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Frontal behavioral syndromes in Prader-Willi syndrome
Kaeko Ogura1, Mayumi Shinohara, Kousaku Ohno
1Department of Behavioral Neurology and Cognitive Neuroscience, Tohoku University Graduate School of Medicine, 2-1, Seiryo-machi, Aoba-ku, Sendai, Japan. ogura-kaeko@umin.ac.jp
Brain & Development
|February 12, 2008
Summary
Prader-Willi syndrome (PWS) is linked to high rates of abnormal eating, stereotypy, and collecting behaviors. These issues may stem from orbitofrontal cortex and anterior temporal lobe dysfunction.
Area of Science:
- Neuroscience
- Genetics
- Behavioral Science
Background:
- Prader-Willi syndrome (PWS) is a genetic neurodevelopmental disorder.
- PWS is characterized by hyperphagia, emotional issues, and compulsive behaviors.
- These behaviors are potentially linked to orbitofrontal cortex and anterior temporal lobe dysfunction.
Purpose of the Study:
- To determine the prevalence of eating and non-eating behavioral disturbances in PWS.
- Utilized assessment tools designed for frontotemporal dementia and frontal lobe injury patients.
Main Methods:
- A 35-question survey was administered to parents of 250 individuals with PWS in Japan.
- The survey covered eating behaviors, stereotypy, and collecting behaviors.
- Participants ranged in age from 1 to 42 years.
Main Results:
- High prevalence rates of symptoms were observed across all behavioral categories.
- Eating behaviors were significantly correlated with stereotypy and collecting behaviors.
- Older individuals exhibited higher prevalence and severity of certain behaviors.
Conclusions:
- Abnormal eating, stereotyped, and collecting behaviors are common in PWS.
- A potential link exists between abnormal eating and non-eating behaviors, characteristic of frontal behavioral syndromes.
- Behavioral abnormalities likely reflect orbitofrontal cortex and anterior temporal lobe dysfunction.
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