Download The main neurostomatological syndromes (neuralgias of

The main neurostomatological
syndromes
(neuralgia of
Sphenopalatinum, Otic, Ciliary
ganglions and
angiotrophoneurosis)
Facial pain can be developed
due to the next processes:
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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
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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
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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
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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
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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.
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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
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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
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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
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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
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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
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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
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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
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Definition Geniculate neuralgia - is episodes
of severe lancinating pain occurring in the
region of the pinna and external auditory
canal
Etiology and Pathology
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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
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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
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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:
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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:
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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
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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 nasal-ciliary
ganglion
Etiology of Pterygopalatine sympathalgy is nasal
sinus infection (in particular, in Maxillar cavity)
and lesion of ganglion pterygopalatine
Charlen syndrome
(Ciliary neuralgia)
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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
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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)
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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.
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Recurrence of Facial neuropathy in
combination with a plicate tongue,
heilitis is determined as disease of
Rossolimo-Melkerson-Rozental
Treatment
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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
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Vascular syndromes are more
frequent (in patients with so-called
Facial sympathalgias)
They manifest as attacks described
before
Are more typical for men
Cluster-syndrome
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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
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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
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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(20-40)
Rarely, comparable cases have evolved in
association with a structural brain lesion, often
located around the cavernous sinus
Symptoms of Cluster headache
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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.
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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
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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
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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
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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
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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
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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
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Headache, usually localized
Pain on chewing (jaw claudication)
General malaise
Proximal muscle stiffness and pain
Visual loss
Clinical manifestations symptoms
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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
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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 artery
Cranial arteritis. Fundus photograph showing
ischaemic optic neuropathy in the acute phase.
Therapy
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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 12-24
months, when treatment can be withdrawn,
though some authorities advocate long-term
therapy.
Cranial arteritis
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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
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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
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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