Overview
Clinial Phenotypes of Motor Neuron Disease
- Amyotrophic lateral sclerosis (ALS) - mixed upper and lower motor neuron involvement
- Primary lateral sclerosis - mainly upper motor neuron involvement
- Progressive muscular atrophy - mainly lower motor neuron involvement
- Progressive bulbar palsy - mainly bulbar involvement
Diagnosis
Signs of Motor Neurone Disease
- Bulbar palsy (LMN) - nasal speech, absent gag reflex, tongue wasting and fasciculations, absent palate rise, absent (i.e. normal) jaw jerk
- Pseudobulbar palsy (UMN) - dysarthria, increased / normal gag reflex, tongue spasticity, absent palate rise, increased jaw jerk
- Upper motor neuron signs - increased tone, clonus, reduced power, hyperreflexia, upgoing plantar reflex
- Lower motor neuron signs - muscle wasting, fasciculations, reduced tone, reduced power, hyporeflexia, downgoing plantar reflex
Management
Management Options for Motor Neuron Disease
Non-Pharmacologic
- Physiotherapy
- Non-invasive ventilation (NIV)
- Speech pathology assessment
- Consideration of PEG feeding
- Psychological sypport
Pharmacologic
- Rulizole
- Anti-spasmodics - baclofen, diazepam, botox
- Neuropathic agents - pregabalin, amitriptyline
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