Motor Neuron Disease Flashcards
what type of neurological conditions is MND
a progressive neurodegenerative condition
outline the pathophysiology of MND
umbrella term of 4 diseases with selective dysfunction of motor neurons, either in motor cortex, cranial nerve nuclei or anterior horn cells of spinal cord
what two genes are linked to MND
C9orf72
SOD1
MND is associated with what other neurological condition
fronto-temporal dementia - means that behavioural and personality changes can occur before motor symptoms begin
what is the main primary symptom of MND
muscle weakness
describe the presentation of muscle weakness in MND
focal, usually begins in upper limbs or bulbar and thorax muscles
can also present with laughing/giggling and feeling tearful at odd times - emotional lability
how can MND be distinguished from other neuro conditions
does not affect the eyes - rules out myasthenia
does not have sensory or sphincter dysfunction - MS and neuropathies
which type of MND presents with mixed upper and lower motor neuron deficit
amyotrophic lateral sclerosis
describe the clinical features of amyotrophic lateral sclerosis
weak hand grip weak shoulder abduction stumbling gait foot drop drooling and dysarthria
what is the most common subset of MND
amyotrophic lateral sclerosis
how does progressive bulbar MND present
begins with bulbar muscle weakness with subsequent limb involvement
drooling, dysarthria, dysphagia, aspiration pneumonia
which area of the body does progressive spinal muscle atrophy affect
anterior horn cells so only LMN signs only
presents similarly to ALS minus UMN signs
what is the least common type of MND
primary lateral sclerosis
how does primary lateral sclerosis present
loss of Benz cells in the motor cortex - only UMN signs
marked spasticity and pseudobulbar palsy
how is MND diagnosed
clinical diagnosis, history and examination only but must rule out other conditions
