Spinocerebellar ataxia type 7 (SCA7): Widespread brain damage in an adult‐onset patient with progressive visual impairments in comparison with an adult‐onset …

U Rüb, ER Brunt, K Seidel, K Gierga… - Neuropathology and …, 2008 - Wiley Online Library
Spinocerebellar ataxia type 7 (SCA7) represents a rare and severe autosomal dominantly
inherited ataxic disorder and is among the known CAG‐repeat, or polyglutamine, diseases …

Cognitive deficits in spinocerebellar ataxia 2

K Bürk, C Globas, S Bösch, S Gräber, M Abele, A Brice… - Brain, 1999 - academic.oup.com
This is one of the first studies assessing the pattern of cognitive impairment in
spinocerebellar ataxia 2 (SCA2). Cognitive function was studied in 17 patients with …

Cognitive dysfunction in spinocerebellar ataxias

HAG Teive, WO Arruda - Dementia & neuropsychologia, 2009 - SciELO Brasil
Spinocerebellar ataxias (SCAs) comprise a heterogeneous group of complex
neurodegenerative diseases, characterized by the presence of progressive cerebellar …

Gray and white matter alterations in spinocerebellar ataxia type 7: an in vivo DTI and VBM study

S Alcauter, FA Barrios, R Díaz, J Fernández-Ruiz - Neuroimage, 2011 - Elsevier
Spinocerebellar ataxia type 7 (SCA7) is a progressive neurodegenerative disorder
characterized by cerebellar ataxia and visual loss. It is caused by a CAG repeat expansion …

Clinical analysis of adult-onset spinocerebellar ataxias in Thailand

P Boonkongchuen, S Pongpakdee, P Jindahra… - BMC neurology, 2014 - Springer
Background Non-ataxic symptoms of spinocerebellar ataxias (SCAs) vary widely and often
overlap with various types of SCAs. Duration and severity of the disease and genetic …

Spinocerebellar ataxia types 1, 2, 3, and 6: disease severity and nonataxia symptoms

T Schmitz-Hubsch, M Coudert, P Bauer, P Giunti… - Neurology, 2008 - AAN Enterprises
Objective: To identify factors that determine disease severity and clinical phenotype of the
most common spinocerebellar ataxias (SCAs), we studied 526 patients with SCA1, SCA2 …

Cognitive impairment in ARCA-1, a newly discovered pure cerebellar ataxia syndrome

R Laforce, JP Buteau, JP Bouchard, GA Rouleau… - The Cerebellum, 2010 - Springer
Cerebellar contribution to non-motor functions has been supported by several animal,
human and functional neuroimaging studies. Which cognitive skills and to what extent the …

Profile of extrapyramidal manifestations in 85 patients with spinocerebellar ataxia type 1, 2 and 3

K Jhunjhunwala, M Netravathi, M Purushottam… - Journal of Clinical …, 2014 - Elsevier
This study aimed to determine the prevalence and type of extrapyramidal signs (EPS) in
spinocerebellar ataxia (SCA) type 1, 2 and 3. Eighty-five patients with genetically confirmed …

Cognitive deficits in Machado–Joseph disease correlate with hypoperfusion of visual system areas

P Braga-Neto, LA Dutra, JL Pedroso, AC Felício… - The Cerebellum, 2012 - Springer
Cognitive and olfactory impairments have previously been demonstrated in patients with
spinocerebellar ataxia type 3 (SCA3), also known as Machado–Joseph disease (MJD) …

Focusing attention on cognitive impairment in spinocerebellar ataxia

DH Geschwind - Archives of neurology, 1999 - jamanetwork.com
COGNITIVE OR behavioral abnormalities are not generally considered to be impor-tant
features of the autosomal dominant cerebellar ataxias (ADCAs). However, Harding1 …