Week 3 Motor Neurone Disease Flashcards
What is Motor Neurone Disease?
- progressive, degenerative disorder of cortical, brainstem, and spinal motor neurones
- it causes the denervation and atrophy of corresponding mm fibres
What are the Clinical Presentations of MND?
- Insidious Onset
- 50% of Motor neurons can be lost before weakness becomes apparent
- Typical wasting and weakness of limbs
- Cramps and fasciculations
- Combination of UMN and LMN signs: 10% of patient only ever develops LMN signs
Clinical presentation of MND if LMN degeneration predominates…
- Profound atrophy of specific muscles
- Hyporeflexia
- Normal or low tone (hypotonia)
- Fasciculations
Clinical Presentation of MND if UMN predominates….
- general (widespread) wasting of muscles
- hyperreflexia
- hypertonia
- primitive reflexes (positive babinksi or glabellar tap)
How is MND diagnosed?
- there is no specific diagnostic tool for MND yet
- Diagnosis is a mix of clinical diagnosis
- It can be confirmed by EMG
- Other investigations can also be performed such as, genetic testing, MRIs, and pathology
- It may take several months to finalise a diagnosis
- One of the strongest diagnostic feature is its RAPID PROGRESSION
What is Amyotrophic Lateral Sclerosis?
- most common type of MND (accounts for 66% of all patients with MND)
- a mix of LMN AND UMN effects and a mix of limb and bulbar signs
- most commonly affects the hands and presents as clumsiness, and wasting of the thenar eminence
What is Progressive Bulbar Palsy (PBP)?
- next most common form (accounts to about 25% of patients with MND
- Mix of LMN and UMN
- Bulbar symptoms appear first - Dysarthria, Dysphasia, emotional liability, tongue tends to atrophy and fasciculates
What is Progressive Muscular Atrophy?
- accounts for 5-10% of patients with MND and it affects men more than women
- patient presents with progressive neuromuscular atrophy
- initially LMN signs - wasting and weakness of the arms, can progress to bulbar signs - rare and later on the disease
What is Progressive Lateral Sclerosis?
- Very rare condition <1% of MND
- Mainly UMN changes which causes stiffness and paralysis
What are the early symptoms of MND?
- cramping
- slowed movement
- muscle stiffness, twitching, jerking
- difficulty walking or holding objects
- Emotional response triggered easily
- Dysphasia
- Dysarthria
What are the advance symptoms of MND?
- depression
- weight loss, malnutrition, dehydration
- venous thromboembolism
- contractures
- respiratory failure
Important to note that Sensory signs are absent (they remain intact) and cognitive function is rarely affected. Also there is no sphincter involvement.
MND
In the management of MND what is one disease modifying medication?
- Riluzole: has modest reduction in rate of disease progression - this inhibits the release of glutamate
What is Phase 1 of MND?
Phase I - Independent Phase
Stage 1 - Mild weakness and independent with ADLs
Stage 2 - Moderate weakness, difficulty with stairs
Stage 3 - severe weakness and decreased independence in ADLS, Fatigue
What is Phase II of MND?
Phase II - Partially Independent Phase
Stage 4 - likely to have shoulder pain and oedema in the hand. Severe lower limb weakness with or without spasticity
Stage 5 - severe lower limb weakness. Moderate to sever upper limb weakness
What is Phase III of MND?
Phase III - Dependent
Stage 6 - complete dependence on ADLs
Exercise and MND
- combine formal exercise with enjoyable physical activities
- focus more on concentric than eccentric (eccentric exercises may cause greater hypertrophy but it can be more taxing on patients with MND - it can lead to microtraumas)
- moderate resistance has been shown to be better than high resistance exercise
- muscles with less antigravity strength will not improve so FOCUS on stronger muscles
- Monitor for overwork weakness and include adequate rest periods
How does a physiotherapy manage a patient with Stage 1 MND?
- Increase activity if sedentary
- Begin flexible exercise program
- Add strengthening exercise
How does a physiotherapy manage a patient with Stage 2 MND?
- implement stretching to avoid contracture
- Continue cautious strengthening
- Consider orthotic support (AFO, thumb and wrist splint)
- Adaptive equipment (physios work with OT)
How does a physiotherapy manage a patient with Stage 3 MND?
- Continue stage 2 programme as tolerated
- Maintain independence for as long as possible through pleasurable activities such as walking, etc…
- Deep breathing exercises and postural drainage if needed
- Wheelchair prescription to reduce fatigue and maintain function for as long as possible
How does a physiotherapy manage a patient with Stage 4 MND?
- heat and massage to control spasm and pain
- preventative anti oedema massage
- Active assisted ROM exercises
- Encourage isometric contractions as tolerated
- Consider motorised wheelchair for independent mobility
How does a physiotherapy manage a patient with Stage 5 MND?
- teach family and carers transfer and turning techniques
- Involve OT for home modifications and assistance with ADLs
- electric hospital beds and anti-pressure mattress
- Home mechanical ventilation
How does a physiotherapy manage a patient with Stage 6 MND?
- medication for excess saliva
- electronic speech aids for dysarthria
- For breathing difficulty; clear airways, postural drainage, positioning
- Maintain comfort
