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We report a case of type I Arnold-Chiari malformation that is very peculiar because of its particular onset especially characterized by psychiatric symptoms. These symptoms were so prevailing that, for fifteen years, they masked the neurological aspects and the patient was treated with high doses of psychotropic drugs without any benefit. If the Arnold-Chiari malformation had been diagnosed before the development of severe hydro-syringomyelia, the patient could have underwent decompressive neurosurgery which may have improved her quality of life.   It is worthwhile to highlight that psychotic symptoms may be caused by this congenital malformation, that typically has an aspecific onset. Therefore it’s important to consider an eventual organic etiology while challenging a resistant clinical picture with unusual presentation.   Case description:  A 51-year-old woman reported neurological symptoms consisting of headaches, blurred vision, diplopia, tinnitus, vertigo and psychiatric symptoms including obsessive ideas about the fear of killing her son, auditory and visual pseudo-hallucinations. The symptoms had developed suddenly at the age of 35 years and persisted thereafter. She underwent multiple hospitalizations in psychiatric units and was treated with a variety of psychopharmacological approaches without substantial improvement. We performed a brain MRI that identified a type I Arnold-Chiari malformation. We assessed psychiatric symptoms using the Structured Clinical Interview for DSM IV Axis I Disorders, the Brief Psychiatric Rating Scale, and the Yale-Brown Obsessive-Compulsive Scale. A tailored psychopharmacological therapy led to a partial improvement in mood and anxiety but not in hallucinations.   Discussion:  We want to highlight how important is, in everyday psychiatric clinical practice, not to focus only on psychiatric aspects but consider the patient globally, because in this case psychiatric problems were the onset presentation of a rare neurological syndrome.

Case Report: Atypical psychotic onset of type I Arnold-Chiari malformation