Peripheral Neuropathies Flashcards

1
Q

What is a peripheral neuropathy?

A
  • When peripheral nerves are affected by pathological processes resulting in motor, sensory &/or autonomic dysfunction
  • Acquired or inherited
  • AKA polyneuropathy
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2
Q

What are the types of acquired peripheral neuropathies?

A
  • Diabetic
  • Metabolic due to renal disease, alcoholism, drugs/toxins & vitamin deficiency
  • Acute & chronic inflammatory demyelinating (GBS, CIDP)
  • Neuropathy associated with infection or malignancy
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3
Q

What are the types of inherited peripheral neuropathies?

A
  • Motor neuron disease
  • Charcot-Marie Tooth disease
  • Hereditary sensory neuropathy
  • Spinal muscular atrophy
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4
Q

What is Guillian-Barre syndrome?

A
  • Acute inflammatory demyelinating peripheral neuropathy
  • Predominantly motor, although 42-75% of patients experience altered sensation
  • Autonomic dysfunction may occur in severe cases
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5
Q

What is the aetiology of GBS?

A
  • Unknown
  • Usually preceded by respiratory tract infection or GI infection
  • Some reports of immunisation or surgery preceding symptoms
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6
Q

What is the pathophysiology of GBS?

A
  • Auto-immune reaction resulting in inflammation & destruction of myelin sheath of peripheral nerves
  • Loss of myelin causes slowed conduction causing weakness
  • If multiple adjacent nerve fibres become demyelinated it can cause a complete nerve block causing paralysis
  • In 20% of patients, axon will also be destroyed
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7
Q

What are the different forms of GBS?

A
  • Ascending paralysis (most common)
  • Miller Fisher syndrome
  • Acute motor axonal neuropathy
  • Acute motor sensory axonal neuropathy
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8
Q

What is Miller Fisher syndrome?

A
  • Rare form of GBS
  • Involves descending paralysis
  • Presents as ophthalmoplegia, ataxia (due to significant loss of sensation) & areflexia
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9
Q

What is acute motor axonal neuropathy?

A
  • Form of GBS
  • Purely motor neuropathy
  • Recovery can be slow or rapid
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10
Q

What is acute motor sensory axonal neuropathy?

A
  • Form of GBS
  • Also affected sensory nerves
  • Associated with severe axonal damage
  • Recovery is slow & often incomplete
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11
Q

How is GBS diagnosed?

A
  • Clinically, supported by lumbar puncture or nerve conduction studies which may show conduction slowing/block and/or reduced amplitude of action potentials
  • Typical CSF findings include elevated protein level
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12
Q

What is the required clinical criteria for GBS diagnosis?

A
  • Progressive, relatively symmetrical weakness of 2 or more limbs
  • Areflexia
  • Disease course < 4 weeks
  • Exclusion of other causes
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13
Q

What is the prognosis for GBS?

A
  • Most often recovery commences 4 weeks from onset
  • 80% of patients recover completely in 12 months
  • 5-15% have severe disability due to weakness, contracture or sensory loss
  • 5% fatal
  • Approx 5-10% have one or more relapses (classified as having CIDP)
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14
Q

What does the medical management of GBS involve?

A
  • Drug therapy including steroids & immunosuppressants
  • Plasmaphoresis (plasma exchange - shown to decrease recovery time)
  • Immunoglobulin therapy (injection)
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15
Q

What does the physio management of GBS involve?

A
  • Restore motor performance & ADLs to previous level
  • Maintain cardiorespiratory & neuromuscular system while awaiting remyelination
  • Utilise principles of specificity, repetition & feedback
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16
Q

What are the common impairments in GBS?

A
  • Loss of strength
  • Loss of sensation
  • Autonomic dysfunction
  • Contracture
17
Q

What should the physio assessment of GBS include?

A
  • Strength
  • Contracture
  • Sensation
  • Activities (bed mobility, sitting up, sitting, standing up, standing, walking, R&M)
18
Q

What are the physio interventions for GBS?

A
  • Respiratory physio
  • Strength training
  • Serial casting
  • Sensory retraining
  • Activity training (part, modified or whole task)
19
Q

What did Ruhlands & Shields 1997 find regarding strengthening in CIDP?

A
  • Home exercise program including mild strengthening, stretching & aerobic exercise over 6 weeks
  • Resulted in significant increase in strength & QOL
20
Q

What did Khan et al 2011 find regarding rehab in GBS?

A
  • RCT of GBS patients
  • Compared high intensity vs low intensity rehab (additional 3 hours over 3 months)
  • Significant but small improvement in FIM scores & Perceived Impact Problem Profile at 12 months
21
Q

What did Bussmann et al 2007 find regarding general exercise programs in GBS?

A
  • Examined effects of exercise in severely fatigued GBS patients on physical fitness, fatigue, objectively measured actual mobility, perceived physical functioning & perceived mental functioning
  • 12 week cycling program 30 mins 3 times/week
  • Significant improvement in all domains
  • Strong correlation between fatigue & perceived physical function
22
Q

What is motor neurone disease?

A
  • Terminal progressive degenerative disease
  • Affects anterior horn cells of spinal cord, corticospinal tract & motor nuclei of brain stem
  • Results in upper & lower motor neuron lesions & bulbar palsy (dysarthria, dysphagia)
23
Q

What is the clinical presentation of MND?

A
  • Insidious onset, aetiology unknown

- Up to 50% of motor neurons can be lost before weakness is evident as a consequence of neural plasticity

24
Q

What are the 3 main forms of MND?

A
  • Amyotrophic lateral sclerosis
  • Progressive bulbar palsy
  • Progressive muscular atrophy
    (although patients can present with a mixture of signs & symptoms)
25
Q

What is amyotrophic lateral sclerosis (ALS)?

A
  • Accounts for 65% of all MND
  • Involves UMN, LMN & bulbar signs
  • Hands most commonly affected first (report dropping things frequently etc)
26
Q

What is progressive bulbar palsy?

A
  • Accounts for approx 25% of MND
  • Initially results in dysarthria & dysphagia (often unable to communicate verbally or swallow)
  • May have atrophied/spastic tongue
  • Associated with emotional lability
  • Upper & lower limb occurs with progression of the disease
27
Q

What is progressive muscular atrophy?

A
  • Accounts for approx 10% of MND
  • Predominantly affects men < 50 years
  • Only affects LMN & usually upper limbs first
  • With progression there is lower limb weakness & bulbar involvement
  • Progression is slower than in other forms & most patients live 5-10 years after diagnosis
28
Q

What are some of the other problems associated with MND?

A
  • Pain
  • Dyspnoea (reported in approx 50%)
  • Sore eyes (unable to blink enough)
  • Constipation
  • Insomnia (pain, orthopnoea, fear of not waking up)
  • Fatigue
  • Pressure areas (less common than SCI due to intact sensation)
  • Urinary retention
29
Q

What is the role of physio in MND?

A
  • Maintenance of strength, length & ROM
  • Promote mobility & independence with aids & adaptations
  • Education & reassurance
  • Pain & fatigue management
  • Management of respiratory dysfunction
30
Q

What did Lunetta et al 2016 find regarding exercise in patients with ALS?

A
  • RCT of patients with ALS
  • Compared supervised exercise program (cycling & strength training) for 6 months with passive exercise & stretching
  • Significantly better motor score in ALS functional rating scale in experimental group
  • No difference in respiratory decline or QOL
31
Q

What are the guidelines for exercise in MND?

A
  • Encourage patients to continue sports/activities if safe
  • Encourage low intensity activities
  • Encourage active lifestyle when sport is no longer possible
  • Exercise for short bursts often to minimise fatigue
  • Include stretching, strengthening & aerobic work
  • Educate patients that exercise will not cure the condition or prevent deterioration but may improve QOL if they enjoy exercise
  • Rigid adherence to exercise is not necessary
  • Let the patient do what they want
32
Q

What needs to be considered for seating in MND?

A
  • Meeting individual needs
  • Maintaining muscle length
  • Preventing deformity
  • Improving comfort
  • Preventing pressure areas
  • Maximising function & social interaction
33
Q

What are some of the assistive devices used in MND?

A
  • Orthoses: Improve mobility & protect soft tissue from trauma due to malalignment
  • Neck collars for cervical weakness
  • Mobile arm supports & splints for upper limb function
  • Communication systems (ipad apps, pointer charts, light-writers, computers)
34
Q

What does management of the terminally ill patient involve?

A
  • Palliative care starts from diagnosis
  • Incorporates psychological care, care of the family & counselling
  • Problems must be anticipated so symptoms are well controlled & patient/family are well supported
  • Final deterioration is usually very short (< 12 hours), most commonly from respiratory infection
35
Q

How is the physio management different for a GBS patient compared to a stroke patient?

A
  • No dexterity training
  • No spasticity management
  • No e stim (doesn’t work when the peripheral nerve is not intact)