MOTOR NEURON DISEASES.ppt .neurology subject

Motor neuron medical subject neurology department

1. MOTOR NEURON DISEASESMOTOR NEURON DISEASES
(MOTOR SYSTEM DISEASES)(MOTOR SYSTEM DISEASES)
Dr. Nawaj Pathan
Dept of Neurophysiotherapy
MGM Institute of Physiotherapy
Chh. Sambhajinagar

2. General term used to designate a progressive General term used to designate a progressive
degenerative disorder of motor neurons in the degenerative disorder of motor neurons in the
spinal cord, brain stem, and motor cortexspinal cord, brain stem, and motor cortex
Manifestations are muscular weakness, Manifestations are muscular weakness,
atrophy and corticospinal tract signsatrophy and corticospinal tract signs
Disease of middle lifeDisease of middle life
Progresses to death in a matter of 2-5 years or Progresses to death in a matter of 2-5 years or
longerlonger

3. TypesTypes
Amyotrophic lateral sclerosisAmyotrophic lateral sclerosis
Amyotrophy and hyperreflexia are combinedAmyotrophy and hyperreflexia are combined
Progressive spinal muscular atrophyProgressive spinal muscular atrophy
Weakness and atrophy occur alone without evidence Weakness and atrophy occur alone without evidence
of cortico spinal dysfunctionof cortico spinal dysfunction
Progressive bulbar palsyProgressive bulbar palsy
Weakness and wasting predominate in muscles Weakness and wasting predominate in muscles
innervated by motor nuclei of lower brainsteminnervated by motor nuclei of lower brainstem
Primary lateral sclerosisPrimary lateral sclerosis
Clinical state dominated by spastic weakness, Clinical state dominated by spastic weakness,
hyperreflxia, and Babinski signs with lower motor hyperreflxia, and Babinski signs with lower motor
neuron affection becoming apparent only at a later neuron affection becoming apparent only at a later
stage of the illnessstage of the illness

4. Amyotrophic lateral Amyotrophic lateral
sclerosissclerosis
Incidence -0.4 to 1.76 per 10000Incidence -0.4 to 1.76 per 10000
Men are affected twice as of womenMen are affected twice as of women
Starts with a weakness in the distal part of the Starts with a weakness in the distal part of the
armarm
Foot drop or awkwardness in tasks requiring Foot drop or awkwardness in tasks requiring
fine finger movementsfine finger movements
CrampingCramping
Slight spasticity of the arms and legs with Slight spasticity of the arms and legs with
hyperreflexia without sensory changehyperreflexia without sensory change

5. Babinski anfd Hoffman’s signs are variably Babinski anfd Hoffman’s signs are variably
presentpresent
Cadaveric or skeletal handCadaveric or skeletal hand
The muscles of the upper arm and The muscles of the upper arm and
shoulder girdles are typically involved latershoulder girdles are typically involved later
The affected part may ache and feel coldThe affected part may ache and feel cold
Sphincteric control is well maintained Sphincteric control is well maintained
Abdominal reflexes may elicitable even Abdominal reflexes may elicitable even
when the plantar reflexes are abnormalwhen the plantar reflexes are abnormal

6. Other patterns of Other patterns of
evolutionevolution
Leg may be affected before the handsLeg may be affected before the hands
Early involvement of thoracic, abdominal Early involvement of thoracic, abdominal
or posterior neck musclesor posterior neck muscles
Early diaphragmatic weaknessEarly diaphragmatic weakness

7. Progressive muscular Progressive muscular
atrophyatrophy
More common in men than women (4:1)More common in men than women (4:1)
In about half of the patients, symmetrical In about half of the patients, symmetrical
weakness of intrinsic hand muscles, slowly weakness of intrinsic hand muscles, slowly
advancing to more proximal parts of the armsadvancing to more proximal parts of the arms
Fascicular twitching or cramping are invariably Fascicular twitching or cramping are invariably
presentpresent
Tendon reflexes are diminished or absent and Tendon reflexes are diminished or absent and
signs of corticospinal tract disease cannot be signs of corticospinal tract disease cannot be
detecteddetected

8. Progressive bulbar palsyProgressive bulbar palsy
Weakness and laxity of muscles innervated by Weakness and laxity of muscles innervated by
motor nuclei of lower brain stem (m. of jaw, motor nuclei of lower brain stem (m. of jaw,
face, tongue, pharynx &larynx)face, tongue, pharynx &larynx)
Early defect in articulationEarly defect in articulation
Syllables lose their clarity and run togetherSyllables lose their clarity and run together
Speech sounds as if the patient is eating food Speech sounds as if the patient is eating food
that is too hotthat is too hot

9. Voice is modified by a combination of atrophic Voice is modified by a combination of atrophic
and spastic weaknessand spastic weakness
Defective modulation with variable degrees of Defective modulation with variable degrees of
rasping and nasalityrasping and nasality
Pharyngeal reflex is lostPharyngeal reflex is lost
Mastication and deglutition also become Mastication and deglutition also become
impairedimpaired
Muscles of the lower face weaken and sagMuscles of the lower face weaken and sag

10. Fasciculations and focal loss of the tissues of Fasciculations and focal loss of the tissues of
the tongue are early manifestationsthe tongue are early manifestations
The jaw jerk may be present or exaggerated at The jaw jerk may be present or exaggerated at
a time when the muscles of mastication are a time when the muscles of mastication are
markedly weakmarkedly weak
Bulldog reflex- jaws snap shut involuntarily Bulldog reflex- jaws snap shut involuntarily
when attempting to open mouthwhen attempting to open mouth

11. Course is progressiveCourse is progressive
Weakness spreads to the respiratory Weakness spreads to the respiratory
muscles and deglutition fails entirelymuscles and deglutition fails entirely
Patient dies within 2-3 years of onsetPatient dies within 2-3 years of onset

12. Primary lateral sclerosisPrimary lateral sclerosis
Slowly progressive corticospinal tract Slowly progressive corticospinal tract
disorder that begins with a pure spastic disorder that begins with a pure spastic
paraparesis later and to a lesser degree paraparesis later and to a lesser degree
the arms and oropharyngeal becomes the arms and oropharyngeal becomes
involvedinvolved
Insidious onset in fifth or sixth decadeInsidious onset in fifth or sixth decade
Stiffness in one leg and then the other Stiffness in one leg and then the other
Slowing of gaitSlowing of gait

13. Spasticity predominating over weakness Spasticity predominating over weakness
as the years go onas the years go on
No sensory symptoms or signsNo sensory symptoms or signs
Progression for 3 years without evidence Progression for 3 years without evidence
of lower motor neuron dysfunctionof lower motor neuron dysfunction
Pathologic studies reveal stereo typed Pathologic studies reveal stereo typed
pattern of reduced numbers of Betz cells pattern of reduced numbers of Betz cells
in the frontal and prefrontal motor cortex, in the frontal and prefrontal motor cortex,
degeneration of the cortico spinal tracts degeneration of the cortico spinal tracts
and preservation of motor neurons in the and preservation of motor neurons in the
brain stem and spinal cordbrain stem and spinal cord

14. Laboratory features of Laboratory features of
motor neuron diseasemotor neuron disease
EMG- widespread fibrillations and EMG- widespread fibrillations and
fasciculations and enlarged motor unitsfasciculations and enlarged motor units
NCV- slight slowing without focal motor NCV- slight slowing without focal motor
conduction blockconduction block
CSF protein is usually normal or marginally CSF protein is usually normal or marginally
elvatedelvated
Serum CK is moderately elevated in patients Serum CK is moderately elevated in patients
with rapidly progressive atrophy and weaknesswith rapidly progressive atrophy and weakness

15. PathologyPathology
Loss of nerve cells in the anterior horn cells of Loss of nerve cells in the anterior horn cells of
the spinal cord motor nuclei of the lower brain the spinal cord motor nuclei of the lower brain
stemstem
Slight gliosis and proliferation of microglial cellsSlight gliosis and proliferation of microglial cells
Surviving nerve cells are small, shrunken and Surviving nerve cells are small, shrunken and
filled with lipofuscinfilled with lipofuscin
Swelling of the proximal axonsSwelling of the proximal axons
Disproportionate loss of large myelinated fibers Disproportionate loss of large myelinated fibers
in motor nervesin motor nerves

16. Corticospinal tract degeneration are most Corticospinal tract degeneration are most
evident in the lower part of the spinal evident in the lower part of the spinal
cord, but I can be traced up to the cord, but I can be traced up to the
brainstem to the posterior limb of internal brainstem to the posterior limb of internal
capsule and corona radiata by means of capsule and corona radiata by means of
fat strainsfat strains
Loss of Betz cells in the motor cortexLoss of Betz cells in the motor cortex

17. Differential diagnosis of Differential diagnosis of
ALSALS
Central spondylotic barCentral spondylotic bar
Ruptured cervical discRuptured cervical disc
Hemiparesis or monoparesis due to Hemiparesis or monoparesis due to
multiple sclerosismultiple sclerosis
Chronic motor poly neuropathyChronic motor poly neuropathy

18. MEDICAL MANAGEMENTMEDICAL MANAGEMENT

19. No specific treatment for motor neuron No specific treatment for motor neuron
diseasesdiseases
Supportive measures are importantSupportive measures are important
Early discussions avoid devastating Early discussions avoid devastating
statementsstatements
Rituzole- anti glutamate agent, slows the Rituzole- anti glutamate agent, slows the
progression of the disease and improve progression of the disease and improve
survival with disease of bulbar onsetsurvival with disease of bulbar onset

20. Muscle spasms and painMuscle spasms and pain
Quinine or baclofenQuinine or baclofen
Aching and muscle sorenessAching and muscle soreness
Pain medicationsPain medications

21. DysphagiaDysphagia
Nutritious diets should be followedNutritious diets should be followed
Risk of aspiration, sialorrheaRisk of aspiration, sialorrhea
Viscosity of saliva can be treated by Viscosity of saliva can be treated by
hydration or papaya tablets, tenderizerhydration or papaya tablets, tenderizer
Bulbar symptoms- nasogastric or Bulbar symptoms- nasogastric or
jejunostomy tubesjejunostomy tubes

22. Respiratory managementRespiratory management
Evaluations of respiratory statusEvaluations of respiratory status
Postural drainage with cough facilitationPostural drainage with cough facilitation
Breathing exercises, chest stretchingBreathing exercises, chest stretching
Incentive spirometryIncentive spirometry
Long term mechanical ventilationLong term mechanical ventilation
Home mechanical ventilationHome mechanical ventilation
Morphine for air hungerMorphine for air hunger

23. Oxygen 2L/minOxygen 2L/min
Non invasive mechanical ventilationNon invasive mechanical ventilation
Negative pressure deviceNegative pressure device
Bi-level positive airway pressureBi-level positive airway pressure

24. Therapeutic management Therapeutic management
of impairment and of impairment and
disabilitiesdisabilities
Patients with severe respiratory and bulbar Patients with severe respiratory and bulbar
problems may not get benefitted from active problems may not get benefitted from active
exercisesexercises
Goal in the end stages- optimize health and Goal in the end stages- optimize health and
improve quality of life improve quality of life
Efficacy is related to timing of interventions Efficacy is related to timing of interventions
the motivation and persistence of the the motivation and persistence of the
patient in carrying out the program and patient in carrying out the program and
support from family memberssupport from family members

25. Evaluation Evaluation
Review of patient’s medical activity recordReview of patient’s medical activity record
Discussion of the patient’s lifestyle or Discussion of the patient’s lifestyle or
interests, work focus, respiratory status, interests, work focus, respiratory status,
psychosocial support issues and patient psychosocial support issues and patient
concerns and goalsconcerns and goals
Baseline testing of muscle strength and Baseline testing of muscle strength and
ROMROM

26. Evaluation of functional activity levelEvaluation of functional activity level
Evaluation of pain, identify what makes Evaluation of pain, identify what makes
the pain worse or betterthe pain worse or better
Evaluation of bulbar and respiratory Evaluation of bulbar and respiratory
functionfunction

27. Intervention goalsIntervention goals
Maintenance of maximal muscle strength Maintenance of maximal muscle strength
within limits imposed by ALS within limits imposed by ALS
Prevention and minimization of Prevention and minimization of
secondary consequences of the disease secondary consequences of the disease
such as contractures, thrombophlebitis, such as contractures, thrombophlebitis,
decubitus ulcers, and respiratory decubitus ulcers, and respiratory
infectionsinfections

28. Therapeutic Therapeutic
considerationsconsiderations
Two major factors should be considered Two major factors should be considered
when planning and implementing activity when planning and implementing activity
or exercise program for patients with ALSor exercise program for patients with ALS
Prevention of disuse atrophyPrevention of disuse atrophy
Prevention of overuse injury Prevention of overuse injury

29. Exercise prescription Exercise prescription
recommendationsrecommendations
To improve compliance consider both a To improve compliance consider both a
formal exercise program and enjoyable formal exercise program and enjoyable
physical activitiesphysical activities
Include activities with opportunities for social Include activities with opportunities for social
development and personnel accomplishmentdevelopment and personnel accomplishment
Strengthening program should emphasize Strengthening program should emphasize
concentric rather than eccentric muscle concentric rather than eccentric muscle
contractionscontractions

30. High resistance strengthening programs High resistance strengthening programs
probably have no benefit over moderate probably have no benefit over moderate
resistance programsresistance programs
Muscles with less than antigravity Muscles with less than antigravity
strength have little capacity to improve; strength have little capacity to improve;
the program should focus on stronger the program should focus on stronger
musclesmuscles

31. Periodically monitor muscles to assess Periodically monitor muscles to assess
for possible overwork weakness, for possible overwork weakness,
particularly in unsupervised programsparticularly in unsupervised programs
Activity modifications should include Activity modifications should include
periods of physical activity with restperiods of physical activity with rest

32. Therapeutic interventionsTherapeutic interventions
Before finalizing the intervention plan based Before finalizing the intervention plan based
on patient goals, the therapist must on patient goals, the therapist must
consider the followingconsider the following
1)1)Typical rate of patient progression Typical rate of patient progression
2)2)Distribution of weakness and spasticity, Distribution of weakness and spasticity,
respiratory factors leading to hypoxemia, respiratory factors leading to hypoxemia,
and easy fatigability and bulbar involvementand easy fatigability and bulbar involvement
3)3)Phase of the diseasePhase of the disease

33. EXERCISE AND EXERCISE AND
REHABILITATION REHABILITATION
PROGRAMS PROGRAMS
ACCORDING TO ACCORDING TO
STAGE OF THE STAGE OF THE
DISEASEDISEASE

34. Phase I (independent)Phase I (independent)
stage 1stage 1
Patient characteristicsPatient characteristics
Mild weaknessMild weakness
ClumsinessClumsiness
AmbulatoryAmbulatory
Independent in ADLIndependent in ADL

35. TreatmentTreatment
continue normal activities or increase continue normal activities or increase
activitiesactivities
Begin program of ROM exercisesBegin program of ROM exercises
Add strengthening program of gentle Add strengthening program of gentle
resistance exercises with cautionresistance exercises with caution
Provide psychological support as neededProvide psychological support as needed

36. Stage 2Stage 2
Patient characteristicPatient characteristic
Moderate selective weaknessModerate selective weakness
Slightly decreased independence in ADLSlightly decreased independence in ADL
AmbulatoryAmbulatory

37. TreatmentTreatment
Continue stretching to avoid contracturesContinue stretching to avoid contractures
Continue cautious strengthening of Continue cautious strengthening of
muscles with grade above 5muscles with grade above 5
Consider orthotic supportConsider orthotic support
Use adaptive equipment to facilitate ADLUse adaptive equipment to facilitate ADL

38. Stage 3Stage 3
Patient characteristicsPatient characteristics
Severe selective weakness in ankles, wrists, and Severe selective weakness in ankles, wrists, and
handshands
Moderately decreased independence in ADLModerately decreased independence in ADL
Easy fatigability with long distance ambulationEasy fatigability with long distance ambulation
AmbulatoryAmbulatory
Slightly increased respiratory effortSlightly increased respiratory effort

39. TreatmentTreatment
Continue stage 2 program as toleratedContinue stage 2 program as tolerated
Keep patient physically as long as Keep patient physically as long as
possible through pleasurable activitiespossible through pleasurable activities
Encourage deep breathing exercise, chest Encourage deep breathing exercise, chest
stretching, postural drainage if neededstretching, postural drainage if needed
Prescribe wheel chairPrescribe wheel chair

40. Phase II(partially Phase II(partially
independent)independent)
Stage 4Stage 4
Patient characteristicsPatient characteristics
Hanging arm syndrome with shoulder Hanging arm syndrome with shoulder
pain and sometimes edema in the handpain and sometimes edema in the hand
Wheel chair dependentWheel chair dependent
Sever lower extremity weaknessSever lower extremity weakness
Able to perform ADL but fatigues easilyAble to perform ADL but fatigues easily

41. TreatmentTreatment
Heat, massage, preventive anti edema Heat, massage, preventive anti edema
measuresmeasures
Active assisted ROM exercise to weakly Active assisted ROM exercise to weakly
supported jointssupported joints
Encourage isometric contractionsEncourage isometric contractions
Try arm slings, overhead slingsTry arm slings, overhead slings
Motorized chair if want to be independentMotorized chair if want to be independent

42. Stage 5Stage 5
Patient characteristicsPatient characteristics
Severe lower extremity weaknessSevere lower extremity weakness
Moderate to severe upper extremity Moderate to severe upper extremity
weaknessweakness
Wheel chair dependentWheel chair dependent
Increasingly dependent on ADLIncreasingly dependent on ADL
Possible skin break down secondary to poor Possible skin break down secondary to poor
mobilitymobility

43. TreatmentTreatment
Encourage family to learn proper transfer Encourage family to learn proper transfer
positioning principles, and turning techniquespositioning principles, and turning techniques
Encourage modifications at home to aid patients Encourage modifications at home to aid patients
mobility and independencemobility and independence
Electric hospital bed with anti pressure mattressElectric hospital bed with anti pressure mattress
If there is HMV adapt chair to hold itIf there is HMV adapt chair to hold it

44. Phase III (dependant)Phase III (dependant)
stage 6stage 6
Patient characteristicsPatient characteristics
BedriddenBedridden
Completely dependent in ADLCompletely dependent in ADL

45. TreatmentTreatment
For dysphagia soft diet, long spoons, tube feeding, For dysphagia soft diet, long spoons, tube feeding,
percutaneous gastrstomypercutaneous gastrstomy
To decrease flow of accumulated saliva: medication, To decrease flow of accumulated saliva: medication,
suction, surgerysuction, surgery
For dysarthria: palatal lifts, electronic speech For dysarthria: palatal lifts, electronic speech
amplification, eye pointing electronicamplification, eye pointing electronic
For breathing difficulty: clear airway, tracheostomy, For breathing difficulty: clear airway, tracheostomy,
respirator, medications to decrease the impact of respirator, medications to decrease the impact of
dyspnea dyspnea