Motor neurons are neurons that control muscles and glands. Their cell bodies are located in the brainstem or spinal cord, and their axons project to muscles. Motor neuron disease (MND) refers to conditions where motor neurons degenerate, leading to muscle weakness and atrophy. The most common type is amyotrophic lateral sclerosis (ALS), where both upper and lower motor neurons are affected. In ALS, muscles weaken and waste away as motor neurons die, and symptoms may include limb weakness, bulbar problems like slurred speech, and respiratory issues. The disease progresses as motor neurons continue to deteriorate over time.

1. MOTOR NEURON
DISEASE
DHRUVI MISTRY
2ND YEAR MPT IN NEUROLOGICAL
SCIENCES

2. What is motor neuron?
 A motor neuron (or motoneuron) is
a neuron whose cell body is located in
the motor cortex, brainstem or
the spinal cord, and
whose axon (fiber) projects to the
spinal cord or outside of the spinal
cord to directly or indirectly control
effector
organs,mainly muscles and glands.

3. Types of neurons
upper motor neurons Lower motor neurons
 UMN have their cell
bodies located in
cerebral hemisphere.
 They form descending
pathways of brain and
synapse with the nuclei
of brainstem and spinal
cord.
 Involved in the
voluntary control of
muscular activities.
 LMN have their cell
bodies located in the
brainstem and spinal
cord.
 The axons from the lower
motor neurons
are efferent nerve
fibers that carry signals
from the spinal cord to
the effectors.

4. MOTOR NEURON DISEASE
 MND refers to a heterogeneous
group of conditions characterized
by degeneration of lower motor
neurons and/or upper motor
neurons.
 Progressive degeneration and loss
of motor neurons in the spinal cord
with or without similar lesions in
the motor nuclei of the brainstem
or the motor cortex

5. AETIOLOGY
 A mutation of the superoxide
dismutase (SOD-1) gene has been
identified in approximately 20% of
families with the familial manifestation
of MND.
 Superoxide dismutases are a group
of enzymes that eliminate oxygen free
radicals that, although products of
normal cell metabolism, have been
implicated in neurodegeneration.

6. There are three isoform of SOD in
humans:
- CuZnSOD, MnSOD, ECSOD.
 SOD-1, a gene on chromosome 21,
encode CuZnSOD.
 When mutation in SOD1 occur, SOD
enzyme activity is decreased lead to
accumulation of free radicals, causing
damage.

7.  Glutamate, an excitatory
neurotransmitter, has also been
implicated in neurodegeneration
 Excess glutamate trigger a cascade of
events leading to cell death
 Clumping of neurofilament proteins
into spheroids in the cell body and
proximal axon is one of the cause.

8.  Autoimmune reaction is also
etiological factor.
- serum factors toxic to AHC
- antibodies to calcium channels

9. EPIDEMIOLOGY
 In UK ,the incidence is the thought to
be 2 per 1,00,000 and the prevalence
7 per 1,00,000.
 Occurrance is slightly higher in men
approximate ratio- 1.7 : 1
 and onset is most common between
the ages of 50 and 70 years.

10. PATHOPHYSIOLOGY
 MND is characterized by a
progressive and irreversible
deterioration of upper and lower motor
neurons.
 Degeneration within the corticospinal
and corticobulbar tracts gives rise to
upper motor neuron symptoms.
 Brainstem nuclei for cranial nerves
trigeminal (V), facial (VII),
glossopharyngeal (IX), vagus (X), and
hypoglossal (XII)

11.  and anterior horn cells in the spinal
cord are also involved.
 As motor neurons degenerate, they
can no longer control the muscle
fibers they innervate.
 Healthy, intact surrounding axons can
sprout and reinnervate the partially
denervated muscle, in essence
assuming the role of the degenerated
motor neuron

12. and preserving strength and function
early in the disease, however, the
surviving motor units undergo
enlargement.
 Reinnervation can compensate for the
progressive degeneration until motor
unit loss is about 50% and EMG
studies have found evidence of motor
unit reinnervation in individual with
MND.

13.  As the disease progresses,
reinnervation cannot compensate for
the rate of degeneration, and a variety
of impairments develop.
 The progression of MND is thought to
spread in a contiguous manner.

14. Possible risk factors for MND
 Trauma
- skeletal trauma
- fractures
- severe electrical shock with
unconsciosness
 Diet
- high fat intake
- high glutamate intake
- low fiber intake
- low antioxidant intake

15.  Neurotoxicant exposure
- lead & mercury
- pesticide exposure
- solvent exposure
 Lifestyle factors
- cigarette smoking
- alcohol intake

16. TYPES OF MND
 1) amyotrophic lateral sclerosis
 2)progressive muscular atrophy
 3)progressive bulbar palsy
 4)primary lateral sclerosis
 5)kennedy’s syndrome
 6)multifocal motor neuropathy
 7)spinal muscular atrophy

17. AMYOTROPHIC LATERAL
SCLEROSIS (ALS)
 ALS is disease of part of the nervous
system that controls voluntary
movements
 “Amyotrophic” = without muscle
nourishment, resulting in loss of nerve
signals to muscles
 “Lateral” = to the side, referring to
location of damage in spinal cord
(corticospinal tract)
 “Sclerosis” = hardened nature of spinal
cord (Pathological Change)

18.  Degenerative disorder if UMN and
LMN resulting in a mixed picture of
upper and lower motor signs in limbs,
trunk, respiratory muscles and
affecting bulbar functions.
 The process is remarkably selective,
leaving special senses, cerebellar,
sensory and autonomic functions
intact.

19.  Typically sparing of certain areas until
the later stages of disease i.e. cranial
nerves : 3rd , 4th ,6th and spinal nerves
S1-S3.
 This explains the preservation of ocular
movement and bladder and bowel
function.
 Initially the involvement tends to be
either lower or upper motor neuron in
nature.
 The coexistence of both, in the absence

20. Pathology of ALS
 ALS is characterized by motor neuron
degeneration and death with gliosis
replacing lost neurons.
 Gliosis in corticospinal tract results in
the bilateral white matter changes
seen in MRI.
 The spinal cord becomes atrophic and
the ventral root become thin and loss
of large myelinated fibers.

21.  Loss of neurons in anterior horn of
spinal cord and motor nuclei of lower
brainstem
 Pathologic features include cell body
shrinkage, pyknosis (irreversible
condensation of cromatine in nucleus
of a cell), ghost cells, neuronophagia
(destruction of neurons by
Phagocytes), and gliosis
 Loss of Betz cells in precentral motor
cortex

22. Clinical presentation of ALS
 Typical clinical presentation- patient
with progressive motor deterioration
manifesting with both UMN and LMN
signs and symptoms. i.e. combination
of marked weakness and wasting but
brisk reflexes, spasticity and
pathological reflexes.
 Muscle weakness begins in a focal
area before involving contiguous
muscles in same region and then
other region.

23.  So may resemble focal
mononeuropathy (
PSEUDONEURITIC or FLAIL –LEG
presenation)
 Limb weakness : progressive
assymetrical weakness of limbs.
 Upper limb > lower limb
 Begins in the legs as foot drop and
begins in the hands with difficulty of
fine movements

24.  Localized wasting
 Severe cramps : Common in legs,
thighs, abdomen, back or even tongue
at night
◦ Often resolve spontaneously with
disease progression
◦ May be severe later in disease:
Associated with prominent spasticity
◦ Fasciculation :ALS rarely presents with
fasciculations in the absence of
weakness

25.  Bulbar symptoms :
 Presenting feature in about 25% of
patients
◦ Dysarthria
◦ Nasal speech
 Speech rate: Slow
 Voice quality: Reduced
◦ Dysphagia

26.  Pseudobulbar symptoms :
Coticobulbar tracts involvement
Spastic dysarthria, dysphonia,
dysphagia
Emotional lability(forced crying or
laughter)
Brisk jaw jerk
Hyperactive gag reflex

27.  Areas of weakness with some
specificity for ALS
- Very proximal denervation
◦ Paraspinous
◦ Posterior neck muscle – head fall
forward & neck completely flexed
- Jaw weakness: Closure; Opening
- Voice
◦ Nasal, slurred speech
◦ Continuous emission of sound

28. Associations:
 Loss of sense of taste; Weight loss;
Depression
 Weakness of respiratory group of
muscles
 Muscle wasting: Usually associated
weakness
 Wasting of hands & arms in ALS
 Stiffness, weakness, wasting in hand
muscles; impaired fine finger
movements (e.g. buttoning, turning
ignition keys)

29.  Finger abductors, adductors,
extensors weak before finger flexors
(resulting in clawed hand with
hollowing of dorsal interosseus
spaces)
 Leg involvement with foot drop

31. Progression:
 Regional symptoms usually progress
first in affected region
 Continuous progression once region
(bulbar, arms, or legs) involved
 Other regions involved later
◦ Spread usually to adjacent region
◦ No predictor of time of involvement
◦ Periods of symptomatic stabilization may
occur
 Remissions in symptoms or signs rare

32. UMN
•Weakness
•Slowed rapid
alternating movements
•Spasticity, clasp knife
•Hyperreflexia
•Pathologic reflexes,
including Babinski
•Pseudo bulbar palsy
(labile affect, spastic
speech, dysarthria,brisk
jaw jerk, and gag reflex)
LMN
 Weakness
 Muscle atrophy
 Fasciculations
 Cramps
 Attenuated or
absent reflexes
 Bulbar palsy
 hypotonia

33. Other symptoms :
 In case of dysphagia, weight loss is
often rapidly progressive
 Sleep disturbances from hypopnea
and apnea
 Cognitive dysfunction occurs in 20-
50% of cases, and 5-15% develop
dementia.

34. World Federation of Neurology
criteria for diagnosis of ALS
1) The presense of
 evidence of LMN degeneration on
clinical, electrophysiologic or
neuropathogenic examination
 Evidence of UMN degeneration on
clinical examination
 Progression of the motor syndrome
within a region or to other regions, as
determined by history or examination;

35. 2)The absence of
 electrophysiological and pathological
evidence of other process that might
explain UMN and /or LMN signs;
 Neuroimaging evidence of other
disease processes that might explain
the observed clinical and
electrophysiological signs

36. World Federation of Neurology EI Escorial revisited
criteria for dx of MND

37. PROGRESSIVE MUSCULAR
ATROPHY
 It is pure LMN presentation.
 Most benign variety of MND
 Atrophy and fasciculations : wasting of
one hand and tendon become
prominent as the hand muscles waste,
giving guttered appearance.
 Loss of tendon reflexes

38. Weakness:
◦ Distal & Proximal: Either may be
more prominent
◦ Patients develop distal limb
wasting
◦ Long clinical course, with slow
progression to proximal limb
muscles
◦ Asymmetric
◦ Often involves paraspinous &
respiratory muscles
◦ Often spares bulbar musculature

39.  Spontaneous motor activity
◦ Cramps: Common in legs, at night
◦ Fasciculations
 No upper motor neuron signs
 Time course
◦ Progressive
◦ Similar to, more rapid, or slower
than, typical ALS

40. Pathology
Loss of motor neurons in anterior horn
of spinal cord
Shrinkage of remaining motor neurons

41. PROGRESSIVE BULBAR
PALSY
 Progressive bulbar palsy present with
a combination of corticobulbar
degeneration and lower cranial
nerve (V, VII, IX, X & XII) motor
nuclei involvement with sparing of
cranial nerve III, IV, VI.

42.  In bulbar palsy (LMN type):-
- dysphagia follow by dysarthria
- nasal regurgitation of fluid and
nasal
slurred voice
- tongue wasted and folded,
fasciculation
visible.
- absent jaw jerk and gag reflex
brisk.

43.  In pseudobulbar palsy (UMN type):-
- weakness of muscle of mastication and
difficulty in chewing.
- expressionless face
- exaggerated jaw jerk & brisk gag reflex
- palatal weakness (X) allowing food &
fluid to
enter nasopharynx.
- monotonous speech
- tongue immobile & can’t be protruded
(XII)
- emotional lability ( pathological laughing
&
crying).

44. PRIMARY LATERAL
SCLEROSIS
 PLS is uncommon MND.
 PLS was first described by Charcot
in 1865.
 Slowly progressive spasticity and
weakness of the limbs.
 Adult onset- age- 50-55 years.
 Absent of family history.

45.  progressive corticospinal
involvement and characterized by
lower limbs spasticity followed by
upper limbs involvement, rare
cranial nerve involvement.
 Associated with pseudobulbar palsy.
 Eventually, spastic bulbar palsy may
evolve speech, swallowing and
emotional problems

46.  It has pure motor presentation
 It has better prognosis.

47. KENNEDY’S SYNDROME
 X-linked LMN disease.
 A rare form of MND with near normal
life expectancy.
 Bulbar and spinal atrophy
 Presents in males between age of 20
and 50.
 c/f : mild dysarthria, wasted
fasciculating tongue, tremor, proximal
weakness with wasting and
fasciculations, gynecomastia and
reduced fertility.

48. MULTIFOCAL MOTOR
NEUROPATHY (MMN)
 Occurs in men under 45 with
assymetrical, progressive, mainly
distal weakness with onset in the
arms.
 c/f: fasciculation, wasting and reduced
reflexes.
 EMG shows multifocal conduction
block.
 High serum titres of antibodies to GM1
ganglioside often detactable.

49. SPINAL MUSCULAR
ATROPHY(SMA)
 Disease that usually present in infancy
or childhood.
 It affect only lower motor neurons.
 SMN1 (Telomeric SMN (SMNT)) gene
mutated in 95% of SMA
 Contains 9 exons
 Codes for protein containing 294
amino acids

50. Types of
SMA
Type 1
SMA
Type ||
SMA
Type|||
SMA
Type 1V
SMA

51.  Type | SMA : Werdnig Hoffman
disease
 Autosomal recessive disease
 It presents at birth with hypotonia,,
reduced movements, proximal
weakness, wasting and reduced
reflexes.
 Respiratory insufficiency: Paradoxical
respirations
 Death before one year of age.

52.  Swallowing & sucking are impaired.
 All motor milestones are delayed.
 Type || SMA : preesnt at 6 months
and has a more protracted course.
 Death from respiratory failure at age of
10.

53.  Type ||| SMA : Kugelberg-Welander
disease
 Present at about 10 years of age with
life expectancy of about 35years.
 Progression tend to be slower than
lower motor neuron forms of MND.
 Autosomal recessive disease
Onset: Often > 18 months

54.  Stand independently
 Life span
◦ Shortened
 Cramps may be 1st symptom
 Weakness of limb muscle, face,
tongue, diaphragm.
 Limb girdle dystrophy
 Tremor
 Tendon reflexes: Reduced

55.  Type 1V SMA : has an early adult
onset. Survival may be normal.
Onset:- b/w 2nd to 5th decade
 Involvement of only LMN
 Foot drop
 Weakness & wasting of hand muscle
 Deep tendon reflex absent or
diminished

56. Pathology of SMA
Muscle Pathology:-
◦ Grouped atrophy of large fibers:
Mostly type 1;
◦ Hypertrophy of small fibers mixed or
type 2
◦ Development: Muscle weakness &
atrophy may precede loss of motor
neurons

57. Spinal cord:-
◦ Loss of motor neurons in anterior
horn
 Time period: 12 weeks of gestation
to birth; Similar to period of normal
cell death
 Apoptosis: Increased only at 12 to
16 weeks, Not post-natal

58. Complications of SMA
 Scoliosis, contractures, and pneumonia
are predictable complications
 Forced vital capacity decreased
 Development of orthopedic deformities in
patients with SMA II and III
 SMA I patients rarely survive long
enough to develop spinal deformities
 SMA patients who walked, but never
normally, typically lose walking ability by
15 years. Those who developed
weakness after walking normally can
walk until the 30s or 40s

59. INVESTIGATIONS OF MND
Genetic test
Laboratory studies
Electromyography
Nerve conduction velocity studies
Muscle & nerve biopsies
Neuroimaging studies
Pulmonary function test

60. ◦ EMG and NCV reveal anterior horn
cell damage but do not specify the
cause.
◦ EMG : Active denervation (fibrillations
and positive waves)
- Fasciculations
- Chronic denervation (large
amplitude, prolonged duration
polyphasic MUAPs)
 NCV : shows normal velocities and

61. exclude in all limbs multifocal
neuropathy with conduction block
 MRI : MRI exclude foramen magnum
or spinal cord compression in neck.
 Cranial MRI in patients with bulbar to
exclude brainstem lesion.

62. Laboratory studies
 Serum and urine studies to exclude
other metabolic causes
 Lumbar puncture usually yields normal
CSF
 CK usually normal or slightly elevated
Thyroid and calcium studies exclude
endocrine or metabolic diseases.

63. DIFFERENTIAL DIAGNOSIS
1. Cervical spondylosis – produce UMN
and LMN signs
2. Spinal tumours – produce combined
UMN and LMN signs.
3. Hexosaminidase deficiency – mimic
MND.
4. Hyperthyroidism & hyperparathyroidism
– produce muscle wasting and
hyperreflexia.
5. Cerebrovascular disease – produce
pseudobulbar palsy.

64.  Syringomyelia : MRI
 Syringobulbia : cranial MRI
 Cervical radiculomyelopathy : CT scan
, cervical MRI
 Cervical myelopathy with lumbosacral
radiculopathy : MRI , EMG studies

65. management
medical physiotherapy

66.  MEDICAL management:
- Disease modifying agents
- Symptomatic management

67. MEDICAL MANAGEMENT
1. DISEASE MODIFYING AGENTS:-
 Riluzole (Rilutek) - glutamate inhibitor
standard dose – 50mg – 2 time/day
extending survival for 2-3 months.
side effect – liver toxicity , asthenia ,
Nausea , vomiting
Dizziness

68. Botulinum toxin to control drooling
Baclofen for spasticity

69. Symptomatic treatment
1. For sialorrhea : Anti-cholinergic
medications:-
- Glycopyrrolate
- Benztropine
- Transdermal hyoscine
- Atropine
Botulinum type injection into parotid and
submandibular glands.

70.  Mechanical suction – to remove
oropharyngeal secretion.
 Dysphagia : food thickner initially, but
definitive procedure is percutaneous
endoscopic gastrostomy.
 A PEG is a type of gastrostomy tube
inserted via endoscopic surgery that
creates a permanent opening into the
stomach for the introduction of food.

71.  Ventilatory failure : Respiratory
impairments place the patient at risk
for respiratory infections .
 non invasive IPPV in patients who
have good bulbar and limb function

72. Medication for muscle cramps :-
Anticonvulsant medication-
- phenytoin
- carbamazepine
- benzodiazepines – diazepam ,
clonazepam
 Side effects:- gastrointestinal upset,
rash, sedation, dizziness, respiratory
depression and increased weakness

73. Medication for brisk, widespread
fasciculations:-
1. Avoid or minimize caffeine and
nicotine.
2. Lorazepam to decrease the
intensity of the fasciculation.

74.  Medication for pain:-
1. Mild pain or pain associated
with joint discomfort:- analgesic-
acetaminophen & NSAIDs.
2. severe refractory pain:-
- narcotics- codeine,
hydrocodone,
3. In terminal stage of ALS:-
morphine

75. PHYSIOTHERAPY
MANAGEMENT
GOALS:-
 Promoting independence &
maximizing function throughout the
disease.
 Minimizing potential impairment
 Improve ROM
 Improve aerobic capacity or strength
 Prevent respiratory complication-
pneumonia atelectasis

76.  Promoting health & wellness
 Providing alternative means of
carrying out functional activities and
performing task
 Providing education & psychological
support.

77. 1. Restorative intervention:-
 It is directed toward remediating or
improving impairments and activity
limitation.
2. Compensatory intervention:-
 It is directed toward modifying activities,
tasks, or the environment.
3. Preventive intervention:-
 It is directed toward minimizing potential
impairments.

78. Framework For Rehabilitation-
Early stage
Common impairment:-
- Mild to moderate weakness in
specific muscle groups
- Difficulty with ADL and mobility
toward the end of disease.

79. Restorative/preventive
intervention:-
- Strengthening exercise
- Endurance exercise
- Active ROM
- Active-assisted ROM
- Stretching exercise

80. Framework For Rehabilitation-
Middle stage
Common impairment:-
- Progressive decrease in mobility
- Severe muscle weakness
- Progressive decrease in ADL skills
- Pain

81. Compensaroty intervention:-
- Support weak muscle by using
assistive and supportive devices,
adaptive devices, slings, orthoses
- Modification of home/workplace
- Use wheelchair

82. Preventive intervention:-
- Active ROM exercise
- Active-assisted ROM exercise
- Passive ROM exercise
- Stretching exercise
- Strengthening and endurance
exercise

83. Framework For Rehabilitation-
Late stage
Common impairment:-
- Wheelchair dependent
- Complete dependence with ADL
- Severe muscle weakness of UE, LE,
neck
and trunk muscles
- Dysarthria & Dysphagia
- Respiratory compromise
- Pain

84. Preventive intervention:-
- Passive ROM
- Pulmonary care
- Skin care & hygiene
- Use pressure relieving device to
prevent
pressure-sore
 Compensatory intervention
- educate about transfers, positioning,
turning.

85. Common impairment & its
management
Cervical muscle weakness
 For mild to moderate cervical muscle
weakness, a soft foam collar may be
worn during specific activities.
 Soft collars are comfortable and
usually well tolerated.
 For moderate to severe weakness, a
semirigid or rigid collar is use.

86.  Some patient with combined cervical
and upper thoracic weakness may
benefit from a cervical-thoracic
orthosis, or a sternal occipital
mandibular immobilizer (SOMI).

87.  In addition to wearing collar,
individuals with cervical weakness
may also benefit from……..
 Taking frequent rest periods,
 Supportive seating, such as high-
back chairs or recliners, tilt in space
or reclining wheelchairs,
 Elevating reading material,
 Good arm support for prolong sitting.

88. Dysarthria & Dysphagia
PT can play a role in managing
dysarthria & dysphagia by addressing
the patient’s head and trunk control
and position in sitting.
In addition, the PT can reinforce the
use of strategies for eating and
swallowing (chin tuck), the use of
communication devices and food
consistency modifications.

89. Dysarthria impairments are managed
primarily by a speech-language
pathologist.
 Initial speech changes are usually
managed with environmental
modifications, such as decreasing
background noise.
As the severity of dysarthria
progresses, management will focus on
decreasing the patient’s dependence on
speech as the primary method of

90. A palatal lift prosthesis may be
prescribed for individuals with good
articulation but who have a breathy
voice quality or decreased loudness
because of excessive air loss through
the nose.
It allows the soft palate to close
around the surrounding structures
such as the pharynx, making verbal
communication more understandable
by reducing or eliminating hypernasal

91.  The device also lowers the hard
palate, which reduce tongue
movement allowing speech to be less
fatiguing.

92. Upper limb muscle weakness
Patient with a painful shoulder due to
subluxation may benefit from a sling.
Splinting of the wrist or hand may
be indicated to prevent contracture
or to improve the patient’s function,
such as the ability to grasp.

93. Shoulder pain
Modalities,
 ROM exercise,
 Passive stretching,
 Joint mobilization,
 Proper joint support & protection.

94. Respiratory muscle weakness
 Education:-
 Patient and caregivers must be taught
how to balance activities and rest and
educated about energy conservation
techniques.
 Also educate about sign and
symptoms of aspiration, positioning to
avoid aspiration, such as upper
cervical spine flexion during eating.

95. Also educate cause and signs of
respiratory infection,
Strategies for managing oral
secretions.
Also teach specific breathing exercise
and positioning.
Airway clearance techniques may
be use when conditions that cause
secretion retention, such as
pneumonia or atelectasis arise.

96. To compensate for a weakened cough,
the patient and caregiver may be
instructed in the use of manually
assisted coughing techniques &
mechanical insufflation - exsufflation
(MI-E) device.
High-frequency chest wall
oscillation:-
HF-CWO is an external noninvasive
modality that trasmits high-frequency
oscillatory pressure through the chest

97. thereby mobilizing secretions from the
small peripheral airway and
enhancing secretion clearance and
gas exchanges.
 HF-CWO has been effectively used in
patient populations in which secretion
retention and hypersecretion are
issues.

99. Lower limb weakness & gait
impairment
Orthoses:-
Orthoses may be recommended to
improve function by……
- offering support to weakened muscle
& the
joints they surround,
- decrease the stress on remaining
functioning or compensatory
muscles,
- conserve energy,
- minimize local or general muscle

100. Controlling knee impairment can
often be achieved through an ankle-
foot orthosis (AFO), such as,
addressing the ankle should be first.
It is important to consider the weight of
the orthosis an individuals with MND
will have energy expenditure issues,
and it may be more fatiguing for the
patient to ambulate with a heavy
orthosis, for that reason KAFO is not
recommended.

101. Solid AFOs are a good choice for
patients who have medial/lateral
instability of the ankle with
quadriceps weakness.
The fixed ankle position, combined
with the quadriceps weakness, may
make it difficult for sit-to-stand
transfer, climbing stairs.
Hinged AFOs allow dorsiflexion and
may be appropriate for the patient with
adequate knee extensor strength with
mild ankle strength loss.

102. Ambulatory assistive device:-
The type of ambulatory assistive device
prescribed is dependent on…..
- the degree of proximal muscle
strength,
- function of the UEs,
- the pattern, extent and rate of
disease
progression,
- acceptance by the patient,
- financial constraints.

103. Wheeled walkers, which do not
require the patient to lift the device,
are usually recommended.
In general, crutches are not
recommended, if crutches are
warranted, loftstrand (canadian)
crutches are preferred.

106. Decreased mobility
Patient with LE weakness may have
difficulty with sit-to-stand or car transfers.
 Simple intervations:-
 Placing a firm cushion 2-3 in thick under
the buttocks in chair or elevating the
chair by placing the leg in prefabricated
blocks.
 Self powered lifting cushion
 Upholstered reclining chair with powered
seat lifts.

108. Transfer boards:-
Transfer boards may be used for
transfers once the individual is unable
to stand and adequate arm strength &
good sitting balance.
 Swivel cushion or seats.
 Transfer belts

110. Once an individual cannot perform
transfer, following techniques are use.
- hydraulic or mechanical lifts
- easy pivot
- electric bed
- stairway lift
- wheelchair

112. Muscle cramps & spasticity
 Muscle cramps may be alleviated with
massage and a stretching.
 Manage spasticity with…..
- cold
- slow prolonged stretches
- passive ROM exercises
- postural and positioning techniques
- splinting to prevent contractures.

113. Psychosocial issues
A diagnosis of MND is devasting for
both the patient and family.
 Because of the progressive nature of
the disease, impairments readily leads
to activity limitation and participation
restrictions, which may affect quality of
life.
 Much is lost when living with a
terminal, progressive disease:
physical health and

114. abilities, body image, work and family
roles, identity; family and social
networks, lifestyle, independence,
control, hope, meaning.
The physiotherapist must be able to
recognize the patient’s ability to cope
and adapt, and his or her
psychological reactions, level of
acceptance, and willingness and
ability to integrate therapeutic
recommendations.

115.  The physiotherapist must be able to
know the presence of clinical anxiety
and depression, and refer the patient
to the appropriate health care team
member, when necessary.

116. PROGNOSIS
Age at time of onset has the strongest
relationship to prognosis.
Patient less than 35 to 40 years of age
at onset had better 5 year survival
rates than older individuals.
Individuals with limb onset have a
better prognosis than those with
bulbar onset.

117.  1. ROWLAND LP , SCHEIDER N : Amyotrophic lateral
sclerosis. New. Engl J. Med. (2002) 344 (22)
 2. A.F. Golwalla & S.A. Golwalla : Golwalla’s MEDICINE for
students; 25th edition
 3. M. Stokes & E Stack; Physical management for
neurological conditions ;3rd edition
 4. J. DelLisa; DeLisa’s Physical Medicine and Rehabilitation;
5th edition