Este síndrome, que se hereda de manera autosómica por un gen recesivo incompleto, se caracteriza por: degeneración retiniana, retraso mental, obesidad, hipogenitalismo y polidactilia, se acompaña de nefropatía.
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La degeneración de la retina es del tipo llamado «degeneración de conos y bastones» (RP atípica). A menudo, las alteraciones observadas en el fondo de ojo son mínimas, a pesar de la disminución de las funciones visuales y las anomalías del ERG, muy marcadas.
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