| | National conference of gerontological nurse practitioners
General nurse practitioner care guidelines: Dementia with lewy bodies  Dementia with Lewy bodies (DLB) is a cortical neurodegenerative disorder in which abnormal structures are found in the brain, mainly in the brainstem and the limbic area. This condition probably is a distinct clinical syndrome that is the second most common cause of dementia after Alzheimer disease (AD). DLB, AD, and Parkinson's disease (PD) affect some of the same areas of the brain, especially the substantia nigra; share some of the same symptoms; and can occur concomitantly. Given these similarities, experts are not certain that the syndrome is distinct. However, many scientists believe it is and that clinical variations occur within the DLB syndrome. Terms used to describe DLB are Lewy body variant of AD, Lewy body dementia, senile dementia-Lewy body type, cortical Lewy body disease, and diffuse Lewy body disease. The latter is a variant that seems to occur in middle-age adults (40 years and older) and results in prominent dementia. Incidence Dementia with Lewy bodies is the second most common form of dementia and affects 15% to 20% of people with late-onset dementia. It occurs predominantly in men, as does PD. DLB has only recently been recognized in the clinical setting as being fairly common. Pathology Lewy bodies are eosinophilic inclusion bodies in neurons, and when they are present in subcortical nuclei, they are the pathological criterion for the diagnosis of idiopathic PD. Lewy bodies contain deposits of a protein, alpha-synuclein, that is linked with PD and multiple system atrophy. Clinical features Many DLB symptoms are the same or similar to those of PD, such as bradykinesia, loss of postural reflexes, resting tremor, and rigidity. DLB is similar to AD in that memory loss and confusion occur. The core features of DLB are fluctuating cognition (great variations from day to day) with pronounced variation in attention and alertness. The elder may be alert, oriented, and sociable one day and near stuporous the next. Complex visual hallucinations occur and may be an early symptom; they may be pleasant or fearful. Delusions and depression also may occur. Clinical features that support the diagnosis include syncope, repeated falls, systematized delusions, and hallucinations. Attentional dysfunction and visuospacial impairment is seen more often in DLB than in AD. The deficit is evidenced by more difficulty with clock drawing and figure copying. Differential diagnosis AD and PD are the main considerations in the differential; cerebral vascular disease or Binswanger's disease can look similar. DLB differs from AD and PD in that it often has an abrupt onset and rapid, fluctuating progression that at first may be considered delirium. Functional decline is often rapid; however, DLB also may be slowly progressive. DLB does not always include loss of recent memory, but it does include problems retrieving information. In the late stage, memory is significantly impaired. AD is less likely to involve hallucinations, parkinsonism, and the dramatic fluctuations seen in DLB or the severe neuroleptic-induced parkinsonism or frequent falls of DLB. However, AD and DLB can occur together, and their respective signs will be present. DLB is more likely and AD less likely to cause the sensitivity reaction (mild to severe) that occurs with neuroleptic drug use in DLB, even at low doses. Diagnostic tools Detailed psychometry confirms the clinical impression of the pattern of dementia. Routine laboratory tests used in dementia evaluation are normal (complete blood count, electrolytes, glucose, calcium, blood urea nitrogen, creatinine, liver function tests, thyroid function, and B12). Computed tomography scan or magnetic resonance imaging may show generalized cerebral atrophy with frontal predominance. However, the imaging may reveal an incidental vascular disease that may make diagnosis difficult. Other imaging revelations, such as diffuse subcortical white matter, are similar to those seen in Binswanger's disease. Electroencephalogram shows generalized slowing of background activity and occasionally will show a complex similar to Creutzfeldt-Jacob disease. Brain biopsy is not justifiable. DLB, like AD, can be definitively diagnosed only at autopsy. The usual cognitive tests and other testing performed to diagnose AD (eg, Mini Mental State Exam, instrumental activities of daily living, activities of daily living, nutrition, hearing, vision, and depressions scales) are helpful for establishing the baseline and progression of DLB. Consultation with a neuropsychologist can be useful in confirming the clinical diagnosis and planning the patient's management. Diagnosis The diagnosis of probable DLB includes the central feature—dementia, as defined by DSM-IV criteria—and two core clinical features (above) that support the diagnosis. The diagnosis of possible DLB includes dementia and one core clinical feature. A positive diagnosis can be made only with biopsy or at autopsy. Pharmacologic management There is no cure for DLB; it ends in death, often more quickly than AD. Pharmacotherapy is directed at managing the neuropsychiatric symptoms and movement disorders. Anti-PD medications may reduce tremor and rigidity in DLB, but they often worsen the hallucinations, delusions, and behavioral disturbances. Neuroleptic drugs prescribed for the psychiatric symptoms (hallucinations) of DLB usually worsen the movement symptoms markedly and cause deterioration of cognitive function, known as the “neuroleptic malignant syndrome,” which can be fatal. Atypical antipsychotic drugs, such as clozapine and olanzapine, are sometimes more useful, more effective, and less likely to precipitate parkinsonism yet will improve the psychiatric symptoms. These drugs also are useful in PD with early dementia. DLB sometimes can be managed with cholinesterase inhibitors, such as rivastigmine or donepezil, for management in early dementia. How long this management should last is unclear. Nonpharmacologic management Management for DLB is the same as that for AD. Insufficient research has been conducted to determine the best management; however, the National Institute of Neurological Disorders and Stroke is supporting a number of studies at this time. One study has shown that DLB and AD can be differentiated by a set of qualitative performance characteristics. Researchers found that inattention, visual distractibility, impairments in establishing and shifting mental set, incoherence, confabulatory responses, perseveration, and intrusions were significantly more common in DLB than AD patients. Nevertheless, educating the family, managing caregiver stress, preserving skills, and giving love should all be part of the care of elders with DLB as with those having AD.
Pharmacologic update: Use of folic acid supplementation to prevent alzheimer disease  A 2000 study1 in the Journal of Neuroscience described the results of an animal study that adds new knowledge to the possible cause and prevention of Alzheimer disease (AD). In this study, transgenic mice with mutant genes that cause AD in people were randomly assigned to receive a diet either deficient or adequate in folic acid. Mice with low amounts of dietary folic acid had high levels of homocysteine, an amino acid in the blood and brain. Increased levels of homocysteine are believed to damage the DNA of nerve cells in the hippocampus. The mice that received sufficient folic acid could repair this DNA damage. These findings suggest a possible cause-and-effect relationship between folic acid and AD. Unfortunately, the diagnosis of folate deficiency still presents problems, particularly if hematologic abnormalities are absent. This imprecision relates to the interrelationship between the duration and degree of folate deficiency and other predisposing factors such as genetic make-up. Further research is ongoing to better understand both diagnostic issues and how much folic acid is sufficient. Currently, dosage recommendations for folic acid vary between 0.4 mg/d and 10 mg/d, depending on the severity of the deficiency. Older adults certainly should be encouraged to eat a diet that includes beans; raw, green, leafy vegetables; and foods supplemented with folate.
Putting research into practice: Behavioral and pharmacologic management of dementia  The management of older adults with dementia must include both behavioral (and other nonpharmacologic interventions) and pharmacologic interventions as appropriate. Several recent studies addressing nonpharmacological interventions are worthy of review. Beck et al1 tested the impact of several different behavioral interventions, all built on the premise that if an individual's basic psychosocial needs were met, disruptive behavior would decrease. Specifically, residents in long-term care facilities were randomized to one of five interventions. No significant differences emerged for the intervention by time interaction for any disruptive behavior. The participants seemed to experience benefit during the one-on-one interaction, but these effects did not last. Aromatherapy was another nonpharmacologic intervention tested in a randomized clinical study with older adults with dementia. This study2 included 72 older adults with dementia and associated agitation who were randomly assigned to receive aromatherapy with essential oil of lemon balm. The oil was combined with a base lotion and applied to the residents' faces and arms twice a day. The control group received sunflower oil in the same way. The results indicated that exposure to the oils decreased agitation and improved quality of life. In light of the low risk for side effects from this type of treatment, aromatherapy should be considered for residents in whom pharmacotherapy may not be an option or is not safe. The current standard of care for pharmacologic management of dementia is a combination of a cholinesterase inhibitor and high-dose vitamin E.3 This standard is based on the results of large-scale, double-blind, placebo-controlled randomized trials. Large-scale placebo-controlled trials of tacrine, donepezil, rivastigmine, and galantamine have demonstrated moderate benefits in patients with mild to moderate dementia.3, 4, 5, 6, 7, 8 Rivastigmine is a dual acetylcholinesterase/butyrylcholinesterase (AChE/BuChE) inhibitor, whereas donepezil and galantamine are acetylcholinesterase-selective inhibitors. Donepezil, rivastigmine, and galantamine are the first-line choices because of their lower side effect profile. Treatment benefits include improved function and behavior. Table 1 compares the different AChE inhibitors.
| | |  | Characteristic | Donepezil | Rivastigmine | Galantamine |  |
 | Safety | Caution in peptic ulcer disease, asthma, and bradycardia | Same | Same |  |
 | Tolerability | Insomnia, agitation, leg cramps, GI upset | Agitation, GI upset | GI upset |  |
 | Dosing/half-life /metabolism | Daily dosing, 72-hour half-life, liver metabolism | Twice daily, 8-hour half-life, AChE metabolism | Twice daily, 7-hour half-life, liver metabolism |  |
 | Behavioral symptom response | Yes | Yes | Yes |  | | | |
Older adults with dementia and their caregivers should be provided with information about the benefit and risks of using AChE inhibitors and helped to make a decision about initiation and termination of this treatment option.
Health policy pearls for advance practice nurses who care for geriatric patients  In July, President Bush signed nursing shortage legislation, the Nurse Reinvestment Act, into law. This important bill includes nurse scholarships in exchange for commitment to work in underserved areas, nurse retention grants, comprehensive geriatric training grants, faculty loan cancellation programs, career ladder grant programs, and public service announcements. The National Conference for Gerontological Nurse Practitioners (NCGNP) and the 60-plus other nursing and allied health organizations that form the Americans for Nursing Shortage Relief (ANSR) Alliance helped it happen. The ANSR Alliance met regularly to discuss methods to promote the bill and regularly contacted Capitol Hill legislators. Enactment of this law authorizes the appropriate agencies to establish or expand programs. The second phase of enacting the bill is underway, thus ensuring that funding is appropriated to fully implement all parts and programs. The ANSR Alliance is working with Congress to obtain funds for fiscal year 2003. The budget is tight, but NCGNP and other nursing groups in the coalition will continue to push. Much of the administrative implementation of the act will be delegated to the Health Resources and Services Administration's Division of Nursing. They have the authority to administer the various nursing scholarship and grant programs that address nursing education. For more information, visit www.bhpr.hrsa.gov/nursing .
References  1.
1
Kruman I, Kumaravel T, Lohani A, Pedersen R, Cutler Y, Kruman N, et al.
Folic acid deficiency and homocysteine impair DNA repair in hippocampal neurons and sensitize them to amyloid toxicity in experimental models of Alzheimer's disease.
J Neurosci. 2002;22:1752–1762. 1.
1
Beck C, Vogelpohl T, Rasin J, Uriri J, O'Sullivan P, Walls R, et al.
Effects of behavioral interventions on disruptive behavior and affect in demented nursing home residents.
Nurs Res. 2002;51:219–228. MEDLINE |
CrossRef
2.
2
Ballard CG, O'Brien JT, Reichelt K, Perry EK.
Aromatherapy as a safe and effective treatment for the management of agitation in severe dementia: The results of a double-blind, placebo-controlled trial with Melissa.
J Clin Psychiatry. 2002;63:553–558. MEDLINE 3.
3
Bonner LT, Peskind ER.
Pharmacologic treatments of dementia.
Med Clin North Am. 2002;86:657–674. Full Text |
Full-Text PDF (106 KB)
|
CrossRef
4.
4
Doraiswamy PM, Krishnan KR, Anand R, Sohn H, Danyluk J, Hartman RD, et al.
Long-term effects of rivastigmine in moderately severe Alzheimer's disease: Does early initiation of therapy offer sustained benefits?.
Prog Neuropsychopharmacol Biol Psychiatry. 2002;26:705–712. MEDLINE |
CrossRef
5.
5
Olin J, Schneider L.
Galantamine for Alzheimer's disease (Cochrane Review).
Cochrane Database Syst Rev. 2002;(3):CD001747. 6.
6
Potki SG, Anand R, Hartman R, Veach J, Grossberg G.
Impact of Alzheimer's disease and rivastigmine treatment on activities of daily living over the course of mild to moderately severe disease.
Prog Neuropsychopharmacol Biol Psychiatry. 2002;26:713–720. MEDLINE |
CrossRef
7.
7
Wilkinson DG, Passmore AP, Bullock R, Hopker SW, Smith R, Potocnik FC, et al.
A multinational randomised 12-week comparative study of donepezil and rivastigmine in patient with mild to moderate Alzheimer's disease.
Int J Clin Pract. 2002;127(Suppl):20–36. 8.
8
Wolfson C, Oremus M, Shukla V, Momoli F, Demers L, Perrault A, et al.
Donepezil and rivastigmine in the treatment of Alzheimer's disease: A best-evidence synthesis of the published data on their efficacy and cost-effectiveness.
Clin Ther. 2002;24:862–886. Abstract |
Full-Text PDF (1546 KB)
|
CrossRef
*.
10.
Adams AC.
Neurology in primary care.
www.fadavis.com2000;
. *.
11.
Ballard C, Grace J, Holmes C.
Neuroleptic sensitivity in dementia with Lewy bodies and Alzheimer's disease.
Lancet. 1998;351:1032–1033. Full Text |
Full-Text PDF (61 KB)
|
CrossRef
*.
12.
Campbell S, Stephens S, Ballard C.
Dementia with Lewy bodies: Clinical features and treatment.
Drugs Aging. 2001;18:397–407.
CrossRef
*.
13.
Doubleday EK, Snowden JS, Varma AR, Neary D.
Qualitative performance characteristics differentiate dementia with Lewy bodies and Alzheimer's disease.
J Neurosurg Psychiatr. 2002;72:565–566. *.
14.
Leverenz JB, McKeith IG.
Dementia with Lewy bodies.
Med Clin North Am. 2002;86:519–535. Full Text |
Full-Text PDF (164 KB)
|
CrossRef
*.
15.
McKeith IG, Burn D.
Spectrum of Parkinson's disease, Parkinson's dementia and Lewy body dementia.
Neurol Clin. 2000;18:865–902. Full Text |
Full-Text PDF (1172 KB)
|
CrossRef
*.
16.
McKeith IG, et al.
Guidelines for the clinical and pathologic diagnosis of dementia with Lewy bodies.
Neurology. 1996;47:1113–1124. MEDLINE *.
17.
National Institute of Neurological Disorders and Stroke. National Institutes of Health.
www.ninds.nih.gov/health_and_medical_disorders/dementiawithlewybodies_doc.htm2002 Oct 8;
. *.
18.
Pompeu F, Growdon J.
Diagnosing dementia with Lewy bodies.
Arch Neurol. 2002;59(1):29–30. MEDLINE |
CrossRef
*.
19.
Santa Cruz K, Walker Z, Swagerty D, et al.
Clinical presentation in monozygotic twins with dementia with Lewy bodies.
J Am Med Directors. 2002;3:175–179. *.
20.
Bilikiewicz A, Opala G, Podemski R, Puzynski S, Lapin J, Soltys K, et al.
An open-label study to evaluate the safety, tolerability and efficacy of rivastigmine in patients with mild to moderate probable Alzheimer's disease in the community setting.
Med Sci Monitor. 2002;8(2):PI9–P15. *.
21.
Hageman PA, Thomas VS.
Gait performance in dementia: The effects of a 6-week resistance training program in an adult day-care setting.
Int J Geriatr Psychiatry. 2002;17:329–334. MEDLINE |
CrossRef
*.
22.
Lopez OL, Becker JT, Wisniewski S, Saxton J, Kaufer DI, DeKosky ST.
Cholinesterase inhibitor treatment alters the natural history of Alzheimer's disease.
J Neurol Neurosurg Psychiatry. 2002;72:310–314. MEDLINE |
CrossRef
*.
23.
Rosler M.
The efficacy of cholinesterase inhibitors in treating the behavioural symptoms of dementia.
Int J Clin Pract. 2002;56:441–446. MEDLINE ANN S. LUGGEN, PhD, BC, GNP, CNAA, is a professor of nursing at Northern Kentucky University and a geriatric nurse practitioner at Evercare. She was chairwoman of the NCGNP Research Committee and now serves as NCGNP section editor for Geriatric Nursing and the NCGNP newsletter. JOANNE M. MILLER, PhD, APN/GNP, is an assistant professor at Rush University's College of Nursing in Chicago and a geriatric nurse practitioner at Chicago Senior Center. KATHLEEN JETT, ARNP, BC, PhD, is an assistant professor in the Christine E. Lynn College of Nursing at Florida Atlantic University in Boca Raton. She is a geriatric nurse practitioner working with underserved elders. BARBARA RESNICK, PhD, CRNP, FAAN, FAANP, is President of NCGNP and an associate professor in the University of Maryland School of Nursing in Baltimore. LYNN CHILTON, DSN, GNP/FNP, is chair of the NCGNP Health Affairs Committee PII: S0197-4572(02)09001-8 doi:10.1067/mgn.2003.2 © 2003 Mosby, Inc. All rights reserved. | |
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