Manic phenomena in an adult with Prader-Willi syndrome.
نویسندگان
چکیده
references 1. Butler MG. Prader-Willi syndrome: obesity due to genomic imprinting. Curr Genomics 2011; 12: 204-15. a 37 year old man presented with genetically confirmed Prader-Willi syndrome, a genetic multisystem disorder caused, in this case, by a deletion of the paternal genes in the 15q11-q13 region. The most striking characteristic symptom is relentless pursuit of food with devastating effects on body weight, health and quality of life. The patient lives in a residential home for adults with PraderWilli syndrome where diet is strictly controlled. In the last few years his primary problem was severe behavioral disruption which was treated with a combination of psychiatric medications (risperidone, sertraline, topiramate and clotiapine). During a recent trial to taper down the psychiatric medications, he developed an acute maniphorm psychotic state during which he ate uncontrollably and developed abdominal pain. X-ray demonstrated the presence of metallic objects such as batter2. Cassidy SB, Schwartz S, Miller JL, Driscoll DJ. Prader-Willi syndrome. Genet Med 2012; 14 (1): 10-26.
منابع مشابه
Dental Management of Patients with Prader Willi Syndrome
Prader–Willi syndrome (PWS) is a genetic disorder which occurs with a frequency of about one in 10,000–30,000 live newborns. Both males and females, and all races are equally affected. PWS is a complex disorder with multiple disabilities, and the main defect is found in the hypothalamus. Child with PWS at the age between 2 and 3 years becomes constantly hungry and if the diet is not controlled,...
متن کاملExperience of severe desaturation during anesthetic induction period in an obese adult patient with Prader-Willi syndrome -A case report-
Prader-Willi syndrome is characterized by infantile hypotonia, childhood-onset obesity, short stature, mental retardation, hyperphagia, hypogonadism. After infantile hypotonia phase, patient is prone to morbid obesity due to hyperphagia. Complications associated with morbid obesity are recognized as the main risk factors for death the lifespan of patients with Prader-Willi syndrome. We experien...
متن کاملAdult Prader-Willi Syndrome: An Update on Management
With the advancement of medical care, the survival of most patients with syndromal genetic disease is greatly improved. In this case report, we have reported an adult Prader-Willi syndrome patient who is being diagnosed at the age of 33. The clinical features and their associated complications during adulthood have been reviewed.
متن کاملAnomalous basal ganglia connectivity and obsessive-compulsive behaviour in patients with Prader Willi syndrome.
BACKGROUND Prader Willi syndrome is a genetic disorder with a behavioural expression characterized by the presence of obsessive-compulsive phenomena ranging from elaborate obsessive eating behaviour to repetitive skin picking. Obsessive-compulsive disorder (OCD) has been recently associated with abnormal functional coupling between the frontal cortex and basal ganglia. We have tested the potent...
متن کاملSuccessful treatment of heart failure in an adult patient with Prader-Willi syndrome.
Prader-Willi Syndrome (PWS) is a rare genetic disorder characterized by physical, psychological and physiological abnormalities. Obesity and related cardiovascular diseases are a common problem in adult patients with PWS. This report describes a case of adult PWS with heart failure associated with marked obesity and sleep-disordered breathing that was successfully treated with oxygen therapy, a...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
- The Israel Medical Association journal : IMAJ
دوره 16 1 شماره
صفحات -
تاریخ انتشار 2014