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Presented by: Ishan

Singh (37); Itee


1
Sharma (38)
Trigeminal Neuralgia
Sphenopalatine Neuralgia
Burning Mouth Syndrome
Orolingual Paresthesia
Auriculotemporal Syndrome
Bells Palsy
Glossopharyngeal Neuralgia
Motor System Disease
Multiple Sclerosis
Orofacial Dyskineasia
Menieres Disease
Migraine
Temporal/Giant Cell Arteritis
Complex Regional Pain Syndrome
Atypical Facial Pain
Horners Syndrome

2
Severe Generalized Familial Muscular
Dystrophy
Mild Restricted Muscular Dystrophy
Dystrophic Myotonia
Congenital Myotonia
Acquired Myotonia
Hemifacial Spasm
Periodic Paralysis
Hypotonias
Myasthenia Gravis
Dermatomyositis
3
Congenital myotonia is an anomaly of
muscular contraction in which an
inheritance pattern has been established in
about 25% of the reported cases.

It is an autosomal dominant trait but with


incomplete penetrance in some families.

4
Congenital myotonia commences early in
childhood and may be first noticed
because of difficulties in learning to stand
and walk.
Muscular contraction induces severe,
painless muscular spasms, actually a delay
in relaxation.
Electrical or physical stimulation of a
muscle produces characteristic prolonged
contraction or percussion contraction.
The muscles are large, and patients with
this disease are described as presenting a
Herculean appearance. 5
The muscles of the thighs, forearms and
shoulders are especially affected, as well as
the muscles of the neck and the masseter
muscles of the face.
The muscles of the tongue are not reported
to be affected by the hypertrophy,
although they may be involved by the
myotonia.
Blinking with strong closure of the eyes will
sometimes produce a prolonged
contraction of the lids. Spasms of the
extraocular muscles may lead to
convergent strabismus.
6
Muscle biopsy reveals no alterations from
normal except for hypertrophy of all
muscle fibers.

7
There is no specific treatment of the
disease, but the prognosis is good.

In fact, some regression of the disease


occurs in occasional patients.

8
Acquired myotonia, refers to spasms of
muscles, although such spasms are
generally considered to be more intense
than those occurring in typical myotonia.
If these spasms are intermittent, the
condition is called clonus (myoclonic
contractions); if constant, the term trismus is
applied (myotonic contractions).
All gradations in the degree of spasmodic
contraction occur, ranging from slight
muscular twitches to severe, painful,
prolonged muscular cramps.
9
Spasm involving the facial muscles is seen
in a variety of situations such as epilepsy,
diseases of the CNS and tetany.
Such spasms on a local basis are far more
common; however, and these occur in a
variety of conditions such as pericoronal
infection, especially of third molars;
infectious myositis; and hysteria (hysterical
trismus).
The spasms, which are usually painful, may
be transitory or may persist for a period of
several days or until the cause of the
disease is treated.
10
The head and neck region is a common site for
neuralgias.
Facial neuralgias produce pain that often
mimics pain of odontogenic origin, the dental
practitioner is frequently called on to rule out
odontogenic or inflammatory causes.
A comprehensive understanding of the
disorders affecting the nerve pathways and
the nerve supply of the various anatomic sites
and structures associated with the oral cavity is
essential for the dentist if he is to determine
successfully the true nature of the pain and
take appropriate measures to effect its relief.
11
Other Names: Tic doulourex; trifacial neuralgia;
Fothergills disease
Folllows the anatomical distribution of the fifth
cranial nerve.
Mainly affects the second and third divisions of
the trigeminal nerve.
Almost always exhibits a trigger zone,
stimulation of which, initiates paroxysm of pain.
Pain often accompanied by brief facial spasm
or tic.
Unilateral pain (most often) and lasts for a few
seconds to a minute.
12
The causative mechanism of pain in this condition
remains controversial.
Most cases are idiopathic but compression of the
trigeminal roots by tumors or vascular anomalies may
cause similar pain.
Uncommonly, an area of demyelination, such as may
occur with multiple sclerosis , may be the precipitant.
Development of trigeminal neuralgia in a young
patient suggests possibility of multiple sclerosis.
Infrequently, adjacent dental fillings composed of
dissimilar metals may trigger attacks (galvanism).

13
Older adults more commonly
affected by trigeminal
neuralgia then young persons.
(Rare before 35 years of age)
Females are more commonly
affected. ( M:F::3:2)
Right side of the face is affected more than
the left side by the ratio 1.7:1
Pain is of a searing, stabbing, or lancinating
type which many times is initiated when the
patient touches a trigger zone' on the
face.
14
The term tic doulourex is applied when the patient
suffers from spasmodic contractions of the facial
muscles.
In early stages, the pain is relatively mild, but as the
attacks progress over a period of months or years,
they become more severe and tend t occur more
frequently.
Pretrigeminal neuralgia: early pain described as dull,
aching or burning or resembling a sharp toothache.
Later the pain may be so severe thata the patient
lives in constant fear of an attack, and many sufferers
have attempted suicide to put an end to their
torment.

15
Each attack of excruciating pain persists for only a
few seconds to several minutes and characteristically
disappears as promptly as it arises.
As the attack occurs, the patient may clutch his face
as if in terror of the dreaded pain.
The patient is free of symptoms between two attacks.
Any portion of the face may be involved by the pain,
depending upon which branches of the fifth nerve
are affected.
The maxillary and mandibular divisions are more
commonly affected then the ophthalmic; in some
instances two divisions may be simultaneously
affected.

16
The trigger zones which precipitate
an attack when touched are
common on the vermillion border of
the lips, the alae of nose, the
cheeks, and around the eyes.
Usually any given patient manifests
only a single trigger zone.
The patient learns to avoid touching the skin over the
trigger area and frequently goes unwashed or
unshaven to forestall any possible triggering of an
attack.
In some cases, exposure to a strong breeze or simply
the act of eating or smiling has been known to
precipitate an attack.

17
The presence of a trigger zone, the fleeting but
severe type of pain occasioned and the location of
the pain usually provides the key for establishing the
diagnosis of trigeminal neuralgia.
One of the more common conditions mistaken for
trigeminal neuralgia is migraine or migrainous
neuralgia (Hortons syndrome), but this severe type
of periodic headache is persistent, at least over a
period of hours, has no trigger zone.
Sinusitis has also been confused with this disease so
should be kept in differential diagnosis.
Tumors of nasopharynx can also produce a similar
type of pain, generally manifested in the lower jaw,
tongue and side of the head with an associated
middle ear deafness.
18
A condition clinically similar to trigeminal neuralgia often
occurs after attacks of herpes zoster of fifth nerve called
post herpetic neuralgia- The pain involves ophthalmic
division of trigeminal nerve and commonly regresses within
two or three weeks. The history of skin lesions prior to onset
of neuralgia aids in diagnosis.
Trigeminal neuritis or trigeminal neuropathy is a condition
that differs from trigeminal neuralgia by being described
more often as an ache, variously stated as a burning,
boring, pulling drawing or pressure sensation which
continues over a period of hours, days or weeks rather
than the instantaneous jolt of pain in trigeminal neuralgia
A pain of dental origin may also stimulate this disease and
diligent search is necessary to establish a dental diagnosis.

19
Each of the many types of treatment suggested has
its advocates, but none is successful in all cases.
One of the earliest forms of treatment was peripheral
neurectomy-- sectioning of the nerve at the mental
foramen or at the supra or infra orbital foramen. Only
provides temporary relief.
The injection of alcohol either into a peripheral nerve
area or centrally into the gasserian ganglion provides
respite from all symptoms for a period of six months to
several years after alcohol injection.
The injection of boiling water into the gasserian
ganglion has also been reported to be beneficial in
causing respite from pain.

20
The use of phenytoin (dilantin)
in the management of
trigeminal neuralgia has been
found to be efficacious in some
cases. The use of the drug must
be continuous as there are cases
of return of pain following
cessation of drug use.
If a patient who is presumed to have this disease
does not respond to carbamazepine in 24-48 hours
the diagnosis is seriously in doubt.
New procedures like microsurgical decompression of
the trigeminal nerve have been shown to produce
good results.

21
Other names: Sphenopalatine ganglion
neuralgia, lower half headache, Sluders
headache, vidian nerve neuralgia, atypical
facial neuralgia, hortorns syndrome, cluster
headache.
Described by Sluder as a symptom complex
referable to the nasal ganglion.
Idiopathic syndrome consisting or recurrent
brief attacks of sudden, severe, unilateral
periorbital pain.

22
Its typical periodicity attributed to
hypothalamic hormonal influences.
The pain is thought to originate at the
level of carvernous sinus complex.
Cases of this syndrome affecting multiple
members of the same family have been
reported suggesting a genetic
predisposition in some individuals.

23
Characterized by unilateral paroxysms of intense pain in
the region of eyes, the maxilla, the ear and mastoid, base
of nose, and beneath the zygoma.
These pain proxysms have rapid onset, persist for about 15
minutes to several hours and disappear rapidly.
There is n trigger zone. The pain is often described as
toothache by patients.
The attacks develop regularly often once a day over a
prolonged period of time.
For some patients the onset of paroxysm occurs at exactly
the same time of day, and for this reason the disease has
been referred to as Alarm clock headache.
After some weeks or months the attacks disappear
completely and reoccur subsequently.

24
In addition to pain sensation
experienced by the patients,
other symptoms have also
been reported
Sneezing, swelling of nasal
mucosa and severe nasal
discharge often appear simultaneously with the
painful attacks, as well as epiphora, or watering of
the eyes, and bloodshot eyes.
It has been noticed in many patients that the attacks
are precipitated either by emotional stress or
injudicious intake of alcohol.
Men are affected more than women (5:1) and
common age of onset is before the age of 40 years.

25
Most widely used treatment has been
cocainization of the sphenopalatine ganglion
or alcohol injection of this structure.
Ergotamine will often produce immediate and
complete relief of symptoms. In cases where it
is not totally effective, combining it with
methysergide, an antiserotonin agent, appears
to produce a synergistic action providing total
relief.
Invasive nerve blocks and ablative
neurosurgical procedures have been
implemented successfully in refractory cases.
26
Burning mouth syndrome is a burning or
stinging of mucosa of lips, tongue in
absence of visible mucosal lesions.
Defined by van der Waal as idiopathic
cases in which the main symptoms are
located in the oral mucosa, with or without
involvement of any other part of the body.
Strong female predilection mostly
postmenopausal and approx age of onset
being 50 years.
27
Local causes:
Dry mouth
Mucosal disorders- geographic tongue, lichen planus etc.
Trauma to oral mucosa (eg: poorly fitting dentures).
Repetitive oral habits such as tongue thrusting.
Gastroesophageal reflux disease.
Sensory nerve damage due to trauma.

Systemic medical causes:


Vitamin B12, folate, iron deficiencies.
Medication eg. ACE inhibitors such as captopril.
Immunologically mediated diseases such as Sjogren syndrome.
Psychogenic disorders such as depression, anxiety.
Stressful lifestyle.
Menopause.
Diabetes mellitus

28
The burning sensation may be felt either as a continuous or
intermittent discomfort which most frequently affects the
tongue, and sometimes the lips or palate.
Onset of the symptoms may be sudden or gradual after
months. Psychosomatic factors are associated with the
onset of burning mouth syndrome.
No oral mucosal lesions will be detected on examination.
50% of the patients report an associated sensation of dry
mouth which is not confirmed on investigation.
Affected patients may also report altered taste sensation
with reduced taste perception or presence of a persistent
unusual taste mostly bitter or metallic.
Drinking or eating may temporarily reduce symptoms.
Patients may have associated anxiety or depression.

29
Treatment modalities include
antidepressants, vitamins or dietary
supplements such as alpha lipoic acid;
analgesic sprays or mouthwashes such
as benzydamine hydrochloride, and in
postmenopausal female patients,
hormone replacement or topical
estrogen applied to the oral mucosa.
Saliva substitutes may be considered in
cases of dry mouth.
30
Other names: Glossodynia; painful tongue, glossopyrosis, burning
tongue.
Etiology:
Deficiency states such as pernicious anemia and pellagra.
Diabetes
Gastric disturbances such as hyeracidity or hypoacidity.
Psychogenic factors such as stress, sexual maladjustment and
cancerophobia
Periodontal disease
Xerostomia
Hypothyroidism
Referred pain from abscessed teeth or tonsils
Oral habits such as excessive use of tobacco, spices and the like.
Antibiotic therapy
Local dental causes such as dentures, irritating clasps or new fixed
bridges.

31
The tongue is most frequently the site of the
paresthetic sensations however any site in
the oral cavity may be affected.
The sensations encountered are pain,
burning, itching, and stinging of the mucous
membranes.
The appearance of the tissues is usually
normal and there are no apparent lesions
to explain the sensations.
Seen most frequently in women of the
postmenopausal age.
32
Drugs that have been tried include topical
anesthetics, anagesics, smooth and skeletal
muscle relaxants, sedatives, antibacterial
and antifungal agents, antihistamines,
vitamins, enzyme digestants, CNS
stimulants, salivary stimulants, vasodilators
and sex hormones.
Permanent remission of the condition
cannot be expected after drug therapy
except in certain cases.
33
Other names: Freys syndrome, gustatory sweating.
It is an unusual phenomenon , which arises as a result
of damage to the auriculotemporal nerve and
subsequent reinnervation of swear glands by
parasympathetic salivary fibers.
Etiology: The syndrome follows some surgical
procedure such as removal of a parotid tumor orr the
ramus of the mandible, or a parotitis of some type
that has damaged the auriculotemporal nerve
After a considerable time following surgery, during
which the nerve regenerates, the parasympathetic
salivary nerve supply develops, innervating the sweat
glands, which then function after salivary or psychic
stimulation.

34
Patient exhibits flushing and
sweating of the involved side
of the face, chiefly in the
temporal area, during eating.
Profuse sweating may be
evoked by the parentral
administration of pilocarpine or
eliminated by the administration of atropine or by a
procaine block of the auriculotemporal nerve.
A similar condition known as crocodile tears in which
patients exhibit profuse lacrimation when food is eaten,
particularly hot or spicy foods.
Auriculotemporal syndrome is not a common condition
but the possibility of its occurrence should always be
considered after surgical procedures in the area supplied
by the ninth cranial nerve.

35
Treatment of auriculotemporal syndrome
by intracranial division of the
auriculotemporal nerve has been
reported to be successful.

36
Bells palsy is a self limiting, unilateral facial paralysis.
Suspected causes include reactivation of herpes
simplex or zoster in the geniculate ganglion, nerve
demylination, nerve edema or ischemia,
autoimmune damage to nerves and vasospasm of
vessels associated with nerves.
Incidence: 24 of every 100,000 persons each year
with increased frequency in the fall and winter
seasons.
Occurs more frequently in demyelinating diseases
such as multiple sclerosis.
Also associated with Melkersson- Rosenthal
syndrome, Lyme disease and sarcoidosis.

37
38
Susceptible age group: All ages but middle
aged people are most frequently affected.
Gender: Women affected more often than
men (71%)
Childhood involvement associated with a
viral infection, Lyme disease or earache.
Palsy is characterized by an abrupt loss of muscular control
on one side of the face, imparting a rigid masklike
appearance and resulting in the inability to smile, to close the
eye, to wink, or to raise the eyebrow.
A few patients experience prodromal pain on the affected
side before the onset of paralysis.

39
Patients usually awaken in the morning with a full fledged
case.
Rapid onset of bilateral facial weakness should alert to the
possibility of Gullian-Barre syndrome or other types of
vasculitis causing multiple cranial neuropathies.
When vertigo or tinnitus is a major symptom, an occult
herpes zoster ear infection should be suspected changing
the diagnosis to Ramsay Hunt syndrome.
Cornor of the mouth drops, causing saliva to drool onto
the skin.
Slurred speech and abnormal taste may also be present.
Conjuctival dryness or ulceration may occur because the
eyelid cannot close.

40
Histamine and other vasodilators,
corticosteroids and hyperbaric oxygen therapy
may shorten the duration of palsy.
Topical ocular antibiotics and artificial tears
may be required to prevent corneal ulceration
and the eyelid may have to be taped shut.
Symptoms begin to regress slowly within 1-2
months of onset. Sever cases as well as older
patients may take longer.
Residual symptoms that remain after 1 year will
probably remain indefinately.
Recurrence is seen in Melkersson-Rosenthal
syndrome.
41
42
43
This condition is not as common as trigeminal
neuralgia but when it occures, the pain may be as
severe and excruciating.
Clinical features: No gender predilection. Occurs in
middle-aged or older persons.
Manifests itself as a sharp, shooting pain in the ear,
the pharynx, the nasopharynx, the tonsil or the
posterior portion of the tongue.
Almost always unilateral, paroxysmal, rapidly
subsiding.
Numerous mild attacks may be interspersed by
occasional severe ones.
Trigger is usually the posterior oropharynx or tonsillar
fossa.
44
Inciting actions include actions like
swallowing, talking, yawning or
coughing.
The etiology is unknown.
Treatment: Treatment consists of
resection of the extracranial portion of
the nerve or intracranial section.
Periods of remission with subsequent
recurrence are common in this disease.
45
Other names: Motor neuron disease,
amyotrophies.
Constitutes a group of closely related
conditions f unknown etiology which
occur in three clinically variant forms
referred to as progressive muscular
atrophy, amyotrophic lateral sclerosis,
and progressive bulbar palsy.

46
Progressive muscular atrophy: Characterized
by progressive weakness of the limbs with
associated muscular atrophy, reflex loss and
sensory disturbances.
Shows a strong hereditary pattern, affects
males more than females and tends to occur in
childhood.
Symptoms consist of difficulty in walking , with
leg pain and paresthesia.
Atrophy of the foot, leg and hand muscles
ultimately occurs with appearance of a typical
foot-drop, steppage gait and stork legs.

47
Amyotrophic lateral sclerosis: Occurs between
ages 40-50 and affects males more frequently.
Precipitating factors include fatigue, alcohol
intoxication, trauma and certain infections
such as syphilis, influenza, typhus and epidemic
encephalitis.
Initial symptoms consist of weakness and
spasticity of the limbs, difficulty in swallowing
and talking with indistinct speech and
hoarseness. Atrophy and fasciculation of the
tongue with impairment or loss of palatal
movements may also occur.
48
Progressive bulbar palsy: Characterized by difficulties
in swallowing and phonation, hoarseness, facial
weakness and weakness of mastication.
Occurs in patients in the fifth or sixth decades of life
with a familial pattern in some instances.
Symptoms include difficulty in articulation, with
impairment and finally loss of swallowing.
Chewing is difficult as the muscles become
weakened.
Patients exhibit atrophy of the face, masseter and
temporal muscles, and tongue with fasciculation of
the face and tongue.
There is also impairment of the palate and vocal
cords.
49
Other name: Disseminated sclerosis.
It is an idiopathic inflammatory demyelinating
disease of the CNS.
Etiology: Multiple sclerosis
is believed to result from
an autoimmune process.
There is a contributory role
of environmental agents to
genetic predisposition in the
causation of this disorder.

50
Rarely occurs in individuals younger than 20 years .
Female gender prediliction (2:1) and a familial
incidence is often observed.
A variety of ocular disturbances including visual
impairment as a manifestation of retrobulbar neuritis,
nystagmus and diplopia.
Fatigability, weakness and stiffness of extremities with
ataxia or gait difficulty involving one or both legs.
Superficial or deep paresthesia.
Personality and mood deviation toward friendliness
and cheerfulness.
Autonomic effector derangements, such as bladder
or rectal retention or incontinence.

51
Charcots triad is a well known diagnostic
triad characteristic of multiple sclerosis
consists of intention tremor, nystagmus and
dysarthria or scanning speech.
Facial and jaw weakness occurs in some
patients and a staccato type of speech
has been described.
Bells palsy and trigeminal neuralgia have
been reported in some patients with
multiple sclerosis.
There is no treatment for multiple sclerosis.
52
It is condition thought to result from either an
extrapyramidal disorder or a complication of
phenothiazine therapy.
Clinical features: This disorder occurs more frequently in
persons over the age of 60 years than in the young.
Characterized by severe, involuntary, dystonic movements
of the facial, oral and cervical musculature.
Irregular and involuntary movements such as lip-smacking
and lip-licking, protrusion of the lips as in pouting,
protrusion of tongue and mandible with uncoordinated
movements, and grimacing are all typical manifestations.
Dyskinesia may occur alone or in association with torticolis
or generalized dystonia.

53
Surgical operations
similar to those carried
out in the treatment of
parkinsons disease
generally cause improvement in the
symptoms of the disease.
It has also been suggested that
correction of denture occlusion may be
an effective therapeutic procedure.
54
Other names: Menieres disease, Menieres
syndrome, endolynphatic hydrops.
It is an inner ear disorder in which there is an increase
in volume and pressure of the endolymph of the inner
ear.
Typically presents with waxing and waning hearing
loss and tinnitus associated with vertigo.
Etiology: The exact cause is unknown.
Current theory states that it is the response of inner
ear to injury.
There is an increase in the volume of endolymph with
distention of the entire endolymphatic system which
esults in permanent damage to both the vestibular
and cochlear apparatus.
55
Characterized by deafness, tinnitus and vertigo
usually beginning in middle age.
Commences with tinnitus and deafness which are
unilateral in approx 90% of the cases.
Vertigo is often a late symptom of the disease and
many times is accompanied by attacks of nausea
and vomiting which may be incapacitating.
The vertigo usually has a sudden explosive onset and
persists for several minutes to several hours.
These same signs and symptoms commonly occur in
cases of cardiovascular disorders or with cerebral
arteriosclerosis.
True Menieres disease has histopathology changes in
the labyrinth.
56
In true Menieres disease there is dilation of
endolymphatic spaes of the labyrinth with
absence of an inflammatory reaction.
The increased endoplymphatic fluid
pressure appears to diminish and distort the
response to stimulation of the end organs of
the labyrinth and cochlea.
It has been suggested that the basic cause
of this disorder is either an autonomic
vasomotor dysfunction or an intrinsic
allergy.
57
No treatment of Minieres disease is
wholly effective.
Management by drugs in generally
unsuccessful.
Some patients react favorably to
vasodilators such as histamine or niacin.
Surgical intervention may be considered
to relieve the vertigo. Section of eighth
nerve or destructive labrynthotomy.
58
A dominantly inherited disorder
characterized by varying degrees of
recurrent vascular headache,
photophobia, sleep disruption and
depression.
Etiology: Headache during migraine attack
was thought to be a reactive hyperemia in
response to vasoconstriction-induced
ischemia during aura. This explains the
throbbing quality of the headache, its
varied localization and the relied obtained
from ergots.
59
Usually begins in the second decade of life and is
common in professional persons.
Migraine headaches are reported to affect women more
than men.
They may occur at frequent intervals over a period of
years or on only a few occasions during the lifetime of the
patient.
A prodromal stage/ preheadache phenomenon is noted
by some patients, consisting of lethargy and dejection
several hours before the headache.
Visual phenomenon such as scintillations, hallucinations or
scotomas are often described.
Other less common prodromal phenomenon include
vertigo, aphasia, confusion, unilateral paresthesia or facial
weakness.

60
The headache phase consists of severe pain in the
temporal, frontal and retro orbital areas. Other sites such
as parietal, postauricular, occipital or suboccipital are also
involved.
The pain is usually unilateral but may become bilateral
and generalized.
The pain is not necessarily confined to the same side of
the head in successive attacks.
Pain is described as deep, aching , throbbing pain.
The patient may appear extremely ill at the time of
headache. Face is usually pale, sallow and sweaty.
Patient is irritable and fatigued with impaired memory and
concentration.
Anorexia and vomiting may occur with visual
disturbances.

61
Treatment includes a wide variety of
drugs ranging from acetylsalicylic acid
and codeine to ergotamine,
methysergide and norepinephrine.
The disease has a good prognosis and
may undergo complete and permanent
remission.

62
It is a cause of headache which is
frequently diagnosed erroneously as
atypical migraine.
It is a focal granulomatous inflammation of
arteries, especially the cranial vessels.
The temporal arteries are particularly prone
to develop these lesions.
Similar lesions found throughout the skeletal
muscles related to their vasculature are
termed as polymyalgia arteritica.

63
Etiology: It is primarily a
disease of cellular immunity.
The vasculitic damage is
mediated by activated
CD4+ T helper cells
responding to antigen
presented by macrophages.
The primary inflammatory response affects
the internal elastic lamina.
The actual inciting antigen remains
unknown, but elastin remains an important
suspect.
64
Temporal arteritis occurs most frequently in older
persons usually between the ages of 55 and 80 years.
Affects women far more frequently than men.
The onset may be slow and insidious or the disease
may develop suddenly with a headache or a
burning, throbbing type of pain.
A general malaise, chills and fever and weight loss
with anorexia, nausea, and vomiting may precede
any manifestation of pain.
These symptoms are sometimes followed by aching
and stiffness of the muscles of shoulders and his which
is often termed as polymyalgia rheumatica.

65
The pain frequently may be localized first in
the teeth, temporomandibular joint, scalp,
or occiput.
Many patients complain of tiredness,
fatigue and pain on repetitive chewing.
Ultimately there is localized inflammation or
cellulitis over the swollen, nodular, tortuous
artery.
The ESR is markedly elevated in the majority
of these patients and a mild leukocytosis
may also be found. The are non specific
findings however.
66
The histopathology of the diagnostic arterial lesion
includes intimal proliferation with resulting luminal
stenosis, disruption of the internal elastic lamina by a
mononuclear cell infiltrate, invasion and necrosis of
the media progressing to panarteritic involvement by
mononuclear cells, giant cell formation with
granulomata within the mononuclear cell infiltrate,
and less consistently, intravascular thrombosis.
Treatment and prognosis: The response of temporal
arteritis to corticosteroid therapy is excellent, and
clinical manifestations subside within a few days.
In cases with widespread systemic vascular
involvement, the course of the disease may be
progressively downhill and may be fatal.

67
Other names: Causalgia,
reflex sympathetic
dystrophy syndrome.
Causalgia is a term
applied to severe
pain which arises after
injury to or sectioning
of a peripheral sensory
nerve.
It is readily identifiable signs and symptoms and is
treatable if recognized early or may become disabling if
unrecognized.
Etiology: It is believed that development of causalgia
requires the following triad of conditions: an injury, an
abnormal sympathetic response, and a predisposing
personality.

68
It may develop in patients of any age.
Usually follows extraction of a multirooted
tooth, particularly when the extraction is
difficult or traumatic. The pain arises within a
few days or several weeks after the extraction
and has a typical burning quality from which
the condition derives its name.
The attack may be elicited by hot or cold
application or by contact or simply by
emotional disturbances.
The pain is reported to be intensified by head,
ingestion of alcohol, during menstrual periods
or when the patient is frustrated or upset.
69
It should be differentiated from local pain
due to simple traumatic injury to soft tissue
or bone during the extraction procedure.
There is another disease which typically
produces referred pain in the posterior
portion of the mandible: Subacute
thyroiditis. Its etiology is unknown. It has
been reported to occcur in many patients
with thyroitditis.
Treatment of thyroiditis invariably results in
subsidence of the jaw pain.
70
The treatment of intraoral causalgia is
indeed a difficult one.
The injections of procaine, alcohol nerve
block, phenol cauterization and surgical
curettement of the bone in the involved
area have generally proved ineffective.
Resection of nerves in the retrogasserian
region has afforded relief.
It has been revealed that the patient
submits to numerous procedures but still
continues to suffer from the severe pain.
71
Other names: Atypical facial neuralgia, facial causalgia.
Constitutes a group of conditions in which there is a
vague, deep, poorly localized pain in the regions suppied
by the fifth and ninth cranial nerves and the second and
third cervical nerves.
The distribution of this pain is unanatomic. It involves
portions of the sensory supply of two or more nerves and
may cross the midline.
This pain lacks trigger zone and is constant and persists for
weeks, months or even years.
Atypical facial pain occurs in the territory of trigeminal
nerve but its pattern and quality are different. The
distinction is important for making treatment decisions.

72
It occurs usually without a specific cause. Injury
of any peripheral or proximal branch of the
trigeminal nerve due to facial trauma or basal
skull fracture can produce the disorder.
One condition that always must be considered
in differential diagnosis is Eagles Syndrome. This
syndrome consists of either elongation of the
styloid process or ossification of the stylohyoid
ligament causing dysphagia, sore throat,
otalgia, glossodynia, headache, vague
orofacial pain or pain along the distribution of
the internal and external carotid arteries.
73
The most consistent symptom is pharyngeal
pain. It is a common difficulty to arise after
tonsillectomy because of the fibrous tissue
that is formed and is stretched and rubber
over the elongated styloid process.
Another form known as carotid artery
syndrome in which pressure exerted by
either a deviant styloid process or an
ossified ligament causes impingement on
the internal or external carotid arteries
between the styloid process lies.
74
Medical treatment of atypical facial
pain is less satisfactory than hat of
trigeminal neuralgia.
Tricyclic antidepressants give the best
results of the non narcotic drugs.
Phenytoin is of intermediate
effectiveness, and carbamazepine is
least effective.

75
Other name: Sympathetic ophthalmoplegia.
It is a condition
characterized by:
Miosis, or contraction
of the pupil of the eye
due to paresis of the
dilator of the pupil.
Ptosis, or drooping of
the eyelid due to paresis
of the smooth muscle elevator of the upper lid.
Anhidrosis and vasodilation over the face due
to interruption of sudomotor and vasomotor
control.
76
The exact features of the syndrome depend upon the
degreee of damage of sympathetic pathways to the
head and the site of this damage.
Lesions in the brainstem chiefly tumors or infections, or in
the cervical or high thoracic cord occasionally will
produce this syndrome.
Preganglionic fibers in the anterior spinal roots to the
sympathetic chain in the low cervical and high thoracic
area are rather commonly involved by infection, trauma
or pressure as by aneurysm or tumor to produce Horners
syndrome.
Involvement of the carotid sympathetic plexus by lesions
of the gasserian ganglion or an aneurysm of the internal
carotid artery may produce the typical facial sweating
defect as well as facial pain and sensory loss.

77
78
Also known as pseudohypertrophic
muscular dystrophy of Duchenne.
Rapidly progressive muscle disease
usually beginning in early childhood.
Familial transmission through unaffected
females, predominantly occurring in
males.
Most common type of muscular
dystrophy.
79
Clinical Features
Earliest signs are inability to walk or run ,
falling readily due muscular enlargement
and weakness.
Muscles of extremities are affected first
but facial muscles may also be involved.
Muscular enlargement ultimately leads to
atrophy.
This atrophy leads to ambulatory and
postural defects such as waddling gait.
Muscles of mastication, facial and ocular,
laryngeal and pharyngeal muscles are
involved in late stages.
80
Gradual disappearance of muscle fibres ,
ultimately replaced entirely by connective
tissue and fat.

Fibres show variation in size in early stages


of disease , some being hypertrophic others
being atrophic.

81
Serum creatine phosphokinase levels are
elevated in affected males and in about
70% of carrier females.

CPK elevation occurs prior to clinical


manifestations of disease in males.

82
Disease remains incurable.

With proper care and attention patients


have chances of better life, but most die
by the age of 30 years, due to
cardiopulmonary failure.

83
Also known as Facioscapulohumeral
dystrophy of Landouzy and Dejerine.
It is a slowly progressive proximal
myopathy which involves the muscles of
the shoulder and face and has weak
familial incidence.
It frequently presents long remissions and
complete arrest.
One variant of this is a slowly progressive
one without facial weakness. 84
It is an autosomal dominant disease in
70-90% of patients and is sporadic in the
rest.

One of the causative has been localized


to chromosome band 4q35.

85
Begins at any age 2-60 years, although
onset in majority of cases is in first 2
decades.
Frequency of occurrence is higher in males.
Earliest signs maybe inability to raise arms
above the head and inability to close the
eyes even during sleep due to weakness of
facial muscles.
The lips develop a characteristic looseness
and protrusion which have been descibed
as tapir-lips, a part of myopathic facies
and patients are unable to whistle or smile.
86
Scapular muscles become atrophic and
weak with subsequent alteration in
posture.

Cardiac abnormalities, including


cardiomegaly and tachycardia are
often present and many patients die of
sudden cardiac failure.

87
No specific microscopic findings are
found in this disease.

There is some variation in size of muscle


fibers and moderate infiltration of fiber
bundles by connective tissue.

Individual fibers ultimately become


atrophic.

88
There is no treatment for the disease.

Some patients undergo temporary


periods of remission or even complete
arrest.

89
Described as steadily progressive,
familial, distal myopathy with associated
weakness of muscles of face, jaw and
levators of eyelids, a tendency for
myotonic persistence of contraction in
affected parts and testicular atrophy.

It is inherited as autosomal dominant


characteristic.

90
Atrophy of muscles is characteristic feature
of this disease, generally manifested in
hands and forearms.
This muscular wasting does not appear until
third decade of life.
Alterations in facial muscles is one of the
prominent features. These include ptosis of
eyelids and atrophy of masseter and
sternocleidomastoid muscle.
The masseteric atrophy produces a
narrowing of the lower half of the face
which, with the ptosis and generalized
weakness of the facial musculature, gives
the patient a characteristic myopathic
facies and swan neck.
91
The muscles of the tongue commonly show
myotonia but seldom atrophy.
Pharyngeal and laryngeal muscles in patients
with dystrophic myotonia also exhibit
weakness manifested by a weak,
monotonous, nasal type of voice and
subsequent dysphagia.
Other clinical features frequently associated
with dystrophic myotonia include testicular
atrophy, which is so common as to be
considered an integral part of the syndrome;
cataracts, even in a high percentage of
young patients; hypothyroidism with coldness
of extremities, slow pulse and loss of hair; and
functional cardiac changes.
92
Enlargement of scattered muscle fibers and
the presence of centrally placed muscle
nuclei in long rows have been described as
being characteristic of atrophy.
True hypertrophy of some fibers is almost
invariably found, as well as isolated fibers
which show extreme degenerative
changes, including nuclear proliferation,
intense basophilic cytoplasmic staining and
phagocytosis.
In advanced muscular atrophy, fibers
appear small, and there may be interstitial
fatty infiltration.
93
There is no treatment for this disease.

It progresses inevitably over a period of


many years, producing disability and
ultimately death.

94
Also known as Facial myoclonus, facial
dystonia.
Hemifacial spasm is a disease
characterized by repeated, rapid, painless,
irregular, nonrhythmic, uncontrollable,
unilateral contractures of the facial muscles
in adults, chiefly women.
The cause of this condition is unknown, but
appears to be a peripheral facial nerve
lesion.

95
Hemifacial spasm usually begins in the
periorbital muscles, but soon spreads to the
entire half-face.
It is first manifested as a brief transitory
twitching, but may progress to sustained
spasms.
These spasms are often triggered by
fatigue, tension or facial activity and are of
brief duration, usually only a few seconds.
In cases of long-standing hemifacial spasm,
mild facial contracture may occur, as well
as lid closure and lip pursing. 96
There is no treatment for this disease, but
decompression of the facial nerve in its
canal has offered relief in some cases.

It is a progressive, nonfatal illness.

It almost always responds favorably to


treatment.

97
The heterogeneous group of muscle diseases
known as periodic paralyses (PP) is
characterized by episodes of flaccid muscle
weakness occurring at irregular intervals.
They can be divided conveniently into
primary and secondary disorders.
General characteristics of primary PP include
the following:
They are hereditary
Most are associated with alteration in
serum potassium levels
Myotonia sometimes coexists
Both myotonia and PP result from
defective ion channels.
98
Paramyotonia is manifested by cramping,
stiffness and weakness of the muscles of the
face and neck, fingers and hands upon
exposure to cold.
The eyelids are closed, and the face
assumes a mask-like appearance.
The tongue may exhibit a similar cramping
after drinking cold liquids, and the speech
becomes slurred.
In many cases, myotonia of the tongue
may be induced by percussion, although
this is not true of other muscles.
99
Reports of microscopic study of muscles
from patients with paramyotonia are
almost entirely lacking.

Information available indicates that


there are no significant histologic
changes in muscle fibers.

100
There is no specific treatment for
paramyotonia, but the prognosis is
excellent with frequent improvement
during adult life.

101
Hypotonia is a reduction or complete absence
of tonus in muscles.
There are many causes of hypotonia and delay
in motor development in infants, so that this
condition should be regarded only as a
symptom which may be found in many
diseases.
Certain congenital diseases may result in
hypotonia, such as:
Diseases of the central nervous system (e.g.
atonic diplegia)
Lipoid and glycogen storage diseases (e.g.
Tay-Sachs disease)
102
Mongolism
Cretinism
Achondrodysplasia.
Hypotonia also may result from strictly
neuromuscular diseases, however,
including:
Infantile muscular atrophy
Amyotonia congenita
Congenital nonprogressive myopathy
Neonatal myasthenia gravis.

103
Infants have a generalized weakness so
that their bodies hang limply with inability to
sit, stand or walk.
The hypotonia involves the muscles of the
face and tongue as well, but these findings
are secondary to the generalized
condition.
The term floppy infant syndrome has
sometimes been applied to describe the
chief clinical manifestations of this disease.

104
Myasthenia is an abnormal weakness and
fatigue in muscle following activity.

The myasthenias constitute a group of


diseases in which there is a basic disorder
of muscle excitability and contractility
and include myasthenia gravis; familial
periodic paralysis; and aldosteronism.

105
Myasthenia gravis (MG) is an acquired
autoimmune disorder characterized
clinically by weakness of skeletal muscles
and fatigability on exertion.

106
Myasthenia gravis is idiopathic in most
patients but autoimmunity is also
implicated to be responsible.

Theantibodies in MG are directed


toward the acetylcholine receptor
(AChR) at the neuromuscular junction
(NMJ) of skeletal muscles.

107
Myasthenia gravis occurs chiefly in adults in
the middle-age group, with a predilection for
women, and is characterized by a rapidly
developing weakness in voluntary muscles
following even minor activity.
Muscles of mastication and facial expression
are involved by this disease, frequently before
any other muscle group.
The patients chief complaints may be
difficulty in mastication and in deglutition, and
dropping of the jaw. Speech is often slow and
slurred. Disturbances in taste sensation occur in
some patients.
108
Diplopia and ptosis, along with dropping of
the face.
The neck muscles maybe be so weak that
the head cannot be held up without
support.
Patients with this disease rapidly become
exhausted, lose weight, become further
weakened and may eventually become
bedfast. Death frequently occurs from
respiratory failure.
two forms of the disease are now
recognized: one, a steadily progressive
type; the other, a remitting, relapsing type.
109
Focal collections of small lymphocytes,
or lymphorrhages, are found
surrounding small blood vessels in the
interstitial tissue of affected muscles.

Foci of atrophy or necrosis of muscle


fibers have been described. There are
no pathognomonic features however.

110
Physostigmine, an anticholinesterase,
administered intramuscularly, improves
the strength of the affected muscles in a
matter of minutes, although the remission
is only temporary.

111
Myositis refers to an inflammation of
muscle tissue and is entirely nonspecific,
since a great many bacterial, viral, fungal
or parasitic infections, as well as certain
physical and chemical injuries, may give
rise to the condition.
Four specific forms of myositis are:
dermatomyositis; myositis ossificans,
generalized and traumatic; proliferative
myositis; and focal myositis.

112
Dermatomyositis (DM) is an idiopathic
inflammatory myopathy (IIM) with
characteristic cutaneous findings.
Four of the five criteria are related to the
muscle disease, and are as follows:
progressive proximal symmetrical
weakness, elevated muscle enzymes, an
abnormal finding on electromyograph,
and an abnormal finding on muscle
biopsy. The fifth criterion is compatible
cutaneous disease.
113
The cause of DM is unknown. The
pathogenesis of the cutaneous disease is
poorly understood.

DM is probably caused by complement-


mediated (terminal attack complex)
vascular inflammation, while PM is caused
by the direct cytotoxic effect of CD8+
lymphocytes on muscle.

114
Dermatomyositis may occur in patients of
any age from very young children to elderly
adults, but the majority of cases occur in
the fifth decade of life.
There is no sex predilection in its
occurrence.
The more acute form of the disease, seen
more commonly in children, begins with an
erythematous skin eruption, edema,
tenderness, swelling and weakness of the
proximal muscles of the limbs.
115
Accompanying these manifestations are
fever and leukocytosis.
The skin lesions frequently calcify and form
calcium carbonate nodules with a foreign
body reaction.This is known as calcinosis
cutis, whereas the term calcinosis
universalis is applied when these calcified
masses are found generalized throughout
the soft tissues.
The chronic form of the disease is similar,
but may not show dermal involvement
(polymyositis only), although all gradations
are present between the two extremes.
116
In addition, Raynauds phenomenon or
paroxysmal digital cyanosis may be an
early manifestation.
The muscular stiffness and weakness are
often symmetric in distribution.
The cutaneous lesions usually consist of a
diffuse erythema with desquamation,
although other types of rashes have been
described. This rash is most frequently seen
on the face, eyelids, ears, anterior neck
and overlying articulations.

117
The oral lesions, consisting of diffuse
stomatitis and pharyngitis, are extremely
common.
Telangiectatic lesions of the vermilion
border of the lips and cheeks may also
occur.
In addition, involvement of the muscles of
the jaws, tongue and pharynx may pose
problems in eating and phonation.

118
The muscle fibers in dermatomyositis exhibit
widespread degeneration and
hyalinization.
In advanced cases the muscle fibers
disappear, leaving only the fibrous stroma.
Many fibers show vacuolization,
granulation and fragmentation with
phagocytosis of disintegrating fibers.
Diffuse leukocytic infiltration is also
frequently pronounced.
119
There is no specific treatment for the
disease, although symptomatic
treatment may be of considerable
benefit to the patient.
In the more acute forms of the disease,
death may occur rapidly.
In other cases there may be recovery,
sometimes with a residual disability.

120