Aslı Demirtaş Tatlıdede1, Haşmet A. Hanağası2, Murat Çabalar3, Vildan Yayla3

1Behavioral Neurology And Movement Disorders Unit, Department Of Neurology, Istanbul University Istanbul Faculty Of Medicine, Istanbul, Turkey Ve Department Of Neurology, Bakirkoy Dr. Sadi Konuk Research And Training Hospital, Istanbul, Turkey
2Behavioral Neurology And Movement Disorders Unit, Department Of Neurology, Istanbul University Istanbul Faculty Of Medicine, Istanbul, Turkey
3Department Of Neurology, Bakirkoy Dr. Sadi Konuk Research And Training Hospital, Istanbul, Turkey

Keywords: Multiplesystematrophy, cerebellum, cognitive affective cerebellar syndrome, psychosis

Abstract

Besides its well-established motor functions, the modulatory role of cerebellum in the neural networks subserving cognition and emotion has been supported by several lines of evidence obtained from neuroanatomical investigations, functional neuroimaging and clinical research. Damage to cerebellar posterior lobe and vermis may lead to a cognitive affective cerebellar syndrome primarily comprising cognitive disorders characterized by executive dysfunctioning, language and visuospatial impairments in addition to disorders of affect and other psychiatric impairments. Cognitive and affective symptoms have often been reported in neurodegenerative cerebellar diseases while psychotic symptoms occur less frequently. In this article, we describe an extraordinary case of multiple system atrophy (MSA-C), who presented with cognitive affective cerebellar syndrome and psychotic symptoms before cerebellar ataxia was evident. The wide variety of symptoms observed in this case underlines the critical role of cerebellum in modulation of networks engaged in cognition, affect, thought and perception, and provides further evidence regarding the contribution of cerebellar dysfunction to several neuropsychiatric symptoms.