The spinocerebellar ataxias (SCAs) are a genetically heterogeneous group of autosomal
dominantly inherited progressive disorders, the clinical hallmark of which is loss of balance …

Autosomal dominant cerebellar ataxias are hereditary neurodegenerative disorders that are
known as spinocerebellar ataxias (SCA) in genetic nomenclature. In the pregenomic era …

Objective: To develop a reliable and valid clinical scale measuring the severity of ataxia.
Methods: The authors devised the Scale for the Assessment and Rating of Ataxia (SARA) …

The autosomal dominant cerebellar ataxias (ADCAs) represent a heterogeneous group of
neurodegenerative diseases with progressive ataxia and cerebellar degeneration. The …

A growing number of neurodegenerative diseases have been found to result from the
expansion of an unstable trinucleotide repeat. Over the past 6 years, researchers have …

Polyglutamine (polyQ) diseases are a group of inherited neurodegenerative disorders
caused by expanded cytosine-adenine-guanine (CAG) repeats encoding proteins with …

The spinocerebellar ataxias type 1 (SCA1), 2 (SCA2), 3 (SCA3), 6 (SCA6) and 7 (SCA7) are
genetically defined autosomal dominantly inherited progressive cerebellar ataxias (ADCAs) …

Autosomal dominant cerebellar ataxias (ADCAs) are a group of neurodegenerative
disorders characterized by degeneration of the cerebellum and its connections. All ADCAs …

Two decades ago, molecular genetic analysis provided a new approach for defining the
roots of inherited disorders. This strategy has proved particularly powerful because, with …

Objective: To obtain quantitative data on the progression of the most common
spinocerebellar ataxias (SCAs) and identify factors that influence their progression, we …