Abstract
BACKGROUND: Shwachman-Diamond Syndrome (SDS) is a rare genetic disorder with documented cognitive and behavioral challenges. However, its socio-pragmatic dynamics remain underexplored, particularly in cooperative interactions where social norms and economic considerations intersect. OBJECTIVE: This study investigates the socio-behavioral dynamics of SDS, focusing on how children with the condition navigate cooperative interactions. Using computational pragmatics, we aimed to identify the underlying principles guiding their social behavior. METHODS: A cohort of 10 children (5 SDS, 5 matched controls) participated in ecological and cognitive tasks, including the WISC-V "Comprehension" subtest, NEPSY-II social perception tasks, and the Trognon Ecological Side Task for the Assessment of Speech-Act Processing (TEST-ASAP). Dialogues were analyzed using the Topological and Kinetic (2TK) model and a Recurrent Neural Network (RNN), enabling fine-grained computational insights into their interaction patterns. RESULTS: Children with SDS exhibited cooperative behaviors shaped by perceived economic benefits, often at the expense of established social norms. Unlike behaviors classically observed in other pathologies such as autism spectrum disorders, where responses are influenced by the directness of communication, SDS behaviors were driven by personal gain, regardless of the indirectness of requests. Computational analyses revealed strong divergences in dialogical alignment when tasks lacked direct benefits, even with corrective prompts. CONCLUSION: SDS children demonstrate a transactional approach to social interactions, prioritizing personal benefits over cooperative norms. Using our unique dialogic and computational frameworks, we show that perceived personal gain strongly shapes their cooperation patterns. These findings underscore the need for targeted interventions to enhance pragmatic skills and adaptive functioning in SDS, given their unique interaction profiles.