Neurodegenerative Disorders Chapter 23 Neurodegenerative Disorders Copyright © 2016 by Elsevier, Inc. All rights reserved.

Neurodegenerative Disorders Neurodegenerative disorders are seen in older adults more than any other age group May be barely noticeable in the beginning, slight exacerbations and remissions, but the ultimate trajectory is a downward slope Parkinson’s, Alzheimer’s, and dementia with Lewy bodies (LB) are three terminal disorders that will be discussed; other nonterminal neurocognitive disorders (NCDs) are listed in Box 23-1 The word “dementia” has been replaced with the phrase “neurocognitive disorder” (NCD) Copyright © 2016 by Elsevier, Inc. All rights reserved.

Neurodegenerative Disorders (Cont.) Diagnosis Evaluation is initiated by the person, significant other, or health care provider when changes are noted in comparison to a prior state of cognition, especially memory or physical stability, such as balance or tremors The diagnostic process begins with the assessment of potentially all reversible causes for changes, such as delirium, infection, vitamin deficiencies, or endocrine disturbances (Box 23-4) Copyright © 2016 by Elsevier, Inc. All rights reserved.

Signs and Symptoms of NCDs Copyright © 2016 by Elsevier, Inc. All rights reserved.

Parkinson’s Disease (PD) The 14th cause of death, affecting 6.3 million people worldwide More common in men than women Second most common neurodegenerative disease after Alzheimer’s disease (AD) In late stages many develop NCDs, referred to as PD dementia Persons of all races and ethnicities are affected Copyright © 2016 by Elsevier, Inc. All rights reserved.

Parkinson’s Disease (PD) (Cont.) Diagnosis As a movement disorder, diagnosis can be made with reasonable certainty considering the absence or presence of classic signs and symptoms Confirmed by a “challenge test” when symptoms improve dramatically after the administration of levodopa Early falls, poor response to levodopa, symmetry of motor symptoms, lack of tremor, and early autonomic dysfunction are characteristic of other movement disorders (Box 23-5) Copyright © 2016 by Elsevier, Inc. All rights reserved.

Parkinson’s Disease (PD) (Cont.) Etiology Deficiency of the neurotransmitter dopamine, a reduction in dopamine receptors, and the accumulation of LB, especially in the basal ganglia Severity associated with the degree of neuron loss By the time a person becomes symptomatic, 70-90% of dopamine-producing cells are lost Epigenetic factors influence development 15-25% of those with PD have a relative with PD Copyright © 2016 by Elsevier, Inc. All rights reserved.

Parkinson’s Disease (PD) (Cont.) Signs and Symptoms The four core signs of PD are resting tremor, muscular rigidity, bradykinesia, and asymmetric onset Resting tremor is the first sign in 70% of those with PD Rigidity can be assessed with passive ROM Muscle rigidity and bradykinesia worsen, affecting the striated muscles in the extremities, trunk, and ocular areas, including muscles of chewing, swallowing, and speaking Other symptoms experienced (Box 23-8) Copyright © 2016 by Elsevier, Inc. All rights reserved.

Parkinson’s Disease (PD) (Cont.) Treatment No cure for PD, but when symptoms interfere with function, pharmacological interventions are initiated First-line medication is levodopa Other drugs: carbidopa and dopamine agonists When medications don’t provide relief from disabling symptoms, patients may elect to have surgical intervention, such as deep brain simulation or ablation Nonpharmacological approaches: gait training and muscle strengthening Copyright © 2016 by Elsevier, Inc. All rights reserved.

Alzheimer’s Disease Incidence increases dramatically with age By 2050 the number of persons with AD is expected to grow to 16 million Researchers may have found a gene placing African Americans at twice the risk for AD Hispanics are one and a half times more likely to develop AD; no known genetic risk identified 6th leading cause of death in whites, 10th for blacks, and 9th for Asian American and Native Hawaiian/Pacific Islander Copyright © 2016 by Elsevier, Inc. All rights reserved.

Alzheimer’s Disease (Cont.) Etiology Familial AD, diagnosed between 30 and 60 years of age, is caused by a single gene mutation on one of three chromosomes: 21, 14, or 1 Most AD diagnosed in persons over 60 years is likely due to a number of factors, including genetic risk APOE gene type ε4 is found in 40% of late onset AD Persons with NCD due to AD also have an increased number of -amyloid proteins outside the neuron and accumulation of tau proteins inside the neuron Copyright © 2016 by Elsevier, Inc. All rights reserved.

Alzheimer’s Disease (Cont.) Symptoms The initial symptom is memory loss, specifically the ability to remember new information Additional signs and symptoms develop over time Functional decline correlates to cognitive decline Copyright © 2016 by Elsevier, Inc. All rights reserved.

Alzheimer’s Disease (Cont.) Diagnosis Diagnosis of NCD due to AD requires: a decline from previous level of functioning. the onset was insidious. there has been a gradual progression in cognitive abilities. NCD disorders are now characterized as possible or probable, and major or minor Copyright © 2016 by Elsevier, Inc. All rights reserved.

Alzheimer’s Disease (Cont.) Pharmacological Treatment Pharmacological therapy is aimed at slowing cognitive decline Medications used: Cholinesterase inhibitors: donepezil (Aricept) galantamine (Razadyne) rivastigmine (Exelon) Regulation of the activity of glutamate: Memantine HCL (Namenda) Copyright © 2016 by Elsevier, Inc. All rights reserved.

Neurocognitive Dementia with LB NCD due to LB affects 1.3 million people in the United States It is second in prevalence to AD Often confused with PD, but distinctly different in chronology of symptomology In LB, cognitive changes always precede movement disorders Men affected slightly more than women Copyright © 2016 by Elsevier, Inc. All rights reserved.

Neurocognitive Dementia with LB (Cont.) Signs and Symptoms Severe loss of thinking, especially problem solving, use of language, and numerical concepts Fluctuating attention and alertness About 80% have hallucinations, which may lead to delusions and paranoia Disordered sleep Mood problems: depression, apathy, anxiety, and agitation Autonomic signs and symptoms (Box 23-9) Copyright © 2016 by Elsevier, Inc. All rights reserved.

Neurocognitive Dementia with LB (Cont.) Etiology Normal healthy brains have alpha-synuclein proteins that help neurons communicate with each other at their synapse, but in LB abnormal spherical protein aggregates are found within neurons Alpha-synuclein is highly expressed with these bodies, which displace other cellular structures and may contribute to cell death Progression of the disease leads to significant deficits in neurotransmitter production along the cholinergic and dopaminergic pathways Copyright © 2016 by Elsevier, Inc. All rights reserved.

Neurocognitive Dementia with LB (Cont.) Diagnosis According to the new DSM-5 diagnostic criteria, the person identified as one with NCD due to LB must first meet all of the initial mild or moderate criteria for other NCDs and then be classified as “possible” or “probable” Copyright © 2016 by Elsevier, Inc. All rights reserved.

Neurocognitive Dementia with LB (Cont.) Pharmacological Treatment Persons have a wide range of symptoms over time, and changes in these symptoms result in change in priority of treatment Medications: Cholinesterase inhibitors Benzodiazepines Clonazepam SAFETY ALERT: Typical antipsychotics can never be used in persons with NCD due to LB due to a very high rate of irreversible side effects and possible death Copyright © 2016 by Elsevier, Inc. All rights reserved.

Neurocognitive Dementia with LB (Cont.) Complications Decreasing risk for NCDs (Box 23-11) Complications: Pressure ulcers Pneumonia Dysphagia Aspiration Under nutrition and weight loss Behavioral disturbances Copyright © 2016 by Elsevier, Inc. All rights reserved.

Promoting Healthy Aging: Implications for Gerontological Nursing A skilled and caring multidisciplinary team is essential Treatment focuses on: relieving symptoms with medication. increasing functional ability. preventing excess disability. decreasing risk for injury. Key factors in care are: appropriate use of nonpharmacological and pharmacological interventions. prompt treatment of reversible conditions. coordination between all care providers. Copyright © 2016 by Elsevier, Inc. All rights reserved.

Question 1 Signs and symptoms related to the diagnosis of PD includes all of the following except: dramatic improvement with administration of levodopa. resting tremor. symmetry of motor symptoms. muscular rigidity. ANS:  C Copyright © 2016 by Elsevier, Inc. All rights reserved.

Question 2 Which group is at greatest risk for developing AD? African American Hispanic White (Caucasian) Asian American/Native Hawaiian ANS: A Copyright © 2016 by Elsevier, Inc. All rights reserved.