Patients with cerebellar damage often present with the cerebellar motor syndrome of
dysmetria, dysarthria and ataxia, yet cerebellar lesions can also result in the cerebellar …

Niemann-Pick disease type C (NP-C) is a rare, progressive, irreversible disease leading to
disabling neurological manifestations and premature death. The estimated disease …

Friedreichʼs ataxia (FA) is caused by a variant of the Frataxin (FXN) gene, leading to its
downregulation and progressively impaired cardiac and neurological function. Current gold …

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) …

Niemann–Pick disease type C (NP-C) is a rare inherited neurovisceral disease caused by
mutations in either the NPC1 (in 95% of cases) or the NPC2 gene (in around 5% of cases) …

Measuring the severity of neurologic dysfunction in patients with inherited ataxias, including
Friedreich ataxia (FA), is difficult because of the variable rate of progression, the variable …

To develop a brief ataxia rating scale (BARS) for use by movement disorder specialists and
general neurologists. Current ataxia rating scales are cumbersome and not designed for …

Objective: To develop and evaluate a clinical Spastic Paraplegia Rating Scale (SPRS) to
measure disease severity and progression. Methods: A 13-item scale was designed to rate …

Background We assessed the clinimetric properties of ataxia rating scales and functional
tests, and made recommendations regarding their use. Methods A systematic literature …

The objective of this study was to test the reliability and validity of the Scale for the
Assessment and Rating of Ataxia (SARA) in ataxia patients not suffering from autosomal …