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ADELE-Noodles of Neurology > Nerve and muscle disease > Flashcards

Nerve and muscle disease Flashcards

1
Q

What are the three types of muscle?

A

Skeletal, smooth, cardiac

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2
Q

Describe skeletal muscle

A

Highly organised

There is an endomysium, perimysium and epimysium

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3
Q

What is a fascicle encapsulated by?

A

Perimysium

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4
Q

What are a large group of fascicles encased in?

A

Epimysium

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5
Q

What is the smallest contractile unit surrounded by?

A

Endomysium

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6
Q

Describe smooth muscle

A 
Cells not striated
Single central network
Significant connective tissue around them
No sarcomeres
Actin:Myosin ratio 10:1
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7
Q

What are the three types of smooth muscle fibres?

A

Type I
Type IIa
Type IIb

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8
Q

Describe type 1 fibres

A

Slow oxidative- dense capillary network, myoglobin, resist fatigue

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9
Q

Describe type IIa fibres?

A

Fast oxidative- aerobic metabolism

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10
Q

Describe type IIb fibres?

A

Fast glycolytic, easily fatigued

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11
Q

What is myotonia?

A

Failure of muscle relaxation after use

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12
Q

What are fasciculations?

A

Visible, fast, fine, spontaneous twitch

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13
Q

Fasciculations only appear if muscle has pathology TRUE/FALSE

A

May occur in healthy muscle-triggers include stress, caffeine and fatigue
Usually a sign of disease in motor neurone

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14
Q

What myopathy can statins cause?

A

Autoimmune myositis (always check CK)

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15
Q

What 5 drugs can cause myopathies?

A 
Statins
Hydroxycloroquine
Amiodarone
Diuretics
A-interferon
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16
Q

What is the MRC muscle power grading?

A

0-No movement at all
1-Flicker of movement when attempting to contract muscle
2-Some muscle movement if gravity removed but none against gravity
3- Movement against gravity but not against resistance
4- Movement against resistance but not full strength
5-Normal strength

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17
Q

Name a postsynaptic disorder

A

Myasthenia gravis

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18
Q

Name 2 presynaptic disorder

A

Botulism-Clostridium botulinum

Lambert Eaton Myasthenic Syndrome (LEMS)

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19
Q

What are the synapses formed between motor neurones and muscle called?

A

Motor end plates

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20
Q

Where do the cell bodies that innervate the motor neurones that innervate the skeletal muscle fibres arise?

A

Ventral horn of the spinal cord

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21
Q

TRUE/FALSE

A single motor neuron may control many muscles but each muscle cell responds only to one motor neuron

A

TRUE

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22
Q

What is curare?

A

Muscle relaxant used in anaesthetics that competes with acetylcholine and blocks transmission of information

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23
Q

What is McArdle’s disease?

A

AR, usually presents in 1st decade of life
caused by myophosphorylase deficiency
Muscle pain soon after starting exercise
Second wind phenomenon

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24
Q

Why should patients with McArdles disease stop if they get severe pain when exercising?

A

As it may increase risk of rhabdomyolysis with myoglobinuria and subsequent AKI

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25
Name the chronic autoimmune disease affecting post-synaptic nicotinic acetylcholine receptors at NMJ
Myasthenia gravis
26
What is the most common disorder of the NMJ?
Myasthenia gravis
27
What is the typical Myasthenia gravis patient?
F>M (ratio 3:2) Females in 3rd decade Males in 6/7th decade
28
When do the symptoms of myasthenia gravis start?
When the number of Ach receptors decreases to 30% of normal
29
Clinical features of myasthenia gravis
Muscle fatiguability/weakness Occular features commonly appear first Proximal muscle weakness more marked than distal muscles Jaw, facial muscles, speech and/or swallow and resp muscles are also affected weakness on neck flexion
30
Name two complications of myasthenia gravis
Myasthenia crisis | Cholinergic crisis
31
What is myasthenia crisis?
Weakness of muscles of respiration cause ventilator failure
32
How do you monitor for myasthenia crisis?
Measure vital capacity and tidal volume | If vital capacity falls below 15mL/kg then urgent elective tracheal intubation and ventilation considered
33
What is the treatment for myasthenia crisis?
Plasmapheresis IV immunoglobulins Systemic steroids
34
What are the symptoms of cholinergic crisis?
Sweating Hypersalivation Bronchial hyper secretions and miosis
35
Name 4 common associations with myasthenia gravis
Thymic hyperplasia Thymoma Hyperthyroidism SLE
36
What is the drug to look out for which can exacerbate myasthenia?
Gentamicin
37
What testing is done for myasthenia gravis?
Anti-AchR | If this is negative can check for anti-MuSK
38
what are the three investigations for those you suspect to have myasthenia gravis?
``` Antibody testing (anti-AchR and anti-MuSK) Repetitive nerve stimulation tests Thyroid function test and CT thymus ```
39
What is symptomatic control of acetylcholinesterase inhibition with?
Pyridostigmine
40
What immunosuppressive agents are used to improve myasthenic weakness and establish remission
Corticosteroids | Azathioprine
41
In myasthenia gravis what is morbidity due to?
Resp failure and aspiration pneumonia
42
Describe muscular dystrophy
Inherited, non inflammatory, progressive, no central or peripheral abnormality
43
What is the commonest muscular dystrophy in adults?
Myotonic dystrophy
44
What is myotonic dystrophy and how is it inherited?
Multisystem progressive disease | AD: tri-nucleoide repeat on Ch 19 (with anticipation)
45
What are the clinical features of myotonic dystrophy?
``` Sternocleidomastoid & distal limb muscle affected first Facial weakness ptosis, ophthalmoplegia Christmas tree like cataracts Hollowing of temples Early frontal balding ```
46
What is Lambert-Eaton Myasthenic syndrome (LEMS)?
NMJ disorder caused by impaired release of acetylcholine by pre-synaptic terminal due to autoimmune antibodies
47
What has LEMS got a strong association with?
Underlying small cell carcinoma
48
What are the clinical features of LEMS?
``` Insidious onset of weakness of proximal muscles (lower extremity mainly affected) Autonomic features (constipation, postural hypotension, impotence and dry mouth) Deep tendon reflexes diminished/abscent ```
49
What is diagnosis of LEMS established by?
Presence of anti-VGCC antibodies Characteristic electrophysiological findings using a 20-50Hz repetitive stimulation CT scan (to rule out malignancy)
50
What is the treatment for LEMS?
3,4-diaminopyridine (amifampridine)
51
What is Charcot-Marie-Tooth disease?
A group of hereditary neuropathies Can be inherited or AD (most common), AR or X-linked Onset in first 2 decades with motor symptoms In lower limbs Sensory loss follows same pattern as muscle weakness Inverted champagne bottle appearance
52
What does Charcot Marie Tooth disease look like histopathologically?
Schwann cells proliferate and form concentric arrays of demyelination around the demyelinated axon - onion bulb appearance
53
What is Guillian Barre syndrome?
Acute neuromuscular weakness causing demyelination and axonal injury
54
With GBS what infections do most patients have a preceding history of?
Campylobacter
55
How does GBS present?
Symmetrical, progressive ascending sensorimotor paralysis | Usually stops progressing 4wks from its onset
56
How is GBS diagnosed?
Usually clinical However LP (show high CSF protein) and nerve conduction studies (suggestive of patchy proximal and distal demyelination ) sometimes helpful
57
What is the management of GBS?
Plasma exchange or IV immunoglobulins
58
What is chronic inflammatory demyelinating polyradiculoneuropathy (CIPD)
Acquired demyelinating peripheral nervous system disease characterised by progressive or relapsing proximal and distal weakness with hyporeflexia/areflexia and a distal sensory loss (paraesthesia and numbness)
59
How is CIDP diagnosed?
CliniCAL | Supported by electrophysial nerve conduction studies & CSF analysis (high protein)
60
What is the treatment for CIPD?
Oral steroids or IV immunoglobulins
61
What is Spinal muscular atrophy?
AR neuromuscular disorder Congenital degeneration of anterior horns of spinal cord (LMN lesions) which leads to progressive muscular wasting often leading to early death
62
What does the severe form of spinal muscular atrophy manifest itself as in early life?
Floppy baby syndrome
63
What is poliomyelitis?
Symmetrical progressive proximal weakness developing over weeks to months
64
What is poliomyelitis caused by?
Poliovirus (faecal-oral transmission | The infection causes destruction of cells in the anterior horn of spinal cord (LMN death)
65
What is dermatomyositis similar to?
Clinically similar to poliomyelitis but ass with skin lesions, "heliotrope" rash on face up to 50% have underlying malignancy
66
How does inclusion body myositis present?
Typically slowly progressive weakness in 6th decade of life with characteristic thumb sparing
67
What is rhabdomyolysis?
Dissolution of muscle | Damage to skeletal muscle causes leakage go large quantities of toxic intracellular contents into plasma
68
How does rhabdomyolitis present?
Triad Myalgia Muscle weakness Myoglobinuria
69
What can be complications of Rhabdomyolysis?
Acute renal failure | DIC
70
What is botulism- clostridium botulinum from?
Organism present in soil Food and wounds can become infected ALSO IV drug users-black tar heroin
71
How does botulism-clostridium botulinum present?
Rapid onset weakness without sensory loss
72
How does botulism-clostridium botulinum work its magic?
It cleaves presynaptic proteins involved in vesicle formation and blocks vesicle docking with the presynaptic membrane

ADELE-Noodles of Neurology (38 decks)

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