Othello syndrome as a presenting manifestation of progressive supranuclear palsy

Background: Othello syndrome, or delusional jealously, has been reported to occur in a variety of psychiatric and neurologic disorders, but no cases have been described in patients with progressive supranuclear palsy. This case study describes a patient with progressive supranuclear palsy who presented with an Othello syndrome, posing a major diagnostic challenge. Awareness of the full clinical spectrum of behavioral manifestations of this and other neuropsychiatric disorders is quintessential for early and correct management of these patients. Increased collaboration between Psychiatrists and Neurologists is needed in a time where the frontiers between the two specialties are becoming increasingly blurred. Case report: The case describes an eighty-year-old white male, with no previous psychiatric or neurological history, who developed a two-year history of delusional jealously (Othello syndrome). Over the last year he became progressively apathetic and socially withdrawn. His overall state of apathy was punctuated by intermittent episodes of verbal aggression and violent behavior towards his wife and her alleged lovers, sometimes accompanied by an increased libido and sexually aggressive behavior. His past medical history was significant for diabetes, hypertension and erectile dysfunction following a radical prostatectomy. He had no family history of psychiatric or neurological disorders. He denied alcohol or other substance abuse, and his developmental history was unremarkable. At the time of his initial evaluation, he was alert, temporally disoriented, aspontaneous in speech and movement with a masked facies and a generalized slowness of movement. His mini-mental state examination score was 24. Laboratory investigation and neuroimaging were unremarkable and did not explain his clinical picture. He was diagnosed as having a delusional disorder and was started on quetiapine 25 mg/d, which was effective at reducing his delusional thoughts and aggressive behavior. After discharge from the hospital, he developed a progressive akinetic-rigid syndrome over the course of several months. His neurological examination revealed vertical gaze palsy, masked facies and a startled expression, dysartria, dysphagia, axial rigidity, cervical dystonia, bradykinesia, unstable posture, widely based and unstable gait with freezing, and frontal release signs. Neuropsychological evaluation revealed apathy, motor perseveration, slowed cognitive processing, complex attentional deficits, executive dysfunction and mild memory impairment. Magnetic resonance imaging scan showed cortical and subcortical atrophy, T2-white-matter hyperintensities, selective midbrain atrophy and periaqueductal signal changes. The patient was started on levodopa/carbidopa, with poor improvement. Discussion: A large spectrum of behavioral abnormalities are increasingly recognized as common in progressive supranuclear palsy and other neurodegenerative disorders.[1] These symptoms can be the first manifestation of the disorder and they are often indistinguishable from psychiatric disorders. This results in Psychiatrists being the first clinicians to evaluate these patients, who normally don't possess the necessary training to identify subtle neurological abnormalities, such as slow vertical saccades and square wave jerks on ocular examination, that are often present early in the course of the disease. Delayed diagnosis and initiation of treatment leads to a faster disease progression with decreased quality of life for the patient. This case raises awareness about the importance of interdisciplinary teamwork involving Psychiatrists and Neurologists. References: Gerstenecker, A., Duff, K., Mast, B., Litvan, I., 2013. Behavioral abnormalities in progressive supranuclear palsy. Psychiatry Res 210(3), 1–13.