ALS & Guillain Barre (Exam 2) Flashcards
(19 cards)
ALS stands for
Amyotrophic Lateral Sclerosis
“Lou Gehrig Disease”
ALS Definition
A rare, progressive neuro disorder characterized by loss of motor neurons
Progressive muscle weakness
What neurons are loss in ALS
Upper and Lower motor neurons
ALS survival?
Death usually occurs around 3 years after diagnosis
r/t respiratory failure
ALS: Risk Factors
Age: 40-70
Gender: Male
Genetics
Smoking
ALS: Pathogenesis
Degeneration of upper and lower motor neurons
Neurons in the brainstem and spinal cord degenerate
Electrical and chemical messages from the brain do not reach the muscles
ALS: What could cause it?
“Excitotoxicity Hypothesis”
-excessive levels of glutamate initiate a cascade of events that lead to neuron death
-glutamate = excitatory neurotransmitter
What is glutamate?
Excitatory neurotransmitter thought to be impactful in the neuron death r/t ALS
Why do we think glutamate plays a role in ALS?
Because elevated glutamate levels have been found in the CSF of people with ALS
What drug reduces glutamate and can increase survival?
Riluzole
Antiglutamatergic drug
ALS: Clinical Manifestations
-Weakness of upper extremities (muscle wasting, spasticity)
-Dysarthria, dysphagia, drooling
-Cognitive and behavioral changes
-Constipation
-Sleep problems
-Breathing problems (most common cause of death)
What is guillain-barre syndrome?
Rare autoimmune disorder where the myelin sheath is damaged by AUTOANTIBODIES
What does the mylin sheeth do?
Covers axons that carry nerve impulses away from the body.
Myelin helps them travel faster
What is the Onset of GBS?
Days to Weeks following a VIRAL infection
GBS: Comes From?
Viral Infection (resp/GI)
Bacterial Infection
(campylobacter jejuni)
Post Surgery (5-10%)
FLU-Vaccines
GBS: Disease Process
-Weakness/tingling in LOWER extremities
-This ascends up the body (recovery = opposite = top down)
-Severity of symptoms increases over hours or weeks (fast or slow) (may be intubated quickly)
-Potentially life-threatening if respiratory muscles are involved
-May require mechanical ventilation
GBS: Clinical Manifestations
-Weakness going up the body
-Uncoordinated movements
-Numbness and decreased sensation
-Loss of bowel/bladder control
-Blurred vision
-Difficulty, breathing, swallowing, chewing
GBS: Pharmacotherapy Goal
-NO cure
Goal = Reduce severity and accelerate recovery
Steroid therapy + High dose Immunoglobulin therapy
GBS: Prognosis
95% survive
75% completely recover
25% have residual weakness after 3 years
3% may suffer relapse of muscle weakness