neuro (class 9) degenerative diseases Flashcards
dementia
cognitive decline caused by any disorder that permanently damages areas of the brain necessary for memory & learning.
progressive impairment of cognitive function, personality & behavior.
um brella term for disorders that result in neuron death or loss of communication between cells.
dementia risk factors
age, family history, smoking/ETOH use, ethnicity, diseases: high cholesterol, atherosclerosis, DM, down’s syndrome, TBI.
various types of dementia
Alzheimer's disease vascular dementia lewy body dementia fronto-temporal dementia mixed dementia reversible causes
Alzeimer’s disease
pathophysiology
cause unknown-interaction of multiple factors.
brain changes: loss of nerve cells & vascular supply causing brain atrophy.
neurotic plaques
group of degenerated cells that clump around amyloid core begin in areas for cognition & memory, disrupt transmission of nerve impulses
tangles
twisted fibers of tau protein that build up cytoplasm of affected neurons
progressive alzheimers
more areas of the brain over time- loss of neurons/neurotransmitters with enlargement of ventricles & structural changes.
alzheimer’s manifestations
dementia often categorized as mild-moderate-severe.
7 stage classification systme.
stages 1-2 mild
trouble finding words, decrease memory & planning, lose objects, social & work problems, stage 3 may become noticable to others & measurable in exams.
stages 4&5 moderate
ST memory loss, loss of mental functions, can’t perform complex tasks, become withdrawn, may need ADL assist, poor recall, disorientation to place, time, context
stages 6&7 severe
worsening memory, personality changes, lose context, need supervision, sleep-wake cycle disruptions, wandering, personality changes, suspicious, in final stage lose ability to respond to environment, speech, & purposeful movement, decrease reflexes, muscle rigidity, swallowing impairments, vision loss, malnutrition & aspiration are frequent complications.
alzheimers diagnosis
no single specific test to R/O things that mimic.
medical & medication hx. unintentional wt loss. hx of behavior patterns over time, lab work. physical exam. neuro exams, mood evaluation, cognitive testing. brain scans: CT, MRIs
treatment meds
meds for memory- slow progression.
acetylcholinesterase inhibitors-donepezil.
prevents breakdown of ACH=enhanced neuron activity
NMDA receptor antagonist-memantine.
reduces glutamate & blocks excessive Ca entry into cells =prevent cell death
meds for behaviors-antidepressants, anxiolytics, antipsychotics.
meds for sleep changes
treatment: non-med strategies
reminiscence, validation therapy, cueing, reality orientation, manage aggression: space, low stimuli environment, distraction, alternative activities, touch-caution.
dementia
group of cognitive disorders could have problems with memory, judgement, using and understanding language and motor function. result in death of neurons. gradual onset, slow decline, no cure
delirium
caused by underlying medical problem.
occurs abruptly
symptoms fluctuate throughout day.
more profound state of confusion, delusions, and agitation.
other medical problem is identified and treated
confusion
mild form of delirium
acute change in mental status
abnormal & fluctuation change in attention.
may give warning of development of more severe disorder.
creutzfeldt-jakob disease
cause
prion disorder(protein) cns disease, effects dependent on where proteins are active. familial, sporadic, or infections causes buildup of abnormal prion proteins. 4+ yr incubation
creutzfeldt-jakob disease symptoms
rapid progression (wks to months). behavior & personality changes. CNS: memory, vision loss, dysphagia, abnormal movement.
creutzfeldt-jakob disease diagnosis
can only by confirmed by autopsy
creutzfeldt-jakob disease treatment
supportive only
parkinsons disease pathophysiology
chronic & gradually progressive, classes as movement disorder but sensory s/s.
risk factors: age, exposure to toxins, head injury, sex.
neurotransmitter problem: death of dopaminergic neurons in substantia nigra.
parkinsons disease manifestations- movement
bradykinesia-slow movement. rigidity-stiffness cogwheel rigidity. tremors- resting. postural instability additional motor manifestations: stooped posture & gait disturbances-no arm swing, shuffling, festinating, dyskinesia, dystonia, freezing, facial masking, swallowing problems, drooling, micrographia, dysphonia.
parkinsons disease manifestations- nonmovement
cognitive changes: attention, planning, language, memory, dementia, constipation, fatigue, loss of taste or smell, urinary urgency, frequency, sleep problems, mood disorders: depression, anxiety, paathy, irritability. early satiety, wt loss, orthostatic hypotension, dizziness.
parkinsons disease diagnosis
no specific test- must have 2 of the 4 symptoms.
neurologist movement disorders specialist- challenging dx, pts have variable symptoms.
R/O other conditions that mimic.
tests: ct scan mri, datscan SPECT: shows density of dopamine transporter, qualatative.
PET SCAN
parkinsons disease treatment
no cure, meds & treatments control symptoms, eventually not effective.
medications: dopamine replacement: levodopa/carbidopa
dopamine agonists= stimulate areas of brain where dopamine works.
Anticholinergics- treat tremor by blocking acetycholine.
MAO inhibitors- prevent breakdown of dopamine
medical marijuanna.
surgery-deep brain stimulator.
therapy.
multiple sclerosis
autoimmune= B cells invade/stimulate inflammation & scarring.
central nervous system.
destruction of myelin sheath.
motor & sensory nerves are impacted.
lesions happen in different areas so clinical s/s individualized.
MS manifestations
fatigue is very common. visual impairments, nystagmus.
sensory: pain, paresthesia, numbness, balance impairments (+rombergs)
cognitive: memory loss, judgement/planning, concentration impaired.
mood: unstable emotions, depression, anxiety.
motor: stiff, slow, weakness, spastic paresis, impaired gait.
bladder: urgency, retention, incontinence.
bowel: constipation, incontinence
sexual dysfunction.
MS diagnosis
process- no specific test.
medical history- exacerbations of symptoms followed by remission
neuro exam: cranial nerves, coordination, strength, reflexes, sensation
MRI: lesions, atrophy.
lumbar puncture: looking for elevate immune proteins/
evoked potential studies: apply stimuli & measure how long it takes to reach the brain.
MS treatment
goal to arrest progress early- remyelination can occur if damage isn’t too deep.
MS medications
disease-modifying therapies: decrease lesions, decrease frequency & severity of relapse, slow progression.
corticosteroids decrease inflammation, immunsuppressants decrease immune response, interferon decreases immune response, glatiramer acetate stimulates myelin proteins.
fingolimod traps immune cells in the lymph nodes.
MS symptom management
fatigue-amanatadine, modafinial.
bladder-anticholinergics for OAB, cholinergic for retention, spasticity-muscle relaxants, benzodiaepine.
bowel-stool softeners, laxatives.
tremors-beta blockers.
MS treatment
surgery- tendon release, placement of intrathercal infusion pump for baclofen.
lifestyle: diet nutrition various diets in the research, supplements: vitamin D, probitoics, safety concerns- problems with chewing and swalling.
sleep
activity
identify triggers: stress fatigue infection smoking med changes heat
amyotrophic lateral sclerosis
progressive neurodegeneration, degeneration/death of upper and lower motor neurons in brain & spinal cord.
begins as a focal process then spreads to impact all levels of the motor system.
weakness, wasting, and paralysis of the muscles of the limbs and trunk controlling voluntary movement.
most sporadic but 5-10% familiail.
risk factors age 40-70
men more likely. smoking. veterans twice as likely.
amyotrophic lateral sclerosis manifestations
gradual onset- initial symptoms vary and can be subtle progression of painless muscle weakness.
s/s: tripping, dropping things, fatigue of extremities, slurred speech, muscle cramps, fasciculations(twitching), uncontrollable periods of laughing/crying.
amyotrophic lateral sclerosis diagnosis
difficult to diagnos- no specific test & involves ruling out other neuro disease.
diagnostic procedures to r/o other causes: electromyography & nerve conduction tests, blood & urine tests, thyroid/parathyroid hormone levels, heavy metals, lumbar puncture, MRI, muscle nerve biopsy
amyotrophic lateral sclerosis treatment
supporitve care.
medication:
riluzole: reduce damage to motor neurons by decreasing levels of neurotransmitter messages between nerve cells & motor neuron.
edaravone: decrease oxidative stress & slows progression of disease.
symptom management.
therapy: PT OT ST
nutrition support
respiratory support
end of life care planning.
Huntington’s disease
genetic disorder- autosomal dominant.
onset: @ 30-50 yrs old.
degenerative and fatal- long duration of illness 15-20 yrs
abnormal folded proteins. damage to basal ganglia which regulates voluntary movement, learning, decision making, cognition, & emotion
huntingtons disease manifestations
abnormal muscle movement- progressive loss of control of movement chorea.
intellectual decline- impaired interaction & communication
memory & attention defecits
emotional disturbances- depression, personality change- can be aggressive, paranoid, unstable mood.
progressive-late stage can’t walk, talk, or eat
huntingtons deases diagnosis & treatment
diagnosis: dna testing, number of abnormal copies varies & predicts severity of disease, genetic counseling.
treatment- symptomatic only- no way to delay onset or slow progression.
Myasthenia gravis
chronic autoimmune neuromuscular disorder of PNS.
destroys acetylcholine receptors-> muscle contraction impaired, especially with repetition.
causes: thymus gland hyperplasia or tumors produce antibodies- most.
other causes- hyperthyroid, RA, lupus.
myasthenic crisis
sudden exacerbation, motor weakness, risk of respiratory failure.
S/S: tachycardia, tachypnea, respiratory distress, dysphagia, restlessness, speech impairments, anxiety.
causes: undermedication, missed med doses, infection/stress
cholinergic crisis
life-threatening, motor weakness, risk of respiratory failure.
S/S: GI, muscle weakness, vertigo, respiratory distress.
cause: overdose of anticholinesterase meds.
manifestations of myasthenia gravis
dependent on muscles involved. initially eye muscles. facial muscles. fatigue & weakness dysphagia & PNA-respiratory muscles often involved.
exac: stress, fever, infection overexertion, heat.
relief: rest, better in the AMs.
diagnosis of myasthenia gravis
careful hx & phsyical s/s (occular, respiratory, facial,)
testing: nerve stimulation tests
antiacetylcholine receptro antibodies in seru,
anticholinesterase testing
complications
aspiration
pneumonia.
myasthenia gravis treatment
medications: anticholinesterases , immunosuppressants
surgery: thymectomy.
plasma exchange (plasmapheresis)- temporary.
Guillain-Barre
segmental demyelination with edema & inflammation of affected nerves.
acute & rapidly progressing motor paralysis.
impacts muscles, nerves, cranial nerves, LOC not affected.
autoimmune, affects all ages, ethnicites & genders.
triggers: infections, immunizations, surgery.
Guillain barre manifestations
acute onset- progresses over days to 4 weeks.
most report precipitating event within 28 days before onset.
s/s: bilateral & symmetrical loss of sensation & weakness. begins in feet & spreas upward
pain
reflexes lost
autonomic features at times: up/down BP, arrhythmia, paralytic ileus. may lead to difficulty in breathing
guillain barre diagnosis
no specific test. by presettion & correlating hx and tests.
lumbar puncture: often proteins in the csf
nerve conduction studies show a neuropathy.
guillain barre treatment
plasma exchange (plasmapheresis) IVGg infusions to suppress antibodies. supportive care-multisystem impacts. rehabilitation: recovery weeks to years.
approximately 5% of CBS pts die & up to 20% have persistent disability immunotherapy
