An autosomal recessive muscular dystrophy caused by mutations in the POMT1 gene, encoding protein O-mannosyl-transferase 1. It is associated with characteristic brain and eye malformations, profound mental retardation, and early death. The disease is muscular dystrophy-dystroglycanopathy (congenital with brain and eye anomalies), type A1 (MONDO_0009364). Also known as: muscle-eye-brain-POMT1 related, muscular dystrophy-dystroglycanopathy (congenital with brain and eye anomalies) type A, 1.