ALS

What is ALS short for, what is it also called and what is it?

Amyotrophic Lateral Sclerosis (AKA Lou Gehrig's disease) and it is a progressive neuromuscular disorder

Why can health providers only manage symptoms for diseases like ALS and MS?

Because there is no cure

How is ALS diagnosed?

By ruling out everything else like electrolyte imbalances, infections, EMG, MRI to ensure no structual issues, stenosis or tumors, nerve condition velocity test to rule out peripheral neuropathies and myopathy.

Describe how physical function symptoms occur in the early stage of ALS?

Can be in one area of the body or more than one. Usually progresses to adjacent area - arms first.

What physical symptoms start to present in the middle stage of ALS?

Contractures, dysphagia, choking, respiratory muscle weakness and pseudobulbar effect

If ALS causes upper motor neuron symptoms, what part of the CNS is involved?

Cortex. Hint: Upper - for the tippy top of the brain

What are general symptoms that occur in the late stage of ALS? 7

Decreased motility, confusion due to hypoxia, respiratory muscle paralysis, dysphagia, bowel and bladder issues.

Name 3 characteristics of the drug riluzole

Delays need for ventilator support by 3-6 months, It is a glutamate agonist and it works for pts whose nerve involvement began in the medulla not spinal cord - so for people whose ALS started higher above the spinal cord.

If ALS causes lower motor neuron symptoms, what part of the CNS is involved?

Everything from the brain stem down

Describe general symptoms of Early ALS. 4

Fatigue, balance issues, gait and slurred speech

Do general symptoms of ALS have a slow onset or do they progess quickly?

General ALS symptoms have a slow onset

In ALS, what kind of muscles are NOT affected?

Involuntary muscles

What are the initial symptoms of ALS?

It depends on what neurons are involved but usually starts on one side and progresses.

Define areflexia

Lack of spinal reflexes

Define the pseudobulbar affect

Loss of control over emotional expression. Similar to labile.

What is the pathophysiology of ALS? 4 steps

Loss of motor neurons in the anterior horns of spinal cord and motor nuclei of the lower brain stem which leads to motor nerve cell death which causes muscle atrophy which causes weakness and dysfunction.

What sex is more prone to getting ALS and what is the average age onset?

Males are at greater risk for ALS and age of onset is 40 -60 y.o.

What are the 4 goals for caring for someone with ALS?

Manage symptoms, Maintain quality of life, Caregiver/family support and collaborative treatment.

What does Nuedexta (dextromethrophan hydrobromide + quinidine sulfate) combat symptoms of?

Management of pseudobulbar affect by acting on the CNS in some unknown way

What happens with muscle symptoms in the middle stage of ALS?

More widespread involvement

Define dysarthria

Motor speech disorder - the muscles of the mouth, face, and respiratory system become weak.

There are 10 general symptoms of ALS when lower motor neurons are involved, what are they?

Muscle weakness, stiffness, cramping, fatigue, dysarthria, hoarseness, areflexia, hypotonia, fasciculations including tongue and atonia

How can a nurse be supportive and preventive when caring for a patient with ALS? 3 main

Nutrition - make sure patient is getting adequate amounts - may need a G-tube. Skin Care - Make sure patient is properly positioned and that bed and equipment are not causing pressure ulcers. Respiratory - maintain patency of airway - ventilator may be required.

When discussing collaborative treatment for a patient with ALS, what should be discussed? 2 main topics.

Palliative care - relieve symptoms, pain and stress. End-of-Life decisions - heroic meausres? DNR? Patient needs to know they are not going to get better and all family members need to be on the same page.

What does frontal lobe dementia look like?

Problems with planning, decision making and executive function but no memory loss

What is the most common cause of death in patients with ALS?

Respiratory complications due to respiratory muscle weakness and paralysis.

There are 10 general symptoms of ALS when Upper motor neurons are involved, what are they?

Spasticity, changes in mood/behavior/personality, Sialorrhea, increased muscle tone, Frontal lobe dementia, depression in 90% of the patients, pseudobulbar affect

The etiology of ALS is unknown but what is the main theory?

That over-excitement of the nerve cells by the NT glutamate causes nerve cell injury and neuronal degeneration

What are the muscular symptoms in the early stage of ALS? 6

The symptom starts in one area of the body and then spreads - weakness, cramping, atrophy, fasciculations and aches

What neurons does ALS affect?

Upper and lower motor neuron involvement

What happens to the muscles in the late stage of ALS?

Voluntary muscle paralysis

Is ALS fatal, if so how long does one have to live after diagnosis?

Yes, 3-5 years

Name 7 side effects of riluzole

asthenia, N/V/D, Dizziness, vertigo, somnolence, decreased respiratory function and hepatotoxic.

Define Sialorrhea

excess salivation and drooling

Define Asthenia

lack or loss of strength

Define fasciculations

muscle twitching

Define atonia

muscle wasting

Name 2 drugs that help relieve symptoms for ALS.

riluzole and Nuedexta (Combo med of dextromethrophan hydrobromide and quinidine sulfate)