Autoimmune nerve Conditions Flashcards

1
Q

What is MS?

A

Chronic T-cell autoimmune attack of myelin leading to sensory & motor impairment

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2
Q

What is the pathophysiology of MS?

A

T-cell mediated attack of myelin
Leads to plaques of demyelination of CNS neurons = sclerosis
Reduces/blocks transmission of signals from CNS
Impairment of sensory & motor functions

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3
Q

What are the types of MS? Describe them

A
  • Relapsing & remitting: 80% poor demyelination but periods of good health
  • Primary progressive: From onset Sx are progressive
  • Secondary progressive: 50% w/relapsing & remitting disease. Prolonged demyelination causes axonal loss + clinically progressive Sx
  • Progressive & relapsing
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4
Q

What is the main trigger of primary progressive MS?

A

Infection

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5
Q

What are the RFs for MS?

A

Western world
30yo
Female
Vit D Deficiency

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6
Q

What type of hypersensitivity is MS?

A

Type 4

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7
Q

How does MS present?

A
Monosymptomatic initially
Sx WORSE AFTER HOT SHOWER
Charcot's triad: Dysarthria + Intention tremor + Nystagmus
U/L Optic neuritis
Paraesthesia + Numbness
Brainstem/cerebellar Sx
Fatigue
Autonomic effects
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8
Q

What are the Sx of optic neuritis?

A

Demyelination of optic nerve
Pain on eye movement
Rapid ↓central vision & ↓acuity → hemianopia/blindness
Red desaturation (colours not as vibrant)
Nystagmus
CN6 palsy

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9
Q

What are the Sx of paraesthesia in someone with MS?

A

Tingling/itching/burning
Numbness
OFTEN LEGS: Acute leg weakness & incontinence= transverse myelitis

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10
Q

What are the Sx of brainstem & cerebellar MS?

A
Cerebellar = DANISH
Diplopia & Nystagmus
Trunk & limb ataxia
Trigeminal neuralgia
Deafness & BPPV
Headache
Facial weakness- Bell's palsy, dysarthria
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11
Q

What are the autonomic effects of MS?

A

Bladder: Urgency, frequency, incontinence
Sexual: Impotence, altered sensation

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12
Q

How is MS investigated?

A

Prior to referral =
Bloods: FBC, ESR/CRP, LFTs, U&E, Ca2+, Glucose, TFT, B12, HIV
REFER to neurologist post-bloods
MRI: Multiple disseminated lesions
LP: Oligoclonal IgG bands in CSF or ↑IgG index

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13
Q

Other than suspecting MS when else should someone be referred to a neurologist for ?MS ?

A

Ophthalmologist confirms optic neuritis

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14
Q

How is MS diagnosed?

A

Can only be done by consultant neurologist

>2 episodes at 2 SEPARATE SITES at 2 DIFFERENT POINTS IN TIME

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15
Q

What advice is given to someone with MS?

A

Stop smoking
Exercise
Vit D: Cholecalciferol aim >50 of 25(OH)D levels

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16
Q

How is MS managed?

A

Methylprednisolone: 0.5-1g PO OD shortens acute relapses- use sparingly + PPI
DMARDs: Alemtuzumab (R&R)

17
Q

How are the Sx of MS treated?

A
Spasticity: Baclofen
Tremor: Botulinum toxin
Urgency/frequency: Catheterisation
Neuro pain: TCA, Pregabalin
Emotions: TCA
18
Q

What are the complications of MS?

A

Transverse myelitis: Urgent hospital admission
Relapse: Over 12-24hours
Pressure ulcers
Cognitive decline = 50%

19
Q

What is myasthenia gravis?

A

Autoimmune disorder of neuromuscular transmission leading to muscle fatigue

20
Q

What is the pathophysiology of myasthenia?

A

Auto antibodies bind to postsynaptic Ach receptors

Deficiency of nicotinic acetylcholine receptors

21
Q

What is myasthenia associated with?

A

<50yo women: Thymic hyperplasia
>50yo men: Thymic atrophy, tumour, RA, SLE
Other: thymoma, pernicious anaemia, AI thyroid disorders

22
Q

What are the Sx of myasthenia?

A

Bilateral, symmetrical Sx
Increasing muscle fatigue WITH ACTIVITY
Slow improvement with rest
Proximal weakness of muscles
Extraocular muscle weakness: Diplopia & ptosis
Bulbar weakness: Dysphagia, dysarthria, dysphonia

23
Q

What are the triggers for weakness in myasthenia?

A
Pregnancy
Infection 
Climate change
Emotion
Exercise
Drugs
24
Q

Which drugs can exacerbate myasthenia?

A
BB
Abx: Gentamicin, Ciprofloxacin, tetracyclines
Anti-Arrhythmias: Verapamil
Opiates
Lithium
Statins
Quinine
GA- neuromuscular blockers
25
Q

In what order does weakness occur in myasthenia?

A

extra-occular → bulbar (swallowing, chewing) → face → neck → limb girdle → trunk

26
Q

How is myasthenia investigated?

A

90% have auto-Ab:
Anti-AchR Ab = DIAGNOSTIC
Anti-MuSK Ab = DIAGNOSTIC
CXR/CT thorax: ESSENTIAL (exclude thymoma)
Neurophysiology
Count to 50: Voice weakens due to fatigue

27
Q

How is myasthenia managed?

A

Pyridostigmine
Relapse: Prednisolone 5mg or DMARD (Azathioprine) if steroids not covering it
Crisis: Plasma exchange + IV immunoglobulin

28
Q

What is a myasthenic crisis?

A

Respiratory compromise
Weak muscles of ventilation = acute respiratory failure
Weak pharyngeal muscles = compromise airways
REGULAR PO2 + FVC
Tx: Plasma exchange + IV immunoglobulin

29
Q

What is Lambert-Eaton myasthenic syndrome?

A

Paraneoplastic syndrome causing muscle weakness IMPROVING w/exercise
CXR: LUNG CANCER

Patho: Autoimmune destruction of Ca2+ channels on presynaptic terminal

30
Q

What are the Sx of Guillain Barre?

A

Symmetrical ascending muscle weakness
AND
Paraesthesia (glove-stocking)
↓Reflexes depressed/absent

31
Q

How is Guillain Barre treated?

A

IV Immunoglobulins 400mg/kg/24hr

32
Q

How is Guillain Barre investigated?

A

LP: ↑PROTEIN

Nerve conduction: Slow