Amyotrophic Lateral Sclerosis
A.K.A Lou Gehrig’s Disease
Bobbette Miller DPT, NCS
Objectives
Describe the incidence and prevalence of ALS
Describe the pathophysiology of ALS
Describe the clinical manifestations of ALS
Describe the course of the disease
ALS Pathophysiology
Amyotrophy – atrophy of the muscle fibers as their
corresponding anterior horn cells degenerate
Lateral sclerosis – hardening of the anterior and lateral
columns of the spinal cord as the motor neurons
degenerate and are replaced by fibrous astrocytes
(gliosis)
Epidemiology of ALS
Approx. 5,000 new cases per year
One – two per 100,000
Approx. 25,000 - 30,000 people living with ALS at any given
time
6 per 100,00
5% - 10% of cases are genetic/familial ALS
Affects all races and ethnic groups
Men more than women – 1.7 to 1
Age of onset 55-75; peak age is 62
More cases in the Western Pacific and Guam
ALS Pathophysiology
Irreversible, progressive disorder of the nervous system,
which we do not understand well.
Degeneration of both UMN & LMN
Neurons in the motor cortex (UMN) and brainstem and
anterior horn cells in the spinal cord (LMN) stop relaying
messages to the muscles - weakness, atrophy, and
fasciculations
Loss of all voluntary movement, then death.
The chest and diaphragm muscles become involved and the
person is no longer able to breathe.
Respiratory failure is the leading cause of death
Video
https://www.als.net/what-is-als/?gclid=Cj0KCQjwnpXmBRDUARIsAEo71tSAk2RaBBMqF8scAQ4RRku
n-GGscCVQ36mJtNmW_e3F8wHuI1pw75QaAtjZEALw_wcB
Proposed ALS classification system
This is a proposed
classification system for
ALS. It has not been
adopted by medical
community. It is for your
reference only.
Disease Course
Onset to ventilator dependence or death is 2-4 years
After the ventilator mean life expectancy is approx. 5 years, but can
exceed 10 years
Cognition, occulomotor function, sensation, and bowel & bladder function
remain normal
Skin integrity concerns due to lack of mobility.
Research is focused on finding the source of neuron degeneration and
stopping it
Clinical Manifestations:
Vary from patient to patient
LMN UMN
Asymmetrical weakness + Babinski
Cramping with voluntary + Hoffman’s sign
movement in the early Hyperreflexivity
morning
Fasciculations
Extensor muscles become
weaker than flexor
muscles; hand
Pharmacological Management
Cost $407 a tablet.
Symptoms Disease modifying
Baclofen for spasticity or Rilusole/Rilutek – has
anticonvulsants such as been clinically shown to
Gabapentin or Neurotin increase time by 3-5
NSAIDs for pain or months until
Benzodiazepines for tracheostomy or death
severe fasciculations But no improvement of
muscle strength or
neurological function
Indicators for Therapy
Fatigue
Spasticity
Functional losses are noted when isometric strength declines
Progressive weakness of scapulohumeral musculature and
subluxation
Contractures
Unable to shift weight for pressure relief
Loss of diaphragm
Teach use abdominals to help with inspiration
Most important!
Understand the wants and needs of the person
Anticipate the progressive nature of this disease
Problem solve with them to determine how you can best assist
them; including making recommendations for AT
Understand and appreciate the impact of the environment to
help them remain as functional for as long as possible
Work with caregiver and other providers
Steve Gleason
References
Lewis, M. & Rushanan. S. (2007). The role of physical therapy and occupational therapy in the
treatment of amyotrophic lateral sclerosis. NeuroRehabilitation, 22 (6), 451-61.
University of Pennsylvania MD-ALS Center Occupational and Physical Therapy
http://www.alsclinic.pitt.edu/patients/pt_ot_therapy.php
Bello-Haas VD. Physical therapy for individuals with amyotrophic lateral sclerosis: current
insights. Degener Neurol Neuromuscul Dis. 2018;8:45-54. Published 2018 Jul 16.
doi:10.2147/DNND.S14694
