A neurological examination score for the assessment of spinocerebellar ataxia 3 (SCA3)

C Kieling, CRM Rieder, ACF Silva… - European journal of …, 2008 - Wiley Online Library
Spinocerebellar ataxias (SCAs) are characterized by a heterogeneous set of clinical
manifestations. Our aims were to assess the neurological features of SCA3, and to describe …

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 …

[HTML][HTML] Clinical characteristics of patients with spinocerebellar ataxias 1, 2, 3 and 6 in the US; a prospective observational study

T Ashizawa, KP Figueroa, SL Perlman… - Orphanet journal of rare …, 2013 - Springer
Abstract Background All spinocerebellar ataxias (SCAs) are rare diseases. SCA1, 2, 3 and 6
are the four most common SCAs, all caused by expanded polyglutamine-coding CAG …

Spinocerebellar ataxia type 1: one-year longitudinal study to identify clinical and MRI measures of disease progression in patients and presymptomatic carriers

A Nigri, L Sarro, A Mongelli, A Castaldo, L Porcu… - The Cerebellum, 2022 - Springer
Abstract Spinocerebellar ataxias type 1 (SCA1) is an autosomal dominant disease usually
manifesting in adulthood. We performed a prospective 1-year longitudinal study in 14 …

Cognitive impairment in spinocerebellar ataxia type 12

A Agarwal, H Kaur, A Agarwal, A Nehra… - Parkinsonism & Related …, 2021 - Elsevier
Introduction Cognitive impairment has now been recognised to be present in patients with
several of spinocerebellar ataxias (SCAs). Cognitive impairment in patients with …

[HTML][HTML] A longitudinal investigation into cognition and disease progression in spinocerebellar ataxia types 1, 2, 3, 6, and 7

A Moriarty, A Cook, H Hunt, ME Adams… - Orphanet Journal of …, 2016 - Springer
Background The natural history of clinical symptoms in the spinocerebellar ataxias (SCA) s
has been well characterised. However there is little longitudinal data comparing cognitive …

[HTML][HTML] The progression rate of spinocerebellar ataxia type 2 changes with stage of disease

TL Monte, ER Reckziegel, MC Augustin… - Orphanet journal of rare …, 2018 - Springer
Background Spinocerebellar ataxia type 2 (SCA2) affects several neurological structures,
giving rise to multiple symptoms. However, only the natural history of ataxia is well known …

Spinocerebellar ataxia type 1 (SCA1): new pathoanatomical and clinico‐pathological insights

U Rüb, K Bürk, D Timmann… - Neuropathology and …, 2012 - Wiley Online Library
U. Rüb, K. Bürk, D. Timmann, W. den Dunnen, K. Seidel, K. Farrag, E. Brunt, H. Heinsen, R.
Egensperger, A. Bornemann, S. Schwarzacher, H.‐W. Korf, L. Schöls, J. Bohl and T. Deller …

The S-factor, a new measure of disease severity in spinocerebellar ataxia: findings and implications

LP Selvadurai, SL Perlman, GR Wilmot, SH Subramony… - The Cerebellum, 2023 - Springer
Spinocerebellar ataxias (SCAs) are progressive neurodegenerative disorders, but there is
no metric that predicts disease severity over time. We hypothesized that by developing a …

The natural history of spinocerebellar ataxia type 1, 2, 3, and 6: a 2-year follow-up study

H Jacobi, P Bauer, P Giunti, R Labrum, MG Sweeney… - Neurology, 2011 - AAN Enterprises
Objective: To obtain quantitative data on the progression of the most common
spinocerebellar ataxias (SCAs) and identify factors that influence their progression, we …