Resting-state functional connectivity and cognitive dysfunction correlations in spinocerebelellar ataxia type 6 (SCA6).

Objective: The aim of this study is to evaluate the correlation between resting state functional MRI (RS-fMRI) activity and motor and cognitive impairment in spinocerebellar ataxia type 6 (SCA6).

Methods: Twelve patients with genetically confirmed SCA6 and 14 age matched healthy controls were imaged with RS-fMRI. Whole brain gray matter was automatically parcellated into 1000 regions of interest (ROIs).

Structural cerebellar correlates of cognitive and motor dysfunctions in cerebellar degeneration.

See King et al. (doi:10.1093/aww348) for a scientific commentary on this article.

Landmark Based Shape Analysis for Cerebellar Ataxia Classification and Cerebellar Atrophy Pattern Visualization.

Cerebellar dysfunction can lead to a wide range of movement disorders. Studying the cerebellar atrophy pattern associated with different cerebellar disease types can potentially help in diagnosis, prognosis, and treatment planning. In this paper, we present a landmark based shape analysis pipeline to classify healthy control and different ataxia types and to visualize the characteristic cerebellar atrophy patterns associated with different types.

A toolbox to visually explore cerebellar shape changes in cerebellar disease and dysfunction.

The cerebellum plays an important role in motor control and is also involved in cognitive processes. Cerebellar function is specialized by location, although the exact topographic functional relationship is not fully understood. The spinocerebellar ataxias are a group of neurodegenerative diseases that cause regional atrophy in the cerebellum, yielding distinct motor and cognitive problems.

Survival in Frontotemporal Dementia Phenotypes: A Meta-Analysis.

Background: Survival in frontotemporal dementia (FTD) is not well understood. We conducted a mixed effects meta-analysis of survival in FTD to examine phenotype differences and contributory factors.

Methods: The PubMed, Medline, EMBASE, CINAHL, PsycINFO and Cochrane databases were searched for studies describing survival or natural history of behavioral variant FTD (bvFTD), progressive non-fluent aphasia (PNFA), semantic dementia (SD), FTD with amyotrophic lateral sclerosis (FTD-ALS), progressive supranuclear palsy and corticobasal degeneration.

The use of cerebrospinal fluid and neuropathologic studies in neuropsychiatry practice and research.

The gold standard for diagnosis of neurodegenerative diseases (ie, Alzheimer disease, frontotemporal dementia, Parkinson disease, dementia with Lewy bodies, amyotrophic lateral sclerosis) is neuropathologic examination at autopsy. As such, laboratory studies play a central role in antemortem diagnosis of these conditions and their differentiation from the neuroinflammatory, infectious, toxic, and other nondegenerative etiologies (eg, rapidly progressive dementias) that are encountered in neuropsychiatric practice. This article summarizes the use of cerebrospinal fluid (CSF) laboratory studies in the diagnostic evaluation of dementia syndromes and emerging CSF biomarkers specific for underlying neuropathology in neurodegenerative disease research.