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    Trigeminal Neuralgia - Case Report and Review

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    The document describes a case report of a patient with trigeminal neuralgia who was treated with gamma knife radiosurgery. It then provides background on trigeminal neuralgia including its history, pathogenesis, classification, and treatment approaches. Gamma knife radiosurgery was used to non-invasively treat the patient's trigeminal neuralgia by creating a lesion on the trigeminal nerve.

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    Trigeminal Neuralgia - Case Report and Review

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    The document describes a case report of a patient with trigeminal neuralgia who was treated with gamma knife radiosurgery. It then provides background on trigeminal neuralgia including its history, pathogenesis, classification, and treatment approaches. Gamma knife radiosurgery was used to non-invasively treat the patient's trigeminal neuralgia by creating a lesion on the trigeminal nerve.

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    The document describes a case report of a patient with trigeminal neuralgia who was treated with gamma knife radiosurgery. It then provides background on trigeminal neuralgia including its history, pathogenesis, classification, and treatment approaches. Gamma knife radiosurgery was used to non-invasively treat the patient's trigeminal neuralgia by creating a lesion on the trigeminal nerve.

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    Trigeminal Neuralgia - Case Report and Review

    Uploaded by

    Garry
    The document describes a case report of a patient with trigeminal neuralgia who was treated with gamma knife radiosurgery. It then provides background on trigeminal neuralgia including its history, pathogenesis, classification, and treatment approaches. Gamma knife radiosurgery was used to non-invasively treat the patient's trigeminal neuralgia by creating a lesion on the trigeminal nerve.

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    of 4

    Trigeminal Neuralgia: Case Report

    and Review
    Benjamin M. Zussman, BS1; Yaron A. Moshel, MD PhD2
    Jefferson Medical College, Philadelphia, PA
    Neurosurgery Department, Thomas Jefferson University Hospitals, Philadelphia, PA

    1
    2

    PATIENT VIGNETTE
    We report the case of an 86-year-old female who described 15-years of sharp, stabbing pain that
    radiated down the distribution of the second and third divisions of her right trigeminal nerve. She
    described two trigger points, one on her right cheek and a second intra-oral trigger. Her symptoms
    were often triggered by eating and she had begun to loose weight secondary to the pain. She denied
    having any baseline pain between the episodes of lancinating pain. She denied any contralateral pain,
    dysasthetic pain or any burning pain sensation. She denied any pain along the first trigeminal division
    and did not recently undergo any dental work or have a history of dental carries. Her symptoms had
    been initially well controlled with Carbamazepine 200 mg BID, but had recently worsened despite
    increasing the dosage to 600 mg BID when she started to develop medication-related side effects.
    Her past medical history was unremarkable, and on examination she was neurologically intact. Her
    brain magnetic resonance imaging (MRI) showed no masses or gross abnormalities, and she was
    diagnosed with trigeminal neuralgia.
    Neurosurgical intervention was pursued because the patient was refractory to medical therapy and
    had worsening symptoms. Non-invasive Gamma Knife radiosurgery was recommended because
    of the patients surgical risk profile and preference for a non-invasive procedure. Using Leksell
    stereotactic frame, a stereotactic MRI dataset was generated with a gradient echo and CISS sequence.
    On the Leksell gamma planning workstation we identified the right trigeminal nerve and prescribed
    90 Gy to the 100% isodose line using a single 4-mm shot such that the 20% isodose line was tangential
    to the brainstem. The patient tolerated the procedure well and reported complete resolution of her
    symptoms at one-month follow-up.

    DISCUSSION
    For patients complaining of facial pain, the differential diagnosis is broad. It includes dental
    abnormalities, such as caries, root abscesses,
    and broken teeth; temporomandibular joint
    pain; eye pain due to glaucoma, orbital cellulitis,
    or trauma; facial trauma including bony fractures; tumors of the facial bones or infiltrating
    the trigeminal nerve; giant cell arteritis; trigeminal autonomic cephalgias, such as cluster
    headache and paroxysmal hemicrania; primary
    headache syndromes including migraine and
    tension types; postherpatic neuralgia; systemic
    autoimmune disorders including lupus erythematosis; and other structural pathology, such as
    cerebellopontine angle tumors and cysts.1
    The International Headache Society (ihsclassification.org) defines classical trigeminal
    neuralgia as follows: (1) Paroxysmal attacks of
    pain lasting from a fraction of a second to two
    minutes, affecting one or more divisions of the
    trigeminal nerve; (2) Pain has at least one of

    Figure 1

    Figure 2

    Gamma Knife Radiosurgery treatment plan screen shot

    Bausch (National Library of Medicine)

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    Pain

    the diagnosis and the decision for potential


    surgical exploration remains a purely clinical
    diagnosis irrespective of MRI presence of vascular compression.

    HISTORICAL CONTEXT
    The earliest reported case of trigeminal
    neuralgia was Johannes Laurentis Bausch
    (1605-1665), physician and president of the
    Imperial Leopoldian Academy of Natural
    Sciences. Bausch himself suffered from severe
    trigeminal neuralgia, and the course of his
    illness was detailed in his eulogy, which was
    published posthumously by the Academy.4
    Next, John Locke (1632-1704), physician and
    famed philosopher, was summoned to visit the
    Countess of Northumberland (wife of an English Ambassador), who suffered from crippling
    trigeminal neuralgia. Locke recorded the case.4.

    Figure 3
    Locke (National Library of Medicine)

    the following characteristics: intense, sharp,


    superficial or stabbing; precipitated from trigger areas or by trigger factors; and (3) Attacks
    are stereotyped in the individual patients.
    Additionally, there can be no clinically evident
    neurological deficit, and the symptoms must not
    be attributed to another disorder. In contrast,
    atypical trigeminal neuralgia refers to symptoms
    that are not paroxysmal, have associated chronic
    pain, and/or a dysasthetic quality.

    The French term for trigeminal neuralgia, tic


    douloureux, was coined by surgeon Nicolaus
    Andre (born 1704), who reported a case series
    of five tic patients and, amazingly, practiced
    trigeminal nerve ablation by applying caustic
    substances through infraorbital foramina.4
    John Hunter (1728-1793), an English anatomist
    and physician, further narrowed in on the neurological basis for trigeminal neuralgia, stating
    that:
    There is one disease of the jaw which seems in
    reality to have no connection with the teeth, but
    of which the teeth are generally suspected to be

    Bell (National Library of Medicine)

    the cause...I have known cases of this kind where


    all the teeth of the affected side of the jaw have
    been drawn out, and the pain has continued in
    the jaw...Hence it should appear that the pain
    in question does not arise from any disease in
    the part, but it is entirely a nervous affection.4
    Sir Charles Bell (1774-1842), famed Scottish
    neuroanatomist, characterized the separate
    functions of the trigeminal (V) and facial (VII)
    nerves. Bell was the first to localize trigeminal
    neuralgia to the trigeminal (V) nerve.1
    Armed with this neuroanatomical knowledge,
    surgeons began to explore how they might
    treat this disorder. Harvey Cushing (18691939) pioneered a temporal fossa approach
    beneath the middle meningeal artery, aiming
    to completely extirpate the trigeminal ganglion.
    Cushing later shifted to isolated sensory root
    avulsion to spare motor function.5 Walter
    Dandy (1886-1946) performed trigeminal root
    neurotomies via a posterior fossa approach and
    noted that The [trigeminal nerve] sensory root
    is frequently indented, lifted up, or bent at an
    angle by the artery...This I believe is the cause
    of tic douloureux.6

    More recently, Burchiel and colleagues2 developed a simplified framework for defining and
    classifying trigeminal neuralgia (Table 1).
    Importantly, trigeminal neuralgia traditionally
    referred to as classical-type is redefined as TN1,
    while idiopathic facial pain that is aching, throbbing, or burning for more than 50% of the time
    (constant pain) is termed TN2. This distinction
    between shock-like (TN1) and constant (TN2)
    pain is the most significant predictor of longterm success after treatment.3
    Preoperative MRI is useful to rule out any other
    potential pathological etiologies such as tumor
    or demyelinating disease. More advanced MR
    imaging can identify the presence of vascular
    compression at the root entry zone, which
    can aid pre-operative planning. Nevertheless,

    Figure 5

    Figure 4
    Hunter (National Library of Medicine)

    More recently, Peter Jannetta, an American (and


    Pennsylvanian!) neurosurgeon, established
    open surgical microvascular decompression of
    the trigeminal nerve as an effective procedure
    for treating trigeminal neuralgia7 (discussed
    below). Lars Leksell (1907-1986), Swedish neurosurgeon and inventor, famous for stating that

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    Table 1. Burchiel definition and classification of trigeminal neuralgia (2)


    Diagnostic Classification

    Defining Symptomatology

    Idiopathic
    TN1

    Sharp, shooting, electrical shock-like, episodic


    pain

    TN2

    Aching, throbbing, burning, >50% constant pain

    Trigeminal Injury
    Neuropathic Pain

    Unintentional (facial trauma; oral op; ear, nose,


    & throat op; skull base op; posterior fossa op; or
    stroke)

    Deafferentation Pain

    Intentional (neurectomy, gangliolysis, rhizotomy,


    nucleotomy, tractotomy, or other denervating
    procedure)

    Symptomatic

    Associated with multiple sclerosis

    Postherpetic

    Resulting from an outbreak of facial herpes zoster

    Atypical Facial Pain

    Somatoform pain disorder

    tools used by the surgeon must be adapted to


    the task and where the human brain is concerned, no tool can be too refined, invented
    Gamma Knife Radiosurgery, which has since
    been used to introduce radiosurgical lesions
    of the trigeminal nerve to non-invasively
    treat trigeminal neuralgia (discussed below).
    Indeed, todays multimodality armamentarium
    for treating trigeminal neuralgia is broad.

    PATHOGENESIS
    Our understanding of the pathogenesis of
    trigeminal neuralgia continues to evolve. The
    leading explanation involves trigeminal nerve
    root compression that causes demyelination.
    Multiple different anatomical variants (classically the superior cerebellar artery) may
    cause arterial and/or venous compression of
    the trigeminal nerve root, usually within a few
    millimeters of its entry into the brainstem.8
    Pathological analyses demonstrate a focal
    zone of chronic demyelination immediately
    beneath the region of nerve indentation and
    limited to the immediate vicinity of the point
    of vascular indentation.9
    In turn, this demyelination causes ectopic
    (erratic) impulses and ephaptic (indirect)
    cross-firing between nerve fibers. Experimental evidence from a model of purposefully
    demyelinated nerve fibers in cat dorsal spinal
    cord white matter demonstrates spontaneous,
    ectopic activity.10 Ephaptic coupling refers to
    the indirect firing that occurs between neural
    fibers through extracellular electrical fields,

    JHN JOURNAL

    as opposed to direct synaptic transmission.


    Ephaptic coupling has been experimentally
    demonstrated between immediately adjacent
    non-myelinated axons.11
    Abnormal ectopic and ephaptic electrical
    impulses may lead to the development of
    clinical pain hypersensitivity. Neuronal
    plasticity is believed to be the underlying
    cause of hypersensitivity phenomena including allodynia, hyperalgesia, and trigeminal
    neuralgia.12
    Knowledge of pathogenesis can directly
    inform treatment approaches. Structural
    nerve root compression can be addressed with
    surgical decompression. Abnormal neuronal
    firing can be addressed with medications such
    as Carbamazepine, which stabilize neuronal
    ion channels, reducing neuronal excitability.
    Finally, hypersensitivity mediated by misdirected neuronal plasticity can be addressed
    with ablative techniques directed to the nerve
    itself.

    TREATMENTS
    Medical therapy for trigeminal neuralgia
    includes anticonvulsant drugs. In 1963
    Carbamazepines efficacy in treating this disorder was first demonstrated by Sigfrid Blom.13
    Carbamazepine stabilizes inactivated voltagegated sodium channels, down-regulating
    cellular excitability. It remains the first-line
    drug of choice today. Alternative medications
    include Oxcarbazepine, Baclofen, Gabapentin,
    Lamotrigine, and Pimozide.

    When medical therapy fails, surgical therapy


    may be indicated. Surgical therapy for trigeminal neuralgia consists of two broad categories,
    microvascular decompression, and ablative
    procedures. Microvascular decompression
    usually entails a retrosigmoid approach to the
    upper cerebellopontine angle and identification
    of the offending vessel causing nerve decompression. The most frequently identified culprit
    is a superior cerebellar artery loop which, once
    freed by sharp dissection, can be mobilized
    away from the nerve and kept separated from
    the nerve with a piece of shredded Teflon felt.14
    Ablative procedures include rhizotomy (nerve
    obliteration) and radiosurgery. Rhizotomy is
    a percutaneous procedure, whereby a cannula
    is passed through the foramen ovale and the
    sensory component of the trigeminal nerve
    is destroyed by radiofrequency thermocoagulation, mechanical balloons (e.g., Fogarty
    catheter), or neurotoxic chemicals (e.g., glycerol). Radiosurgery utilizes Gamma Knife to
    stereotactically direct conformal, high-dose
    radiation to the cisternal portion of the trigeminal nerve. The procedure involves placement of
    a head frame, which allows for the acquisition
    of a stereotactic MRI scan to accurately identify
    the nerve and safely target the radiation dose.
    While medical therapy is generally accepted as
    the first-line treatment for trigeminal neuralgia,
    best surgical practice for patients refractory to
    medical therapy remains undefined. In a landmark 1996 New England Journal article, Barker
    and colleagues demonstrated that microvascular decompression is safe and effective, with a
    high rate of long-term success.15 More recently
    a review of ablative procedures found that
    radiofrequency thermocoagulation offers the
    highest rates of complete pain relief but also the
    highest rates of complications (e.g., masticatory weakness, corneal numbness, dysesthesia
    (impairment of sensitivity)), when compared
    with mechanical balloon, chemical injection,
    and radiosurgical ablation techniques.16 The
    sole prospective study of Gamma Knife Radiosurgery showed that radiosurgery is a safe and
    effective treatment, associated with delayed
    pain cessation but fewer complications than
    minimally-invasive ablative techniques.17
    In practice, treatment decisions are centered
    around individual patient and treatment
    team characteristics and preferences. Young
    patients with TN1-type TN are often excellent
    candidates for open surgery, and microvascular
    decompression will typically achieve a complete

    Pain

    cure. Elderly patients with increased medical


    risks are often appropriate candidates for ablative therapies, which produce immediate relief.
    Finally, for elderly patients willing to wait a few
    weeks for symptoms to resolve, radiosurgery is
    an ideal choice because of its high efficacy and
    low complication profile.

    4. Eboli, P., Stone, J. L., Aydin, S. & Slavin, K. V. Historical


    characterization of trigeminal neuralgia.Neurosurgery 64,
    1183-6; discussion 1186-7 (2009).

    REFERENCES

    7. Jannetta, P. J. Arterial compression of the trigeminal nerve at


    the pons in patients with trigeminal neuralgia.J. Neurosurg.
    26, Suppl:159-62 (1967).

    1. Prasad, S. & Galetta, S. Trigeminal neuralgia: historical notes


    and current concepts. Neurologist 15, 87-94 (2009).
    2. Eller, J. L., Raslan, A. M. & Burchiel, K. J. Trigeminal
    neuralgia: definition and classification. Neurosurg. Focus.
    18, E3 (2005).
    3. Miller, J. P., Acar, F. & Burchiel, K. J. Classification of trigeminal neuralgia: clinical, therapeutic, and prognostic implications in a series of 144 patients undergoing microvascular
    decompression. J. Neurosurg.111, 1231-1234 (2009).

    5. Adams, H. et al. Harvey Cushings case series of trigeminal


    neuralgia at the Johns Hopkins Hospital: a surgeons quest
    to advance the treatment of the suicide disease. Acta
    Neurochir. (Wien) 153, 1043-1050 (2011).
    6. Dandy, W. E. The Treatment of Trigeminal Neuralgia by the
    Cerebellar Route. Ann. Surg. 96, 787-795 (1932).

    8. Hardy, D. G. & Rhoton, A. L.,Jr. Microsurgical relationships


    of the superior cerebellar artery and the trigeminal nerve. J.
    Neurosurg. 49, 669-678 (1978).

    11. Ramon, F. & Moore, J. W. Ephaptic transmission in squid


    giant axons. Am. J. Physiol. 234, C162-9 (1978).
    12. Woolf, C. J. & Salter, M. W. Neuronal plasticity: increasing
    the gain in pain. Science 288, 1765-1769 (2000).
    13. Blom, S. Tic Douloureux Treated with New Anticonvulsant;
    Experiences with G 32883. Arch. Neurol.9, 285-290 (1963).
    14. McLaughlin, M. R. et al. Microvascular decompression
    of cranial nerves: lessons learned after 4400 operations. J.
    Neurosurg. 90, 1-8 (1999).
    15. Barker, F. G.,2nd, Jannetta, P. J., Bissonette, D. J., Larkins,
    M. V. & Jho, H. D. The long-term outcome of microvascular
    decompression for trigeminal neuralgia. N. Engl. J. Med. 334,
    1077-1083 (1996).

    9. Love, S. & Coakham, H. B. Trigeminal neuralgia: pathology


    and pathogenesis. Brain 124, 2347-2360 (2001).

    16. Lopez, B. C., Hamlyn, P. J. & Zakrzewska, J. M. Systematic


    review of ablative neurosurgical techniques for the treatment
    of trigeminal neuralgia. Neurosurgery 54, 973-82; discussion
    982-3 (2004).

    10. Smith, K. J. & McDonald, W. I. Spontaneous and evoked


    electrical discharges from a central demyelinating lesion. J.
    Neurol. Sci. 55, 39-47 (1982).

    17. Regis, J. et al. Prospective controlled trial of gamma knife


    surgery for essential trigeminal neuralgia. J. Neurosurg. 104,
    913-924 (2006).

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