The RAPID Neuropsychological Test Battery for Subjects Aged 50-89: From Norms to Cognitive Profile.

BACKGROUND/AIMS: It is well established that healthy adults obtain low performances when simultaneously interpreting the results of multiple tests. The aim of this study was to estimate the proportion of French-speaking healthy older adults with low scores for the RAPID (Réseau d'Aide au diagnostic et à la PrIse en charge des Détériorations cognitives et de maladies neurologiques chroniques en Franche-Comté et au niveau national) battery test and consider different combinations of test scores within a specific domain and across different domains. METHODS: The prevalence of low scores (i.e., ≤5th percentile) on the 14 RAPID primary measures was calculated from the RAPID normative sample (n = 476), based on 4 ages (50-89 years) and 3 levels of education. RESULTS: A high percentage (40.1%) of the normative sample obtained at least one or more low scores (i.e., false positives). In contrast, the risk of having low scores was much less important (<2%) when considering the combinations of 2 test-scores. CONCLUSION: Low scores are very common in healthy older subjects and are thus not necessarily pathological or indicative of truly impaired functioning. The information derived from a cognitive profile may provide a greater clinical relevance in an individual, since very few of the healthy older adults obtained low scores on combinations of 2 test-scores.

Executive and Attentional Disorders, Epilepsy and Porencephalic Cyst in Autosomal Recessive Cerebellar Ataxia Type 3 Due to ANO10 Mutation.

BACKGROUND: ANO10 mutations have recently been reported in autosomal recessive cerebellar ataxia type 3 (ARCA3). The objective of this study was to describe the phenotype of 2 siblings with compound heterozygous ANO10 mutations and progressive cerebellar ataxia, epilepsy, and cognitive impairment. A porencephalic cyst was also described in one of them and a coenzyme Q10 deficiency in the other one. METHODS: We performed neurological, neuropsychological, electromyographic, electroencephalic and MRI examinations in 2 siblings with compound heterozygous ANO10 mutations. RESULTS: We reported for the first time the neuropsychological profile of 2 ARCA3 patients showing an adult-onset executive and attentional syndrome. Both presented epilepsy. One of them presented a porencephalic cyst. CONCLUSION: These results suggest that executive and attentional disorders are impaired in ANO10 mutation. In addition, epilepsy and porencephalic cysts were also described in our ARCA3 patients, the cyst thus expanding the clinical phenotype of ARCA3 patients due to ANO10 mutation.

Left Prefrontal Repetitive Transcranial Magnetic Stimulation in a Logopenic Variant of Primary Progressive Aphasia: A Case Report.

BACKGROUND/AIMS: High frequency repetitive transcranial magnetic stimulation (hf-rTMS) improves language skills in Alzheimer's disease (AD). We report the use of hf-rTMS in a patient with logopenic primary progressive aphasia (LPPA) due to AD. METHOD: hf-rTMS was applied to the left dorsolateral prefrontal cortex of a LPPA patient. Cerebral perfusion, neuropsychological and linguistic performances were evaluated before and 1 month after hf-rTMS. RESULTS: The tolerance was good. Improvements on linguistic (fluency, naming, lesser paraphasia) and cognitive skills (Mini Mental State Examination, verbal memory free recall, speed processing) and cerebral perfusion were observed. CONCLUSION: hf-rTMS can be used in LPPA patients. A procognitive effect persisting several weeks after stimulation in LPPA patients was suggested and should therefore be evaluated in a clinical trial as an adjunctive therapeutic tool.

Conversion, dissociative amnesia, and Ganser syndrome in a case of "chameleon" syndrome: anatomo-functional findings.

The term "chameleon" was first used in the seventeenth century by Sydenham to describe a patient with a protean semiology. We report a single case of "chameleon" syndrome that challenges the current international criteria for somatoform disorders, dissociative amnesia, and Ganser syndrome. The florid symptoms were as follows: anterograde and retrograde amnesia (including semantic, episodic, and procedural deficits), loss of identity, atypical neuropsychological impairment (approximate answers), left sensitive and motor deficit, and left pseudochoreoathetosis movement disorders. Additional behavioral disorders included the following: anxiety, clouded consciousness, hallucinations, and "belle indifference". A single photon emission computed tomography examination showed bilateral temporal, frontal and a right caudate (in the head of the caudate nucleus) hypoperfusion concordant with a common mechanism of repression in these disorders.

Cognitive Impairment in Late-Life Depression: A Comparative Study of Healthy Older People, Late-Life Depression, and Mild Alzheimer's Disease Using Multivariate Base Rates of Low Scores.

Late-Life Depression (LLD) is often associated with cognitive impairment. However, distinction between cognitive impairment due to LLD and those due to normal aging or mild Alzheimer's Disease (AD) remain difficult. The aim of this study was to present and compare the multivariate base rates of low scores in LLD, mild AD, and healthy control groups on a battery of neuropsychological tests. Participants (ages 60-89) were 352 older healthy adults, 390 patients with LLD, and 234 patients with mild AD (i.e., MMSE ≥ 20). Multivariate base rates of low scores (i.e., ≤ 5th percentile) were calculated for each participant group within different cognitive domains (verbal episodic memory, executive skills, mental processing speed, constructional praxis, and language/semantic memory). Obtaining at least one low score was relatively common in healthy older people controls (from 9.4 to 17.6%), and may thus result in a large number of false positives. By contrast, having at least two low scores was unusual (from 0.3 to 4.6%) and seems to be a more reliable criterion for identifying cognitive impairment in LLD. Having at least three low memory scores was poorly associated with LLD (5.9%) compared to mild AD (76.1%) and may provide a useful way to differentiate between these two conditions [ χ ( 1 ) 2 = 329.8, p < 0.001; Odds Ratio = 50.7, 95% CI = 38.2-77.5]. The multivariate base rate information about low scores in healthy older people and mild AD may help clinicians to identify cognitive impairments in LLD patients, improve the clinical decision-making, and target those who require regular cognitive and clinical follow-up.

Neuropsychological and neuroimaging phenotype induced by a CAMTA1 mutation.

BACKGROUND/AIMS: CAMTA1 mutations have recently been reported in families with intellectual disability and/or non-progressive congenital ataxias. The objective of this study was to describe the neuropsychological and neuroimaging phenotype of CAMTA1 mutation. METHODS: We performed neuropsychological examinations, MRI and FDG-PET imaging in three patients with autosomal dominant mild intellectual disabilities and ataxia induced by a CAMTA1 intragenic deletion at 1p36.31p36.23. RESULTS: Neuropsychological tests showed similar findings in two patients, with low information processing speed, slow memory consolidation, phonological disorders, working memory deficits, but mainly preserved executive function. Bilateral parietal and medial temporal abnormalities were found on brain MRI. Diffuse parieto-occipital and local left temporo-parietal decrease of FDG uptake was observed on PET images. CONCLUSION: These results suggest that CAMTA1 mutation may induce an unusual neuropsychological profile and parieto-temporal developmental abnormalities. We recommend screening for CAMTA1 mutations in patients with autosomal dominant mild intellectual disability presenting with similar a phenotype.

Initial neuropsychological profile of a series of 20 patients with logopenic variant of primary progressive aphasia.

BACKGROUND: Logopenic variant of primary progressive aphasia (LPPA) is classically considered as an isolated language disorder, but verbal short-term memory deficit induces difficulties in neuropsychological tests that are not intended to evaluate language. OBJECTIVE: The aim of this study is to describe the initial symptoms and neuropsychological profiles of LPPA. METHODS: A retrospective study was conducted with a series of 20 consecutive patients diagnosed with LPPA. Clinical, neuroimaging, neuropsychological, and linguistic examinations are reported. The first neuropsychological examinations (mean time between neuropsychological assessment and diagnosis: 11 months) were then compared to 20 patients with mild cognitive impairment (MCI) and 20 patients with Alzheimer's disease (AD) matched by age, gender, and education level. RESULTS: A recent onset or aggravation of anxiety disorders was frequently reported. An unusual neuropsychological profile, different from that of AD or MCI, was observed: dissociation between verbal and visual memory performances, poor encoding performances on verbal memory tests, and preserved orientation to time, difficulties with mental calculation and fluency tasks. Biparetal abnormality and left hippocampal diaschisis was frequently observed. Asymptomatic dopaminergic depletion was observed in four patients. CONCLUSION: Our study identifies that de novo or recently worsening anxiety and specific neuropsychological profiles call for screening for LPPA, including a linguistic examination. Sometimes, there may be a continuum between LPPA and corticobasal syndrome.

Logopenic syndrome in posterior cortical atrophy.

Few language disorders have been reported in posterior cortical atrophy (PCA). Furthermore, no study has focused on screening for them and described these language deficits. The goal of this work was to describe linguistic examination of PCA patients and the impact of language disorders on neuropsychological performances compared to patients with other neurodegenerative syndromes and control groups. Linguistic examination of 9 PCA patients was carried out. The neuropsychological performance of the PCA group (16 patients) in the RAPID battery tests was compared with performances of patients with a logopenic variant of primary progressive aphasia (LPPA), patients with Alzheimer's disease and patients with amnestic mild cognitive impairment, as well as the control group. A "logopenic syndrome" with anomia, fluency impairment, and length-dependent deficit was found in 8/9 PCA patients. A comparison with other neurodegenerative syndromes showed that not only visual disorders but also language and verbal short-term memory disorders, such as those found in LPPA, can explain neuropsychological performances. A "logopenic syndrome" is frequently found in PCA and may be associated with poor performance on other verbally mediated neuropsychological tasks (e.g., verbal memory). Specific logopedic rehabilitation should be offered to these patients.