motor neurone disease Flashcards
what is motor neurone disease? what doesn’t it affect?
relentless and unexplained destruction of UMN and anterior horn cells in the brain and spinal cord
no involvement of the sensory system or motor nerves to the eyes
cause of death in motor neurone disease
die within 3 yrs from resp failure resulting from bulbar palsy & pneumonia
what is bulbar palsy
impairment of function of the CN 9-12 which occurs due to LMN lesion in the medulla or from lesions lower in the CNS outside the brainstem
who gets motor neurone disease and when
M > F
middle age
what are the 4 clinical patterns in motor neurone disease
1 - progressive muscular atrophy
2 - amyotrophic lateral sclerosis
3 - progressive bulbar & pseudobulbar palsy
4 - primary lateral sclerosis - rare
progressive muscular atrophy in motor neurone disease
LMN causing weakness, wasting and fasciculation in hands and feet
amyotrophic lateral sclerosis in motor neurone disease
disease of the lateral corticospinal tracts and anterior horn cells producing a progressive spastic tetraparesis or parapesis with added LMN signs (wasting & fasciculation)
progressive bulbar & pseudobulbar palsy in motor neurone disease
results from destruction of upper and lower motor neurones in the lower CNs.
There is dysarthria (unclear speech), dysphagia with wasting and fasciculation of the tongue
primary lateral sclerosis in motor neurone disease
rare
progressive tetraparesis
investigation MND
diagnosis clinical
fasciculation is characteristic
EMG shows muscle denervation
diff diag MND
cervical spine lesion, but will have sensory signs too
management MND
riluzone - sodium channel blocker that inhibits glutamate release, slows progression slightly
ventilatory support & feeding via a PEG helps prolong survival for some months
