Class 16: ALS and GBS

ALS is also known as

Lou Gehrigs disease

ALS 

Loss of motor neurons in spinal cord and brain stem

Atrophy of muscles in hands and feet, and other parts of body as it progresses 

ALS cause

Unknown

Combination of genetic component and environmental factors

ALS average survival after diagnosis

3-5 years

ALS respiratory effects

Main cause of death in patients with ALS

Eventually diaphragm stops working aand can no longer breathe

Earliest signs of ALS

Tripping and dropping things 

May write it off as being clumsy/tired, so people often get diagnosed late 

ALS signs and symptoms

Hard to doagnose bc very general, Depends on location of affected motor neuron:

Tripping and dropping things

Slurred speech

Difficulty swallowing 

Weight loss and decreased muscle tone 

SOB

Increased or decreased reflexes 

Uncontrollable periods of laughing or crying 

Weakness, fatigue

Muscle cramping, twitching, stiffness or rigidity 

ALS diagnosis

Clinical symptoms

No definitive lab tests, ruling out other diagnoses 

MRI and electromyography (looks at muscle and nerve cells to check conduction)

Muscle and nerve function tests 

ALS management

Emotional support

Supportive measures that address symptoms 

Prevent skin breakdown (pressure devices and turning)

Nutrition (trying to maintain weight)

Prevent aspiration (elevate HOB, thickened fluids)

Exercise to prevent spasticity 

Facilitate communication 

End of life decisions 

Asstive devices 

Pain management (not common but help if present)

Mechanical ventilation 

Medication

ALS medication

Riluzole (Rilutek)

Helps protects nerves to slow disease progression

Not a cure 

ALS end of life discussion

Terminal disease, so need to have hard discussions

Need to figure out before disease progresses

Do you want a ventilator/trach? Help when you can no longer feed yourself? etc

ALS communication

“locked in”: body deteriorates but mind is unaffected

Need to get creative to communicate (pointing, blinking for yes/no/letters etc)

ALS early recognition of respiratory insufficiency

Very important!

ALS suctioning

Risk for aspiration, so have suction ready

may suction themselves if still able 

ALS falls

High fall risk

Ensure home is safe to reduce risk of fall related injury

ALS activities

Diversional activities

Helps them cope/be happy

Find acitivies they enjoy and are still able to do 

GBS 

Autoimmune attack on peripheral nervous system

What causes GBS

Illness (most often a a recent viral infection)

How common is it for GBS patients to regain function?

80% regain most or all of their function

GBS signs and symptoms

1. Symmetrical muscle weakness

2. Distal parenthesia-feet first, then hands 

3. Absent or depressed deep tendon reflexes 

4. Ascending weakness-starting in lower limbs, upper extremities, then face and respiratory muscles 

5. May progress to complete paralysis 

6. Pain 

7. Respiratory failure 

Major complication of GBS

Respiratory failure

Often need ventilator and go to ICU

Key signs of GBS

1. Recent viral infection

2. Notice symptoms quickly (hours to days)

3. Muscle weakness starting in the lower limbs and gradually moving up 

4. Nerve pain-shooting, stabbing, burning

5. Respiratory failure  

GBS diagnostic studies 

1. History and physical:

Recent infection, muscle weakness on both sides starting in legs and feet

2. CSF-protein (supports diagnosis but not definitive)

3. EMG and nerve conduction studies (looking at muscle and nerve cell impulses)

Whatis an extremely rare cause of GBS

Vaccines

GBS Supportive care

Cant completely stop, but want to slow progression and symptoms:

Ventilation

Prevent DVT, PE and skin breakdown

Feeding tube bc aspiration risk

GBS med 

IVIG

How does IVIG help GBS

Not a cure, helps suppress/stop progression

Boosts immune system

GBS corneal reflexes

Diminshed

May need eye drops and eye care bc cant blink

Why would someone with GBS get an ECG

Respiratory issues can cause dysryhthmias 

GBS urinary symptoms

May have urinary retention

May need foley or in and out catheter

GBS nutrition

Aspiration risk

May need tube feed

Monitor electrolytes

GBS communication

Alert and oriented, but may be unable to speak 

Facilitate communication using aids, blinking etc