Motor neuron disease
Motor Neuron Diseases

 The
motor neuron diseases (MNDs) are a group of
progressive neurological disorders that destroy cells
that control essential muscle activity such as
speaking, walking, breathing, and swallowing.
ALS Demographics
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Incidence 2 per
100,000
Male slightlymorethan
Female
Peak age of onset: 6th
decade (range 20 to
90)
Classification of Motor neuron disease
UMN:
 Primary Lateral Sclerosis (PLS)
LMN:
 Spinal Muscular Atrophy (SMA)
 Progressive Muscular Atrophy
UMN and LMN:
 Amyotrophic Lateral Sclerosis (ALS)
cause


No known cause
Possible cause could be
 Mitochondrial dysfunction
 Gene defects
 Cytoskeletal protein defects
 Autoimmune dysfunction/ Inflammatory Damage
 Virus
Amyotrophic Lateral Sclerosis (ALS)


originally described by Charcot and thus it is
often called Charcot's disease in Europe.
Classical Amyotrophic Lateral Sclerosis (ALS) is
a distinct syndrome characterized by a
combination of Upper motor neuron (UMN) and
Lower motor neuron (LMN) signs and symptoms
Clinical features
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
(65% cases)affects
the hands first
fasciculation is seen
on examination
Clinical features


wasting of the
muscles and
paralysis of
movement.
Weakness of the
muscles of the chest
wall and diaphragm
leads to difficulty in
breathing.
Clinical features


Weakness of muscles
controlling facial
expression, the tongue,
the palate
ability to cough
effectively and clear
secretions is lost.
ALS: Prognosis

Poor prognosis if:
 Bulbar onset
 Older age at diagnosis
ASSESSMENT
 Level
of consciousness and orientation
 Basic ability to communicate
 Motor ability
 Co-ordination
 Respiratory system
 Swallow
 Function
 Equipment and environment
 Bladder and bowel function
 Mood
 Pain
Goals of Physical Therapy
Management

The overall goals of intervention will vary as the
condition progresses.
 Early stage - to optimise remaining function
 Middle stage - to maintain functional mobility
 Late stage - to maximise quality of life
Early stage

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Preserving optimum level of mobility
Active range of movement exercises
Strengthening exercises
Aerobic activities
Training in pacing activity/fatigue management
Stretching of affected joints
Teaching breathing exercises
Provision of appropriate equipment and assistive
devices to support weak muscles.
Provision of appropriate walking aids
Middle stage

The patient may remain
ambulatory but have
severe weakness in certain
muscle groups
 provision
of adaptive
equipment (e.g. Ankle Foot
Orthosis, splints, arm
slings/trays, and wheelchair.
 access to equipment,
provision of walking aids
(Advice on footwear)
 management of oedematous
limbs
Middle stage
 soft
collar provision
 active,
assisted active, passive
and /or aerobic exercise, as
appropriate
Middle stage
 teaching
breathing exercises and
assisted coughing
 advice
regarding pressure care
and provision of pressure
relieving seating/mattress
 management
of spasticity.
prevention of contractures
 position
for comfort
Late stage

The patient uses a wheelchair when out of bed or
remains in bed
 teaching carers to perform gentle passive stretches,
heat treatment and/or massage to relieve pain
and improve comfort
 advising carers regarding positioning and
movement of the patient in bed
 modified postural drainage to decrease retention
of secretions, self-assisted or carer-assisted
coughing and airway clearance techniques may
need to be taught.