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4th Year Neurology > Neuromuscular Disorders > Flashcards

Neuromuscular Disorders Flashcards

1
Q

What is motor neuron disease?

A

Progressive degeneration of motor neurons, leading to muscle wasting and weakness

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2
Q

What is the average survival time for a patient with MND?

A

3 years

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3
Q

90% of MND involves lower motor neuron. True/False?

A

True

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4
Q

What is the most common phenotype of MND?

A

Amyotrophic lateral sclerosis (ALS)

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5
Q

What is the main difference between ALS and PLS?

A

ALS has both UMN and LMN features, PLS UMN only

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6
Q

ALS is associated with which type of dementia?

A

Frontotemporal dementia

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7
Q

List investigations for MND

A 
Clinical diagnosis (onset: bulbar, chest etc.)
Neurophysiology (EMG)
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8
Q

Outline management of MND

A

Assess needs and organise care
Speech therapy
Nutrition support
Ventilatory support

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9
Q

What is the classic sign of MND in the hands?

A

Split hand syndrome (preferential wasting of thenar group in the hand)

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10
Q

MND causes does not cause sensory problems. True or false?

A

True

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11
Q

Describe the basic pattern of symptoms in ALS

A

Both UMN and LMN features that starts in one limb and spread to other limbs and trunk

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12
Q

Progressive bulbar palsy involves which cranial nerves?

A

CN IX, X & XII

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13
Q

Progressive muscular atrophy has purely _____ motor neuron features

A

Lower

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14
Q

PLS has purely _____ motor neuron features

A

Upper

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15
Q

Botulism causes what symptoms?

A

Rapid onset weakness without sensory loss

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16
Q

What are the risk factors for botulism?

A
  • IV drug use
  • Food (clostridium botulinum is present in soil)
  • Wounds
17
Q

Botulism is a ___synaptic disorder?

A

Pre

18
Q

Lambert Eaton Myasthenic Syndrome (LEMS) is a ___synaptic disorder?

A

Pre

19
Q

Lambert Eaton Myasthenic Syndrome (LEMS) is strongly associated with which type of cancer?

A

Small cell lung cancer

20
Q

Myasthenia gravis is a ___synaptic disorder?

A

Post

21
Q

Myasthenia gravis is an autoimmune disease of which receptors?

A

Acetyl choline receptors

22
Q

What is the most common presentation of myesthenia gravis?

A

Extraocular weakness, facial and bulbar weakness (eyes drooping by end of day, choke after big meal etc)

23
Q

Facial weakness in myesthenia gravis is typically bilateral. True or false?

A

True

24
Q

Ptosis in myesthenia gravis is worse at the beginning of the day. True or false.

A

False - typically bilateral and worse at end of the day/prolonged upgaze

25
Q

Which antibodies indicate myesthenia gravis?

A

Anti-Ach Receptor Antibody (80-90% of patients

26
Q

What tests can indicate myesthenia gravis?

A
  • Fatigue tests (eg blink 20 times – MG patients will fatigue)
  • Neck flexion – very few things have neck flexion weakness
  • Ice test – ptosis improved after applying ice for 2 mins
27
Q

What is the treatment for myesthenia gravis?

A
  • Pyridostigmine (anticholinesterase)
  • Steroids
    _Steroid sparing agents – azathioprine / mycophenolate
  • Thymectomy
28
Q

75% of myesthenia gravis patients have abnormalities in what organ?

A

Thymus

29
Q

Polymyositis causes symmetrical distal muscle weakness. True or false?

A

False - it cases symmetrical, proximal muscle weakness

30
Q

What are the cutaneous features of dermatomyositis

A
  • V-shaped rash over chest
  • Gottron’s papules (on hands)
  • Heliotrope rash (on eyes)
  • Shawl sign
31
Q

What abnormalities are seen in blood tests in poly/dermatomyositis

A

Raised inflammatory markers and Ck

32
Q

What antibodies are seen in polymyositis

A

Anti-Jo-1

33
Q

What is the treatment of polymyositis

A

Steroids + immunosurpressive drugs (eg Methotrexate/azathioprine/ciclosporin)

34
Q

How does inclusion body myositis differ from polymyositis?

A

It is progressive weakness and wasting of both distal and proximal muscles (often asymmetrical) whereas polymyositis is symmetrical, proximal muscle weakness

35
Q

What is the muscle breakdown product that causes kidney damage in rhabdomyolysis?

A

Myoglobin

4th Year Neurology (13 decks)

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