Successful neuropsychological rehabilitation in a patient with Cerebellar Cognitive Affective Syndrome

Abstract

The objective of this case study was to describe the neuropsychological rehabilitation of a 16-year-old patient who presented a Cerebellar Cognitive Affective Syndrome (CCAS) following a bilateral cerebellar hemorrhage. The patient presented severe and diffuse cognitive deficits, massive behavioral disorders, and emotion regulation difficulties. The cognitive rehabilitation was performed in the chronic phase (one year after the onset of the hemorrhage) using a transdisciplinary neurobehavioral approach based on the patient's favorite interest (soccer). A significant behavioral and cognitive improvement was observed. The patient became progressively independent in all activities of daily living and was discharged home. The Functional Independence Measure at discharge was 124=126 (vs. 37=126 at entry). The patient was able to complete his schooling despite the mild cognitive and behavioral sequelae. This first description of the use of neurobehavioral therapy in a case of chronic CCAS suggests that (a) major clinical improvement can occur more than one year after the onset of the CCAS, showing the importance of long-term and intensive neurorehabilitation; and (b) when the cerebellum cannot properly play its regulator role in cognition, neuropsychological intervention through a behavioral and cognitive approach can be of great help by acting as an external modulator to help the patient regain control over himself.

Virtual Reality Social Prediction Improvement and Rehabilitation Intensive Training (VR-SPIRIT) for paediatric patients with congenital cerebellar diseases: study protocol of a randomized controlled trial.

“…These findings are in line with clinical reports on the importance of behavioural, affective and social skills alterations presented by patients with cerebellar diseases (12,31,54,55). Though, available rehabilitative interventions for these pathologies typically address motor and cognitive alterations (26,(28)(29)(30), but not social cognition deficits. Our study tries to fill this gap, proposing a protocol that aims to improve social prediction skills.…”

“…In this light, rehabilitation programs for children and adolescents affected by congenital cerebellar malformation should not only involve the recovery of motor functions, but also of higher-orders abilities, such as cognitive processing of social stimuli (25). However, only few single-case studies have reported data concerning cognitive rehabilitation for patients with cerebellar diseases, either congenital (26) or acquired (27)(28)(29)(30). Crucially, none of these previous studies has focused on social cognition and has exploited the boosting of the specific predictive abilities of the cerebellum in order to treat and rehabilitate neuropsychiatric symptoms shown by cerebellar patients (31).…”

Virtual Reality Social Prediction Improvement and Rehabilitation Intensive Training (VR-SPIRIT) for paediatric patients with congenital cerebellar diseases: study protocol of a randomized controlled trial.

“…These findings are in line with clinical reports on the importance of behavioural, affective and social skills alterations presented by patients with cerebellar diseases(12,31,60,61). Though, available rehabilitative interventions for these pathologies typically address motor and cognitive alterations(26,(28)(29)(30), but not social cognition deficits. Our study tries to fill this gap, proposing a protocol that aims to improve social prediction skills.Rehabilitation treatments based on specific neural mechanisms of the brain offer the opportunity to design interventions with a clear rationale, but they also directly serve as a clinical validation of theoretical knowledge on brain functioning(62).…”

“…In this light, rehabilitation programs for children and adolescents affected by congenital cerebellar malformation should not only involve the recovery of motor functions, but also of higher-orders abilities, such as cognitive processing of social stimuli (25). However, only few single-case studies have reported data concerning cognitive rehabilitation for patients with cerebellar diseases, either congenital (26) or acquired (27)(28)(29)(30). Crucially, none of these previous studies has focused on social cognition and has exploited the boosting of the specific predictive abilities of the cerebellum in order to treat and rehabilitate neuropsychiatric symptoms shown by cerebellar patients (31).…”

Recent Advances in the Treatment of Cerebellar Disorders

“…There are no systemic studies on cognitive rehabilitation [78]. Since there are only a few case reports that confirm the notion of Schmahmann syndrome [78][79][80][81][82], appropriate rehabilitation protocols and their outcomes remain unclear [78] (Table 3).…”