Effects of Computer-Based Cognitive Rehabilitation on Attention, Executive Functions, and Quality of Life in Patients with Parkinson's Disease: A Randomized, Controlled, Single-Blinded Pilot Study.

BACKGROUND: Cognitive decline in Parkinson's disease (PD) has become increasingly recognized in recent years, and there is a need to identify methods for cognitive rehabilitation in PD patients. OBJECTIVE: The aim of this study was to explore the feasibility and effects of 2 different computer-based cognitive rehabilitation (CBCR) interventions on attention, executive functions, and quality of life (QoL) in PD patients. METHODS: Thirty nondemented PD patients were randomly assigned to one of 3 groups: one passive control group and 2 intervention groups with 2 different CBCR programmes. The intervention period was 8 weeks with follow-up visits in clinic every second week. Before and after the intervention period, patients were tested with a neuropsychological battery of attention, executive functions, and QoL. RESULTS: Twenty-four patients completed the study. Patients in one of the CBCR groups experienced a significant within-group increase on the primary measures of attention, executive functions, and QoL. However, this effect was not significant between groups. No significant differences were observed for the other CBCR group or the control group. CONCLUSIONS: CBCR is a feasible intervention for cognitive rehabilitation in nondemented PD patients. The effects of training were modest and should be further explored in larger clinical trials. Some CBCR programmes might be more effective than others for PD patients. The protocol for this study was published prospectively at ClinicalTrials.gov on September 18, 2017 with ID: NCT03285347.

Severe and rapidly progressing cognitive phenotype in a SCA17-family with only marginally expanded CAG/CAA repeats in the TATA-box binding protein gene: a case report.

BACKGROUND: The autosomal dominant spinocerebellar ataxias (SCAs) confine a group of rare and heterogeneous disorders, which present with progressive ataxia and numerous other features e.g. peripheral neuropathy, macular degeneration and cognitive impairment, and a subset of these disorders is caused by CAG-repeat expansions in their respective genes. The diagnosing of the SCAs is often difficult due to the phenotypic overlap among several of the subtypes and with other neurodegenerative disorders e.g. Huntington's disease. CASE PRESENTATION: We report a family in which the proband had rapidly progressing cognitive decline and only subtle cerebellar symptoms from age 42. Sequencing of the TATA-box binding protein gene revealed a modest elongation of the CAG/CAA-repeat of only two repeats above the non-pathogenic threshold of 41, confirming a diagnosis of SCA17. Normally, repeats within this range show reduced penetrance and result in a milder disease course with slower progression and later age of onset. Thus, this case presented with an unusual phenotype. CONCLUSIONS: The current case highlights the diagnostic challenge of neurodegenerative disorders and the need for a thorough clinical and paraclinical examination of patients presenting with rapid cognitive decline to make a precise diagnosis on which further genetic counseling and initiation of treatment modalities can be based.

[Dementia in amyotrophic lateral sclerosis]. / Demens ved amyotrofisk lateral sklerose.

New clinical findings suggest a connection between Amyotrophic Lateral Sclerosis (ALS) and Frontotemporal Dementia (FTD). The two diseases are often seen simultaneously, which complicates the course of the disease for the ALS-patient and the relatives. This paper illustrates this problem and describes a way to monitor the ALS-patient's cognitive and personal changes in an effort to improve his or her rehabilitation and palliation.

Cognitive deficits in long-term survivors of childhood brain tumors: Identification of predictive factors.

BACKGROUND: To describe cognitive function and to evaluate the association between potentially predictive factors and cognitive outcome in an unselected population of survivors of childhood brain tumors. PROCEDURE: We studied a consecutive sample of 133 patients (76 had received radiotherapy (RT)) who had a brain tumor diagnosed before the age of 15 years and were treated during the period January 1970 through February 1997 in the Eastern part of Denmark. Biologic effective dose of irradiation (BED) was assessed in 71 patients. One hundred twenty-seven patients were able to cooperate to WISC-R and WAIS-R. Multiple regression models were constructed to evaluate relationships between possible risk factors and cognitive outcome. RESULTS AND CONCLUSIONS: The mean intelligence (IQ) scores were substantially lower than the expected means of the general population. Younger age at diagnosis, tumor site in cerebral hemisphere, hydrocephalus treated with shunt, and treatment with RT were found to be significant predictors of lower cognitive functions. RT was the most important risk factor for impaired intellectual outcome. The mean observed full scale IQ was 97.1 (SD = 14.3) for the non-irradiated patients and 78.8 (SD = 14.3) for the irradiated patients (adjusted P < 0.001). Verbal IQ, but not performance and full scale IQ, had a significant negative correlation to BED to the tumor site (P < 0.05). These results can be used to identify subgroups of children who are at increased risk for cognitive deficits allowing early and goal-directed intervention.