Amyotrophic lateral Sclerosis- ALS Flashcards
What is ALS
devastating fatal Motor Neuron Disease -UMN
most common
charaterized by degeneration and loss of motor neurons in spinal cord, brain stem and brain
symptoms present from LMN and UMN
pathophysiology
progressive degeneration and loss of motor neurons in spinal cord, brain stem and motor cortex
UMN in cortex are affected and corticospinal tracts
brainstem nuclei and anterior horn cells in spinal cord are affected
does not affect eyes or sensory system
spreads contiguous patter-spreads locally within region and then spreads to other areas
Upper motor neuron dysfunction
lesion in corticospinal and pyramidal tracts in brain or SC
-spasticity and clonus
-hyperreflexia
-pathological reflexes
-bulbar signs
respiratory impairments
-cognitive impairments
Bulbar signs
dysfunction due to medulla oblongata lesion
-dysarthria-speech difficulty
dysphagia-swallow dysfunction
sialorrhea-excessive saliva
pseudobulbar affect-poor emotional control
Lower Motor neuron Dysfunction
lesion distal to ventral or anterior horn of spinal cord
- muscle weakness
- muscle atrophy
- fasciculations
- hyporeflexia
- hypotonicity
- muscle cramps
Respiratory Impairments
early S&S:
- fatigue
- exertional dyspnea
- notctural respiratory difficulty
- recurrent sighing
- excessive daytime sleepiness
- morning headaches from hypoxia
progression:
- truncated speech
- orthopnea
- dyspnea at rest
- accessory muscle use
- weak cough
medical management
RILUZOLE: prolongs survival 2-3 months
ANTI-CONVULSANT: control muscle spasms
ANTICHOLINGEGICS: decrease excessive saliva
DIAZAPM, ZANAFLEX,BACLOFEN: control spasticity
*anxiety and depression management
Dysphagia treatment
SLP
change consistency of diet
educate pt./caregivers
chin tuck during swallowing
- use writting board or pen&pad
- computer with voice synthesizer
- swing-switch, scanning computerized communication
respiratory impairments
pneumo and flu vaccines
prevention of aspiraition
when VC decreases to 50% will need NIV
when NIV doesnt work will need invasive ventilatory
STAGE 1 rehab
mild muslce weakness, pt. is minimally deconditioned and independent with ambulation
- pt. education
- ther ex: AROM of involved, low resistive ROM of non-involved muscles, aerobic activity
- energy conservation
- home and work place modification
- psychological support
STAGE 2 rehab
mod muscle weakness, pt. is mod deconditioned and ind. with ambulation w/ or w/o AD
- ther ex:
- continue AROM but may need to begin AA-PROM to effected muscles
- avoid over fatigue to avoid accelation of muscle weakness
- pace exercises: shorter more frequent ex
STAGE 3 rehab
severe muscle weakness, pt. severely deconditoined and needs assist with all mobility
- continue AAROM with involved muscles
- strengthen non-involved
- continue pace exercise
aerobic activity usually limited to funcitional activitiy
- adaptive equipment
- AFO and cervical support
- power recline chair
- commode and shower chair
- WC
STAGE 4
Severe LE-> WC primary mobility and mild to mod. UE weakness
- AAROM and positioning to prevent contractures
- W/C presscription if not already fitted
- general mobility to decrease:
- skin breakdown
- pressure assessment
- pressure relief
- bed coushin
- W/C coushin
STAGE 5
progressive weakness and deterioration of mobility & endurance–W/C or bed bound
- THER EX and postioning to prevent contracture
- Adaptive Equipment: slide board, lift, semi-rigid cervical orthosis
- paid due to LOM and spasticity/cramps
- *stretching, massage, modalities, orthotics, soft tissue mobs
STAGE 6
essentially bed bound and max/total assist with ADL
- ther ex and positioning to preserve ROM
- adaptive equipment: hospital bed
Progressive respiratory distress
- body-positioning/postural drainage
- self-assisted/manually assisted cough
- airway clearance techniques
