Motor neuron disease Flashcards
What is motor neuron diseases?
A cluster of major degenerative diseases characterised by selective loss of neutrons in the motor cortex, cranial nerve nuclei and anterior horn cells.
What type of nerves are affected?
Upper and lower motor neurone, but no sensory loss of sphincter disturbance. It never affects eye movements
What are the four types of MND?
ALS, progressive bulbar palsy, progressive muscular atrophy and primary lateral sclerosis.
What nerves are affects in ALS?
Loss of motor neurone in motor cortex and the anterior horn of the cord.
What are the symptoms of ALS?
Weakness and UMN signs and LMN wasting/fasciculation. Progressive tetra paresis or paraparesis with wasting and fasciculation.
What makes a worse prognosis of ALS?
Bulbar onset, old, thenar wasting.
Which are the most common types of MND?
ALS (50%), The others are 10/20%
What nerves are affected by a bulbar palsy?
Only affects cranial nerves 9-12 in the medulla. LMN lesion of the tongue and muscles of talking and swallowing,
What are the signs of a bulbar palsy?
Flaccid, fasciculating tongue, jaw jerk is normal or absent, speech is quiet, horse or nasal. Dysphagia with wasting.
What nerves are affected by progressive muscular atrophy?
Anterior horn cell lesion only, therefore no UMN signs.
Which muscle groups are affected first by progressive muscular atrophy?
Affects distal muscle groups before proximal. Presents with weakness, wasting and fasciculations in the hands and arms.
Does progressive muscular atrophy or ALS have a better prognosis?
Progressive muscular atrophy
What nerves are affected in primary lateral sclerosis?
Loss of Betz cells in motor cortex, thus mainly UMN signs.
What are the symptoms of primary lateral sclerosis?
Marked spastic leg weakness and psuedobulbar palsy. No cognitive decline. Progressive tetraparesis
How do you diagnose MND?
There is no diagnostic test, it is on clinical features
How common is MND?
6 in 100,000
Is it more common in men or women?
Men:women, 3:2
What is the median age of onset?
60 years
What is the prognosis?
Often fatal in 2-4 years
Who is in the team to help treatment?
A multidisciplinary approach is best: neurologist, palliative nurse, hospice, physiology, speech therapist, OT, dietician, social services
What medication can you give to prolong life?
Riluzole prolongs life by approx. 3 months, it is costly
What are the side effects of Riluzole?
Liver damage (check every 3 months), vomiting, headache, vertigo, increased pulse
What medications can you give to help the drooling?
Amitriptyline, doctors think this is good but patients tend to disagree.
What medications can you give for spasticity?
Baclofen
What can you do to help the dysphagia?
Blend food, or possible NG tube
What medications can you give to help the joint pain and distress?
Analgesic ladder
What can you do to prevent respiratory failure?
Non-invasive ventilation at home may give valuable palliation.
Is it hereditary?
Most cases are sporadic with no family history.
What does motor neurone disease not affect?
Eye movements, the senses (sight, touch, sound) or the bladder or bowel
Will I get cognitive changes?
Around 35% of people with MND experience mild cognitive change, which can cause issues in executive functions such as planning, decision-making and language
What blood test can you get with MND?
It can increase your creatinine kinase, but this is not specific
What nerve tests are there?
Nerve conduction tests and an EMG
Are there any packs I can have in case of emergencies?
MND associate have a just in case kit that has two kits, one for carer’s and one for doctors in case anything bad happens
What is non-invasive ventilation?
a portable machine supports your own breathing by providing extra air through a mask
Is there any preventative measures I can take against any other diseases?
You are advised to get a flu jab each year.
Is there any end of life care decision I should make?
It is easiest to make these decisions whilst you can still speak and communicate properly, it is advised to put someone as your power of attorney.
