Motor Neurone Disease Flashcards
what is the most common form of motor neurone disease
amyotrophic lateral sclerosis (ALS)
what is motor neurone disease
a progressive neurodegenerative disorder of cortical, brainstem and spinal motor neurons (lower and upper motor neurons)
what are the types of motor neurone disease
amyotrophic lateral sclerosis
progressive muscular atrophy variant
progressive bulbar palsy
primary lateral sclerosis
what is ALS
combined degeneration of upper AND lower motor neurons, so patients present with a mix of both symptoms
what is progressive muscular atrophy
only LMN symptoms and has a better prognosis than other types of motor neurone disease
what is progressive bulbar palsy
dysarthria, dysphagia, wasted fasciculating tongue, brisk jaw jerk reflex
what is primary lateral sclerosis
UMN patten of weakness, brisk reflexes, no LMN signs
what are some upper motor neurone signs
hyperreflexia, hypertonia, no fasciculations, no atrophy, Babinskis sign present, spastic weakness
what are some lower motor neurone signs
hyporeflexia, hypotonia, fasciculations present, wasting present, absent Babinskis sign, patient will show flaccid weakness
difference between flaccid and spastic weakness
Flaccid paralysis causes your muscles to shrink and become flabby. It results in muscle weakness.
Spastic paralysis involves tight and hard muscles. It can cause your muscles to twitch uncontrollably, or spasm
what type of dementia is motor neurone disease associated with
frontotemporal
some signs and symptoms of motor neurone disease
Weakness of limbs
Speech disturbance (slurring or reduction in volume)
Swallowing disturbance (e.g. choking on food)
Behavioural changes (e.g. disinhibition, emotional lability)
what will you see in a sensory examination
should be normal
investigations for motor neurone disease
bloods, EMG, MRI, spirometry
management of MND
Riluzole (an antiglutamatergic drug which dampens motor nerve firing) has been shown to prolong life by 3 month
