PERIPHERAL Neuropathies Flashcards

1
Q

How different are the large nerve fibres from the small ones?

A
  • Large fibres - more well myelinated

whereas the small nerve fibres are either THINLY myelinated or unmyelinated

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2
Q

What occurs with damage to the small sensory fibres?

A
  • PAIN
  • dysesthesia (UNPLEASANT sensation when touched)
  • reduced pin prick and temperature sensation
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3
Q

Name the small sensory fibres.

A
  • Adelta: thinly myelinated

- C: unmyelinated

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4
Q

What occurs with damage to the autonomic fibres?

A
  • dizziness (d.t POSTURAL hypotension)
  • impotence (ED)
  • Nausea and vomiting (GASTROPARESIS)
  • constipation/diarrhoea (esp. at night)
  • bloating and belching
  • tachycardia
  • excessive sweating
  • can’t empty bladder/ loss of bowel control
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5
Q

What are the symptoms of large sensory fibre damage?

A
  • PARAESTHESIA
  • unsteadiness
  • numbness
  • diminished vibration and JPS
  • Reflexes may be lost
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6
Q

What occurs with large fibre motor damage?

A

PC:
- weakness/ unsteadiness/ wasting

SIGNS:

  • REDUCED power
  • absent reflexes
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7
Q

Name patterns of peripheral loss.

A
  • glove and stocking
  • mild/moderate/severe
  • mononeuritis/ mononeuropathy
  • radiculopathy
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8
Q

How may plexopathy occur?

A
  • by traction of the nerve plexus
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9
Q

How are nerve damaged?

A
  • axonal loss

- Peripheral nerve demyelination

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10
Q

Name an ACUTE demyelinating neuropathy.

A
  • GBS (Guillaine Barre Syndrome)

- occurs within DAYS-wks

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11
Q

Name a chronic, progressive demyelinating neuropathy.

A
  • CIDP (chronic inflammatory demyelinating polyradiculopathy)
  • Hereditary sensory motor neuropathy (Charcot-Marie Tooth $)
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12
Q

How common is GBS?

A

1-2/ 100,000 per year

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13
Q

How does GBS present as?

A
  • progressive paraplegia over days-4 weeks
  • sensory symptoms precede weakness
  • PAIN
  • peak symptoms 10-14 days INTO onset of illness
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14
Q

When causes GBS?

A
  • post-infectious CAMPYLOBACTER association

- campylobacter jejuni

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15
Q

WHat complications may arise from GBS?

A
  • 25% require MECHANICAL ventilation

- 10% DIE mainly from autonomic failure (CARDIAC arrhythmia)

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16
Q

How is GBS treated?

A
  • immunoglobulin infusion
    AND/OR
  • PLASMA exchange
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17
Q

What is key about the pattern of muscle weakness in GBS?

A
  • starts DISTALLY moves proximal
18
Q

What is hereditary neuropathy?

A
  • inherited

- NUMEROUS MUTATIONS (A.D, A.R and X-linked)

19
Q

How may hereditary neuropathy present as?

A
  • pure motor/ sensory/ sensorimotor/ small fibre and AUTONOMIC variants
20
Q

What is the most common genetic mutation for GBS?

A

CMT1a

21
Q

What is gr.1 of HMSN/ Charcot-Marie-Tooth?

A
  • has SLOW nerve conduction velocities

- evidence of hypertrophic demyelinating neuropathy

22
Q

What is usually seen with HMSN type 1?

A
  • WEAKNESS and ATROPHY of lower legs in adolescence

- later WEAKNESS of HANDS

23
Q

What is the type 2 HMSN variety characterised with?

A
  • normal nerve conduction velocity

- axonal degenration

24
Q

What are infectious causes of axonol neuropathy?

A
  • HIV
  • syphilis
  • Lyme
  • Hepatitis B and C
25
Q

What are drugs/toxins that may cause axonal neuropathies?

A
  • alcohol
  • amiodarone
  • phenytoin
  • chemo (CISPLATIN/Vincristine)
26
Q

What metabolic/endocrine causes result in axonal neuropathies?

A
  • diabetes
  • B12/Folate
  • hypothyroidism
  • chronic uremia
  • porphyria
27
Q

What vasculitic condition results in axonal neuropathies?

A
  • ANCA +ve

- Rheumatoid Arthritis/ Sjrogens Syndrome

28
Q

Can a paraneoplastic condition cause axonal neuropathies?

A

yes

  • Myeloma
  • Ab mediated (breast cancer)
29
Q

What are 3 CAUSES of autonomic neuropathy?

A
  • diabetes
  • amyloidosis
  • hereditary
30
Q

What are acute causes of autonomic neuropathy?

A

GBS

porphyria

31
Q

How to manage axonal peripheral neuropathy?

A
  • treat cause

- symptomatic rx= physiotherapy/ orthotics/neuropathic pain relief

32
Q

How to treat vasculitic P. neuropathy?

A
  • pulsed IV PREDNISOLONE

+ cyclophosphamide

33
Q

How to treat demyelinating, inflammatory neuropathy?

A
  • IV immunoglobulin
  • steroids
  • azathioprine, mycophenate, cyclophosphamide
34
Q

What is the MOA mycophenolate?

A

it suppresses the immune system the recipient

35
Q

What is cyclophosphamide?

A
  • it is used as chemotherapy and as a DMARD
36
Q

What is a HNPP?

A

Hereditary Neuropathy with Pressure Palsy

  • causes Numbness, tingling and muscle weakness
  • d.t mutation in the (AD) PMP22 gene resp. for producing myelin in nerve cells ——weak myelin= slightest pressure> muscle weakness
37
Q

Define autonomic neuropathy.

A
  • SYMPTOMS that occur with damage to the nerves that MANAGE every day functions
  • BP/ sweating/ bowel and bladder emptying/ HR/ digestion
38
Q

What is porphyria?

A
  • group of abnormalities in the chemical steps that lead to heme prodn
  • the enzymatic defects of biosynthesis of heme causes tissue accumulation of porphyrins
39
Q

How to recognize if its the origin of nerve damage is in the axon or the demyelination of the the axon?

A
  • do a Nerve Conduction Study (NCS)
  • demyelinating neuropathies will have reduced velocity of nerve conduction
  • Axonal neuropathies will demonstarte DECR. amplitude
40
Q

Describe the presentation of a person with AIP.

Acute intermittent Porphyria

A
  • severe abdominal pain
  • symmetrical limb weakness (Quadraplegia)
  • autonomic instability
  • SEIZURES