CRANIAL NEURALGIAS Presented by: Neha Sharma M.D. Date: September 27th, 2019 TYPES OF NEURALGIAS ❖ TRIGEMINAL NEURALGIA ❖ GLOSSOPHARYNGEAL NEURALGIA ❖ NASOCILIARY NEURALGIA ❖ SUPERIOR LARYNGEAL NEURALGIA ❖ SUPRAORBITAL NEURALGIA ❖ OCCIPITAL NEURALGIA ❖ SPHENOPALATINE NEURALGIA ❖ GREAT AURICULAR NEURALGIA ❖ NERVUS INTERMEDIUS 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 ❖ Anatomy of Trigeminal Nerve ❖ Cranial Nerve V ❖ Three Branches: Ophthalmic, Maxillary and Mandibular ❖ Sensory supply to forehead/supraorbital, cheeks and jaw https://www.nf2is.org/cn5.php TRIGEMINAL NEURALGIA – TRIGGERS ❖ Mastication (73%) ❖ Eating (59%) ❖ Touch (69%) ❖ Talking (58%) ❖ Brushing Teeth (66%) ❖ 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 ❖ Classifications for the Study of Pain ❖ I – idiopathic, sharp, shooting, electric, ❖ Sudden, unilateral, brief, episodic intermittent pain in at least ❖ II – idiopathic, aching, burning, >50% one branch of CN V constant ❖ III – secondary to injury, trauma, surgery to face/cranium, stroke ❖ IHS – International Headache Society ❖ IV – deafferentation by intentional injury (ex: rhizotomy) ❖ Excruciating, unilateral, ❖ V – associated with MS electric pain in at least one branch of CN V ❖ 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 https://www.slideshare.net/drhaydarmuneer/nervouse-system-chapter-two canthus and conjunctiva 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- https://link.springer.com/chapter/10.1007/978-3-319-27482-9_14 anterior scalp 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 Nervus intermedius 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 ❖ IHS – International Association for the Study Headache Society of Pain ❖ Classifications