Transcript 03. Neurostomatological syndromes

The main neurostomatological
syndromes
(neuralgias of
Sphenopalatinum, Otic, Ciliary
ganglions and
angiotrophoneurosis)
Facial pains can be the display
of four processes:
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Trigeminal neuralgia and (rarely)
Glossopharingeal neuralgia
Facial forms of migraine, including
cluster vascular pains
Charlen and Sluder sympathalgia
Psychogenic head pains
Cranial
neuralgias and
central reasons
of pain
Trigeminal neuralgia
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It is characterized by short,
intensive lancinating pain,
recurring at frequent intervals
and confined to one or more
territories of Trigeminal nerve
innervation.
Trigeminal neuralgia
Polyetiological disease, arising up mainly (90 %
and more) in age senior 40 years. Among
patients are women in 60-70 %.
Frequent reasons:
 Mechanical compression of nerve
 Formation of sclerotic plate
 Infections in oral cavity
 Vascular malformations or tumors
 Basic lesions of brainstem
Clinical features
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The second branch of Trigeminal nerve are usually
involved – 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 inter attack period.
Paresthesia with hypesthesia can appear only after
injection-destructive treatment - signs of neuropathy
Autonomic supplement of pain attack is observed less than
in 1/3 patients
Resistant autonomic disorders (changes of skin colour,
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 a 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 do not complain on attacks months
and years
 Disease course is with 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 min
 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 in an inter attack period
 suggestion of pain from triggers zones (cold,
eating, talk, washing, cleaning of teeth,
speaking or touching the face ).
 stereotype of attacks for the same patient
Distribution of trigger zones.
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Thus on the basis of information of
somatic and neurological
inspection, and if necessary instrumental, must be eliminated
other reasons of head and facial
pain, local processes in nasal
sinuses, teeth, jaws, pharynx or
basis of skull.
Glossopharyngeal neuralgia
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Meets rarely.
Typical short paroxysms of pain
centered in throat or the base of the
tongue, tonsils
Typically triggered by swallowing
(especially cold solution), cough,
mastication, talk, yawning
Sometimes pain irradiated in ear and
neck, or only in ear (Oticalgic form)
Glossopharyngeal neuralgia
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Triggers zones - touch to the soft palate or tonsils,
sometimes by pressure on ear
Attack lasts a few seconds or minutes and can be
associated with bradycardia, falling BP, sometimes
syncope
Especially often syncope happen at neuralgia due to
tumour of pharynx
Disorders of sensitiveness or paresis are absent
Often there are spontaneous remission from a few
months to a few years
Combination with Trigeminal neuralgia is possible
(compression of both nerve by complete arched vessel)
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 it is necessary to suspect for
young people with permanent pains and symptoms of
neurological deficit
 Treatment are same, as well as at Trigeminal neuralgia
Neuralgia of Upper
laryngeal nerve
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Characterized by paroxysmal one-sided or bilateral pain
in larynx (usually at the level of upper part of Thyroid
cartilage or hyoid) and corner of mandible
Irradiation in eye, ears, thorax and forearm
Pain is associated with hiccup, hypersalivation, cough
Pains are provoked by swallowing, yawning, cough,
blowing, motion of head.
Some neuralgias of Upper laryngeal nerve are coursed
by malformations of neck
Karbamazepin (Finlepsin) and local anesthesia in hyoid
thyroid membrane are effective
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 lasts from ten minutes to days
(rarely)
Typical is an acute, often unendurable
pain in face of burning, holding apart,
pressing, 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 peace 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,
due to 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 aetiology is not enough clear.
Herpetic rash in case of Charlen syndrome tells
about herpetic ganglionitis of nasal-ciliary
ganglion
Etiology of Pterygopalatine sympathalgia is nasal
sinus infection (in particular, in maxillary sinus)
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 typically observed in internal corner of eye
Usually an attack is associated with acute
lacrymation, changes in frontal 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 (Gasserii) ganglion,
manifest symptoms in area of I
Trigeminal branch innervation.
Expressed 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 clatter)
The attack of pain begins and finishes gradually
Course of attack is quite long
Certain periodicity is absent
Pain is of middle severity with out motor anxiety
After an attack - paresthesia in face and noise in ear
are observed.
Differential diagnosis
Typical signs of neuralgia:
 one-side autonomic features.
 one-side 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 meet
considerably more frequent – at
great part of patients with so-called
Facial sympathalgias.
They manifests by the attacks
described before
Meet more frequent for men
Cluster-syndrome
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Horton Histamine migraine,
Harris migraines neuralgia
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 vessels 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/or temporal
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 ARTERIITIS
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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
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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,
has been attributed to abnormal activity of the jaw
muscles, cither triggered by malocclusion of the jaw
or the result of excessive jaw contraction in an
anxious individual. Treatment is symptomatic.