PBZ (Pini, Bonuccelli, Zappella): Italian scale for clinical evaluation in Rett syndrome

Giorgio Pini, Laura Congiu, Michele Zappella, Ubaldo Bonuccelli, Anna Maria Romanelli

Compared to other scales used up to now, such as the ISS (Kerr et al., 2001), the PBZ is particularly useful for its sensitivity in grasping symptomatological modifications in the observational field, such as in clinical and pharmacological trials, during rehabilitative programmes, and environmental enrichment.

Keywords
Classical Rett syndrome, Zappella Variant, Italian evaluation scoring (PBZ), International Scoring System (ISS), MECP2, CDKL5 and FOXG1 genes.

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