The cerebellar cognitive affective syndrome

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

Archives of Psychiatry and Psychotherapy, 2013

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.

Applied Neuropsychology: Child, 2016

Psychosomatics, 2010

Background: Pathology of the cerebellum has traditionally been associated with motor symptoms, vertigo, and nystagmus. Patients with cerebellar disorders do not usually receive psychiatric evaluations. Objective: The authors seek to alert clinicians to the association between cerebellar disease and psychiatric symptoms. Method: The authors describe a patient with uncommon psychiatric morbidity associated with cerebellar dysfunction, and provide a brief review of previous research on this phenomenon. Results: Neurology consultants suggested that physical exam findings and behavioral changes could be accounted for by cerebellar cognitive affective syndrome. This syndrome involves dysfunction of the cerebellum, including classic cerebellar findings, in addition to cognitive difficulties and affective/personality changes. Conclusion: The suspected etiology was post-infectious cerebellitis from Epstein-Barr virus infection.

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.

Aim. Growing number of research shows the role of the cerebellum in the regulation of affect. Lesions of the cerebellum can lead to emotional disregulation, a significant part of the Cerebellar Cognitive Affective Syndrome. The aim of this article is to analyze the most recent studies concerning the cerebellar participation in emotional reactions and to present three cases: two female and one male who suffered from cerebellar damage and presented post-traumatic affective and personality change.

During the past decades, the role of the cerebellum as sole modulator of motor coordination, balance and motor speech has been substantially redefined. Results from neuroanatomical, neuroimaging and clinical studies have extended the functional role of the cerebellum to cognitive and affective regulation. Neuroanatomical studies convincingly demonstrated cerebellar connectivity with the supratentorial association areas involved in higher cognitive functioning, whereas functional neuroimaging studies provided evidence of cerebellar activation during a variety of cognitive tasks. In this contribution, a concise overview of the cerebellar role in various cognitive processes, such as executive functioning, memory, learning, attention, visuospatial regulation, language and behavioral-affective modulation, is presented. In addition, cerebellar syndromes such as the cerebellar cognitive affective syndrome (CCAS) and the posterior fossa syndrome (PFS) will be discussed. Although extensive research has substantially broadened the insights into the cognitive and affective role of the cerebellum, the precise nature of the cerebellar contribution to cognitive, linguistic and affective processing is not yet clear. In this review, clinical and functional neuroimaging data will be discussed that substantiate some of the current hypotheses dealing with the presumed neurobiological mechanisms underlying the cognitive and affective modulatory role of the cerebellum.

The Cerebellum, 2019

Sporadically advocated over the last two centuries, a cerebellar role in cognition and affect has been rigorously established in the past few decades. In the clinical domain, such progress is epitomized by the “cerebellar cognitive affective syndrome” (“CCAS”) or “Schmahmann syndrome.” Introduced in the late 1990s, CCAS reflects a constellation of cerebellar-induced sequelae, comprising deficits in executive function, visuospatial cognition, emotion–affect, and language, over and above speech. The CCAS thus offers excellent grounds to investigate the functional topography of the cerebellum, and, ultimately, illustrate the precise mechanisms by which the cerebellum modulates cognition and affect. The primary objective of this task force paper is thus to stimulate further research in this area. After providing an up-to-date overview of the fundamental findings on cerebellar neurocognition, the paper substantiates the concept of CCAS with recent evidence from different scientific angle...

Brain, 2000

neuropsychological data in 19 children who underwent resection of cerebellar tumours but who received neither produce impairments in higher function as exemplified cranial irradiation nor methotrexate chemotherapy. by the cerebellar cognitive affective syndrome. It is not Impairments were noted in executive function, including yet known whether similar findings occur in children with planning and sequencing, and in visual-spatial function, acquired cerebellar lesions, and whether developmental expressive language, verbal memory and modulation of factors influence their presentation. In studies to date, affect. These deficits were common and in some cases survivors of childhood cerebellar tumours who demoncould be dissociated from motor deficits. Lesions of the strate long-term deficits in cognitive functions have vermis in particular were associated with dysregulation undergone surgery as well as cranial irradiation or of affect. Behavioural deficits were more apparent in methotrexate treatment. Investigation of the effects of older than younger children. These results reveal that the cerebellar lesion independent of the known deleterious clinically relevant neuropsychological changes may occur effects of these agents is important for understanding following cerebellar tumour resection in children. Age at the role of the cerebellum in cognitive and affective the time of surgery and the site of the cerebellar lesion development and for informing treatment and rehabilitainfluence the neurobehavioural outcome. The results of tion strategies. If the cerebellar contribution to cognition the present study indicate that the cerebellar cognitive and affect is significant, then damage in childhood may affective syndrome is evident in children as well as in influence a wide range of psychological processes, both adults, and they provide further clinical evidence that the as an immediate consequence and as these processes fail cerebellum is an essential node in the distributed neural circuitry subserving higher-order behaviours. to develop normally later on. In this study we evaluated