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Degenerative Neurologic Disorders

Unit 11 · Topic 66Degenerative Neurologic Disorders
1.Overview & Pathophysiology

Degenerative neurologic disorders cause progressive loss of neurons. Most have no cure; care aims to relieve symptoms, preserve function and safety, prevent complications (especially aspiration and falls), and support families.

Parkinson disease (PD)

  • Loss of dopamine-producing neurons in the substantia nigra → imbalance between dopamine and acetylcholine in the basal ganglia.
  • Cardinal motor features: resting tremor ("pill-rolling," disappears with purposeful movement and sleep), rigidity (lead-pipe or cogwheel), bradykinesia (slowness — the key feature), and postural instability.
  • Nonmotor features often come first: loss of smell, constipation, REM sleep behavior disorder, depression, orthostatic hypotension.

Atypical parkinsonism (poor response to levodopa)

  • Multiple system atrophy (MSA): parkinsonism or ataxia with prominent autonomic failure — orthostatic hypotension, urinary dysfunction, erectile dysfunction.
  • Progressive supranuclear palsy (PSP): early falls (often backward), axial rigidity, and vertical supranuclear gaze palsy — difficulty looking down (and up) is characteristic; downgaze problems cause trouble with stairs and eating.

Alzheimer disease (AD) and dementia

  • AD is the most common cause of dementia: amyloid-beta plaques and tau neurofibrillary tangles, loss of cholinergic neurons, brain atrophy.
  • Other dementias: vascular, Lewy body (visual hallucinations, parkinsonism, severe sensitivity to antipsychotics), frontotemporal (early personality and behavior change).
  • Stages: mild (forgetfulness, trouble with complex tasks), moderate (confusion, wandering, needs help with ADLs, sundowning), severe (dependent, loss of speech, dysphagia).

Amyotrophic lateral sclerosis (ALS)

  • Degeneration of both upper and lower motor neurons. Weakness, atrophy, fasciculations, spasticity, hyperreflexia, dysarthria, dysphagia, and eventually respiratory muscle failure.
  • Sensation, bladder/bowel control, and eye movements are usually spared; cognition is often intact, though some clients develop frontotemporal dementia.
  • Death usually results from respiratory failure and aspiration pneumonia.

Huntington disease (HD)

  • Inherited in an autosomal dominant pattern: each child of an affected parent has a 50% chance of inheriting the gene (CAG repeat expansion in the HTT gene). Men and women are equally affected.
  • Triad: chorea (involuntary jerky movements), cognitive decline, and psychiatric symptoms (depression, irritability, suicide risk). Onset typically in mid-adulthood.
2.Assessment Findings
DisorderKey findings
ParkinsonShuffling, short, fast steps with a forward-leaning (festinating) gait; reduced arm swing; freezing of gait (feet feel stuck to the floor, especially when starting to walk or turning); masklike face; soft voice; small handwriting (micrographia); drooling; dysphagia
MSAOrthostatic hypotension, urinary retention or incontinence
PSPVertical gaze palsy, early backward falls
AlzheimerMemory loss (recent first), disorientation, aphasia, apraxia, agnosia, poor judgment, sundowning (worse confusion late in the day), day–night reversal, wandering
ALSAsymmetric limb weakness, fasciculations, dysphagia, dysarthria, weak cough, dyspnea, orthopnea
HuntingtonChorea, clumsiness, dysphagia, weight loss (high energy use), mood changes

Assess swallowing, nutrition and weight, fall risk, cognition (e.g., Mini-Cog, MoCA), mood, caregiver strain, and respiratory function (forced vital capacity in ALS).

3.Diagnostics
  • Parkinson: clinical diagnosis; clear improvement with levodopa supports it. Dopamine transporter imaging may be used when unclear.
  • Alzheimer: cognitive testing; labs to exclude reversible causes (vitamin B12, thyroid function, metabolic panel, syphilis or HIV when indicated, depression screening); MRI. Amyloid PET, CSF biomarkers, or validated blood tests confirm amyloid pathology, required before anti-amyloid therapy.
  • ALS: clinical findings plus EMG/nerve conduction studies; exclude mimics (e.g., cervical myelopathy). Genetic testing (e.g., SOD1).
  • Huntington: genetic test for CAG repeat length. Predictive testing of at-risk adults should follow genetic counseling.
4.Medical Management

Parkinson disease

DrugNursing points
Carbidopa-levodopaMost effective drug. Carbidopa blocks peripheral conversion of levodopa to dopamine, so more levodopa reaches the brain and nausea, vomiting, and orthostatic hypotension are reduced. High-protein meals compete with levodopa absorption — spread protein evenly across the day or take the dose 30–60 minutes before meals. Adverse effects: nausea, orthostatic hypotension, hallucinations, and, with long-term use, dyskinesias (involuntary writhing movements that occur at peak dose) and "wearing-off" or on–off fluctuations. Do not stop abruptly — risk of a syndrome resembling neuroleptic malignant syndrome. Avoid nonselective MAO inhibitors (hypertensive crisis). Pyridoxine (vitamin B6) does not reduce the effect when carbidopa is included
Dopamine agonists (pramipexole, ropinirole, rotigotine patch)Impulse control disorders (gambling, shopping), sudden sleep attacks (driving risk), hallucinations, orthostatic hypotension, edema
MAO-B inhibitors (selegiline, rasagiline, safinamide)Avoid meperidine, tramadol, methadone, and dextromethorphan; caution with serotonergic drugs (serotonin syndrome); tyramine restriction at higher selegiline doses; selegiline can cause insomnia
COMT inhibitors (entacapone)Extend levodopa effect; harmless orange urine; diarrhea
AmantadineReduces dyskinesia; livedo reticularis, confusion; adjust in kidney disease
Anticholinergics (trihexyphenidyl)For tremor in younger clients; avoid in older adults (confusion, urinary retention, constipation)
  • Deep brain stimulation for motor fluctuations; physical, occupational, and speech therapy.
  • Avoid dopamine-blocking drugs: haloperidol and most first-generation antipsychotics, metoclopramide, prochlorperazine. For psychosis, low-dose quetiapine, clozapine (requires neutrophil monitoring), or pimavanserin are used; all antipsychotics, including pimavanserin, carry a boxed warning for increased mortality in older adults with dementia-related psychosis.

Alzheimer disease

  • Cholinesterase inhibitors (donepezil, rivastigmine, galantamine) — modest symptomatic benefit. Adverse effects: nausea, diarrhea, weight loss, bradycardia and syncope, vivid dreams (give donepezil in the morning if this occurs). Caution with conduction disease; additive bradycardia with beta blockers; GI bleeding risk rises with NSAIDs.
  • Memantine (NMDA antagonist) for moderate to severe AD — dizziness, confusion, constipation; reduce dose in kidney impairment.
  • Anti-amyloid monoclonal antibodies (lecanemab, donanemab) for early AD with confirmed amyloid: IV infusions that modestly slow decline. Main risk (boxed warning) is amyloid-related imaging abnormalities (ARIA) — brain edema or microhemorrhage — requiring scheduled MRI monitoring. ARIA can mimic stroke, so clients should carry a treatment card; thrombolysis needs extra caution. Risk is highest in APOE e4 homozygotes; caution with anticoagulants. Watch for infusion reactions; report headache, confusion, visual change, or seizures.
  • Behavioral symptoms: treat nonpharmacologically first; antipsychotics increase mortality in dementia (boxed warning) and are reserved for severe distress or danger.

ALS

  • Riluzole — modestly prolongs survival; monitor liver enzymes and neutrophil count. Edaravone (IV or oral). Tofersen (intrathecal) for SOD1-ALS.
  • Noninvasive ventilation when respiratory muscles weaken (e.g., FVC below 50% predicted or symptoms of nocturnal hypoventilation) — improves survival and quality of life.
  • Gastrostomy (PEG) early, before FVC falls too low for a safe procedure.
  • Mechanically assisted cough, secretion management, advance care planning.

Huntington disease

  • Chorea: tetrabenazine, deutetrabenazine, valbenazine (VMAT2 inhibitors). All three carry a boxed warning for depression and suicidality in Huntington disease — contraindicated in clients who are actively suicidal or have untreated depression; screen mood at every visit. Do not combine with MAO inhibitors or reserpine. Also sedation, parkinsonism, QT prolongation. Antipsychotics may also be used.
  • Treat depression; high-calorie diet; genetic counseling.
5.Nursing Interventions

Listed in priority order.

  1. Airway and aspiration prevention
    • Swallow evaluation; upright for meals; thickened liquids and soft foods as ordered; small bites; suction available.
    • ALS: the most dangerous complications are aspiration pneumonia and respiratory failure — monitor FVC, SpO₂, cough strength, and dyspnea lying flat; teach breathing exercises and assisted-cough techniques as ordered; elevate head of bed; support NIV use.
  2. Fall prevention
    • PD: teach to lift the toes, take large steps, walk with a wide base, and rock side to side or step over a visual line to overcome freezing; remove rugs; grab bars; rise slowly (orthostatic hypotension, especially in MSA).
    • PSP: early backward falls — supervision, assistive devices.
  3. Medication timing (PD)
    • Give levodopa on time in the hospital — delayed doses cause rigidity, immobility, and aspiration risk.
    • Recognize peak-dose dyskinesia versus end-of-dose wearing-off and report patterns.
  4. Cognitive support and safety (dementia)
    • Communicate with short, simple sentences, one step at a time, spoken slowly, with visual cues and gestures; approach from the front; call the client by name; avoid arguing or reality confrontation when it causes distress.
    • Consistent routine, clocks and calendars, familiar caregivers.
    • Sleep–wake: encourage daytime activity and sunlight exposure, limit daytime napping, reduce evening stimulation and caffeine to lessen sundowning and night waking.
    • Wandering: identification bracelet, door alarms; never encourage the client to go out alone.
  5. Nutrition — high-calorie foods (HD, ALS); monitor weight; finger foods for dementia; constipation management (PD).
  6. Communication — speech therapy, communication boards, eye-gaze devices for ALS.
  7. Psychosocial and caregiver support — screen for depression and suicide risk (HD, PD); respite care; advance directives early while decision-making capacity remains.
6.Client Education
  • PD: take levodopa at consistent times; spread protein through the day; change position slowly; report hallucinations, compulsive behaviors, or sleep attacks; never stop medication suddenly; exercise (walking, cycling, tai chi) improves mobility.
  • Dementia caregivers: secure medications, knives, car keys, stove knobs, and firearms; remove trip hazards; use labeled doors and night lights; consider supervised driving evaluation; plan for legal and financial decisions early.
  • ALS: discuss ventilation, feeding tube, and end-of-life preferences early.
  • HD genetic counseling: autosomal dominant, 50% risk for each child; the decision to have predictive testing is personal and requires counseling about emotional, family, insurance, and employment implications.
7.Complications & Red Flags
ComplicationWhat to watch for
Aspiration pneumoniaCoughing with meals, fever, crackles — leading cause of death in PD, ALS, advanced dementia
Respiratory failure (ALS)Morning headache, orthopnea, weak cough, falling FVC
Falls, hip fractureFreezing, orthostatic hypotension, impulsivity
Levodopa withdrawal syndromeFever, rigidity, confusion after missed or stopped doses
Delirium superimposed on dementiaSudden change from baseline — look for infection, drugs, dehydration
SuicideHD, PD, early dementia
Malnutrition, dehydrationWeight loss, dysphagia
8.High-Yield Points
  • PD = dopamine loss: resting tremor, rigidity, bradykinesia, postural instability.
  • PD gait: short shuffling fast steps, forward lean; freezing = feet stuck when starting.
  • Carbidopa prevents peripheral conversion of levodopa; high protein reduces absorption.
  • Long-term levodopa → peak-dose dyskinesia and wearing-off; never stop abruptly.
  • Avoid haloperidol, metoclopramide in PD.
  • MSA → orthostatic hypotension; PSP → vertical gaze palsy (downgaze) and early falls.
  • Dementia communication: short simple sentences, slowly, visual cues; day activity and sunlight for sundowning; never let client go out alone.
  • Cholinesterase inhibitors → bradycardia, GI upset; anti-amyloid antibodies → ARIA, MRI monitoring.
  • ALS: upper + lower motor neurons; most dangerous complication = aspiration pneumonia / respiratory failure; riluzole (liver tests); early NIV and PEG.
  • Huntington: autosomal dominant, 50% per child; chorea; suicide risk; VMAT2 inhibitors.

Country Notes

United States

  • Anti-amyloid antibodies are FDA-approved for early AD; they require confirmed amyloid, baseline and scheduled MRIs, and APOE counseling.
  • The Genetic Information Nondiscrimination Act protects against genetic discrimination in health insurance and employment, but not in life or long-term care insurance — relevant to Huntington predictive testing.

Philippines

  • X-linked dystonia-parkinsonism ("Lubag") is a hereditary movement disorder found mainly in men with ancestry from Panay Island (especially Capiz); consider it in a Filipino man with dystonia and parkinsonism.
  • Family members are usually the main caregivers for dementia and ALS; caregiver teaching and respite planning are central nursing tasks.

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