2. General term used to designate a progressive
degenerative disorder of motor neurons in the
spinal cord, brain stem, and motor cortex
Manifestations are muscular weakness,
atrophy and corticospinal tract signs
Disease of middle life
Progresses to death in a matter of 2-5 years or
longer
3. Types
Amyotrophic lateral sclerosis
Amyotrophy and hyperreflexia are combined
Progressive spinal muscular atrophy
Weakness and atrophy occur alone without evidence
of cortico spinal dysfunction
Progressive bulbar palsy
Weakness and wasting predominate in muscles
innervated by motor nuclei of lower brainstem
Primary lateral sclerosis
Clinical state dominated by spastic weakness,
hyperreflxia, and Babinski signs with lower motor
neuron affection becoming apparent only at a later
stage of the illness
4. Amyotrophic lateral
sclerosis
Incidence -0.4 to 1.76 per 10000
Men are affected twice as of women
Starts with a weakness in the distal part of the
arm
Foot drop or awkwardness in tasks requiring
fine finger movements
Cramping
Slight spasticity of the arms and legs with
hyperreflexia without sensory change
5. Babinski anfd Hoffman’s signs are variably
present
Cadaveric or skeletal hand
The muscles of the upper arm and
shoulder girdles are typically involved later
The affected part may ache and feel cold
Sphincteric control is well maintained
Abdominal reflexes may elicitable even
when the plantar reflexes are abnormal
6. Other patterns of
evolution
Leg may be affected before the hands
Early involvement of thoracic, abdominal
or posterior neck muscles
Early diaphragmatic weakness
7. Progressive muscular
atrophy
More common in men than women (4:1)
In about half of the patients, symmetrical
weakness of intrinsic hand muscles, slowly
advancing to more proximal parts of the arms
Fascicular twitching or cramping are invariably
present
Tendon reflexes are diminished or absent and
signs of corticospinal tract disease cannot be
detected
8. Progressive bulbar palsy
Weakness and laxity of muscles innervated by
motor nuclei of lower brain stem (m. of jaw,
face, tongue, pharynx &larynx)
Early defect in articulation
Syllables lose their clarity and run together
Speech sounds as if the patient is eating food
that is too hot
9. Voice is modified by a combination of atrophic
and spastic weakness
Defective modulation with variable degrees of
rasping and nasality
Pharyngeal reflex is lost
Mastication and deglutition also become
impaired
Muscles of the lower face weaken and sag
10. Fasciculations and focal loss of the tissues of
the tongue are early manifestations
The jaw jerk may be present or exaggerated at
a time when the muscles of mastication are
markedly weak
Bulldog reflex- jaws snap shut involuntarily
when attempting to open mouth
11. Course is progressive
Weakness spreads to the respiratory
muscles and deglutition fails entirely
Patient dies within 2-3 years of onset
12. Primary lateral sclerosis
Slowly progressive corticospinal tract
disorder that begins with a pure spastic
paraparesis later and to a lesser degree
the arms and oropharyngeal becomes
involved
Insidious onset in fifth or sixth decade
Stiffness in one leg and then the other
Slowing of gait
13. Spasticity predominating over weakness
as the years go on
No sensory symptoms or signs
Progression for 3 years without evidence
of lower motor neuron dysfunction
Pathologic studies reveal stereo typed
pattern of reduced numbers of Betz cells
in the frontal and prefrontal motor cortex,
degeneration of the cortico spinal tracts
and preservation of motor neurons in the
brain stem and spinal cord
14. Laboratory features of
motor neuron disease
EMG- widespread fibrillations and
fasciculations and enlarged motor units
NCV- slight slowing without focal motor
conduction block
CSF protein is usually normal or marginally
elvated
Serum CK is moderately elevated in patients
with rapidly progressive atrophy and weakness
15. Pathology
Loss of nerve cells in the anterior horn cells of
the spinal cord motor nuclei of the lower brain
stem
Slight gliosis and proliferation of microglial cells
Surviving nerve cells are small, shrunken and
filled with lipofuscin
Swelling of the proximal axons
Disproportionate loss of large myelinated fibers
in motor nerves
16. Corticospinal tract degeneration are most
evident in the lower part of the spinal
cord, but I can be traced up to the
brainstem to the posterior limb of internal
capsule and corona radiata by means of
fat strains
Loss of Betz cells in the motor cortex
17. Differential diagnosis of
ALS
Central spondylotic bar
Ruptured cervical disc
Hemiparesis or monoparesis due to
multiple sclerosis
Chronic motor poly neuropathy
19. No specific treatment for motor neuron
diseases
Supportive measures are important
Early discussions avoid devastating
statements
Rituzole- anti glutamate agent, slows the
progression of the disease and improve
survival with disease of bulbar onset
20. Muscle spasms and pain
Quinine or baclofen
Aching and muscle soreness
Pain medications
21. Dysphagia
Nutritious diets should be followed
Risk of aspiration, sialorrhea
Viscosity of saliva can be treated by
hydration or papaya tablets, tenderizer
Bulbar symptoms- nasogastric or
jejunostomy tubes
22. Respiratory management
Evaluations of respiratory status
Postural drainage with cough facilitation
Breathing exercises, chest stretching
Incentive spirometry
Long term mechanical ventilation
Home mechanical ventilation
Morphine for air hunger
24. Therapeutic management
of impairment and
disabilities
Patients with severe respiratory and bulbar
problems may not get benefitted from active
exercises
Goal in the end stages- optimize health and
improve quality of life
Efficacy is related to timing of interventions
the motivation and persistence of the
patient in carrying out the program and
support from family members
25. Evaluation
Review of patient’s medical activity
record
Discussion of the patient’s lifestyle or
interests, work focus, respiratory status,
psychosocial support issues and patient
concerns and goals
Baseline testing of muscle strength and
ROM
26. Evaluation of functional activity level
Evaluation of pain, identify what makes
the pain worse or better
Evaluation of bulbar and respiratory
function
27. Intervention goals
Maintenance of maximal muscle strength
within limits imposed by ALS
Prevention and minimization of
secondary consequences of the disease
such as contractures, thrombophlebitis,
decubitus ulcers, and respiratory
infections
28. Therapeutic
considerations
Two major factors should be considered
when planning and implementing activity
or exercise program for patients with ALS
Prevention of disuse atrophy
Prevention of overuse injury
29. Exercise prescription
recommendations
To improve compliance consider both a
formal exercise program and enjoyable
physical activities
Include activities with opportunities for
social development and personnel
accomplishment
Strengthening program should
emphasize concentric rather than
eccentric muscle contractions
30. High resistance strengthening programs
probably have no benefit over moderate
resistance programs
Muscles with less than antigravity
strength have little capacity to improve;
the program should focus on stronger
muscles
31. Periodically monitor muscles to assess
for possible overwork weakness,
particularly in unsupervised programs
Activity modifications should include
periods of physical activity with rest
32. Therapeutic interventions
Before finalizing the intervention plan
based on patient goals, the therapist
must consider the following
1) Typical rate of patient progression
2) Distribution of weakness and spasticity,
respiratory factors leading to hypoxemia,
and easy fatigability and bulbar
involvement
3) Phase of the disease
34. Phase I (independent)
stage 1
Patient characteristics
Mild weakness
Clumsiness
Ambulatory
Independent in ADL
35. Treatment
continue normal activities or increase
activities
Begin program of ROM exercises
Add strengthening program of gentle
resistance exercises with caution
Provide psychological support as needed
37. Treatment
Continue stretching to avoid contractures
Continue cautious strengthening of
muscles with grade above 5
Consider orthotic support
Use adaptive equipment to facilitate ADL
38. Stage 3
Patient characteristics
Severe selective weakness in ankles,
wrists, and hands
Moderately decreased independence in
ADL
Easy fatigability with long distance
ambulation
Ambulatory
Slightly increased respiratory effort
39. Treatment
Continue stage 2 program as tolerated
Keep patient physically as long as
possible through pleasurable activities
Encourage deep breathing exercise,
chest stretching, postural drainage if
needed
Prescribe wheel chair
40. Phase II(partially
independent)
Stage 4
Patient characteristics
Hanging arm syndrome with shoulder
pain and sometimes edema in the hand
Wheel chair dependent
Sever lower extremity weakness
Able to perform ADL but fatigues easily
41. Treatment
Heat, massage, preventive anti edema
measures
Active assisted ROM exercise to weakly
supported joints
Encourage isometric contractions
Try arm slings, overhead slings
Motorized chair if want to be independent
42. Stage 5
Patient characteristics
Severe lower extremity weakness
Moderate to severe upper extremity
weakness
Wheel chair dependent
Increasingly dependent on ADL
Possible skin break down secondary to
poor mobility
43. Treatment
Encourage family to learn proper transfer
positioning principles, and turning
techniques
Encourage modifications at home to aid
patients mobility and independence
Electric hospital bed with anti pressure
mattress
If there is HMV adapt chair to hold it
45. Treatment
For dysphagia soft diet, long spoons,
tube feeding, percutaneous gastrstomy
To decrease flow of accumulated saliva:
medication, suction, surgery
For dysarthria: palatal lifts, electronic
speech amplification, eye pointing
electronic
For breathing difficulty: clear airway,
tracheostomy, respirator, medications to
decrease the impact of dyspnea
