Background: Hereditary cerebellar ataxias (HCA) and hereditary spastic paraplegias (HSP)
are two groups of neurodegenerative disorders that usually present with progressive gait …

Abstract Machado–Joseph disease (MJD), also known as spinocerebellar ataxia type 3
(SCA3), may be the most common dominantly inherited ataxia in the world. Here I will review …

This study describes the frequency of spinocerebellar ataxias and of CAG repeats range in
different geographical regions of Brazil, and explores the hypothetical role of normal CAG …

There are over 40 autosomal dominant spinocerebellar ataxias (SCAs) now identified. In this
chapter we delineate the phenotypes of SCAs 1-44 and dentatorubral-pallidoluysian atrophy …

The hereditary ataxias are clinically and genetically very heterogeneous. They are mainly
characterized by gait and limb ataxia, dysarthria, a variable degree of oculomotor …

Motivation: Resolving tandemly repeated genomic sequences is a necessary step in
improving our understanding of the human genome. Short tandem repeats (TRs), or …

This paper reviews and summarizes three main aspects of spinocerebellar ataxias (SCA) in
the Asian population. First, epidemiological studies were comprehensively reviewed …

Objectives: Identify the etiology of spinocerebellar ataxia medical conditions and
emergencies. Review the appropriate evaluation of spinocerebellar ataxia. Outline the …

Common genetic risk factors are associated with frontotemporal dementia (FTD) and
amyotrophic lateral sclerosis (ALS). Intermediate repeat expansions at the Ataxin-2 locus …

Abstract Spinocerebellar ataxia type 1 (SCA1) is one out of nine polyglutamine diseases, a
group of late-onset neurodegenerative diseases present only in humans. SCA1, the first …