Trigeminal Neuralgia (Tic Douloureux)
Trigeminal neuralgia, a condition affecting the fifth cranial nerve, is characterized by unilateral
paroxysms of shooting and stabbing pain in the area innervated by any of the three branches, but
most commonly the second and third branches of the trigeminal nerve. The pain ends as abruptly as
it starts and is described as a unilateral shooting and stabbing sensation. The unilateral nature of the
pain is an important feature. Associated involuntary contraction of the facial muscles can cause
sudden closing of the eye or twitching of the mouth, hence the former name tic douloureux (painful
twitch). Trigeminal neuralgia occurs most often before 35 years of age. Pain-free intervals may last
minutes, hours, days, or longer. With advancing years, the painful episodes tend to become more
frequent and agonizing. The patient lives in constant fear of attacks.
Pathophysiology
Although the cause is not certain, vascular compression and pressure are suggested causes. The
disorder occurs more commonly in women and in people with multiple sclerosis (MS) compared with
the general population.
Clinical Manifestations
Paroxysms are aroused by any stimulation of terminals of the affected nerve branches (eg,
washing the face, shaving, brushing teeth, eating, and drinking). Patients may avoid these
activities (behavior provides a cue to diagnosis).
Drafts of cold air and direct pressure against the nerve trunk may cause pain.
Trigger points are areas where the slightest touch immediately starts a paroxysm.
Assessment and Diagnostic Methods
Diagnosis is based on characteristic behavior: avoiding stimulating trigger point areas (eg, trying not
to touch or wash the face, shave, chew, or do anything else that might cause an attack).
Medical Management
Pharmacologic Therapy
Antiseizure agents, such as carbamazepine (Tegretol), reduce transmission of impulses at certain
nerve terminals and relieve pain in most patients. Carbamazepine is given with meals. The patient is
observed for side effects, including nausea, dizziness, drowsiness, and aplastic anemia. The patient
is monitored for bone marrow depression during long-term therapy. Gabapentin and baclofen are also
used to treat pain. If pain control is still not achieved, phenytoin (Dilantin) may be used as adjunctive
therapy.
Surgical Management
In microvascular decompression of the trigeminal nerve, an intracranial approach (craniotomy) to
decompress the trigeminal nerve is used. Percutaneous radiofrequency produces a thermal lesion on
the trigeminal nerve. Although immediate pain relief is experienced, dysesthesia of the face and loss
of the corneal reflex may occur. Use of stereotactic magnetic resonance imaging (MRI) for
identification of the trigeminal nerve followed by gamma knife radiosurgery is being used at some
medical centers. Percutaneous balloon microcompression disrupts large myelinated fibers in all three
branches of the trigeminal nerve.
Nursing Interventions
Assist patient to recognize the factors that trigger excruciating facial pain (eg, hot or cold food
or water, jarring motions). Teach patient how to lessen these discomforts by using cotton pads
and room temperature water to wash face.
Instruct patient to rinse mouth after eating when tooth brushing causes pain and to perform
personal hygiene during pain-free intervals.
Advise patient to take food and fluids at room temperature, to chew on unaffected side, and to
ingest soft foods.
Recognize that anxiety, depression, and insomnia often accompany chronic painful conditions,
and use appropriate interventions and referrals.
Provide postoperative care by performing neurologic checks to assess facial motor and
sensory deficits. Instruct patient not to rub the eye if the surgery results in sensory deficits to
the affected side of the face, because pain will not be felt in the event there is injury. Assess
the eye for irritation or redness. Insert artificial tears, if prescribed, to prevent dryness to
affected eye. Caution patient not to chew on the affected side until numbness diminishes.
Observe patient carefully for any difficulty in eating and swallowing foods of different
consistencies.
Trigeminal neuralgia (TN, or TGN), also known as prosopalgia, tic doloureux, or Fothergill's disease
Historically TN has been called "suicide disease"
Trigeminal neuralgia was first described by physician John Fothergill and treated surgically by John
Murray Carnochan, both of whom were graduates of the University of Edinburgh Medical School.
is a neuropathic disorder characterized by episodes of intense pain in the face. It has been
described as among the most painful conditions known. The pain originates from a variety of
different locations on the face and may be felt in front of the ear, eye, lips, nose, scalp,
forehead, cheeks, mouth, or jaw and side of the face.
The pain of TN is from the trigeminal nerve. The trigeminal nerve is a paired cranial nerve that has
three major branches: the ophthalmic nerve (V1), the maxillary nerve (V2), and the mandibular nerve
(V3). One, two, or all three branches of the nerve may be affected. 16% of cases occur on both
sides of the face but extremely rare for both to be affected at the same time. Trigeminal neuralgia
most commonly involves the middle branch (the maxillary nerve or V2) and lower branch (mandibular
nerve or V3) of the trigeminal nerve.
The trigeminal nerve is one of 12 pairs of nerves that are attached to the brain. The nerve has three
branches that conduct sensations from the upper, middle, and lower portions of the face, as well as
the oral cavity, to the brain.
The ophthalmic, or upper, branch supplies sensation to most of the scalp, forehead, and front of the
head.
The maxillary, or middle, branch stimulates the cheek, upper jaw, top lip, teeth and gums, and to the
side of the nose.
The mandibular, or lower, branch supplies nerves to the lower jaw, teeth and gums, and bottom
lip. More than one nerve branch can be affected by the disorder. Rarely, both sides of the face may
be affected at different times in an individual, or even more rarely at the same time (called bilateral
TN).
Surgery
Several neurosurgical procedures are available to treat TN, depending on the nature of the pain; the
individuals preference, physical health, blood pressure, and previous surgeries; presence of multiple
sclerosis, and the distribution of trigeminal nerve involvement (particularly when the upper/ophthalmic
branch is involved). Some procedures are done on an outpatient basis, while others may involve a
more complex operation that is performed under general anesthesia. Some degree of facial
numbness is expected after many of these procedures, and TN will often return even if the procedure
is initially successful. Depending on the procedure, other surgical risks include hearing loss, balance
problems, leaking of the cerebrospinal fluid (the fluid that bathes the brain and spinal cord), infection,
anesthesia dolorosa (a combination of surface numbness and deep burning pain), and stroke,
although the latter is rare.
A rhizotomy (rhizolysis) is a procedure in which nerve fibers are damaged to block pain. A rhizotomy
for TN always causes some degree of sensory loss and facial numbness. Several forms of rhizotomy
are available to treat trigeminal neuralgia:
Balloon compression works by injuring the insulation on nerves that are involved with the
sensation of light touch on the face. The procedure is performed in an operating room under
general anesthesia. A tube called a cannula is inserted through the cheek and guided to where
one branch of the trigeminal nerve passes through the base of the skull. A soft catheter with a
balloon tip is threaded through the cannula and the balloon is inflated to squeeze part of the
nerve against the hard edge of the brain covering (the dura) and the skull. After about a minute
the balloon is deflated and removed, along with the catheter and cannula. Balloon
compression is generally an outpatient procedure, although sometimes the patient may be
kept in the hospital overnight. Pain relief usually lasts one to two years.
Glycerol injection is also generally an outpatient procedure in which the individual is sedated
with intravenous medication. A thin needle is passed through the cheek, next to the mouth, and
guided through the opening in the base of the skull where the third division of the trigeminal
nerve (mandibular) exits. The needle is moved into the pocket of spinal fluid (cistern) that
surrounds the trigeminal nerve center (or ganglion, the central part of the nerve from which the
nerve impulses are transmitted to the brain). The procedure is performed with the person
sitting up, since glycerol is heavier than spinal fluid and will then remain in the spinal fluid
around the ganglion. The glycerol injection bathes the ganglion and damages the insulation of
trigeminal nerve fibers. This form of rhizotomy is likely to result in recurrence of pain within a
year to two years. However, the procedure can be repeated multiple times.
Radiofrequency thermal lesioning (also known as "RF Ablation" or RF Lesion) is most often
performed on an outpatient basis. The individual is anesthetized and a hollow needle is passed
through the cheek through the same opening at the base of the skull where the balloon
compression and glycerol injections are performed. The individual is briefly awakened and a
small electrical current is passed through the needle, causing tingling in the area of the nerve
where the needle tips rests. When the needle is positioned so that the tingling occurs in the
area of TN pain, the person is then sedated and the nerve area is gradually heated with an
electrode, injuring the nerve fibers. The electrode and needle are then removed and the
person is awakened. The procedure can be repeated until the desired amount of sensory loss
is obtained; usually a blunting of sharp sensation, with preservation of touch. Approximately
half of the people have symptoms that reoccur three to four years following RF
lesioning. Production of more numbness can extend the pain relief even longer, but the risks of
anesthesia dolorosa also increase.
Stereotactic radiosurgery (Gamma Knife, Cyber Knife) uses computer imaging to direct highly
focused beams of radiation at the site where the trigeminal nerve exits the brain stem. This
causes the slow formation of a lesion on the nerve that disrupts the transmission of sensory
signals to the brain. People usually leave the hospital the same day or the next day following
treatment but wont typically experience relief from pain for several weeks (or sometimes
several months) following the procedure. The International RadioSurgery Association reports
that between 50 and 78 percent of people with TN who are treated with Gamma Knife
radiosurgery experience "excellent" pain relief within a few weeks following the procedure. For
individuals who were treated successfully, almost half have recurrence of pain within three
years.
Microvascular decompression (MVD) is the most invasive of all surgeries for TN, but also
offers the lowest probability that pain will return. About half of individuals undergoing MVD for
TN will experience recurrent pain within 12 to 15 years. This inpatient procedure, which is
performed under general anesthesia, requires that a small opening be made through the
mastoid bone behind the ear. While viewing the trigeminal nerve through a microscope or
endoscope, the surgeon moves away the vessel (usually an artery) that is compressing the
nerve and places a soft cushion between the nerve and the vessel. Unlike rhizotomies, the
goal is not to produce numbness in the face after this surgery. Individuals generally recuperate
for several days in the hospital following the procedure, and will generally need to recover for
several weeks after the procedure.
A neurectomy (also called partial nerve section), which involves cutting part of the nerve, may be
performed near the entrance point of the nerve at the brain stem during an attempted microvascular
decompression if no vessel is found to be pressing on the trigeminal nerve. Neurectomies also may
be performed by cutting superficial branches of the trigeminal nerve in the face. When done during
microvascular decompression, a neurectomy will cause more long-lasting numbness in the area of
the face that is supplied by the nerve or nerve branch that is cut. However, when the operation is
performed in the face, the nerve may grow back and in time sensation may return. With neurectomy,
there is risk of creating anesthesia dolorosa.
carbamazepine
Drug class
Antiepileptic
Therapeutic actions
Mechanism of action not understood; antiepileptic activity may be related to its ability to inhibit
polysynaptic responses and block post-tetanic potentiation. Drug is chemically related to the TCAs.
Indications
Trigeminal neuralgia (tic douloureux): Treatment of pain associated with true trigeminal neuralgia;
also beneficial in glossopharyngeal neuralgia
gabapentin
Drug class
Antiepileptic
Therapeutic actions
Mechanism of action not understood; antiepileptic activity may be related to its ability to inhibit
polysynaptic responses and block posttetanic potentiation.
Indications
Management of postherpetic neuralgia or pain in the area affected by herpes zoster after the
disease has been treated
Unlabeled uses: Hot flashes, neuropathic pain
baclofen
Drug class
Centrally acting skeletal muscle relaxant
Therapeutic actions
Precise mechanism not known; GABA analogue but does not appear to produce clinical effects by
actions on GABA-minergic systems; inhibits both monosynaptic and polysynaptic spinal reflexes;
CNS depressant.
Indications
Unlabeled uses: Trigeminal neuralgia (tic douloureux); may be beneficial in reducing spasticity in
cerebral palsy in children (intrathecal use); intractable hiccups unresponsive to other therapies,
alcohol and opiate withdrawal (oral), GERD, migraine prevention
phenytoin
Drug classes
Antiarrhythmic, group 1b
Antiepileptic
Hydantoin
Therapeutic actions
Has antiepileptic activity without causing general CNS depression; stabilizes neuronal membranes
and prevents hyperexcitability caused by excessive stimulation; limits the spread of seizure activity
from an active focus; also effective in treating cardiac arrhythmias, especially those induced by
cardiac glycosides; antiarrhythmic properties are very similar to those of lidocaine; both are class IB
antiarrhythmics.
Indications
Unlabeled uses: Antiarrhythmic, particularly in arrhythmias induced by cardiac glycosides (IV
preparations); treatment of trigeminal neuralgia (tic douloureux); rectal administration