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Cognitive and visual impairment are common in Huntington’s Disease (HD) and may precede motor diagnosis. We investigate the early presence of visual cognitive deficits in 181 participants, including HD carriers (40 pre-manifest, 30 early manifest, 27 manifest, and 6 reduced penetrance) and 78 healthy controls (HC). Significant differences in visual memory were observed between reduced penetrance and pre-manifest groups ( p  = .003), with pre-manifest showing worse performance. Age, education, CAG repeats, motor status, executive function, and verbal fluency, accounted for up to 72.8% of the variance in general and visual cognitive functions, with motor status having the strongest impact on visual domains in HD carriers. In pre-manifest HD, visual cognitive domains were primarily influenced by executive function, verbal fluency, age, and CAG repeats, while in early and manifest stages motor status and verbal fluency becomes more influential. ROC analyses showed that especially visuospatial abilities, visual memory, and visual attention (AUC = 0.927, 0.878, 0.874, respectively) effectively differentiated HC and pre-manifest from early and manifest HD. Early assessment of visual cognitive domains, particularly visual memory, could be an early marker of cognitive decline in HD. Our findings highlight the different profiles of impairment in visual cognition across HD carriers.

Background and objective Cognitive impairment, including impairment of episodic memory, is frequently found in newly diagnosed Parkinson's disease (PD). In this longitudinal observational study we investigated whether performance in memory encoding, retention, recognition and free recall is associated with reduced hippocampal radial distance. Methods We analysed baseline T1-weighted brain MRI data from 114 PD subjects without cognitive impairment, 29 PD subjects with mild cognitive impairment and 99 normal controls from the ParkWest study. Age- and education-predicted scores for the California Verbal Learning Test 2 (CVLT-2) and tests of executive function were regressed against hippocampal radial distance while adjusting for imaging centre. Results There was no association between encoding or performance on executive tests and hippocampal atrophy in the PD group. In the full PD sample we found bilaterally significant associations between lower delayed free recall scores and hippocampal atrophy in the CA1, CA3 and subiculum area (left, p=0.0013; right, p=0.0082). CVLT-2 short delay free recall scores were associated with bilateral hippocampal CA1 and subicular atrophy in the full PD sample (left, p=0.013; right, p=0.047). CVLT-2 recognition scores showed a significant association with right-sided subicular and CA1 atrophy in the full PD sample (p=0.043). Conclusions At the time of PD diagnosis, subjects’ verbal memory performance in recall and recognition are associated with atrophy of the hippocampus, while encoding is not associated with hippocampal radial distance. We postulate that impaired recall and recognition might reflect deficient memory consolidation at least partly due to structural hippocampal changes.

Patients with Alzheimer’s disease (AD) show a deficit in processing sentences with psychological verbs. Evidence from English suggests that their comprehension of object Experiencers is more impaired relative to subject Experiencers. Since Spanish is structurally different from English, in the present study we wanted to determine whether Spanish–speaking AD patients would show a different comprehension pattern. We tested comprehension of semantically irreversible sentences involving psych verbs with subject Experiencers, and accusative– and dative–marked object Experiencers in 10 native speakers of Spanish diagnosed with mild to moderate AD. We also tested their comprehension of semantically reversible sentences involving dative object Experiencers in two word orders (SVO, OVS). A paired–samples t–test revealed no statistically significant differences in their comprehension of subject vs. object Experiencers, or accusative vs. dative object Experiencers in semantically irreversible sentences, with one–sample t–test indicating comprehension above chance. However, their overall comprehension of psych verbs in semantically reversible sentences was poor, regardless of word order, and it was significantly worse than their comprehension of sentences with action verbs in the same word orders. Thus, our data indicate that the deficit in comprehension of psych verbs found in Spanish speakers with mild to moderate AD dementia differs from the pattern found in English, and that the deficit in AD patients’ comprehension of psych verbs is more heterogeneous than previously thought.

Background/Aims: Dementia with Lewy bodies (DLB) and Alzheimer’s disease (AD) are the two most common neurodegenerative dementias. During the early stages, clinical distinction between them is often challenging. Our objective is to compare hippocampal atrophy patterns in mild AD and mild DLB. We hypothesized that DLB subjects have milder hippocampal atrophy relative to AD subjects. Methods: We analyzed the T1-weighted magnetic resonance imaging data from 113 subjects: 55 AD, 16 DLB and 42 cognitively normal elderly (normal controls, NC). Using the hippocampal radial distance technique and multiple linear regression, we analyzed the effect of clinical diagnosis on hippocampal radial distance, while adjusting for gender and age. Three-dimensional statistical maps were adjusted for multiple comparisons using permutation-based statistics with a threshold of p < 0.01. Results: Compared to NC, AD exhibited significantly greater atrophy in the cornu ammonis (CA)1, CA2–3 and subicular regions bilaterally while DLB showed left-predominant atrophy in the CA1 region and subiculum. Compared directly, AD and DLB did not reveal statistically significant differences. Conclusion: Hippocampal atrophy, while present in mildly impaired DLB subjects, is less severe than atrophy seen in mildly impaired AD subjects, when compared to NC. Both groups show predominant atrophy of the CA1 subfield and subiculum.

The objective of this study is to assess how the non-motor symptoms of Parkinson’s disease (PD), such as depression, cognitive deterioration, neuropsychiatric and sleep disorders, affect the quality of life, and to compare them with the motor symptoms in order to determine their real impact. A cross-sectional study was designed including 99 patients (mean age 68.5 ± 9.9 years, duration of disease 8.7 ± 6.2 years). Demographic data, onset of PD, years on treatment with levodopa (LD), class of dopaminergic drug prescribed, and dosages were obtained. The following scales were used: quality of life (PDQ-39), Unified Parkinson’s Disease Rating Scale (UPDRS I–IV), Parkinson Disease Sleep Scale (PDSS) and daytime sleepiness (Epworth), Mini-Mental State Examination, depression (HAM-D), and the neuropsychiatric inventory (NPI-10). The PDQ-39 summary index (PDQ-39 SI) was 24.7 ± 13.2. A linear regression model including all variables showed that four independent variables accounted for 67.2% of the variance in the PDQ-39 SI ( F  = 33,277; p  < 0.001): NPI, PDSS, UPDRS IV, and UPDRS I. When sub-items of the NPI, PDSS and UPDRS IV scales are analyzed, significant correlations ( p  < 0.001) are found between the PDQ-39 SI and depression, agitation, apathy, anxiety, hallucinations, delusions, incontinence of urine, morning painful posturing, restlessness in bed, morning fatigue, duration of off periods, unpredictable and predictable off periods, early morning dystonia, and sudden off periods. Neuropsychiatric symptoms, especially depression, nighttime sleep disorders such as urinary incontinence, nighttime restlessness, morning fatigue and somnolence, off-period dystonia and motor fluctuations are the variables that most affect the quality of life of patients with PD.

BackgroundHD is an autosomal dominant, hereditary, and neurodegenerative disease that presents neurological, psychiatric, and cognitive impairment, with visual cognition being one of the affected areas.AimsThis study aims to analyze the visual cognition profile of asymptomatic and symptomatic carriers of Huntington’s disease (HD), compared with healthy controls (HC), and to evaluate the differences between asymptomatic and symptomatic patients with different years of progression of HD.MethodsWe evaluated 99 participants, 51 HD carriers [17 asymptomatic, 13 symptomatic ( < 5 years of evolution), 21 symptomatic ( > 5 years of evolution)] and 48 HC matched by sex and educational level. Motor function was rated with UHDRS scale, the general cognitive status was assessed with MoCA test, and a comprehensive battery of visual cognitive instruments was used. The following visual cognitive domains were assessed: visual memory, visuospatial skills and visuoconstructive abilities. One- way ANOVA and Tukey’s test for post hoc analysis were performed to analyze and compare the cognitive performance between the four groups.ResultsStatistically significant differences were found in the motor function (F(3.1)=14.129; p < .001) and in the general cognitive status (F(6.1)=9.63; p < .001) between groups. Specifically, we found significant differences in visual memory and visuospatial and visuoconstructive abilities between asymptomatic and both symptomatic subgroups of HD patients (p=.058), and also between the two groups of symptomatic patients with different years of evolution of HD (p=.014).ConclusionsFindings suggest that both symptomatic and asymptomatic HD patients present an increased visual cognitive impairment compared to HC. This impairment worsens with HD progression.

Orthostatic hypotension and supine hypertension frequently coexist in Parkinson’s disease (PD) patients, leading to visceral damage and increased mortality rates. The aim of this paper is to analyze the frequency and association of both conditions in a sample of outpatients with PD. A total of 111 patients, diagnosed with PD, were studied. Disease duration, treatment, cardiovascular risk factors, UPDRS I-IV and Scopa Aut scale scores were reported. Subjects underwent 24-h ambulatory blood pressure (BP) monitoring and were assessed for orthostatic hypotension. We compared our results with those published in 17,219 patients using the same protocol and the same type of device. Overall, 71.1 % had no proper circadian rhythm. This frequency was significantly higher than that of the control population (48 %). The prevalence of the nondipper or riser patterns was higher in patients with orthostatic hypotension (77.8 vs. 66.7 %). There was a correlation between nightly increases in diastolic blood pressure and changes in BP during the orthostatic test. Patients taking higher doses of treatment had less decreases in SBP (cc:−0.25; p  = 0.007) and DBP (cc:−0.33; p  < 0.001) at night, however there was no relation with drug type. The majority of patients with Parkinson’s disease show an altered circadian rhythm of blood pressure. Patients with a non-dipper or riser pattern on 24 h ABPM exhibited a higher prevalence of autonomic disorders (orthostatic hypotension) and received higher doses of dopaminergic treatment. A day–night variation in diastolic blood pressure was the most important marker of these findings.

The applause sign has been associated with various neurodegenerative diseases. We investigate its validity in the differential diagnosis of progressive supranuclear palsy and Parkinson's disease, and its relationship with neuropsychological tests. 23 patients with progressive supranuclear palsy and 106 patients with Parkinson's disease were included and administered the following scales: progressive supranuclear palsy rating scale, unified Parkinson's disease rating scale (UPDRS), mini-mental state examination (MMSE), frontal assessment battery (FAB), neuropsychiatric inventory and three-clap test. 73.9% with progressive supranuclear palsy and 21.7% with Parkinson's disease showed a positive applause sign. Only a positive applause sign, UPDRS II score and disease duration were found to be predictors of progressive supranuclear palsy. Both patient-groups showed statistically significant correlations between the applause sign and neuropsychological tests: in progressive supranuclear palsy patients MMSE correlation coefficient: 0.62 (p: 0.002) and FAB correlation coefficient: 0.48 (p: 0.02), and in Parkinson's disease patients MMSE correlation coefficient: 0.47 (p<0.001) and FAB correlation coefficient: 0.43 (p<0.001). Verbal fluency and inhibitory control (FAB) and writing and orientation in time (MMSE) discriminated between patients with normal and positive applause sign. A positive applause sign is not specific to progressive supranuclear palsy and may also be observed in Parkinson's disease patients with altered cognition, and it's related to cortical frontal abnormalities such as language disorders and inhibitory control.

The presence of asymmetry in symptoms and clinical signs favours the diagnosis of Parkinson's disease (PD). The aim of this study is to analyse this symptom asymmetry as a function of different variables and compare it with other parkinsonisms. 201 Patients with PD were studied. The sample was supplemented with 29 patients diagnosed with MSA-P (according to the criteria established by the American Academy of Neurology) and 17 with PSP (according to the criteria established by the NINDS-SPSP International Workshop). The symmetry was evaluated, based on items 20–23, 25 and 26 of the Unified Parkinson's Disease Rating Scale, by subtracting the motor score for the left side from that for the right side. Those patients with a difference of one point or more were designated as being asymmetric. Around 16.4% of patients presented symmetrical clinical symptoms. There were no differences between those patients with or without family history of the disease. Those patients with symmetric symptoms were found to have longer symptomatic disease duration (10.8 vs. 7.9 years), a worse mental state (UPDRS I: 3.9 vs. 3.2), a higher incidence of complications (UPDRS IV: 4.5 vs. 3.2) and had their activities of daily living (ADL) affected to a greater degree (UPDRS II: 13.0 vs. 11.0). Around 48.3% of the MSA-P patients and 52.9% of the PSP patients showed symmetric symptoms. The degree of symmetry is not useful in differentiating between sporadic and familial PD. However, the observation of highly symmetrical symptoms in a patient with short evolution time indicates that an atypical parkinsonism should be suspected.