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Flashcards in Week 4 Deck (81):
1

When do patients with MS typically present first?

30s and 40s

2

What demyelinating disorder is optic neuritis associated with?

MS

3

Give three features of pyramidal dysfunction in patients with MS?

1. Increased tone
2. Spasticity
3. Weakness

4

Name some sensory symptoms in MS?

1. Pain
2. Paraesthesia
3. Dorsal column loss - proprioception and vibration
4. Numbness
5. Trigeminal neuralgia

5

What dysfunction is present with ataxia, intention tremor, nystagmus, past pointing, pendular reflexes, dysdiadokinesis and dysarthria?

Cerebellar dysfunction

6

Give two features of brainstem dysfunction in MS?

1. Diplopia
2. Facial weakness

7

Medial longitudinal fasciculus
Distortion of binocular vision
Failure of adduction- diplopia
Nystagmus in abducting eye
Lag

Internuclear ophthalmoplegia - MS

8

What is used to treat fatigue in MS?

Amantadine
Modafinil if sleepy
Hyperbaric oxygen

9

Give three investigations for possible MS?

1. MRI
2. CSF
3. Neurophysiology

10

How is a mild acute exacerbation of MS treated?

Symtpomatic treatment

11

How is a moderate acute exacerbation of MS treated?

Oral steroids

12

How is a severe acute exacerbation of MS treated?

Admit and IV steroids

13

Give four ways to treat spasticity in MS?

1. pHYSIOTHERAPY
2. Oral baclofen and tizanidine
3. Botulinum toxin
4. Intrathecal baclofen/phenol

14

Give two drugs for treating sensory symptoms in MS?

1. Gabapentin
2. Amitriptyline

15

Give four ways to treat lower urinary tract dysfunction in MS?

1. Bladder drill
2. Anti cholinergics oxybutynin
3. Desmopressin
4. Catheterisation

16

List three first line disease modifying therapies for MS?

1. Interferon Beta - avonex, rebif
2. Glitiramer Acetate (copaxone)
3. Tecfedira

17

List two second line therapies for MS?`

1. Monoclonal antibody
2. Fingolimod

18

Name a third line therapy for MS?

Mitoxantrone

19

What drug is first line, oral agent in relapsing remitting MS?

Tecfidera

20

List the three cells involved in direct pathway for signal transmission in the retina?

Photoreceptors
Bipolar cells
Ganglion cells

21

What cells in teh retina receive input from photoreceptors and project to other photoreceptors and biopolar cells?

Horizontal cells

22

What cells in the retina receive input from bipolar cells and project to ganglion cells, biopolar cells and other amacrine cells?

Amacrine cells

23

What is the role of photoreceptors?

Transduction

24

In relation to the dark current - when is the cGMP sodium channel open/

In the dark and closes in the light

25

What is rhodopsin (for rods) made from?

Retinal vitamin A derivative + opsin (GPCR)

26

What does light convert 11-cis-retinal to?

All-trans-retinal (activated form)

27

What activates transfucin leading to molecular cascade that decreases cGMP, leading to closure of sodium channels and hyperpolarisatio?

All-trans-retinal

28

Is there more glutamate in the dark or light?

In the dark

29

What photoreceptors are for dim light?

Rods - more convergence

30

Where in the retina are rods found?

Peripheral retina

31

Where in the retina are ocnes fround?

Central retina - fovea

32

Do rods have higher visual acuity than cones?

No they have low visual acuity

33

Are bipolar cells (OFF pathway) more positive in light or dark?

Dark - more glutamate

34

Are bipolar cells (ON pathway) more positive in light or dark?

Light

35

What cells in signal transmission (vision) produce APs?

Ganglion cells

36

In relation to vision what does lateral inhibition imporve?

Stimulus localisation

37

What modifies the receptive fields of ganglion cells to have a center-surround organisation?

Lateral inhibition

38

What does center-surround organisation/lateral inhibtion serve to do?

Emphasise areas of difference (contrast) i.e,. sharpens boundary between objecys of different luminance

39

Nerve fibres from what half of each retina cross over at optic chiasm?

Nasal half

40

What are motor end plates?

The synapses formed between motor neurons and muscle are called the motor end plate

41

Who is lkely to be affected by the presynaptic disorder botulism?

IV drug users - black tar heroin

42

What symptoms does botulism give?

Rapid onset weakness without sensory loss

43

What involves antibodies to presynaptic calcium channels leading to less vesicle release. Strong association with underlying small cell carcinoma?

Lambert Eaten Myasthenic Syndrome

44

Name a post synaptic disorder?

Myasthenia Gravis

45

What is the most common disorder of neuromuscular junction?

Myasthenia grais

46

What condition is related to antibodies to acetyl choline receptors (AChR)?

Myasthenia gravis

47

In Myasthenia Gravis what happens to the number of ACh receptors and endplate folds?

Reduced number and flattening of endplate folds

48

What do 75% of myasthenia gravis patients also have?

Hyperplasia of thymus or thymoma

49

What are some clnical features of myasthenia gravis?

Weakness fluctuating - worse through day
Extraocular weakness, facial and bulbar weakness
limb weakness typically proximal

50

How do you treat myasthenia gravis?

Acetylcholinesterase inhibitor - pyridostigmine
Thymectomy

51

What is the actin myosin ratio in smooth muscle?

10 : 1

52

What is a fasciculation?

Visible, fast, fine spontaneous twitch

53

What precipitates fasciculations?

Stress, caffeine and fatigue

54

What is usually a sign of disease in the motor neurone, not the muscle?

Fasciculations

55

What is myotonia?

Failure of muscle relaxation after use - chloride channel

56

What infalmmatory condition is symmetrical, progressive proximal weakness, raised CK and responds to steroids?

Polymyositis

57

Heliotrope rash on face and up to 50% have underlying malignancy>?

Dermatomyositis

58

Give a degenerative muscle disease?

Inclusion body myositis

59

Slowly progressive weakness in 6th decade of life with characteristic thumb sparing?

Inclusion body myositis

60

What is the commonest muscular dystrophy?

Myotonic dystrophy

61

What heritance is myotonic dystrophy?

Autosomal dominant

62

Myotonia, weakness, cataracts, ptosis, frontal balding, cardiac defects
Trinucleotide repeat disorder with anticipation

Myotonic dystrihyt

63

Myalgia, muscle weakness and myoglobinuria?

Rhabdomyolysis

64

Gie two complications of rhabdomyolysis?

Acute renal failure
DIC

65

What are the two peaks of incidence for myasthenia gravis?

Females in 3rd decade
Males in 6th/7th decade

66

Name an untreatable and rapidly progressing neurodegenerative condition?

Motor neuron disease

67

Name some symptoms of MND?

Muscle weakness
speech, swallo and breathing

68

What condition shows upper and/or lower motor neurone signs without sensory problems

MND

69

What does MND lead to?

Focal onset, continuous spread and finally generalised paresis

70

How long is average MND survival time?

3 years

71

90% sporadic, 10% familial?

MND

72

wHAT IS THE 70% site of onset for MND?

Extremities

73

What motor neuron is more affected in MND?

Lower motor neurone 90%

74

Is the bulbar variant of MND more common in women or men?

Women (60-80 years)

75

How is MND diagnosed?

Diagnosis of exclusion

76

What kind of ementia is asociated with MND?

Fronto temporal

77

Are these signs upper or lower motor neuron - spasticity, babinski sign?

UMN

78

Name some bulbar UMN signs?

1. Exaggerated snout reflex
2. Clonic jaw jerk
3. Emotional lability
4. Forced yawning

79

Name some cervical and lumbar region UMN sings?

1. Clonic deep tendon reflexes
2. Wasted limb
3. Hoffman reflex
4. Hyperreflexia

80

Upper or lower MN dysfunction - severe weakness, fasciculations, muscle cramps, muscle hypotonicity, muscle atrophy, hyporeflexia?

Lower

81

upper or lower MN dysfunction - pseudobulbar affect, moderate weakness, spasticity, hyperreflexia, extensor plantar reflexes?

Upper