3360 neurodegenerative disorders

parkinson’s disease causes

lack of dopamine, link to pesticides and chemicals, lewy bodies, genetics

parkinson’s clinical manifestations

TRAP, fine tremor in hands, jaw, and tongue, decreased arm swing, flat affect, cogwheel rigidity (jerky stiff movements cause muscle soreness), shuffling, propulsive gait with arms flexed, loss of postural reflexes, hypokinetic dysarthria, dysphagia, loss of taste→lack of interest in food→malnutrition and aspiration, sleep problems, constipation, panic attacks, loss of smell, hypotension, impulse control disorder, delusional behavior, bladder dysfunction, depression, anxiety, hallucinations, sweating, dementia

TRAP

tremor, rigidity, akinesia, postural instability

complications of parkinson’s

weakness, neurologic problems, neuropsychiatric problems, dementia associated with increased mortality, poor nutrition status, orthostatic hypotension→fall risk, debilitation→pneumonia, UTI, skin breakdown

parkinson’s diagnosis

2/4 TRAP manifestations, asymmetric onset, positive response to antiparkinsonian drugs

parkinson’s treatment goals

maximize neurologic function, maintain independence in ADLs for as long as possible, prevent injury/falls, optimize well-being

parkinson’s drug therapy

aimed at correcting imbalance of NTs, start with 1 drug at a time and add combination therapy as disease progresses

antiparkinsonian drugs effects

enhance or release DA or block effects of overactive cholinergic neurons in striatum, excess amounts of dopaminergic drugs can worsen symptoms and cause paradoxical intoxication

Sinemet (Levodopa+Carbidopa) considerations

side effects- xerostomia, GI distress

high protein meal decreases absorption, consume majority of daily protein at night

levodopa

precursor of DA, can cross BBB, converted to DA in basal ganglia

carbidopa

inhibits enzyme that breaks down levodopa before it reaches brain

COMT inhibitors

block breakdown of levodopa in peripheral circulation, prolongs effects of Sinemet

entacapone/Comtan, tolcapone/Tasmar

pramipexole

dopamine agonist, mimics endogenous DA, can cause dizziness/sleepiness/orthostatic hypotension, monitor bp and safety

amantadine

antiviral, increases DA release and blocks reuptake, can cause dizziness/GI distress/CP/drowsiness, hepatotoxic, monitor LFTs, rotate injection sites, don’t stop abruptly

rasagiline, selegiline

MAO-B inhibitors, increase levels of DA, prolong half-life of levodopa, can cause dizziness/xerostomia/HA/orthostatic hypotension, avoid tyramine, many drug interactions

benztropine

anticholinergic drug, decreases activity of ACh, can cause dizziness/xerostomia/urinary retention/vision changes, avoid heat, avoid ETOH, stop if experiencing dysphagia

drugs for dementia/memory loss

rivastigmine/Exelon, donepezil/Articept

parkinson’s nutrition therapy

adequate fiber and fruit, 6 small meals/day, plan ample time to avoid frustration, limit protein intake to evening meal bc levodopa absorption can be impaired by protein and vitamin B6 ingestion, enteral tube feedings

parkinson’s interprofessional care

PT, OT, speech therapy for swallowing

parkinson’s treatment expected outcomes

maintain optimal muscle function, use assistive devices appropriately, maintain adequate nutrition, have unimpaired swallowing, use methods of communication that allow interaction with others (difficulty communicating can lead to isolation)

huntington’s disease

chronic progressive nerve degeneration of ACh and GABA, excess DA→spastic chorea

huntington’s disease inheritance

autosomal dominant, 50% chance of kid inheriting disease if one parent has it

huntington’s motor manifestations

chorea, dystonia, ataxia, oculomotor problems, parkinsonian symptoms (rigidity, spasticity, bradykinesia, myoclonus)

huntington’s cognitive manifestations

executive dysfunction, processing speed, visuoperceptual alterations, episodic memory, subcortical dementia

huntington’s psychiatric manifestations

depression, apathy, irritability, agitation, anxiety, psychosis, obsessive-compulsive behavior

leading cause of death in huntington’s disease

pneumonia secondary to aspiration, suicide

huntington’s nonpharmacological treatment

genetic counseling, memory-boosting activities, nutrition (tube feeding/PEG), palliative care

huntington’s pharmacology

dopamine depleting agents decrease spasticity- tetrabenazine/Xenazine, reserpine

antipsychotics- haloperidol, risperidone

SSRIs- sertraline, paroxetine

ALS/lou gehrig’s disease

rare progressive neuromuscular disorder, loss of motor neurons, upper and lower motor neurons in brainstem and spinal cord degenerate, myelin sheath degenerates

ALS/lou gehrig’s disease prognosis

3-5 years after diagnosis, 10 max

ALS/lou gehrig’s etiology

associated with genetics, smoking, athleticism, metals, solvents, radiation, pesticides, viruses, military

ALS/lou gehrig’s S/S

muscular atrophy, breathing problems, speech challenges, stiffness, cramps, hand tremors, unsteady gait, heavy legs, dysphagia

risk for falls and aspiration

ALS/lou gehrig’s diagnosis

increased creatinine kinase, rule out lyme disease and heavy metal poisoning, positive sharp waves, fibrillation

ALS/lou gehrig’s treatment

riluzole, edaravone, aid in communication, reduce risk for aspiration, early identification of respiratory problems, encourage diversional activities, exercises for trunk and limbs, safety and fall prevention, end of life care

myasthenia gravis

autoimmune, fluctuation of weakness of certain MSK groups, weakness of voluntary muscles after repetitive movement, ptosis/drooping eye

myasthenia gravis pathophysiology

antibodies attack ACh receptors, resulting in decreased number of ACh receptor sites at neuromuscular junction

myasthenia gravis link to thymus gland

decreased secretions, 15% of pts have thymic tumor, 65% have overactive thymus

myasthenia gravis onset

20-30 years in females

50s in males

myasthenia gravis S/S

ptosis and diplopia in 90% of cases, strabismus, lazy eye, dysphagia, difficulty chewing, dysphonia, difficulty holding head up over time, neck weakness with head bobbing, difficulty breathing, diminished breath sounds, respiratory paralysis and failure, mask-like facial expressions, fatigue worsens throughout day as muscles are used

myasthenic crisis

acute exacerbation of muscle weakness affecting swallowing and breathing, rapid unrecognized progression of disease triggered by sun/extreme heat, stress from surgery/illness/pregnancy/trauma, smoking

myasthenic crisis and drugs

inadequate medication dosage, beta blockers, calcium channel blockers, lithium, aminoglycosides, erythromycin, xylocaine, ciprofloxacin

myasthenia gravis diagnostics

PMH and PA, 6 cardinal fields (can’t hold upward gaze), CXR, CT, MRI, EMG, positive tensilon test

tensilon test

edrophonium chloride drug given in controlled environment with crash cart and oxygen and doctor present, symptoms/appearance should return back to normal short-term to confirm MG diagnosis, too much causes deadly cholinergic crisis (increased salivation, sweating, urination, defecation, lacrimation)=not MG, atropine (anticholinergic) antidote

myasthenia gravis interventions

monitor VS and respiratory status, assess muscle strength, aspiration precautions, monitor for myasthenic crisis and cholinergic crisis

myasthenia gravis nonpharmacological treatment

thymectomy, plasmapheresis (filter antibodies out, 1-2mo relief), IV IgG

myasthenia gravis anticholinesterase drugs

neostigmine, pyridostigmine drugs of choice for long term treatment

administer 30-60mins before eating

immunosuppressants for myasthenia gravis

corticosteroids, azithioprine, cyclosporine, methotrexate, tacrolimus, rituximab

myasthenia gravis pt education

conserve strength, avoid stress/infection/fatigue/OTC meds, wear medic-alert bracelet, resources/support groups, plan activities for early in the day to avoid fatigue or rest before activities

myasthenia gravis nursing diagnoses

risk for aspiration, respiratory failure, impaired verbal communication, risk for falls, impaired nutrition, activity intolerance, disturbed body image, depression, isolation

bell’s palsy

acute episode affecting CN VII, symptoms result from injury to facial nerve that supplies muscle function and sensations to face, affecting tears, salivation, taste, and expression

only affects one side of face, rule out stroke

bell’s palsy causes

inflammation caused by herpes simplex or herpes zoster that attacks CN VII

bell’s palsy S/S

pain in neck/behind ear, dry eyes, drooling, facial twitch, xerostomia, numbness on affected side, alterations in taste or hearing

bell’s palsy management

acetaminophen, moist heat, facial sling, PT, facial massage, sunglasses, artificial tears, eye shield/tape eyes shut at night, chew on unaffected side, oral care, oral corticosteroids, antivirals (acyclovir)

SLE

chronic autoimmune disease causing widespread issue damage, has periods of flares and remission, can affect skin, joints, heart, lungs, kidneys, blood cells, and brain

SLE causes

genetics, environmental factors, hormones, infections (EBV), silica dust, medications, emotional stress, injury/stress to body (childbirth, surgery)

SLE S/S

arthritis, low fever, extreme fatigue, headaches, pain when breathing deeply/lying down, swelling in hands/feet/around eyes, sensitivity to sun (rashes), malar/butterfly rash, round scaly rash on body, skin issues, raynaud’s phenomenon, swollen glands, kidney issues, eye dryness/redness, loss of vision, easy bleeding/bruising, muscle weakness/aching, joint stiffness, hair loss, sores on mouth/nose, seizures, confusion, dizziness, depression, unilateral weakness/numbness, anorexia, weight changes

SLE diagnosis

PMH, PA, CXR, echocardiogram, ANA, CBC, CMP, CRP, antiphospholipid antibodies, anti-smith, anti-double-strand DNA, kidney/skin biopsy, urinalysis

SLE treatment goals

manage symptoms, reduce inflammation, immunosuppression, prevent and treat flare ups

SLE pharmacology

anti-malarials (hydroxychloroquine), prednisone, NSAIDs, immunosuppressives, blood thinners, monoclonal antibodies

lupus nephritis S/S

foamy urine, LE edema, HTN, joint pain, muscle pain, fever with unknown origin

lupus nephritis diagnosis

urinalysis, labs, kidney biopsy

lupus nephritis treatment

control inflammation/immune system, control bp

lupus nephritis

increases risk for cancer

affects 50% of adults and 80% of kids with SLE, more common in POC

pain and fatigue are debilitating, forced to cut back on social activities, most can no longer work full time due to complications