Motor Neuron Disease Flashcards
What is another name for MND?
ALS
Lou Gehrig’s Disease
What is MND?
Untreatable rapidly progressive neurodegenerative condition
How common is MND?
200 cases per year in Scotland
What is the median survival for a patient diagnosed with MND?
3 years
What causes MND?
90% sporadic
10% familial
Name the four classifications of MND
- Primary lateral sclerosis
- Progressive muscular atrophy
- Amyotrophic lateral sclerosis
- Bulbar onset
Describe primary lateral sclerosis
Very rare, motor dysfunction but upper motor neuron phenotype - no muscle wasting and very tight muscles, good prognosis
Describe progressive muscular atrophy
Very rare, lower motor neuron phenotype, muscle wasting, weakness and spasticity - variable prognosis
Describe amyotrophic lateral sclerosis
Mixed upper and lower motor neuron signs - muscle wasting, fasciculations, brisk reflexes and increased tone. Most common type, poor prognosis.
Describe bulbar onset MND
No peripheral involvement, arms and legs are fairly well preserved. Wasted fasciculated tongue, hypertonic nasal speech. Confined to the mouth, more common in women and usually generalises to ALS.
What interventions can be used in bulbar onset MND?
Speech and language therapy
URT management
Eating and nutritional advice
State the upper motor neuron signs
- increased tone
- hyper-reflexia
- extensor plantar responses
- spastic gait
- exaggerated jaw-jerk
- slowed movements
State the lower motor neuron signs
- muscle wasting or contractures
- weakness
- fasciculations
- absent/reduced tendon reflexes
Do patients more commonly have upper or lower motor neuron signs?
90% have lower motor neuron signs
What is a bad survival sign in MND?
MND katabolism - increased metabolic function and weight loss
What is split hand syndrome?
Wasting of the thenar group, typical pattern of atrophy seen in ALS and preservation of the abductor digit minmi
What indicates a benign prognosis?
ALS variant with flail arm or leg syndrome
Primary lateral sclerosis
Focal distal spinal muscular dystrophy
Kennedy’s disease
What is the name of the classification system used for the diagnosis of MND?
El Escorial Criteria - includes EMG and other neuroimaging to rule out other diseases
What is the only drug currently that can be used in MND?
Riluzole - prolongs life by 3 months at most disabled but causes severe liver damage
Describe the communication aids that can help patients when they are unable to communicate properly
Ipad Eye gaze Pen and paper Word board Voice bank may be created early on in the disease
What can be done for patients who are struggling to eat?
PEG inserted endoscopically
RIG inserted radiologically
NG tube
All have a narrow window of efficacy
Define sialorrhoea
Excess saliva
How can excess saliva be managed?
Buscopan, glycopyrronium, botox into glands, suction or humification, carbocistein thickens saliva
How can a dry mouth be managed?
Fluids
Artificial salivation
As patient’s disease progresses how can nutrition and swallowing difficulties be managed?
Liquid drug preparations
Supplements and thickeners
How can muscle cramps be managed?
Quinine, baclofen
How can muscle spasms be managed?
Baclofen, gabapentin, dentrolene, cannabis therapy spray
What is one of the main causes of MND death?
Weakness of respiratory muscles
What are the red flag respiratory symptoms?
Breathlessness Orthopnoea Recurrent chest infections Disturbed sleep Nightmares Poor concentration Type 2 respiratory failure
What is used in MND patients with respiratory problems?
Non-invasive ventilation - start at night and evolve into daytime use - mask needs to be tight and this is sometimes not well tolerated
How can SOB and anxiety be managed?
Lorazepam
