Hallervorden-Spatz disease in a psychiatric setting

Neurodegenerative disorders of sufficient severity to be lethal are also likely to generate psychiatric symptomatology. At times, behavioral changes may predate neurologic manifestations, whereas at other times disturbances in mental status and physical functioning may coexist. In either situation, accurate assessment and appropriate treatment may prove challenging. The case of Hallervorden-Spatz disease reported here illustrates this difficulty; the authors present it to highlight the general issues that often arise in this group of illnesses. In this patient, as well as in three of his relatives, initially subtle neurologic signs were preceded by and then intermingled with significant and sometimes severe symptoms of depression. The authors emphasize the importance of attending to the neurologic symptom picture and family history in order to more appropriately assess the psychiatric manifestations of the disorder. Knowledge of neurodegenerative illnesses, even those as admittedly rare as Hallervorden-Spatz disease, can facilitate accurate and prompt diagnostic assessment, guide treatment strategies (including avoidance of inappropriate interventions), and help to more realistically define outcome expectations.