Lewy Body Dementia Health Article

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.

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. eMedicine—Dementia 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