chapter 32 Flashcards
huntingtons
Autosomal dominant
Progressive neurological disorder
Involuntary motor symptoms, cognitive decline, and emotional and behavioral symptoms
Symptoms appear between ages 30 to 50, with progression for 10 to 30 years
Gene mutation on chromosome 4, disrupt the protein called huntingtin
Common clinical manifestations
Involuntary motor symptoms
Dyskinesia
Chorea: brief, dancelike movements
Athetosis: twisting and writhing
Emotional and behavioral symptoms
Major depressive disorder
Cognitive symptoms
Apathy, slowed thought processes
Diagnosis
Genetic testing
Treatment
Symptom management
Antipsychotic medications
Help with movement problems
Antidepressants
epilepsy treatment
Antiepileptic agents:
***Phenytoin, valproic acid, carbamazepine, etc.
Modify ion channels or neurotransmitters in brain
Decrease seizure risk while limiting adverse side effects of medications
myasthenia gravis
Autoimmune disease
Attacks connection between nerves & muscles, neuromuscular junction
Diagnosis
Ach receptor (AChR) antibodies
Muscle weakness, worsened by activity and fatigue
Commonly, the extraocular muscles often affected first; ptosis (eye lid lag)
B-cell and T-cell mediated
Thymus may be involved in this disease
Approximately 70% of patients with MG have thymus gland hyperplasia (enlargement or increased reproduction rate of its cells)
Presents in two ways
Ocular form: often patients first present with ocular changes. Muscles that move the eyes & control the eyelids are easily fatigued & weakened; vision affected
General form: face & throat muscles, chewing & swallowing, impaired speaking, neck & limb weakness
True muscle fatigue
Not a sense of tiredness
Decreased contractile force
Occurs with repetitive motions
Blinking, walking, talking, chewing
Swallowing and respiratory function may be affected
Monitor the forced vital capacity & negative respiratory force
Rest improves muscle function
**Forced vital capacity is the max amount of air that can be forcibly exhaled from the lungs after fully inhaling. Normal is 4.8 liters
**Negative inspiratory force is the measurement of respiratory muscle strength & ventilator reserve
Diagnosis
Edrophonium (Tensilon®) testing
Acetylcholinesterase inhibitor
Allows more Ach to remain in the synapses for a longer period of time
30–45 seconds after administration, improved muscle contraction
Serum Ach receptor antibodies
Electromyography (EMG) assesses the health of muscles & the nerve sells that control them
Physostigmine (fai-suh-stig-meen)
Acetylcholinesterase inhibitor
Other treatments
Intravenous immune globulin
Plasmapheresis – unhealthy plasma is swapped for healthy plasma or plasma substitute, before the blood is returned to the body
Thymectomy, removal of the thymus, may be recommended
***The respiratory rate of a client w/MG should be monitored closely
guillen barre syndreome
Post-infectious disease
Acute inflammatory demyelination
Weakness varies from mild weakness to complete paralysis, including respiratory muscles
Recovery is often steady over weeks and months
Treatment
Supportive as life-threatening sequelae may develop
Immunomodulating agents: substances that stimulate or suppress the immune system & may help fight cancer, infection, or other diseases.
The agents may include cytokines, vaccines etc
