trigeminal neuralgia tic douloureuxweb.brrh.com/.../trigeminalneuralgia_final_0928.pdftrigeminal...
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Trigeminal Neuralgia (tic douloureux)
Gregg Goldin, MD
Timothy Miller, MD
9/28/18
Neurology and Neurosurgery Grand Rounds
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Disclosures
-None
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Objectives
• 1) Epidemiology, pathophysiology, and medical management (GG)
• 2) Surgical options (TM)
• 3) Radiosurgical option (GG)
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Debilitating neuropathic condition involving the fifth (trigeminal) cranial nerve
• Chronic facial pain disorder
• Severe/episodic
• Typically unilateral
• Usually affects mandibular and maxillary divisions
• Isolated involvement of the ophthalmic division is much less common
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• Early 1500’s: Leonardo da Vinci sketches the cranial nerves
• 1600: Johannes Fehr and Elias Schmidt (secretaries of philosopher John Locke) describe the clinical entity
• 1756: Nicolas Andre coined the term tic douloureux
• 1773: John Fothergill described the first case series
• 1820: Charles Bell first localized the pain to the trigeminal nerve
• 1925: Walter Dandy sectioned the trigeminal nerve
• Late 1960’s: Peter Jannetta introduced the microvascular decompression (MVD)
• 1971, Lars Leksell treated first patient with stereotactic radiosurgery (SRS)
Nicolas Andre John Fothergill
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Epidemiology • Prevalence = ~40,000
• Incidence = ~15,000/year
• Condition of elderly
• Slightly more common in Women
• >$100 million spent on procedures alone annually in the US
Harsha KJ, et al. Imaging of vascular causes of trigeminal neuralgia. J Neuroradiol. 2012;39(5):281–9
Mauskop A. Trigeminal neuralgia (tic douloureux). J Pain Symptom Manage. Apr 1993;8(3):148-54
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Natural History (Classical TN)
• Misdiagnosed and under treated
• No clear natural history
• Paroxysmal attacks
• Pain tends to occur in cycles
• Pain-free intervals typically grow shorter
• Patients often live in fear and anticipation
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Natural History
• Triggers: sensory stimulation • touch, certain head movements, talking, chewing,
swallowing, shaving, brushing teeth, or even a cold draft.
•
• In many, pain is generated spontaneously
• Stabbing electric shocks, burning, sharp, pressing, crushing, exploding or shooting pain
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Pathophysiology • Believed to involve loss of the myelin
around the trigeminal nerve
• Etiologies include: • Neurovascular compression (most
commonly accepted) • at TN entrance into the brainstem (pons).
• In one study, 64% of the compressing vessels were identified as an artery, • most commonly the superior cerebellar (81%).
• Multiple Sclerosis
• Tumor
• Arteriovenous Malformation
• Facial Injury
Pamir MN, et al. Microvascular decompression in the surgical management of trigeminal
neuralgia. Neurosurg Rev. 1995;18(3):163–7.
Lummel N, et al. Diffusion tensor imaging of the trigeminal nerve in patients with trigeminal
neuralgia due to multiple sclerosis. Neuroradiology. 2015;57(3):259–67.
Samadian M, et al. Trigeminal Neuralgia Caused by Venous Angioma: A Case Report and
Review of the Literature. World Neurosurg. 2015;84(3):860–4.
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Pathophysiology
• Mechanical compression often occurs as the nerve leaves the pons
• Nerve region especially susceptible to pathologic changes from vascular contact is the Redlich-Obersteiner’s zone, also known as the root entry zone (REZ).
• REZ is characterized by nerve axons ensheathed in central myelin, transitioning to peripheral myelin.
Agrawal SM, Kambalimath DH. Trigeminal neuralgia
involving supraorbital and infraorbital nerves. Natl J Maxillofac Surg. 2010;1(2):179–82.
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Neurophysiological Mechanism “Ignition Therory”
• Vascular contact leads to instability and atrophy
• Nerves become hyperexcitable and generate abnormal discharges
• Spontaneous firing results in burning and paresthesias
• Cascade propagation to the trigeminal nucleus is perceived as burst of pain.
• Intense flares from synchronization of bursts • “ephaptic crosstalk” • increased ions or neurotransmitters in the
interstitial space Devor M, Amir R, Rappaport ZH. Pathophysiology of
trigeminal neuralgia: the ignition hypothesis. The Clinical
journal of pain. 2002;18(1):4–13.
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Diagnosis • Diagnosis of exclusion
• Neuro exam findings are usually normal.
• Rapid spreading of pain, bilateral affliction, or involvement of other cranial nerves suggests a systemic cause • multiple sclerosis
• expanding cranial tumors
• Nerve compression on MRI often not visualized
• Blood work indicated if medical therapy is contemplated or to exclude collagen vascular diseases
Haines SJ, Jannetta PJ, Zorub DS. Microvascular relations of the trigeminal nerve: an anatomical study with clinical correlation. Journal of
neurosurgery. 1980;52(3):381–386.
Hamlyn PJ, King TT. Neurovascular compression in trigeminal neuralgia: a clinical and anatomical study. Journal of
neurosurgery. 1992;76(6):948–954.
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Differential Diagnosis • Trigeminal neuropathy
• postherpetic neuralgia (PHN)
• Neoplasms
• Granulomatous inflammation
• odontogenic pain, geniculate neuralgia, glossopharyngeal neuralgia, temporomandibular disorders, sinusitis, cluster headache, hemicrania, and SUNCT (short-lasting, unilateral neuralgia from headache attacks with conjunctival injection and tearing) syndrome
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MRI Characteristics • MRI (using heavily T2 weighted (FIESTA) sequences) to visualize cranial
nerves and exclude neoplasia
• Anatomical
• Volume: depletion of the trigeminal nerve volume is correlated with TN
• Neurovascular compression
• Functional • Decreased Fractional Anisotropy: indicator of microstructural changes and reduced “connectivity”
• Increased Apparent Diffusion Coefficient: associated with nerve atrophy
World Neurosurg. 2017 Feb;98:89-97. doi: 10.1016/j.wneu.2016.10.104. Epub 2016 Oct 27.
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Treatment
• Medical treatment is first line
• Surgical interventions (including SRS) for refractory cases
• up to 50% of patients with classic TN will eventually require surgery
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Medical Therapy
• Interrupt the temporal summation of afferent impulses
• After breakthrough on a single agent, a second and even third medication may be required • Anticonvulsant medicines—carbamazepine (most effective), oxcarbazepine, topiramate,
clonazepam, phenytoin, lamotrigine, and valproic acid. • many adverse CNS side effects
• up to 44% relapse or have unsatisfactory relief
• Gabapentin or baclofen
• Tricyclic antidepressants used to treat pain described as constant, burning, or aching
• Opiates are typically not helpful
Dalessio DJ. Trigeminal neuralgia. A practical approach to treatment. Drugs. Sep 1982;24(3):248-55.
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Surgical Management of Facial Pain
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Classification of Facial Pain
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Classification of Facial Pain
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Trigeminal Neuralgia Diagnostic Questionnaire
https://neurosurgery.ohsu.edu/tgn.php
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Facial Pain Treatment Algorithm
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Trigeminal Tract Neuroanatomy
-Semilunar Ganglion -Gasserian Ganglion -Gasser’s Ganglion -Trigeminal Ganglion
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Trigeminal Tract Neuroanatomy VPM
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Trigeminal Tract Neuroanatomy
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Peripheral Trigeminal Nerve Interventions
V3 block V2 block
Peripheral nerve blockade
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Peripheral Trigeminal Nerve Interventions
Neurectomy
Infraorbital
Supraorbital
Inferior Alveolar
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Peripheral Trigeminal Nerve Interventions
Peripheral Neurostimulation
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Interventions at the Ganglion
Percutaneous Radiofrequency Trigeminal Gangliolysis
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Interventions at the Ganglion
Balloon Compression and
Glycerol Rhizolysis
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Interventions at the Ganglion
Gasserian Stimulation
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Dorsal Root Entry Zone
Microvascular Decompression
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Dorsal Root Entry Zone
Trigeminal Rhizotomy
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Trigeminothalamic Tract
-Trigeminal Tractotomy -Nucleus Caudalis DREZ
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Trigeminothalamic Tract
High Cervical Spinal Cord Stimulation
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Thalamus
Deep Brain Stimulation
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Cortex
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Temporary Trials
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Stereotactic Radiosurgery • Absence of surgical risk
• Targets focused beams of high dose radiation to the trigeminal nerve at exit from the brainstem
• Axonal degeneration and necrosis disrupt transmission of pain signals
• Single treatment: maximum dose of 70 to 90 Gy
• Average pain-free is about the same as for radiofrequency (RF) rhizotomy, or around 3 years
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Delineate the retrogasserian sensory root of the prepontine TN
• 1) anatomy tends to be symmetric.
• 2) Find the trigeminal eminence
• 3) Find Meckel’s cave
• 4) trigeminal sensory root will travel between brainstem and Meckel’s cave
• 5) Reference the coronal and sagittal images
• 6) target volume encompassed by 2 to 3, two mm thick MR slices
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Gammaknife
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Cyberknife Frameless SRS
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• initial pain relief in up to 90% of cases,
• lag time ranging from 1 to 3 months.
• side effects: facial numbness, ranging from 20% to 32%, and less commonly dysesthesias and weakness
• One of the challenges of SRS is the long-term durability of pain relief
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• Of 59 studies, 41 reported on SRS (n =5242), 12 reported on MVD (n= 8028), and 5 reported on both SRS (n = 535) and MVD (n =237).
• Efficacy • In the group treated with SRS, the initial success rate was 71.1%. Success rates
at follow-up (2 years, between years 2 and 5, and after 5 years) amounted to 77.8%, 63.1%, and 63.8%, respectively.
• In the group treated with MVD, the initial success rate was 86.9%. At followup, success rates were 91.4% (2 years), 80.6% (between years 2 and 5), and 84% (>5 years), respectively.
• With a median follow-up time of 36 months, the median recurrence rate was 11% for MVD and 25% for SRS, respectively
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Complications
• less frequent complications: • MVD: perioperative mortality (0.31%),
cerebrospinal fluid leak (2.73%), and the combined percentage of cerebrovascular, cardiologic, pneumologic, or thromboembolic events (3.92%) in MVD.
• SRS: Keratitis, Anesthesia dolorosa (0.04%), tinnitus (0.15%), brainstem edema (0.06%), and chronic fatigue (0.79%)
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Conclusions
• MVD yields more immediate and better long-term relief
• Although surgical complications associated with MVD are not negligible, dysesthesia and other CN effects after SRS may significantly compromise the patient’s quality of life too.
• MVD is a valid first-line treatment option for patients free of comorbidities. SRS would be advised in patients with a high surgical and anesthetic risk.
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Questions?