A JOURNAL OF NEUROLOGY The cerebellar cognitive profile

Brain, 2011

The cerebellar role in non-motor functions is supported by the clinical finding that lesions confined to cerebellum produce the cerebellar cognitive affective syndrome. Nevertheless, there is no consensus regarding the overall cerebellar contribution to cognition. Among other reasons, this deficiency might be attributed to the small sample sizes and narrow breadths of existing studies on lesions in cerebellar patients, which have focused primarily on a single cognitive domain. The aim of this study was to examine the expression of cerebellar cognitive affective syndrome with regard to lesion topography in a large group of subjects with cerebellar damage. We retrospectively analysed charts from patients in the Ataxia Lab of Santa Lucia Foundation between 1997 and 2007. Of 223 charts, 156 were included in the study, focusing on the importance of the cerebellum in cognition and the relevance of lesion topography in defining the cognitive domains that have been affected. Vascular topography and the involvement of deep cerebellar nuclei were the chief factors that determined the cognitive profile. Of the various cognitive domains, the ability to sequence was the most adversely affected in nearly all subjects, supporting the hypothesis that sequencing is a basic cerebellar operation. proposed that cerebro-cerebellar connectivity is based on discrete 'parallel circuits'. These parallel loops are organized such by guest on December 2, 2015 http://brain.oxfordjournals.org/ Downloaded from The data are reported as mean (SD). BilCb = bilateral cerebellar group; CA = cerebellar atrophy group; Cb = total cerebellar group; focal = focal cerebellar lesion; LCb = left cerebellar group; RCb = right cerebellar group; M = male; F = female. Cerebellar cognitive profile Brain 2011: 134; 3672-3686 | 3673 by guest on December 2, 2015 http://brain.oxfordjournals.org/ Downloaded from LesNclCb = involvement cerebellar deep nuclei within lesion; LesNclSurg = involvement cerebellar deep nuclei in surgical lesions; NoLesNclCb = no involvement cerebellar deep nuclei within lesion; NoLesNclSurg = no involvement cerebellar deep nuclei in surgical lesions; PICA = posterior inferior cerebellar artery; RM-I = subjects with magnetic resonance image; SCA = superior cerebellar artery; M = male; F = female. 3674 | Brain 2011: 134; 3672-3686 A. M. Tedesco et al. by guest on December 2, 2015 http://brain.oxfordjournals.org/ Downloaded from Cerebellar cognitive profile Brain 2011: 134; 3672-3686 | 3675 by guest on December 2, 2015 http://brain.oxfordjournals.org/ Downloaded from Cerebellar cognitive profile Brain 2011: 134; 3672-3686 | 3677 by guest on December 2, 2015 http://brain.oxfordjournals.org/ Downloaded from ICA = idiopathic cerebellar ataxia; OPA = olivo-ponto-cerebellar atrophy; SCA1 = spinocerebellar atrophy type 1. Cerebellar cognitive profile Brain 2011: 134; 3672-3686 | 3679 by guest on December 2, 2015 http://brain.oxfordjournals.org/ Downloaded from Vascular territory effects Anatomical, developmental and neuropsychological data indicate the existence of cerebellar motor functions in the anterior lobe and of cognitive functions in the posterior lobe (Timmann et al., 2008; Stoodley and Schmahmann, 2010). The vascular territory of the superior cerebellar artery primarily involves the anterior lobe, and that of the posterior inferior cerebellar artery mostly involves the Cerebellar cognitive profile Brain 2011: 134; 3672-3686 | 3681 by guest on December 2, 2015 http://brain.oxfordjournals.org/ Downloaded from LCb = left cerebellar damage; LesNcl = lesions of the deep cerebellar nuclei; NoLesNcl = no lesions of the deep cerebellar nuclei; PICA = posterior inferior cerebellar artery; RCb = right cerebellar damage; SCA = superior cerebellar artery. Cerebellar cognitive profile Brain 2011: 134; 3672-3686 | 3683 by guest on December 2, 2015 http://brain.oxfordjournals.org/ Downloaded from A. M. Tedesco et al. by guest on December 2, 2015 http://brain.oxfordjournals.org/ Downloaded from

Cortex, 2010

The traditional view on cerebellar functioning has recently been challenged by results from neuroanatomical, neuroimaging and clinical studies. In this contribution, eighteen patients with primary cerebellar lesions (vascular: n=13; neoplastic: n=5) were systematically investigated by means of an extensive neuropsychological test battery. Fifteen patients (83%) presented with a broad variety of cognitive and linguistic deficits following cerebellar damage. Disturbances of attention (72%), executive functioning (50%) and memory (50%) were most commonly found.

The Cerebellum, 2019

The cerebellar cognitive affective syndrome (CCAS) was first described by Schmahmann and Sherman in 1998. Despite their clear depiction of the syndrome, it is our experience that the CCAS has not yet found solid ground as a disease entity in routine clinical practice. This made us question the dimension of the CCAS in cerebellar patients. We performed a systematic review of the literature according to the PRISMA guidelines, in order to answer the question whether patients with acquired isolated cerebellar lesions perform significantly worse on neuropsychological testing compared to healthy controls. Studies were selected based on the predefined eligibility criteria and quality assessment. The systematic search resulted in ten studies, mainly observational cohorts consecutively including adult patients with isolated cerebellar lesions. Patients were compared to healthy controls, and neuropsychological investigation was done within one year of diagnosis. Meta-analysis of the twelve tests that were done in two or more studies showed that cerebellar patients perform significantly worse on Phonemic Fluency, Semantic Fluency, Stroop Test (naming, reading and interference), Block Design test and WMS-R visual memory. Cerebellar patients have significant and relevant deficits in the visuospatial, language and executive function domain. This meta-analysis therefore emphasizes the importance of the cerebellar cognitive affective syndrome as described by Schmahmann and Sherman.

Archives of Psychiatry and Psychotherapy, 2013

Brain, 1998

Anatomical, physiological and functional neuroimaging studies suggest that the cerebellum participates in the organization of higher order function, but there are very few descriptions of clinically relevant cases that address this possibility. We performed neurological examinations, bedside mental state tests, neuropsychological studies and anatomical neuroimaging on 20 patients with diseases confined to the cerebellum, and evaluated the nature and severity of the changes in neurological and mental function. Behavioural changes were clinically prominent in patients with lesions involving the posterior lobe of the cerebellum and the vermis, and in some cases they were the most noticeable aspects of the presentation. These changes were characterized by: impairment of executive functions such as planning, set-

Neurological Sciences, 2004

Cerebellar pathology commonly shows important motor signs and less evident cognitive dysfunction. The ’cerebellar cognitive affective syndrome’ is characterised by impairment on executive function, spatial cognition, language and behaviour. We report the case of a man with acute onset of transitory motor features and severe mental disorders. Cranial CT and brain MRI revealed extended cerebellar lesions. Neuropsychological assessment disclosed deficits of attention, executive function and memory. Auditory event–related potentials showed abnormal P300. These data suggest a pure “cerebellar cognitive affective syndrome” and strengthen the hypothesis of cerebellar cognitive function modulation.

The Cerebellum, 2010

Cerebellar contribution to non-motor functions has been supported by several animal, human and functional neuroimaging studies. Which cognitive skills and to what extent the cerebrocerebellar loops contribute remain unclear, however. Among other reasons, this may be explained by the fact that authors have studied patients with extracerebellar lesions. The goal of this study was to explore the role of the cerebellum in cognition and affect in patients with autosomal recessive cerebellar ataxia type 1 (ARCA-1), a newly described inherited cerebellar disease characterised by middle-age onset of ataxia as well as pure, severe and diffuse cerebellar atrophy. To this end, the performance of 21 ARCA-1 patients was compared to that of 21 normal controls paired for age and education on a 3-h battery of attention, executive, visuospatial and memory skills. Results indicated similar IQ, naming and declarative memory abilities between groups. ARCA-1 patients showed significant deficits in attention (attention span, speed of information processing, sustained attention), verbal working memory and visuospatial/visuoconstructional skills (3-D drawings, copy of a complex figure). Functional brain imaging in a subset of patients showed diffuse severe cerebellar hypometabolism associated with a small area of right parietal hypometabolism. None of the patients presented a significant affective syndrome. Correlational analyses suggested that cognitive deficits could not be explained by the severity of motor deficits, duration of disease or mood. Altogether, this study confirms that pure cerebellar damage as seen in ARCA-1 is associated with significant cognitive impairments but not with psychiatric comorbidity. These deficits are correlated with an overall moderate impact on patient's autonomy. Our data favour an indirect participation of the dorsolateral prefrontal and posterior parietal cortical areas to the cerebrocerebellar circuit.

Encyclopedia of Clinical Neuropsychology, 2011

Anatomical, physiological and functional neuroimaging studies suggest that the cerebellum participates in the organization of higher order function, but there are very few descriptions of clinically relevant cases that address this possibility. We performed neurological examinations, bedside mental state tests, neuropsychological studies and anatomical neuroimaging on 20 patients with diseases confined to the cerebellum, and evaluated the nature and severity of the changes in neurological and mental function. Behavioural changes were clinically prominent in patients with lesions involving the posterior lobe of the cerebellum and the vermis, and in some cases they were the most noticeable aspects of the presentation. These changes were characterized by: impairment of executive functions such as planning, set

Dev Disabil Res Revs, 2008

The postoperative cerebellar mutism syndrome (CMS), consisting of diminished speech output, hypotonia, ataxia, and emotional lability, occurs after surgery in up to 25% of patients with medulloblastoma and occasionally after removal of other posterior fossa tumors. Although the mutism is transient, speech rarely normalizes and the syndrome is associated with long-term adverse neurological, cognitive, and psychological sequelae. The clinical, neuroradiographic, and neuropsychological findings associated with CMS as well as possible mechanisms of injury are reviewed. Theories about the pathophysiology of CMS have evolved along with our understanding of the cerebellum as an important structure in the distributive neurocircuitry underlying complex speech, cognition, and behavior. CMS shares many similarities with the cerebellar cognitive affective syndrome, more commonly described in adults and consisting of disturbances of executive function, visuospatial skills, nonmotor language, and affect regulation. Future directions include more thorough neuropsychological characterization, functional and diffusion tensor imaging studies, and investigations into the underlying differences that may make some patients more vulnerable to CMS. Wiley-Liss, Inc. Dev Disabil Res Rev 200814:221-228.

Neuropsychologia, 1992