Lecture 3: Amyotrophic Lateral Sclerosis

Question 1

What is another name for ALS?

Answer 1

Lou Gehrig’s disease

Question 2

What is usual age, gender and race of ALS pts?

Answer 2

mid 50’s, mostly white (93%), 60% male

Question 3

What is pathogenesis of ALS?

Answer 3

progressive degenerative motor neuron disease affects both UMN and LMN?

Question 4

How does ALS affect LMN?

Answer 4

loss of anterior horn cells and CN motor nuclei by degeneration of axons leads to denervated muscles and atrophy (amyotrophic)

Question 5

How does ALS affect UMN?

Answer 5

degeneration of Betz cells in motor cortex with demylelination and gliosis of corticospinal tract (lateral sclerosis) and corticobulbar tract

Question 6

What is the process of gliosis?

Answer 6

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

Question 7

What areas of CNS are typically spared or less affected?

Answer 7

CN 3, 4 and 6 plus sensory function and spinecerebellar tracts

Question 8

What are physiological changes in CNS from ALS?

Answer 8

mitochondrial dysfunction, inflammation and apoptosis

Question 9

What is prognosis for ALS?

Answer 9

variable depending on bulbar onset or limb onset, slower progression with young age and bulbar onset however death usually 3-5 years after diagnosis

Question 10

What is the etiology of ALS?

Answer 10

not well understood potentially triggered by genentic disposition and/or environmental stimulus such as viral infections, toxins or autoimmune reaction

Question 11

What is medical diagnosis of ALS?

Answer 11

nothing definitive but can use MRI, SPECT, EMG, CSF

will show atrophy of precentral and postcentral gyrus, extra motor activation during limb activity

Question 12

What is medical management of ALS?

Answer 12

meds to help pain, spasticity, anxiety, respiratory care(BiPAP), nutritional support

Question 13

What is clinical presentation of ALS?

Answer 13

isolated weakness, cramping and fasiculations, vague sensory changes, possible cognitive impairments (dementia), possible irregularities in ANS ( BP, HR, thermoreuglation)

Question 14

What is difference in presentation between limb onset and bulbar onset?

Answer 14

limb- 70% of cases, asymmetrical weakness of hand and foot

bulbar- 30% of cases, difficulties with speech or swallowing

Question 15

What percentage of motor neurons are lost by the time patient c/o of weakness?

Answer 15

80%

Question 16

What are primary LMN sx of ALS?

Answer 16

weakness, hyporeflexia, hypotone, atrophy

Question 17

What are primary UMN sx of ALS

Answer 17

spasticity, babinski sign, hyperreflex, impaired motor control

Question 18

What are secondary signs and symptoms?

Answer 18

fatigue, weight loss, loss of ROM, balance loss, pressure ulcers, contractures

Question 19

What are clinical sx of bulbar onset ALS?

Answer 19

dysarthria, disphagia, sialorrhea, DOE, nocturnal difficulty

Question 20

What is stage 1 of ALS?

Answer 20

indept mobility, and ADL’s weakness in specific muscle groups

Question 21

What is stage 2 of ALS?

Answer 21

early, moderate weakness in muscle groups minor difficulty in with mobility, fine motor skills or ADL’s

Question 22

What is stage 3 of ALS?

Answer 22

mild to moderate impairments, ambulatory with orthotics

Question 23

What is stage 4 of ALS?

Answer 23

severe muscle weakness, w/c use may be moderately indpt with AdL’s

Question 24

What is stage 5 of ALS?

Answer 24

w/c dept for all mobility pain and pressure ulcers,

Question 25

What is stage 6 of ALS?

Answer 25

repspiratory complications begin

Question 26

During early stage of ALS what are some restorative/preventative PT interventions?

Answer 26

strength- maintain maximal muscle strength, prevent disuse atrophy, avoid overwork damage

AROM, stretching, AC and endurance

Question 27

During early stage of ALS what are some compensatory PT interventions?

Answer 27

adaptive equipment, home/work modifications, energy conservation

Question 28

During middle stage of ALS what are some restorative/preventative PT interventions?

Answer 28

AROM to PROM, stretching, endurance, pressure relief/skin protection

Question 29

During middle stage of ALS what are some compensatory PT interventions?

Answer 29

adaptive equipment, splints, ortohotics, caregiver training

Question 30

During late stage of ALS what are some restorative/preventative PT interventions?

Answer 30

PROM, pulmonary care/hygiene, pressure relief and skin care

Question 31

During late stage of ALS what are some compensatory PT interventions?

Answer 31

mechanical lift, ventilator support, caregiver education and training, hospice care

Question 32

What are disease specific standardized test and measures for ALS?

Answer 32

ALS functional rating scale, severity scale, Q-40

can also use FIM, sickness impact profile and fatigue severity scale