1. AMYOTROPHIC LATERAL SCLEROSIS
NEURO CONDITIONS 3
AMYOTROPHIC LATERAL SCLEROSIS
AND
GUILLAIN-BARRE
Jane E. Binetti RN MSN

2. Amyotrophic Lateral Sclerosis
“Lou Gehrig's Disease”
Progressive neurological disorder
Pts die within 2-6 yrs of diagnosis
Onset:
Typically years old
Men more than women by 2:1
5000 new cases diagnosed each year in the US
There is no cure

3. What happens? Motor neurons in the brain and brainstem degenerate Sx:
No signal is brought to the muscles
No chemical or electrical transmission
Sx:
Weakness in upper and lower extremities
Dysarthria
Dysphagia, drooling
Muscle wasting, pain, spasticity

4. Diagnosis No specific tests Thorough H and P Tracking of symptoms
Some tests help rule out other diseases:
EMG
MRI
Antibody testing

5. Treatment There is very little that helps ALS
We provide comfort and symptomatic relief
Riluzole (Rilutek) slows the progression of disease in most cases
As the illness progresses, the patient is cognitively intact

6. What do you do? Care is supportive:
Assess for respiratory insufficiency
Risk of aspiration
Pain Control
Reduce safety risks
Find ways to continue to communicate
Support family through care and grieving
Remember the patient’s mind is intact!!!

7. GUILLAIN BARRE Acute polyneuropathic syndrome
Rapidly progressive, potentially fatal
Typically ascending in progression
Affects both peripheral and cranial nerves
More male than female
More common in adults but is seen in all ages
% of patients recover

8. What happens? Cause is unclear
It is due to a cellular and humoral response
Segmental demyelination
Transmission slows and then stops
Remyelination takes time, and happens in reverse
Triggers:
Viral infections, viral immunizations, HIV, trauma
Camphylobacter jejuni 30% of cases
CMV, mycoplasma pna, Epstien Barr

9. What do you see? Symptoms can range from mild to severe
Commonly onset ~ 3 weeks after URI or GI infection
Weakness of lower extremities
Parasthesia, pain
Hypotonia, areflexia
Autonomic nervous dysfunction
Respiratory muscle paralysis, orthostatic hypotension, HTN
Vagal stimulation – brady, HeartBlocks, asystole
Bowel and bladder
SIADH

10. Complications Respiratory failure – mechanical ventilation Immobility
Fever
Ileus
DVT and possible PE
Orthostatic hypotension
Compromised nutrition

11. Diagnostics H and P CSF samples EMG
Initially low in protein but eventually high
EMG
abnormal

12. Collaborative Care Ventilation support Plasmaphoresis
IV immunoglobin therapy
Tube feedings
NGT for ileus

13. Nursing Diagnoses Impaired Spontaneous Ventilation Acute Pain
Impaired Verbal Communication
Fear
Self Care Deficit