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N - C > Nerve and Muscle Disease > Flashcards

Nerve and Muscle Disease Flashcards

1
Q

Describe the basic route for the movement of a muscle

A 
Upper Motor Neuron
Anterior Horn Cell
Lower Motor Neuron
NMJ
ACh release
ACh receptor
Muscle stimulated
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2
Q

What are these disorders, disorders of?

A

Muscle
Neuromuscular junction
Peripheral nerve
Anterior horn cell

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3
Q

What is muscle?

A

An intricate machine designed to convert chemical energy to mechanical energy

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4
Q

What are the components of the process for chemical energy -> mechanical energy

A

Excitation-contraction coupling

The contractile mechanism

Structural components

The energy system

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5
Q

What is the presentation of muscle disease?

A
  • Weakness of skeletal muscle
  • Short of breath (respiratory muscle)
  • Cardiomyopathy
  • Poor suck/ feeding/ Failure To Thrive/ Floppy
  • Cramp, pain
  • Myoglobinuria
  • Wasting/ hypertrophy
  • Motor weakness… NOT sensory
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6
Q

Explain why myoglobinuria occurs and what the results of it are

A

Muscles break down
Myglobin in blood
Filtered by kidneys
Makes urine look like coca cola

Large molecule so can clog kidneys up in large amounts -> renal failure

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7
Q

How are muscle diseases classified?

A
  • Muscular dystrophies
  • Channelopathies
  • Metabolic muscle disease
  • Inflammatory Muscle disease
  • Congenital myopathies
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8
Q

What parts can fail in muscle disease

A 
Dystrophin
Actin
Sarcoglycan complex
Systroglycan complex
Laminin
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9
Q

Name the muscular dystrophies

A 
Duchenne's MD
Becker's MD
Facioscapulohumeral MD
Myotonic Dystrophy
Limb-Girdle MD
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10
Q

What is Duchenne’s MD?

A

X linked
No functional muscular dystrophin produced

Poor motor milestones
(arent walking when young, wheelchair by teens)

Investigations:
CK, Genetics, Muscle biopsy

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11
Q

What is Becker’s MD?

A

Allelic with DMD
Cardiomyopathy

Some dystrophin produced so less severe than DMD

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12
Q

What is Facioscapulohumeral MD?

A

Autosomal dominant

Affects the skeletal muscles of the face, scapula and upper arms initially

Usually present in teens

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13
Q

What is myotonic dystrophy?

A

Autosomal dominant trinucleotide repeat disorder

Multisystem disorder characterised by weakness and myotonia

myotonia = hard to relax muscles once contracted

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14
Q

What is limb-girdle MD?

A

Large family of autosomal dominant and recessive disorders affecting primaryily the limb girdles

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15
Q

What are channelopathies?

A

Disorders of Ca, Na and Cl channels

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16
Q

How are channelopathies divided?

A

Periodic paralysis:

  • Familial hypokalemic periodic paralysis
  • Hyperkalemic periodic paralysis

Partial Paralysis:

  • Paramyotonia congenita
  • Myotonia congenita
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17
Q

What is the difference between paramyotonia congenita and myotonia congenita?

A

Myotonia = the more you move the easier it gets

Paramyotonia = the more you move the harder it gets

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18
Q

What is metabolic muscle disease?

A

Muscle isnt supplied very well with what it needs to function.

Muscle breakdown during exercise is therefore excessive

Myogobinuria can cause renal failure

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19
Q

How can someone with metabolic muscle disease present?

A

Muscle pain (particularly after exercise) which is unusually excessive

Coca Cola urine

Renal failure

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20
Q

How is metabolic muscle disease divided?

A

Disorders of carbohydrate metabolism

DIsorders of lipid metabolism

Mitochondrial myopathies/ cytopathies

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21
Q

How can a patient get around disorders of carbohydrate metabolism?

A

Push through during exercise.

After 10-15 mins start to break down fat stores so will get “second wind”

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22
Q

What will mitochondrial myopathies/ cytopathies effect?

A

Many different systems, not just skeletal

23
Q

What is Myositis?

A

Inflammation of the muscles

24
Q

What is Polymyositis?

A

Inflammation of all the muscles

25
Q

What is dermatomyositis?

A

Inflammation of muscles and skin

Gives a characteristic rash

26
Q

Describe inflammatory muscle disease

A 
Acute or subacute
Painful weak muscles
Characteristic rash of DM
Any age
Other symptoms may be involved
27
Q

What is the characteristic rash of dermatomyositis?

A

Gottron’s sign = erythematous, scaly eruption occurring in symmetric fashion over the Metacarpal and interphalangeal joints (can mimic psoriasis).

The heliotrope or “lilac” rash = eruption on the upper eyelids and in rare cases on the lower eyelids as well, often with itching and swelling

28
Q

What are the investigations for inflammatory muscle disease?

A

History and examination
Increased CK
EMG

Biopsy

  • PM = CD8 cells
  • DM = humeral-mediated, B cells and CD4 cells

Genetic testing (more common than biopsy nowadays)

29
Q

What is the treatment for inflammatory muscle disease?

A

Immunosuppression

30
Q

What is EMG?

A

Electromyography

Evaluates and records the electrical activity produced by skeletal muscles.

Can see if muscle is normal or abnormal
Can see if the problem is msucle or nerve

31
Q

Describe the function of the NMJ in normal patients

A

Ca mediated exocytosis of ACh
Binds to ACh receptor
Anticholinesterase breaksdown ACh so that muscle can relax

32
Q

What happens to the NMJ in Myasthenia Gravis?

A

Autoantibodies block ACh receptors.
Less receptors so ACh less effective

Patients are fatiguable

33
Q

What is the clinical presentation of Myasthenia Gravis?

A

Fatiguable Weakness:

  • Limbs
  • Eyelids (ptosis)
  • Muscles of mastication (chewing is tiring, may switch diet to easier foods)
  • Talking
  • SOB
  • Diplopia
34
Q

What is the investigations into myasthenia gravis?

A

AChR antibody
Anti MuSK antibody
EMG
Tensilon test

CT chest

35
Q

What is the tensilon test?

A

Nerve conduction of which you then look at the action potential

In myasthenia gravis you do repetative stimulation and AP will get smaller and smaller

36
Q

What is the treatment for Myasthenia gravis?

A

Symptomatic
-Acetylcholinesterase inhibitor

Immunosuppression:

  • Prednisolone
  • Steroid saving agent (azathioprine)
37
Q

Explain myasthenia gravis and the thymus

A

Must check thymus in MG

  • In young associated with thymic hyperplasia
  • In old may be associated with a malignant thymoma

?Thymectomy
-Females

38
Q

What does peripheral nerve disease involve?

A

Lower motor neuron

Sensory axons:

  • Small fibres (pain + temperature)
  • Large fibres (joint position sense + vibration)

Motor axons

39
Q

How is peripheral nerve disease divided?

A

Root disease

Lesion of individual peripheral nerve

Generalised peripheral neuropathy

40
Q

What is Root nerve disease?

A

Degenerative spine disease
Nerve root disorders are precipitated by acute or chronic pressure on a root in or adjacent to the spinal column. The most common cause is
A herniated intervertebral disk (C5, 6, 7 or L5, S1)

Bone changes due to RA or osteoarthritis, especially in the cervical and lumbar areas, may also compress isolated nerve roots. (outside of load bearing areas

41
Q

What are the symptoms of root nerve disease?

A

Dermatomal sensory change

Myotomal wasting and weakness

Fasciculations

42
Q

What is a lesion of an individual peripheral nerve?

A

Compressive/ entrapment neuropathy

Vasculitis (mononeuritis multiplex)

43
Q

What are the symptoms of a lesion of an individual peripheral nerve?

A

Specific sensory change

Wasting and weakness of innervated muscle

Fasciculations

44
Q

What may generalised peripheral neuropathy involve?

What is it?

A

Motor/ sensory/ both
+/- autonomic features

Nerves start to die back from periphery

45
Q

What are the causes of Generalised peripheral neuropathy?

A

Metabolic: DIABETES, ALCOHOL, B12

Toxic: drugs

Infectious: HIV, leprosy, Lymes disease

Malignancy: paraneoplastic

Inflammatory demyelination

  • Acute: Guillain Barre syndrome
  • Chronic = CIDP
46
Q

What does CIDP stand for?

A

Chronic inflammatory demyelinating polyneuropathy

47
Q

What are the symptoms of general peripheral neuropathy?

A

sensory and motor symptoms starting distally and moving proximally

48
Q

What are the investigations for nerve disease?

A 
Blood tests
Genetic analysis
EMG
Lumbar puncture (CSF analysis)
Nerve biopsy
49
Q

What does anterior horn disease affect?

A

Upper motor neurone in corticospinal or corticobulbar tracts

Anterior horn cell

50
Q

Describe Motor Neuron Disease (Amyotrophic Lateral Sclerosis)

A

Usually limb onset, later bulbar (muscles of mouth and throat) and respiratory involvement

Combination of UMN and LMN signs
-The more mixed the worse the prognosis

M:F 3:2

Terrible prognosis

51
Q

What lower motor neuron symptoms may you see in ALS?

A

Wasting
weakness
muscle fasciculations

52
Q

What upper motor neuron symptoms may you see in ALS?

A

Pyramidal weakness
Stiff
Brisk reflexes

53
Q

How do you diagnose Motor neuron disease?

A

Unique combination of UMN and LMN signs

EMG

54
Q

What is the treatment of ALS?

A

Supportive

  • counseling
  • multi diciplinary clinic

riluzole

N - C (35 decks)

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