Motor Neurone Disease Flashcards
What is the definition of motor neurone disease?
Devastating condition causing progressive weakness and eventually death, usually as a result of respiratory failure or aspiration.
What are the types of MND?
Amyotrophic lateral sclerosis (most common)
Primary lateral sclerosis
Progressive bulbar palsy
Progressive muscular atrophy
What is the pathophysiology of MND?
Degeneration of predominantly upper and lower motor neurones in the spinal cord, cranial nerve motor nuclei and cortex
What are some general clinical features of MND?
Upper motor neurone signs (spasticity, hyperreflexia, upgoing plantars)
Lower motor neurone signs (fasciculations, atrophy)
Generally eye and sphincter muscles spared until late disease course
No sensory disturbance
Focal onset and continuous spread, finally generalised paresis
What are some features of ALS?
Simultaneous involvement of upper and lower motor neurones, usually in one limb and spreading to others and trunk
Split hand syndrome (wasting of thenar eminence)
What are some features of progressive bulbar palsy?
Early tongue and bulbar involvement
- Dysarthria
- Dysphagia
- Nasal regurgitation of fluids and choking
What are some features of progressive muscular atrophy?
Lower motor neurone features only
Starting in one limb and spreading to adjacent spinal segments
What are some features of PLS?
Upper motor neurone features only
Slowly progressive tetraparesis and pseudobulbar palsy
What is the prognosis for ALS and PLS?
ALS = poor (3-5 years)
PLS = better (>5 years)
What are some investigations for MND?
Clinical diagnosis
EMG to confirm degeneration of lower motor neurones
Investigations to exclude other disorders and confirm involvement of muscle groups (eg paraspinal muscles)
What is the ongoing management for MND?
Assessment of needs and coordination of care
Speech therapy, AAC
Nutritional needs (metabolic rate doubled in MND)
Respiratory needs (ventilation)
Riluzole (slows progression slightly and increases life expectancy by 3-4 months)
What is the symptomatic treatment of sialorrhoea in MND?
Hyoscine/buscopan
Glycopyrronium
Botox
Suction
What is the symptomatic treatment of muscle cramps and spasms in MND?
Quinine
Baclofen
Tizanidine
Dantroline
Gabapentin
What is the symptomatic treatment of emotional lability in MND?
Sometimes treated with antidepressants
