Highlights•Cognitive impairment in SCAs is well-established, but not universal in all
patients.•The CCAS Scale is the current standard for identifying cognitive impairments in …

ATXN2 is one of the few genes where a single gene causes several diseases and/or
modifies several and disparate neurological disorders. Hence, understanding mutagenesis …

Background Riluzole has been reported to be beneficial in patients with cerebellar ataxia;
however, effectiveness in individual subtypes of disease is unclear due to heterogeneity in …

Immune-mediated cerebellar ataxias (IMCAs) have diverse etiologies. Patients with IMCAs
develop cerebellar symptoms, characterized mainly by gait ataxia, showing an acute or …

In degenerative neurological disorders such as Parkinson's disease, a convergence of
widely varying insults results in a loss of dopaminergic neurons and, thus, the motor …

Gait ataxia is one of the most common and impactful consequences of cerebellar
dysfunction. Purkinje cells, the sole output neurons of the cerebellar cortex, are often …

With many viable strategies in the therapeutic pipeline, upcoming clinical trials in hereditary
and sporadic degenerative ataxias will benefit from non-invasive MRI biomarkers for patient …

Currently, nine polyglutamine (polyQ) expansion diseases are known. They include
spinocerebellar ataxias (SCA1, 2, 3, 6, 7, 17), spinal and bulbar muscular atrophy (SBMA) …

Background and Objectives Brain MRI abnormalities and increases in neurofilament light
chain (NfL) have mostly been observed in cross-sectional studies before ataxia onset in …

Objective Transcranial sonography (TCS) is a noninvasive neuroimaging technique,
visualizing deep brain structures and the ventricular system. Although widely employed in …