Amyotrophic lateral sclerosis (als)

ALS

1. AMYOTROPHIC LATERAL SCLEROSIS (ALS)

2. What is ALS? Progressive neurodegenerative disease in which the motor neurons gradually degenerates and after some time, they eventually die.

3. Upper Motor Neuron - evolving from layer five of motor cortex to descend via the pyramidal tract to synapse with motor neuron Lower Motor Neuron - consisting of anterior horn cells in spinal cord and their brainstem homologues innervating bulbar muscle . L oss of function in: * Although at its onset, ALS may involve selective loss of function of only upper or lower motor neurons, it ultimately causes progressive loos of both categories of motor neurons.

4. MOTOR NEURONS Motor neuron is a type of nerve that can be found in the spinal cord, brain, and brain stem. It provides a connection between voluntary muscle of the body and nervous system

5. Causes In ALS, MN get affected and so messages cannot be initiated or transferred to the muscles, this means movement will not be supported in the muscles leads to weakness of the muscles and they also get thinner. -As the condition reaches its later stages, more and more MN are damaged and more voluntary muscles fail to function then a person will be unable to move their limbs and body eventually leads to complete paralysis

6. PATHOPHYSIOLOGY Familial – 5-10% Sporadic – 90-95%

7. PATHOPHYSIOLOGY It is unknown why the motor neuron degenerate in ALS but researchers suggested certain possibilities which are: EXCITOTOXICITY GENE MUTATION

8. PATHOPHYSIOLOGY EXCITOTOXICITY – Excitotoxic neurotransmitters such as GLUTAMATE

9. PATHOPHYSIOLOGY GENE MUTATION – SOD1 (superoxide dismutase) is mutated in some familial cases of ALS. SOD1 which is a cellular defense against excitotoxicity which detoxifies free radicals.

10. Summary of pathophysiology

11. !!! The denervation of the nerves now causes the clinical manifestations

12. Clinical manifestations Early stage Dysphagia (bulbar onset) Dysarthria (bulbar onset) Emotional lability (bulbar onset) Spasticity (limb’s onset) Fasciculation's (limb’s onset) Cramps (limb’s onset) Muscle weakness ( limb’s onset) Atrophy (limb’s onset)

13. Progression Dyspnea Loose the ability to walk, or use hands and arms Loose the ability to speak, and swallow

14. Late stage Dementia Respiratory failure Aspiration pneumonia Occulomotor nerve of extra ocular muscle is affected May resemble locked in syndrome

15. TREATMENT NO curative agent RILUZOLE- the only drug approved by the FDA

16. THERAPY Physical Therapy -involves exercises Speech Therapy -text -to-speech applications

17. Dental health for ALS patients

18. Why keep the mouth clean? -Maintain comfort -Reduce halitosis -Reduce risk for gum disease, which can further endanger your health -Reduce the risk of pneumonia (infection in the lungs from bacteria in the mouth ) -Help manage saliva which can reduce drooling or stimulate saliva flow

19. Physical changes that may occur with ALS might make it difficult to keep the mouth clean. These include 1 . Changes in hand/arm function 2. Changes in function of the mouth, lips, cheeks, and tongue 3 . Changes in function of the swallowing muscles 4 . Changes in mobility 5 . Changes in breathing

20. Dental hygiene -Brush w/ soft bristle - floss -use tools that may help

21. During Dental Procedure -use bite block -semi supine position -use strong suction -have a short break

22. Maintenance -get regular check ups -nutritional counseling