Motor Neurone Disease Flashcards
What is Motor Neurone Disease (MND)?
Degenerative disease of upper/lower motor neurones in spinal cord, CN motor nuclei & cortex
What is the aetiology of MND?
Cause unknown, genetic factors
Incidence 2/100,000
Onset b/ 50-70yrs
What are the four patterns of MND?
Amylotrophic lateral sclerosis
Progressive muscular atrophy
Primary lateral sclerosis
Bulbar presentation
What is amylotrophic lateral sclerosis?
Most common
Loss of spinal/brain stem LMB & cortical UMN
Associated w/ FTD
How does amylotrophic lateral sclerosis present?
LMN weakness - starting in hands, progressing to upper arms/legs, wasting, fasciculation
UMN spastic weakness - progressive spastic tetraparesis
Bulbar/pseudobulbar palsy
What signs are present in amylotrophic lateral sclerosis?
Muscle wasting
Fasciculation
Brisk reflexes
Extensor plantars
What are the features of progressive muscular atrophy?
Loss restricted to LMN
Painless wasting in small muscles of hands, spreads
Wasting/fasciculation
What are the features of primary lateral sclerosis?
Rare, disease confined to cortical UMN
Progressive tetraparesis
What are the features of a bulbar presentation?
Bulbar sx w/ preservation of limb function in early stages
Poor prognosis due to early resp involvement
What investigations are appropriate in suspected MND?
Bloods - rule out differentials
Spinal cord MRI - rule out myelopathy/radiculopathy
EMG - evidence of denervation
What is the management of MND?
Specialist MDT care Social/carer assessment Riluzole Nutritional support Resp support (NIPPV) Treatment of complications (as per MS)
How does Riluzole work?
Increases pre-synaptic glutamate release
Increases survival in ALS pts by 3-4mo
What is the prognosis of MND?
Remission unknown
Death from bronchopneumonia/ventilatory failure due to weakness of resp muscles
