Abstract

An increasing number of findings confirm the significance of cerebellum in affecting regulation and early learning. Most consistent findings refer to association of congenital vermis anomalies with deficits in nonmotor functions of cerebellum. In this paper we presented a young woman who was treated since sixteen years of age for polysubstance abuse, affective instability, and self-harming who was later diagnosed with borderline personality disorder. Since the neurological and neuropsychological reports pointed to signs of cerebellar dysfunction and dysexecutive syndrome, we performed magnetic resonance imaging of brain which demonstrated partially developed vermis and rhombencephalosynapsis. These findings match the description of cerebellar cognitive affective syndrome and show an overlap with clinical manifestations of borderline personality disorder.

Highlights

  • IntroductionThere are an increasing number of findings supporting the fact that cerebellum, apart from its significance in movement coordination (sensomotoric cerebellum), plays an important role in cognitive and emotional regulation (cognitive and limbic cerebellum) [1]

  • There are an increasing number of findings supporting the fact that cerebellum, apart from its significance in movement coordination, plays an important role in cognitive and emotional regulation [1]

  • Most consistent findings refer to association of congenital vermis anomalies with deficits in nonmotor functions of cerebellum

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Summary

Introduction

There are an increasing number of findings supporting the fact that cerebellum, apart from its significance in movement coordination (sensomotoric cerebellum), plays an important role in cognitive and emotional regulation (cognitive and limbic cerebellum) [1]. In comparison with malformations of other parts of cerebellum and the acquired lesions, congenital vermis deficit presents as pronounced slow psychomotor development and worse cognitive functioning (lower IQ) and language skills, as well as affective dysregulation [2]. Rhombencephalosynapsis (RS) is a rare congenital posterior fossa malformation characterised by hypogenesis or agenesis of the vermis, dorsal fusion of cerebellar hemispheres, and fusion of the dentate nuclei and superior cerebellar peduncles [3]. Brain MRI, which was performed because of severe clinical picture and neurological and cognitive deficits, showed partially developed vermis and rhombencephalosynapsis. This sheds a new light on the severity of and nonresponsiveness to medication

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