neuromuscular diseases power point

1. Neurological diseases

2. Multiple sclerosis
 It is characterized by multiple areas of CNS white matter inflammation,
demyelination, and gliosis.
 Women (2:1)
 White people
 4th
decade of life (20-40y)
Symptoms
 Impairment of vision,
 Muscular incoordination,
 Bladder dysfunction.

3. Etiology
 Presence of activated T lymphocytes and autoantibodies to
glycoproteins.
 Genetic – HLA DR 15, DQ 6, Dw2, MHC 6p21
 Environmental factors-
Viral & Bacterial infections
Antibody titers against measles, rubella, mumps, EB, HSV 1 &
2, & H HV- 6 found in the CSF & serum.
 Most common in northern Europe, Canada, and New Zealand.

4. Types
 RRMS- relapsing remitting MS
 SPMS- Secondary progressive MS
 PPMS- Progressive relapsing MS

5. Clinical Manifestations
 Depend on the site of the demyelinating lesion.
Visual disturbances
 Loss of vision - 2nd
cranial nerve
 Color blindness, diplopia, nystagmus, blurring, visual field
defects - 3, 4, 6 cranial nerves.
 Limb weakness, gait disturbances

6.  Uhthoff’s sign-
Rapid vision loss following a body temperature increase that is
associated with exhausting exercise
 Marcus Gunn’s pupillary sign-
A bright light is shone into each eye separately; when this light
is moved from the normal to the affected eye, the pupil of the
latter dilates rather than constricts.
 Lhermitte’s symptom
Electric shock like sensations that are evoked by neck flexion
and radiate down the back and into the legs

7.  Weakness or paresthesia of the extremities
 Increase in the deep tendon reflexes
 These symptoms may remit for long periods and then suddenly reverse,
leading to paraplegia.
Other signs
 Bladder dysfunction,
 Euphoria,
 Ataxia,
 Vertigo, and
 Generalized incoordination.
 Spasticity associated with painful muscle spasm.
 Chronic and are characterized by exacerbations and remissions over a
period of many years
 Patients may have a normal life span

8. Diagnosis
 Clinical findings of the patient,
 Neurologic signs
 History of exacerbations and remissions
 Demyelinating changes can be seen on MRI in more than 90%
of cases ( Hypointense areas)
 Increased IgG, TNF in the CSF

9. Treatment
 Use of carbamazepine, baclofen, gabapentin, or Phenytoin.
 High doses of IV corticosteroids may arrest the progress
 Long-term treatment with immunosuppressants may reduce the
frequency of relapse in patients with MS
 Azathioprin
 Methotrexate
 Interferon-γ-1b and -1 alpha

10. Nonpharmacologic measures
 Total lymphoid irradiation,
 Plasmapheresis,
 Immunoglobulin therapy
 Thermocoagulation,
 Surgical sectioning of the nerve
 Alcohol injection

11. Oral health considerations
 TGN is present in about 2% of cases of MS
 Symptoms are commonly Bilateral
 Pain is severe and lancinating, but trigger zones may be
absent.
 Pain often becomes less severe but more continuous
 Burning, tingling, reduced sensation
 Numbness of the chin, lower lip- neuropathy of the mental
nerve
 Myokymia

12.  Facial weakness and peralysis
 Dysarthria
 Avoid elective dental treatment
 Evaluate the level of the motor dysfunction
 Under GA
 Electric tooth brush

13. Myasthenia gravis

14. Characterized by
 Progressive skeletal muscular weakness on exertion
 Autoantibodies that combine with and may destroy the
acetylcholine receptor sites at the neuromuscular junction.
 Preventing the transmission of nerve impulses to the muscle.
 Thymoma
 Association with other diseases,
( Pemphigus, Pemphigoid, SLE, and RA.)
 Women
 2nd
-3rd
decades of life- women
 7th
-8th
decades of life- men.

15. Clinical Manifestations
 Initial signs - Eye muscles- Ptosis, Diplopia,
 Difficulty in chewing or swallowing,
 Respiratory difficulties,
 Limb weakness
 Usually best in the morning and worse as the day progress
 Exacerbations and remissions occur frequently.
 Respiratory difficulty arises.
 Weakness in the facial muscle expression, masticatory muscles,
muscles used for swallowing and speech

16. Diagnosis
 On the basis of clinical presentation.
 Inability to continually blink the eyes voluntarily
 Administration of a short-acting anticholinesterase; it will
antagonize the effect of cholinesterase on acetylcholine,
 Detecting the antiacetylcholine receptor antibody for
confirmation of diagnosis.

17. Treatment
 Anticholinesterase drugs such as neostigmine and
pyridostigmine bromide
 Thymectomy.
 Long-term corticosteroids and immunosuppressive drugs
 Plasmapheresis.
 IV immunoglobulins

18. Oral Health Considerations
 Facial muscles are commonly involved
 Patient an immobile and expressionless appearance
 Tongue edema- making eating difficult
 Difficulty with prolonged opening and swallowing presents
challenges to the dental treatments
 Difficulty in chewing can affect diet, and the design prosthesis

19.  Dental treatment should be performed in a hospital where
endotracheal intubation can be performed
 Avoiding drugs - Narcotics, Tranquilizers And Barbiturates
Tetracyclins, Sulfonamides, Clindamycin

20. Muscular dystrophy

21.  It is a genetic disease characterized by muscle atrophy
that causes severe progressive weakness.
 Enzymatic dysfunction at the muscle surface membrane.

22. Classification
According to mode of inheritance, age at onset, and C/F
 Duchenne’s muscular dystrophy
 Becker’s muscular dystrophy
 Facio scapulo humeral dystrophy
 Limb-girdle dystrophy
 Oculo pharyngeal muscular dystrophy
 Myotonic dystrophy

23. Duchenne’s muscular dystrophy
 Most common form
 Young males
 Mutation of the dystrophin gene
 Begin during the first 3 years of life
Early signs
 Difficulty in walking, frequent falling,
 Inability to run.

24. Symptoms
 Initially, the muscles may appear even larger than normal,
primarily because of the fat deposition in the muscles.
 Pseudo hypertrophy
 Intellectual retardation
 Skeletal deformities,
 Muscle contractures,
 Cardiac involvement.
 Serum levels of Creatine Phosphokinase are elevated.

25.  At the end of the 1st
decade of life, the child will be unable to
walk and will be bedridden.
 Respiratory muscles will begin to be affected
 Most patients die in the late teenage years or early twenties.
 The muscles of the pelvis and femoral region -affected
 Muscles of the face, head, and neck are not involved.

26. Becker’s muscular dystrophy
 Between 5 - 25
 Patients may have a normal life span.
Facio scapulo humeral dystrophy
 Autosomal dominant trait
 Affects both males and females.
 Symptoms do not usually begin until the 2nd
decade of life.
 Muscles of the face and pectoral girdle are severely involved,
 Exhibit weakness of the arms,
 Winging of the scapulae, and
 Weakness of the muscles of the eyes and mouth.

27. Limb-girdle dystrophy
 Autosomal recessive trait;
 It affects both sexes
 Onset in the 2nd
-3rd
decades of life.
 The weakness starts in either the shoulders or the pelvis
 But will eventually spread to both.
 Facial muscles are not involved.

28. Oculopharyngeal muscular dystrophy
 Autosomal dominant trait
 Characterized by the late onset of ptosis and dsyphagia.
 Symptoms may begin at any age
 Progressive weakness of levator palpebrae and chronic
contraction of the frontalis muscle.
 The patient will maintain a chin-up head position
 Difficulty in swallowing solid food initially and liquids later.

29. Myotonic dystrophy
 Autosomal dominant trait
 From birth to the age of 40 years,
 Progressive muscular weakness, myotonia, cataracts, cardiac
abnormalities, hypogonadism, and frontal balding.
 My occurs in the muscles of the head and neck and in the distal
extremities.
 Unable to relax the muscles after contraction.
 Observed in the forearm, thumb, and tongue.
 Wasting of muscles and subsequent weakness
 Prolapsed mitral valve and atrial flutter;

30. Treatment
 All forms of MD are incurable, and no satisfactory method
of retarding the muscle atrophy exists.
 Corticosteroids have been shown to decrease the rate of
muscle loss, but only in the short term.
 A physical therapy program will help to delay the
development of joint contractures
 Orthopedic procedures may help to counteract deformities.

31. Oral Health Considerations
Facio scapulo humeral
Myotonic forms of MD.
 Difficulty in the ability to turn the head.
 The muscles of facial expression and mastication are also
commonly affected.
 The patient has difficulty in chewing or in pursing the lips.
 Weakness of the facial muscles and enlargement of the
tongue due to fatty deposits.

32. Oculopharyngeal form
 Difficulty in swallowing.
 Occlusal abnormalities
 Lack of the proper muscle tension necessary to keep the teeth
properly aligned in the dental arch.
 If the tongue is enlarged and the facial muscles are weak, the
teeth will be pushed out.
 Macroglossia,
 Anterior open bite,
 TMJ dysfunction.

33. Guillain-barré syndrome
(Acute idiopathic polyneuropathy)
 Acute symmetrical polyneuropathy, often occurring 1 to 3
weeks after an acute infection.
 Often follows a nonspecific respiratory or GIT illness and
specific infections (CMV, EBV, and after immunization).

34. Clinical Manifestations
 Begins with myalgia or paresthesias of the lower limbs,
followed by weakness
 Involve abdominal, thoracic, and upper-limb muscles.
 Respiration is compromised.
 ANS - may induce changes in BP and pulse rate.

35.  Impaired swallowing or paresthesias of the mouth and
face
 7th
cranial nerve is frequently involved, and bilateral
facial weakness is common.
 Ptosis
 Dysarthria, dysphagia and diplopia

36. Treatment
 Prednisone is ineffective and may actually affect the
outcome adversely by prolonging recovery time
 Plasmapheresis performed within the first few days of
illness.

37. Epilepsy
 Epilepsy is a condition characterized by abnormal,
recurrent, and excessive neuronal discharge precipitated
by many different disturbances within the CNS.

38. Oral Health Considerations
Medical history
 What type of seizures the patient has,
 How well the seizures are controlled,
 The frequency and duration of seizures,
 The potential triggers for seizures, and
 What to expect if the patient has a seizure.

39.  Treatment planning may be altered
 Depending on the status of the seizure disorder.
 Better to place a FPD rather than a removable appliance.
 Gingival overgrowth. Phenytoin
 Increase in the number of fibroblasts in the connective tissues.
 Younger patients
 Men and women are equally affected.
 Does not correlation between dosage and the incidence of gingival
overgrowth.
 Strong correlation between poor oral hygiene and the amount of
tissue

40.  Starts in the interdental papillae and occurs only where teeth are
present.
 The papillae enlarge buccally and lingually.
 The enlarged areas are firm, pink, and covered with normal mucosa.
Treatment
 Begins with prevention.
 Careful oral hygiene can minimize the gingival enlargement.
 Each patient should be referred to a dentist for oral hygiene
instruction and gingival curettage.
 Gingivectomy.
 Curettage

41. Other side effects
 Megaloblastic anemia,
 Hirsutism, and
 Lymphadenopathy.
 Osteomalacia,
 Thickening of the calvarium, and coarse facies
 Patients with well-controlled epilepsy may be performed with
no change from normal treatment.

42. Bell’s palsy
 Unilateral paresis of the facial nerve.
 Inflammatory reaction involving the facial nerve.
 Herpes simplex virus 1
 Bell’s palsy must be differentiated from other causes of facial
nerve palsy,
lyme disease,
Ramsay Hunt Syndrome
Acoustic neuromas.

43. Clinical Manifestations
 Begins with slight pain around one ear, followed by an
abrupt paralysis of the muscles on that side of the face.
 Eye on the affected side stays open,
 The corner of the mouth drops,
 Drooling.
 Masseter weakness,
 Food is retained in both the upper and lower buccal and
labial folds.

44.  The facial expression changes remarkably,
 The creases of the forehead are flattened.
 Due to impaired blinking, corneal ulcerations from foreign
bodies can occur.
 Involvement of the chorda tympani nerve leads to loss of
taste perception on the anterior two-thirds of the tongue.
 Reduced salivary secretion

45. Treatment
 Systemic corticosteroids within the first few days after the onset
of paralysis,
 Combining steroids with antiherpetic drugs
 Protect the eye with lubricating drops or ointment
 Facial plastic surgery
 Anastomosis between the facial and hypoglossal nerves -
restore partial function.