Introduction: myelomeningocele (MMC) is a closure defect in neural tube, which has been associated with various forms of cognitive and behavior impairments. Studies indicate that hydrocephalus is not sufficient to explain the observed deficits and a possible cause for those deficits would be the Chiari malformation type II (MF-CII).

Patients and Methods: Ten children with ages between 6 and 13 years old with MMC and MF-CII were evaluated using a neuropsychological test battery composed of 15 subtests of the Wechsler Intelligence Scale for Children 4th Edition (WISC - IV), Wisconsin Card Sort Test (WCST), Rey Complex Figures Test and Pediatric Symptom Checklist (PSC).

Results: Four patients had IQs below 70. In neuropsychological assessment all mean scores were below the normative sample mean, and lower scores were found on comprehension skills social rules, sustained attention and mental flexibility. No patients achieved scores above the cut-off score in the PSC for psychiatric disorders, but a Spearman correlation was found linking symptoms reported by parents and the child's age in the amount of 0.64 (p < 0.05), indicating that the psychological impact of the disease increase with age. Five patients presented in the clinical examination signs and symptoms consistent with anxiety disorders according to the DSM-5, indicating that the scale had low sensitivity in this group.

Conclusion: The MMC is a multifaceted disease that includes cognitive and affective aspects and it is important an interdisciplinary rehabilitation model and validated instruments for this population.