It’s not always Alzheimer’s: Introducing LBD
By Angela Taylor
If a person experiences memory loss, does that mean they probably have Alzheimer’s disease? Not by a long shot. There are more than 100 disorders that cause dementia, including hypothyroidism, vitamin deficiencies, alcohol abuse, brain tumors and AIDs. The most frequent cause of dementia, and the first name that pops into nearly everyone’s mind, is Alzheimer’s disease.
But if it’s not Alzheimer’s disease, what’s the next most likely cause of progressive memory loss? With a strange name and a clinical presentation that masquerades as other more well-known disorders, Lewy body dementia is the most misdiagnosed form of dementia. Unlike Alzheimer’s disease, people with undiagnosed LBD may be exposed to medications that can be very harmful to them. Misdiagnosis or lack of diagnosis also delays comprehensive symptom management that could improve their quality of life.
A primer on dementia
Dementia itself is not a disease. It is actually a clinical syndrome with many different potential causes. The term dementia means a significant decline in a person’s ability to think and reason that renders them unable to independently manage aspects of their everyday lives, such as their finances, medications, personal hygiene, or household tasks.
Dementia affects a person’s abilities in several cognitive areas such as:
Memory — the ability to recall what has been learned or experienced
Language — the ability to speak and understand words
Judgment — the ability to reason and make good decisions
Executive function — the ability to plan, solve problems and understand abstract concepts
Visuospatial function — understanding how items or places are physically related to each other in space
Attention — the ability to focus awareness, such as on someone talking or one’s surroundings.
Unfortunately, primary care physicians may not recognize cognitive impairment in up to 76 percent of people with dementia or probable dementia.1 Sadly, most of these patients are not diagnosed until they are at moderate to severe stages.2 This is a substantial missed opportunity and can leave families unprepared for crisis situations and the future.
A systematic approach to dementia diagnosis provides a vital window of opportunity to both the physician and the family. In some cases, a reversible cause such as vitamin deficiency or adverse medication side effect can be identified and readily addressed. Comprehensive medical care can only begin after diagnosis and can improve quality of life and extend independence. Families can and should learn all about the diagnosed disorder, including symptoms, treatments and prognosis. Individuals diagnosed with dementia should also consult with legal and financial experts and have important discussions with their families about their wishes regarding medical decisions, long-term care and end of life issues.
What is Lewy body dementia?
Lewy body dementia is a brain disorder that affects thinking, movement, behavior, sleep, and automated body functions like blood pressure control, temperature regulation, and digestion. The Lewy Body Dementia Association (LBDA) estimates that approximately 1.3 million Americans have LBD, though the number of people diagnosed during life is far less. Autopsy studies indicate that at approximately 20 percent of all dementias cases are caused by LBD. The prognosis for a person with LBD is five to eight years after the presentation of clinical symptoms and may have a faster rate of decline than Alzheimer’s.
There are two clinical diagnoses that are included in the term Lewy body dementias. These disorders share very similar symptom profiles but have different patterns of symptom onset. “Dementia with Lewy bodies” is diagnosed when a person develops dementia and any other LBD symptoms before or within a year of developing Parkinson-like motor signs (parkinsonism). Conversely, people who have parkinsonism and are diagnosed with Parkinson’s disease may also develop dementia a year or more later. Those individuals are then diagnosed with Parkinson’s disease dementia.3 Both disorders have the same underlying disease pathology, the presence of Lewy bodies in multiple brain areas.
For simplicity’s sake, this article will use LBD to refer to both clinical diagnoses; where more clarification is needed, the specific form of LBD will be indicated.
A Complex Array of Symptoms
While Alzheimer’s and LBD both feature dementia as a main symptom, the cognitive domains that are affected differ considerably. Early in the disease course, LBD severely impacts executive function, such as the ability to solve problems or do abstract or analytical thinking. Visuospatial skills and attention are also impaired. However, in early Alzheimer’s disease, short-term memory loss is more pronounced.
The pathology of LBD and Alzheimer’s differs as well. LBD is associated with the presence of misfolded deposits of alpha-synuclein protein in the brain (called Lewy bodies). Alzheimer’s disease is associated with structural brain changes called plaques and tangles of two different proteins, beta-amyloid and tau.
A diagnosis of “dementia with Lewy bodies” requires that the person have dementia plus any two of the following symptoms:
Hallucinations: seeing or hearing things that are not really present
Cognitive fluctuations: unpredictable changes in concentration and attention
Parkinsonism: rigidity or stiffness, shuffling gait, tremor, slowness of movement (bradykinesia)
Severe sensitivity to neuroleptics (medications used to treat hallucinations and paranoid thinking)
REM sleep behavior disorder: a sleep disorder where people seemingly act out their dreams
Low dopamine transporter uptake in the brain’s basal ganglia as seen on SPECT and PET imaging scans.
Other symptoms commonly seen in dementia with Lewy bodies that support the diagnosis include:
Repeated falls and syncope (fainting)
Transient, unexplained loss of consciousness.
Hallucinations of other modalities.
Other psychiatric disturbances.
LBD can only be diagnosed with certainty after death, via a brain autopsy. Researchers are studying a variety of promising methods to diagnose LBD, but until reliable tests are widely available, doctors must rule out other causes for the dementia and identify clinical symptoms of LBD to make the diagnosis.
The wide array of cognitive, behavioral, movement, and autonomic symptoms can delay diagnosis of LBD if the physician has an incomplete picture. Many primary care physicians are not yet familiar with the rather complex diagnostic criteria for dementia with Lewy bodies. If physicians do not ask about the full range of LBD symptoms or if differentiating symptoms are too mild for the clinician to observe, a timely LBD diagnosis becomes difficult. Additionally, patients do not report some LBD symptoms that seem unrelated to their cognitive complaint. The Lewy Body Dementia Association has developed a symptom checklist to help clinicians and their patients identify key signs of Lewy body dementia. The Lewy Body Dementia (LBD) Diagnostic Symptoms: Patient Checklist can be downloaded for free at www.lbda.org/go/diagnoseLBD.
If LBD is suspected, a referral to a specialist is strongly recommended for differential diagnosis. A complete neuropsychological examination is helpful in the diagnostic process. These assessments are much more extensive and sensitive than routine office tests of mental status and can help differentiate among LBD, Alzheimer’s disease, the usually mild changes associated with normal aging, and other neurological conditions.
According to a survey by the Lewy Body Dementia Association of 962 caregivers, a typical LBD diagnosis can involve three or more doctors and approximately 6 office visits over the course of 12 to 18 months. These caregivers reported that only 6 percent of LBD diagnoses were made by primary care physicians. Specialists make the vast majority of LBD diagnoses, with 62 percent made by neurologists, but the majority of LBD patients were referred back to primary care more follow-up care.4 This underscores the importance of more education for primary care physicians about diagnosing and treating non-Alzheimer’s dementias.
Higher Caregiver Burden than Alzheimer’s
LBD affects many aspects of a person — their mood, the way they think, and the way they move. The combination of cognitive, motor, and behavioral symptoms creates a highly challenging set of demands for continuing care and can begin early in the disease course. LBD patients and families need considerable resources and assistance from health care professionals and agencies since caregiver burden may be higher in Lewy body dementias than in Alzheimer’s disease.
Individuals with LBD tend to require more resources than those with Alzheimer’s, specifically outpatient care, informal care, community resources, and pharmaceutical therapy.5 The cost of care for persons with LBD is usually higher than for those with Alzheimer’s.6 Crisis situations are common as well. In one study, 64 percent of LBD caregivers also reported that a crisis situation had occurred within the previous 12 months, with 73 percent involving a visit to the emergency room. 7
Up to three-quarters of all people with dementia with Lewy bodies will experience parkinsonism, with half having it at the time of diagnosis. Research indicates people with LBD are more functionally impaired than those with Alzheimer’s. These impairments are primarily due to parkinsonism and especially affect mobility and the ability to perform self-care.8
LBD caregivers report medium to high levels of subjective emotional burden associated with caring for a person with LBD. A sense of isolation is also common in those with LBD, which may be a result of low public awareness and low diagnostic rates.7
LBD is slightly more common in men than women, compared to Alzheimer’s disease, which is more common in women than men. Average mortality or age of death in LBD is approximately six years younger than Alzheimer’s. 9 Combined, these two facts may explain some of the difference in caregiver demographics between Alzheimer’s and LBD as well. The Alzheimer’s Association reports that caregivers of people with Alzheimer’s are 60 percent women, where in a survey of LBD caregivers by the Lewy Body Dementia Association, 88 percent of respondents were women. Only 6 percent of Alzheimer’s caregivers are spouses, while 40 percent of respondents to LBDA’s caregiver survey were spouses.7 This higher percentage of elderly spouses providing care for people with LBD underscores the importance of providing adequate support to these potentially vulnerable caregivers.
Despite the high toll of caregiving in LBD, most caregivers are not currently utilizing paid help to assist with care. In LBDA’s caregiver survey, only 29 percent of LBD caregivers reported using paid care in the home and only 21 percent used adult day services. Forty percent of caregivers reported needing additional help but 75 percent of them indicated that the cost of the services was a problem.7
There is a difference between spousal and non-spousal caregivers when it comes to utilizing community resources. Non-spousal caregivers are more likely to use meal services, while spousal caregivers are more likely to attend support groups, obtain case manager and legal services, and use hospice services.7
Symptom Management — A Delicate Balancing Act
Some symptoms of LBD are highly treatable, while others can pose more of a challenge. Nearly all medications prescribed for LBD are used off-label and were designed for other disorders.
Medications designed for cognitive symptoms of Alzheimer’s disease, acetylcholinesterase inhibitors (AChEIs), have been demonstrated to improve cognitive measures and may be effective in treating apathy and visual hallucinations. Memantine may also be helpful.
Levodopa is used to manage parkinsonism in LBD; however there is a risk of worsening behavioral and psychotic symptoms. If parkinsonism is mild, it may be preferable to not treat it. Other medications for motor symptoms in Parkinson’s disease may worsen cognition and should be avoided in LBD.
Atypical antipsychotic medications (such as quetiepine and clozapine) should be used sparingly and under close monitoring. (See Treating Behavioral Disturbances below.)
REM sleep behavior disorder is responsive to treatment with melatonin or clonazepam.
SSRIs/SNRIs are commonly used to treat depression and anxiety in LBD.
Other important symptoms to manage include constipation and orthostatic hypotension.
Not all treatments involve medications. Physical therapy options include gait training, cardiovascular strengthening, and flexibility exercises. Physicians may also recommend exercise programs such as aerobic exercise, strengthening programs, or water exercise. LBD patients may be responsive to speech therapy for low voice volume and poor enunciation. Speech therapy may also improve inspiratory muscular strength and swallowing difficulties. Occupational therapy may help maintain skills and promote function and independence.
For more detailed information on LBD therapeutics, visit http://www.lbda.org/content/therapeutics.
Treating Behavioral Disturbances — Less is Better
Of special importance are the medication sensitivities common in LBD as it pertains to antipsychotic medications. Visual hallucinations occur in up to 80 percent of people with LBD and the goal of addressing any behavioral disturbances in LBD is to ensure the safety of the patient and others. If the hallucinations are not frightening, treatment with medications may not be needed.
Up to 50 percent of people with LBD who are treated with any antipsychotic medication have a severe reaction, including worsening cognition, sedation, increased or possibly irreversible acute onset parkinsonism or symptoms resembling neuroleptic malignant syndrome, which can be fatal. Typical antipsychotic medications (such as haloperidol) should be avoided due to the risk of neuroleptic malignant syndrome, parkinsonism, somnolence, and orthostatic hypotension. Atypical antipsychotic medications (such as quetiepine and clozapine) should be used only in situations where the safety of the patient or others is at risk and require close monitoring.
Resources for you and your patients
Get essential information at your fingertips quickly and easily. Visit the Lewy Body Dementia Association (LBDA) at www.lbda.org for more clinical information on LBD, as well as treatment recommendations, patient and caregiver handouts, and more. Please refer your LBD patients and caregivers to LBDA for educational and support resources, such as local and virtual support groups, educational webinars and publications, online communities and more.
The author would like to thank Dr. Daniel Kaufer for serving as technical consultant on this article and to the LBD community for providing vital feedback to the Lewy Body Dementia Association about the impact of LBD on caregivers.
Plassman BL, Kanga KM, Fisher GG, et al. Prevalence of dementia in the United States: the aging demographics and memory study. Neuroepidemiology. 2007;29:125-32.
Mitchell A. The clinical significance of subjective memory complaints in the diagnosis of mild cognitive impairment and dementia: a meta-analysis. Int J Geriatr Psychiatry. 2008;23:1191-2012.
McKeith IG, Dickson DW, Lowe DM, et al. Diagnosis and management of dementia with Lewy bodies; Third report of the DLB consortium. Neurology. Dec 2005;65(12):1863-72.
Galvin JE, Duda JE, Kaufer DI, Lippa CF, Taylor A, Zarit SH. Lewy body dementia: the caregiver experience of clinical care. Parkinsonism Relat Disord. Jul 2010;16(6):388-92.
Bostrom F, Jonsson L, Minthon L, Londos E. Patients with dementia with lewy bodies use more resources than those with Alzheimer’s disease. Alzheimer Dis Assoc Disord. Aug 2007;22(8):713-9.
Zhu CW, Scarmeas N, Stavitsky K, et al. Comparison of costs of care between patients with Alzheimer’s disease and dementia with Lewy bodies. Alzheimers Dement. Jul 2008;4(4):280-4.
Galvin JE, Duda JE, Kaufer DI, Lippa CF, Taylor A, Zarit SH. Lewy body dementia; caregiver burden and unmet need. Alzheimer Dis Assoc Disord. Apr-Jun 2009;24(2):177-81.
McKeith IG, Rowan E, Askew K, et al. More severe functional impairment in dementia with lewy bodies than Alzheimer disease is related to extrapyramidal motor dysfunction. Am J Geriatr Psychiatry. Jul 2006;14(7):582-8.
Williams MM, Xiong C, Morris J, Galvin JE. Survival and mortality differences between dementia with Lewy bodies vs Alzheimer disease. Neurology. Dec 2006;12(67):1935-41.
About the Author: Angela Taylor is the Director of Programs for the Lewy Body Dementia Association. In 2004, Angela joined LBDA as a member of LBDA’s Board of Directors when she was a caregiver for her father who had Lewy body dementia. Angela oversees all of LBDA’s programs and services and serves as the liaison to the Scientific Advisory Council. She can be reached at firstname.lastname@example.org.