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

Amyotrophic Lateral Sclerosis “Lou Gehrig's Disease” Progressive neurological disorder Pts die within 2-6 yrs of diagnosis Onset: Typically 40-70 years old Men more than women by 2:1 5000 new cases diagnosed each year in the US There is no cure

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

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

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

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!!!

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 85 - 95% of patients recover

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

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

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

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

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

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