Cognitive changes in patients with
Huntington’s disease (HD) and
asymptomatic carriers of the HD mutation

Objective Objective information about the onset and progression of cognitive impairment in Huntington’s disease (HD) is very important in the light of appropriate outcome measures when conducting clinical trials. Therefore, we evaluated the progression of cognitive functions in HD patients and asymptomatic carriers of the HD mutation (AC) over a 2.5–year period.We also sought to detect the earliest markers of cognitive impairment in AC. Methods A prospective study comparing HD patients, clinically asymptomatic HD mutation–carriers (AC) and non–carriers (NC). These groups were examined three times during a period of 2.5 years. At baseline the study sample consisted of 49 subjects. Forty–two subjects (19 HD patients, 12 AC and 11 NC) completed three assessments. A battery of neuropsychological Tests measuring intelligence, attention, memory, language, visuospatial perception, and executive functions was performed. Results The performance of HD patients deteriorated on the following cognitive Tests: Symbol Digit Modalities Test (SDMT), Stroop Colour and Word, Boston Naming Test (BNT), Object and Space Perception and Trail Making Test–B. Longitudinal comparison of AC and NC revealed that performances on SDMT, Block Span, Digit Span Backwards, Hopkins Verbal Learning Test (Learning and delayed recall) and Conditional Associative Learning Test are impaired in AC. Conclusions Tasks measuring mainly attention, object and space perception and executive functions adequately assess the progression of HD disease. Other cognitive functions do not significantly deteriorate. Furthermore, problems in attention, working memory, verbal Learning, verbal long–term memory and Learning of random associations are the earliest cognitive manifestations in AC.

Cognitive changes in patients with Huntington’s disease (HD) and asymptomatic carriers of the HD mutation: A longitudinal follow-up study

Objective information
about the onset and progression
of cognitive impairment
in Huntington’s disease (HD) is
very important in the light of appropriate
outcome measures when

conducting clinical trials. Therefore,
we evaluated the progression
of cognitive functions in HD patients
and asymptomatic carriers
of the HD mutation (AC) over a
2.5–year period.We also sought to
detect the earliest markers of cognitive
impairment in AC. A prospective study comparing HD
patients, clinically asymptomatic
HD mutation–carriers (AC) and
non–carriers (NC). These groups
were examined three times during
a period of 2.5 years. At baseline
the study sample consisted of 49
subjects. Forty–two subjects (19 HD
patients, 12 AC and 11 NC) completed
three assessments. A battery
of neuropsychological Tests measuring
intelligence, attention, memory,
language, visuospatial perception,
and executive functions was
performed. The performance
of HD patients deteriorated
on the following cognitive Tests:
Symbol Digit Modalities Test
(SDMT), Stroop Colour and Word,
Boston Naming Test (BNT), Object
and Space Perception and Trail
Making Test–B. Longitudinal comparison
of AC and NC revealed that
performances on SDMT, Block
Span, Digit Span Backwards, Hopkins
Verbal Learning Test (Learning
and delayed recall) and Conditional
Associative Learning Test are
impaired in AC. Tasks
measuring mainly attention, object
and space perception and executive
functions adequately assess the
progression of HD disease. Other
cognitive functions do not significantly
deteriorate. Furthermore,
problems in attention, working
memory, verbal Learning, verbal
long–term memory and Learning of
random associations are the earliest
cognitive manifestations in AC.

Cognitive changes in patients with
Huntington’s disease (HD) and
asymptomatic carriers of the HD mutation

Objective Objective information about the onset and progression of cognitive impairment in Huntington’s disease (HD) is very important in the light of appropriate outcome measures when conducting clinical trials. Therefore, we evaluated the progression of cognitive functions in HD patients and asymptomatic carriers of the HD mutation (AC) over a 2.5–year period.We also sought to detect the earliest markers of cognitive impairment in AC. Methods A prospective study comparing HD patients, clinically asymptomatic HD mutation–carriers (AC) and non–carriers (NC). These groups were examined three times during a period of 2.5 years. At baseline the study sample consisted of 49 subjects. Forty–two subjects (19 HD patients, 12 AC and 11 NC) completed three assessments. A battery of neuropsychological Tests measuring intelligence, attention, memory, language, visuospatial perception, and executive functions was performed. Results The performance of HD patients deteriorated on the following cognitive Tests: Symbol Digit Modalities Test (SDMT), Stroop Colour and Word, Boston Naming Test (BNT), Object and Space Perception and Trail Making Test–B. Longitudinal comparison of AC and NC revealed that performances on SDMT, Block Span, Digit Span Backwards, Hopkins Verbal Learning Test (Learning and delayed recall) and Conditional Associative Learning Test are impaired in AC. Conclusions Tasks measuring mainly attention, object and space perception and executive functions adequately assess the progression of HD disease. Other cognitive functions do not significantly deteriorate. Furthermore, problems in attention, working memory, verbal Learning, verbal long–term memory and Learning of random associations are the earliest cognitive manifestations in AC.

Cognitive changes in patients with Huntington’s disease (HD) and asymptomatic carriers of the HD mutation: A longitudinal follow-up study

Objective information
about the onset and progression
of cognitive impairment
in Huntington’s disease (HD) is
very important in the light of appropriate
outcome measures when

conducting clinical trials. Therefore,
we evaluated the progression
of cognitive functions in HD patients
and asymptomatic carriers
of the HD mutation (AC) over a
2.5–year period.We also sought to
detect the earliest markers of cognitive
impairment in AC. A prospective study comparing HD
patients, clinically asymptomatic
HD mutation–carriers (AC) and
non–carriers (NC). These groups
were examined three times during
a period of 2.5 years. At baseline
the study sample consisted of 49
subjects. Forty–two subjects (19 HD
patients, 12 AC and 11 NC) completed
three assessments. A battery
of neuropsychological Tests measuring
intelligence, attention, memory,
language, visuospatial perception,
and executive functions was
performed. The performance
of HD patients deteriorated
on the following cognitive Tests:
Symbol Digit Modalities Test
(SDMT), Stroop Colour and Word,
Boston Naming Test (BNT), Object
and Space Perception and Trail
Making Test–B. Longitudinal comparison
of AC and NC revealed that
performances on SDMT, Block
Span, Digit Span Backwards, Hopkins
Verbal Learning Test (Learning
and delayed recall) and Conditional
Associative Learning Test are
impaired in AC. Tasks
measuring mainly attention, object
and space perception and executive
functions adequately assess the
progression of HD disease. Other
cognitive functions do not significantly
deteriorate. Furthermore,
problems in attention, working
memory, verbal Learning, verbal
long–term memory and Learning of
random associations are the earliest
cognitive manifestations in AC.

Cognitive changes in patients with
Huntington’s disease (HD) and
asymptomatic carriers of the HD mutation

Objective Objective information about the onset and progression of cognitive impairment in Huntington’s disease (HD) is very important in the light of appropriate outcome measures when conducting clinical trials. Therefore, we evaluated the progression of cognitive functions in HD patients and asymptomatic carriers of the HD mutation (AC) over a 2.5–year period.We also sought to detect the earliest markers of cognitive impairment in AC. Methods A prospective study comparing HD patients, clinically asymptomatic HD mutation–carriers (AC) and non–carriers (NC). These groups were examined three times during a period of 2.5 years. At baseline the study sample consisted of 49 subjects. Forty–two subjects (19 HD patients, 12 AC and 11 NC) completed three assessments. A battery of neuropsychological Tests measuring intelligence, attention, memory, language, visuospatial perception, and executive functions was performed. Results The performance of HD patients deteriorated on the following cognitive Tests: Symbol Digit Modalities Test (SDMT), Stroop Colour and Word, Boston Naming Test (BNT), Object and Space Perception and Trail Making Test–B. Longitudinal comparison of AC and NC revealed that performances on SDMT, Block Span, Digit Span Backwards, Hopkins Verbal Learning Test (Learning and delayed recall) and Conditional Associative Learning Test are impaired in AC. Conclusions Tasks measuring mainly attention, object and space perception and executive functions adequately assess the progression of HD disease. Other cognitive functions do not significantly deteriorate. Furthermore, problems in attention, working memory, verbal Learning, verbal long–term memory and Learning of random associations are the earliest cognitive manifestations in AC.

Cognitive changes in patients with Huntington’s disease (HD) and asymptomatic carriers of the HD mutation: A longitudinal follow-up study

Objective information
about the onset and progression
of cognitive impairment
in Huntington’s disease (HD) is
very important in the light of appropriate
outcome measures when

conducting clinical trials. Therefore,
we evaluated the progression
of cognitive functions in HD patients
and asymptomatic carriers
of the HD mutation (AC) over a
2.5–year period.We also sought to
detect the earliest markers of cognitive
impairment in AC. A prospective study comparing HD
patients, clinically asymptomatic
HD mutation–carriers (AC) and
non–carriers (NC). These groups
were examined three times during
a period of 2.5 years. At baseline
the study sample consisted of 49
subjects. Forty–two subjects (19 HD
patients, 12 AC and 11 NC) completed
three assessments. A battery
of neuropsychological Tests measuring
intelligence, attention, memory,
language, visuospatial perception,
and executive functions was
performed. The performance
of HD patients deteriorated
on the following cognitive Tests:
Symbol Digit Modalities Test
(SDMT), Stroop Colour and Word,
Boston Naming Test (BNT), Object
and Space Perception and Trail
Making Test–B. Longitudinal comparison
of AC and NC revealed that
performances on SDMT, Block
Span, Digit Span Backwards, Hopkins
Verbal Learning Test (Learning
and delayed recall) and Conditional
Associative Learning Test are
impaired in AC. Tasks
measuring mainly attention, object
and space perception and executive
functions adequately assess the
progression of HD disease. Other
cognitive functions do not significantly
deteriorate. Furthermore,
problems in attention, working
memory, verbal Learning, verbal
long–term memory and Learning of
random associations are the earliest
cognitive manifestations in AC.