The main

neurostomatological
syndromes
(neuralgia of
Sphenopalatinum, Otic,
Ciliary ganglions and
angiotrophoneurosis)

Facial pain can be

developed due to the next
processes:
Trigeminal neuralgia and (rarely)
Glossopharyngeal neuralgia
Facial forms of migraine, including
cluster vascular pain
Charlen and Slader sympathalgia
Psychogenic head pains

Cranial neuralgia
and central
reasons of pain

Trigeminal neuralgia

Characterized by short,
intense lancinating pain,
often recurring at frequent
intervals and confined to
one or more zones of
Trigeminal nerve.

Trigeminal neuralgia
Polyetiological disease, arising up mainly
(more than in 90 % cases) at the age more
then 40 years. 60-70 % of patients are
women.
The most frequent reasons:
Mechanical compression of nerve
Formation of sclerotic plate
Infections in an oral cavity
Vascular malformations or tumors
Basic lesions of the brainstem

Clinical
features

The second branch of Trigeminal nerve is

involved in pathological process in 44 % cases,
third in 36 %, first in 20 % cases.
The repeated attacks usually arise up in the same
zones of face
Emotional and physical trauma can increase
frequency of attacks
Through some time dull pain or feeling of burning
appears in patients in an inter-attack period.
Paresthesia with hypoesthesia can appear only after
injection-destructive treatment - signs of neuropathy
Autonomic supplement of pain attack is observed in
less than 1/3 of all patients
Resistant autonomic disorders (changes colour of skin,
desquamation, hypotrophy of soft tissue) can be only
in patients, which had injection-destructive treatment

CLINICAL MANIFESTATIONS
- SYMPTOMS

Usually unilateral. Only 4% of patients complain

on bilateral paroxysms
The right side of the face is more often affected
The third segment is more often affected than
the second which, in turn, is more often affected
than the first
Brief paroxysms of intense, lancinating pain,
seldom lasting longer than one minute are often
triggered by cold, eating, speaking or touching
the face (trigger zones)
Typically, remission of pain occurs, lasting
months or even years. Eventually, the pain
becomes chronic

Course
Disease has typical remittent course
Many patients describe, that attacks do
not disturb months and years
Disease course includes exacerbation
and remission
In the period of exacerbation
patients answer on questions
monosyllabically, barely opening
slightly a mouth (the least motion of
facial muscles can cause pain attack)
During attacks patients stop beating

The diagnosis of
Trigeminal neuralgia
is based on characteristic signs:
Paroxysm of pain in area of face or
forehead, lasting from a few seconds to 2
minutes
localization of pain in area of one or a few
branches of Trigeminal nerve
Brief paroxysms of intense, lancinating
pain,
seldom lasting longer than a minute and
absence of it in an inter-attack period
suggestion of pain from triggers zones
(cold, eating, talk, washing, cleaning of
teeth, speaking or touching the face ).
stereotyped attacks for the same patient

Distribution of trigger
zones.

Thus on the basis of information

about somatic and neurological
examination, other reasons of
head and facial pain, local
processes in nasal sinuses,
teeth, jaws, pharynx or basis of
skull should be eliminated

Glossopharyngeal
Neuralgia
Definition Glossopharyngeal neuralgia is the

occurrence of spasms of pain in the sensory

distribution of the ninth and tenth Cranial nerves
Etiology and Pathology
The cause is unknown (can be due to the
pressured on or entrapment of the IX-th and X-th
CN)
Glossopharyngeal neuralgia usually occurs after
acute infection of the pharynx
It can be associated with compression in different
sites - the cerebellopontine angle, jugular
foramen, base of the skull, pharynx, and tonsils

Glossopharyngeal
neuralgia
Clinical features

Rare condition
Typical short spasm paroxysm of pain in the
pharynx or the base of the tongue, tonsils
Attacks are usually brief but may last for
several minutes in severe cases
Attacks are occasionally associated with
bradycardia, cardiac arrhythmias, falling BP or
hypertension, and syncope due to associated
Vagal stimulation (caused tumour of pharynx)
Typically triggered by swallowing (especially
cold solution), coughing, chewing, talking,
yawning, sneezing, turning the head to one
side

Glossopharyngeal
neuralgia
Clinical features

Triggers zones - touch to the soft palate or

tonsils, sometimes by touching the tragus of
the ear
The neurological status is normal - disorders
of sensitiveness or paresis are absent
Often there are spontaneous remission from
a few months to a few years
Hypersecretion of the Parotid gland has been
reported
Combination with the Trigeminal neuralgia is
possible (compression of both nerve by
complete arched vessel)
Sometimes pain irradiated to ear and neck,
or only into the ear (Oticalgic form)

Diagnostic Procedures

A diligent search should be made

for a compressive lesion in the
area of the cerebellopontine angle
or at the base of the skull using
magnetic resonance imaging (MRI)
or CT scanning, which provides
clear views of the jugular foramen

Differential diagnosis
Sometimes a similar pain syndrome is marked at the:
Tumour of pharynx (including nasopharynx),
tongue, posterior cranial fosse
Tonsillitis
Paratonsilar abscess
Hypertrophy of processus stiloideus. In last case
pain increases at the turn of head
Neurinoma of Glossopharyngeal nerve is observed
exceptionally rarely
A symptomatic neuralgia should be differentiated
in young people with permanent pains and
symptoms of neurological deficit
Treatment are same, as well as at Trigeminal
neuralgia

Treatment

Carbamazepine (Tegretol) as described

under Trigeminal neuralgia.
Intracranial sectioning of the
Glossopharyngeal nerve has been
performed in intractable cases. This
procedure entails a section of the upper
two rootlets of the Vagus nerve and
may be associated with postoperative
hypotension and cardiac arrhythmias.

Superior Laryngeal
Neuralgia

Typical paroxysmal one-sided or bilateral episodic

lancinating pains in larynx (usually at the level of
upper part of Hyothyroid membrane) and angle of
mandible
Irradiation in eye, ears, thorax and forearm
Pain is associated with hiccup, hypersalivation,
cough
Pain is provoked by swallowing, yawning, cough,
blowing, motion of head.
Some Superior Laryngeal neuralgias are caused
malformations of neck
Karbamazepin (Finlepsin) and local anesthesia in
hyothyroid membrane are effective

Geniculate Neuralgia
Definition Geniculate neuralgia - is
episodes of severe lancinating pain
occurring in the region of the pinna and
external auditory canal
Etiology and Pathology
The etiology of this condition is unknown
Neuralgia affecting the nervus Intermedius
(the bipolar neurons of the n. Intermedius
are located in the Geniculate ganglion and
the afferent axons enter the spinal tract of
the Trigeminal nerve. The peripheral fibers
are distributed to the external auditory
canal and the pinna. There may also be
some distribution to deeper structures of
the face and hard palate.

Clinical Features

Typical spasmodic attacks of

severe pain in the region of the
pinna and external auditory canal.
The pain is occasionally felt in the
throat, deep in the face and in the
orbit.
Treatment Same as for Trigeminal
neuralgia. Surgical excision of the
Geniculate ganglion has been
performed in some cases.

Facial sympathalgias

Is the group of the similar clinical states

As a rule they have paroxysmal courses
and normal state between attacks
Attacks last from ten minutes to days
(rarely)
Typical sign is the acute, often
unendurable pain in face, sometimes
pulsating character

The important clinical sign are

autonomic signs on the side of
pain:

Lacrymation
Conjunctival injection
Rhinorrhoea from one half of nose and nasal
obstruction
Forehead and facial sweating
Syndrome meets more frequent among men
Attacks happen up acutely, mainly at nights,
the sharpest pain does make a patient to
move, because at rest pain becomes yet
sharper

Facial sympathalgias are

expression two on principle
different forms of pathology:

1)
2)
3)

1)
2)
3)

Sympathalgic syndromes (lesion of

autonomic peripheral ganglions and
nerves)
Nasal-ciliary neuralgia Charlen syndrome
Pterygopalatal neuralgia Sluder
syndrome
Neuralgia of Large Petrosal superficial
nerve Gartner syndrome
Vascular syndromes, as migraine:
Cluster headache
Cluster-effect (Horton histamine migraine,
Garris migrainous neuralgia)
Glyazer Carotid syndrome

Sympathalgic
syndromes, caused
the lesion of
autonomic peripheral
ganglions and nerves

Pathogenesis

Sympathalgic syndromes (Charlen and

Sluder) are caused by pathological process of
peripheral autonomic ganglions or their
irritation.
The etiology is not enough clear
Herpetic rashes in case of Charlen syndrome
tells about herpetic ganglionitis of nasalciliary ganglion
Etiology of Pterygopalatine sympathalgy is
nasal sinus infection (in particular, in Maxillar
cavity) and lesion of ganglion pterygopalatine

Charlen syndrome
(Ciliary neuralgia)

Typical attack of one-sided pain in internal corner

of eye and base of the nose, in an eyeball
Irradiation in a nose
Rhinorrhoea and nasal obstruction
Conjunctival injection, blepharospasm
Pain is typical in internal corner of eye
Usually an attack is associated with acute
lacrymation, changes in the front parts of eyeball,
cornea (phenomena of keratitis or iritis)
Herpetic exanthema at skin of the nose

The Charlen syndrome should be

differentiated with Herpetic
ganglionitis of Trigeminal ganglion
It manifests as symptoms in zone of
1 branche of Trigeminal nerve
innervation
Bright autonomic supplement is not
typical

Sluder syndrome
(neuralgia of ganglion
pterygopalatal)
Pain is localized in base of the nose, maxilla,

teeth, tongue, soft palate, ear, neck-shoulder

area
Then with irradiation in an eye and temple
Sometimes there is reduction of muscles of soft
palate (typical click)
The attack of pains begins and finishes
gradually
Course of attack protracted
Certain periodicity absents
Pain of middle cruelty with out motor anxiety
After an attack - paresthesias in face and noise
in ear

Differential diagnosis
Typical signs of neuralgia:
one-sided autonomic features.
one-sided oedema of face in the attack period.
Differential diseases:
Kvinke Angiotrophoneurotic oedema:
Typical localization in lips, cheeks,
bilateral oedema does not cause diagnostic
difficulties
Local oedema of orbit:
oedema yet and pain syndrome
Angiotrophic oedema in channel of Facial nerve
conduce to insufficiency of Facial nerve.

Recurrence of Facial
neuropathy in combination
with a plicate tongue, heilitis is
determined as disease of
Rossolimo-Melkerson-Rozental

Treatment

Application of autonomic tropic drugs

(ganglioblockercs Pyrroxanum)
uninnervation effect on the ganglion
Antiepileptic drugs - Carbamazepinum
(Tegretol, Finlepsin)
Psychotropic medications - tranquilizers
and antidepressants
Effectively in acute period greasing a
cocaine or lidocaine of middle nasal
cavity
Novocaine or Lidocaine blockade of
autonomic ganglions

Vascular
syndromes

Vascular syndromes are more

frequent (in patients with socalled Facial sympathalgias)
They manifest as attacks
described before
Are more typical for men

Cluster-syndrome
Horton Histamine migraine,
Harris migrainous euralgia
Term Cluster headache (CH)
is most correct, as reflects the
characteristic features of
disease and shuts out mixing
of him with a migraine

Etiology
The etiology is unknown.
In basis of diseases are
paroxysmal dilatation of branches
of Carotids, deficit of sympathetic
innervation of vesels with the
increase (rarer decrease) of local
cerebral blood circulation.

Epidemiology

The condition is considerably less common

than migraine, perhaps 50 times less, although
not so in the author's own out-patient series
Approximately 80% of patients are male
A family history of the condition occurs in
about 2% of cases
Mean age of onset is in the late twenties(2040)
Rarely, comparable cases have evolved in
association with a structural brain lesion, often
located around the cavernous sinus

Symptoms of Cluster
headache

Unilateral pain (although it may transfer sides in

subsequent attacks)
It is orbital or peri-orbital but occasionally radiates
ipsilaterally to the forehead, jaw or neck.
Continuous, characteristically intense
Attacks last from 15 to 180 min and usually occur
once or twice a day
Alcohol can trigger the pain
Nausea may occur but vomiting is rare
The paroxysms typically occur at the same time of
the day or night.
The pain is not usually pulsation but more continuous

Autonomic manifestations
They include the following:
Lacrimation
Conjunctival injection
Rhinorrhoea or nasal obstruction
Altered forehead sweating
Homer's syndrome

Horner's syndrome during an attack of cluster

headache.

Clinical course
Typically, bouts of pain last for
a few weeks, then remit but
return at intervals thereafter.
Chronic cluster headache is
defined as a headache phase
persisting beyond 12 months.

Criteria for diagnosing

CLUSTER HEADACHE
At least five attacks fulfilling the following criteria:
1. Severe unilateral orbital, supra-orbital
and/ortemporal pain lasting 15-180 min untreated
2. Headache is associated with at Least one of the
following signs ipsilateral to the headache:
Conjunctival injection
Lacrimation
Nasal congestion and Rhinorrhoea
Forehead and facial sweating
Miosis
Ptosis
Eyelid edema
3. Frequency from every other day to eight each day

CLUSTER HEADACHE

Cluster headache is strictly unilateral and

concentrated round the eye
Typically it is accompanied by a number of
autonomic signs
Ergotamine, oxygen and sumatriptan are
effective for attacks
Prophylactic agents used include ergotamine,
lithium and corticosteroids

CHRONIC PAROXYSMAL
HEMICRANIA

A rare condition
Predominating in women
Typical very frequent, short-lived attacks of
pain occur of a type similar to cluster
headache
Autonomic symptoms are common
Indometacin, in a dose of approximately
150 mg/day, aborts the attacks within 48 h.
In most patients, the attacks become
chronic.

IDIOPATHIC STABBING
HEADACHE

Defined as recurrent, very brief,

stabbing pains, usually confined to
the head, occurring singly or in
runs.
They tend to occur in subjects with
migraine.
They respond to regular
indomethacin therapy

Differential
diagnosis

CRANIAL ARTERITIS

Also known as temporal or giant cell arteritis.

This is a granulomatous inflammation of the
medium and large-sized arteries of the
cranium and elsewhere.
Within the cranium, the arteritis is confined
to those arteries possessing an internal
clastic lamina
The loss of vision is the consequence of
involvement of the posterior ciliary artery
leading to anterior ischaemic optic
neuropathy.

Epidemiology

The condition is rare under the age

of 50 years
The mean age at diagnosis is
approximately 70 years
The condition is slightly more
common in women and almost
confined to Caucasians

Cranial arteritis. Superficial temporal artery biopsy showing

(a)intimal thickening and medial damage and
(b) giant cells with inflammatory cell infiltration
in the internal elastic lamina.

Symptoms of Cranial
arteritis

Headache, usually localized

Pain on chewing (jaw claudication)
General malaise
Proximal muscle stiffness and pain
Visual loss

Clinical manifestations
symptoms

Headache occurs in the vast majority of patients

It particularly localizes to one or other temple
but may be more generalized
Constant, boring, intense pain, exacerbated by
contact - brushing the hair and also by exposure
to cold, pain on chewing occurs (jaw
claudication)
Many patients feel generally unwell, with
malaise, anorexia, weight loss and night sweats
Stiffness and pain in the shoulder and pelvic
girdle muscles (polymyalgia rheumatica) may
antedate or coincide with the headache or
appear as a separate entity
Before persistent visual loss there may be
amaurosis fugax

Clinical manifestations signs

The scalp vessels are tender and thickened

Pulsation is reduced more often than it is absent
Scalp necrosis is rare.
The optic disc is swollen and later pale
Ophthalmoplegia is sometimes seen either as a
sixth nerve or a pupil-sparing, third nerve palsy.
Stroke incidence is low.
Rarely, involvement of the aortic arch or of the
mesenteric or limb vessels is symptomatic

Cranial arteritis. Thickened superficial temporal

Cranial arteritis. Fundus photograph showing

ischaemic optic neuropathy in the acute phase

Therapy

Corticosteroid therapy should be started

immediately while biopsy is being
arranged
There are advocates of both high-dose
and low-dose regimes initially (e.g. 60
mg or 20 mg daily of prednisolone).
Eventually, patients are maintained on
7.5-10 mg daily.
The condition tends to resolve after 1224 months, when treatment can be
withdrawn, though some authorities
advocate long-term therapy.

Cranial arteritis

Cranial arteritis is an important condition to

exclude in an elderly patient with a newly
acquired headache
The affected arteries are tender but usually
still pulsatile
The erythrocyte sedimentation rate is usually
markedly elevated but rarely normal
Corticosteroids are the drugs of choice

Meige Syndrome

This condition is the result of dystonic stimulation

mediated through the Facial nerve
Occur in middle-aged or elderly individuals
The signs of orofacial mandibular dystonia and
blepharospasm are characteristic
The changes, which resemble tardive dyskinesia,
are not induced by neuroleptic drugs and are unlike
the open mouth dystonia of Bruegel syndrome
Lower pontine lesions can be identified in some
cases
There may be temporary improvement with
haloperidol, tetrabenazine, or intravenous
Cogentin.
Botulinum toxin injection into the affected facial
muscles is the treatment of choice.

Temporomandibular joint
dysfunction

Degenerative changes in the temporomandibular

joint are associated with pain in the jaw
sometimes referred to the ear or temple.
The pain is exacerbated by chewing.
Pain in a similar distribution, without evidence of
joint derangement,
Pain has been attributed to abnormal activity of
the jaw muscles, either triggered by
malocclusion of the jaw or the result of excessive
jaw contraction in an anxious individual
Treatment is symptomatic