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Neuropsychological functioning in a young adult case of schizencephaly
B J Allen1, S A Reid-Arndt, T Rolan
1Health Psychology, University of Missouri, Columbia, MO 65212, USA. brittanyjeanallen@gmail.com
Insights
Schizencephaly, a rare brain disorder, can cause significant cognitive and motor impairments. Neuropsychological evaluation is crucial for understanding these effects and guiding functional support.
Area of Science:
- Neurology
- Neuroscience
- Developmental Neuroscience
Background:
- Schizencephaly is a rare neuromigrational disorder causing cerebral clefts and associated neurological deficits.
- Existing literature on neuropsychological manifestations of schizencephaly, particularly in adults, is limited.
- This study presents a case of adult-onset bilateral schizencephaly.
Observation:
- A 29-year-old woman with bilateral schizencephaly underwent comprehensive neuropsychological testing.
- Testing revealed preserved intelligence and memory.
- Significant impairments were observed in attention, executive functions, expressive language, visual-spatial skills, and bilateral motor coordination.
Findings:
- Despite intact general intelligence and memory, the patient exhibited specific cognitive and motor deficits.
- These deficits substantially impacted her functional abilities, including employability.
- The findings highlight the heterogeneous nature of schizencephaly's cognitive and functional sequelae.
Implications:
- This case underscores the importance of detailed neuropsychological assessments in individuals with schizencephaly.
- Understanding specific deficits is crucial for tailoring interventions and support.
- The study contributes to the limited literature on adult schizencephaly, emphasizing its variable presentation.
Abstract:
Schizencephaly is a rare neuromigrational/organizational disorder characterized by the development of cerebral clefts, which are typically associated with neurological sequelae including seizures, motor disturbances, and cognitive dysfunction. Although there are multiple case reports of schizencephaly and associated neurological sequelae, primarily in children, the literature regarding neuropsychological manifestations of schizencephaly is limited. This article reviews the case of a woman diagnosed with bilateral schizencephaly at age 29. Neuropsychological testing revealed intact intelligence and memory functioning. However, impairments were noted in attention, executive functioning, expressive language skills, visual-spatial abilities, and bilateral manual motor skills, all of which were adversely impacting her functional abilities (e.g., ability to be gainfully employed). Given the potential variability in deficits associated with schizencephaly, this case demonstrates the utility of neuropsychological evaluation for understanding cognitive and functional consequences of bilateral schizencephaly.
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