muscle and nerve disease Flashcards

1
Q

what is the function of a muscle?

A

Intricate machine designed to convert chemical energy → mechanical energy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

what are the components of this process:

Intricate machine designed to convert chemical energy → mechanical energy

A

Structural components
Contractile mechanism
Excitation-contraction coupling (ion channels)
Energy system

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

what are the symptoms of muscle disease?

A

Weakness of skeletal muscle
Short of breath (respiratory muscles)
Poor swallow / aspiration (bulbar muscles)
Cardiomyopathy
Cramp, pain, stiffness, myoglobinuria
(Babies: poor suck / feeding / failure to thrive / floppy)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

what are the signs of muscle disease?

A

Wasting / hypertrophy
Normal or reduced tone and reflexes
Motor weakness…NOT sensory

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

what are the investigations for muscle disease

A
History and examination
Creatine Kinase (CK)
EMG
Muscle biopsy
Structure
Biochemistry
Inflammation
Genetic testing
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

what are congenital classifications of muscle disease?

A

Structural: muscular dystrophies

Contractile: congenital myopathies

Coupling: channelopathies

Energy: enzymes / mitochondria

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

what are acquired classifications of muscle disease?

A

Metabolic (Ca2+, K+)
Endocrine (thyroid, adrenal, vit D)
Inflammatory muscle disease
Iatrogenic: medication (steroids / statins)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

are there specific treatments for muscular dystrophies?

A

no

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

what is channelopathies?

A

disorders of Ca2+, Na+, K+ and Cl- channels
often paroxysmal symptoms e.g. periodic paralysis / myotonia
partially treatable

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

what is metabolic myopathy?

A

Exercise induced: early = disorders of carbohydrate metabolism
late = disorders of lipid metabolism

Mitochondrial problems
partially treatable

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

what are Polymyositis and dermatomyositis?

A

are disorders of the body’s connective tissues, which include tendons, ligaments and the dense sheets of collagen-based tissue that cover the ends of the muscles. These diseases cause swelling and tenderness in the muscles (polymyositis) and sometimes the skin (dermatomyositis).

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

what separates polymmyositis and dermatomyositis?

A

Characteristic rash of DM

High CK, autoantibodies, tumour screen (esp DM)
EMG & biopsy
Polymyositis: CD8 cells
Dermato: humeral-mediated, B cells and CD4 cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

what is the treatment for Polymyositis and dermatomyositis (DM)

A

immunosuppression

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

what is the clinical presentation myasthenia gravis?

A

Fatiguable weakness

Ocular: eyelids (ptosis)
muscles of eye movement (diplopia)

Generalised: limbs
bulbar (chew, swallow, talk)
breathing

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

what are the investigations for myasthenia gravis?

A

AChR, Anti MuSK antibodies
Neurophysiology (Repetitive stimulation, Jitter)
CT chest (thymoma)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

what is the treatment for myasthenia gravis?

A
Symptomatic
Acetylcholinesterase inhibitor (pyridostigmine)

Immunosuppression
Prednisolone
Steroid saving agent (e.g. azathioprine)

Immunoglobulin / plasma exchange

Thymectomy

17
Q

what does the peripheral nerve consist of?

A
Sensory axons
Small fibres (pain + temperature)
Large fibres (joint position sense + vibration)
Motor axons
Autonomic axons
Nerve sheath (myelin)
18
Q

what type of disease is a nerve disease?

A

root disease

19
Q

causes of generalised peripheral neuropathy

A

Hereditary

Metabolic: diabetes, alcohol, renal failure, B12

Toxic: drugs

Infectious: Lyme, HIV, leprosy

Malignancy: paraneoplastic

Inflammatory demyelinating:
Acute = Guillain Barre syndrome
Chronic = chronic inflammatory demyelinating polyneuropathy

20
Q

symptoms and signs of nerve root?

A

Myotomal wasting and weakness
Reflex change
Dermatomal sensory change

21
Q

symptoms and signs of individual nerve of nerve disease

A

Wasting and weakness of innervated muscle

Specific sensory change

22
Q

symptoms and signs of generalised peripheral neuropathy

A

Sensory and motor symptoms, usually starting distally and moving proximally

23
Q

investigations for nerve diseases?

A
Blood tests
Genetic analysis
Nerve Conduction Studies
Lumbar puncture (CSF analysis)
Nerve biopsy (nb sensory nerve)
24
Q

treatment of nerve disease?

A

Treat the cause eg stop drug, surgery, immunoglob

25
Q

diagnosis of motor neuron disease

A

Unique combination of UMN + LMN signs with no sensory signs

EMG

26
Q

treatment of motor neuron disease

A

Supportive
PEG feed, non invasive ventilation, physio, OT, SALT, care
Riluzole
Anticipatory / palliative care