Degenerative Motor Neuron Diseases Flashcards
characterized by
weakness and wasting of muscles without sensory changes
Examples
5
- bulbar palsy
- pseudobulbar palsy
- progressive spinal muscular atrophy
- primary lateral sclerosis
- amyotrophic lateral sclerosis
common sx of all disorders
6
- dysphagia
- problems w/ chewing
- problems w/ coughing
- dyspnea
- trouble w/ talking
- limb weakness
general tx options
- edaravone (60mg infusion for 10-14d monthly)
- slows disease progression in mild cases
other general tx options
5
- botox injections
- lots of PT/OT
- suction machines
- feeding tube
- tracheostomy
Bulbar Palsy
primarily affects what?
motor nuclei of cranial nerves
Bulbar Palsy
hallmark sx
2
- drooping of palate
- weak tongue
Bulbar Palsy
worry with this?
all the sx combined can lead to…..
depressed gag reflex + weak cough + pooling of saliva in pharynx = aspiration
Pseudobulbar Palsy
dysfunction with what
upper motor neurons
Pseudobulbar Palsy
hallmark sx
4
- uncontrolled episodes of laughing or crying without cause
- tongue is contracted + spastic, difficult to move
- stiff and weak limbs, muscle wasting, fasciculations
- sphincters are spared
differentiate bulbar palsy and pseudobulbar palsy
- Gag reflex: bulbar absent, pseudo normal
- jaw jerk: bulbar absent, psuedo normal
- tongue: bulbar wasted w/ fasciculation, pseudo spastic w/out fasciculation
- emotional: bulbar emotional liability, pseudo no emotional liability
- speech: bulbar nasal, pseudo hot potato?
Progressive Spinal Muscular Atrophy
describe
lower motor neuron deficit in limbs due to degeneration of anterior horn cells of spinal cord
Primary Lateral Sclerosis
describe
- upper motor neuron deficits only in the limbs
- typically have longer life but profound quadriparesis and spasticity
Amyotrophic Lateral Sclerosis
describe location of deficits
- mixed upper and lower motor neuron deficit but no sensory deficits
- affects frontal motor neurons w/ UMN degeneration and LMN degeneration
Amyotrophic Lateral Sclerosis
sx of UMN degeneration
3
- weakness
- hyperreflexia
- spasticity
Amyotrophic Lateral Sclerosis
sx of LMN degeneration
3
- weakness
- atrophy
- fasciculation
Amyotrophic Lateral Sclerosis
describe onset
gradual, over a period of time over several months w/ progressive worsening
Amyotrophic Lateral Sclerosis
what is required for official dx
changes in all 3 spinal regions
Amyotrophic Lateral Sclerosis
PE Findings
9 components
- clumsiness, gait abnormalities, limb weakness, wrist/foot drop, poor fine motor skills
- muscle fasciculations
- voice changes
- involuntary laughing/crying
- drooling
- dyspnea on exertion
- cognitive changes
- sesnation in tact
- hyperreflexia + pronator drift
Amyotrophic Lateral Sclerosis
dx
3 components
- EMG to r/o other causes
- labs to r/o other causes
- no specific test to dx ALS, dx based on H&P
Amyotrophic Lateral Sclerosis
tx
- focused on survival and symptom management (respiratory function)
- use orthotic and assistive devices to keep pt mobile
- physical, occupational, speech therapies
Amyotrophic Lateral Sclerosis
meds which can help slow progression
3 components
- riluzole (50mg PO QD)
- edaravone (60mg IV QD for 2 wks on and off; then 10 days on, 2 wks off)
- muscle relaxants and botox injections for spasticity
Amyotrophic Lateral Sclerosis
usually fatal within?
3-5 yrs because of pulm infections
Amyotrophic Lateral Sclerosis
survival rate 5-10 yrs post onset
20%
