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
after carbohydrates have been broken down with is then broken down to provide energy in the body?
lipids
26
if muscles aren't provided with energy/ATP what occurs?
the muscle can't contract as the muscle needs ATP to relax and to release the myosin form the actin
27
give two examples of inflammatory muscle disease
Polymyositis | Dermatomyositis
28
do inflammatory muscle disease run in families ?
no
29
do inflammatory muscle disease start suddenly?
yes | - over a few days or few weeks
30
what two symptoms are common with inflammatory muscle diseases?
painful and weak muscles | purple rash around eyes and rash behind elbows and finger with dermatomyositis
31
what age range are more likely to have inflammatory muscle disease?
can affect any age
32
what affect can inflammatory muscle diseases have on the heart?
can affect the conducting system and cause arrhythmias
33
would the CK of inflammatory muscle disease be increased or decreased ?
increased CK
34
what investigations should be done for inflammatory muscle disease ?
EMG Biopsies CK blood test
35
what is the treatment of inflammatory muscle disease ?
immunosuppression
36
what does the enzyme acetylcholinesterase do?
breaks down ACh in the synaptic cleft to stop APs being stimulated
37
what is the common disorder of the neuromuscular junction called?
myasthenia gravis
38
what is the main clinical sign of myasthenia gravis?
muscles get tired quickly
39
what are the main symptoms of myasthenia gravis ?
``` limb weakness ptosis chewing weakness slurred talking after a few sentences/words SOB diplopia ```
40
what causes myasthenia gravis ?
- 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
what are 3 treatment options for myasthenia gravis ?
immunosupressors acetylcholinesterase inhibitor steroids
42
what 5 investigations can be done for myasthenia gravis ?
``` AChR ab Anti MuSK ab EMG/repetitive stimulation Tensilon test CT chest ```
43
what does the tension test involve ?
- Short acting inhibtor of acetylcholinesterase - Will relieve symptoms for 1 min - Used for a diagnositic test
44
what doe the repetitive stimulation test involve ?
Send lots of APs to tire out the muscle and the contraction will get less and less every time
45
what conditions are associated with myasthenia gravis in young and older people?
young = thymic hyperplasia older = malignant thymoma
46
what surgical option is there for treatment of myasthenia gravis ?
thymectomy
47
what sensory axons are larger ? - pain/temperature - proprioception/vibration
proprioception/vibration
48
state two causes of lesion of an individual peripheral nerve ?
compression | inflammation (vasculitic)
49
state three causes of spinal root disease
- degenerative spine disease - inflammation - infiltration
50
give some examples of disease which cause inflammation in spinal roots?
lymes disease shingles HIV
51
give the two main causes of peripheral nerve disease
- alcohol | - diabetes
52
what investigations can be done for nerve disease?
``` blood tests - LFTs, glucose genetic analysis NCS lumbar puncture nerve biopsy ```
53
what is a good way to determine whether a nerve disease condition is hereditary or not?
the feet will not be normal if it is hereditary because development wouldn't have been normal
54
a cause of nerve disease can be inflammatory demyelination. give an example of an acute and chronic example...
acute = Guillain Barre Syndrome chronic = CIDP
55
what symptoms would be seen if a nerve root was damaged ?
muscle wasting and weakness dermatomal sensory change reflex change
56
what symptoms would be seen if a individual nerve was damaged ?
wasting and weakness in innervated muscle | specific sensory change
57
what clinical sign would be seen with UMN disease?
brisk reflexes | increased tone
58
what clinical sign would be seen with LMN disease?
muscle wasting muscle weakness muscle fasciculations
59
does UMN diseases sometimes have mixed symptoms which may indicate a LMN disease?
yes
60
why might a UMN disease have symptoms of a LMN disease?
because UMN diseases may involve the anterior horn head of the lower motor neuron so muscle wasting may also be seen
61
what type of muscle disease is amyotrophic lateral sclerosis ?
motor neuron disease
62
what are the clinical signs of amyotrophic lateral sclerosis ?
starts in one limb progressively moving over to contralateral limb then starts to involve breathing and swallowing - muscle weakness and atrophy
63
what is the prognosis from symptom onset?
3-5yrs
64
what is the prognosis from diagnosis ?
2-3yrs
65
how many patients with motor neuron disease will have died within 14 months of diagnosis ?
50%
66
what is the treatment options for motor neuron disease?
medication - riluzole (trials) supportive - physical therapist - Non Invasive Ventilation - Percutaneous endoscopic gastrostomy