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Y4R3 - Medicine II 🩺 > L6: Myopathy > Flashcards

L6: Myopathy Flashcards

1
Q

Skeletal muscle constitutes the principal organ of locomotion.

A

…

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2
Q

There is more than 600 separate muscles, this tissue makes up as much as 40% of the weight of adult human beings.

A

…

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3
Q
  • The muscle fibers are attached at their ends to tendon fibers —-> connect with the skeleton.
  • By this means, muscle contraction maintains posture and produce movement.
A

…

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4
Q

Histology of Muscle Fibers

  • Types of Muscles
A

Red and White (human muscles are composed of both types)

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5
Q

Histology of Muscle Fibers

A
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6
Q
  • Each lower motor neuron supplies a bundle of muscle fibers but each M.F. is innervated by only one axon.
A

…

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7
Q

Def of Motor Unit

A

is a motor neuron and all the muscle fibers it innervates.

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8
Q

Examples of Motor Unit

A
  • In Ocular muscles: a motor unit contains only 6 to 10 muscle fibers.
  • In Gastrocnemius: a motor unit contains as many as 1,800 fibers
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9
Q

Physiology of Muscle Fibres & muscle Contraction

A
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10
Q

Manifestations of Muscle Diseases

A
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11
Q

Def of Progressive Muscular Dystrophy

A
  • Group of genetically determined primary degenerative muscular disorders characterized by muscular weakness and wasting (myopathy).
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12
Q

Pathogenesis of Progressive Muscular Dystrophy

A
  • A defect in muscle fiber plasma membrane → uncontrolled entry of calcium → activates calcium dependent protease → muscle fiber necrosis.
  • The missing gene product is a protein called Dystrophin.
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13
Q

Pathology in Progressive Muscular Dystrophy

A
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14
Q

Classification of Progressive Muscular Dystrophy

A
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15
Q

Classification of Progressive Muscular Dystrophy

  • X-Linked Recessive
A
  1. Severe (Duchenne).
  2. Benign (Becker).
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16
Q

Classification of Progressive Muscular Dystrophy

  • AD
A
  1. Facio-scapulo-humeral.
  2. Scapulo-peroneal.
  3. Limb girdle.
  4. Distal.
  5. Ocular
  6. Oculo-pharyngeal.
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17
Q

Classification of Progressive Muscular Dystrophy

  • Autosomal Recessive
A
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18
Q

Age in Becker Muscle Dystrophy

A

at 5-20 Years

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19
Q

Course of Becker Muscle Dystrophy

A

Slowly progressive and does not shorten life.

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20
Q

Sex in Duchenne Muscle Dystrophy

A

Affects Boys & Females are carriers. → due to It is X-linked s

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20
Q

Age in Duchenne Muscle Dystrophy

A
  • Between 3 -10 years.
  • The child appears normal at birth and in the first year of life.
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21
Q

Onset of Duchenne Muscle Dystrophy

A

Gradual

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22
Q

Course of Duchenne Muscle Dystrophy

A

Rapidly progressive

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23
Q

Site affected in Duchenne Muscle Dystrophy

A

Pelvic girdle, then Shoulder girdle muscles.

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24
Q

CP of Duchenne Muscle Dystrophy

A
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25
Q

CP of Duchenne Muscle Dystrophy

  • Which Side?
A

Bilateral & Symmetrical

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26
Q

CP of Duchenne Muscle Dystrophy

  • Proximal or Distal?
A

Proximal more than distal

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27
Q

CP of Duchenne Muscle Dystrophy

  • Symptoms
A
  • Slow Walking, inability to Run, frequent Falling.
  • Difficulty in Climbing stairs, Putting on and Taking off clothes.
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28
Q

CP of Duchenne Muscle Dystrophy

  • Shoulders
A

Sloping shoulders

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29
Q

CP of Duchenne Muscle Dystrophy

  • Scapulae
A

Winging of scapulae

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30
Q

CP of Duchenne Muscle Dystrophy

  • Spine & pelvis
A
  • Exaggerated Lumbar Lordosis, protuberant abdomen, pelvis is tilted forward due to: Weak Glutei, so lordosis compensates this tilting and it disappears with sitting.
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31
Q

CP of Duchenne Muscle Dystrophy

  • Muscle Hypertrophy?
A
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32
Q

CP of Duchenne Muscle Dystrophy

  • Gait
A
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33
Q

CP of Duchenne Muscle Dystrophy

  • Signs
A
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34
Q

CP of Duchenne Muscle Dystrophy

  • Ambulation
A

Ambulation is lost at age of about 10 years.

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35
Q

CP of Duchenne Muscle Dystrophy

  • Reflexes
A

Reflexes are lost → With increasing weakness and progressive muscle atrophy

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36
Q

CP of Duchenne Muscle Dystrophy

  • No …….
A

No fasciculations, sphincter disturbance, sensory changes.

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37
Q

CP of Duchenne Muscle Dystrophy

  • which Muscles are involved?
A
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38
Q

CP of Duchenne Muscle Dystrophy

  • Speech
A

Delayed speech: development may occur.

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39
Q

CP of Duchenne Muscle Dystrophy

  • IQ
A

May be affected

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40
Q

Age in Facio-Scapulo-Humeral Type

A

starts in the 2nd decade

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41
Q

Course in Facio-Scapulo-Humeral Type

A

Benign, arrest is common.

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42
Q

Incidence of Facio-Scapulo-Humeral Type

A

Females are less severely affected than males.

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43
Q

CP of Facio-Scapulo-Humeral Type

A
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44
Q

CP of Facio-Scapulo-Humeral Type

  • Transverse Smile
A

Elevators > Retractors

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45
Q

Incidence of Scapulo-Peroneal Type

A

Rare Syndrome

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46
Q

CP of Scapulo-Peroneal Type

A

Like Facioscapulohumeral but with no facial affection.

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47
Q

Inheritance of Limb-Girdle Ms Dystrophy

A
  • Autosomal recessive in 90%,
  • Autosomal dominant in 10%.
  • Sporadic cases were reported
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48
Q

Age in Limb-Girdle Ms Dystrophy

A

Starts at 3rd Decade

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49
Q

Course of Limb-Girdle Ms Dystrophy

A

Slowly Progressive

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50
Q

CP of Limb-Girdle Ms Dystrophy

A
  • More commonly starts in Shoulder Girdle more than Pelvic Girdle
  • There may be enlarged calf muscles mildly.
  • Usually limited for years before spread
  • Severe disability occurs after about 20 years.
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51
Q

Inheritance of Congenital Type Ms Dystrophy

A

Autosomal recessive

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52
Q

CP of Congenital Type Ms Dystrophy

A
  • Characterized by congenital or early infantile onset of weakness with variable CNS involvement including ocular.
  • Epilepsy is common and although mental development is normal,
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53
Q

MRI in Congenital Type Ms Dystrophy

A

white matter abnormalities

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54
Q

Inheritance of Distal Myopathy

A

Some Are Autosomal Dominant & Some Are Autosomal Recessive

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55
Q

Onset of Distal Myopathy

A
  • Some begin in 4th - 6th decade
  • Some patients show onset in childhood or early adult life-
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56
Q

Age in Occular Type Ms Dystophy

A

Before 30 years

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57
Q

CP of Occular Type Ms Dystophy

A
  1. External opthalmoplegia,
  2. Progressive bilateral ptosis
  3. Orbicularis oculi affection.
  4. No pupillary changes
  5. No diplopia (gradual bilateral symmetrical affection).
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58
Q

Age in Occulo-Pharyngeal Type

A

Around 40 years old.

59
Q

CP of Occulo-Pharyngeal Type

A

Similar to ocular type but with dysphagia.

60
Q

Investigations in Progressive Muscular Dystrophy

A
60
Q

Investigations in Progressive Muscular Dystrophy

  • EMG
A

Diminished duration and amplitude of motor action potential.

61
Q

Investigations in Progressive Muscular Dystrophy

  • Urine
A
  • Diminished creatinine
  • Appearance of creatine.
62
Q

Investigations in Progressive Muscular Dystrophy

  • Serum Enzymes
A

Increase and that’s used to detect
- Female carriers and
- Preclinical diagnosis in males.

63
Q

Investigations in Progressive Muscular Dystrophy

  • Muscle Bx
A

…

64
Q

TTT of Progressive Ms Dystophy

A
65
Q

TTT of Progressive Ms Dystophy

  • Steroids
A

It is now accepted that it prolongs ambulation

66
Q

TTT of Progressive Ms Dystophy

  • Myoblast Transfer
A

Injecting the muscle with a huge number of myoblasts will give a transient improvement (but cost exceeds benefit).

67
Q

Def of Myotonic Disorders

A

A group of M. disorders characterized by delayed relaxation of skeletal muscles after voluntary, mechanical or electrical stimulation.

68
Q

What Improves Myotonic Disorders?

A
  1. Exercise (Repetition of movement)
  2. Calcium
  3. Warmth
  4. Quinine and Procainamide.
69
Q

What Worsens Myotonic Disorders?

A
  1. Rest
  2. Cold
  3. Potassium
  4. Prostigmine.
70
Q

Types of Myotonic Disorders

A
  1. Myotonic Dystrophy
  2. Myotonia congenita
  3. Paramyotonia congenita
  4. Myotonia paradoxica
  5. Acquired myotonia
  6. Chondrodystrophic myotonia (Schwartz-Jample syndrome)
71
Q

Age in Myotonic Dystrophy

A

20 - 50 Years

72
Q

Incidence in Myotonic Dystrophy

A

Male › female

73
Q

CP of Myotonic Dystrophy

A
74
Q

Age in Myotonia Congenita

A

1st & 2nd Decade

75
Q

Incidence of Myotonia Congenita

A
  • Female more than male.
  • Autosomal recessive & less frequently autosomal dominant
76
Q

CP of Myotonia Congenita

A
  • Present since birth (Difficult Suckling).
  • Generalized muscular hypertrophy and stiffness.
77
Q

PPT Factors for Paramyotonia Congenita & Myotonia Paradoxica

A
  • Precipitated only by exposure to cold
  • Exacerbated by exercise
78
Q

Acquired Myotonia is associated with …..

A

Occurs with polymyositis and some cases of polyneuropathy.

79
Q

Another Name of Chondrodystrophic Myotonia

A

Schwartz-Jample Syndrome

80
Q

CP of Chondrodystrophic Myotonia

A
  • AR
  • Dwarfism
  • Myotonia
  • Skeletal deformities
81
Q

Prognosis of Myotonic Disorders

A
82
Q

Prognosis of Myotonic Disorders

  • Myotonia Congenita
A
83
Q

Prognosis of Myotonic Disorders

  • Myotonia Dystrophica
A
84
Q

Prognosis of Myotonic Disorders with anasethia

A
85
Q

TTT of Myotonic Disorders

A
86
Q

TTT of Myotonic Disorders

  • Genetic Counseling
A
  • Female carrying the gene may be asymptomatic but still at risk of having a child with sever congenital form of the disease so antenatal diagnosis based on chorionic villus sampling is needed.
87
Q

TTT of Myotonic Disorders

  • ECG
A

regular

88
Q

TTT of Myotonic Disorders

  • Chest Infections
A

may be reduced by influenza and pneumococcus vaccination.

89
Q

TTT of Myotonic Disorders

  • Anesthetia
A

The patient must carry a warning card explaining hazards.

90
Q

TTT of Myotonic Disorders

  • Cataract
A

Treated surgically.

91
Q

TTT of Myotonic Disorders

  • Drugs
A
  • Procainamide (500 mg t.d.s.) & Phenytoin (100 mg t.d.s.)
  • They are theoretically contraindicated because of their potential action on cardiac conduction but practically few problems have been reported.
92
Q

TTT of Myotonic Disorders

  • Speech therapy
A

to correct swallowing and dysarthria.

93
Q

Def of Myasthenia Gravis

A
  • Disorder of transmission of the myoneural junction characterized clinically by fatigability on repetition of muscular activity which may be relieved by rest and or cholinergic drugs.
94
Q

Pathogenesis of Myasthenia Gravis

A

It is due to an autoimmune process in which antibodies against acetylcholine receptors cause a disordered conduction in neuromuscular junction.

95
Q

Pathology of Myasthenia Gravis

A
96
Q

Sex in Myasthenia Gravis

A

Female: male = 2: 1

97
Q

Age in Myasthenia Gravis

A

Mean age of onset: 26y in Females, 30y in Males.

98
Q

Myasthenia Gravis is closely related to ……

A
  • Thyrotoxicosis
  • Rheumatoid arthritis
  • M.S. (Multiple sclerosis)
  • SLE. (Systemic Lupus Erythematosus)
  • Biliary cirrhosis
  • Cancer
99
Q

CP of Myasthenia Gravis

A

…

100
Q

Onset of Myasthenia Gravis

A

Gradual

101
Q

Course of Myasthenia Gravis

A

progressive with remissions.

102
Q

CP of Myasthenia Gravis

  • site
A

Only skeletal muscles (smooth muscles are not involved).

103
Q

CP of Myasthenia Gravis

  • Symptoms
A
104
Q

CP of Myasthenia Gravis

  • Descending march
A
105
Q

CP of Myasthenia Gravis

  • Fluctiations in Myasthenia
A
106
Q

CP of Myasthenia Gravis

  • Muscle Bulk Affection
A

No

107
Q

CP of Myasthenia Gravis

  • smooth Musce affection
A

No

108
Q

CP of Myasthenia Gravis

  • Smile?
A

Retractors of mouth angle › elevators → Vertical or snarling smile.

109
Q

What induces remission of Myasthenia Gravis?

A

Pregnancy

110
Q

Dx of Myasthenia Gravis

A
111
Q

Dx of Myasthenia Gravis

  • Clinical Fatigue Test
A

Inducing fatigue by asking the patient to:
- Count to 50 (will induce dysarthria) or
- Maintain looking up (will precipitate ptosis).

112
Q

Dx of Myasthenia Gravis

  • Prostigmine test
A

1.5 mg Prostigmine + 0.5 mg Atropine IM → Improve myasthenia.

113
Q

Dx of Myasthenia Gravis

  • EMG
A

Repetitive stimulation test shows:
- Diminished amplitude after repeated stimulation (decremental response).

114
Q

Dx of Myasthenia Gravis

  • Tensilon Test
A

2 mg IV followed, if no idiosyncrasy, by 8mg → improve myasthenia after a minute.

115
Q

Dx of Myasthenia Gravis

  • Most Sensitive Test
A

Detection of Anti-Ach R Antibodies

116
Q

Dx of Myasthenia Gravis

  • Chest tomogram
A

to detect Thymoma or Thymic Hyperplasia.

117
Q

Dx of Myasthenia Gravis

  • Thyroid Function Tests
A

…

118
Q

Dx of Myasthenia Gravis

  • tests for Collagen Disorders or Cx
A

…

119
Q

TTT of Myasthenia Gravis

A
120
Q

TTT of Myasthenia Gravis

  • Anticholinestrase
A
  • Mestinon
  • Prostigmine: titrate until adequate response.
121
Q

TTT of Myasthenia Gravis

  • Prednisone
A
  • 50 mg daily then taper to 5-10 mg per day as a maintenance dose.
122
Q

TTT of Myasthenia Gravis

  • Azathioprine
A

2mg/ Kg/day.

123
Q

TTT of Myasthenia Gravis

  • Plasmapharesis
A

to get rid of harmful antibodies.

124
Q

TTT of Myasthenia Gravis

  • IVIG
A

0.4 gm/ Kg/day divided over five successive days.

125
Q

TTT of Myasthenia Gravis

  • Thymectomy
A

Obligatory for Thymoma or Thymic hyperplasia.

126
Q

Def of Crises in Myasthnia

A

Severe deterioration in a myasthenic patient which necessitate Mechanical Ventilation.

127
Q

Types of Crises in Myasthnia

A
128
Q

Types of Crises in Myasthnia

  • Myasthenic Crises
A
129
Q

Types of Crises in Myasthnia

  • Cholinergic Crises
A
130
Q

Managment of Crises

A
131
Q

Managment of Crises

  • Tensilon test
A

For differentiation (it will improve only the myasthenic crisis)

132
Q

Managment of Crises

  • Avoid ……
A
  • Aminoglycosides
  • Ampicillin
  • Phenytoins
  • Propranolol
  • Morphine
  • Barbiturates (has a neuromuscular blocking effect)
133
Q

Managment of Crises

  • Re-introduction of Drugs
A
134
Q

age in Neonatal Myasthenia

A

Seen in infants from a myasthenic mother

135
Q

Etioogy of Neonatal Myasthenia

A

It is due to antibodies from the mother serum.

136
Q

TTT of Neonatal Myasthenia

A
  • Recovers spontaneously (1 week - 3 months after delivery).
  • The infant need treatment with anti cholinesterase for short duration
137
Q

Def of Congenital Myasthenia

A

Genetically determined non-auto-immune disorder.

138
Q

Pathogenesis of Congenital Myasthenia

A
  • Mutation affecting one of A.Ch.R. subunits
  • Mutation affecting the end plate protein.
139
Q

Incidence of Congenital Myasthenia

A
  • Rare disease
  • Begin in infancy (floppy infant).
140
Q

CP of Congenital Myasthenia

A
141
Q

Etiology of Lambert-Eaton Syndrome (Myasthenic-Myopathic)

A
142
Q

CP of Lambert-Eaton Syndrome (Myasthenic-Myopathic)

A
143
Q

TTT of Lambert-Eaton Syndrome (Myasthenic-Myopathic)

A
  1. Tumour treatment often improves the picture.
  2. Prednisolone and azathioprine often induces remission → In absence of tumor
  3. Plasma exchange and IVIG are effective.

Y4R3 - Medicine II 🩺 (16 decks)

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