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Neurology > Disorders of the PNS > Flashcards

Disorders of the PNS Flashcards

Peripheral nerve compartments, Mononeuritis multiplex, Facial Nerve palsy, Hereditary sensory motor neuropathy, Guillain-Barré syndrome

1
Q

Ulnar nerve entrapment/ compression site

A

Cubital Tunnel (elbow)

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1
Q

Median Nerve entrapment/ compression site

A

Carpal Tunnel (wrist)

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2
Q

Radial Nerve entrapment/ compression site

A

Spiral groove (of Humerus)

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3
Q

Posterior Interosseous Nerve entrapment/ compression site

A

Supinator Muscle (forearm)

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4
Q

Lateral cutaneous nerve of thigh entrapment/ compression site

A

Inguinal Ligament

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5
Q

Common Peroneal Nerve entrapment/ compression site

A

Neck of fibula

Trauma here associated with foot drop.

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6
Q

Posterior tibial nerve entrapment/ compression site

A

Tarsal Tunnel (flexor retinaculum - foot)

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7
Q

What is mononeuritis Multiplex?

A

A Type of Peripheral neuropathy which is characterised by simultaneous or sequential loss of sensory or motor function in individual non-contiguous nerve trunks.

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8
Q

What are some causative / Associated medical conditions in Mononeuritis Multiplex?

A
  • Diabetes Mellitus
  • Leprosy
  • Vasculitis (eosinophilic granulomatosis with polyangitis amyloidosis - Most common cause)
  • Malignancy
  • Neurofibromatosis
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9
Q

Is mononeuritis multiplex symmetrical or Asymmetrical?

A

Asymmetrical.

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10
Q

When is pain caused by Mononeuritis Multiplex the worst?

A

Worse at night and frequently in the lower back, hip or leg.
Deep, aching pain.

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11
Q

When would patients with mononeuritis multiplex encounter acute, unilateral and severe thigh pain followed by anterior muscle weakness and loss of knee reflex?

A

When it’s a patient who also has Diabetes Mellitus.

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12
Q

How do upper motor neurons innervate the forehead?

A

Each side of the forehead has upper motor neurone innervationbyboth sidesof the brain

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13
Q

How do lower motor neurones innervate the forehead?

A

Each side of the forehead only has lower motor neurone innervation from one side of the brain.

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14
Q

Would the forehead be spared in a Stroke?

A

Yes
- In an upper motor neurone lesion (stroke / tumour) the forehead will be spared as its innervated by both hemispheres.
- The patient will be able to move their forehead on the affected side.

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15
Q

Would a patient with Bell’s Palsy have their forehead spared?

A

NO
- In lower motor neuron lesions (Bell’s Palsy, Ramsay-Hunt) the forehead is not spared and the patient will not be able to move it on the affected side.

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16
Q

Is Ramsay-Hunt syndrome an Upper or Lower motor neurone lesion?

A

Lower

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17
Q

What causes Bell’s palsy?

A
  • Idiopathic
  • Viral infections (herpes simplex virus type 1)
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18
Q

What is the treatment of choice for Bell’s palsy?

A

If patient presents within 72hrs of symptoms - PREDNISOLONE

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19
Q

How long does Bell’s palsy take to resolve?

A

Majority of Patients fully recover over several weeks but recovery may take up to 12 months.

20
Q

What is the cause of Ramsay-Hunt Syndrome?

A

Varicella Zoster Virus (VZV)

21
Q

Apart from the Unilateral Lower motor neurone facial nerve palsy.. what can patients with Ramsay-Hunt syndrome present with?

A

Stereotypically have a painful, tender VESICULAR RASH in the ear canal, pinna and around the ear on the affected side.
- can extend to the anterior 2/3rds of the tongue.

22
Q

What is the management of Ramsay-Hunt syndrome?

A

Oral Prednisolone + Acyclovir

23
Q

What is Hereditary sensory motor neuropathy also known as?

A

Charcot marie-tooth disease

24
Q

What is Charcot marie-tooth disease?

A

An Inherited disease that affects the peripheral motor and sensory nerves.

25
Q

What is the most common type of Mutation in Hereditary sensory motor neuropathy?

A

HSMN Ia is the most common type (70%)
- An autosomal dominant demyelinating neuropathy.

26
Q

What is the typical presenting symptom in Charcot-Marie tooth disease?

A

Weakness of the feet and Ankle.

27
Q

In what disease does an affected adult typically have a bilateral foot drop, symmetrical atrophy of muscles below the knee, pes cavus, atrophy of intrinsic hand muscles (esp thenar muscles of thumb) and absent tendon reflexes in both upper and lower extremities?

A

Hereditary sensory motor neuropathy (charcot marie tooth disease)

28
Q

What is “stork leg appearance”?

A

Symmetrical atrophy of muscles below the knee.
Seen in Charcot-marie tooth disease.

29
Q

What are the Investigations done in Hereditary sensory motor neuropathy?

A
  • Tests done to exclude other causes of neuropathy. e.g. bloods, serum and urine protein elecrophoresis, muscle biopsy, CSF.
  • Genetic studies
  • Nerve conduction studies.
30
Q

What is the management of Charcot marie tooth disease?

A

Currently no effective Tx.
Management is supportive.

31
Q

What is Guillain-barré syndrome?

A

An ‘Acute paralytic polyneuropathy’ that affects the peripheral nervous system.

32
Q

What is the difference between Demyelinating and axonal neuropathies?

A

Demyelinating: A disorder that causes degeneration of the myelin around the axon.

Axonal: A disorder that causes degeneration of the Axon itself.

Given enough time Demyelinating neuropathies will eventually damage the Axon.

33
Q

Is Guillain-barré syndrome a Demyelinating or an Axonal neuropathy?

A

Usually Demyelinating but can occasionally be Axonal.

34
Q

After what infection does Guillain-barré syndrome typically present?

A

Campylobacter jejuni, CMV or EBV.
within 4 weeks post infection.

35
Q

What is the name given to the process that causes damage to peripheral neurones in Guillain-barré syndrome?

A

Molecular Mimicry.
- B cells create antibodies against antigens on the pathogen that the host is infected with.
- These antibodies also match proteins on the nerve cells and may target proteins on the myelin sheath of the motor nerve cell or the nerve axon.

36
Q

What are the symtoms associated with Guillain-barré syndrome?

A
  • Symmetrical ascending weakness (starting in the feet and moving up the body)
  • May progress to the cranial nerves and cause facial nerve weakness.
37
Q

Are reflexes increased or reduced in Guillain-barré syndrome?

A

Reduced

38
Q

Is there loss of sensation in Guillain-barré syndrome?

A

There may be peripheral loss of sensation or neuropathic pain.

39
Q

How is Guillain-barré syndrome usually diagnosed?

A

Clinically.

40
Q

What investigations can be used to support a diagnosis of Guillain-barré syndrome?

A
  • Nerve conduction studies
  • Lumbar puncture
41
Q

What would a lumbar puncture show in Guillain-barré syndrome?

A

Raised protein with a normal cell count and glucose.

42
Q

What is essential to monitor in Guillain-barré syndrome patients?

A
  • Bedside spirometry (FVC)
    Essential due to risk of Respiratory failure.
43
Q

What is the treatment in Guillain-barré syndrome?

A
  • IV Immunoglobulin (first line)
    or
  • Plasma Exchange (typically if IV Ig is ineffective)
44
Q

What is the leading cause of death in Guillain-barré syndrome patients?

A

Pulmonary embolism.
- VTE prophylaxis is essential.

45
Q

What do polyneuropathies such as Metabolic neuropathies, Toxic neuropathies, Neuropathies in cancer, Neuropathies in systemic disease and Autonomic neuropathies cause damage to?

A

All result in Axonal Loss peripherally.

46
Q

What is an example of an Acute Demyelinating neuropathy? A Chronic Demyelinating Neuropathy?

A

Acute: Guillain-barré syndrome. (days to weeks)

Chronic Charcot-marie tooth disease (Months to years)

47
Q

How is Ramsay-Hunt syndrome Investigated?

A

Otoscopy (vesicular rash in the ear canal)
- Ramsay-Hunt syndrome can also cause hypersensitivity to loud noise (likely due to the rash)

48
Q

What is the difference between Charcot-Marie tooth disease type 1 and type 2?

A

Type 1 - Is a Demyelinating syndrome.

Type 2 - Is an axonal syndrome

Neurology (13 decks)

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