Escolar Documentos
Profissional Documentos
Cultura Documentos
CHAPTER 11
Ronald I. Apfelbaum
223
224 / CHAPTER 11
frequent occurrence of herpes virus within the gasserian ence brief repetitive paroxysmal spasms of unilateral
ganglion, the virus was advocated as the etiologic agent pain of extreme intensity, confined entirely to the terri-
of trigeminal neuralgia—until control studies demon- tory of one or more divisions of the trigeminal or the
strated an equally high incidence in asymptomatic indi- glossopharyngeal nerve. The pain is frequently described
viduals. as a "bolt of lightening" or an electric shock-like phe-
The one consistent observation, which holds true both nomenon, and patients will often, in describing the pain,
for patients with demyelinating disease and for those make a characteristic gesture, flinging open a closed
without, is that there are segmental pathological changes hand to demonstrate the rapid onset and spread of the
at the root entry zone of the nerve affecting the centrally pain.
myelinated portion of the nerve (4-7). Central myelin The pain may occur spontaneously but is often trig-
(derived from oligodendroglial cells) extends a few milli- gered by nonpainful tactile stimuli, usually, but not in-
meters from the brainstem into the nerve before being variably, within the territory of the affected nerve. Thus,
replaced by myelin formed by Schwann cells (8). In elec- in the case of trigeminal neuralgia, the pain may be acti-
tron-microscopic studies there has been consistent de- vated by a breeze on the face, touching the face lightly,
myelination in this area, as well as axonal disruption. In chewing, talking, or eating. Common activities, such as
patients suffering from multiple sclerosis who also have face washing, hair combing, and tooth brushing, become
trigeminal neuralgia, demyelinating plaques are invari- difficult or impossible. The attack of pain is always uni-
ably located in this region (and may also be found in lateral, with an approximately 60 to 40 right to left pre-
other portions of the trigeminal pathway within the dominance. Some patients may be unfortunate enough
brainstem). to suffer from this condition bilaterally (1 to 3 percent),
Walter Dandy first observed the high incidence of vas- but the pain is neither simultaneous nor synchronous on
cular channels impinging upon the root entry zone of the the two sides.
nerve and suggested that this might be the cause of tri- Most patients will not have any underlying back-
geminal neuralgia (9). It was not until Dr. Peter Jannetta ground pain. But an occasional patient may complain of
applied the operating microscope to the systematic study a burning or drawing feeling after the sharp paroxysm
of these problems that the truly high incidence of such subsides, and some will note a prickling, burning or
root entry zone compression was accurately appreciated throbbing sensation before the tic starts (18). In obtain-
(over 97 percent) (10,11). Several postmortem studies ing a history, one must also be careful that repetitive
have confirmed the abnormally high incidence of vascu- paroxysms occurring in long volleys are not mistaken for
lar lesions in patients suffering from trigeminal neuralgia one steady, long pain.
as compared to control populations (12-14). This com- When the diagnosis is doubtful (usually because the
pression appears to be the cause of the demyelination patient is a poor historian) one should inquire about the
and is accepted by most, but not all, specialists in this patient's behavior during the pain attacks. Patients with
field as the cause of the symptoms in most patients. The trigeminal neuralgia tend to remain as immobile as possi-
major exception is in patients with multiple sclerosis, ble and to guard against any contact with the exposed
who of course have intrinsic neural disease. area. This is in contradistinction to other types of pain
The anatomical derangement in the nerve apparently such as cluster headache (Horton's cephalgia), in which
alters the physiology in an as yet incompletely under- patients tend to pace or throw themselves about, often
stood manner. Gardner has hypothesized the develop- crying out. These latter patients may often place hot or
ment of a "short circuit" in the nerve at the site of de- cold compresses on the face or massage the affected area
myelination, resulting in touch sensation carried by the in an attempt to diminish the pain.
larger afferent fibers actuating thinner pain fibers (15). Both trigeminal and glossopharyngeal neuralgia are
Loeser, Calvin, and Howe have suggested that antidro- conditions that seem to occur with increasing frequency
mic reflectance of a normal nerve impulse sets up a re- with advancing age. Contrary to some descriptions, how-
verberating circuit, explaining how a light touch can trig- ever, they are not exclusively diseases of elderly patients
ger a painful paroxysm (16,17). and may occur as early as the teenage years in rare in-
Because of its rarity, similar pathological studies have stances. The peak age of onset is in midlife, during the
not been performed in patients with glossopharyngeal fifth to sixth decades.
neuralgia, but the conditions are otherwise analogous In the natural history of trigeminal neuralgia, remis-
and are thought to have the same physiological basis. sions are common and may last for extended periods of
time. With increased duration of the illness, however,
remissions tend to become shorter and periods of exacer-
Symptoms bation longer.
Another useful characteristic in the differential diag-
These conditions are characterized by a very consis- nosis is the infrequent occurrence of nocturnal pain.
tent clinical pattern among patients. Patients will experi- Many patients are able to find a comfortable position
TRIGEMINAL AND GLOSSOPHARYNGEAL NEURALGIA AND HEMIFACIAL SPASM / 225
and get adequate rest. This condition therefore is not a a careful and adequate history. A magnetic resonance
chronic pain but rather an acute pain syndrome occur- imaging (MRI) scan should be performed, with particu-
ring repetitively. Narcotic analgesics are impotent in this lar attention paid to the affected nerve in the middle and
situation, and thus it is extremely uncommon to find a posterior fossa, on every patient who has this problem so
patient who is habituated or even a frequent user of nar- as to exclude an underlying neoplasm. Multiple sclerosis
cotics. will usually be readily detected on the MRI scan, but if
There is a well-established association between trigemi- doubt exists other appropriate tests may include audi-
nal neuralgia and multiple sclerosis. In a large series of tory and visual evoked responses and spinal fluid analy-
patients with multiple sclerosis, 1 to 2 percent will be sis to help establish the diagnosis.
found to have trigeminal neuralgia (19). Conversely, a In addition to excluding neoplasms and ruling out
similar percentage of patients suffering from trigeminal multiple sclerosis, MRI scanning in trigeminal neuralgia
neuralgia will be found to have multiple sclerosis. As has demonstrated the flow voids of vascular loops adja-
previously mentioned, in these patients there is a demye- cent to the root entry zone of the affected nerve, with
linating plaque at the root entry zone of the trigeminal distortion of the nerve. Abnormal uptake of gadolinium
nerve. The site of pathology, therefore, is in exactly the in the compressed region also may be seen. These
same area as that found in trigeminal neuralgia pro- changes in the preganglionic segment of the trigeminal
duced by vascular compression, but in these patients the nerve are best viewed on coronal T,-weighted sequences
disease is intrinsic within the nerve rather than due to an (Fig. 1) (20). Scanning also alerts the surgeon to the pres-
extrinsic cause. The clinical symptoms are also identical. ence of dolichoectatic major vessels (Fig. 2), aneurysms,
The association of glossopharyngeal neuralgia and multi- and vascular malformations. All are infrequent causes of
ple sclerosis has been documented only rarely. vascular compression but ones that may require differ-
The symptoms of glossopharyngeal neuralgia are gen- ent treatment or surgical strategics.
erally similar to those of trigeminal neuralgia but have In glossopharyngeal neuralgia, in addition to the
some additional features. The pain resembles that of tri- above, a useful diagnostic test is the application of a topi-
geminal neuralgia and occurs as brief paroxysms in the cal solution of 10 percent cocaine to the oropharynx.
throat or ear. A higher number of patients, however, ex- This will provide several hours of relief, during which the
perience a dull, aching, or burning feeling. As in trigemi- majority of patients with this problem will be able to eat
nal neuralgia, the episodes of pain are usually triggered, and drink without experiencing pain. This time helps to
the common provocative maneuvers being swallowing, establish the diagnosis and also aids in predicting the
chewing, coughing, or talking. Patients may experience outcome of surgical treatment.
paroxysms of coughing with their attacks of pain. Syn-
cope has also been reported, but appears to be a very
uncommon associated symptom. It may be due to hyper-
sensitivity of the carotid sinus nerve, producing asystole.
Signs
FIG. 3. Placement of the needle through the lateral cheek * The percentages expressed here represent my personal experience
and into the gasserian ganglion for percutaneous radiofre- with 134 patients treated over 10 years by this technique. On analysis,
quency lesioning. (From reference 51, with permission.) these percentages agree with many large published series (26-30).
228 / CHAPTER 11
abscesses, and injury to the third, fourth, and sixth cra- cellent pain relief with usually only minimal sensory loss
nial nerves. Punctures of the carotid artery and cavern- at times none. Some patients, however, will get
ous sinus usually are benign, but on occasion carotid significant sensory loss (4 percent), even anesthesia,
cavernous fistula and/or cerebral vascular accidents though the latter usually occurs only with sequential de-
have been reported. These latter complications, how- structive procedures. In 158 patients treated with this
ever, are all exceedingly rare. technique over seven years, we have only rarely observed
Patients may resume full activity and diet immedi- corneal anesthesia (4 percent) and have never seen kera-
ately after recovery from the anesthetic and are usually titis develop as a sequela to the procedure. Dysesthetic
discharged the following day. There are no restrictions of sensations are also very infrequent (3 percent) and
their activities after this procedure. usually quite mild. Anesthesia dolorosa has not been re-
Percutaneous chemoneurolysis with glycerol was intro- ported from this procedure (33).
duced in 1981 by Hakanson (32). In this approach, pure We perform the procedure in the x-ray suite, using the
anhydrous glycerol (99.5 percent) is instilled into the tri- same technique for puncturing the foramen ovale under
geminal cistern under fluoroscopic control. It is not clear direct fluoroscopic guidance that we use for radiofre-
whether the effect of the glycerol is due to chemical or quency lesioning (25). The entry site, approximately 2 to
hyperosmotic damage to the trigeminal nerve pregan- 3 cm from the corner of the mouth, is selected fluoro-
glionic rootlets. In any case, the effect is to produce ex- scopically (Fig. 4A) by placing the patient with his neck
B
FIG. 5. (A) View through the operating microscope of the left trigeminal nerve, compressed by an
elongated superior cerebellar artery (SCA) as it traverses the brainstem. The artery is caught in the axilla
between the nerve and brainstem. (B) View after elevation of the arterial loop and placement of the
plastic prosthesis to prevent the reapposition of artery and nerve. (From reference 36, with permission.)
(Fig. 5B). The goal is to redirect the arterial pulsation duces a lower incidence of dysesthesia and of numbness
away from the root entry zone. Our operative findings than more peripheral destruction of the nerve.
fully support those of Jannetta, and our results and fol- There is a somewhat higher incidence of bilateral tri-
low-up closely agree with those he has reported (38,39). geminal neuralgia in patients afflicted with multiple scle-
After satisfactory decompression, the dura is closed in rosis than occurs otherwise. In a small percentage of pa-
a watertight fashion and the wound is closed in layers. tients, trigeminal neuralgia may be the initial presenting
We routinely place our patients on steroids preopera- symptom of multiple sclerosis. This may explain the oc-
tively and for 24 hours postoperatively. Most patients casional negative posterior fossa exploration, especially
tolerate this procedure well and are able to begin oral in the younger patients.
intake and get out of bed on the first postoperative day. If
the patients are operated on in the sitting position, they Glossopharyngeal Neuralgia
usually have a moderate postoperative headache, which
can be controlled with oral analgesics. The majority of The medical principles of treatment as outlined for
patients can be discharged five to seven days postopera- trigeminal neuralgia apply equally to this condition. If
tively and usually take another week or two of additional these are unsuccessful, the Jannetta micro vascular de-
convalescence at home. During this period, they are en- compression appears to be the definitive procedure of
couraged to increase their activities gradually. choice, offering, as with trigeminal neuralgia, the oppor-
As with any operation, this procedure is not without tunity for the relief of pain without sacrificing neural
risk and fatal complications have ensued (1 percent). function (38,39).
Cerebellar hematomas or hemorrhagic infarction (1.6 Radiofrequency lesioning at the jugular foramen has
percent) and supratentorial strokes (1 percent) have oc- been attempted, but this only produces a lesion distal to
curred and at times have been responsible for fatalities, the ganglion. Thus, recurrences in a few months can be
despite vigorous appropriate treatment. Other signifi- expected, as with peripheral nerve sectioning.
cant complications have included transient fourth-nerve An alternative procedure involves sectioning of the
palsies (4 percent), transient facial nerve palsies (1.6 per- glossopharyngeal nerve and the upper several fascicles of
cent), and unilateral hearing loss (2 percent; 1 percent the vagus nerve in the posterior fossa (2). This procedure,
severe). employed since its introduction by Love in 1948, is pref-
In trigeminal neuralgia associated with multiple sclero- erable in more elderly patients, rather than exposing
sis, if medical treatment is unsuccessful, relief can be them to increased operating time and the risks of manip-
effected only by a destructive procedure. Percutaneous ulating their intracranial vessels.
lesioning is therefore the procedure of choice. If this is Potential complications and postoperative care are as
unsuccessful for technical reasons, section of the nerve in described above for trigeminal neuralgia. After section-
the posterior fossa will produce a similar benefit and, if ing of the nerve, dysphagia may occur, as well as unpleas-
performed immediately adjacent to the brainstem, pro- ant pharyngeal sensations.
TRIGEMINAL AND GLOSSOPHARYNGEAL NEURALGIA AND HEMIFACIAL SPASM / 231
Outcome decompression approximates that seen in trigeminal neu-
ralgia and is less with nerve sectioning if the upper vagal
Success in relieving the pain of trigeminal neuralgia rootlets are included.
has been achieved in about 95 percent of the patients
treated with either of these two types of procedures. Re-
currences following percutaneous radiofrequency le- HEMIFACIAL SPASM
sioning have varied significantly in different series, but
average approximately 35 percent within four years. Incidence
There is an increasing incidence of recurrences with the
passage of time after surgery. Recurrences may occur in Hemifacial spasm is a relatively rare condition. There
the treated area but are more common in adjacent un- are no published epidemiologic studies, so its true inci-
treated regions. If necessary, the procedure can be re- dence is not known. From personal experience I would
peated and many surgeons have preferred to produce estimate this condition to be approximately 25 percent
minimal lesions, minimizing numbness and decreasing as common as trigeminal neuralgia. This may not be a
the risk of corneal anesthesia and dysesthetic sequelae, true figure, however, because many individuals may en-
while accepting a higher recurrence rate and repeating dure rather than seek treatment for this condition, which
the procedure as often as necessary (27). is neither life threatening nor painful. Also great regional
With the Jannetta microvascular decompression, re- variation in incidence occurs, with an especially high fre-
currences, when they occur, tend to occur early, usually quency of occurrence noted in Oriental (especially Japa-
within the first year to 18 months, with only a rare recur- nese) patients.
rence being reported after that. Severe refractory recur-
rences have occurred in 13 percent of our patients. An
additional 19 percent of our patients have had some Etiology and Pathology
pain, which was well controlled with medication, usually
at very low dosages. Many of these patients (about one- Hemifacial spasm appears to be the direct motor ana-
half) subsequently have been able to discontinue the logue of trigeminal neuralgia. That is, both are disorders
medication. All were refractory to medical treatment pre- of paroxysmal hyperactivity occurring in a cranial nerve.
operatively, and most have reported that they feel the Hemifacial spasm involves the facial nerve and as such
procedure was of significant benefit to them, even if they manifests pure motor hyperactivity, in comparison with
have required additional medication. Thus the overall trigeminal neuralgia, which occurs in a primarily sen-
success rate for excellent (painfree) and good (controlled sory nerve and causes paroxysms of pain.
pain) results is 87 percent. Significant historical observations have noted that, if
Both of these procedures, therefore, offer great poten- the nerve is sectioned at the stylomastoid foramen and
tial for the relief of this severe, incapacitating pain. Pa- anastomosed to itself, the spasms return with reinnerva-
tient satisfaction with either has been gratifying, though tion. If, however, an anastomosis to another cranial
patients undergoing microvascular decompression are nerve is performed, the spasms do not return. Thus the
significantly happier, since they do not have any sensory site of pathology must be proximal to the stylomastoid
loss, with its annoyance serving as a constant reminder foramen. It has also been observed that a supratentorial
of their problem. The lack of dysesthesia and fewer recur- stroke producing a hemiplegia does not relieve the spasm
rences also contribute to this increased satisfaction. even in an otherwise plegic face (40). Therefore, the
The choice of procedure must ultimately be made by pathological process must be confined to the lower mo-
the patient, after he or she has achieved a thorough un- tor neuron.
derstanding of the potential risks and benefits of each. Isolated case reports dating back to an autopsy study
The physician's recommendation also must be based on by Schultze in 1875 (41) have demonstrated vascular
a careful analysis of the patient's general health. For the lesions compressing the facial nerve in the posterior
patient under age 65, in good general health, we usually fossa. Campbell and Keedy published two cases in 1947
recommend the Jannetta microvascular decompression. in which a "cirsoid aneurysm" (nowadays known as ver-
Glycerol chemoneurolysis is our preferred technique for tebral basilar dolichoectasia) was found compressing the
those patients who do not meet the above criteria, who facial nerve (42). Other isolated case reports support
have multiple sclerosis, who do not wish to accept the these observations. Tumors compressing the facial nerve
risks of microvascular decompression, or who have a re- in the posterior fossa and causing hemifacial spasm were
current tic after a prior surgical procedure. noted by Dandy (43,44) and Gushing (45). In 1961
The results in glossopharyngeal neuralgia are similar, Gardner and Sava presented their experience with 19
in a smaller number of reported cases treated with the patients (46). In 14 of these they found mass lesions. In
Jannetta procedure, and also with sectioning of the the majority of cases, the problem appeared to stem from
nerve. The frequency of recurrence after microvascular either normal or abnormal blood vessels. Gardner and
CHAPTER 11
Sava also suggested that hemifacial spasm was a revers- with trigeminal neuralgia. Gushing apparently was the
ible pathophysiological state. first to describe this under the term "tic convulsif" (49).
It was not until the systematic application of the oper- Most commonly, mass lesions in the posterior fossa, re-
ating microscope by Peter Jannetta, commencing in sulting in compression of both the fifth and seventh
1967, that a large series of observations was made in nerves, have been associated with this combination. Sep-
patients with hemifacial spasm. Jannetta not only firmly arate independent lesions, however, may also occur.
established that root exit zone compression of the facial
nerve is present in virtually every patient afflicted with
Signs
this problem but also devised a nondestructive tech-
nique for moving the blood vessel (which is the common
The clinical appearance of patients suffering from this
cause) away from the nerve and securing it with a small
problem is quite characteristic. Intermittent, uncontrol-
sponge prosthesis to decompress the nerve effectively.
lable, brief, repetitive, and painless spasms of the facial
Jannetta's observations have been substantiated by sev-
musculature, most prominent in the midface, are noted.
eral other surgeons (36,47), firmly establishing the etiol-
Occasionally the "tonus" phenomenon occurs, in which
ogy of hemifacial spasm.
sustained forceful contractures, usually of the midface
The exact physiological effect that this root exit zone
musculature, last for several seconds or even longer.
compression produces is not fully understood. Hunt in
Physical examination will usually reveal abnormalities
1909 detailed the role of sensory afferent fibers in the
of the cranial nerves. Mild facial paresis (between
facial nerve. Gardner suggested a reverberating circuit
spasms) may be noted, as well as mild decreased hearing
produced by afferent-efferent transaxonal "short-circuit-
in the ipsilateral ear, in long-standing cases. Deficits in
ing" (46).
cranial nerves V, VII, or IX or cerebellar or brainstem
findings suggest a mass lesion in the cerebellopontine
Symptoms angle.
Treatment