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 affectedthan the first

Brief paroxysms of intense, lancinating pain,seldom lasting longer than one minute are oftentriggered by cold, eating, speaking or touchingthe face (trigger zones)

Typically, remission of pain occurs, lastingmonths or even years. Eventually, the painbecomes chronic

Course Disease has typical remittent course Many patients describe, that attacks do

not disturb months and years Disease’ course includes exacerbation

and remissionIn 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: Sympathalgic syndromes (lesion of

autonomic peripheral ganglions and nerves)

1) Nasal-ciliary neuralgia – Charlen syndrome 2) Pterygopalatal neuralgia – Sluder

syndrome 3) Neuralgia of Large Petrosal superficial

nerve – Gartner syndrome Vascular syndromes, as migraine:1) Cluster headache2) Cluster-effect (Horton histamine migraine,

Garris migrainous neuralgia)3) 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 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)

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

T r e a t m e n t

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 so-called 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(20-

40) Rarely, comparable cases have evolved in

association with a structural brain lesion, oftenlocated 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 untreated2. 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 edema3. 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 artery.

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 12-24 months, when treatment can be withdrawn, though some authorities advocate long-term therapy.

Cranial arteritis

Cranial arteritis is an important condition toexclude in an elderly patient with a newlyacquired headache

The affected arteries are tender but usually still pulsatile

The erythrocyte sedimentation rate is usuallymarkedly 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