Motor Neuron Disease Flashcards
What is MND?
A cluster of neurodegenerative diseases characterised by selective loss of neurons in the motor cortex, cranial nerve nuclei and anterior horn cells
What distinguishes MND from MS and polyneuropathies?
UMN and LMN can be affected but there is no sensory loss or sphincter disturbance
What is amyotrophic lateral sclerosis?
Loss of motor neurons in motor cortex and in the anterior horn of the spinal cord
What is progressive bulbar palsy?
Only cranial nerves IX-XII are affected
What is progressive muscular atrophy?
Anterior horn cell lesion so LMN signs only. Distal muscles are affected first
What is primary lateral sclerosis?
Loss of Betz cells in motor cortex
What features are common in MND?
Stumbling spastic gait, wrist/foot drop, change in appearance of hands, excessive fatigue
What are UMN signs?
Babinski reflex, increased muscle tone, muscle weakness, overactive reflexes
What are LMN signs?
Fasciculations, decreased muscle tone, muscle weakness, decreased reflex response
What are bulbar signs?
Speech or swallowing difficulties
What diagnostic criteria is used?
El Escorial
What medications can improve survival?
Riluzole, NMDA receptor antagonists
What end of life care should you consider?
Involve a palliative care team from diagnosis, opioids, discuss wishes early
What are some potential complications of ALS?
Respiratory failure, nutritional deficit, aspiration pneumonia
What are poor prognostic factors in ALS?
Bulbar onset, older, decreased forced vital capacity
