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Objective: To determine frequency and type of cognitive disorders in cross-sectional analysis of a Parkinson's disease (PD) cohort, and explore its relations to motor symptoms, modifiable vascular risk factors and white matter lesions (WML) volume., Methods: In a group of 133 PD patients, mild cognitive impairment (PD-MCI) and dementia (PDD) were diagnosed according to Movement Disorders Society Task Force criteria (level 2 for PD-MCI). Detailed motor measurements were applied, including rigidity, axial, bradykinesia, tremor and postural instability gait disorders (PIGD) scores. Vascular risk was estimated by the Framingham General Cardiovascular Disease risk scoring algorithm and WML volume was measured for whole brain and frontal lobe., Results: Sixty-one (46.9%) patients fulfilled criteria for PD-MCI, and 23 (17.7%) for PDD. Non-amnestic multiple domain MCI was most frequent (52% of PD-MCI patients). Motor scores were significantly higher in cognitively impaired patients, but only axial score discriminated between MCI and dementia. High vascular risk was related to impaired cognition, bradykinesia, axial, PIGD and freezing of gait (FOG) score, while whole brain WML volume was associated with PDD, FOG and attention deficits. Furthermore, high vascular risk was identified as a potential predictor of both MCI and dementia in PD. Additionally, age and bradykinesia score were independently associated with PD-MCI and age, axial score and whole brain WML volume with PDD., Conclusion: Cognitive disorders in PD are associated with more severe, predominantly axial motor deficits and increased, but partly modifiable vascular burden, thus opening a possibility for development of preventive strategies in PD.

Background: Frontotemporal dementia (FTD) is the second most common cause of early-onset dementia (EOD), characterized by behavioral changes (behavioral variant; bvFTD) or language deficits. A hexanucleotide repeat expansion in a noncoding region of chromosome 9 open reading frame 72 (C9orf72) has been proved to be a major cause of both familial and sporadic amyotrophic lateral sclerosis or FTD, with or without concomitant motor neuron disease (MND)., Methods: The aim of this study was to assess the frequency of the C9orf72 hexanucleotide expansion in a cohort of 117 Serbian patients with EOD and to report phenotypic features of identified carriers., Results: We identified 4 of 117 (3.4%) patients with EOD to have C9orf72 hexanucleotide expansions. All patients were classified in the FTD disease spectrum group (8.2%): 3 patients fulfilled the criteria for bvFTD, and 1 patient had FTD-MND. None of the patients with the C9orf72 hexanucleotide expansion fulfilled the diagnostic criteria for language variants of FTD, FTD-progressive supranuclear palsy overlap syndrome, dementia with Lewy bodies or Alzheimer's dementia., Conclusion: In a cohort of consecutive patients with EOD, 3.4% had the C9orf72 hexanucleotide expansion with clinical phenotypes of bvFTD or an overlap of bvFTD and MND., (© 2015 S. Karger AG, Basel.)

Our objective was to determine whether the presence of executive dysfunction in non-demented amyotrophic lateral sclerosis (ALS) patients might affect the longevity of survival. Forty-eight consecutive non-demented ALS patients (mean age = 52.93 ± 12.37) were followed for five years. All patients underwent clinical and neuropsychological assessments at baseline visit. Further, a yearly follow-up check for associated dementia (ALS-Dem) was completed and the time of death was recorded, when applicable. Executive deficits were shown in 49.5% of ALS patients, with the most striking differences found on the tests of verbal fluency (both phonemic and category,p < 0.01); as well as inefficient strategy on a working memory test (p < 0.05); as on the more demanding levels of the planning and problem solving task (p < 0.01). It appears that the baseline executive status might predict survival in ALS (p = 0.075), and the patients presenting executive dysfunction could have up to three times greater risk of death, after adjustment by several potential confounding factors. In conclusion, this study suggests that executive dysfunction could potentially influence survival in ALS patients. The cognitive testing might give us important clues about the prognosis of the disease. Further studies with larger sample size are necessary. [ABSTRACT FROM PUBLISHER]