Journal of Neuropsychiatry

Mental Health Manifestations in a Case of Dandy-Walker Variant

Dandy-Walker variant disorder is a defined as cerebellar dysgenesis, enlarged posterior fossa with variation of cerebellar vermis hypoplasia and less commonly agenesis/thinning of the corpus callosum. Commonly reported symptoms in the literature are intellectual disabilities, epilepsy, and developmental delay. The pathogenesis of psychological manifestations in the illness is believed to be attributed to abnormal corticocerebellar tracts, resulting in what is recognized as cerebellar cognitive affective syndrome. Reports about the clinical manifestation and management challenges associated with Dandy-Walker variant coexisting with impulsive/destructive behavior are very few. We report a case of young adult man with Dandy-Walker variant who presented with challenging behaviors outbursts including verbal abusive, physically assaultive, environmental destruction and sexually disinhibited behavior and destructive behavior causing high risk of injury and financial consequences. His presentation suggests and organic psychosis diagnosis in the context of uncontrolled epilepsy. This report highlights the challenges in diagnostic formulation and management of this case; especially the collaboration of different services and the need of monitoring the pharmacotherapy, behavioral support plans and risk management to safeguard the patient and his family. The report also explores the possible factors contributing to the progression of his behavioral and mental health. In comparison with previously published cases.

Author(s):

Mohamed El Tahir,  Ibrahim Abdelhafez, and Salma Salman

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