CRANIALNEURALGIAS

Presented by: Neha Sharma M.D.

Date: September 27th, 2019

TYPES OF NEURALGIAS

❖ TRIGEMINAL NEURALGIA

❖ NASOCILIARY NEURALGIA

❖ SUPRAORBITAL NEURALGIA

❖ SPHENOPALATINE NEURALGIA

❖ NERVUS INTERMEDIUS NEURALGIA

❖ GLOSSOPHARYNGEAL NEURALGIA

❖ SUPERIOR LARYNGEAL NEURALGIA

❖ OCCIPITAL NEURALGIA

❖ GREAT AURICULAR NEURALGIA

❖ TROCHLEAR NEURALGIA

WHAT IS CRANIAL NEURALGIA?

❖ Paroxysmal pain of head, face and/or neck

❖ Unilateral sensory nerve distribution

❖ Pain is described as sharp, shooting, lancinating

❖ Primary or Secondary causes

❖ Multiple triggers

TRIGEMINAL (CN V)

NEURALGIA

TRIGEMINAL NEURALGIA

❖ Also called Tic Douloureux

❖ Sudden, unilateral, electrical, shock-like, shooting, sharp

pain. Presents affecting Cranial Nerve V; primarily V2 and

V3 branches

❖ F>M; 3:1

TRIGEMINAL NEURALGIA

https://www.nf2is.org/cn5.php

❖ Anatomy of Trigeminal

Nerve

❖ Cranial Nerve V

❖ Three Branches:

Ophthalmic,

Maxillary and

Mandibular

❖ Sensory supply to

forehead/supraorbital,

cheeks and jaw

TRIGEMINAL NEURALGIA – TRIGGERS

❖ Mastication (73%)

❖ Touch (69%)

❖ Brushing Teeth (66%)

❖ Eating (59%)

❖ Talking (58%)

❖ Cold wind (50%)

TYPES OF TRIGEMINAL NEURALGIA

❖ Primary/Classic/Idiopathic

❖ Vascular compression of the nerve – superior cerebellar artery

❖ Secondary/Symptomatic

❖ Caused by intracranial lesions

❖ Tumors, Strokes, Multiple Sclerosis (4%)

❖ Typical vs. Atypical

❖ Paroxysmal (79%) vs. Continuous (21%)

IASP/IHS & CLASSIFICATIONS OF TRIGEMINAL NEURALGIA

❖ IASP – International Association

for the Study of Pain

❖ Sudden, unilateral, brief,

intermittent pain in at least

one branch of CN V

❖ IHS – International Headache

Society

❖ Excruciating, unilateral,

electric pain in at least one

branch of CN V

❖ Classifications

❖ I – idiopathic, sharp, shooting, electric,

episodic

❖ II – idiopathic, aching, burning, >50%

constant

❖ III – secondary to injury, trauma, surgery to

face/cranium, stroke

❖ IV – deafferentation by intentional injury

(ex: rhizotomy)

❖ V – associated with MS

❖ VI – postherpetic neuralgia

❖ VII – facial pain with somatoform disorder

PRIMARY TRIGEMINAL NEURALGIA

❖ Pathophysiology

Vascular compression of nerve/root

Demyelination of CN V

Hyperexcitability, ectopic discharge and impaired inhibition

DIAGNOSING TRIGEMINAL NEURALGIA

❖ History

❖ Physical Examination

❖ Pain mapping

❖ Labs and MRI/MRA

❖ To rule out other

underlying pathology

http://www.austinfacepain.com/faqs/

TREATMENT OF TRIGEMINAL NEURALGIA

❖ 1st line therapy

❖ Pharmaceutical – carbamezapine (80% symptomatic relief)

❖ Others – oxcarbazine, phenytoin, baclofen, lamotrigine, gabapentin, valproate – best to be used with carbamezapine

❖ 2nd line therapy

❖ Local anesthetics – alcohol, tetracaine, bupivacaine, botulinum A

❖ Inhibits signal transmission

❖ Surgical interventions – rhizotomy, ablation, microvascular decompression, cryotherapy

❖ Help to alleviate pressure along the nerve or inhibit signal altogether

NASOCILIARY

NEURALGIA

NASOCILIARY NEURALGIA

❖ Described as sharp, stabbing pain in distribution of nasociliary nerve (conjunctiva

and nose)

❖ Triggers include pressure to nasal bridge and touching ipsilaterally affected nostril

❖ Charlin's Syndrome – additional symptoms include unilateral nasal congestion,

rhinorrhea, sneezing, keratitis, photophobia, conjunctivitis

NASOCILIARY NEURALGIA

❖ Anatomy of Nasociliary nerve

❖ Branch of Ophthalmic

nerve; V1 branch

❖ Passes through orbital

cavity anteroinferior to

ethmoid enters nasal

septum

❖ Sensory supply to nasal

mucosa, nasal tip, medial

canthus and conjunctivahttps://www.slideshare.net/drhaydarmuneer/nervouse-system-chapter-two

ETIOLOGY OF NASOCILIARY NEURALGIA

❖ Primary causes – vascular compression of the

nerve – nasociliary artery

❖ Secondary causes – inflammation, infection,

tumors, fractures, surgery (rhinoplasty)

DIAGNOSIS OF NASOCILIARY NEURALGIA

❖History

❖ Physical Exam

❖ Pain mapping

❖MRI/XR to rule out underlying pathology

TREATMENT FOR NASOCILIARY NEURALGIA

❖ 1st line therapy

❖ Pharmaceutical – carbamezapine, gabapentin

❖ 2nd line therapy

❖ Local anesthetics – nerve block – lidocaine and

triamcinolone

❖ Surgical – transection of nerve or neurovascular

bundle electro-cauterization

SUPRAORBITAL

NEURALGIA

SUPRAORBITAL NEURALGIA

❖ Described as constant pain with intermittent shock like

paresthesia along nerve distribution, especially in

supraorbital notch

❖ Known as "goggle headache" or "swimmer's headache"

❖ Triggered by exertion such as exercise or sexual activity or

compression (by helmet or goggles)

❖ Can have additional autonomic symptoms such as

lacrimation and rhinorrhea

SUPRAORBITAL NEURALGIA

❖ Anatomy of Supraorbital nerve

❖ Terminal branch of CN V; V1 – Ophthalmic –Frontal nerve

❖ Sensory supply to conjunctiva, forehead, and mid-anterior scalp

https://link.springer.com/chapter/10.1007/978-3-319-27482-9_14

ETIOLOGY OF SUPRAORBITAL NEURALGIA

❖ Primary causes – vascular compression –

supraorbital artery or muscular compression of the

nerve

❖ Secondary causes – infection, trauma,

tumors, surgery (plastic reconstruction to

eyelid/eyebrow region)

DIAGNOSIS OF SUPRAORBITAL NEURALGIA

❖ History

❖ Physical Exam

❖ Pain mapping

❖ MRI/XR to rule out underlying pathology

❖ Diagnostic local anesthesia alleviating pain and symptoms

TREATMENT FOR SUPRAORBITAL NEURALGIA

❖ 1st line therapy

❖ Pharmaceutical – carbamezapine, gabapentin

❖ Alternative – acupuncture and physio/massage

therapy

❖ 2nd line therapy

❖ Local anesthetics – nerve block – bupivacaine and

triamcinolone, botulinum toxin

❖ Surgical – endoscopic supraorbital nerve neurolysis,

microvascular decompression, radiofrequency

ablation

SPHENOPALATINE

NEURALGIA

SPHENOPALATINE NEURALGIA

❖ Described as pressure/fullness, unilateral pain in head, gums,

maxillary teeth that can radiate to neck and upper back

❖ Additional symptoms – nasal congestion, rhinorrhea,

orbit pain, paresthesia over mandible, lacrimation

❖ Known as Sluder's neuralgia

❖ Triggered by exposure to irritants or infection through nasal

mucosa, stress, smoking

SPHENOPALATINE

NEURALGIA

❖ Anatomy of Sphenopalatine nerve

❖ Also known as Pterygopalatine nerve

❖ Sensory branch of CN V; V2 maxillary

❖ Connected to Nervusintermedius nerve as well

❖ Located in pterygopalatine fossa posterior to middle turbinate

❖ Sensory supply to soft palate, pharynx, nasal membrane, lacrimal gland

http://cden.tu.edu.iq/images/New/2016/Lectures/Dr.ban/2/7Pterygopalatine-fossa.pdf

ETIOLOGY OF SPHENOPALATINE NEURALGIA

❖ Primary causes – vascular compression of ganglion

or nerve – sphenopalatine artery

❖ Secondary causes – infection, inflammation, nasal

bone or septal abnormalities, surgery (rhinoplasty)

DIAGNOSIS OF SPHENOPALATINE NEURALGIA❖ History

❖ Physical Exam

❖ Pain mapping

❖ MRI/CT to rule out underlying pathology

❖ Diagnostic local anesthesia alleviating pain

and symptoms

TREATMENT FOR SPHENOPALATINE NEURALGIA

❖ 1st line therapy

❖ Pharmaceutical – carbamezapine

❖ 2nd line therapy

❖ Local anesthetics – nerve block – lidocaine soaked

cotton tip applicator

❖ Surgical – radiofrequency ablation, neurostimulation

NERVUS INTERMEDIUS

NEURALGIA

NERVUS INTERMEDIUS NEURALGIA

❖ Described as brief, paroxysms of stinging and burning pain deep in

the auditory canal that can radiate to parieto-occipital regions and

mandibular region

❖ Diagnostic criteria per International Classification of

Headache Disorder 3 (ICHD-3)

❖ Sub-organization of IHS

❖ Also known as Geniculate neuralgia

❖ Triggered by stimulation of external acoustic meatus

NERVUS INTERMEDIUS NEURALGIA

❖ Anatomy of Nervus Intermedius

❖ Branch of Facial Nerve (CN VII)

❖ Sits between motor component of CN

VII and CN VIII

❖ Sensory branch supplies skin of external

acoustic meatus, mucous membrane of

nasopharynx, soft palate and taste from

anterior 2/3 of tongue

https://www.sciencedirect.com/science/arti

cle/pii/B9780124103900000251

ETIOLOGY OF NERVUS INTERMEDIUS NEURALGIA

❖ Primary causes – vascular compression of the nerve

–AICA/PICA

❖ Secondary causes – herpes zoster, TMJ dysfunction,

tumor, infection, surgery (to face/ear)

DIAGNOSIS OF NERVUS INTERMEDIUS NEURALGIA

❖ History

❖ Physical Exam

❖ Pain mapping

❖ Labs, Cultures, MRI/MRA to rule out underlying pathology

❖ Can be difficult to visualize nervus intermedius nerve

TREATMENT FOR NERVUS INTERMEDIUS NEURALGIA

❖ 1st line therapy

❖Pharmaceutical – carbamezapine, amitriptyline,

lamotrigine, prednisolone

❖ 2nd line therapy

❖Surgical – transection of the nerve or ablation of

geniculate nucleus, microvascular decompression

GLOSSOPHARYNGEAL

(CN IX)

NEURALGIA

GLOSSOPHARYNGEAL NEURALGIA

❖ Described as spasmodic, brief, severe, sharp shooting pains in

pharynx, tonsillar fossa, ear canal, base of tongue and inferior to

gonial angle

❖ International Association for the Study of Pain (IASP)

definition

❖ Triggered by deglutition (cold water), tussive action, mastication,

yawning, talking, touching the ear, and sudden head/neck

movement

❖ Middle aged females commonly affected; L>R for Females; R>L

for Males

GLOSSOPHARYNGEAL NEURALGIA

❖ Anatomy of Glossopharyngeal

nerve (CN IX)

❖Somatic sensory for

oropharynx, posterior 1/3

tongue, eustachian

tube, middle ear, and

mastoid region

http://www.clinicalexams.co.uk/9th-cranial-nerve-tests-for-the-

glossopharyngeal-nerve/

ETIOLOGY OF GLOSSOPHARYNGEAL NEURALGIA

❖ Primary causes – vascular compression of the nerve

– PICA/AICA

❖ Secondary causes – trauma, radiation,

tumors, surgery, Multiple Sclerosis

IASP/IHS & CLASSIFICATIONS OF GLOSSOPHARYNGEAL NEURALGIA

❖ IASP – International

Association for the Study

of Pain

❖Sudden, severe, brief,

recurrent pain in

distribution of CN IX

❖ IHS – International

Headache Society

❖ Classifications

❖ I – classic – episodic

❖Tympanic or peritonsillar –

history of surgery

❖ II – symptomatic – constant

DIAGNOSIS OF GLOSSOPHARYNGEAL NEURALGIA

❖ History

❖ Physical Exam

❖ Pain mapping

❖ CT-A, MRI/MRA

❖ Identify vascular compression or malignancies

❖ Panoramic X-ray/CT to rule out Eagle's syndrome

❖ EKG to rule out cardiac pathology

TREATMENT FOR GLOSSOPHARYNGEAL NEURALGIA

❖ 1st line therapy

❖ Pharmaceutical – carbamezapine, gabapentin, Vitamin B12,

SSRIs (low-dose)

❖ Secondary – baclofen, dextromethorphan, lamotrigine

❖ 2nd line therapy

❖ Local anesthetics – nerve block -- alcohol, bupivacaine, steroids

❖ Surgical – transection of nerve or gamma-knife surgery

SUPERIOR LARYNGEAL

NEURALGIA

SUPERIOR LARYNGEAL NEURALGIA

❖ Described as an anterior neck pain syndrome that presents as

unilateral, paroxysmal stabbing and burning pain

❖ Pain is sharp and begins posterolateral to thyrohyoid cartilage,

radiates up to gonial angle and inferior to earlobe and can radiate

inferiorly along anterolateral neck

❖ Triggered by deglutition, coughing, talking, ipsilateral head

turning, and foreign body sensation is common

SUPERIOR LARYNGEAL NEURALGIA

❖ Anatomy of Superior Laryngeal nerve

❖ Branch of the vagus nerve

❖ From inferior ganglion of vagus

nerve

❖ Located at posterior thyrohyoid

space

❖Lateral to pharynx, posterior

to internal carotid artery

❖ Sensory branch is internal

supplying laryngeal mucosa,

epiglottis and base of tongue

https://journals.sagepub.com/doi/pdf/10.1177/0145561318823373

ETIOLOGY OF SUPERIOR LARYNGEAL NEURALGIA

❖ Primary causes – local inflammation of the nerve or

excessive talking leading to microtrauma

❖ Secondary causes – infections, trauma,

tumors, surgery to anterolateral neck region (carotid

endarterectomy)

DIAGNOSIS OF SUPERIOR LARYNGEAL NEURALGIA

❖ History

❖ Physical Exam

❖ Pressure point on thyrohyoid region elicits pain

❖ Pain mapping

❖ Diagnostic local anesthesia alleviating pain and symptoms

TREATMENT FOR SUPERIOR LARYNGEAL NEURALGIA

❖ 1st line therapy

❖ Pharmaceutical – carbamezapine, gabapentin,

lacosamide, amitriptyline

❖ 2nd line therapy

❖ Local anesthetics – nerve block – Lidocaine and

triamcinolone

❖ Surgical – transection of the nerve

OCCIPITAL

NEURALGIA

OCCIPITAL NEURALGIA❖ Described as paroxysmal shooting or stabbing pain in distribution of

greater occipital nerve (GON) or lesser occipital nerve (LON) with

tenderness to palpation

❖ Classified by the International Headache Society (IHS)

❖ Also known as C2 Neuralgia; Pain spreads to upper neck, posterior scalp,

behind the eyes, usually unilateral but can be bilateral, occipital allodynia

is common

❖ Triggered by compression of GON/LON, head on pillow with

hyperextension or rotation of neck elicits pain

❖ F>M; GON (90%), LON (10%)

OCCIPITAL NEURALGIA

❖ Additional symptoms include:

❖ Vision impairment or ocular pain (67%)

❖ Tinnitus (33%)

❖ Dizziness (50%)

❖ Nausea (50%)

❖ Nasal congestion (17%)

OCCIPITAL NEURALGIA

❖ Anatomy of the Occipital

nerve

❖ GON medial branch of

dorsal ramus at C2

❖ LON medial branch of

dorsal ramus at C2

and C3

❖ Sensory supply to skin

of occipital region to

vertex

https://painendshere.com/treatments/occipital-nerve-block-injections/

ETIOLOGY OF OCCIPITAL NEURALGIA

❖ Primary causes – vascular – occipital artery or

muscular compression of nerve

❖ Secondary causes – inflammation, trauma, arthritis,

cranio-cervical instability, tumors, congenital

abnormalities

DIAGNOSIS OF OCCIPITAL NEURALGIA

❖ History

❖ Physical Exam

❖ Pain mapping

❖ Tinel's sign +

❖ MRI/CT/XR to rule out underlying pathology

❖ Diagnostic local anesthesia alleviating pain and symptoms

TREATMENT FOR OCCIPITAL NEURALGIA

❖ 1st line therapy

❖ Pharmaceutical – carbamezapine, gabapentin, SSRIs

❖ Alternates – physical therapy, CBT

❖ 2nd line therapy

❖ Local anesthetics – nerve block – lidocaine with

steroid, botulinum toxin A

❖ Pulsed radiofrequency inhibits firing of A-delta and

small C fibers

❖ Surgical – rhizotomy, occipital neurolysis, nerve root

decompression

GREAT AURICULAR

NEURALGIA

GREAT AURICULAR NEURALGIA

❖ Presents with paroxysmal spells of unilateral, sharp

pain along the preauricular-parotid, gonial angle

and mastoid regions

❖ Triggered by head movement to the opposite side

GREAT AURICULAR

NEURALGIA

❖ Anatomy of the Great Auricular

nerve

❖ The great auricular nerve is

a purely sensory branch of

C2-C3 anastomosing with

ansa cervicalis

❖ Anterior branch

supplies parotid region

skin

❖ Posterior branch

supplies mastoid

region skin

http://www.ajnr.org/content/21/3/568

ETIOLOGY OF GREAT AURICULAR NEURALGIA

❖Primary causes – idiopathic in most cases

❖Secondary causes – surgery or tumors of the neck

DIAGNOSIS OF GREAT AURICULAR NEURALGIA

❖History

❖Physical Exam

❖MRI/CT/XR to rule out underlying pathology

TREATMENT FOR GREAT AURICULAR NEURALGIA

❖ 1st line therapy

❖Pharmaceutical – pregabalin, amitriptyline

❖ 2nd line therapy

❖Local anesthetics – nerve block – lidocaine with

steroid

TROCHLEAR

NEURALGIA

TROCHLEAR NEURALGIA

❖ Described as frontal, periorbital, with or

without eye pain, peri-trochlear inflammation

❖ Dull, achy, photophobia, binocular diplopia, pain in

superomedial orbit

❖ ICHD3/IHS

❖Aggravated by downward eye movement

TROCHLEAR NEURALGIA

❖ Anatomy of the

Trochlear nerve

❖ Trochlear nerve

cranial nerve IV

superior oblique

❖ downward and

adduct eye

http://notezonnursing.blogspot.com/2011/05/cranial-nerve-iv-trochlear-nerve-review.html

ETIOLOGY OF TROCHLEAR NEURALGIA

❖ Primary causes – idiopathic, with or without

associated headache

❖ Secondary causes – inflammation, autoimmune

disorders

DIAGNOSIS OF TROCHLEAR NEURALGIA

❖ History

❖ Physical Exam

❖ MRI/CT to rule out underlying pathology

❖ Diagnostic local anesthesia alleviating pain and symptoms

TREATMENT FOR TROCHLEAR NEURALGIA

❖ 1st line therapy

❖ Pharmaceutical – carbamezapine

❖ 2nd line therapy

❖ Local anesthetics – nerve block –

lidocaine/dexamethasone

OTHERS

TO

CONSIDER

OTHER NEURALGIAS/HEADACHES

❖ External Compression headache

❖ DDx for Supraorbital Neuralgia

❖ Cold Stimulus headache

❖ DDx for Sphenopalatine Neuralgia or Glossopharyngeal Neuralgia

OTHER NEURALGIAS/HEADACHES

❖ Eagle's Syndrome

❖ DDx for Glossopharyngeal Neuralgia

❖ Neck Tongue Syndrome

❖ DDx for Occipital Neuralgia or Glossopharyngeal

Neuralgia

THANK YOU!

QUESTIONS?

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