Motor Neurone Disease Flashcards
What is motor neurone disease?
progressive deterioration of lower and upper motor neurones
in the spinal cord, in cranial nerve motor nuclei and within cortex
What rules out the diagnosis of MND?
any sensory signs
Who usually gets MND?
slight male predominance
usually over 50
What are the 4 subtypes of MND?
1 - ALS (amyotrophic lateral sclerosis)
2 - progressive bulbar palsy
3 - progressive muscle atrophy
4 - primary lateral sclerosis
What is ALS?
loss of motor neurones in motor cortex and anterior horn of corn (UMN and LMN signs and symptoms)
What is progressive bulbar palsy?
affects cranial nerves IX-XII
tongue and muscles of talking and swallowing
What is progressive muscular atrophy?
anterior horn lesion only (no UMN symptoms)
affects distal muscle groups before proximal
What is primary lateral sclerosis?
loss of Betz cells in motor cortex (mainly UMN signs)
marked leg weakness and pseudo bulbar palsy
What is the most common MND?
ALS
What is the presentation of ALS?
UMN and LMN signs and symptoms
- weakness and muscle wasting
- fasciculations
- stiffness with poor coordination and balance
- painful muscle spasms
- spastic, unsteady gait
- foot drop
What is the split hand sign?
in ALS
thenar muscles disproportionately wasted compared to hypothenar
What gives a worse prognosis in ALS?
bulbar onset
increased age
What are the key diagnostic factors for ALS?
risk factors
upper extremity weakness
stiffness, poor coordination and balance
spastic, unsteady gait
What are the risk factors for ALS?
genetic predisposition or family history
age >40
What is the average survival time for MND?
3 years
