Magnetic resonance imaging (MRI) findings in 21 patients with congenital muscular dystrophy (CMD) complicated by cerebral anomalies were analysed from data collected at several Departments of Child Neurology, The Netherlands: Free University Hospital, and Emma Children’s Hospital, Amsterdam; Sophia Children’s Hospital, Rotterdam; Leiden University Hospital; Groningen University Hospital; and St Radboud University Hospital, Nijmegen. Four subgroups of CMD were defined by MRI characteristics: Grp 1, Walker-Warburg syndrome (WWS) (5 cases), CMD with retardation, macrocephaly, hydrocephalus, and eye abnormalities, showed posterior fossa abnormalities, aqueductal stenosis, polymicrogyria, and cerebral white matter demyelination on MRI; Grp 2, less severe form of muscle-eye-brain disease than WWS (4 patients), with normal or only focal white matter changes, less severe cerebral cortical dysplasia, frequent myopia, and similar posterior fossa abnormalities; Grp 3, CMD with isolated occipital agyria (3 patients), normal intelligence, no eye abnormalities, white matter changes, and hypoplasia of pons and vermis; and Grp 4, classic merosin-deficient CMD (MD-CMD) (8 patients), with diffuse cerebral white matter abnormalities only. 
COMMENT. The MRI is important in the classification of patients with congenital muscular dystrophy, both in terms of prognosis and in genetic studies. Four subgroups may be defined: Walker-Warburg syndrome, muscle-eye-brain disease, merosin-deficient CMD, including the classic form and that of Fukuyama, and a newly recognized subtype with occipital agyria.