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afya MotorNeuroneDisease

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20160425222440 Ben  
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''features''

unknown cause 
presents with upper and lower motor neuron signs
rare before 40yr
various patterns -  amyotrophic lateral sclerosis, progressive muscular atrophy and bulbar palsy

''clues''
fasciculation (always think MND)
absence of sensory signs/symptoms (may have vague symptoms early on but no signs)
LMN arms, UMN legs 
wasting of the small hand muscles/tibialis anterior is common

Note
no ocular muscle signs
no cerebellar signs
abdominal reflexes usually preserved and sphincter dysfunction if present is a late feature

Diagnosis is clinical
nerve conduction studies help exclude neuropathy
Electromyography - reduced number action potentials with inc amplitude 
MRI done to exclude cervical cord compression and myelopathy