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Treatment of a Prader-Willi Patient with Recurrent Catatonia
Hana M Poser1, Alexandru E Trutia2
1Medical College of Virginia, 1201 E. Marshall Street, Richmond, VA 23284, USA.
Insights
Prader-Willi syndrome, a genetic disorder, can present with recurrent catatonia. Electroconvulsive therapy (ECT) offered partial symptom improvement in a complex case, highlighting treatment challenges.
Area of Science:
- Genetics
- Neurology
- Psychiatry
Background:
- Prader-Willi syndrome is a complex genetic disorder with varied clinical manifestations.
- Individuals with Prader-Willi syndrome, particularly maternal uniparental disomy, face an elevated risk for psychosis.
- Catatonia is a severe neuropsychiatric syndrome that can occur in Prader-Willi syndrome.
Purpose of the Study:
- To report the treatment course of a 24-year-old female with Prader-Willi syndrome and recurrent catatonia.
- To illustrate the complexities and challenges in managing catatonia within the context of Prader-Willi syndrome.
Main Methods:
- Case report detailing the clinical presentation and treatment interventions.
- Initial assessment included a lorazepam challenge test.
- Treatment involved benzodiazepines and subsequently electroconvulsive therapy (ECT).
Main Results:
- The patient initially responded to a lorazepam challenge test but failed benzodiazepine treatment.
- Eight sessions of electroconvulsive therapy (ECT) led to initial improvement in catatonic symptoms.
- Symptom resolution was not linear, characterized by periods of improvement followed by relapse.
Conclusions:
- Treatment of catatonia in Prader-Willi syndrome can be complex and challenging.
- Electroconvulsive therapy (ECT) may provide partial and temporary relief for catatonia in this population.
- Further research is needed to establish optimal treatment strategies for catatonia in Prader-Willi syndrome.
Abstract:
Prader-Willi is a genetic disorder characterized by neonatal hypotonia, hyperphagia, short stature, hypogonadism, and mental delay. This disorder can result from multiple mechanisms, most commonly a deletion of paternal chromosome 15, leaving a single maternally derived chromosome 15. Individuals who have a maternal uniparental disomy of chromosome 15 have a higher risk for developing psychosis compared to other forms of Prader-Willi. The following report details the treatment course of a 24-year-old female with Prader-Willi and recurrent catatonia. The patient initially had a positive lorazepam challenge test but subsequently failed treatment with benzodiazepines. She then received eight electroconvulsive therapy (ECT) treatments after which she showed improvement from initial catatonic state. However, the resolution in her symptoms did not follow a linear course but would show periods of improvement followed by a return of catatonic features. This case provides an example of the complexity of treatment of a patient with a genetic disorder and recurrent catatonia.
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