Neuropsychological Features of Patients with Spinocerebellar Ataxia (SCA) Types 1, 2, 3, and 6
Open Access
- 26 May 2010
- journal article
- research article
- Published by Springer Science and Business Media LLC in The Cerebellum
- Vol. 9 (3), 433-442
- https://doi.org/10.1007/s12311-010-0183-8
Abstract
A subtype-specific impairment of cognitive functions in spinocerebellar ataxia (SCA) patients is still debated. Thirty-two SCA patients (SCA1, 6; SC2, 3; SCA3, 15; SCA6, 8) and 14 matched healthy controls underwent neuropsychological evaluation testing attention, executive functions, episodic and semantic memory, and motor coordination. Severity of ataxia was assessed with the Scale for the Assessment and Rating of Ataxia (SARA), nonataxia symptoms with the Inventory of Non-Ataxia Symptoms. Depressive symptoms were evaluated with the Beck Depression Inventory. The SARA scores of our SCA patients (range 1–19.5) indicated an overall moderate ataxia, most pronounced in SCA6 and SCA1. Mean number of nonataxia symptoms (range 0–2.2) were most distinct in SCA1 and nearly absent in SCA6. SCA1 performed poorer than controls in 33% of all cognitive test parameters, followed by SCA2, SCA3, and SCA6 patients (17%). SCA 1–3 patients presented mainly attentional and executive dysfunctions while semantic and episodic memory functions were preserved. Attentional and executive functions were partly correlated with ataxia severity and fine motor coordination. All patients exhibited mildly depressed mood. Motor and dominant hand functions were more predictive for depressed mood than cognitive measures or overall ataxia. Besides motor impairments in all patients, SCA patients with extracerebellar pathology (SCA 1–3) were characterized by poor frontal attentional and executive dysfunction while mild cognitive impairments in predominantly cerebellar SCA6 patients appeared to reflect mainly cerebellar dysfunction. Regarding the everyday relevance of symptoms, (dominant) motor hand functioning emerged as a marker for the patient’s mood.Keywords
This publication has 46 references indexed in Scilit:
- Unique and shared validity of the “Wechsler logical memory test”, the “California verbal learning test”, and the “verbal learning and memory test” in patients with epilepsyEpilepsy Research, 2009
- Cognitive Impairment in Spinocerebellar DegenerationEuropean Neurology, 2009
- New insights into the pathoanatomy of spinocerebellar ataxia type 3 (Machado–Joseph disease)Current Opinion in Neurology, 2008
- Social economic costs and health‐related quality of life in patients with degenerative cerebellar ataxia in SpainMovement Disorders, 2008
- Reliability and validity of the scale for the assessment and rating of ataxia: A study in 64 ataxia patientsMovement Disorders, 2007
- Diagnostik und Behandlung alkoholbezogener StörungenFortschritte der Neurologie · Psychiatrie, 2005
- Cognitive deficits in Machado‐Josephs diseaseAnnals of Neurology, 1996
- The cerebellum and cognitive functions in humans.Behavioral Neuroscience, 1993
- Visual Learning Deficits in Nonresected Patients with Right Temporal Lobe EpilepsyCortex, 1991
- The Purdue Pegboard: norms and studies of reliability and validity.Journal of Applied Psychology, 1948