MOTOR NEURON DISEASE
Definition
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Motor Neuron Diseases
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group of diseases which include progressive degeneration and loss of
motor neurons with or without similar lesion of the motor nuclei of the
brain
replacement of lost cells with gliosis
ALS Demographics
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Incidence 2 per 100,000
Male slightly > Female
Peak age of onset: 6th
decade (range 20 to 90)
No racial predilection
95% sporadic
5% hereditary
classification
UMN:
 Primary Lateral Sclerosis (PLS)
 Familial Spastic Paraparesis
LMN:
 Spinal Muscular Atrophy (SMA)
 Progressive Muscular Atrophy
UMN and LMN:
 Amyotrophic Lateral Sclerosis (ALS)
cause
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No know cause
Possible cause could be
Defective glutamate metabolism
 Free radical injury
 Mitochondrial dysfunction
 Gene defects
 Programmed cell death (apoptosis)
 Cytoskeletal protein defects
 Autoimmune dysfunction/ Inflammatory Damage
 Protein aggregation
 Virus
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Amyotrophic Lateral Sclerosis
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originally described by Charcot and thus it is often
called Charcot's disease in Europe. Classical ALS is
a distinct syndrome characterized by a combination
of UMN and LMN signs and symptoms
Clinical features
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(65% cases)affects the hands first
fasciculation is seen on examination
wasting of the muscles and paralysis of movement.
Weakness of the muscles of the chest wall and
diaphragm leads to difficulty in breathing.
muscles controlling facial expression, the tongue, the
palate
ability to cough effectively and clear secretions is
lost.
Diagnostic Triad:
ALS
Upper motor
neuron
Progression
Lower motor
neuron
ALS: Prognosis
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Prognosis
 50% dead in 3 years
 20% live 5 years
 10% live 10 years
Worse prognosis if:
 Bulbar onset
 Simultaneous arm/leg onset
 Older age at diagnosis (onset < 40: 8.2 yr duration, onset
61-70: 2.6 yr duration)
medication
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Riluzole, a drug given orally may have some effect in
slowing the progression of the disease
Symtomatic treatment
Baclofen, dantrolene, diazepam, gabapentin to relieve
muscle stiffness and cramps
 Analgesics – paracetamol,
 Hyoscine, atropine to reduce salivation
 Antidepressants e.g. amitriptiline, fluoxetine to alleviate
symptoms of anxiety, depression and emotional lability
 Benzodiazepines e.g. diazepam to reduce anxiety
 Opiates e.g. morphine, fentanyl, oxycodone to control pain,
treat breathlessness, choking attacks and reduce anxiety
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ASSESSMENT
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Subjective assessment:
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Course of the disease
Social history and support network
Lifestyle
Expectations
Objective assessment:
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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
Outcome Measurement
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Modified fatigue impact scale
Berg balance scale
Elderly Mobility Score
Timed walk
Timed Unsupported Steady Stand
Ashworth scale
intervention
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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
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The patient may remain ambulatory but have severe weakness in certain
muscle groups
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provision of adaptive equipment (e.g. Ankle Foot Orthosis, splints, arm slings/trays, and
wheelchair.
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access to equipment, provision of walking aids (Advice on footwear)
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management of oedematous limbs
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soft collar provision
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active, assisted active, passive and /or aerobic exercise, as appropriate
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teaching breathing exercises and assisted coughing
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advice regarding pressure care and provision of pressure relieving seating/mattress
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management of spasticity. prevention of contractures
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position for comfort
Late stage
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The patient uses a wheelchair when out of bed or remains in
bed
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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.
A suction machine maybe required at this stage. Carers may be
shown how to use suction to keep the patient's mouth clear of
secretions but only experienced health professionals should
attempt deep suction,