Neurodegenerative Disorders

1. Neurodegenerative Disorders Rita Carey-Nita

2. Neurodegenerative disorders • Any disorder that causes degeneration or wasting of the neurons in the nervous system • Includes disorders such as: • Dementia • Delirium • Parkinson’s Disease • Huntington’s Disease • Alzheimer’s Disease

3. Dementia • Dementia • Symptom not disorder • Impairs intellectual functioning, normal ADL’s & relationships • Personality changes, behavioral problems, inability to problem solve & memory loss are all issues • May be mild to severe • Mild is referred to as mild cognitive impairment • Does not interfere with ADL as drastically

4. Dementia • Etilogy • Huntington’s disease • Parkinson’s • Alzheimer’s • Multiple infarcts • Chronic ETOH • Neurological infections • TBI • Medications

5. Dementia • Pathophysiology • Vary depending on cause • Thinking is affected by changes in brain • Reduced blood flow or structural changes • Signs & Symptoms • Recent memories are lost first • Ask same question repeatedly • May get lost in familiar area • Disoriented to time • May not recognize surrounding • May not recognize family • With progression of dementia • Long-term memory lost • Forget how to perform simple daily tasks • Wander & become lost • Aphasia

6. Dementia • Diagnostic Tests • Neuropsychological tests • Mini-mental exam • Examine for depression • Review of medications • MRI • PET Scan • Blood Tests

7. Dementia • Therapeutic Interventions • Medical interventions depend on cause • If can not reverse or alter than focus is on slowing progression • Establish patient’s wishes in regards to POA—code status—guardianship—finances

8. Delirium • Delirium • Disorganized thinking and difficulty staying focused • Mental disturbance that is temporary • Can progress rapidly or gradually • Medical emergency that needs to be diagnosed & treated promptly • Causes: • Medication—acute illness—pain—oxygen deficiency—urinary catheters—fluid & electrolytes imbalance—change in environment—nutritional deficiency • Interventions: • Reorient with family member present • Same nurses providing care • Correct underlying cause • Monitor for safety • Communicate clearly

9. Parkinson’s Disease • Parkinson’s Disease • Chronic degenerative disease arises from basal ganglia in cerebrum • Onset in 40’s-50’s & progressively worsens • Communication from brain to muscles is impaired • Profoundly impacts patient

10. Parkinson’s Disease • Pathophysiology • Destruction of substantianigra cells which produce dopamine results in decrease production of dopamine • Dopamine facilitates the transmission of impulses from one neuron to another • Loss of dopamine results in impairment of semiautomatic movements • Acetylcholine production remains normal but with decrease in dopamine an imbalance occurs • The imbalance results in • Tremors—muscle rigidity—akinesia • Affects motor neurons along the extrapyramidal tract

11. Parkinson’s Disease • Etiology • Unknown • Not sure why cell destruction occurs • May be genetic • May be environmental toxins

12. Parkinson’s Disease • Classic symptoms • Tremors • Begin in hand & progress to ipsilateral foot then contralateral • Worse on one side • Referred to as pill rolling • Lessen or disappear with movement • Noted at rest or while holding object • Disappear when asleep • Change in posture • Forward stooped position • Knees, hips & neck flexed • Shuffled gait with short steps

13. Parkinson’s Disease • Signs & Symptoms • Muscular rigidity • Bradykinesia • Akinesia • Broad stance noted to compensate for imbalance • Difficulty initiating movement or slow with voluntary movement • May actually halt movement • Once moving may then have difficulty stopping movement • Affects extensor muscle more than flexors

14. Parkinson’s Disease Secondary Symptom’s • Generalized weakness • Muscle fatigue & weakness • Difficulty with fine motor skills • Deterioration of hand writing • Soft monotone voice • Masklike facial expression • Diminished blink reflex

15. Parkinson’s Disease Autonomic system involvement • Diaphoresis • Constipation • Orthostatic hypotension • Drooling • Dysphagia • Seborrhea • Diaphoresis • Frequent urination

16. Parkinson’s Disease • Late symptoms • Slowed mental function • Dementia • Death

17. Parkinson’s Disease Complications patient is at risk for • Falls • Injury • Incontinence • Constipation • Aspiration • Impaired mobility • Hygiene deficit • Isolation • Depression

18. Parkinson’s Disease Diagnostic Tests • No specific test • Based on history & physical exam • MRI to rule out alternative cause

19. Parkinson’s Disease Therapeutic Interventions • No cure • Treatment aimed at management of symptoms

20. Parkinson’s Medications Medication • On-off phenomenon • Drug holiday • Anticholingerics • trihexyphenidyl (Artane)—blocks action of acetylcholine to control tremor & salivation • Dopamine agonist • Amantadine (Symmetrel)—facilitates the release of dopamine • Levodopa (L-Dopa)—converts into dopamine in the brain • Pramipexole (Mirapex)—stimulates dopamine receptors in the brain • Monoamine Oxidase B Inhibitor • Selegiline (Eldepryl, Carbex)—blocks the metabolism of central dopamine which increases dopamine in CNS • Catechol O Methyltransferase inhibitor • Entacapone (Comtan) Blocks the enzyme COMT to prevent breakdown of levodopa prolonging its action—used with Sinemet

21. Parkinson’s Disease Surgical Treatment • Pallidotomy • Performed to manage uncontrollable rigidity—tremors—bradykinesia • Stereotactic procedure that places a destructive lesion in the basal ganglia • Performed on one side at a time • Patient is awake for procedure to ensure proper placement of lesion • Stem cell implantation into brain • Controversial due to ethical issues surrounding stem cell research

22. Nursing Assessments related to Parkinson’s Disease • Assess the symptoms & level of function • Monitor gait & ability to perform ADL’s • Is safety an issue • Assess nutritional status • Assess for side effects of medication • Psychosocial assessment

23. Huntington’s Disease • Progressive, hereditary, degenerative, incurable neurological disorder Etiology • Inherited autosomal dominant • 50% of affected parents offspring inherit disorder Pathophysiology • Characterized by degeneration of corpus striatum, caudate nucleus and other deep nuclei of the brain and portions of the cerebral cortex • Results in progressive loss of normal movement & intellect

24. Huntington’s Disease Signs & Symptoms • Personality changes & inappropriate behavior • Mood swings between euphoria to irritability • Paranoia • May become violent as dementia progresses • Progression to complete dementia • Emotional upset—stress with symptoms • Depression & suicide common during early stages before cognitive impairment

25. Huntington’s Disease • Physical Symptoms • Involuntary—jerky—irregular—choreiform movements • Initially appears as mild fidgeting with facial grimacing • Start with involuntary movement of arms—face—neck • Progresses to entire body • Hesitant speech • Eye blinking • Irregular trunk movements • Abnormal tilt of head • Constant motion • Wide gait • Dysphagia

26. Huntington’s Disease • Patient becomes dependent • Aspiration with respiratory failure is primary cause of death • Life Span after onset is 10-20 years

27. Huntington’s Disease • Diagnostic Tests • Based physical exam & family history of disease • Genetic testing • Therapeutic Interventions • No cure • Minimize symptoms & prevent complications • Medications • Antipsychotic—antidepressants—antichoreic • Transplant fetal nerve tissue • Controversial ethical debate

28. Huntington’s Disease • Nursing Management • Give directions in calm firm manner • Monitor for safety • Swallowing • Involuntary movements • Discuss end of life decisions before dementia progresses

29. Alzheimer’s Disease Alzheimer’s Disease • Most common type of dementia • Progressive degenerative disease • Characterized by the loss of mental functioning that interferes with memory—thinking—learning—function • More common in women than men

30. Alzheimer’s Disease • Etiology • Unknown • Theories include: • Viral or bacterial infection—autoimmune dysfunction—correlation with Chromosome 21

31. Alzheimer’s Disease • Pathophysiology • Abnormality within the protein layer in the cell membrane of a neuron • Axon terminals & dendrite branches disintegrate & collect in plaques • Neurofibrillary tangles present • The tangles & plaques spread via axons to other areas in the brain • Acetylcholine deficiency in cerebral cortex • The younger the onset the faster the progression the shorter the life span

32. Alzheimer’s Disease Signs & Symptoms • 3 stages • Early stage • 2-4 years characterized by increasing forgetfulness • Loss of interest in day-to-day activities—acquaintances—surroundings • Job performance deteriorates & reluctant to take on new tasks

33. Alzheimer’s Disease • Middle stage • 2-12 years • Progressive cognitive deterioration • Irritability • Depression • Aphasia • Increase in physical activity • Disruption in sleep-wake cycle • Anxiety • Hallucinations • Seizures • Deterioration of personal hygiene & social behavior

34. Alzheimer’s Disease • Third Stage • Length is dependent on overall health & stamina • Progressive to point of total dependence • Unable to communicate • Incontinent of bowel & bladder • Safety is issue due to wandering • Loss of emotional control • Unable to recognize loved ones • Progresses to inability to move independently—swallow—communicate needs

35. Alzheimer’s Disease • Diagnostic Tests • Only determined on autopsy • Diagnosis is based on history & physical • MRI • May reveal neurofibrillary tangles & neuritic plaques • PET & SPECT • Show areas of neuronal inactivity

36. Therapeutic Interventions • Minimize effects & maintaining independence • Medications • Acetylcholinesterase (AChE) inhibitors—Aricept (donepezil) & Exelon (rivastigmine) • inhibit the breakdown of acetylcholine which increases levels in the brain allowing better function of remaining neurons • Used with mild to moderate symptoms • Takes time for effect to be noticed • Delays progression of symptoms therefore dependence on care • NMDA (N-methyl-D-aspartate) antagonists—Namenda, Axura (Memantine) • Prevents overexcitation of NMDA receptors in brain & allows for normal function • Given at any stage • Slows progression

37. Alzheimer’s Disease • Other medications • Antidepressants—antipsychotics—antianxiety • Control symptoms exhibited • Does not treat dementia