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PubMed1990

Age-related cognitive recovery after general anesthesia.

This study was designed to quantify the rate of mental recovery in elderly and young patients after general anesthesia for intraabdominal surgery (cholecystectomy). Forty patients (25-83 yr) were given four tests assessing neuropsychological function once preoperatively and on five occasions postoperatively. Two of the four neuropsychological tests showed impairment in scores in the elderly patients on the first postoperative day (Symbol Digit Modalities Test, P less than 0.004; The Trail Making Test, P less than 0.03). In addition, one of the tests (Symbol Digit Modalities Test) showed a deterioration in the younger patients (P less than 0.05). The changes that did occur in these tests on the first postoperative day reverted to baseline levels thereafter. There were no significant changes in the remaining two tests, the Mini Mental State Test or the Digit Span Test, at any time in either group. We conclude that postoperative mental deterioration is no greater in elderly than in young patients.

Adult↗
PubMedDate not supplied

[Executive functioning in unipolar depression: a review].

While several neuropsychological studies have demonstrated that cognitive deficits are seen across a broad range of cognitive domains, executive deficits associated with frontal lobe dysfunction may be prominent in depression. Executive function refers to cognitive processes that control and integrate other cognitive activities such as episodic memory. These executive functions involve a set of cognitive behaviors which include: dealing with novelty, selecting strategies, inhibiting incorrect responses, monitoring performance and using feedback to adjust future responding. The measurement of executive function relies mainly on the use of neuropsychological tests known to be sensitive to frontal lobe damage such as the Wisconsin and California Card Sorting Tests, verbal fluency tests, Stroop-test, Tower of London Task and Trail Making Test. The present review focuses on studies investigating executive functions in primary unipolar depression with these neuropsychological tasks. Unipolar depressed patients mainly exhibit cognitive inhibition deficits, problem-solving impairments and planning deficits. Cognitive inhibition deficits in depressed patients have been related to a reduction of cognitive resources and psychomotor retardation. Inhibition disturbance could lead depressed patients to process irrelevant information and consequently reduce their capacity to control transient mood changes. Several studies have found evidence of problem solving impairments in depressed patients. Depressed subjects show with card sorting tests difficulties in hypothesis testing with a loss of spontaneous and reactive cognitive flexibility. The cognitive rigidity and hypothesis-testing associated with dorsolateral prefrontal dysfunction in depression may prevent patients to cope with life events and lead to a perpetuation of depressed mood by a continuation of stress exposure. Planning tasks, such as the Tower of London Test, also demonstrate that depressed patients fail to use negative feedback as a motivational boost to improve their performance. Both trait and state factors influence the executive level of depressed patients. Executive deficits have been reported in more severely depressed subjects with melancholic or psychotic features. Executive functioning also might predict a poorer outcome in depression. Thus initiation and perseveration scores - a measure of cognitive flexibility - is associated with relapse and recurrence of depression and residual depressive symptoms. Brain imaging studies show that reduced blood flow, particularly in medial prefrontal cortex and dorsal anterior cingulate cortex subserve executive impairments in depression. However neuroimaging studies underscore the importance of mood-cognitive interactions in depression. A recent working model of depression (Mayberg et al., 1999) implicates failure of the coordinated interactions of distributed cortical-limbic pathways in the neuropsychopathology of depression. According to this model, neocortical (prefrontal and parietal regions) and superior limbic elements (dorsal anterior cingulate) are postulated to mediate impaired attention and executive function, whereas ventral limbic regions (ventral anterior cingulate, subcortical structures) are postulated to mediate circadian and vegetative aspects of depression. Further studies are needed to validate this model at the neuropsychological level as well as the brain level and to elucidate the complex interactions between mood, cognitive resources and executive function in depression.

Cognition Disorders↗
PubMedDate not supplied

[Early postoperative assessment of cognitive functions after stereotactic pallidotomy in patients with primary dystonia - preliminary results].

BACKGROUND AND PURPOSE: The newest studies notify that globus pallidus, besides the motor control, plays an important role in cognitive functioning of: memory, attention, linguistic skills, visuospatial ability, and executive functions. Stereotactic pallidotomy (the lesion of the motor region of the globus pallidus) is one of the surgery options that is used in the treatment of the primary dystonia. Motor region is located in the postero-ventral part of the internal globus pallidus. The goal of the study was to assess of the influence of pallidotomy on cognitive functioning in the group of patients suffering from the primary dystonia. MATERIAL AND METHODS: Eighteen patients diagnosed with primary dystonia were treated by stereotactic pallidotomy and they were investigated neuropsychologically. The study was performed between March 2004 and February 2005. Neuropsychological assessment was conducted 1-2 days prior to operation and 2 days following the surgery. The clinical course of the cognitive functions and emotional status were assessed by the following neuropsychological tests: Mini Mental State Examination (MMSE), Trail Making Test A and B (TMT A and B), Stroop Color-Word Interference Test, N-back Test, Auditory Verbal Learning Test (AVLT), Benton Visual Retention Test (BVRT), Wisconsin Card Sorting Test (WCST), Beck Depression Inventory (BDI), Montgomery-Asberg Depression Rating Scale (MADRS). RESULTS: The statistically significant differences have been obtained between the results before and after the surgery in the depression scales: BDI (p<0.02) and in MADRS (p<0.01). None of the neuropsychological tests revealed significant differences between the results before and after the surgery. The only trend (p=0.06-0.07) was noted between results of AVLT before and after the surgery. The average results were worse after the surgery in the first, fifth and sixth attempt of the reply. CONCLUSIONS: Stereotactic pallidotomy has not significantly changed cognitive functions in the patients suffering from primary dystonia. Whereas, the emotional state has significantly improved in consequence of the applying surgery. Stereotactic pallidotomy in primary dystonia seems to be a treatment option safe for the patients' cognitive functions.

Adult↗
PubMed2006

Visual search and eye movements in patients with chronic solvent-induced toxic encephalopathy.

Various aspects of visual perception have been found to be impaired in patients with occupational chronic solvent-induced toxic encephalopathy (CSE). The purpose of the study was to characterise the changes in eye movements and visual search performance in CSE patients. We measured eye movements of 13 CSE patients and 22 healthy controls during dynamic visual search task by using a fast video eye tracker. The task was to search for and identify a target letter among numerals presented in a rectangular stimulus matrix (3x3-10x10 items). Threshold search time, i.e. the duration of stimulus presentation required for identifying the target with a given probability was determined by using a psychophysical staircase method. The visual search times of the CSE patients were clearly longer, and they needed considerably more eye fixations than healthy controls to find the target. Thus, their reduced performance in this task was mainly related to the reduction in the number of items which could be processed during a single eye fixation (perceptual span). This reduction probably reflects a limited capacity of visual attention, since visual acuity, contrast sensitivity, and the oculomotor saccade velocity were found to be normal. The results suggest that motor slowness or low-level visual factors do not explain the poor performance of CSE patients in visual search tasks. The results are also discussed with respect to the effects of education, and compared to the performance in the widely used neuropsychological Trail Making Test, which uses similar stimuli and requires visual search.

Aged↗
PubMed2001

Short cognitive/neuropsychological test battery for first-tier fitness-to-drive assessment of older adults.

Since fitness-to-drive evaluation of elderly drivers has become an important issue, we developed a short first-tier screening battery to evaluate the necessity for further referral to specialised centres. Our sample consisted of 84 subjects between 65 and 96 years who came to the Belgian Road Safety institute for a fitness-to-drive evaluation. Using cross-validated discriminant analyses, the predictive power of a battery consisting of the Trail Making Test, Part A, a visual acuity test, a clock drawing test, the Mini-Mental State Examination (MMSE) and age was analysed. The judgement by an independent driver instructor (fit-to-drive vs. not unconditional fit-to-drive), based on a real world road test was used as the dependent variable. Classification functions based on the significant discriminant function yielded a specificity score of 85% (subjects fit-to-drive correctly classified) and a sensitivity score of 80% (subjects as not unconditional fit-to-drive correctly classified). These results highlight the potential value of a short screening instrument that can be used in primary health care settings. This instrument may be useful as a first step in a multi-tier assessment procedure.

Aged↗
PubMed2004

Trail making and olfaction in schizophrenia: implications for processing speed.

BACKGROUND: Previous research has established a relationship between smell identification deficits (SID) and particular aspects of cognitive function among patients with schizophrenia. OBJECTIVE: To expand the extant literature, we examined the relationship between SID and the Trail Making Test to determine if processing speed is related to SID. METHODS: Our sample included 60 inpatients from the New York State Psychiatric Institute's Schizophrenia Research Unit. We considered age, deficit syndrome, verbal intelligence quotient, and education in our analyses due to their documented relationship to smell identification ability. RESULTS: Trails A errors and Trails A seconds accounted for a significant amount of the variance in University of Pennsylvania Smell Identification Test scores in a regression analysis (R2=.10, P=.008 and R2=.05, P=.04). CONCLUSION: Linking neurocognition to smell identification deficits may prove to be an essential marker for schizophrenia research.

Adult↗
PubMed1982

Neuropsychological dysfunction following elective cardiac operation. II. A six-month reassessment.

Three neuropsychological tests were administered to 245 men and women, ages 25 to 69 years, before and 6 months after coronary bypass and cardiac valve operations to provide current information regarding the incidence of long-term postoperative decrements in neuropsychological function and the factors associated with them. Biographical, psychological and medical-surgical data were studied together with changes on the Trail Making Test from the Halstead-Reitan Battery, and Visual Reproduction (VR) and Logical Memory tests, both from the Wechsler Memory Scale (WMS). Although 28% of this group showed a deterioration in one or more test scores at a 9 day postoperative examination as compared to their preoperative scores, over 80% of these patients had returned to normal range by 6 months. Similarly, the majority of the 19% of patients showing a significant decrease in one or more of four scores at 6 months had incurred their performance decrements subsequent to the 9 day examination. Hence it seems inappropriate to attribute these latter dysfunctions to the surgical epidose per se, as others have reported. Only 5% of patients showed consistent postoperative test score deterioration both at 9 days and 6 months. Decrements of function at 6 months appear to be associated with total estimated blood loss greater than 3,000 ml and administration of propranolol during the operation plus several postoperative factors including higher levels of fatigue, depression, and worries related to the operation and the recovery process. These findings underscore the need for clinicians and investigators studying neuropsychological dysfunction following cardiac operations to take concurrent emotional and physical states into account, and to make repeated measures well separated in time, before interpreting the presence or absence of residual neuropsychological problems.

Coronary Artery Bypass↗
PubMed2005

Executive function correlates with walking speed in older persons: the InCHIANTI study.

OBJECTIVES: To study the association between performance on psychological tests of executive function and performance on lower extremity tasks with different attentional demands in a large sample of nondemented, older adults. DESIGN: Cross-sectional study. SETTING: Community-based. PARTICIPANTS: Nine hundred twenty-six persons aged 65 and older, without dementia, stroke, parkinsonism, visual impairment, or current treatment with neuroleptics, enrolled in a large epidemiological study. MEASUREMENTS: Trail Making Test (TMT) parts A and B and two performance-based measures of lower extremity function that require different executive/attentional-demanding skills: walking speed on a 4-m course at usual pace and walking speed on a 7-m obstacle course at fast pace. A difference score (Delta TMT), obtained by subtracting time to perform part A from time to perform part B of the TMT, was used as an indicator of executive function. Based on Delta TMT, subjects were divided into poor performance, intermediate performance, and good performance. RESULTS: After adjustment, no association between Delta TMT and 4-m course usual-pace walking speed was found. Participants with poor Delta TMT and with intermediate Delta TMT performance were more likely to be in the lowest tertile for 7-m obstacle course walking speed. CONCLUSION: In nondemented older persons, executive function is independently associated with tasks of lower extremity function that require high attentional demand.

Aged↗
PubMed2004

Anxious-depression in boys: an evaluation of executive functioning.

The effects of anxiety and depression on frontal lobe functioning were tested in two groups of 9-11-year-old boys. Participants were screened for handedness, health, intelligence and classified as anxious-depressed or non-anxious, non-depressed based on scores from the A-State scale of the State-Trait Anxiety Inventory for Children and the Child Depression Inventory. Previous research in our laboratory has indicated that boys high in anxious-depression may have neuropsychological deficits [e.g., Emerson, C. S., Harrison, D. W., & Everhart, D. E. (1999). Investigation of receptive affective prosodic ability in school-aged boys with and without depression. Neuropsychiatry, Neuropsychology and Behavioral Neurology, 12(2), 102-109; Emerson, C. S., Harrison, D. W., Everhart, D. E., & Williamson, J. B. (2001). Grip strength asymmetry in depressed boys. Neuropsychiatry, Neuropsychology, and Behavioral Neurology, 14(2), 130-134]. In order to assess the effects of anxious-depression on cerebral functioning performance on the Trail Making Test (Forms A and B) and on the Concept Formation subtest of the Woodcock Johnson was compared between groups. As predicted, anxious-depressed boys demonstrated deficits in sequencing, alternation, and problem-solving tasks as evidenced by longer completion times and significantly more errors on the tests. These results provide supportive evidence for deficits in frontal lobe functioning.

Anxiety Disorders↗
PubMed1996

Abnormal subjective experiences in schizophrenia: its relationships with neuropsychological disturbances and frontal signs.

The prevalence of abnormal subjective experiences is high in schizophrenic patients. This study starts from the hypothesis that these subjective disorders are associated with cognitive disturbances. In order to test this hypothesis a study was carried out on 40 patients who were diagnosed as suffering from schizophrenia according to RDC and DSM-III-R criteria. They were consecutively admitted due to a recrudescence of their symptomatology. Version 3 of the Frankfurt Complaint Questionnaire, adapted for Spanish by the authors, was used as an instrument for the assessment of subjective experiences. Eight patients refused to answer the questionnaire. A neuropsychological battery, including the Mini-Mental State, some subtests from the WAIS, Bender's visual-motor test, Rey's Complex Figure, and the Trail Making Test, was used. Frontal neurological signs were evaluated. The abnormal subjective experiences of schizophrenics presented a close association with neuropsychological disturbances and, to a lesser degree, of intensity with frontal neurological signs. Regression analyses by means of stepwise method and partial correlation analyses were undertaken. Many significant relationships between subjective experiences and cognitive disturbances persisted after controlling the influence of age, gender, education, lack of insight, neuroleptic doses, and biperidene doses.

Adult↗
PubMed2006

PET imaging of cortical 11C-nicotine binding correlates with the cognitive function of attention in Alzheimer's disease.

RATIONALE: Patients suffering from Alzheimer's disease (AD) experience a marked reduction in cortical nicotinic acetylcholine receptors (nAChRs). In particular, selective loss of the alpha4beta2 nAChR subtype was observed in postmortem AD brain tissue. The alpha4 and alpha7 nAChR subunits were suggested to play an important role in cognitive function. Positron emission tomography (PET) has so far been used to visualize neuronal nAChRs in vivo by 11C-nicotine binding. OBJECTIVES: To investigate the relationship between measures of cognitive function and in vivo 11C-nicotine binding in mild AD brain as assessed by PET. MATERIALS AND METHODS: Twenty-seven patients with mild AD were recruited in this study. A dual tracer model with administration of 15O-water for regional cerebral blood flow and (S)(-)11C-nicotine was used to assess nicotine binding sites in the brain by PET. Cognitive function was assessed using neuropsychological tests of global cognition, episodic memory, attention, and visuospatial ability. RESULTS: Mean cortical 11C-nicotine binding significantly correlated with the results of attention tests [Digit Symbol test (r = -0.44 and p = 0.02) and Trail Making Test A (TMT-A) (r = 0.42 and p = 0.03)]. No significant correlation was observed between 11C-nicotine binding and the results of tests of episodic memory or visuospatial ability. Regional analysis showed that 11C-nicotine binding in the frontal and parietal cortex, which are the main areas for attention, correlated significantly with the Digit Symbol test and TMT-A results. CONCLUSION: Cortical nicotinic receptors in vivo in mild AD patients are robustly associated with the cognitive function of attention.

Aged↗
PubMed2004

The effect of growth hormone substitution on cognitive performance in adult patients with hypopituitarism.

OBJECTIVE: Adult hypopituitary patients with growth hormone deficiency, though on adequate adrenal, thyroid or sex hormone replacement therapy, complain of attention and memory disabilities. During the past years several studies have evidenced that growth hormone (GH) may exert distinctive effects on the central nervous system and induce beneficial effects on psychological capabilities. The aim of our study was to determine whether a long-term replacement therapy of recombinant human growth hormone (rhGH) affects cognitive performance in adults with GH deficiency. DESIGN: A double-blind, randomized placebo controlled trial over 6 months, followed by an open period of 6 months of rhGH treatment. MEASUREMENTS: The assessment of cognitive performance comprised attention, verbal memory and non-verbal intelligence and was examined at baseline (0), at 3, 6, 9, and 12 months. In addition, emotional well-being and energy were assessed using the Nottingham Health Profile self rating questionnaire. PATIENTS: Eighteen hypopituitary patients, mean age 41.6 (range 21-63) years with adult onset GH deficiency were evaluated. Patients were on adequate and stable adrenal, thyroid, gonadal and desmopressin replacement therapy where necessary, but not substituted for GH deficiency. RESULTS: After 3 and 6 months of rhGH treatment in the closed label phase a significant improvement of attentional performance was observed compared to baseline in the rhGH group but not in the placebo group. After 6 months scores of attention were significantly different between rhGH and placebo treatment for the digit cancellation test and marginally different for the trail-making test. In contrast, long-term verbal memory and non-verbal intelligence did not improve compared to baseline during therapy and short-term memory improved both in the GH and the placebo group after 3 and 6 months. This was considered as a placebo or practice effect. In the open-label phase a further improvement of attention was found in the GH group and subsequent treatment with rhGH for 3 and 6 months in the placebo group also significantly improved attentional performance supporting the results of the rhGH group in the first 6 months of the double-blind phase. CONCLUSION: RhGH treatment appears to have a beneficial effect on attentional performance in adult hypopituitary patients with GH deficiency when treated for at least 3 months. Our study does not support a role for GH in influencing verbal memory or non-verbal intelligence.

Adult↗
PubMed2001

Associations between apolipoprotein E phenotype, glucose metabolism and cognitive function in men. An explorative study in a population sample.

AIMS: To investigate the associations of the apolipoprotein E phenotype (apoE) and disturbed glucose metabolism with cognitive function in a random population sample. METHODS: A cross-sectional study was conducted, in which 528 men aged 54 or 60 years were recruited randomly from a larger population-based sample of 1516 men. A subject was defined as having abnormal glucose tolerance (AGT), if he had a clinical diagnosis of diabetes, with either dietary or oral antidiabetic treatment or showed impaired glucose tolerance in an oral glucose tolerance test. The subjects were divided into three groups according to apolipoprotein E phenotypes: (a) E2/4, E3/4 or E4/4 (apoE E4); (b) E 3/3 (apoE E3); and (c) E2/2 or E2/3 (apoE E2). Memory function was examined using a word-list learning with Buschke's selective reminding method and test. Executive functions were assessed with the Trail Making Test A and B. RESULTS: Those subjects with apoE E2 and abnormal glucose metabolism demonstrated the worst cognitive executive control compared to other groups. Simple cognitive speed did not differ between the groups. CONCLUSIONS: The exploratory analyses revealed that subjects with apoE E2 allele and AGT had worse glycaemic control and cognitive executive control compared to other groups. Different apolipoprotein phenotypes together with impaired glucose tolerance may have different cumulative adverse effects on age-related cognitive performance. Some subgroups of subjects may be especially vulnerable to cognitive impairment.

Apolipoprotein E3↗
PubMed2006

Differential impact of executive function on visual memory tasks.

Despite their common use in neuropsychological evaluation, little is known about the differential contribution of executive functioning to visual memory tests. In this study, hierarchical regression was used to determine the role of executive functioning on the Visual Reproduction subtest of the Wechsler Memory Scale--Third Edition, and the Rey-Osterrieth Complex Figure (ROCF) in a mixed neurological sample of 193 patients. Executive functioning was predictive of Visual Reproduction but not ROCF recall variables after accounting for demographic variables and global cognitive functioning. Only executive tests with a visuospatial component, the Trail-Making Test Part B and Wisconsin Card Sorting Test perseverative responses, were predictive of recall of Visual Reproduction stimuli. Organization of the ROCF was predictive of both Visual Reproduction and ROCF recall. These findings increase our understanding of the executive contribution to two common visual memory tests and may aid in the clinical interpretation of seemingly discrepant visual memory performance.

Adult↗
PubMed2001

Childhood socioeconomic position and cognitive function in adulthood.

BACKGROUND: Risk of dementia and Alzheimer's disease is higher among adults with limited education, and the less educated perform poorer on cognitive function tests. This study determines whether the socioeconomic environment experienced during childhood has an impact on cognitive functioning in middle age. METHODS: A population-based study of eastern Finnish men (n = 496) aged 58 and 64 for whom there were data on parent's socioeconomic position (SEP), their own education level, and performance on neuropsychological tests. Cognitive function was measured using the Trail Making Test, the Selective Reminding Test, the Verbal Fluency Test, the Visual Reproduction Test, and the Mini Mental State Exam. RESULTS: We found a significant and graded association between parental SEP (combined as an index) and cognitive function both prior to and after adjustment for respondent's education. Those from more disadvantaged backgrounds exhibited the poorest performance. When the separate components of the parental SEP measure were used, father's occupation and mother's education were independently associated with the respondent's score for three and five of the tests, respectively (there was no association with father's education and mother's occupation). After adjustment for the respondent's education, father's occupation was no longer associated with respondent's test score, however, the results were essentially unchanged for mother's education. CONCLUSIONS: Higher SEP during childhood and greater educational attainment are both associated with cognitive function in adulthood, with mothers and fathers each contributing to their offspring's formative cognitive development and later life cognitive ability (albeit in different ways). Improvements in both parental socioeconomic circumstances and the educational attainment of their offspring could possibly enhance cognitive function and decrease risk of dementia later in life.

Adult↗
PubMed2002

Long-term benzodiazepine use and cognitive decline in the elderly: the Epidemiology of Vascular Aging Study.

Several cross-sectional studies have found cognitive impairment in subjects taking benzodiazepines for long periods. However, it is not known whether long-term use of benzodiazepines accelerates cognitive decline in the elderly. The authors addressed this issue in a follow-up study of 1,389 people aged 60 to 70 years recruited from the electoral rolls of the city of Nantes (Epidemiology of Vascular Aging study). Data on cognitive functioning (five cognitive tests), depressive symptoms (Center for Epidemiologic Studies-Depression Scale), anxiety symptoms (Spielberger Inventory Scale), use of psychotropics and other drugs, and tobacco and alcohol consumption were collected at baseline, 2-year, and 4-year examinations. People reporting to take benzodiazepines at one, two and three examinations were classified as episodic, recurrent, and chronic users, respectively. Among the 1,176 subjects (85%) who participated in the three examinations, the proportions of episodic, recurrent, and chronic users were 10%, 6%, and 7%, respectively. Chronic users of benzodiazepines had a significantly higher risk of cognitive decline in the global cognitive test (Mini Mental State Examination) and the two attention tests than nonusers (Mini Mental State Examination: odds ratio [OR] [95% confidence interval (CI)] = 1.9 [1.0-3.5]; Digit Symbol Substitution test: OR [95% CI] = 2.7 [1.6-4.7]; Trail Making test, part B: OR [95% CI] = 2.1 [1.2-3.7]). Overall, episodic and recurrent users had lower cognitive scores than nonusers, but differences were not statistically significant. These results were independent of age, sex, education, alcohol and tobacco use, anxiety and depression scores, and use of psychotropic drugs other than benzodiazepines. The findings suggest that long-term use of benzodiazepines is risk factor of increased cognitive decline in the elderly.

Aged↗
PubMed1993

Regional brain atrophy in HIV-1 infection: association with specific neuropsychological test performance.

Quantified magnetic resonance imaging (MRI) was related to neuropsychological (NP) test scores in an asymptomatic HIV-1 seropositive group, a non-demented AIDS/ARC group, a group of subjects with HIV-1 dementia, and a seronegative control group. The MRIs were quantified using three planimetric measures of brain structure: the bicaudate ratio (a measure of caudate region atrophy), the bifrontal ratio (a measure of frontal region atrophy), and the ventricle to brain ratio (a measure of overall cerebral atrophy). Cognitive performance was assessed with standard NP tests. Significant correlations between the MRI ratios and many of the NP tests were observed. Of the tests grooved pegboard, part B of the trail making test, the verbal fluency test, and the digit span forward were associated with MRI abnormalities. The bicaudate ratio was most closely associated with the NP tests. These findings indicate that ventricular enlargement, especially in the region of the caudate, is closely related to poor NP test performance in HIV-1 infection.

Acquired Immunodeficiency Syndrome↗
PubMedDate not supplied

Cognitive alterations in non-demented CADASIL patients.

CADASIL is an inherited small-artery disease of the brain due to mutations of the Notch3 gene on chromosome 19. It is characterized by strokes, migraine with aura, and severe mood disturbances during mid adulthood and leads progressively to subcortical dementia. The precise onset of the cognitive decline in CADASIL remains unknown. We report here the cognitive evaluation of 8 non-demented symptomatic patients with CADASIL from 35 to 66 years of age. Altered performances were found in all subjects with the Wisconsin Card-Sorting Test (WCST), in 5/8 with the Trail-Making Test, and in 3/8 with copying of Rey's figure. Altered performances with codes and similarities of the WAIS-R, the Wechsler Memory Scale, Raven's Progressive Matrices, and the category and letter fluency task were observed less frequently (n < or = 2). The score obtained with the WCST was not significantly correlated with the severity of the white-matter or basal ganglia signal abnormalities at MRI examination. Our data show that: (1) symptomatic CADASIL patients, although non-demented, can present with a subtle cognitive impairment; (2) tasks involving the frontal lobes are found most frequently altered, and (3) this subtle cognitive deficit can develop in the absence of major vascular events and does not appear to be correlated with the severity of brain lesions as seen at MRI examination.

Adult↗