Lecture 3: Amyotrophic Lateral Sclerosis Flashcards
What is another name for ALS?
Lou Gehrig’s disease
What is usual age, gender and race of ALS pts?
mid 50’s, mostly white (93%), 60% male
What is pathogenesis of ALS?
progressive degenerative motor neuron disease affects both UMN and LMN?
How does ALS affect LMN?
loss of anterior horn cells and CN motor nuclei by degeneration of axons leads to denervated muscles and atrophy (amyotrophic)
How does ALS affect UMN?
degeneration of Betz cells in motor cortex with demylelination and gliosis of corticospinal tract (lateral sclerosis) and corticobulbar tract
What is the process of gliosis?
is a nonspecific reactive change of glial cells in response to damage to the central nervous system (CNS). In most cases, gliosis involves the proliferation or hypertrophy of several different types of glial cells
glial cells form myelin
What areas of CNS are typically spared or less affected?
CN 3, 4 and 6 plus sensory function and spinecerebellar tracts
What are physiological changes in CNS from ALS?
mitochondrial dysfunction, inflammation and apoptosis
What is prognosis for ALS?
variable depending on bulbar onset or limb onset, slower progression with young age and bulbar onset however death usually 3-5 years after diagnosis
What is the etiology of ALS?
not well understood potentially triggered by genentic disposition and/or environmental stimulus such as viral infections, toxins or autoimmune reaction
What is medical diagnosis of ALS?
nothing definitive but can use MRI, SPECT, EMG, CSF
will show atrophy of precentral and postcentral gyrus, extra motor activation during limb activity
What is medical management of ALS?
meds to help pain, spasticity, anxiety, respiratory care(BiPAP), nutritional support
What is clinical presentation of ALS?
isolated weakness, cramping and fasiculations, vague sensory changes, possible cognitive impairments (dementia), possible irregularities in ANS ( BP, HR, thermoreuglation)
What is difference in presentation between limb onset and bulbar onset?
limb- 70% of cases, asymmetrical weakness of hand and foot
bulbar- 30% of cases, difficulties with speech or swallowing
What percentage of motor neurons are lost by the time patient c/o of weakness?
80%
What are primary LMN sx of ALS?
weakness, hyporeflexia, hypotone, atrophy
What are primary UMN sx of ALS
spasticity, babinski sign, hyperreflex, impaired motor control
What are secondary signs and symptoms?
fatigue, weight loss, loss of ROM, balance loss, pressure ulcers, contractures
What are clinical sx of bulbar onset ALS?
dysarthria, disphagia, sialorrhea, DOE, nocturnal difficulty
What is stage 1 of ALS?
indept mobility, and ADL’s weakness in specific muscle groups
What is stage 2 of ALS?
early, moderate weakness in muscle groups minor difficulty in with mobility, fine motor skills or ADL’s
What is stage 3 of ALS?
mild to moderate impairments, ambulatory with orthotics
What is stage 4 of ALS?
severe muscle weakness, w/c use may be moderately indpt with AdL’s
What is stage 5 of ALS?
w/c dept for all mobility pain and pressure ulcers,
What is stage 6 of ALS?
repspiratory complications begin
During early stage of ALS what are some restorative/preventative PT interventions?
strength- maintain maximal muscle strength, prevent disuse atrophy, avoid overwork damage
AROM, stretching, AC and endurance
During early stage of ALS what are some compensatory PT interventions?
adaptive equipment, home/work modifications, energy conservation
During middle stage of ALS what are some restorative/preventative PT interventions?
AROM to PROM, stretching, endurance, pressure relief/skin protection
During middle stage of ALS what are some compensatory PT interventions?
adaptive equipment, splints, ortohotics, caregiver training
During late stage of ALS what are some restorative/preventative PT interventions?
PROM, pulmonary care/hygiene, pressure relief and skin care
During late stage of ALS what are some compensatory PT interventions?
mechanical lift, ventilator support, caregiver education and training, hospice care
What are disease specific standardized test and measures for ALS?
ALS functional rating scale, severity scale, Q-40
can also use FIM, sickness impact profile and fatigue severity scale
