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Neurology Year 3 > Neuromuscular Disorders > Flashcards

Neuromuscular Disorders Flashcards

1
Q

What is the neurotransmitter in the NMJ?

A

Acetylcholine

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2
Q

What causes botulism?

A

Clostridium Botulinum

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3
Q

How does a bacterial infection produce the neuromuscular disorder?

A

The toxins cleave presynaptic protein preventing vesicles fusing with the membrane.
Acetylcholine isn’t released in NMJ.

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4
Q

How doe botulism present?

A

Rapid onset weakness with no sensory loss.

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5
Q

Lamberton Eaton Syndrome

A

Anutoantibodies to the Ca2+ so reduced vesicles released as Ca2+ doesn’t enter presynaptic bulb.

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6
Q

What is the common cause of Lamberton Eaton Syndrome?

A

Small Cell Carcinoma

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7
Q

What is the most common Neuromuscular disorder?

A

Myasthenia Gravis

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8
Q

When does myasthenia present in the population

A

Women 3rd decade

Men 6-7th decade

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9
Q

What causes myasthenia gravis?

A

Autoimmune antibodies to the acetylcholine receptors on post synaptic plate.
Reduced receptors mean stamina and increased fatigue.

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10
Q

At what percentage loss of receptors do symptoms present?

A

30%

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11
Q

What is Myasthenia Gravis associated with?

A

Thymic Hyperplasia

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12
Q

If left untreated how do the muscles in myasthenia gravis react?

A

Begin to atrophy

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13
Q

In what percentage of patients with myasthenia gravis do ACh antibodies occur?

A

80-90%

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14
Q

What are the clinical signs of Myasthenia Gravis?

A

Fluctuating weakness
Extraoccular weakness - drooping eyes double vision
Struggle to speak and chew
Proximal limb weakness

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15
Q

What is the acute treatment for Myasthenia Gravis?

A

Acetylcholinesterase inhibitors - Pyridostigmine
IV Immunoglobulin
Plasma Exchange
Thymectomy

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16
Q

What is the acute treatment for chronic Myasthenia Gravis?

A

Steroid

Steroid sparing agents

17
Q

What is the course of steroid used in chronic Myasthenia Gravis?

A

High does 40-60mg prednisolone for a month

18
Q

What steroid sparing agents are used in chronic Myasthenia Gravis?

A

Azathioprine

Mycophenolate

19
Q

What drugs should be avoided in a Myasthenia Gravis?

A

Gentamicin

20
Q

What is the mortality in Myasthenia Gravis?

A

3/4%

21
Q

What are the commonest causes of death in Myasthenia Gravis?

A

Respiratory failure
Aspiration pneumonia
Side effects of immunosuppression

22
Q

What are the signs of muscular disease?

A

Myalgia
Muscle weakness
Wasting
Hypoflexia

23
Q

What are fasciculations?

A

Visible fast fine spontaneous twitches

24
Q

What are some non pathological causes of fasciculations?

A

Stress fatigue caffeine

25
Q

What is a pathological cause of fasciculations?

A

Denervated muscles that are hyper excitable

26
Q

What grading system is used to determine the power in someone’s limbs?

A

MRC - Muscle Power Grading
0 - 5
0 = no movement
5 = normal strength ( subjective to the patient)

27
Q

What investigations are undertaken in a suspected NMJ disease?

A

Blood test - Creatine Kinase
Muscle Biopsy
MRI - Muscle tissue

28
Q

Describe the appearance of polymyositis?

A

Symmetrical rapidly progressing proximal muscle weakness

29
Q

What investigations are undertaken in polymyositis?

A

Raised Creatine Kinase

Very reactive to steroid <24 hours

30
Q

What’s dermatomyositis?

A

Clinically similar to polymyositis but + Heliotrope rash

31
Q

If a diagnosis of dermatomyositis is affirmed what should be investigated?

A

Look for malignancy - 50% have a malignancy

32
Q

Describe inclusion body myositis.

A

6th decade
Sow onset weakness
Spares the thumbs
Non treatable

33
Q

What is the presentation of someone with Myotonic Dystrophy?

A 
Myotonia
Cataracts
Ptosis
Frontal balding
Cardiac abnormalities
34
Q

Myotonia

A

Inability to relax muscle

35
Q

Statins can induce this.

A

Drug myopathy

36
Q

What are some causes of Rhabdomyolysis?

A

Crush injuries
Toxins
Post convulsions
Extreme exercise

37
Q

What is the pathophysiology behind rhabdomyolysis?

A

Damaged muscles releases Ca2+
Triggers the release of K+ PH4+ and myoglobininto the blood.
Acute renal failure and DIC as result.

38
Q

What is DIC (rhabdomyolysis)

A

Disseminated Intravascular coagulation

39
Q

How does rhabdomyolysis present?

A

Myalgia
Weakness
Myoglobulinuria

Neurology Year 3 (29 decks)

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