Key result
Risperidone resolves psychosis within 72 hours in a Danon disease case.
Why the study?
Psychiatric symptoms related to Danon disease have only been studied in a few case reports and a case series, leaving its pathophysiological mechanisms understudied.
Does risperidone improve psychotic symptoms in a patient with Danon disease?
Case Report (n=1)
Does risperidone improve psychotic symptoms in a patient with Danon disease?
This case report highlights the occurrence of acute psychotic symptoms in a patient with Danon disease and their successful treatment with risperidone.
Answers are grounded in this paper and cite their sources.
Psychiatric symptoms may warrant evaluation in Danon disease; extends sparse case reports but leaves mechanisms open.
Introduction Danon disease is an X-linked cardioskeletal myopathy related to a primary deficiency in lysosome-associated membrane protein-2. Danon disease manifests with the triad of hypertrophic cardiomyopathy, myopathy and intellectual disability.Psychiatric symptoms related to the disease have only been studied in a few case reports (Hatz et al, 2010 and Tanidir et al, 2015) and a case series (Yardeni et al, 2016), leaving its pathophysiological mechanisms understudied. Objectives Provide scientific data on psychotic symptoms in patients with Danon disease. Methods We report an unusual case of a 25-year-old-patient affected by Danon disease that showed an acute psychotic episode. Results Mr P is a 25 year-old white male, with past medical history for Danon disease. Mr P presents hypertrophic cardiomyopathy, Wolf Parkinson White arrhythmia and carries an implantable cardioverter-defibrillator. There are previous records of mild intellectual disability and the patient had experienced anxiety symptoms as well as obsessive thoughts in the past without receiving any specific diagnosis or treatment. He was admitted to the Neurology inpatient unit to study behavioural symptoms with atypical visual and auditory hallucinations, accompanied by paranoid delusions during the last 4 days. He was examined by the liaison psychiatric team. Psychosis remitted within 72 hours after introducing risperidone 3mg per day, with good tolerability. Magnetic resonance imaging (MRI) scan was normal. Conclusions Danon disease is caused by heterogeneity genetic mutation which means that patients can present different levels of clinical manifestations. The current case report highlights the variety of psychiatric symptoms in patients with Danon disease, and raises awareness towards its identification and treatment. Disclosure No significant relationships.
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Tuneu et al. (2021) conducted a case report in Danon disease with acute psychotic episode (n=1). Risperidone was evaluated on Remission of psychosis. Psychosis remitted within 72 hours after introducing risperidone 3mg per day in a 25-year-old male with Danon disease.
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