DISEASES OF

MUSCLES&NERVES
Nitika Malhotra
3rd yr
47
 CONTENTS
• INTRODUCTION
• TRIGEMINAL NEURALGIA
• PARATRIGEMINAL SYNDROME
• SPENOPALATINE NEURALGIA
• BURNING MOUTH SYNDROME
• OROLINGUAL PARESTHESIA
• AURICULOTEMPORAL SYNDROME
 INTRODUCTION
Pain in many cases is directly associated with teeth,
others arise from diseases of nerves themselves and
are not connected with teeth.

A complete assessment of disorders affecting the

nerve pathways and nerve supply of various
anatomic sites associated with oral cavity are
essential to determine the true nature of pain and
take appropriate measures to effect its relief.
 TRIGEMINAL NEURALGIA

• It is an archetype of orofacial neuralgia, which

follows the anatomical distribution of 5th
cranial nerve.
• It mainly affects the 2nd&3rd divisions of
trigeminal nerve and always exhibits a trigger
zone, stimulation of which initiates paroxysm of
pain.
• Pain distribution is unilateral and lasts for a few
seconds to a minute.
 ETIOLOGY

• It is most likely not dental in origin.

• Periodontal diseases &traumatogenic occlusion have
also been suggested as causes.
• In many cases, no organic lesion is identified& hence
etiology is labeled “idiopathic”.
• Infrequently, adjacent dental fillings composed of
dissimilar metals may trigger attacks(galvanism)
 CLINICAL FEATURES
• Old people are more commonly affected than young people.
• F>M
• F:M=3:2
• Right side of the face is more affected than left side. L:R=1.7:1
• The pain is : searing, stabbing and lancinating.
• In early stages; pain is mild, but with time it becomes severe. This
early pain is termed as “Pre trigeminal neuralgia”and is dull,
aching or burning as a sharp tooth ache.
• The trigger zones which precipitate an attack when touched, are
common on:
• Vermillion border of lip
• Alae of nose
• Cheeks
• Around the eyes
 DIFFERENTIAL DIAGNOSIS
• Migraine or migranious neuralgia
• Sinusitis
• Tumors of nasopharynx
• Trigeminal neuritis or neuropathy:
It differs from neuralgia bcoz it is most often
defined as an ache, variously stated as a
burning,boring,pulling,drawing or pressure
sensation.
 LAB FINDINGS & TREATMENT
• Elective MRI for all patients to exclude an
uncommon space occupying lesion or vessel
compression on nerve roots.
• Peripheral neurectomy is done
• Injection of alcohol into a peripheral nerve area
or in gasserian ganglion.
• Phenytoin(dilantin) is effective.
• Carbamazipine is often used.
• Microsurgical decompression of trigeminal nerve
is the newest procedure
 PARATRIGEMINAL SYNDROME
- It is a disease characterized by severe headache or pain in
the area of trigeminal distribution, with signs of occular
sympathetic paralysis.

CLINICAL FEATURES/DIFFERENTIAL DIAGNOSIS:

M>F
More common in middle age
Homolateral pain in head or eyes
It presents with some signs of “Horner’s
syndrome”bt can be differentiate from it by
the presence of pain &little or no change in
sweating activities on the affected side.
 SPHENOPALATINE NEURALGIA
• It is a pain syndrome, originally described by SLUDER as a
symptom complex referable to the nasal ganglion.
• Acc to VIAL a similar syndrome, also involved the vidian
nerve & hence called it as VIDIAN NEURALGIA.

ETIOLOGY:
 An idiopathic syndrome
 Its periodicity has been attributed to hypothalamic
hormonal influences.
 Genetic predisposition may exist in some individuals.
 CLINICAL FEATURES:
 M>F (5:1) <40 yrs
 Unilateral paroxysm of pain in region of
eyes,maxilla,ears,mastoid,base of nose beneath zygoma.
 Pain has a rapid onset, persists for about 15 min to several hours
& then disappears
 The attacks develop regularly,atleast once a day& in some
patients the onset occurs at the same time of the day hence it is
referred to as ALARM CLOCK.

TREATMENT:
 Cocainization of sphenopalatine ganglion or alcohol injection.
 Resection of ganglion.
 Surgical correction of septal defects.
 Ergotamine &antiserotonin produce synergistic effects.
 BURNING MOUTH SYNDROME
• It is a burning or stinging of the mucosa,lips,tongue in the absence of
visible mucosal lesions.
• F>M
• Age of onset: approx 50 yrs

CLINICAL FEATURES:
 Burning sensation may be felt either as a continuous or intermittent
discomfort.
 Onset of symptoms may be sudden or gradual over months.
 Dry mouth leading to increased thirst
 Altered taste sensation or persistent unusual taste.
 Anxiety or depression.
 ETIOLOGY
Local causes:
 Dry mouth
 Mucosal disorders
 Trauma
 Oral habits(such as tongue thrusting)
 Gastro esophageal reflux disease
 Sensory nerve damage
SYSTEMIC CAUSES:
 Vit B12,folate,fe deficiencies
 Medications (e.g. ACE inhibitors)
 Immunologically mediated diseases
 Psychogenic disorders
 Psychosocial stress
 Diabetes mellitus
 Menopause
 TREATMENT
• Anti depressants
• Vitamins
• Dietary supplements such as Benzydamine
hydrochloride
• Post menopausal patients, hormone replacement or
topical estrogen applied to oral mucosa.
• When dry mouth is a prominent symptom then
saliva substitutes may be considered.
 OROLINGUAL PARESTHESIA
• It is a symptom rather than a disease.
• It is also known as BURNING TONGUE or glossopyrosis.

ETIOLOGY:
Deficiency states like pernicious anemia,
Diabetes,Gastric disturbances, Psychogenic factors,
Trigeminal neuralgia,Xerostomia,periodontal diseases
CLINICAL FEATURES:
Tongue is the most common site of
parasthetic sensations
Pain,burning,itching & stinging of mucous
membrane
Occurs frequently in women past the
menopause.
TREATMENT:
Topical anesthetics,
analgesics,sedatives,antibacterials and
anti-fungal.
 AURICULOTEMPORAL SYNDROME
• It is an unusual phenomena, arises as a result of damage to
auriculotemporal nerve & subsequent reinnervation of sweat glands
by parasympathetic salivary fibers.

ETIOLOGY: surgical removal of a parotid tumor or ramus of

mandible that has damaged the auriculo temporal nerve
CLINICAL FEATURES:
 Sweating during eating, which may be evoked by parentral
administration of pilocarpine.
 Crocodile tears are seen in which there is profuse lacrimation on
eating.
TREATMENT:
Intra cranial division of auriculotemporal nerve has been successful.
DISTURBANCES OF SEVENTH CRANIAL NERVE
BELL’S PALSY
(SEVENTH NERVE PARALYSIS, FACIAL PARALYSIS)

• It is one of the most common neurologic disorder affecting the cranial nerves.
• It is an abrupt, isolated, unilateral, peripheral facial nerve paralysis without
detectable causes.
• Idiopathic facial paralysis was 1st described more than a century ago by Sir
Charles Bell, however much controversy still surrounds its etiology and
management.
• It is important to keep in mind that Bell’s Palsy is diagnosis of exclusion.
ETIOLOGY
• Idiopathic facial paralysis.

• Herpes Simplex Virus(HSV).

• A popular theory proposes that the inflammation of the facial

nerve with resultant edema causes nerve compression while it
passes through the temporal bone.

• Various inflammatory, demyelinating, ischemic or

compressive processes may impair neural conduction at this
unique anatomic site.
CLINICAL FEATURES
• Paralysis of the facial musculature, usually unilaterally.

• Familial occurrence seen in cases of Burzynski and Weisskopf.

• Women are affected more commonly than men.

•The middle-aged are most susceptible.

• The disease arises more frequently in the spring and fall than at other times of
the year.

• It may develop within a few hours or be present when the patients awakens in
the morning.

• Preceded by pain on the side of the face particularly within the ear, in the
temple or mastoid areas or at the angle of the jaw.
•The pt. has a typical masklike or expressionless appearance.

• Speech and eating usually become difficult and occasionally the taste
sensation on the anterior portion of the tongue is lost or altered.

•Recurrent attacks of facial paralysis, indentically with Bell’s Palsy, associated

with multiple episodes of nonpitting, non inflammatory painless edema of the
face, cheilitis granulomatosa and fissured tongue or lingua plicata is known as
the Melkersson-Rosenthal Syndrome.
 • The facial edema resembles angioneurotic edema and involves the upper lip,
occasionally the lower, and sometimes the nose, tongue or maxillary alveolar
process.

• The fissured or scrotal tongue has been reported in about 25-40% of cases.

TREATMENT
•No specific treatment for Bell’s Palsy, since the etiology of the diseases is
unknown.

• The use of vasodilator drugs, eg. Histamine, has proved beneficial in some
• cases.

• Administration of physiologic flushing doses of nicotinic acid has produced

excellent results.
• In permanent paralysis surgical anastomosis of nerves.
DISTURBANCES OF 9TH CRANIAL NERVES
GLOSSOPHARYNGEAL NEURALGIA

Pain is similar to that of trigeminal neuralgia, may arise from the

glossopharyngeal nerve.
This condition is not as common as trigeminal neuralgia, but when it occurs, the
pain may be as severe and excruciating.
The condition has been reviewed by Bohm and Strang.

CLINICAL FEATURES
• No gender predilection.

• Manifested as sharp, shooting pain in the ear, pharynx, nasopharynx, the tonsil
or the posterior portionof the tongue.

• It is unilateral and the paroxysmal, rapidly subsiding type of pain.

• The pt. usually has a ‘trigger zone’ in the posterior oropharynx or tonsillar
fossa.
• Neural ischemia has been suggested but without conclusive evidence.
• Etiology unknown.
TREATMENT
• The treatment of glossopharyngeal neuralgia has generally consisted in
resection of the extracranial portion of the nerve or intracranial section.
• The injection of alcohol into the glossopharyngeal nerve has not been as
widely accepted as has similar treatment in case of trigeminal neuralgia.
• Periods of remission with subsequent recurrence are common in this
disease.
MISCELLANEOUS DISTURBANCES OF NERVES
MOTOR SYSTEM DISEASE
(MOTOR NEURON DISEASE, AMYOTROPHIES)

• Constitutes a group of closely related conditions of unknown etiology which

occur in 3 clinically variant forms usually referred to as progressive bulbar
palsy.

• These are called the motor system disease , since they all manifest
corticospinal and anterior horn cell degeneration and exhibit either
bulbar(tongue,pharyngeal,laryngeal) or limb muscle involvement.
CLINICAL FEATURES
• Progressive muscular atrophy
- progressive weakness of the limbs with associated muscular
atrophy, reflex loss and sensory disturbances.
- M>F, tends to occur in childhood.
- Initial symptoms consists of difficulty in walking, with leg pain
and paresthesia.
• Amyotrophic lateral sclerosis
- Age: 40-50 yrs
- M>F
- Precipitating factors are fatigue , alcohol intoxication, trauma
and certain infections.
- Initial symptoms consists of weakness and spasticity of the
limbs, difficulty in swallowing and talking.
- Atrophy and fasciculations of the tongue with impairment or
loss of palatal movements may also occur.
Progressive bulbar palsy
- Characterised by difficulties in swallowing and phonation, hoarseness, facial
weakness and weakness of mastication.
- 5th- 6th decade of life.
- Initial symptoms are gradual in onset and consists of difficulty in articulation,
with impairment and finally loss of swallowing

.
- Chewing is difficult as the facial muscles becomes weakened.
- These pts. exhibit atrophy of the face, masseter and temporal
muscles and tongue with fasciculations of the face and tongue.
- There is also impairment of the palate and vocal cords.

Pseudobulbar Palsy
Treatment and prognosis
- No specific treatment required.
- Disease is fatal, although temporary remissions
sometimes occur.
MULTIPLE SCLEROSIS
( Disseminated sclerosis)

- It is an idiopathic inflammator demyelinating ds. Of the

CNS.
- Pts. Commonly present with an individual mixture of
neurological dysfunction, which tends to progress over
years.
ETIOLOGY

• Results from an autoimmune process.

• It is the non random nature of its geographic

distribution that suggests an isolated or additive
environment effect and/or inadvertent activation
and dysregulation of immune processes by a
retroviral infection that was perhaps acquired in
childhood.
• On the basis of research findings, some authorities implicate
human herpes virus-6, while others implicate Chlamydia
pneumoniae as causative agents.
• Polygenic inheritance accounts for a familial rate of 10-20%.
• Most studies confirm that amonozygotic twin has onl 30%
risk of acquiring this ds. , suggesting the contributory role of
environmental agents to genetic predisposition in the
causation of this disorder.
CLINICAL FEATURES

• Rarely occurs in those younger than 20 yrs or those older than 50 yrs.

• Onset of symptoms is most frequently seen between the ages of 20 and 40 yrs.

• F>M , 2:1
• The disease is characterised by:
- A variety of ocular disturbances , including visual impairment
as a manifestation of retrobulbarneuritis , 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 and/or
rectal retention or incontinence.
=> Charcot’s triad is a well-known diagnostic triad characteristic of
multiple sclerosis but not invariably present.
- It consists of intention tremor,nystagmus and dysarthria or
scanning speech, an imperfect speech articulation.
=>Facial and jaw weakness occurs in some pts. , and staccato type
of speech has been described.
- In addition, both Bell’s palsy and trigeminal neuralgia have been
reported in some pts. With multiple sclerosis, but these are not
common and the findings may be fortuitous.
• TREATMENT

• There is no treatment for multiple sclerosis.

• Although remissions of the disease frequently occurs in
patients usually following an ingravescent course leading to
death, often from supervening infection.
OROFACIAL DYSKINESIA

43
 Extra pyramidal disorder
or
A complication of phenothiazine therapy

 Sutcher & his associates

have reported a group of edentulous patient wearing full upper &

lower dentures

suffering from this disorder

44
 CLINICAL FEATURES

• Most common age:- 60 yrs.

• Severe, involuntary, dystonic movements of facial, oral & cervical

musculature.

• Lip licking & protrusion of lips as in pouting, protusion of tongue are all
typical manifestations.

TREATMENT
 Surgical operation
 correction of denture occlusion
45
MENIERE’S DISEASE

46
• Also known as endolymphatic hydrops, is an inner ear disorder.

• Increase in volume & pressure of the endolymph of the inner ear.

ETIOLOGY

 Unknown.

 Current theory have been found that it is associated

with injury.
47
 CLINICAL FEATURES

• Deafness
• Tinnitus beginning in middle age
• Vertigo
& if untreated, remission.

TREATMENT
 No treatment is wholly effective.
 But some patient reacts to VASODILATORS SUCH AS:- HISTAMINE OR
NIACIN.
 When conservative treatment fails:- surgical intervention. (labyrinthotomy)

48
MIGRAINE

49
 Its term applied to certain headache with a vascular quality.
DOMINANTLY INHERITED DISORDER.

 Characterized by recurrent vascular headache, photophobia, sleep

disruption,depression.

Etiology:-
 Not completly understood.
 Atleast 14 different types of migrane exist

 They are broadly classified into two groups:-

• Migrane with aura
• Migrane without aura
50
 COMMON TRIGGERS FOR MIGRANE HEADACHE

Types of trigger subtypes

• Hormonal • Mensturation
• Ovulation
• Oral contraceptives
• Dietary • Alcohol
• Chocolate
• Aged cheese
• Missing a meal
• Psychologic • Stress / poststress
• Anxiety /worry
• depression

51
• Physical/environmental • Glare
• Flashing lights
• Fluorescent lights
• Odors
• Weather changes
• High altitude
• Sleep related • Lack of sleep
• Excessive sleep
• Drugs • Nitroglycerine
• Histamine
• Reserpine
• Estrogen
• miscellanous • Head trauma
52
• fatique
 CLINICAL FEATURES
 2nd decade of life.

 commonly in professional persons.

 women more than men.

 Prodromal stage:- patient having lethargy,

hallucinations, vertigo.

 Headache phase consists of severe pain in the temporal, frontal &

retro-orbital areas.

 Pain is usually deep, aching, throbbing type.

 Patient may appear extremly ill at the time of headache.
 Face is usually pale, sallow & sweaty. 53
 TREATMENT
 Wide variety of drug ranging from acetylsalicylic acid & codeine to
ergotamine, norepinephrine.

 Prognosis is good.

54
TEMPORAL / GIANT CELL
ARTERITIS

55
 Is a cause of headache which is frequently diagnosed as ‘ atypical
migraine’.

 Uncommon condition.

 A focal granulomatous inflammation of arteries especially the cranial

vessels, in severe cases arteries throughout the body may be involved.

Etiology:-
 Primarily a disease of cellular immunity.

 The vasculitic damage is mediated by activated CD4+ T helper cells

responding to an antigen presented by macrophages.
56
 CLINICAL FEATURES

Older persons.
Between the ages of 55-85 yrs.
Affect women more.
Onset is slow & insidious.
Sudden headache or a burning, throbbing type of pain.

TREATMENT
CORTICOSTEROID THERAPY:-subsides the problem.

57
COMPLEX REGIONAL PAIN
SYNDROME (CAUSALGIA)

58
 Severe pain which arises after injury to a sectioning of a peripheral sensory
nerve.

Etiology:-

 Triad of conditions An injury,

An abnormal sympathetic response,
& a predisposing personality.

59
 CLINICAL FEATURES

 Any age.
 Extraction of multirooted tooth, particularly when the extraction is
difficult or traumatic is the main cause.
 Pain arises within a few days to several weeks after extraction & has a
typical burning quality.

TREATMENT

 Injection of procaine, alcohol nerve block, phenol cauterization &

surgical currecttment of the bone in the involved area is affective.

60
ATYPICAL FACIAL PAIN (FACIAL
CAUSALGIA)

61
 Constitutes group of conditions in which there is a vague, deep,
poorly localized pain in the regions.

 Supplied by 5th & 8th cranial nerves & the 2nd &3rd cranial nerves.

Etiology:-

 Injury of any peripheral or proximal branch of the trigeminal nerve due

to facial trauma or basal skull fracture can produce the disorder.

Treatment:-

 Non- narcotic drugs,

Tricyclic antidepressants best result. 62

HORNER’S SYNDROME

63
 Condition characterized by:-

• Miosis, or contraction of the pupil of the eye due to paresis of the

dilator of the pupil.

• Ptosis, or dropping of the eyelid due to paresis of the smooth

muscle elevator of the upper lid.

• Anhidrosis & vasodilatation over the face due to interruption of

sudomotor & vasomotor control.

• It indicates the presence of primary disease.

• Lesions in the brainstem, chiefly tumors or infections or in the

cervical or high thoracic cord will produce this syndrome. 64
MARCUS GUNN JAW-WINKING
SYNDROME

65
 Consist of congenital unilateral ptosis, with rapid elevation of the ptotic
eyelid occuring on movement of mandible to the contralateral side.

 Most commonly in infants.

 Males are commonly affected.

 Left upper eyelid is involved most commonly.

 Some of cases are hereditary, can also follow an injury or disease.

66
 INTRODUCTION
 Diseases of the skeletal muscles of the
face and oral cavity occur with sufficient
frequency that it comes in consideration
of dentist.
 The facial or oral manifestation
represent a major feature of disease.
 Special practices of orthodontics
prosthodontics and periodontics among
are especially allied to solve the problem
created by muscle disease.
 CLASSIFICATION OF
DISEASES OF MUSCLE
1. Primary myopathies limited to or predominant
in muscle
 Dystrophies
 Mytonias (dystrophic, congenital, acquired)
 Hypotonias
 Myasthenias
 Myositis (including dermatomyositis and
myositis ossificans
 Metabolic defects (Glycolytic, Myoglobinuria)
 Miscellaneous (Amyoplasia, contractures,
degenerations)
2. Secondary myopathies representing
muscular reaction to primarily
extramuscular disease
A. Atrophy (traumatic neuropathic,
secondary to metabolic,vascular
nutritional, infection and toxic processes)
1- Denervation
2- Disuse and fixation
3- Aging and cachexia
B. Hypertrophy
1- Developmental
2- Functional
C. Endocrime
D. Internal environment
1. Chemical
2. vascular
E. Infection
1. Specific (trichinella, Toxoplasma,
coxsackie virus)
2. General (rickettsial, typhoid,
pneumococcal pneumonia)
3. Postinfectious asthemia.
 DYSTROPHIES
Muscular dystrophy is a primary
progressively degenerative disease of
skeletal muscle.
Important forms of muscular dystrophy
are : -
 Severe generalized familial muscular
dystrophy.
 Mild restricted muscular dystrophy.
 Myotonic dystrophy.
 Ophthalmoplegic dystrophy.
 Late distal muscular dystrophy.
 SEVERE GENERALIZED FAMILIAL MUSCULAR
DYSTROPHY

(PSEUDOHYPERTROPHIC MUSCULAR DYSTROPHY OF DUCHEME)

Introduction : -
Most common form
Rapidly progressive muscle diseases.
Beginning in early childhood.
Occurring predominantly is males.
With are without pseudohypertrophy.
Clinical features
Begins in childhood
Usually before the age of six year and rarely
after 15 years.
Sign : -
 Inability to walk are run.
 Children falling readily
 With muscular enlargement and weakness
 Muscular enlargement ultimately proceeds to
atrophy.
 Muscle of mastication, facial and ocular
muscles and laryngeal and pharyngeal muscular
are also involved.
Histologic feature
 Gradual disappearance of muscle fibers as the
diseases progresses.
 Ultimately no fibers may be recognized.
Laboratory findings
Serum creatine phosphokinase level is
elevated in all males.
70% the females are carriers.
Tt :
No treatment
Disease remains incurable
 MILD RESTRICTED MUSCULAR DYSTROPHY
(Facioscapulohumeral dystrophy of Landouzy
and Dejerine)
Introduction
Slowly progressive proximal myopathy.
Involves the muscles of the shoulder and
face.
Frequently presents long remission and
sometimes complete arrest.
Etiology
Autosonal dominant diseases in 70-90% of
patients.
Causative genes has been localized to
chromsomes band 4q35.
c/f:
Begins at any age
 From 2-60 yrs
Mazority of cases is in the first two decades
of life.
Frequency of occurrence is higher in males.
Signs :- Inability to raise the arms a bone the
head and inability to close the eyes even during
sleep as a result of weakness of facial muscles.
Lips develop looseness and protrusion has
been described as tapair lips
Pt. are unable whistle or smile.
H/f : No specific microscopic findings are found
Tt : No treatment.
 MYOTONIA
Myotonias is a failure of muscle relaxation
after cessation of voluntary contraction occurs
in 3 forms.
Dystrophic myotonias
Congenital myotonias
Acquired myotonias
 DYSTROPHIC MYOTONIAS
(Myotonic dystrophy Dystrophia
Myotonica)
Introduction
 Progressive, familial, distal myopathy.
 With associates as weakness of the muscles of the
face, jaw and neck and levators of the eyelids.
 Autosomal dominant characteristic.
c/f : -
 Atrophy of muscle
 Generally manifested first in the muscles of hands and
forearms.
 Does not seen in 3rd decade of life
 Seen in childhood.
 Dystrophic myotonia found in pharyngeal & largneal
muscles exhibits nasal type of voice and subsequent
dysphagia.
 Hypothyroidism with coldness of extrimities
 Slow pulse and loss of hair.
H/F :
Enlargement of scattered muscle
fibers presence of centrally placed
muscle nuclei in long rows.
In advanced muscular atrophy, fibers
appears small and there may be
interstitial fatty infiltration.
Tt & prognosis
No treatment
 CONGENITAL MYOTONIA
(Thomsen’s ds – Myotania Congenita)
Introduction
 An anomaly of myscular contraction.
 Inheritance pattern found in 25% of cases.
 An autosomal dominant trait.
 Incomplete penetrat in some families.
 Muscular bypertrophy.
c/f
 In childhood.
 Severe and affects all skeletal muscular, especially those
of the lower limbs.
 Muscular contraction induces severe painless muscular
spasm.
 Hereculean appearance (Muscles are large).
 Muscles of the thighs forearms and shoulders are
especially affected.
 Spasm of the extraocular muscles.
H/f : Muscle biopsy reveals no alternation
from normal except for hypertrophy of all
muscles fibers.
Tt : - No treatment
 ACQUIRED MYOTONIA
Introduction
Spasm of muscle.
Spasm are more intense than those
occurring in typical myotonia.
If the spasm are intermittent CLONUS
(Myoclonic Contraction)
If the constant  (TRISMUS)
(Myotonic Contraction)
Spasmodic contraction occur.
Spasm which are usually painful may be
transitory or may persist for a period of
several days.
 HEMIFACIAL SPASM
(Facial Myoclonus, Facial Dystonia)
Introduction
It is characterized by repeated rapid
painless irregular, nonrhythmic, uncontrollable,
unilateral contractures of the facial muscles in
adults.
Etiology : Unknown
c/f :
Begins in the periorbital muscles.
It is differentiated from emotional ties
and focal convulsive seizures.
Tt. : No treatment
 PERIODIC PARALYSES
(Paramyotonia)
Heterogeneous group of muscle disease.
c/f : - Cramping, stiffness and weakness
of muscle of the face and neck. Eyelids are
closed and the face assumes a mask like
appearance.
H/f : There are no significant histologic
changes.
Tt : No specific treatment.
 HYPOTONIAS
Reduction or complete absence of tonus in
muscles.
CAUSES OF THESE DISEASE
Congenital ds. Neuromuscular ds.

Diseases Of the central nervous Infantile muscular atrophy



system. (Atonic Diplegia)

Lipiod and glycogen storage ds.
 Infantile muscular dystrophy


Mongolism
 Congenital non progress
myopathy.
Cretinism
 Neonatal myasthenia gravis

c/f :
Occur in infancy
Reduced tendan reflexes and
muscular weakness.
Floppy infant syndrome.
Tt. : No treatment
 From:
PRACHI SINGH
ROLL NO. 51
 CONTENTS
• MYASTHENIAS
Myasthenias gravis
• MYOSITIS
Dermatomyositis
• HETEROTROHIC OSSIFICATION
Myositis ossificans progressiva
Traumatic myositis ossificans
Neurogenic heterotrophic ossifications
• PROLIFERATIVE MYOSITIS
• FOCAL MYOSITIS
• MISCELLANEOUS MYOPATHIES
Congenital facial diplegia
Atropy of muscle
Hypertrophy of muscle.
 MYASTHENIAS
• Myasthenias is an abnormal weakness and
fatigue in muscle following activity.
 MYASTHENIA GRAVIS

Definition
Myasthenia gravis is an acquired
autoimmunity disorder characterised clinically
by weakness of skeletal muscles and
fatigability on exertion.
 ETIOLOGY
• Idiopathic
• Autoimmunity
•
 CLINICAL FEATURES
• AGE- It occurs chiefly in adults in the middle- age
group.
• SEX- Predeliction for women.
• SITE- Skeletal muscle, voluntary muscle.
• Rapidly developing weakness in voluntary muscle.
• Minor activity of muscles.
• Difficulty in mastication and deglutition.
• Dropping of the jaw.
• Speech is often slow and sensation.
• Disturbances in taste sensation.
• Dropping of the face.
• The neck muscle may be so weak that the
head cannot be held up without support.
• Lose weight
• Death frequently occurs from respiratory
failure.
 TYPES

• A Steadily progressive type .

• A remitting relapsing type.

 HISTOLOGIC FEATURES
• There is focal collections of small
lymphocytes, or lymphorrhages.
• They are found surrounding small blood
vessels in the intestinal tissue of affected
muscles.
• There are usually no demonstrable
changes in the muscle.
 TREATMENT
• Phyosostigmine, an anticholinesterase
administerd intramuscularly improves the
strength of the affected muscle.
• No cure for the disease is known, even
through the prognosis is good in the relapsing
type.
 MYOSITIS
Myositis refers to an inflammation of muscle
tissue.
DERMATOMYOSITIS
(Juvenile dermatomyositis, childhood
dermatomyositis, polymyositis)

DEFINITION
It is an idiopathic inflammatory myopathy with
characteristic cutaneous findings.
 CLINICAL FEATURES
• Erythematous skin eruption. Weakness of the
proximal muscles of the limbs.
• The skin lesions frequently calcify and form
calcium carbonate nodules with a foreign
body reaction. This is known as calcinosis
cutis.
 HISTOLOGIC FEATURES
• The muscle fibres in dermatomyositis exhibit
widespread degeneration and hyalinization.
• In advanced cases the muscle fibres disappear,
leaving only the fibrous stroma.

TREATMENT
There is no specific treatment, although
symptomatic treatment may be of considerable
benefit to the patient.
 HETEROTOPIC OSSIFICATION
• The term heterotopic ossification describes
bone formation at an abnormal anatomical
site , usually in the soft tissue.
• Strover et al (1975) classify Heterotopic
Ossification.
1- Myositis ossificans progressiva.
2- Traumatic myositis ossificans.
3- Neurogenic heterotopic ossification.
 MYOSITIS OSSIFICANS
PROGRESSIVA

DEFINITION

• It is a disease of unknown etiology which affects

the intestinal tissues of muscles as well tendons,
ligaments, fascia, aponeurosis and even the skin.
 CLINICAL FEATURES
• These are usually painless and are
covered by a reddened skin.
• The patient becomes transformed into a
rigid organism as the petrified man.
 HISTOLOGIC FEATURES
 The muscle in this disease is gradually
replaced by connective tissue which
undergoes osteoid formation and
subsequently ossification.

TREATMENT AND PROGNOSIS

There is no teatment for the disease. It is
progressive disease until death usually from a
pulmonary infection.
 TRAUMATIC MYOSITIS
OSSIFICANS
DEFINITION
• This lesions develops after a single acute
traumatic injury.
CLINICAL FEATURES
• Usuually manifests as a firm, painless
mass in the injured muscle.
• In some cases, motion is limited by
this lesion.
 HISTOLOGIC FEATURES
• It exhibits varying stages from hemorrhage,
degeneration of muscle and connective tissue
hyperplasia to chondrification and ossification.
• The more mature tissue is usually found on the
periphery of the lesion.
• The trabecular pattern is often extremely bizarre.

TREATMENT AND PROGNOSIS

• Surgical excision.
• The prognosis is good, since the lesion is a
localized, inflammatory one.
 NEUROGENIC HETEROTOPIC
OSSIFICATION
• This condition is the one that comes to
mind when the generic phrase
heterotopic ossification is used.
• In 1918, Dejerine and Ceilier first
described heterotopic ossification in
patients with spinal cord injury from the
first world war.
• Now it is recognized as a fairly common
sequela of SCI , especially after traumatic
cord injury.
 PROLIFERATIVE
MYOSITIS
DEFINITION
• It has been defined by Enzinger and Dulcey
as a pseudosarcomatous process of muscle
characterised by an ill- defined
proliferation of basophilic giant cells and
fibroblasts chiefly involving the
pelimysium, epimysium and neighbouring
fascia.
CLINICAL FEATURES
• Lesion is a firm solitary nodule. It is
not attached to overlying skin.
 HISTOLOGIC FEATURES
• A poorly demarcated fibroblastic proliferation
involving the epimysium, perimysium and
endomysium.
• A large, basophilic giant cells are present stromal
tissue are affected.

TERATMENT
• Simple local excision.
• No tendency to recur.
 FOCAL MYOSITIS
DEFINITION
• It is a benign inflammatory pseudo tumour of
skeletal muscle.

CLINICAL FEATURES
• A rapidly enlarging mass within a single
skeletal muscle.
• Some cases are asymptomatic; others are
characterized by a dull, aching pain.
 HISTOLOGIC FEATURES

• There are microscopic changes in the

random muscle fibres.
• Lympocytic infiltration is usually present in
the interstitial tissue.

TREATMENT
• The lesion should be excised.
• It does not recur.
 MISCELLANEOUS MYOPATHIES

CONGENITAL FACIAL DIPLEGIA

(Mobius syndrome)
Definition
Congenital facial diplegia is a non
familial deficient development of cranial
muscles consisting of facial deplegia
with bilateral paralysis of the ocular
muscles, particularly the abducens.
 CLINICAL FEATURES
• It is manifested in infancy during the first few
days of life by failure to close the eyes during
sleep.
• No change in facial expression.
• Mouth remains partially open.
 HISTOLOGIC FEATURES
• There are no conclusive microscopic studies of
muscle in patients with congenital facial
diplegia.

TREATMENT AND PROGNOSIS

• There is no treatment for the disease.

• Prognosis appears to good.
 ATROPHY OF MUSCLE
DEFINITION
• It rerers to decrease in the size of the individual
muscle fibres.
CAUSES
1-Disuse and fixation .
2- Aging and cachexia.
3-Denervation.
4-Muscular dystrophies
5-Nutritional distubances.
6-Infections and toxins.
7-Vascular changes.
8-Metabolic disturbances.
9- Muscular hypotonias
 HYPERTROPHY OF MUSCLE
DEFINITION
It refers to an increase in size of individual muscle
fibres.
CAUSES
1- Developmental defects.
2- Functional disturbances.
3- Neoplasms.

Masseteric hypertrophy occurs in two situations

1- Congenital facial hemihypertrophy.

2- Functional hypertrophy.
 REFERENCES
• Shaffers - VI Edition.
• Neville - 1V Edition.
• www.google.com
• www.yahoo.com