Neuro - Inflammatory Conditions Flashcards

1
Q

What is Myaesthenia Gravis?

A

Autoimmune disease of the Neuromuscular junction that presents with a progressive weakness which improves with rest

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2
Q

When are adults commonly affected with Myaesthenia Gravis?

A

Women <40y

Men >60y

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3
Q

Which cancer is commonly associated with Myaesthenia Gravis?

A

Thymoma

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4
Q

Which autoimmune conditions have an association with Myaesthenia Gravis?

A
Thyroiditis
Graves disease
RA
SLE
Pernicious Anaemia
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5
Q

What is the pathophysiology behind Myaesthenia Gravis?

A

Acetylcholine Receptor antibodies are produced which bind to the postsynaptic neuromuscular junction reducing muscle contraction

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6
Q

Where are clinical signs of Myaesthenia Gravis usually found?

A

Proximal muscles of the head and neck

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7
Q

What are some symptoms of Myaesthenia Gravis?

A
Extraocular muscle weakness giving diplopia
Eyelid weakness giving a Ptosis
Weakness in facial movements
Swallowing difficulties
Fatigue of the jaw when chewing
Slurred speech
Progressive weakness with repetitive movements
No Sensory/Reflex loss
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8
Q

Which factors can affect the course of Myaesthenia Gravis?

A
Emotional stress
Pregnancy
Menses
Secondary illness
Thyroid dysfunction
Trauma
Temperature extremes
Drugs
Surgery
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9
Q

Which drugs can affect the course of Myaesthenia Gravis?

A

Beta Blockers
CCB
ACEi

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10
Q

How is suspected Myaesthenia Gravis diagnosed?

A

Test for ACh R antibodies

CT/MRI Thymus - ?Thymoma

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11
Q

How is Neostigmine used as a clinical test for suspected Myaesthenia Gravis?

A

When given it blocks Acetylcholinesterase enzymes, reducing the breakdown of ACh. When given to Myaesthenia Gravis patients, their weakness temporarily improves

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12
Q

Which medications are used to manage Myaesthenia Gravis?

A

Pyridostigmine - Blocks Acetylcholinesterase increasing ACh levels
Prednisolone/Azathioprine - Immunosuppression
IVIG

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13
Q

What is a Myaesthenic Crisis?

A

Acute exacerbation of Myaesthenia Gravis due to another illness

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14
Q

Is a Myaesthenic Crisis serious?

A

Yes, as it can lead to respiratory failure due to weakness in the muscles of respiration

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15
Q

How should a Myaesthenic Crisis be managed?

A

BPAP, CPAP, Intubation
IVIG
Plasma Exchange

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16
Q

What is Guillian-Barre Syndrome?

A

Acute paralytic polyneuropathy that causes acute symmetrical ascending weakness and can also cause sensory loss

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17
Q

Which infections can trigger Guillian-Barre Syndrome?

A

CMV

EBV

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18
Q

What is the pathophysiology of Guillian-Barre Syndrome?

A

B cells create antibodies to the infection. These antibodies also match proteins found on nerve cells, meaning they are also targeted

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19
Q

What are the different types of Guillian-Barre Syndrome?

A

Acute Inflammatory Demyelinating Polyradiculoneuropathy
Acute Motor Axonal Neuropathy
Acute Motor and Sensory Axonal Neuropathy
Acute Pandysautonomia

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20
Q

What are some symptoms of Guillian-Barre Syndrome?

A

Symmetrical ascending weakness
Reduced reflexes
Peripheral loss of sensation/neuropathic pain
Facial nerve weakness

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21
Q

What is the usual course of Guillian-Barre Syndrome?

A

Symptoms start within 4w of the preceeding infection, and peak at 2-4w. Recovery can take months-years

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22
Q

Which criteria are used to assess for Guillian-Barre Syndrome?

A

Brighton Criteria

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23
Q

What are some potential complications of Guillian-Barre Syndrome?

A
Cardiac Arrhythmia
Postural Hypotension
Hypertension
Urinary retention
Ileus
Respiratory failure
Pain
DVT, PE
SIADH
Renal failure due to IVIG
Hypercalcaemia due to immobility
24
Q

What are some recommended management options for confirmed Guillian-Barre Syndrome?

A
Close observations
Bedside spirometry and ventilatory support where appropriate
ECG/Cardiac monitoring
Nutritional Support +/- NGT
DVT Prophylaxis
Catheter
Laxatives and Bowel care
Pain Control and Opiates
IVIG
Plasmaphoresis if presenting within 2w
25
Q

What is the associated prognosis of Guillian-Barre Syndrome?

A
25% Ventilated
70% Complete Recovery at 1y
25-30% Residual Deficit
3% Recurrent
3-5% Mortality
26
Q

What is Multiple Sclerosis?

A

Chronic Progressive disease of the CNS giving demyelination

27
Q

What pathophysiologically happens in MS?

A

Inflammatory process, activation of immune cells against CNS

28
Q

When does MS initially occur?

A

<50y

29
Q

Why is MS often described as “Disseminated in Time and Space”?

A

Different nerves are affected each time, so symptoms are different

30
Q

What are some causes of MS?

A
Genetic
EBV
Reduced Vitamin D
Smoking
Obesity
31
Q

What are some symptoms suggestive of MS?

A
Optic Neuritis
Eye Movement Anomalies
Focal Weakness
Focal Sensory Symptoms
Cerebellar/Sensory signs
32
Q

How does Optic Neuritis present in MS?

A

Sudden unilateral loss of vision over hours-days

33
Q

What are some eye movement anomalies found in MS?

A

6th Nerve Palsy gives:
Internuclear Opthalmoplegia
Conjugate Lateral Gaze disorder

34
Q

What are some focal weaknesses found in MS?

A

Bellsy Palsy
Horners Syndrome
Limb Paralysis
Incontinence

35
Q

What are some focal sensory symptoms found in MS?

A

Trigeminal Neuralgia
Numbness
Paraesthesia

36
Q

Which Cerebellar sign may be found in MS?

A

Ataxia

37
Q

How can suspected MS be investigated?

A

MRI - Looking for plaques

LP

38
Q

How can MS be managed medically?

A

DMARDs

39
Q

How should a relapse of MS be managed?

A

Methylprednisolone 500mg PO for 5/7

40
Q

What are some general measures advised for MS patients?

A
Exercise
Tricyclics if Neuropathic Pain
SSRI if depression
Oxybutinin if Urge Incontinence
Baclofen and Physio for Spasticity
41
Q

What is Clinically Isolated Syndrome for MS?

A

First episode of attack, 2 or more are needed to diagnose MS

42
Q

What is Relapsing-Remitting MS?

A

Patient experiences episodes with symptoms, but has a complete recovery in between episodes

43
Q

What is Secondary Progressive MS?

A

Patient experiences episodes of MS with symptoms, and does not recover to their baseline between episodes

44
Q

What is Primary Progressive MS?

A

There is a worsening of the disease and its symptoms from initial diagnosis, with no recovery between “attacks”

45
Q

What is Guillian-Barre Syndrome?

A

Acute Paralytic Polyneuropathy that affects the peripheral nervous system

46
Q

How does Guillian-Barre Syndrome present?

A

Acute, symmetrical ascending weakness and potential sensory loss

47
Q

How is Guillian-Barre Syndrome usually triggered?

A

Infection - CMV, EBV

48
Q

What happens in Guillian-Barre Syndrome?

A

B cells create antibodies to the pathogen, which match the proteins of nerve cells. As a result, these are also targeted

49
Q

What is the clinical presentation of Guillian-Barre Syndrome?

A

Symmetrical Ascending weakness
Reduced reflexes
Peripheral loss of sensation/neuropathic pain
Facial nerve weakness

50
Q

What is the usual timeframe of Guillian-Barre Syndrome?

A

Symptoms start within 4w of the preceeding infection and peak at 2-4w. Recovery can take months-years

51
Q

Which criteria can be used to diagnose Guillian-Barre Syndrome?

A

Brighton Criteria

52
Q

Which investigations may be appropriate for suspected Guillian-Barre Syndrome?

A

Nerve Conduction studies

LP - Raised Protein

53
Q

What are some potential complications associated with Guillian-Barre Syndrome?

A
Cardiac Arrhythmia
Postural Hypotension
Hypertension
Urinary retention
Ileus
Respiratory failure
Pain
DVT/PE
SIADH
Renal failure secondary to IVIG
Hypercalcaemia due to immobility
54
Q

How should Guillian-Barre Syndrome be managed?

A
Close observation for progressive weakness
Bedside spirometry
Ventilatory support
ECG +/- Cardiac Monitoring
Nutritional Support +/- NGT
DVT Prophylaxis
Catheter
Laxatives and Bowel Care
Pain control with Opiates
IVIG
Plasmaphoresis if within 2w of presentation
55
Q

What is the prognosis associated with Guillian-Barre Syndrome?

A
25% Ventilated
70% Complete recovery at 1y
25-30% Residual deficit
3% Recurrence
3-5% Mortality