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Neuromuscular Disorders

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Title: Neuromuscular Disorders


1
Neuromuscular Disorders
  • Guillain-Barré Syndrome - Chapter 30
  • Myasthenia Gravis Chapter 31

2
Neuromuscular Diseases
  • Guillain-Barré Syndrome
  • Myasthenia Gravis
  • Amyotrophic Lateral Sclerosis (ALS)
  • Muscular Dystrophy
  • Multiple Sclerosis
  • Cerebral Palsy
  • Spinal cord injury

3
Guillain-Barré Syndrome
4
Definition
  • An acute, usually rapidly progressive form of
    inflammatory polyneuropathy characterized by
    muscular weakness and mild distal sensory loss,
    which in about 2/3 of cases, begins 5 days to 3
    weeks after a banal infectious disorder, surgery,
    or an immunization.
  • Disorder of the peripheral nervous system.
  • Also known as
  • Landry's Ascending Paralysis
  • Acute Inflammatory Demyelinating
    Polyradiculoneuropathy
  • Post-infectious polyneuritis
  • Acute post-infection polyneuropathy

5
Etiology
  • Cause is unknown.
  • Autoimmune?
  • Follows a febrile infection by 1-4 weeks
  • Upper Respiratory Tract
  • GI illness
  • Viruses and bacteria
  • Infectious mononucleosis is associated with 25
    of cases.
  • Followed the swine flu influenza vaccination in
    1976.
  • 500 cases of Guillain-Barré and 25 deaths.

6
Etiology
  • Parainfluenza 2
  • Variola
  • Measles
  • Mumps
  • Hepatitis A and B
  • Mycoplasma Pneumoniae
  • Salmonella typhi

7
Etiology
  • All age groups
  • Greater incidence in age group gt 45 years age.
  • Either sex
  • Greater incidence in males.
  • Greater incident in white population.
  • No seasonal clustering.

8
Pathophysiology
  • Most common acquired demyelinating neuropathy.
  • Inflammation and deterioration of peripheral
    nervous system.
  • Decreased ability of the neurons to transmit
    impulses to the muscles.
  • Paralysis.

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12
Neurilemma
  • The outer membrane of the Schwann cells is called
    the neurilemma.
  • Neurilemma is essential for regeneration of cut
    and injured axons.
  • Axons in the brain and spinal cord have no
    neurilemma and therefore cannot regenerate.

13
Clinical Manifestations
  • Progressive ascending skeletal muscle paralysis.
  • Ground to brain (G-B).
  • Tingling sensation and numbness.
  • Paresthesia
  • Loss of deep tendon reflexes.
  • Sensory nerve impairment.
  • Peripheral facial weakness.
  • Decreased gag reflex.
  • Decreased ability to swallow.
  • Dysphagia

14
Symptoms
  • Fever
  • Malaise
  • Nausea prostration.
  • Tingling or numbness in extremities.
  • Feet/legs affected first.
  • Paralysis and loss of reflexes.

15
Ventilatory Failure
  • Mucous Accumulation
  • Airway Obstruction
  • Alveolar Consolidation
  • Atelectasis

16
Progression of the Disease
  • Paralysis peaks in less than 10 days.
  • Progression of the paralysis may stop at any
    point.
  • As paralysis reaches its maximum, it usually
    remains unchanged for a few days to weeks.

17
Clinical Manifestations
  • ABG
  • Acute ventilatory failure with hypoxemia.
  • Cyanosis
  • PFT
  • Restrictive lung disease.
  • Decreased volumes.
  • Breath Sounds decreased.

18
Diagnosis
  • Neurological symptoms.
  • Cerebral Spinal Fluid.
  • Increased protein and normal cell count.
  • Electro-diagnostic studies.

19
X-ray
  • Normal.
  • If atelectasis is present
  • Radiopaque or radiodense (white).

20
Autonomic Nervous System Dysfunction
  • Develops in 50 of cases
  • Sympathetic
  • Parasympathetic
  • Loss of bowel and bladder sphincter control may
    occur

21
Management
  • Oxygen
  • Frequent monitoring of pulmonary function
    parameters.
  • Vital capacity
  • NIF or MIP MEP
  • Frequent monitoring of PO and ABG.
  • Mechanical ventilation
  • Ventilatory Respiratory Failure.
  • Tracheostomy

22
Management
  • Hyperinflation Protocol.
  • Bronchial Hygiene Protocol.
  • Risk of thromboembolic events
  • Subcutaneous heparin.
  • Elastic stockings.
  • Passive range of motion exercises.
  • Management of bedsores
  • Frequent turning.
  • Monitor for Pneumonia (infection).

23
Management
  • Urinary catheterization.
  • Treat cardiac dysrhythmias.
  • Plasmapheresis
  • Withdraw blood.
  • Separate out the plasma discard.
  • Re-infuse the cells with fresh frozen plasma.
  • Decreases the antibodies.
  • Total of five exchanges of 3 L each over 8-10
    days.
  • Steroid Therapy is controversial.

24
Pulmonary Function Monitoring
  • Mechanical Ventilation is indicated if
  • If VC decreases to 10 - 15 cc/kg or less.
  • NIF (MIP) is 20 cm H20 or lower.
  • f increases above 30/min.

25
Prognosis
  • Spontaneous recovery is expected in 85-95 of
    cases.
  • Improvement may take weeks or months.
  • 30 of adults have residual weakness at 3 years.

26
Myasthenia Gravis
27
Definition
  • A disease characterized by episodic muscle
    weakness caused by loss or dysfunction of
    acetylcholine receptors.
  • Chronic disorder.
  • Axon and receptor site of the voluntary muscle.
  • Periods of fatigue with improvement following
    rest.

28
Etiology
  • Circulating anti-ACh receptor antibodies disrupt
    the chemical transmission of ACh.
  • Blocking the ACh from the receptor site.
  • Accelerating the breakdown of ACh.
  • Destroying the receptor sites.
  • Thymus gland is abnormal
  • Antibodies thought to come from the thymus gland.

29
Etiology
  • Twice as common in women.
  • Peak age of onset for females is 15 to 35 years.
  • Peak age of onset for males is 40 to 70 yrs.
  • Signs/symptoms are provoked by
  • Emotional upset.
  • Physical stress.
  • Exposure to extreme temperature changes.
  • Febrile illness.
  • Pregnancy.
  • Death is possible but incidence of deaths decline
    after 10 years.

30
Anatomic Alterations
  • Isolated groups of muscles (eyelids).
  • Generalized muscle weakness
  • May include the diaphragm.
  • Ventilatory Failure.

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32
Clinical Findings
  • Weakness of striated muscle
  • Eye muscles (ptosis).
  • Extraocular muscles (diplopia).
  • Muscles of the lower portion of the face (speech
    impairment).
  • Chewing and swallowing muscles (dysphagia).
  • Muscles of the arms and legs.

33
Clinical Findings
  • Descending paralysis.
  • Mind to Ground (MG)
  • Gradual onset.
  • First symptoms.
  • Drooping of the eyelids.
  • If only the eyes are involved than it is referred
    to as Ocular Myasthenia.

34
Clinical Findings
  • Generalized skeletal muscle disorder
  • Muscles of neck and face.
  • Unable to chew and swallow.
  • Aspiration
  • Unable to speak clearly.
  • Weakness of neck muscles causes the patients head
    to fall forward.

35
Clinical Findings
  • Weakness of arms and legs results in difficulty
    in
  • Climbing stairs.
  • Lifting objects.
  • Maintaining balance.
  • Walking.
  • Hand cannot lift to the mouth.

36
Clinical Findings
  • Pain is rare.
  • Tendon reflexes remain intact.
  • Patient may demonstrate normal health for weeks
    and months at a time.
  • May only show signs of weakness late in the day
    or at night (exhaustion).
  • Develop a sudden and transient generalized muscle
    weakness that includes the diaphragm.

37
Clinical Findings
  • If ventilatory failure is handled properly the
    chest x-ray findings should be normal.
  • If improperly managed
  • Alveolar consolidation
  • Atelectasis
  • Excessive secretions

38
ABG
  • Acute ventilatory failure with hypoxemia
  • Decreased pH
  • Increased PaCO2,
  • Decreased PaO2
  • Hypoxemia secondary to hypoventilation

39
Clinical Findings
  • Pulmonary Functions
  • Decreased volumes.
  • Cyanosis
  • Diminished BS
  • X-ray
  • Normal or radiopaque (white) if atelectasis is
    present.

40
Diagnosis
  • Clinical history.
  • Clinical response to an IV injection of
    edrophonium chloride (Tensilon).
  • Tensilon Test
  • Tensilon is an anticholinesterase.
  • Electrophysiological tests (EMG).
  • Circulating antibody levels in the blood.
  • Enlarged Thymus gland.

41
Thymus Gland
  • Manufacture T-lymphocytes.

42
Management
  • Patients may live a normal life span.
  • Exacerbation
  • Oxygen
  • Frequent monitoring of VC and NIF.
  • Frequent monitoring of BP and SpO2.
  • Frequent monitoring of ABG.
  • Mechanical ventilation.

43
Management of MG
  • Anticholinesterase Drugs
  • Edrophonium Chloride (Tensilon)
  • Neostigmine (Prostigmine)
  • Pyridostigmine (Mestinon)
  • Steroid Therapy
  • Prednisone
  • ACTH therapy

44
Management of MG
  • Thymectomy
  • Useful in young adult females
  • Plasmapheresis
  • Bronchial hygiene
  • Hyperinflation protocol
  • Atelectasis

45
Myasthenia Crisis vs.Cholinergic Crisis
  • Myasthenia Crisis
  • Exacerbation of the disease.
  • Muscle strength improves after Tensilon Test.
  • Increase dosage of anticholinesterase agents.
  • Cholinergic Crisis
  • Too much anticholinesterase drugs.
  • No muscle strength noted after Tensilon.
  • Stop or decrease dosage of anticholinesterase
    agents and give atropine.
  • MEDICAL EMERGENCY

46
SPINAL CORD INJURY
47
Spinal Nerves
  • 31 Pair
  • 8 Cervical
  • 12 Thoracic
  • 5 Lumbar
  • 5 Sacrospinal
  • 1 Coccygeal

48
Spinal Cord Injury
  • High neck fractures (above C3).
  • Apnea.
  • All respiratory muscles are affected.
  • Mechanical ventilation/Tracheostomy.
  • Injury to C3-C8 is a quadriplegic but retains
    some use of respiratory muscles.
  • Night-time mechanical ventilation.
  • Injury below C8.
  • Cough weak and ineffective secretion removal .
  • Loss of function of abdominal muscles.

49
Amyotrophic Lateral Sclerosis
50
Lou Gehrigs Disease
  • Anterior Horn Cells.
  • Weakness of hands and less often in the feet.
  • The site of onset is random, and progression is
    asymmetric.
  • Dysarthria and dysphagia are due to involvement
    of brain stem nuclei and pathways.
  • Sensory systems, voluntary eye movements, and
    urinary sphincters are spared.
  • Rarely, a patient survives 30 years
  • 50 die within 3 years of onset.
  • 20 live 5 years.
  • 10 live 10 years.

51
  • ALS can strike anyone.
  • Male or female
  • Any ethnic origin
  • At any age
  • Some have been diagnosed as teens
  • Usual onset is in middle age
  • http//www.alsa.org/
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