[P2-167] Temperament and Character in Patients with Gorlin syndrome
[Background] With the advances in medical technology as well as social background, various neuroscientific personality studies have been reported. The personality is largely formed by the neurotransmitters. Among them, the Sonic hedgehog (Shh) signaling plays an important role for the development of neurotransmitter producing cells. Gorlin syndrome, characterized by developmental abnormality and tumorigenesis, shows enhanced Shh signaling from the prenatal period. Gorlin syndrome patients have gentle disposition, so we studied their personality.
[Methods] We enrolled genetically confirmed 13 Gorlin syndrome patients (7 males and 6 females, mean age 24.0) and 14 controls (3 males and 11 females, mean age 34.9). The Japanese version of Temperament and Character Inventory (TCI), a self-fill questionnaire consists of 125 questions, was used. TCI is based on the seven-dimensional model of temperament and character by Cloninger, and is controlled by the neurotransmitter. They were evaluated by using TCI and investigating clinical symptoms.
[Result] The control as a 1.00, Gorlin syndrome patients were 1.04 in novelty seeking, 0.90 (p=0.02) in harm avoidance, 0.96 in reward dependence, 1.08 in persistence, 0.95 in self-directedness, 1.01 in cooperativeness and 1.01 in self-transcendence. In addition, in the Gorlin syndrome patients with developmental abnormality, who had the impact of the Shh signaling enhancement from prenatal period, harm avoidance was significantly decrease as 0.84 (p=0.01).
[Conclusion] Gorlin syndrome patients showed low score in harm avoidance, especially with developmental abnormality were significantly lower. These might be coincident with their gentle disposition.
[Methods] We enrolled genetically confirmed 13 Gorlin syndrome patients (7 males and 6 females, mean age 24.0) and 14 controls (3 males and 11 females, mean age 34.9). The Japanese version of Temperament and Character Inventory (TCI), a self-fill questionnaire consists of 125 questions, was used. TCI is based on the seven-dimensional model of temperament and character by Cloninger, and is controlled by the neurotransmitter. They were evaluated by using TCI and investigating clinical symptoms.
[Result] The control as a 1.00, Gorlin syndrome patients were 1.04 in novelty seeking, 0.90 (p=0.02) in harm avoidance, 0.96 in reward dependence, 1.08 in persistence, 0.95 in self-directedness, 1.01 in cooperativeness and 1.01 in self-transcendence. In addition, in the Gorlin syndrome patients with developmental abnormality, who had the impact of the Shh signaling enhancement from prenatal period, harm avoidance was significantly decrease as 0.84 (p=0.01).
[Conclusion] Gorlin syndrome patients showed low score in harm avoidance, especially with developmental abnormality were significantly lower. These might be coincident with their gentle disposition.