Patho - NMDs Flashcards

1
Q

GBS what it is and causative organisms

A

autoimmune disease; acute + rapid peripheral nervous system disorder/polyneuropathy characterized by ascending paralysis lower limbs –> head where they can only blink

organisms: campylobacter jejuni, CMV
- used to be associated with post-vax but decreased incidence now

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2
Q

GBS pathology

A

Demyelination along peripheral nerves, roots and cranial nerves by macrophages and lymphocytes; elicited in response to the causative organism antigen exposure

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3
Q

GBS S&S

A

ascending skeletal muscle paralysis, paresthesia (tingling), loss of deep tendon reflexes (knee), peripheral facial weakness
resp: vent failure due to diaphragm + intercostals affected, atelectasis and secretion retention, weak/absent cough, decreased gag reflex, dysphagia, resp failure; will eventually require MV support

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4
Q

20/30/40 rule

A

helps diagnose weakened insp muscle strength for NMDs such as myasthenia gravis, GBS
VC<20ml/kg, MIP < 30ml/kg, MEP < 40ml/kg = critical values and means they need MV support

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5
Q

How to Dx GBS

A

CSF findings via lumbar puncture = increased protein level could indicate GBS due to demyelination; insp muscle strength trending (VC/MIP/MEP 20/30/40 rule), EMG/electromyography measures nerve conduction + muscle strength, clinical Hx and S&S

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6
Q

GBS 2 primary treatments and other supportive therapy

A

IVIG = provides health IgG via IV to replace the damaged IgG and dilute the antibodies responsible for GBS autoimmune response; OR
Plasmapheresis/plasma exchange = removes damaged IgG + inflammatory components and transfuse back health blood products (harder to access tho)
supportive therapies: CS in combi with IVIG, O2 therapy, bronchial hygiene, MV, tracheostomy, physiotherapy

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7
Q

Describe ALS and the progression

A

aka Lou Gehrig’s disease; progressive degeneration of upper and lower motor neurons within brain + SC
progression is slower and can begin in limbs –> central (face/cough/swallow) or have bulbar onset central –> limbs

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8
Q

T/F ALS can be definitively diagnosed

A

false… ALS is diagnosed via exclusion (ruling out viruses/GBS; tumours with MRI)

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9
Q

ALS treatment

A

Riluzole - inhibits glutamate release and delays nerve damage;
G-tube feeding, BiPAP/CPAP, bronchial hygiene, tracheostomy;
depends what type of palliative care they want

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10
Q

describe SMA and the types (brief)

A

SMA = spinal muscular atrophy
autosomal recessive disorder involving progressive motor neuron disease
4 types; type 1 most severe, type 4 least severe

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11
Q

SMA Tx

A

Spinraza ($$$ - targets the SMA protein to make it more complete/functional); BiPAP, Trach tube, MV, bronchial hygiene

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12
Q

describe myasthenia gravis pathology

A

NMJ NMD!!!

  • autoimmune response involving IgG attacking ACh receptors at neuromuscular junction
  • results in ACh blocked and receptors destroyed = muscles receive less signal
  • results in motor neuron dysfunction (sensory intact)
  • episodic muscle weakness (rather than continuous/progressive)
  • improves with rest
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13
Q

S&S of myasthenia gravis

A

ocular symptoms ptosis, diplopia = unique to MG;

facial muscle weakness, dysphagia, limbs weak

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14
Q

myasthenia gravis Dx

A

ocular symptoms, serum AChR antibodies!! most specific test; insp muscle strength, tensilon test where tensilon/AChEsterase inhibitor should restore muscle function (! unique), ice pack test (cold reduces AChE action)

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15
Q

myasthenia gravis tx

A

Pyridostigmine or Neostigmine = AChE inhibitors; CS and other immunosuppressants, plasmpheresis

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16
Q

describe muscular dystrophy

A

type of inherited muscular disease (Affects muscles directly) due to lack of dystrophin (muscular structural protein)

17
Q

describe 3 types of muscular dystrophy

A

DMD/Duchenne = most severe + common, sex-linked inherited and appears in childhood; BMD = slower progression and older childhood appearance; myotonic dystrophy = adults

18
Q

MD Dx

A

genetic testing, high creatine kinase (CK) = elevated with muscle damage, Gower sign (crawling to stand up due to muscle weakness)

19
Q

MD Dx

A

no curative tx, all supportive