Blood tests Symptomatic TN

Ablative procedures Gasserian

ganglion

Gamma knife

Trigeminal neuralgia Primary care

Carbamazepine

Initial good control but now failing

Refer pain clinic

Neurology headache

Neurosurgery Poor

quality of

life

Carbamazepine

Oxcarbazepine

Lamotrigine

Baclofen

Pregablin

Gabapetin

Joint neurosurgery

clinic Psychology

© Zakrzewska

Choice, Option, Decision

http://www.advancingqualityalliance.nhs.uk/SDM

References / further

reading

TN Review BMJ 2014

Guideline website

www.aan.com

Resources – e-learning

TMD & trigeminal neuralgia

http://cks.nice.org.uk/tmj-disorders

http://cks.nice.org.uk/trigeminal-neuralgia

Educational material

http://www.efic.org/

Year against orofacial pain

Summary

• Epidemiology provides

clues to aetiology

• Differential diagnosis

• Important diagnostic

features of TN

• Causes

• Investigations

• Management

• References

@ R.Sepple

Face to face Rosa Sepple

Thank you