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Neuropathies Flashcards

1
Q

Define neuropathy (2)

A
  1. Disease or dysfunction of one or more of the peripheral nerves
  2. Typically causes numbness or weakness
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2
Q

What are peripheral nerves?

A
  1. These nerves send messages from the central nervous system, the brain and the spinal cord to the rest of the body
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3
Q

Give 3 types of neuropathy

A
  1. Charcot-Marie-Tooth disease
  2. Guillian-Barre syndrome
  3. Myasethenia gravis
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4
Q

Describe Charcot-Marie-Tooth disease (4)

A
  1. Group of hereditary neuropathy disorders affecting peripheral nerves
  2. Muscles of lower legs become weak and waste away
  3. Over 40 types
  4. Motor and sensory neuropathy
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5
Q

What is the prevalence of Charcot-Marie-Tooth disease?

A

1/2,500

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6
Q

When is the onset of Charcot-Marie-Tooth disease?

A
  1. Symptoms usually begin between 5-15 years, sometimes middle age or later
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7
Q

How is Charcot-Marie-Tooth disease classified?

A
  1. Based on the damage caused:
    Demyelination
    Damage to the axon
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8
Q

What is the aetiology of Charcot-Marie-Tooth disease?

A

Inherited fault in one of the many genes responsible for development of the peripheral nerves

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9
Q

What is the inheritance pattern of Charcot-Marie-Tooth disease? (2)

A
  1. Most inherited in autosomal dominant trait (only one gene from one parent required)
  2. Chances of passing on to offspring depends on the specific genetic faults carried
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10
Q

What are 6 symptoms of Charcot-Marie-Tooth disease?

A
  1. Muscle weakness
  2. Awkward or ‘slapping’ gait
  3. Highly arched or very flat feet, hammer toes
  4. Numbness in feet, arms and hands
  5. Weakness begins in lower legs
  6. Later hand muscles begin to waste away
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11
Q

Where does muscle weakness occur in Charcot-Marie-Tooth disease? (5)

A

Feet, ankles, legs, hips and hands

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12
Q

What is a slapping gait?

A

Feet hit the floor hard whilst walking

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13
Q

How does the weakness in lower legs progress? (3)

A
  1. Weakness begins in lower legs
  2. Causes an inability to flew the ankle to life the front part of the foot (foot drop)
  3. Wasting away of calf muscles (stork leg deformity)
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14
Q

Give a summary of the diagnosis of Charcot-Marie-Tooth disease

A
  1. Electromyography and nerve conduction studies
  2. Physical evaluation
  3. Genetic counselling
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15
Q

What does the physical examination of Charcot-Marie-Tooth disease involve? (3)

A
  1. Which areas are weak
  2. Onset of symptoms
  3. Family history
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16
Q

What is the prognosis of Charcot-Marie-Tooth disease? (5)

A
  1. No cure
  2. Progresses slowly and does not affect life span
  3. Differs in severity
  4. Can make everyday activities difficult
  5. May have significant emotional impact
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17
Q

What may be the symptoms of mild Charcot-Marie-Tooth disease?

A

High arches and hammer toes may be the only symptoms

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18
Q

Summarise the treatment of Charcot-Marie-Tooth disease

A
  1. Physiotherapy
  2. Occupational therapy
  3. Braces and other orthopaedic devices
  4. Pain medication
  5. Orthopedic surgery
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19
Q

What is Guillain-Barre syndrome? (2)

A
  1. Rare, short term but potentially life threatning disorder affecting the nerves in the body
  2. Immune system attacks part of the peripheral system so signals cannot be sent from the brain to the muscles as quickly as they should
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20
Q

What does Guillain-Barre syndrome cause?

A

Muscle weakness which worsens over a few days to weeks

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21
Q

When does Guillain-Barre syndrome occur? (2)

A
  1. Can occur at any age, but especially common in people older than 50 years
  2. Most children recover fully with no complications
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22
Q

Outline the aetiology of Guillain-Barre syndrome (4)

A
  1. Exact cause is unknown
  2. Few days or weeks after viral infection
  3. Rare cases: may run in families
  4. Vaccinations, certain medications and surgical procedures
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23
Q

Give 4 types of viral infections which may be linked to Guillain-Barre syndrome

A
  1. Cytomegalovirus
  2. Epstein-Barr virus
  3. Flu
  4. Zika
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24
Q

Give a type of bacterial infection which is linked to Guillain-Barre syndrome

A

Campylobacter jejuni

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25
Q

Outline the inheritance patterns of Guillain-Barre syndrome (3)

A
  1. Mutations in a particular gene are not known to be associated with GBS
  2. Not thought to be passed directly from parent to child
  3. In rare cases, multiple people in one family have GBS
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26
Q

Is there a genetic component of Guillain-Barre syndrome?

A

Family members may share normal variants in certain genes which may increase the risk of GBS

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27
Q

How long do the symptoms of Guillain-Barre syndrome continue to worsen for?

A

Worsen over first 2-3 weeks

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28
Q

What are 10 symptoms of Guillain-Barre syndrome?

A
  1. Muscle weakness, pain and short term paralysis of face, chest, leg and pharynx muscles
  2. Breathing problems, choking and aspiration
  3. Decreased sensation in fingers and toes
  4. Leg weakness
  5. Leg pain that moves to arms
  6. Problems with walking
  7. Irritability
  8. Dysphagia
  9. Facial weakness eg. facial droop
  10. Vision changes
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29
Q

What 2 symptoms can impact vision in Guillain-Barre syndrome?

A
  1. Double vision

2. Changes in eye movements

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30
Q

What are 3 other symptoms of Guillain-Barre syndrome?

A
  1. Difficulty with bladder/bowel function
  2. Blood pressure
  3. Heart rate
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31
Q

Summarise how Guillain-Barre syndrome is diagnosed (5)

A
  1. Physical exam: symptoms and history
  2. Blood tests and urine tests
  3. Lumbar puncture
  4. Electromyogram (EMG)
  5. Pulmonary function test
32
Q

What symptoms are investigated during the physical exam of Guillain-Barre syndrome?

A
  1. Muscle weakness on both sides of the body that starts from lower leg muscles and spreads upwards
  2. Relatively rapid progression and loss of muscle reflexes
33
Q

Why is a lumbar puncture conducted in Guillain-Barre syndrome?

A

People with GBS have high levels of protein in the CSF

34
Q

Why is an EMG carried out in Guillain-Barre syndrome?

A

Determine if the muscles are responding to signals correctly and nerve conduction studies measure speed of signals

35
Q

What is the prognosis of Guillain-Barre syndrome? (5)

A
  1. Speed of recovery depends on severity of nerve damage
  2. Can occassionally be life-threatening and some are left with long term problems
  3. Often treated in ICU
  4. Some may relapse after an original improvement in symptomms
  5. Depression and anxiety
36
Q

When is recovery expected in Guillain-Barre syndrome? (2)

A
  1. Symptoms can last up 1-2 months

2. Full recovery is at least 1-2 years after diagnosis

37
Q

What percentage of people of GBS continue to have muscle weakness years after the first symptoms?

38
Q

What can be the causes of death in Guillain-Barre syndrome? (4)

A
  1. Acute respiratory distress syndrome
  2. Sepsis
  3. Pulmonary emboli
  4. Cardiac arrest
39
Q

What can be a consequence if GBS is not treated?

A

Paralysis of the chest muscles leading to respiratory problems and death

40
Q

Summarise the treatment of Guillain-Barre syndrome (6)

A
  1. Depends on symptoms, age, severity and general health
  2. Early management is crucial
  3. Intravenous immunoglobulin (IVIG)
  4. Plasma exchange (plasmapheresis)
  5. Supportive care
  6. Symptom reduction
41
Q

What is plasmapheresis? (3)

A
  1. Removes plasma from the blood and replaces it with other fluids
  2. Anti-bodies removed
  3. May help reduce symptoms of GBS
42
Q

What professionals may be used in MDT care? (2)

A
  1. PT to reduce stiffness of muscles and contracted joints and regain muscle strength
  2. SLT: speech and swallowing abilities
43
Q

Define myasthenia gravis

A
  1. Rare, long-term autoimmune disorder causing episodic muscle weakness and easy fatiguability
44
Q

What is myasthenia gravis caused by? (2)

A
  1. Autoantibody and cell-mediated destruction of acetylcholine receptors
  2. Prevents a muscle contraction occuring
45
Q

In what age groups is myasthenia gravis more common in? (3)

A
  1. Young women (20-40)
  2. Older men (50-80)
    But may occur at any age
46
Q

What is the annual incidence in myasthenia gravis?

A

1 in 500,000

47
Q

what is the prevalence of myasthenia gravis?

A

1 in 2,500-200,000

48
Q

Give 4 uncommon forms of myasthenia gravis

A
  1. Ocular MG
  2. Congential myasthenia
  3. Neonatal myasthenia
  4. Juvenile MG
49
Q

Describe ocular MG

A

Involves only the eye muscles (15% of cases)

50
Q

Describe the aetiology of congenital myasthenia (2)

A
  1. Rare autosomal recessive disorder that begins in childhood
  2. Not immune-mediated and results from presynaptic or postsynaptic abnormalities
51
Q

What are the symptoms of congenital myasthenia? (6)

A
  1. Opthamoplegia is common (weakness/paralysis of one or more eye muscles)
  2. Generalised weakness in arms and legs
  3. Delays in motor skills
  4. Feeding difficulties
  5. Weak eyelids
  6. Poor head control
52
Q

Describe neonatal myasthenia (3)

A
  1. Affects 12% of infants born to women with MG
  2. Due to the antibodies that passively cross the placenta
  3. Caused generalised muscle weakness which resolves in days-weeks as antibodies decline
53
Q

Describe juvenile MG (3)

A
  1. Typically develops in female adolescents (10% of MG cases)
  2. Life-long condition that may go in and out of remission
  3. Excessively tired after little activity, problems in swallowing and chewing
54
Q

Describe the aetiology of myasthenia gravis (2)

A
  1. Autoimmune attack on postsynaptic acetylcholine receptors

2. Disrupts neuromuscular transmission at neuromuscular junction

55
Q

What is the trigger for autoantibody production in myasthenia gravis? (4)

A
  1. Unknown
  2. Some cases linked to tumours in thymus gland
  3. Variations in certain genes may increase risk of MG
  4. Associated with abnormalities in the thymus, autoimmune hyperthyroidism and other autoimmune disorders
56
Q

Describe the role of the thymus in myasthenia gravis (2)

A
  1. Unclear but 65% of patients have thymic hyperplasis

2. 10% have thymoma (about half are malignant

57
Q

What are precipitating factors of myasthenia gravis?

A
  1. Infection
  2. Surgery
  3. Certain drugs
58
Q

Describe the inheritance patterns of myasthenia gravis (2)

A
  1. Not directly inherited

2. Genetic predisposition to autoimmune disease can run in families

59
Q

When do the symptoms worsen in myasthenia gravis?

A
  1. Worsen with muscle activity and improve with rest
60
Q

What are the symptoms of myasthenia gravias? (11)

A
  1. Droopy eyelids
  2. Double or blurred vision
  3. Difficulty making facial expressions
  4. Dysphagia
  5. Dysarthria, nasal voice
  6. Weak arms, legs, fingers, hands of neck
  7. Shortness of breath/serious breathing difficulties
  8. Unstable/waddling gait
  9. Chest infections
  10. Difficulty with physical tasks
  11. Aching muscles after use
61
Q

What is a myasthenia crisis? (3)

A
  1. 15-20% have severe episodes at least once in their life
  2. Sometimes triggered by infection
  3. Arms and legs may become extremely weak but do not lose sensation
62
Q

Summarise the diagnosis of myasthenia gravis (7)

A
  1. Ice pack or rest tests
  2. Electromyography (EMG)
  3. Blood tests
  4. Endrophonium injection
  5. CT or MRI to assess thymus gland
  6. Genetic tests
  7. Muscle biopsy
63
Q

Why is an ice test used in myasthenia gravis? (3)

A
  1. Weakness lessens in cooler temperature
  2. Apply ice to eyes for two minues
  3. A positive result is full or partial ptosis
64
Q

Why are blood tests used to diagnose myasthenia gravis? (2)

A
  1. Detect antibodies of acetylcholine receptors and check for other disorders
  2. Not everyone will have high levels, particularly if only affecting eyes
65
Q

Why is endrophonium used to diagnose myasthenia gravis? (3)

A
  1. If patient has sudden improvement in muscle strength - MG is likely
  2. Usually done if still unclear what’s causing symptoms
  3. Rarely done as there is a risk of serious side effects eg. slow heartbeat, breathing problems
66
Q

What is the prognosis of myasthenia gravis? (3)

A
  1. With treatment: most improve their weakness and live full lives
  2. Sometimes respiratory failure - medical emergency
  3. Some may go into remission where muscle weakness disappears
67
Q

Summarise the treatment of myasthenia gravis (5)

A
  1. Medication
  2. Supportive care
  3. Avoiding triggers
  4. Surgery
  5. Immunomodulating treatment
68
Q

Give 5 medications with a used in myasthenia gravis

A
  1. Anticholinesterase drugs to relieve symptoms
  2. Corticosteroids
  3. Steroids (eg. prednisolone)
  4. Pyridostigmine
  5. Immunosuppresents
69
Q

What is the role of pyridostigmine in treatment of myasthenia gravis? (3)

A
  1. Helps electrical signals travel between nerves and muscles
  2. Can reduce muscle weakness but only lasts a few hours
  3. Side effects: stomach cramps, diarrhoea, muscle twitching and nausea
70
Q

What is the role of steroids in treatment of myasthenia gravis? (2)

A
  1. Reduce the activity of the immune system to stop attacking the nervous system
  2. Unpleasant side effects eg. weight gain, mood swings, increased infection risk
71
Q

Give 2 examples of immunosuppresents

A

Azathioprine

Mycophenolate

72
Q

When is immunomodulating treatment used? (3)

A
  1. Severe cases/myasthenic crisis
  2. Slows disease progression and helps relieve symptoms
  3. Not used in congenital treatment
73
Q

What are 2 types of immunomodulating treatment?

A
  1. Plasma exchange

2. Intravenous immunoglobulin

74
Q

What type of surgery is used to treat myasthenia gravis?

A

Remove the thymus gland (thymectomy)

75
Q

What are the benefits of thymectomy in myasthenia gravis?

A
  1. Improves symptoms in those with a large thymus (not in those which has grown abnormally)
  2. Can reduce doses of steroids and immunosuppresents
  3. Reduces hospital visits due to worsening symptoms
  4. 50% have stable, long-lasting remisions after

Neurology (17 decks)

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