Peripheral Neurology Flashcards

1
Q

What are the three most important disorders of the peripheral nervous system?

A

Guillain-Barre syndrome
Length dependent neuropathy
Mysathenia Gravis

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2
Q

What is the peripheral nervous system?

A

All neurons that are not in the brain and spinal cord

- nerve roots -> nerve plexus -> peripheral nerves -> neuromuscular junction -> muscle

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3
Q

What is the function of the peripheral nervous system?

A

Sensory input into the CNS
- pain, temperature, light touch, proprioception
Motor output to the muscles
Innervation of the viscera
- autonomic innervation of abdominal and thoracic viscera (Gut, CV system, breathing)

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4
Q

Where does sensory and motor information enter and exit the spinal cord?

A

Incoming sensory information enters via the posterior (dorsal) root
Motor information exits the cord via the anterior (ventral) root

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5
Q

What is the name of collections of nerve cell bodies in the PNS vs the CNS?

A

PNS - ganglia

CNS - nuclei

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6
Q

Describe the relationship of axons, endoneurium, perineurium and epineurium to each other.

A

Axons are bundled together in little sheaths called the endoneurium.
Lots of axon containing endoneurium bundles are bundles together within sheaths called the perinerium
These perineurum covered bundles are in turn grouped together within epineurium sheaths, and are called nerves

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7
Q

What are the possible nerves and their associated fibre types in the PNS?

A
Large (myelinated) fibres
- motor nerves
- proprioception, vibration and light touch (dorsal column) 
Thinly myelinated fibres
- light touch
- pain and temperature (spinothalamic)
Small (unmyelinated) fibres
- light touch
- pain and temperature
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8
Q

Describe the pysiology of a neuromuscular junction.

A

An action potential arrives along the axon
This causes voltage gated calcium channels to open
The resulting calcium influx causes release of neurotransmitter filled vesicles, which fuse with the cell membrane
ACh is released into the synaptic cleft and binds to receptors, causing ion channels to open
ACh binds to postsynaptic receptors on the muscle, causing a sodium influx which depolarises the cell
This causes voltage gated calcium channels to open in the muscle, allowing an influx of calcium
Calcium influx stimulates muscle contraction

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9
Q

How does a neuropathy of the peripheral motor fibres present clinically?

A

Weakness

Muscle atrophy

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10
Q

How does a neuropathy of the peripheral sensory fibres present clinically?

A
Large myelinated fibres
- sensory ataxia
- loss of vibration sense
- numbness and tingling
Small (thinly myelinated/unmyelinated) fibres
- impaired pin prick/ temperature 
- painful burning
- numbness and tingling
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11
Q

How does a neuropathy of the peripheral autonomic fibres present clinically?

A
Postural hypotension
Erectile dysfunction
GI disturbance
- constipation
Abnormal sweating
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12
Q

What is the most common type of peripheral neuropathy?

A

Length-dependent axonal neuropathy

- so the pathology is within the axon of the nerve itselg

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13
Q

How is length-dependent axonal neuropathy length dependent?

A

Because whatever the toxic cause of the condition it, the longest nerves in the body are exposed to it the most

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14
Q

What is length-dependent axonal neuropathy?

A

A symmetrical, slowly progressive destruction of the longest nerves in the body
- so symptoms begin in the toes and feet

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15
Q

What are the signs and symptoms of length-dependent axonal neuropathy?

A

Burning pain/tingling
in the toes/feet (depending on which nerve are affected)
- slowly spreads up the legs
- starts affecting the hands once the symptoms reach the knee
Tends to affect the thinly/unmyelinated fibres
- no significant sensory ataxia
- little/no weakness

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16
Q

Name at least five causes of length-dependent axonal neuropathy.

A
DIABETES
ALCOHOL
Folate/B12/thiamine/B6 deficiency 
RA
Lupus
Vasculitis 
Renal failure
Hypothyroidism
Drugs (isoniazid, gold, amiodarone)
HIV
Hep B or C
Rarer causes
- charcot-Marie-Tooth
- HNPP
- myeloma
- paraneoplastic syndrome
- critcal illness
17
Q

What is Guillain-Barre syndrome?

A

An acute, inflammatory, demyelinating neuropathy, with a post-infectious autoimmune aetiology

18
Q

What are the clinical features of Guillain-Barre syndrome?

A

Progressive ascending weakness of the limbs over several days
Causes flaccid quadraparesis with areflexia
May or may not have respiratory, bulbar or autonomic involvement

19
Q

What do you expect to find on LP if a peson has Gullain-Barre syndrome?

A

High protein in the CSF

20
Q

Describe the pathophysiology of Guillain-Barre syndrome.

A

Bacteria enter the patients system (e.g. campylobacter, CMV or EBV)
A part of the virus looks anitgenically like the myelin sheath
A few weeks later, the antibodies formed to fight off the virus mistakes the myelin sheath for part of the virus, and attacks it
This starts a cascade in which the immune system starts to destroy the myelin sheath
Demyelination slows down the speed at which action potentials are transmitted

21
Q

How is Guillain-Barre syndrome treated?

A

Immunosuppressants

  • IV immunoglobulin
  • apheresis
22
Q

What is the name of the chronic form of Gullain-Barre syndrome?

A

CIDP

- chronic inflammatory demyelinating polyneuropathy

23
Q

What would a nerve conduction study on someone with Guillain-Barre syndrome show?

A

When a stimulus is applied, you see dispersion of the response

  • it takes longer to travel the same distance as a healthy nerve
  • indicates demyelination
24
Q

List some symptoms of muscle disease.

- depends on which part of the body is affected

A
Proximal limb weakness
Facial weakness
- myopathic facies
- drooling
Eyes
- ptosis
- ophthalmoplegia
Bulbar
- dysarthria, dysphagia 
Neck and spine
- head droop
- scoliosis
Respiratory 
- breathlessness
Myocardial 
- exercise intolerance
- palpitations
25
Q

List some potential different causes of muscle disease.

A
Muscular dystrophies 
- dystrophinopathies
- fascioscapulohumeral dystrophy
- limb girdle
- occulopharyngeal
Metabolic muscle disorders
- glycogen storage disease
- defects of FA metabolism
Mitochondrial disorders
Myotonic dystophies
Inflammatory muscle disorder
- polymyositis
- dermatomyositis
- inclusion body myositis
Neuromuscular junction disorders
- Myasthenia Gravis
- Lambert Eaton syndrome
26
Q

What is Myasthenia gravis?

A

A bilateral, autoimmune disorder of the neuromuscular junction

27
Q

Describe the pathogenesis of Myasthenia Gravis.

A

The body forms autoantibodies to acetylcholine receptors at post-synaptic neuromuscular junctions
This means ACh can’t bind to them, and the muscle can’t contract

28
Q

What other conditions is Myasthenia Gravis associated with?

A

Thymic hyperplasia
Thymoma
Other autoimmune conditions

29
Q

What are the clinical features of myasthenia gravis?

A

Fatiguable weakness of ocular, bulbar, respiratory and or/limb muscles

  • double vision
  • slurred speech
  • difficulty swallowing
  • worse towards the end of the day
  • bilateral
  • poor cough
  • proximal limb weakness
30
Q

What investigations do you perform if you suspect Myasthenia Gravis?

A

Autoanitbody test
- antibodies to AChR present in 85% of cases
Single fibre EMG (electromyography) and repetitive nerve stimulation is abnormal

31
Q

What is the management for Myasthenia Gravis?

A

Pyridostigmine (anti-acetylcholine esterase)
Immunosuppressive therapies
- steroids
- IV immunoglobulin