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Extrapyramidal Signs in Dementia: What do they Mean?

http://www.bcm.tmc.edu/neurol/struct/adrc/adrc6.html

Alzheimer's disease (AD) is not the only illness that causes
progressive dementia. Other less common degenerative diseases of the
brain may also produce intellectual decline. Alzheimer's disease may
sometimes coexist with other conditions to give an atypical
presentation. When memory loss or disorientation is associated with
extrapyramidal signs (EPS) such as muscle stiffness, slowness of
movement, postural instability, gait imbalance, and tremor, special
attention is needed to rule out conditions other than AD. A precise
diagnosis is essential for proper management of care and planning for
the future.

When EPS occur before the onset of dementia and the patient's slowness
and stiffness respond to treatment with medication, Parkinson's
disease is the most likely diagnosis. Other patients experience a
rapid progression of gait imbalance, slowness of movement, muscle
stiffness, and dementia, and unfortunately, do not respond well to
treatment. These patients may have degeneration of brain cells caused
by progressive supranuclear palsy, striatonigral degeneration or
Creutzfeldt-Jakob disease. If the condition has a strong family
history, Huntington's disease, Wilson's disease and
Hallervorden-Spartz disease should be considered. Additionally,
multiple small strokes can sometimes damage the brain and cause the
appearance of EPS and dementia in patients. Most of these disorders,
with their unique cognitive changes, can be distinguished from AD by a
careful neurological examination and a comprehensive
neuropsychological evaluation.

Recently, several distinctive clinical features have been linked to a
condition called "diffuse Lewy body disease" (DLBD) that was
frequently mistaken for AD in the past. Cognitively, patients with
DLBD are usually only mildly affected, but they may have fluctuations
in their performance that exceed normal day to day variations. Visual
hallucinations, unexplained falls, dizziness and EPS may be prominent
and occur in early stages of the disease. The patients may also
exhibit changes in personality ( i.e. lack of interest and
depression), and difficulties with thinking ( i.e. problems with
attention, abstract thinking, decision-making, and reality). The
duration of illness is usually shorter for patients with "diffuse Lewy
body disease" than for those with AD. Distinctive differences are also
evident when brain tissue is examined at autopsy.

Both AD and Parkinson's disease are relatively common in the elderly
and one patient may be diagnosed with both illnesses. Treatment of
Parkinsonian features, such as tremor, slowness and muscle stiffness,
may improve the patients' quality of life substantially. Another
possible variant of AD, "Alzheimer's disease with extrapyramidal
signs," is not well understood. These patients experience a more rapid
deterioration of their symptoms, are frequently depressed, and
unfortunately, do not respond to anti-Parkinsonian treatment.

In summary, the presence of EPS in persons with dementia usually
suggests something atypical and patients may require more assistance
and care. A comprehensive evaluation is essential because the degree
of severity and the course of the illness may vary considerably. Even
with proper diagnosis and treatment, many questions remain unanswered.
How similar and different is AD from other dementing illnesses? What
are the causes of these devastating disorders? At this time, the
examination of brain tissues for large numbers of patients with
dementia is critical. Pathological findings can provide clues as to
how and why the brain is damaged. Such information will be invaluable
to the eventual discovery of a treatment, or even a cure, for
Alzheimer's disease and other degenerative diseases of the brain.
Hopefully, exciting breakthroughs will occur in the very near future.







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