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Lewy Body Dementia

Lewy body dementia

Definition

Lewy body dementia (LBD) is a neurodegenerative disorder that can occur in persons older than 65 years of age, which typically causes symptoms of cognitive (thinking) impairment and abnormal behavioral changes.

Description

The condition was first described by Frederick Lewy in 1941 when he described Lewy bodies, which are abnormal inclusions in the cytoplasm (components of a cell outside the nucleus) of cells found in patients who had Parkinson's disease (PD). There is some controversy concerning the relationship between Lewy body dementia and Parkinson's disease. When cognitive impairment and behavioral disturbance are early and prominent symptoms, then LBD is the likely diagnosis. When motor symptoms are the predominant and early symptoms, then Parkinson's disease is likely to be the diagnosis. Typically, on autopsy examination of the brain, both PD and LBD would probably demonstrate Lewy bodies. Autopsy examination is the only method to available for a definitive diagnosis.

The signs and symptoms of LBD stem from a multi-factorial cause of disrupted bidirectional (two-way) information flow in neurons, especially those located in the frontal lobe; that is, there are abnormalities in the chemicals that regulate and pass on message signals between neurons in the brain. Alterations in neurotransmitter chemicals can also impair nerve cell circuitry, causing abnormalities in bidirectional information flow.

Most patients with LBD also have brain evidence of Alzheimer's disease pathology. Additionally, most patients with LBD possess amyloid plaques in their cerebral cortex. Lewy bodies can also occur in a genetically transmitted form of Alzheimer's disease, Pick's disease, and Down syndrome.

Demographics

Dementia (used as a general term) has been an increasingly common disorder that is especially more frequent in the elderly. Dementia affects 7% of the general population older than 65 years and that incidence increases with age to 30% of those age 80 years and older. Autopsy results in the United States estimate that LBD accounts for 1020% of dementia cases. Approximately 40% of patients with Alzheimer's disease also have LBD. Data from autopsy results in Europe and Japan reveal similar frequencies as reported in studies from the United States. No data is available concerning age, gender, or potential risk factors.

Causes and symptoms

The formation of Lewy bodies is thought to occur because of an abnormal increase in the production of a normally occurring protein in nerve cells called alpha-synuclein. Called upregulation, this overproduction can cause substances to accumulate or multiply in increased numbers. Other theories propose that alpha-synuclein may become insoluble (unable to mix in a watery environment), which could make the molecule more prone to accumulate abnormally in the brain.

Symptoms can include cognitive impairment, neurological signs, sleep disorder, and autonomic failure. Cognitive impairment is the presenting feature of LBD in most cases. Patients have recurrent episodes of confusion that progressively worsen. The fluctuation in cognitive ability is often associated with shifting degrees of attention and alertness. Cognitive impairment and fluctuations of thinking may vary over minutes, hours, or days.

Psychological manifestations

Psychological manifestations of LBD predominantly include:

  • delusions, false beliefs, or wrong judgments held to be true despite incontrovertible evidence to the contrary
  • visual hallucinations, strong subjective perception of an imaginary event or object
  • apathy, an indifference or absence of interest in the environment
  • anxiety, apprehension, or dread that causes symptoms of rapid heart rate, restlessness, tension, and shortness of breath

Neurological symptoms in patients affected with LBD include extrapyramidal features early in the disease. The extrapyramidal symptoms in LBD can be differentiated from other dementias such as Parkinson's disease. Patients affected with LBD tend to show axial involvement with greater postural instability and facial impassivity, and less tremor. Disorders of sleep in patients with LBD typically can include impairment of rapid-eye-movement (REM) sleep; REM sleep behavior disorder causes vivid and frightening dreams. Patients may also exhibit loss of muscle tone or cataplexy, hypersomnolence (an increased inclination to sleep), hallucinations, and narcolepsy . Patients with LBD also have deficits in the autonomic nervous system, part of which regulates specific body functions such as blood pressure and bladder control. Autonomic abnormalities can cause orthostatic hypotension and urinary incontinence.

Diagnosis

Clinically, patients have features of fluctuating cognitive impairment such as from alert to confused state, recurrent visual hallucination , depression , and REM sleep disorder. Patients may have impairment of memory retrieval and they often do poorly on tests that measure visuospatial skills such as copying figures or drawing a clock. Patients may have mild gait (walking) impairment. An accurate diagnosis can include identification of target symptoms, including cognitive impairment, psychological disorders (hallucinations, depression, sleep disorder, and behavioral disturbances), extrapyramidal motor features or autonomic dysfunction (orthostatic hypotension), or urinary incontinence. Standard blood tests are ordered and additional tests are typically required, including thyroid studies, vitamin B-12 levels, and, if appropriate, tests for Lyme disease , syphilis, or HIV since these infections can affect the brain. Currently, there are no specific tests used to diagnose LBD. A magnetic resonance imaging (MRI) scan is indicated to distinguish LBD from another disorder called vascular dementia, which can present with similar clinical signs and symptoms. It is important to exclude diseases or drugs that can cause delirium .

Treatment team

The treatment team can be broad, including general practitioners, geriatric psychotherapists, emergency services, or movement disorder specialists. Additionally, the team can include family members, primary care practitioners, caregivers, and neurologists. Special consultations from a neurologist with special expertise in dementias may be appropriate for caregiver education.

Treatment

The management of LBD can be approached in four stages: accurate diagnosis, identification of target symptoms, nonpharmacological treatment, and pharmacological treatment. Nonpharmacological interventions include management of environment and other necessities associated with LBD patient care. Caregiving skills should be specifically tailored to the patient. Pharmacological treatment can include several different medications, most notably a class of drugs called cholinesterase inhibitors . These medications tend to increase a brain neurochemical called acetylcholine, which is an excitatory brain chemical that is decreased in persons with LBD. With a typical dose of a cholinesterase inhibitor (Donepezil or Aricept), the symptoms of visual hallucinations, apathy, anxiety, sleep disorder, and cognitive impairments can be improved. Generally, medications can be utilized to slow the rate of cognitive decline, treat agitation and hallucinations, treat depression, and improve cognition and/or alertness.

Recovery and rehabilitation

Generally, there are no dietary restrictions for persons affected with LBD, except for those who have swallowing impairment. Physical therapy and an exercise program can be useful to maintain mobility. There are potential problems for patients who drive a motor vehicle, and family members and caregivers should be advised.

Clinical trials

Currently, the National Institute of Neurological Disorders and Stroke (NINDS) supports research concerning diagnosis, prevention, and treatment. Research efforts studying the biological consequences of Lewy body formation and mechanisms of disease progression are funded by NINDS.

Prognosis

LBD is a slowly progressive chronic disorder. However, the rate of progression may be faster than in Alzheimer's disease. The disease is fatal from complications of poor nutrition, swallowing difficulties, and immobility.

Special concerns

Primary caregivers and family members require information concerning management of symptoms such as hallucinations, agitation, and cognitive changes. Children of patients with LBD may require genetic counseling. Family members should be aware that LBD affects job performance and medical leave of absence or early retirement may be advisable. Driving may become problematic and should be addressed with the medical treatment team, patient, and family.

Resources

BOOKS

Goetz, Christopher G., et al, eds. Textbook of Clinical Neurology, 1st ed. Philadelphia: W. B. Saunders Company, 1999.

Goldman, Lee, et al. Cecil's Textbook of Medicine, 21st ed. Philadelphia: W. B. Saunders Company, 2000.

PERIODICALS

McKeith, Ian. "Dementia with Lewy bodies." The Lancet Neurology 3, no. 1 (January 2004).

WEBSITES

Crystal, Howard A. eMedicineDementia with Lewy Bodies. November 11, 2003 (May 23, 2004). <http://www.emedicine.com/neuro/topic91.htm>.

Lewy Body Dementia. <http://www.alzheimer.ca> (May 23, 2004).

National Organization for Rare Disorders (NORD). <http://www.rarediseases.org> (May 23, 2004).

ORGANIZATIONS

National Institute on Aging, National Institutes of Health. Building 31, Room 5C27, Bethesda, MD 20892-2292. (301) 496-1752. <http://nih.gov/nia>.

Laith Farid Gulli, MD

Robert Ramirez, DO

Nicole Mallory, MS, PA-C

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