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Progression of Symptoms in the Early and Middle Stages of Huntington Disease
Sandra Close Kirkwood, PhD;
Jessica L. Su, MS;
P. Michael Conneally, PhD;
Tatiana Foroud, PhD
Arch Neurol. 2001;58:273-278.
Objective To delineate the progression of symptoms in the early and middle stages
of Huntington disease (HD).
Design A survey of individuals with symptomatic HD completed by a first-degree
relative.
Setting The National Huntington Disease Research Roster for Patients and Families,
Indianapolis, Ind.
Participants The survey included 1238 individuals with a minimum of a 6-year history
of symptomatic HD.
Measures Participating families completed a series of surveys, including the
Affected Individual Questionnaire, which consists of 19 physical, emotional,
and cognitive signs commonly thought to occur during disease progression.
The respondent indicates if each of the symptoms occurred and, if so, at what
time during the course of the disease: (1) within 1 year, (2) within 2 to
5 years, (3) within 6 to 10 years, (4) after more than 10 years, (5) has not
occurred, or (6) "don't know."
Results The symptoms are categorized into 6 onset periods. Involuntary movements
are grouped alone as the earliest reported symptom. The second group is composed
entirely of mental and emotional symptoms, including sadness, depression,
and difficult to get along with. The third group includes clumsiness, sexual
problems, lack of motivation, and suspiciousness/paranoia. As the disease
progresses, a variety of motor, emotional/behavioral, and cognitive symptoms
are experienced, including unsteadiness, trouble holding onto things, trouble
walking, changes in sleeping patterns, delusions and hallucinations, intellectual
decline, and memory loss. With the approach of late-stage HD, affected individuals
begin to experience speech difficulty and weight loss. In the late stage,
patients lose bowel and bladder control.
Conclusions Even though the symptoms of HD are fairly well characterized, their
progression, especially in the early and middle stages, remains uncertain.
Clarification of the disease progression is vital to improved understanding
of the pathogenesis of HD and to the evaluation of therapeutic agents that
are designed to slow the progression of disease. The results of this study
assist in clarifying HD progression from early involuntary movements and emotional
changes to more overt motor symptoms and difficulty with activities of daily
living.
From the Department of Medical and Molecular Genetics, Indiana University
School of Medicine, Indianapolis.
Corresponding author and reprints: Sandra Close Kirkwood, PhD, Department
of Medical and Molecular Genetics, Indiana University School of Medicine,
975 W Walnut St IB 130, Indianapolis, IN 46202 (e-mail: scloseki{at}iupui.edu).
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