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Neuro > Muscle and Nerve Disease > Flashcards

Muscle and Nerve Disease Flashcards

1
Q

What are the main components of the motor system?

A

UMN, anterior horn cell, LMN, nerve ending, ACh, ACh receptor, NMJ

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2
Q

What is the main function of muscle?

A

Contraction and relaxation
Intricate machine designed to convert chemical energy to mechanical energy

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3
Q

What are the main parts of the muscle structure?

A

Structural components, Excitation-contraction coupling, energy production by breakdown of glycogen and fatty acids, and contractile mechanism (myosin fibres)

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4
Q

What are some symptoms of muscle disease?

A

Weakness of skeletal muscle - resp. and swallowing is important
Cardiac symptoms
Cramps/ muscle pain and stiffness
Myoglobinuria as breakdown product in urea
Babies - floppy, poor suck, feeding issues

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5
Q

What is used for investigation of muscle disease?

A

History and exam
Bloods - creatine kinase CK, electromyography (EMG), muscle biopsy, and genetic testing

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6
Q

What are some congenital/ genetic cause for muscle disease?

A

Contractile - congenital myopathies
Structural - muscular dystrophies
Coupling - channelopathies
Energy - metabolic myopathies

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7
Q

What are some acquired causes of muscle disease?

A

Electrolyte disturbance - esp. K+
Endocrine - thyroid, adrenal, Vit D deficiency
Autoimmune inflammatory muscle disease
Iatrogenic - medication (steroids and statins)

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8
Q

What are the 2 types of inflammatory muscle disease?

A

Polymyositis vs dermatomyositis (DM)

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9
Q

Describe inflammatory muscle disease

A

Autoimmune, any age, acute or subacute, painful, weak muscles
Characteristic rash of DM
High CK, autoantibodies and tumour screen needed for DM

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10
Q

What is used for diagnosis and treatment of inflammatory muscle disease?

A

EMG and biopsy
Treatment is immunosuppression

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11
Q

What is myasthenia gravis?

A

A neuromuscular junction disorder leading to weakness of skeletal muscles

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12
Q

What is the clinical presentation of myasthenia gravis?

A

Fatigable weakness
Ocular - ptosis and diplopia
Generalised - limbs, bulbar (chew, swallow, talk) and breathing

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13
Q

What is used for investigation of myasthenia gravis?

A

ACh receptor or anti-MuSK antibodies
Neurophysiology
CT chest

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14
Q

What is used for symptomatic treatment of myasthenia gravis?

A

Cholinesterase inhibitors

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15
Q

What treatment is used for disease modifying in myasthenia gravis?

A

Immunoglobulin/ plasma exchange, steroids, steroids sparing immunosuppression, thymectomy

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16
Q

What does a peripheral nerve consist of?

A

Axons - sensory (small for pain and temp. and large fibres for joint perception and vibration), motor and autonomic
Nerve sheath - myelin

17
Q

What is a root disease which causes peripheral nerve disease?

A

Commonly degenerative disc disease

18
Q

How does a lesion of individual peripheral nerve cause peripheral nerve disease?

A

Compressive/ entrapment neuropathy
Vasculitis - mononeuritis multiplex

19
Q

What can cause peripheral nerve disease?

A

Root disease
Lesion of individual peripheral nerve
Generalised peripheral neuropathy - motor/sensory or both and possible autonomic features

20
Q

What are the 2 types of generalised peripheral neuropathy?

A

Axonal vs demyelinated

21
Q

What are the causes for generalised peripheral neuropathy?

A

Hereditary, metabolic (diabetes and alcohol), toxic - drugs, infections, malignancy - paraneoplastic and inflammatory

22
Q

What are the symptoms of peripheral nerve disease?

A

Sensory - loss of sensation, abnormal sensation and pain
Motor - muscle thinning and weakness
Autonomic - skin changes, BP, bowel and bladder

23
Q

What are some signs of peripheral nerve disease?

A

Sensory
Motor - LMN
Distribution - root (myotome/ dermatome), single nerve, generalised nerve so distal first

24
Q

What investigations are used in peripheral nerve disease?

A

Blood tests, nerve conduction studies/EMG, lumbar puncture, nerve biopsy and genetic analysis

25
Q

What is the treatment for peripheral nerve disease?

A

Treat the cause
Stop drug, surgery and immunoglobulin

26
Q

What is motor neuron disease affecting in the spinal cord?

A

Anterior horn cell disease

27
Q

Describe motor neuron disease

A

Amyotrophic lateral sclerosis
Usually a limb first then progresses quickly to bulbar then resp.
Prognosis is median 3-5 years from onset

28
Q

What are the signs of motor neuron disease?

A

Combination of LMN and UMN signs
LMN - muscle fasciculations, wasting, weakness
UMN - spasticity, brisk reflexes and extensor plantar
No sensory involvement

29
Q

How is motor neuron disease diagnosed?

A

Clinical - combination of UMN and LMN signs and no sensory signs
EMG
Genetics

30
Q

What is the treatment for motor neuron disease?

A

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

Neuro (40 decks)

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