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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
What is the associated prognosis of Guillian-Barre Syndrome?
``` 25% Ventilated 70% Complete Recovery at 1y 25-30% Residual Deficit 3% Recurrent 3-5% Mortality ```
26
What is Multiple Sclerosis?
Chronic Progressive disease of the CNS giving demyelination
27
What pathophysiologically happens in MS?
Inflammatory process, activation of immune cells against CNS
28
When does MS initially occur?
<50y
29
Why is MS often described as "Disseminated in Time and Space"?
Different nerves are affected each time, so symptoms are different
30
What are some causes of MS?
``` Genetic EBV Reduced Vitamin D Smoking Obesity ```
31
What are some symptoms suggestive of MS?
``` Optic Neuritis Eye Movement Anomalies Focal Weakness Focal Sensory Symptoms Cerebellar/Sensory signs ```
32
How does Optic Neuritis present in MS?
Sudden unilateral loss of vision over hours-days
33
What are some eye movement anomalies found in MS?
6th Nerve Palsy gives: Internuclear Opthalmoplegia Conjugate Lateral Gaze disorder
34
What are some focal weaknesses found in MS?
Bellsy Palsy Horners Syndrome Limb Paralysis Incontinence
35
What are some focal sensory symptoms found in MS?
Trigeminal Neuralgia Numbness Paraesthesia
36
Which Cerebellar sign may be found in MS?
Ataxia
37
How can suspected MS be investigated?
MRI - Looking for plaques | LP
38
How can MS be managed medically?
DMARDs
39
How should a relapse of MS be managed?
Methylprednisolone 500mg PO for 5/7
40
What are some general measures advised for MS patients?
``` Exercise Tricyclics if Neuropathic Pain SSRI if depression Oxybutinin if Urge Incontinence Baclofen and Physio for Spasticity ```
41
What is Clinically Isolated Syndrome for MS?
First episode of attack, 2 or more are needed to diagnose MS
42
What is Relapsing-Remitting MS?
Patient experiences episodes with symptoms, but has a complete recovery in between episodes
43
What is Secondary Progressive MS?
Patient experiences episodes of MS with symptoms, and does not recover to their baseline between episodes
44
What is Primary Progressive MS?
There is a worsening of the disease and its symptoms from initial diagnosis, with no recovery between "attacks"
45
What is Guillian-Barre Syndrome?
Acute Paralytic Polyneuropathy that affects the peripheral nervous system
46
How does Guillian-Barre Syndrome present?
Acute, symmetrical ascending weakness and potential sensory loss
47
How is Guillian-Barre Syndrome usually triggered?
Infection - CMV, EBV
48
What happens in Guillian-Barre Syndrome?
B cells create antibodies to the pathogen, which match the proteins of nerve cells. As a result, these are also targeted
49
What is the clinical presentation of Guillian-Barre Syndrome?
Symmetrical Ascending weakness Reduced reflexes Peripheral loss of sensation/neuropathic pain Facial nerve weakness
50
What is the usual timeframe of Guillian-Barre Syndrome?
Symptoms start within 4w of the preceeding infection and peak at 2-4w. Recovery can take months-years
51
Which criteria can be used to diagnose Guillian-Barre Syndrome?
Brighton Criteria
52
Which investigations may be appropriate for suspected Guillian-Barre Syndrome?
Nerve Conduction studies | LP - Raised Protein
53
What are some potential complications associated with Guillian-Barre Syndrome?
``` Cardiac Arrhythmia Postural Hypotension Hypertension Urinary retention Ileus Respiratory failure Pain DVT/PE SIADH Renal failure secondary to IVIG Hypercalcaemia due to immobility ```
54
How should Guillian-Barre Syndrome be managed?
``` 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
What is the prognosis associated with Guillian-Barre Syndrome?
``` 25% Ventilated 70% Complete recovery at 1y 25-30% Residual deficit 3% Recurrence 3-5% Mortality ```

Neuro and Special Senses (5 decks)

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