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  • Amyotrophic Lateral Sclerosis A.K.A Lou Gehrig's Disease: Bobbette Miller DPT, NCS

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    This document provides information on amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease. It describes the pathophysiology and epidemiology of ALS, noting that it is a progressive neurodegenerative disease that affects motor neurons in the brain and spinal cord, leading to muscle weakness. The document outlines the clinical manifestations of ALS and discusses its typical disease course, management approaches, and indicators for physical and occupational therapy to help maintain function and mobility.

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    ALS

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    This document provides information on amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease. It describes the pathophysiology and epidemiology of ALS, noting that it is a progressive neurodegenerative disease that affects motor neurons in the brain and spinal cord, leading to muscle weakness. The document outlines the clinical manifestations of ALS and discusses its typical disease course, management approaches, and indicators for physical and occupational therapy to help maintain function and mobility.

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    45 views14 pages

    Amyotrophic Lateral Sclerosis A.K.A Lou Gehrig's Disease: Bobbette Miller DPT, NCS

    Uploaded by

    John Smith
    This document provides information on amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease. It describes the pathophysiology and epidemiology of ALS, noting that it is a progressive neurodegenerative disease that affects motor neurons in the brain and spinal cord, leading to muscle weakness. The document outlines the clinical manifestations of ALS and discusses its typical disease course, management approaches, and indicators for physical and occupational therapy to help maintain function and mobility.

    Copyright:

    © All Rights Reserved
    Download as PPTX, PDF, TXT or read online from Scribd
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    of 14

    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

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