AMYOTROPHIC

LATERAL SCLEROSIS
 Motor neuron diseases (MND)
 Lou Gehrig’s disease
 Degeneration and loss of motor neurons
in the spinal cord, brain stem and brain,
resulting in a variety of UMN and LMN
clinical signs and symptoms.
 Incidence; range of 0.4 to 2.4 cases per
100,000, with the incidence
 Prevalence; 4 to 10 cases per 100,000
 The average age at onset is the mid-to-
late 50s
 Affects men slightly more than women
 In about 5% to 10% of individuals the
disease is inherited as an autosomal
dominant trait
 70% to 80% of individuals develop limb-
onset ALS
 20% to 30 % develop bulbar-onset ALS
 Bulbar-onset ALS is more common in
middle-aged women, and initial
symptoms may include difficulty
speaking, chewing, or swallowing
Motor neuron disorders
Subtype Nervous system pathology
Amyotrophic lateral Degeneration of the corticospinal
sclerosis tracts, neurons in the motor cortex
and brainstem and anterior horn cells
in the spinal cord.
Primary lateral Degeneration of upper motor
sclerosis neurons.
Progressive bulbar Degeneration of motor neurons of
palsy cranial nerves IX to XII.
Progressive muscular Loss of chromatolysis of motor
atrophy neurons of the spinal cord and
brainstem.
Etiology
 Superoxide dismutases (SOD)
 Glutamate
 Clumping of neurofilament proteins into spheroids in
the cell body and proximal axon is one of the
histopathological characteristics of ALS.
 Autoimmune reactions (eg. Calcium channels
blockage)
 Lack of neurotrophic factors could contribute to the
development of ALS and other neurodegenerative
disorders
 Exogenous or environmental factors apoptosis and
viral infections
Pathophysiology
 Progressive degeneration and loss of motor neurons
in the spinal cord, brainstem, and motor cortex
 UMNs in the cortex are affected, as are the
corticospinal tracts.
 Brainstem nuclei for cranial nerves V (trigeminal), VII
(facial), IX (glossopharyngeal), X (vagus), and XII
(hypoglossal) and anterior horn cells in the spinal
cord are also involved.
 Brainstem nuclei for cranial nerves controlling
external ocular muscles (III: oculomotor, IV:
trochlear, and VI: abducens) are usually spared
 Motor neurons of the Onufrowicz
nucleus (Onuf’s nucleus), located in the
ventral margin of the anterior horn in the
second sacral spinal level, are also
generally spared
 These neurons control striated muscles
in the pelvic floor, including anal and
external urethral sphincters
 The sensory system and spinocerebellar
tracts are also generally spared in ALS.
 Re-innervation and ultimate failure of re-
innervation
Clinical Manifestations
 Clinical manifestations of ALS vary depending
on the localization and extent of motor neuron
loss, the degree and combination of LMN and
UMN loss, pattern of onset and progression,
body region(s) affected, and stage of the
disease.
 At onset, signs or symptoms are usually
asymmetrical and focal.
 Progression of the disease leads to increasing
numbers and severity of impairments.
Diagnosis
 Genetic test
 Clinical presentation
 Laboratory studies
 EMG
 Nerve conduction velocity (NCV) studies
 Muscle and nerve biopsies
 Neuroimaging studies
Diagnosis criteria
 The diagnosis of ALS requires the presence of
1. LMN signs by clinical, electrophysiological or
neuropathological examination
2. UMN signs by clinical examination and
3. Progression of the disease within a region or two other
regions by clinical examinations of via the medical history,
 And the absence of
1. Electrophysiological and pathological evidences of other
diseases that may explain the UMN and LMN signs, and
2. Neuroimaging evidence of other disease processes that may
explain the observed clinical and electrophysiological signs
Disease course
 ALS has a progressive and deteriorating disease
trajectory, and the progression from pathology to
impairments to functional limitations to
disabilities is inevitable.
 Time from onset to death ranging from several
months to 20 years, studies have found the
average duration of ALS to be between 27 and
43 months, and the median duration to be
between 23 and 52 months.
 Death occurs within 3 to 5 years after diagnosis
and usually results from respiratory failure
PROGNOSIS
 Age at time of onset
 Individuals with limbonset ALS have a
better prognosis than those with
bulbaronset ALS
 Less severe involvement at the time of
diagnosis
 No symptoms of dyspnea at onset
Psychological status
Management
 Disease modifying agents
Glutamate inhibitor; Riluzole (Rilutec)
 Palliative care is”.. the active total care of patients
whose disease is not responsive to curative
treatments.
 Although there is no cure for ALS, it is still considered
a “treatable disease” and rehabilitation plays a
integral role in the overall comprehensive care of the
patient.
 Medical management is symptomatic and
individualized and involves supportive care to
address impairments as they arise.
 Medical management may include the
prescription of anticramping and antispasticity
agents, drying agents for sialorrhea, and
antidepressants; recommendations and
referrals for precutaneous endoscopic
gastrotomy (PEG) tubes and ventilator support
(noninvasive ventilation, tracheostomy); and
discussion of advanced care directives
 Management of dysphagia, dysarthria, anxiety,
depression
 Pain control
In 1999 a multidisciplinary task force was established to
develop recommendations for management of ALS.

1. Informing the patients and family about the

diagnosis and prognosis.
2. Symptom management of sialorrhea and
pseudobulblar affect
3. Nutrition management and PEG decisions (e.g.
dysphagia)
4. Management of respiratory insufficiency and
ventilation decisions (infections, aspiration, low
oxygen, hypercarbia, respiratory arrest)
5. Advance directives and palliative care
Rehabilitation Framework
Physical therapy
examination
 Psychosocial function
 Pain
 Muscle performance
 Motor function
 Tone and reflexes
 Cranial nerve integrity
 Sensation
 Postural alignment, control and balance
 Gait
 Respiratory function
 Integument
 Functional status
 Environmental barriers
 Fatigue
Disease specific and quality of life
measures
 Disease specific measures;
ALS Functional Rating scale (ALSFRS)
The revised version
Appel ALS Scale (AALS)
ALS Severity Scale (ALSSS)
Norris Scale.
 Quality of life measures
SF-36
The Schedule for Evaluation of Individual Quality
Life-Direct Weighing (SEIQoL-DW)
Sickness Impact Profile (SIP).
Physical Therapy Interventions
 Cervical muscle weakness
 Dyartheria and dysphagia
 Upper extremity weakness
 Shoulder pain
 Respiratory muscle weakness
 Lower extremity muscle weakness
 Gait training
 Activities of daily living
 Decreased mobility
 Muscle cramps and spasticity
 Psychosocial issues
Exercise and ALS
 Disuse atrophy
 Overuse fatigue
Physical Therapy Interventions
 Restorative
 Compensatory
 Preventory
 Early stage
Stages Common impairments and Interventions
functional
limitations
Early Mild to moderate Restorative/prevention
weakness in specific  Strengthening exercises a142,169,170
muscle group  Endurance exercises171
Difficulty with ADLs and  Active ROM, 172 Active –assisted POM,
mobility toward the end of stretching exercise
the stage Compensatory
 Determine potential need for aadaptive of
assistive devices
 Determine potential need for ergonomic
modification of home/workplace
 Energy conversation
 Educate the patients about the disease
process, energy conservation , and support
groups
Middle stage
stage Common impairments and Interventions
functional limitations
Middle Progressive decrease in Compensatory
mobility throughout stages  Support weak muscles(assistive and
Wheelchair needed for long supportive devices, adaptive equipment,
distances; slings, orthoses)
Increased wheelchair use  Modification to work place /home (e.g,
toward end of stage install ramp, move bedroom to first floor)
Serve muscle weakness in  Wheel vchair prescription
some groups; mild to  Education of caregiver regarding functional
moderate weakness in other training
groups Preventative
Progressive decrease in ADKL  Active 172 active assistive , and passive
skills throughout stage ROM, stretching exercise
Plan  Stretching exercise 142,169,1709early
  middle)
 Endurance ecercise 171(early middle)
 Determine need for pressure-relieving
devices(e.g., pressure distributing
matteress)
 Late stage
Stage Common Impairments and Interventions
functional limitations

Late Wheel chair dependent or Preventative

restricted to bed  Passive ROM
Complete dependence with  Pulmonary care a
ADLs  Hyospital bed and
Severe weakness of UE, pressure-relieving
LE, neck and trunk muscles devices
Dysarthria, dysphagia  Skin care, hygiene
Respiratory compromise Compensatory
pain  Caregiver education
  regarding transfers,
positioning,
 Mechanical
 Type of Semi rigid and Rigid Cervical Collars
  Examples Advantages Disadvantage
Collars Without PHILADPHIA® Collar Offers good Support Patient may feel confined may
Anterior neck access (A) cause pressure on trachea
patient may experience difficulty
breathing or swallowing can be
uncomfortably warm
 

Collars with anterior neck access(for tracheotomy) Miami-j® Collar(B) Padding absorbs and Patient may feel confined may
Aspen Collar© wicks moisture away be uncomfortably warm more
Malibu Collar(D) from skin suitable for expensive
  individuals with  
  cervical weakness in  
  all 3 planes May put pressure on chin and
Canadian collar(E)   sternum
Headmaster Collar(E) Open design allow for Some models more expensive
circulation of air light Some models require custom
weight cutting
No pressure on Not adequate if rotation and
trachea some lateral flexion weakness is also
patients consider present
collar more  
cosmetically
appealing
Common Types of Adaptive
Equipment
Feeding and Eating Foam tubing to increase the size of utensil handles; utensil and cup with
modified handles or holders; long –levered jar opener; plate guard;
serrated or rocker knife; wrist splint/adapted cuff(for holding tools and
instruments); mobile arm support; Dycm®

Self-care and Bathing Bathing benches; bath tube seat; shower commode; handle shower head;
grab bars; raised toilet seat; long handle sponge; electric toothbrush or
shaver; strap- fitted hair brushes

Dressing Zipper pulls or hooks; button hooks; long handle shoe horn; Velcro®
clothing closures; elastic shoe laces

Writing and Reading Foam tubing to increase the size of the pen or pencil ; Triangular pencil
grip: pen holders; books holders, automatic page turner; adjustable angle
table
Other Key holder; doorknob adapters; lamp extension switch; Personal alarm
system; switch operated environmental controls; speaker phone with
automatic dialing, telephone holder; use of telecommunication devices for
the deaf(TDD)
Summary
 Amyotrophic lateral sclerosis, the most common and devastatingly
fatal motor neuron disease among adults, causes a progressive
increase in the number and severity of impairments, activity
limitations, and participation restrictions.
 Other than a small percentage of cases, etiology for the most part
is unknown, and it is hypothesized that multiple mechanisms may
be responsible for the disease.
 Although there is no cure for ALS and its course cannot be altered,
it should be considered a “treatable disease.”
 Medical management is primarily symptomatic, and a team
approach to care is considered optimal.
 Rehabilitation management is focused on maximizing function and
promoting independence to the highest level possible, and
ensuring optimal quality of life throughout the course of the disease
and across health care settings.
 The physical therapist plays an integral role in designing and
implementing therapeutic interventions for individuals with ALS that
will allow them to maintain independence and function for as long
as possible.
 The selection of interventions, grounded in evidence-based
research whenever possible, is based on the stage and
progression of the disease and may be restorative, compensatory,
or preventative.
 These interventions should take into consideration the individual’s
goals and psychosocial factors that may affect decision making,
such as the individual’s acceptance of the diagnosis and the
individual’s social and financial resources.
 Because of the progressive nature of the ALS, the physical
therapist must not only address the patient’s current problems, but
also plan for the patient’s future needs.
Thanks!