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Y2 Neurology > Muscle and Nerves Disease > Flashcards

Muscle and Nerves Disease Flashcards

1
Q

state some symptoms of muscle disease

A 
weakness 
short of breathe 
cardiopathy 
poor feeding in infants 
cramp 
pain 
myoglobinuria
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2
Q

what is myoglobinuria ?

A

is the presence of myoglobin in the urine, usually associated with rhabdomyolysis or muscle destruction. Myoglobin is present in muscle cells as a reserve of oxygen.

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3
Q

state three signs of muscle disease

A
  1. wasting/hypertrophy
  2. normal/reduced tone and reflexes
  3. motor weakness
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4
Q

what is the function of muscle ?

A

convert chemical energy to physical energy

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5
Q

what are the four components of the muscle contraction process?

A
  1. excitation contraction coupling
  2. contractile mechanism
  3. structural components
  4. energy system
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6
Q

could someone with muscle disease have a headache when they walk up in the morning?

A

yes because their intercostal muscles aren’t working properly so would have increased retention of CO2

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7
Q

what is myotonia ?

A

when the muscles contract but can’t relax again so are stiff

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8
Q

what does stiff muscles result in?

A

hypertrophy of muscles

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9
Q

is lack of reflexes usually due to muscles or nervous system?

A

nervous system

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10
Q

what investigations should be done for muscle diseases ?

A
  1. History and examination
  2. CK - blood test for enzyme in muscles
  3. EMG
  4. Muscle biopsy
  5. Genetic testing
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11
Q

what three things are usually tested for in a muscle biopsy ?

A

muscle structure
biochemistry
inflammation

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12
Q

what is the EMG test?

A

electromyography
- measures electrical activity in response to a nerve’s stimulation of the muscle. The test is used to help detect neuromuscular abnormalities.

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13
Q

what are the five classifications of muscle disease ?

A 
Muscular dystrophies
Channelopathies
Metabolic muscle disease
Inflammatory muscle disease
Congenital myopathies
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14
Q

out of the 5 classifications of muscle disease, which one is not hereditary ?

A

inflammatory muscle disease

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15
Q

what is the cell membrane which surrounds muscle called?

A

sarcolemma

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16
Q

what causes muscular dystrophies ?

A

when one protein controlling contraction isn’t functioning properly

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17
Q

give some examples of muscular dystrophies

A 
Duchenne’s MD
Becker’s MD
Facioscapulohumeral MD
Myotonic dystrophy 
Limb-Girdle MD
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18
Q

is myotonic dystrophy hereditary ?

A

yes

- it gets worse throughout generations

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19
Q

is limb girdle MD a single disease?

A

no

- it is a spectrum of diseases caused by about 30 gene mutations

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20
Q

is channelopathies common?

A

no

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21
Q

what is the cause of channelopathies ?

A

disorders of Ca, Na and Cl channels

- therefore the muscle can’t function properly

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22
Q

what happens to the muscle during Paramyotonia congenita and Myotonia congenita?

A

can’t relax properly

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23
Q

give three causes of metabolic muscle disease

A
  • disorder of carbohydrate metabolism
  • disorder of lipid metabolism
  • mitochondrial myopathies
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24
Q

what substance is broken down first in the body to get energy?

A

carbohydrate

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25
Q

after carbohydrates have been broken down with is then broken down to provide energy in the body?

A

lipids

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26
Q

if muscles aren’t provided with energy/ATP what occurs?

A

the muscle can’t contract as the muscle needs ATP to relax and to release the myosin form the actin

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27
Q

give two examples of inflammatory muscle disease

A

Polymyositis

Dermatomyositis

28
Q

do inflammatory muscle disease run in families ?

A

no

29
Q

do inflammatory muscle disease start suddenly?

A

yes

- over a few days or few weeks

30
Q

what two symptoms are common with inflammatory muscle diseases?

A

painful and weak muscles

purple rash around eyes and rash behind elbows and finger with dermatomyositis

31
Q

what age range are more likely to have inflammatory muscle disease?

A

can affect any age

32
Q

what affect can inflammatory muscle diseases have on the heart?

A

can affect the conducting system and cause arrhythmias

33
Q

would the CK of inflammatory muscle disease be increased or decreased ?

A

increased CK

34
Q

what investigations should be done for inflammatory muscle disease ?

A

EMG
Biopsies
CK blood test

35
Q

what is the treatment of inflammatory muscle disease ?

A

immunosuppression

36
Q

what does the enzyme acetylcholinesterase do?

A

breaks down ACh in the synaptic cleft to stop APs being stimulated

37
Q

what is the common disorder of the neuromuscular junction called?

A

myasthenia gravis

38
Q

what is the main clinical sign of myasthenia gravis?

A

muscles get tired quickly

39
Q

what are the main symptoms of myasthenia gravis ?

A 
limb weakness 
ptosis 
chewing weakness 
slurred talking after a few sentences/words 
SOB 
diplopia
40
Q

what causes myasthenia gravis ?

A
  • the antibody binds to the ACh receptors are the post ganglionic terminal
  • therefore, less ACh can bind and trigger APs to be fired
  • therefore, initial contraction is present but continued contraction can’t be maintained
41
Q

what are 3 treatment options for myasthenia gravis ?

A

immunosupressors

acetylcholinesterase inhibitor

steroids

42
Q

what 5 investigations can be done for myasthenia gravis ?

A 
AChR ab 
Anti MuSK ab 
EMG/repetitive stimulation 
Tensilon test 
CT chest
43
Q

what does the tension test involve ?

A
  • Short acting inhibtor of acetylcholinesterase
  • Will relieve symptoms for 1 min
  • Used for a diagnositic test
44
Q

what doe the repetitive stimulation test involve ?

A

Send lots of APs to tire out the muscle and the contraction will get less and less every time

45
Q

what conditions are associated with myasthenia gravis in young and older people?

A

young = thymic hyperplasia

older = malignant thymoma

46
Q

what surgical option is there for treatment of myasthenia gravis ?

A

thymectomy

47
Q

what sensory axons are larger ?

  • pain/temperature
  • proprioception/vibration
A

proprioception/vibration

48
Q

state two causes of lesion of an individual peripheral nerve ?

A

compression

inflammation (vasculitic)

49
Q

state three causes of spinal root disease

A
  • degenerative spine disease
  • inflammation
  • infiltration
50
Q

give some examples of disease which cause inflammation in spinal roots?

A

lymes disease
shingles
HIV

51
Q

give the two main causes of peripheral nerve disease

A
  • alcohol

- diabetes

52
Q

what investigations can be done for nerve disease?

A 
blood tests
- LFTs, glucose 
genetic analysis 
NCS 
lumbar puncture 
nerve biopsy
53
Q

what is a good way to determine whether a nerve disease condition is hereditary or not?

A

the feet will not be normal if it is hereditary because development wouldn’t have been normal

54
Q

a cause of nerve disease can be inflammatory demyelination. give an example of an acute and chronic example…

A

acute = Guillain Barre Syndrome

chronic = CIDP

55
Q

what symptoms would be seen if a nerve root was damaged ?

A

muscle wasting and weakness
dermatomal sensory change
reflex change

56
Q

what symptoms would be seen if a individual nerve was damaged ?

A

wasting and weakness in innervated muscle

specific sensory change

57
Q

what clinical sign would be seen with UMN disease?

A

brisk reflexes

increased tone

58
Q

what clinical sign would be seen with LMN disease?

A

muscle wasting
muscle weakness
muscle fasciculations

59
Q

does UMN diseases sometimes have mixed symptoms which may indicate a LMN disease?

A

yes

60
Q

why might a UMN disease have symptoms of a LMN disease?

A

because UMN diseases may involve the anterior horn head of the lower motor neuron so muscle wasting may also be seen

61
Q

what type of muscle disease is amyotrophic lateral sclerosis ?

A

motor neuron disease

62
Q

what are the clinical signs of amyotrophic lateral sclerosis ?

A

starts in one limb progressively moving over to contralateral limb then starts to involve breathing and swallowing
- muscle weakness and atrophy

63
Q

what is the prognosis from symptom onset?

A

3-5yrs

64
Q

what is the prognosis from diagnosis ?

A

2-3yrs

65
Q

how many patients with motor neuron disease will have died within 14 months of diagnosis ?

A

50%

66
Q

what is the treatment options for motor neuron disease?

A

medication
- riluzole (trials)

supportive

  • physical therapist
  • Non Invasive Ventilation
  • Percutaneous endoscopic gastrostomy

Y2 Neurology (52 decks)

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