Amyotrophic Lateral sclerosis (ALS)

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What differential tests are done?

-blood tests -physical exam -Genetic testing -medical history -MRI scan -Nerve conduction test -swallowing studies

What are the signs and symptoms of ALS?

-cramps -fasiculations -dysphagia -weakness and paralysis progressive throughout body -breathing difficulties -Dysarthria -loss of upper motor neurons -damage to lower motor neurons -LOB and falls

What are the first areas of difficulty usually noticed in ALS?

-hands during fine motor tasks -feet while walking

What is the 1st stage of ALS?

-independent with ADLs -minor limitations and some pain may be present -decreased endurance

What is the 2nd stage of ALS?

-muscle imbalance -decreased mobility and function -increased muscle fatigue due to using more energy to perform ADLs

What physical tests are done to diagnose ALS?

-neurological exam -pulmonary funciton -motor control and movement

What is currently used to help manage ALS?

-program of exercise and rest -respiratory support -medication

What is the 3rd stage of ALS?

-progressive weakness in trunk -greater loss of mobility and function -wheelchair for distance -assistance for many ADLs

What is the affect of Riluzole?

-reduce motor neuron damage -decrease glutamate release -reduces the damage and death of nerve cells

What is the 4th stage of ALS?

-totally dependent for ADLs -ventilator support may be necessary -swallowing is difficult or impossible

What are the theories as to the cause for the 90% of ALS cases not due to genetics?

-toxic reaction to build up of glutamate in nerve cell synapse -autoimmune disorder -Hormone abnormality -enzyme deficiency

What is the effects of spinal (progressive) muscular atrophy?

-weakness -atrophy -loss of reflexes -fasciculation

What kind of adaptive equipment is used by people with ALS?

-wheelchair and equipment consultation -AAC

What types of therapeutic interventions are done for people with ALS?

-work simplification -energy conservation -adaptive equipment -swallow intervention -environmental adaptations -compensatory techniques -caregiver education

What are the two types of ALS?

1) spinal (progressive) muscular atrophy 2) progressive bulbar palsy

How many cases of ALS generally exist in the US at any given time?

30,000

What is the age that ALS affects?

40-70 y/o

How many cases of ALS are diagnosed each year in the US?

5,600

When is ALS usually diagnosed?

55 y/o

How many people are diagnosed with ALS

6-8 per 100,000

What surgery is done for people with ALS

Gastrostomy

How is ALS diagnosed?

No specific test-- differential diagnosis

What is the first FDA approved drug for ALS?

Riluzole (Rilutek)

What is a fasciculation?

Small local muscle contraction (twitch)

What type of therapy is currently being tested for effectiveness?

Stem cell therapy

What type of equipment is usually used to support individuals with ALS with breathing difficulties?

Ventilator

What is ALS?

a motor neuron disease that attacks the motor neurons located in the brain, brainstem, and spinal cord

Why is an MRI scan done?

allows view of the brain and spinal cord to help determine if it's something other than ALS causing the signs and symptoms

What is the affect of ALS on cognitive function?

cognitive function stays intact

What does sclerosis mean?

degenerative "hardening"

What is dysarthria?

difficult of unclear articulation of speech that is otherwise linguistically normal

What is dysphagia?

difficulty or discomfort swallowing

When are the most benefits seen with Riluzole?

early in treatment

What is the outcome of damage to lower motor neurons?

flaccid paralysis with decreased muscle tone and reflexes

What is the life expectancy for people with ALS?

generally 3-5 years, but people can live up to 10 years

What is generally the outcome of dysarthria for people with ALS?

inability to communicate verbally

What is the affect of progressive bulbar palsy?

loss of muscles innervated by cranial nerves

What motor neuron is affected in spinal (progressive) muscular atrophy?

lower motor neurons

What is the main goal of intervention for people with ALS?

maintaining optimal level of function

Why is a gastrostomy done for people with ALS?

may be necessary to reduce choking/aspiration in clients experiencing swallowing difficulties

What is the amount of men v women diagnosed with ALS?

men= 60% women= 40%

What does amyotrophic mean?

muscle wasting

what are the first symptoms of ALS?

muscle weakness and atrophy?

ALS causes the death of what kind of cell?

nerve cells controlling voluntary muscles of body

What is the onset of ALS?

no clear onset-- the disease affects people at different rates and can have subtle beginning

Is there a treatment for ALS?

no specific treatment to slow the progression

what is the result of cell death caused by ALS?

paralysis and ultimately death

What is the prognosis of ALS?

progressive and degenerative

What parts of brain function are generally spared in ALS?

sensory systems, cognition, and urinary sphincters

What is the outcome of loss of upper motor neurons?

spastic paralysis and hyper-reflexion

What is a gastrostomy?

surgical opening into the stomach

How do the symptoms of ALS progress?

symmetrically

What is a differential diagnosis?

tests are completed to rule out other diseases that may be the cause

what is the cause of ALS?

the exact cause is unknown

Why is a medical history taken?

to determine if symptoms are caused by prior conditions

Why is a nerve conduction test done?

to rule out peripheral nerve damage or muscle disease

Why is genetic testing done?

to see if there is a family history of ALS

How are therapeutic interventions decided for people with ALS?

treatment is based on symptoms

What motor neuron is affected in progressive bulbar palsy?

upper motor neuron involvement

Why is an EMG done?

used as a recording technique used to detect electrical activity in the muscle

When is respiratory support done?

usually within 2-5 years

How many of cases are caused by being inherited on a dominate gene?

~10%


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