Motor Neuron Diseases Flashcards
T/F motor neuron diseases can affect UMN and LMN
True
Motor neuron diseases can be both what?
Inherited or sporadic
Examples of motor neuron diseases
ALS
Post-polio syndrome
Spinal muscular atrophy
Symptoms of ALS
extremity, trunk, and/or bulbar weakness
muscle twitching, spasticity, and/or cramping
dysarthria and/or dysphagia
respiratory compromise
Structures affected in ALS
motor cortex
corticospinal and corticobulbar tracts
brainstem
anterior horn spinal neurons
Etiology of ALS can be both what?
Familial (10%)
Sporadic (90%)
Pathophysiology of ALS
complex interaction of environmental and genetic factors disrupt metabolic pathways
(hard to pinpoint)
Typical onset presentation of ALS
Spinal/Limb onset
Bulbar onset
Spinal/Limb onset
asymmetric, painless weakness of a limb
Bulbar onset
dysarthria, dysphagia, tongue twitching
Negative symptoms of ALS
lack of progression
sensory impairment
visual decline
bowel/bladder dysfunction
imaging, emg r/o
Factors for longer survival times in ALS
younger
spinal/limb onset
UMN predominance
Factors of shorter survival times in ALS
older
bulbar or respiratory onset
onset of FTD and/or behavior changes
LMN predominance
Onset age in PLS
younger
Limbs affected first in PLS
lower extremities
Rate of progression in PLS
slower rate
What function is spared in PLS?
respiratory function
Is PLS fatal?
Not necessarily, average course lasts >20 years
Describe PLS
UMN degeneration with LMN sparing
Describe PMA
LMN degeneration with UMN sparing
Rate of progression of PMA
slightly slower than ALS
Progression of brachial amyotrophic diplegia
Typically proximal to distal
Progression of leg amyotrophic diplegia
Typically asymmetrical, distal to proximal progression
Describe PBA
Degeneration of lower cranial nerves (IX-XII) with subsequent limb involvement
Kennedy’s Disease is also known as
Spinal Bulbar Muscular Atrophy
Symptoms of Kennedy’s disease
Muscular weakness and cramping (proximal > distal)
Dysarthria and/or Dysphagia
Fasciculations
Tremor
Enlarged breasts and/or infertility
Symptoms of post-polio syndrome
Progressive weakness and atrophy
Fatigue (general and musc)
Breathing/Swallowing problems
Cold intolerance
Muscle and joint pain
Treatment considerations in PPS
Joint protection
Energy conservation strategies
Fall prevention
Light to Mod aerobic intensity
Describe SMA
Family of inherited motor neuron disorders
