Amyotrophic Lateral Sclerosis

What are motor neuron diseases

inherited or sporadic disorders than impact the UMN and LMNs

What is the most common motor neuron disease?

ALS

What are the main characteristics of ALS

degeneration and loss of motor neurons in the spinal cord, brainstem and brain leading to both UMN and LMN signs

What is primary lateral scleorsis

degeneration of UMN

What is progressive bulbar palsy

degeneration of motor neurons from CN 9 to 11

What is progressive muscular atrophy

loss of motor neurons of the spinal cord and brainstem

Is ALS a family disorder

most are sporadic

What is the typical onset of ALS

51-66 years

What is the typical symptoms at onset of ALS

limb onset which indicates spinal cord ALS with extremenity movement impaired

What demographic is more likely to have ALS?

males

What is the cause of ALS

genetic and enviornmental factors

linked to trauma and exposure to lead and mercury

Does ALS only impact nerves and muscles

no it has been found in nonmotor areas

What nervous system areas does ALS impact?

primarily the anterior horn

can lead to ANS, basal ganglia, cerebellum, oculomotor and sensory systems

What is reinnervation

new connections made from healthy nerves to compensate for denervations

Muscle involunment progression with ALS

starts with LE, UE then the bulbar

What ADL activities may indicate ALS

difficulty with fine movements with buttoning, pinching and writing

Gait deviations with ALS

foot slap or trigging

What is the cardinal sign of ALS

WEAKNESS

How long does it take for a diagnosis from onset of symptoms

8 to 16 months; shorter with bulbar-onset

What is the prognosis with ALS

50% after 3 years unless mechanical ventilator is used

What type of onset leads to a greater prognosis

limb

Medications for ALS

Riluzole as a glutamate inhibitor

Racicava as a free radical scavenger

How can you treat cervical weakness

head up collar

head master

aspen

What should avoided with swallowing?

no neck extension due to aspiration rest

Does respiratory muscle training imporve symptoms

no it does not

How can they manage oral secretions

oral suction device

What is an issue with getting assistive devices for patients with ALS

their condition changes so much by the time they get next equipment

What exercise intensity is allowed with ALS

light to moderate

Does exercise return strength to muscles

no if they are already below a 3/5

Why is it so hard to study ALS

they dead before the study even starts