Preserved visuospatial abilities in absence of the right hemisphere: A case of cerebral hemiatrophy with minimal cognitive impairment.

Cerebral hemiatrophy is a rare neurological condition, usually resulting in severe and diffuse cognitive impairment. In this paper we present a 69-year old woman with notable congenital hemiatrophy with strikingly preserved cognitive functions. Cognitive assessment indicated that although her executive functions were found impaired, the remaining cognitive domains were relatively unaffected. We argue that this unexpected cognitive profile may be explained by anomalous hemispheric lateralization, driven by neuroplasticity along the developmental course.

Brief Report: Speech and Language Therapy in Children with ASD in an Aquatic Environment: the ASLT (Aquatic Speech and Language Therapy) Program.

Although water-based approaches have been shown to be beneficial for children with Autism Spectrum Disorder (ASD), no study thus far has directly investigated the effects of such intervention programs on language skills. The present study aims to evaluate the efficacy of the Aquatic Speech and Language Therapy (ASLT) program, which is a new, exclusively aquatic intervention program designed especially for children with ASD. The effects of ASLT were compared to the outcome of a similar classroom-based intervention, in two groups of children with ASD matched for age, gender, and expressive/receptive vocabulary. Our findings show that ASLT results in significantly greater improvement of vocabulary measures, thus providing direct evidence of water-based intervention's beneficial effects on language skills in ASD.

A longitudinal study of cognitive function in multiple sclerosis: is decline inevitable?

BACKGROUND: Numerous cross-sectional studies report cognitive impairment in multiple sclerosis (MS), but longitudinal studies with sufficiently long-term follow-up are scarce. OBJECTIVE: We aimed to investigate the cognitive 10-year course of a cohort of MS patients. METHODS: 59 patients with clinically isolated syndrome (CIS) or relapsing-remitting (RR) MS were evaluated with Rao's Brief Repeatable Battery of Neuropsychological Tests at baseline and follow-up (at least 10 years later). They constituted 47.2% of 124 consecutive CIS and RRMS patients originally evaluated at baseline. Patients assessed at follow-up were well matched for baseline clinical characteristics with dropouts. RESULTS: The proportion of MS patients with overall cognitive impairment was increased by 10% within the 10-year period. When grouped on the basis of impairment in specific cognitive domains at baseline, patients originally impaired showed improvement at follow-up, while the opposite trend was observed for patients non-impaired at first assessment. A detailed case-by-case investigation revealed mixed evolution patterns, several patients fail in fewer domains at follow-up compared to baseline or failing at different domains at follow-up compared to baseline. CONCLUSIONS: This study suggests a more fluid picture for the evolution of cognitive function in a subgroup of MS patients and contradicts the concept of an inevitable, progressively evolving "dementia".

Anti-NMDA receptor encephalitis presenting as isolated aphasia in an adult.

Anti-NMDA receptor (NMDA-r) encephalitis is a relatively rare cause of autoimmune encephalitis with divergent clinical presentations. We report a case of an adult patient with anti-NMDA-r encephalitis presenting with isolated, abrupt-onset aphasia. Her condition remained unaltered over a period of 6 months. The patients' electroencephalogram findings were typical for NMDA-r encephalitis; however, her magnetic resonance imaging and cerebrospinal fluid analysis were normal. She responded well to immunotherapy, and aphasia eventually resolved. The natural course of the present case contradicts the rapidly progressive nature of typical NMDA-r encephalitis. Furthermore, it broadens the clinical spectrum of anti-NMDA-r encephalitis, to incorporate isolated, nonprogressive aphasia.

In Search of Executive Impairment in Pathological Gambling: A Neuropsychological Study on Non-treatment Seeking Gamblers.

Pathological gambling is characterized by a persisting maladaptive and recurrent behavior with severe social and psychological consequences. There is evidence of strong comorbidity with psychiatric manifestations as well as cognitive mainly involving executive functions. This study aimed to investigate impairment in executive functions and working memory, and personality traits in a sample of Greek gamblers. Twenty-four men involved in various gambling activities were recruited from ecological settings as probable pathological gamblers. They were assessed with a comprehensive neuropsychological battery involving several executive tasks, the Zuckerman-Kuhlman Personality Questionnaire, the Hospital Anxiety Depression Scale, and the Difficulties in Emotion Regulation Scale. An age- and education-level matched group of 21 men without history of habitual gambling served as controls. As a group, gamblers displayed significantly lower scores on indices of inhibition, decision making and self-reported emotional awareness, and scored higher on impulsivity/sensation seeking personality traits. Notably, gamblers scored similarly or significantly higher on measures of verbal and visuospatial working memory, cognitive flexibility, processing speed, verbal fluency, and sustained attention. Overall, we argue that gamblers do present with specific cognitive deficits, but there is no evidence for a generalized executive impairment, and further stress the importance of investigating cognitive, personality, and psychiatric aspects of gambling on the basis of an ecologically valid sampling.

The effect of cortical and subcortical lesions on spontaneous expression of memory-encoded and emotionally infused information: Evidence for a role of the ventral stream.

The ventral stream of language processing has been implicated in the spontaneous expression of memory-encoded and emotionally infused information. The present study investigated whether left hemispheric lesions in post-stroke right-handed aphasic patients may be selectively associated with specific language functions. Speech rate was assessed with two tasks, one based on autobiographical memory of an emotionally infused event (stroke story narration) and the other based on information that is visually available at the time of speech generation ("cookie theft" picture description). CT and/or MRI scans were obtained for each patient and lesions located in 16 regions of the left hemisphere were identified and coded. The total number of cortical and subcortical areas affected served as a measure of lesion extent. While mean speech rates were similar across conditions, there were different patterns of association between each index and specific lesion sites. Non-parametric quantile regression statistical models constructed to assess dependence of both speech rate indices on each lesion locus indicated that the speech rate in the stroke story had significant inverse associations with total number of lesioned areas, as well as lesions in the inferior frontal gyrus and the external/extreme capsule region. The cookie theft speech rate had significant inverse associations with total number of lesioned areas as well as lesion in the inferior frontal gyrus, but not with the external/extreme capsule region. In sum, integrity of the extreme/external capsule region appears to be important selectively for the Stroke Story task, supporting the hypothesis that the ventral stream plays a central role in spontaneous expression of memory-encoded and emotionally infused information.

The unbridged gap between clinical diagnosis and contemporary research on aphasia: A short discussion on the validity and clinical utility of taxonomic categories.

Even if the traditional aphasia classification is continuously questioned by many scholars, it remains widely accepted among clinicians and included in textbooks as the gold standard. The present study aims to investigate the validity and clinical utility of this taxonomy. For this purpose, 65 left-hemisphere stroke patients were assessed and classified with respect to aphasia type based on performance on a Greek adaptation of the Boston Diagnostic Aphasia Examination. MRI and/or CT scans were obtained for each patient and lesions were identified and coded according to location. Results indicate that 26.5% of the aphasic profiles remained unclassified. More importantly, we failed to confirm the traditional lesion-to-syndrome correspondence for 63.5% of patients. Overall, our findings elucidate crucial vulnerabilities of the neo-associationist classification, and further support a deficit-rather than a syndrome-based approach. The issue of unclassifiable patients is also discussed.

Frontotemporal Dysfunction in Amyotrophic Lateral Sclerosis: A Discriminant Function Analysis.

BACKGROUND: There is growing evidence for extramotor dysfunction (EMd) in amyotrophic lateral sclerosis (ALS), with a reported prevalence of up to 52%. OBJECTIVE: In the present study, we explore the clinical utility of a brief neuropsychological battery for the investigation of cognitive, behavioral, and language deficits in patients with ALS. METHODS: Thirty-four consecutive ALS patients aged 44-89 years were tested with a brief neuropsychological battery, including executive, behavioral, and language measures. Patients were initially classified as EMd or non-EMd based on their scores on the frontal assessment battery (FAB). RESULTS: Between-group comparisons revealed significant differences in all measures (p < 0.01). Discriminant analysis resulted in a single canonical function, with all tests serving as significant predictors. This function agreed with the FAB in 13 of 17 patients screened as EMd and identified extramotor deficits in 2 additional patients. Overall sensitivity and specificity estimates against FAB were 88.2%. CONCLUSIONS: We stress the importance of discriminant function analysis in clinical neuropsychological assessment and argue that the proposed neuropsychological battery may be of clinical value, especially when the option of extensive and comprehensive neuropsychological testing is limited. The psychometric validity of an ALS-frontotemporal dementia diagnosis using neuropsychological tests is also discussed.

Are memory deficits dependent on the presence of aphasia in left brain damaged patients?

While memory deficits in aphasia have been reported in several studies, it has been suggested that these deficits are not due to the presence of aphasia, but rather to the left hemisphere lesion per se. In order to investigate this hypothesis, we tested 64 aphasic and 15 non-aphasic patients with left brain damage on verbal and visuospatial span tasks. Analyses revealed lower than expected performance on all four primary memory tasks for the aphasic, but not for the non-aphasic group. Moreover, comparison of the three lesion-location groups (posterior, anterior, and global) did not reveal statistically significant differences. The present data show that aphasic patients demonstrate memory deficits, which are not specific to the verbal modality, and contradict the notion that primary memory impairment is not due to the presence of aphasia, but rather to a lesion in the left hemisphere per se. Overall our study suggests that verbal and visuospatial, primary memory deficits in patients with left hemisphere lesions are possibly dependent on the presence of aphasia, but not on lesion location or lesion size.