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Musculoskeletal > Neuromuscular Diseases > Flashcards

Neuromuscular Diseases Flashcards

1
Q

Inflammatory Myopathies

A

-acquired diseases mostly autoimmune (polymyositis, dermatomysitis, inclusion body myosistis, sarcoidosis) or by infection (trichinosis)

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2
Q

What are metabolic myopathies caused by?

A

enzyme deficiencies that block metabolic pathways, depriving muscle cells of energy for muscle contraction and relaxation and to maintain membrane integrats

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3
Q

What happens during metabolic myopathies?

A

Breakdown (rhabdomyolysis) and excretion of muscle proteins in urine (myoglobinuria) when energy demands increase with exercise

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4
Q

Metabolic Myopathies?

A

disorders of glycogen, lipid metabolism, or disorders of mitochondrial respiratory chain

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5
Q

How do metabolic myopathies present?

A
  • muscle weakness or exercise intolerance
  • muscle pain
  • myoglobinuria
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6
Q

Myotonic Dystrophy

A

-percussion and grip myotonia
-facial, neck, distal weakness
-autosomal dominant
-cataracts
-balding
-diabetes
-central sleep apnea
-megaesophagus, megacolon
-heart conduction defects
-mental retardation in newborns of MD moms
-genetic defect increases (increased CGT repeats)
chromosome 19
-proximal myotonic dystrophy is similar, but has proximal weakness & a different genotype (change in chromosome 3)

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7
Q

Myasthenia gravis

A
  • autoimmune disease characterized by fluctuation muscle weakness
  • weakness is caused by antibodies that bind and destroy muscle acetylcholine receptors
  • Patients usually respond to anticholinesterase drugs, immunotherapy and thymectomy
  • also perform repetitive stimulation test*
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8
Q

Tensilon/Edrophonium

A

Ach drug that produces rapid improvement of myasthenia gravis

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9
Q

Treatment of Myasthenia Gravis?

A
  • ach drugs like pyridostigmine
  • corticosteroids
  • immunosuppressants (azathioprine, cyclophosphamide, mycophenolate mofeti)
  • Thymectomy (if large)
  • Plasma exchange (removes abs)
  • Immunoglobulin infusions
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10
Q

Symptoms of Lambert-Eaton myasthenic syndrome?

A
  • proximal limb weakness
  • “fatigue” or fluctuation of symptoms
  • difficulty rising from sitting position
  • reduced ability to walk/climb
  • dry mouth, metallic taste, anticholinergic symptoms
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11
Q

Signs of Lambert-Eaton myasthenic syndrome?

A
  • proximal limb weakness (legs>arms)
  • reduced muscle stretch reflexes
  • transient improvement in muscle power following exercise
  • minimal eyelid droop*
  • small poorly reactive pupils
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12
Q

Gower’s Sign

A

-how a patient with proximal muscle weakness must get up

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13
Q

Myopathy pattern of weakness

A

proximal > distal

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14
Q

Neuromuscular junction pattern of weakness

A

proximal > distal

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15
Q

Polyneuropathy pattern of weakness

A

distal > proximal

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16
Q

When muscle looses innervation??

A

the muscle fibers become atrophic and angulated

-atrophic fibers stain dark

17
Q

polyphasic

A

damaged individual fibers fire asynchronously

18
Q

Myopathies as far as fibers go?

A

nromal # of motor neurons and axons, motor untis

-# of individual fibers that are nonfunctional

19
Q

Myopathies

A
  • decreased in the # of muscle fibers per motor unit
  • During voluntary activity
  • motor unit potentials are small in size and of short duration
  • increased number of motor unit action potentials as compared to the strength of contraction
  • decreased # of muscle fibers per motor unit
20
Q

Myopathies: Signs & Symptoms

A
  • proximal, symmetrical weakness
  • normal sensation
  • normal reflex
  • elevated muscle enzyme in serum
  • small motor unit potentials on EMG with normal or increased recruitment on maximal effort
  • abnormal muscle biopsy, depending on the type of myopathy
21
Q

Infantile Acid Maltase Deficiency

A

“floppy”

22
Q

Congenital Myopathies

A
  • “floppy” infants (weakness, hypotonia) at birth, central core disease, myotubular myopathy or rod myopathy and congenital muscular dystrophy
  • hereditary
23
Q

Muscular Dystrophies

A

PROGRESSIVE

  • early childhood
  • hereditary and progressive conditions
  • some are caused by abnormalities or deficiencies of the muscle membrane or nuclear proteins
    ex: Duchenne’s, Becker’s, limb girdle, fascioscapulohumeral and oculopharyngeal dystrophy’s
  • hereditary
24
Q

Myotonic Dystrophies

A

PROGRESSIVE

  • characterized by the presence of myotonia and weakness
    ex: myotonic dystrophy and proximal myotonic myopathy
  • hereditary
25
Q

Duchenne’s Muscular Dystrophy

A

Lethal Childhood Disorder

  • abnormal gene, no dystrophin
  • 1 in 3,500 male births
  • progressive muscle weakness, pseudohypertryphy, abnormal heart, low IQ
26
Q

Duchenne’s causes what about muscle?

A
  • necrosis
  • atrophy
  • segmental fiber over-contraction
  • connective tissue proliferatioin
27
Q

Duchenne’s Signs/Symptoms

A
  • winging of scapulae
  • lordosis
  • large calf muscles
28
Q

What stain shows dystrophin?

A

immunoperoxidase

29
Q

Becker’s Muscular Dystrophy

A
  • some muscle fibers have protein
  • Lordosis
  • Calf hypertrophy
  • present at older age
30
Q

Endocrine Myopathies

A

proximal

  • develop weakness in hyperparathyroidism
  • Crushing’s disease and hypothyrodism
  • select Type II muscle fiber atrophy
31
Q

Toxic Myopathies

A
  • ethanol
  • anesthetics
  • cholesterol-lowering agents
  • glucocorticoids
  • narcotics
  • herbicides
  • muscle fiber atrophy, vacuolization, myofibrillary degeneration, mitochondrial dysfunction, fiber necrosis*
32
Q

Therapy of Lambert-Eaton Syndrome

A

-romoval of associated neoplasm
-“symptomatic” boost of neuromuscular transmission
Pyridostigmine-cholinesterase inhibitors
3,4-diaminopyridine
Immunosuppressive therapy
-high-dose, long term corticosteroids
-azathioprine
-plasmapheresis

Musculoskeletal (19 decks)

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