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Neuro Pathology > Muscle and Nerve Diseases > Flashcards

Muscle and Nerve Diseases Flashcards

1
Q

Examples of muscles and nerve diseases; disorders of….

A

Muscle
NMJ
Peripheral nerve
Anterior horn cell

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2
Q

What does NMJ stand for?

A

Neuromuscular junction

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3
Q

Definition of a muscle

A

An intricate machine designed to convert chemical energy to mechanical energy

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4
Q

Presentation of muscle disease

A 
Poor suck / feeding / failure to thrive / floppy
Weakness of skeletal muscle 
SOB (resp muscles)
Poor swallow (aspiration)
Cardiomyopathy 
Cramp, pain, myoglobulinuria 
Wasting / hypertrophy of muscle
Normal or reduced tone or reflexes
Motor weakness
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5
Q

Definition of myoglobulinuria

A

Peeing black urine after exercise

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6
Q

Do you get sensory weakness in muscle disease?

A

NO

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7
Q

Classification of muscle diseases

A 
Muscle dystrophies
Channelopathies
Metabolic muscle disease
Inflammatory muscle disease
Congential myopathies
Iatrogenic - medication
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8
Q

How do most people get muscular dystrophies?

A

Genetic

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9
Q

What pattern of symptoms do you get with channelopathies?

A

Episodic symptoms

- completely fine, then an episodes and then back to completely fine

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10
Q

Types of muscular dystrophies

A 
Duchenne's MD
Becker's MD
Facioscapulohumeral MD
Myotonic dystrophy 
Limb Girdle MD
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11
Q

What happens in both duchennes and beckers MD?

A

Hypertrophy of the muscles
Infiltration of fat
Trouble of standing to sitting

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12
Q

Presentation of facioscapulohumeral MD

A

Facial weakness
Biceps
One other upper limb and lower limb proximal weakness

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13
Q

What can go wrong to cause muscle channelopathies?

A

Disorders of channels

  • Ca
  • Na
  • Cl
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14
Q

What is the most common muscle channelopathy?

A

Familial hypokalaemia periodic paralysis

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15
Q

Types of muscle channelopathies

A

Familial hypokalaemia periodic paralysis
Hyperkalaemic periodic paralysis
Paramyotonia congenita
Myotonia congenita

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16
Q

In muscle channelopathies when they are having an episode and are weak, what is the key thing to measure? Why?

A

Potassium

Shows organic disease

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17
Q

Presentation of myotonia congenita

A

Bit of weakness

Muscles cannot relax after contract muscle - therefore feel very stiff

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18
Q

Types of metabolic muscle disease

A 
Disorders of carbohydrate metabolism 
Disorders of lipid metabolism 
Mitochondrial myopathies/cytopathies
Endocrinopathy 
Biochemical abnormalities
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19
Q

How does disorders of carbohydrate metabolism present?

A

Low level weakness with much more marked weakness on exercise

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20
Q

Give an example of an endocrinopathy condition that can give you muscle problems and how it does so

A

Cushings

- adrenal (production of steriods) causing the weakness - a steriod induced myopathy

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21
Q

What biochemical abnormality can give you muscle weakness?

A

Low potassium

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22
Q

What molecule is used for immediate rapid muscle activity e.g. sprinting?

A

Glucose

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23
Q

What is used up when muscles are used?

A

Energy

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24
Q

Examples of inflammatory muscle diseases

A

Polymyositis

Dermatomyositis

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25
Q

What does polymyositis affect?

A

ONLY muscles

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26
Q

What does dermatomyositis affect?

A

Skin

Muscles

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27
Q

What type of disorder, apart from inflammatory, is dermatomyositis?

A

Paraneoplastic

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28
Q

Presentation of inflammatory muscle disease

A

Painful weak muscles
Muscles sore to touch
If DM - characteristic rash
Feeling generally unwell

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29
Q

What age can get inflammatory muscle disease?

A

Any age

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30
Q

What is the characteristic rash of dermatomyositis?

A

Rash around eyes and knuckles

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31
Q

If a patient presents with painful weak muscles and a rash around eyes/knuckles, what should be done?

A

Look for an underlying tumour

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32
Q

Investigations of inflammatory muscle disease

A

Increased CK
EMG
Muscle biopsy

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33
Q

What would be find on biopsy in polymyositis?

A

CD8 cells

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34
Q

What would be found on biopsy in dermatomyositis?

A

Humeral mediated, B cells and CD4 cells

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35
Q

Treatment of inflammatory muscle diseases

A

Steriods

If dont work then a steriod suppressing immunosuppressant

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36
Q

When is CK in the blood elevated?

A

When there is a muscle disorder

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37
Q

Investigation of muscle diseases

A 
History and exam 
CK
EMG
Muscle biopsy 
Genetic testing
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38
Q

What does the muscle biopsy look for?

A

Structure
Biochemistry
Inflammation

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39
Q

Give an example of a disorder of the NMJ

A

Myastenia gravis

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40
Q

Presentation of myastenia gravis

A

Fatiguable weakness

  • eyelids (ptosis)
  • proximal muscle weakness (face, neck, limbs)
  • muscles of mastication (chewing, swallowing)
  • talking
  • SOB
  • Diplopia
  • dysphagia
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41
Q

Investigation of myastenia gravis

A

Single fibre electromyography
AChR ab (85 - 90%)
Anti MuSK ab (40%)
Neurophysiology (repetitive stimulation, jitter)
CT chest (thymoma)
Tensilon test; IV edrophonium reduces muscle weakness temporarily (not commonly used anymore due to risk of cardiac arrhythmia)

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42
Q

Treatment of myastenia gravis

A

Symptomatic - long acting acetylcholinesterase inhibitor e.g. pyridostigmine
Prednisolone or a steriod saving agent (to immunosuppress originally)
Thymectomy

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43
Q

Example of a steriod saving immunosuppresant

A

Azathioprine

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44
Q

How does an Ach Inhibitor give symptomatic relief in myastenia gravis?

A

Blocks the breakdown of Ach do have more of it at your endplate

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45
Q

How does immunoglobulin / plasma exchange work in myastenia gravis?

A

Immunoglobulin binds to the antibody and stops it binding to the NMJ

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46
Q

What does fatiguable weakness mean?

A

As you use the muscle, it is strong then gets weaker

Therefore muscles are fine in the morning but develop weakness later on in the day

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47
Q

Pathology of myastenia gravis

A

Anti AChR antibody blocks AchR so there is no muscle contraction
Flooded with ACh to outcompete this antibody but as the muscle fatigues it cannot do this as well

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48
Q

Where is the anterior horn cell found?

A

Between the UMN and LMN

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49
Q

What type of neurones are present in the LMN / PNS?

A

Both motor and sensory

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50
Q

What does peripheral nerve disease consist of?

A

Sensory axons

Autonomic nervous system

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51
Q

Types of sensory axons

A

Small fibres

Large fibres

52
Q

What do small sensory fibres carry?

A

Pain

Temperature

53
Q

What do large sensory fibres carry?

A

Joint position sense and vibration

54
Q

Example of root disease

A

Degenerative spine disease

55
Q

Examples of a lesion of an individual peripheral nerve

A

Compressive/entrapment neuropathy (entrapment e.g. carpal tunnel syndrome)
Vasculitis (mononeuritis multiplex) - if multiple nerves affected due to if blood vessels become inflamed/enlarged

56
Q

Where does generalised peripheral neuropathy tend to present first?

A

Feet THEN hands

57
Q

Presentation of generalised peripheal neuropathy

A

Motor
Sensory
Both
+/- autonomic features

58
Q

Causes of generalized peripheral neuropathy

A 
Metabolic 
- DM
- alcohol 
- renal 
- B12
Toxic - drugs
Hereditary 
Infectious
- Lymes
- HIV
- leprosy 
Malignancy 
Inflammatory demyelinating
- Acute - GBS
- chronic - chronic inflammatory demyelinating polynueropathy
59
Q

What does GBS stand for?

A

Guillian Barre syndrome

60
Q

Presentation of a nerve root disorder

A

Myotomal wasting and weakness
Reflex change
Dermatomal sensory change

61
Q

Presentation of disorder of an individual nerve

A

Wasting and weakness of innervated muscle

Specific sensory change

62
Q

Presentation of generalised peripheral neuropathy

A

Sensory and motor symptoms starting distally and moving proximally

63
Q

Investigations of peripheral neuropathy

A 
Blood tests
- vit B 12 deficiency 
- LFTs
Genetic analysis (only if clear FH)
Nerve conduction studies
LP
Nerve biopsy
64
Q

Why do we tend to avoid nerve biopsies?

A

As could leave someone with a deficit

65
Q

Presentation of MND

A

Combination of UMN and LMN signs
- UMN = increased tone, brisk reflexes, spasticity
- LMN = muscle fasiculations, wasting, weakness
No sensory involvement

66
Q

Is there sensory involvement in MND?

A

No

67
Q

Prognosis of MND

A

Die 2 - 5 years from symptom onset

Die 2 - 3 years from diagnosis

68
Q

What % of patients die within 14 months of their diagnosis?

A

50%

69
Q

What is usually the cause of death in MND?

A

Respiratory failure

70
Q

Investigations of MND

A

Presentation - the unique combination of UMN and LMN signs

EMG

71
Q

Treatment of MND

A 
Supportive
- PEG
- NIV
- Physio / OT / care
Riluzole
72
Q

How does riluzole work?

A

Anti glutamate antagonist - blocks the action of glutamate

Usually used in amyotrophic lateral sclerosis

73
Q

Effects of riluzole on MND

A

Prolongs survival by a few months

N.B. some trials say it does nothing

74
Q

Types of MND

A

Amyotrophic lateral sclerosis (50%)
Primary lateral sclerosis
Progressive muscle atrophy
Progressive bulbar palsy

75
Q

Clues that would point towards a diagnosis of MND

A

Fasciculations
Absence of sensory symptoms
Mixture of UMN and LMN signs
Wasting of small hand muscles / tibialis anterior is common

76
Q

Other common features of MND

A

Does NOT affect the external ocular muscles
NO cerebellar signs
Abdominal reflexes are usually preserved and sphincter dysfunction if present is a late feature

77
Q

Features of amyotrophic lateral sclerosis

A

Typically LMN signs in the arms and UMN signs in the legs

78
Q

What is the genetic abnormality in inherited amyotrophic lateral sclerosis?

A

Superoxide dismutase - on chromosome 21

79
Q

Features of primary lateral sclerosis

A

UMN signs only

80
Q

Features of progressive muscle atrophy

A

LMN signs only

Affects distal muscles before proximal

81
Q

What type of MND carries the best prognosis?

A

Progressive muscle atrophy

82
Q

Features of progressive bulbar palsy

A

Palsy of tongue, muscles of chewing/swallowing and facial muscles due to loss of function of brainstem motor nuclei

83
Q

What MND type carries the worst prognosis?

A

Progressive bulbar palsy

84
Q

Age of onset Beckers vs Duchennes

A

Duchennes - onset < 5 y/o

Beckers - onset > 10 y/o

85
Q

Which of beckers and duchennes has LD?

A

Duchennes

86
Q

Which of beckers and duchennes are associated with dilated cardiomyopathy?

A

Duchennes

87
Q

Treatment for restless leg syndrome

A

Ropinirole

88
Q

CK level in myasthesia gravis

A

Normal

89
Q

Management of myasthenic crisis

A

Plasmapheresis

IV immunoglobulins

90
Q

When does myotonic dystrophy occur?

A

20 - 30 y/o

91
Q

What types of muscle does myotonic dystrophy affect?

A

Skeletal
Cardiac
Smooth

92
Q

Two main types of myotonic dystrophy

A

DM1

DM2

93
Q

Inheritance of myotonic dystrophy

A

Autosomal Dominant

94
Q

Genetics of each type of Myotonic dystrophy

A

DM1 - CTG repeat at the end of DMPK gene on chromosome 19

DM2 - repeat expansion of the ZNF9 gene on chromosome 3

95
Q

Main differences between DM1 and DM2

A

DM1 - distal weakness more prominent

DM2 - proximal weakness more prominent, and severe congenital form NOT seen

96
Q

Presentation of myotonic dystrophy

A 
Myotonic facies (long "'haggard" appearance)
Frontal balding
Bilateral ptosis
Cataracts
Dysarthria 
Myotonia
Weakness of limbs - distal initially
Mild mental impairment
DM
Testicular atrophy 
Cardiac involvement - heart block, cardiomyopathy 
Dysphagia
97
Q

What is Eaton-Lambert syndrome associated with?

A

Small cell lung cancer
Ovarian cancer
Breast cancer
May also occur independently as an autoimmune disorder

98
Q

Pathology of Eaton Lambert syndrome

A

Antibody directed against presynaptic voltage gated calcium channel in the PNS

99
Q

Presentation of Eaton Lambert syndrome

A 
Repeated muscle contractions lead to an increase in muscle strength (only seen in 50%)
Limb girdle weakness (affects LLs first)
Hyporeflexia
Autonomic symptoms
- dry mouth 
- impotence
- difficulty micturating
100
Q

Investigations of Eaton Lambert syndrome

A

EMG

- incremental response to electrical stimulation

101
Q

Treatment of Eaton Lambert syndrome

A

Treatment of underlying cancer
Immunosuppression (e.g. with pred or azathioprine)
IV immunoglobulins and plasma exchange

102
Q

MRC power scores

A

0 - no muscle movement
1 - trace of contraction
2 - movement at the joint with gravity eliminated
3 - Movement against gravity, but not against added resistance
4 - movement against external resistance with reduced strength
5 - Normal strength

103
Q

What MRC power score indicates that the muscle can only work with gravity removed?

A

2

104
Q

Involvement of T1 in erbs palsy may result in what?

A

Horners syndrome

105
Q

Damage to the ulnar nerve results in what?

A

Wasting of hypothenar muscles
Loss of thumb adduction
Wasting of 1st webspace
Ulnar claw hand (hyperextension at metacarpal phalangeal joint, flexion at interphalangeal joint)

106
Q

What does the straight leg test test for?

A

Radiculopathy

107
Q

What would radial nerve palsy result in?

A

Wrist drop

Loss of sensation from 1st dorsal web space

108
Q

What does musculocutaneous nerve palsy result in?

A

Reduced flexion at elbow

Loss of supination

109
Q

What does median nerve compression result in?

A

Carpal tunnel syndrome

110
Q

What does axillary nerve palsy result in?

A

Wastage of deltoid muscles

Loss of sensation in the badge area

111
Q

Lesions where will cause problems with finger abduction?

A

T1

112
Q

What does a +ve hoffmans sign indicate?

A

UMN lesion

113
Q

Treatment for ocular myasthenia gravis

A

Pyridostigmine

114
Q

What is mononeuritis multiplex?

A

A simultaneous or sequential involvement of individual non contiguous nerve trunks

115
Q

Presentation of mononeuritis multiplex

A 
Acute or subacute loss of sensory and motor function of individual nerves
Asymmetric 
As disease progresses
- confluent
- symmetrical
116
Q

What is mononeuritis multiplex hard to differentiate from as the disease becomes more advanced?

A

Polyneuropathy

117
Q

What suggests GBS?

A

Progressive peripheral polyneuropathy with hyporeflexia

118
Q

What is Saturday night palsy?

A

Compression of the radial nerve against the humeral shaft. Results in unable to extend wrist
Possibly due to sleeping on a hard chair with hand draped over the back

119
Q

What is internuclear opthalmoplegia?

A

Occurs due to a lesion of the median longitudinal fasiculus, tract that allows to conjugate eye movements.

120
Q

What does internuclear opthalmoplegia result in?

A

Impairment of adduction of ipsilateral eye

The contralateral eye abducts, however with nystagmus

121
Q

Causes of internuclear opthalmoplegia

A

MS

Vascular disease

122
Q

What are common precipitators of myasthenia crisis?

A

Beta blockers

123
Q

Treatment of myasthenia crisis

A

Plasmaphresis and IV immunoglobulin

124
Q

What does hoovers sign differentiate between?

A

Between organic and non organic leg weakness

125
Q

What is the Jendrassik manouvre?

A

Compares a reflex with/without distraction e.g. clenching teeth

126
Q

What does a positive rhombergs test indicate?

A

Sensory ataxia

Neuro Pathology (21 decks)

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