1. Motor neuron disease

2. 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.

3. ALS Demographics Incidence 2 per 100,000 Male slightlymorethan Female
Peak age of onset: 6th decade (range 20 to 90)

4. 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)

5. cause No known cause Possible cause could be Mitochondrial dysfunction
Gene defects
Cytoskeletal protein defects
Autoimmune dysfunction/ Inflammatory Damage
Virus

6. 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

7. Clinical features (65% cases)affects the hands first
fasciculation is seen on examination

8. 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.

9. Clinical features
Weakness of muscles controlling facial expression, the tongue, the palate
ability to cough effectively and clear secretions is lost.

10. ALS: Prognosis
Poor prognosis if:
Bulbar onset
Older age at diagnosis

11. 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

12. 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

13. Early stage 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

14. 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

15. Middle stage soft collar provision
active, assisted active, passive and /or aerobic exercise, as appropriate

16. 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

17. 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.