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WESTPORT, CT (Reuters Health) Sept 05 2001 - "Early signs of Huntington's disease
can be detected using neuropsychological measures 2 years before the development of more manifest motor disease," Dr. Jane
S. Paulsen of the University of Iowa, Iowa City, tells Reuters Health.
She and colleagues with the Huntington Study
Group note in the August 28th
issue of Neurology that "there is increasing evidence that neuron loss precedes the phenotypic
expression of HD."
To investigate, they used the Unified Huntington's Disease Rating Scale (UHDRS) to prospectively
study 260 patients who had a parent with the disease.
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The subjects did not have manifest disease at baseline. "Repeat UHDRS data, obtained an
average of 2 years later, showed that 70 persons were given a diagnosis of definite HD based on the quantified neurologic
examination," according to the report.
Patients who did not show manifest disease during the followup period
showed improvement on all cognitive tests. In contrast, patients who showed conversion
to disease during the followup demonstrated a decline in every cognitive area. "All of the change rates remained significantly
different for converters and nonconverters (all p < 0.001)."
"Accurate and early detection of mid-and late-life
diseases is becoming more and more important as therapeutic options to slow disease progression or delay disease onset are
identified," Dr. Paulsen noted.
Neurology 2001;57:658-662.
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