2.9 Neurological and Sensory ALS

Question 1

ALS
Amyotrophic lateral sclerosis
pathophysiology

Answer 1

pathophysiology

Progressive neurodegeneration

Degeneration/death of upper and lower motor neurons in brain & spinal cord

Begins as a focal process then spreads to impact all levels of the motor system

Weakness, wasting, and paralysis of the muscles of the limbs and trunk controlling voluntary movement

Most sporadic (no known cause) but 5-10% familial.

Question 2

ALS
Amyotrophic lateral sclerosis
Risk factors

Answer 2

Risk factors:

Most people develop age 40-70.
Men somewhat more likely
Smoking, environmental toxins
Veterans twice as likely to be diagnosed.

Question 3

ALS
Amyotrophic lateral sclerosis
stats

Answer 3

How fast and in what order this occurs is very different from person to person. While the average survival time is three years, about 20 percent of people with ALS live five years, 10 percent will survive 10 years and 5 percent will live 20 years or longer.

Question 4

ALS
Amyotrophic lateral sclerosis
manifestations

Answer 4

manifestations

Gradual onset –initial symptoms vary and can be subtle progression of painless muscle weakness

S/S: tripping, dropping things, fatigue of extremities, slurred speech, muscle cramps, fasciculations (twitching), uncontrollable periods of laughing/crying

Attacks only motor neurons so sight, hearing etc not generally affected

Variable rate of progression but average survival is less than 5yrs.

Progressive loss of voluntary motor functions, muscle wasting, and paralysis

Loss of respirations and will need permanent ventilatory support

Loss of speech

Loss of ability to swallow and will need enteral feedings

Cognitive decline possible but not common

Question 5

ALS
Amyotrophic lateral sclerosis
diagnosis

Answer 5

diagnosis

Difficult to diagnose – no specific test and involves ruling out other neuro diseases

Diagnostic procedures to rule out other causes:
Electromyography and nerve conduction tests
Blood and urine tests, thyroid/parathyroid hormone levels, heavy metals
Lumbar puncture
MRIs
Muscle/nerve biopsy

Question 6

ALS
Amyotrophic lateral sclerosis
treatment

Answer 6

treatment

Supportive care

Medication
riluzole (Rilutek): reduce damage to motor neurons by decreasing levels of neurotransmitter glutamate, which transports messages between nerve cells and motor neuron
edaravone (Radicava):decreases oxidative stress & slows progression of disease

Symptom management 
Therapy: PT, ST, OT
Nutritional support
Respiratory support: ventilator
End of life care planning

Question 7

ALS
Amyotrophic lateral sclerosis
Nursing Care

Answer 7

Nursing Care
Care planning – resources (ALS.org), home care needs,
Impaired mobility- skin checks, muscle weakness, injury prevention, constipation

Nutrition – frequent dehydration & inadequate calories due to swallowing deficits

Dysphagia – risk of aspiration, inadequate intake

Respiratory – risk of PNA, impaired oxygenation, mechanical life supports

Impaired communication – dysarthria, volume loss, and eventual loss of speech

Coping

Grieving