NEURODEGENERATION WITH BRAIN IRON ACCUMULATION – ATYPICAL PKAN: A CASE REPORT OF HALLERVORDEN-SPATZ SYNDROME WITH NEUROPSYCHIATRIC FEATURES AND SUICIDAL BEHAVIOR

Abstract

Background: Syndromes with Neurodegeneration with Brain Iron Accumulation (NBIA) are a rare group of neurodegenerative disorders, being Pantothenate Kinase-Associated Neurodegeneration (PKAN), also known as Hallervorden-Spatz Syndrome, the most common NBIA disorder, with an estimated prevalence of 1 in 1 million. In atypical PKAN, motor involvement tends to be less severe, while cognitive decline and psychiatric features, may be the leading, even presenting sign. Objectives: We present a clinical report of suicidal behavior in a patient with this diagnosis, illustrating the relevance of an adequate evaluation and intervention. Methods: In addition to describing the clinical case, searches were undertaken in PubMed and other databases using keywords such as “Iron accumulation”, “Hallervorden-Spatz”, “PKAN”, “Suicide” and “Suicidal behavior”. Results: We here report the case of a 52-year-old female patient who attempted suicide by hanging. As far as we know, there are no published reports involving suicidal behavior in this type of neurodegenerative disorders, nor particularly in PKAN. Discussion: Some patients eventually diagnosed with PKAN will initially carry a diagnosis of a psychiatric disease. A high index of suspicion is essential for an adequate intervention involving all neuropsychiatric symptoms.