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C-55 Normal Brain Cells in Abnormal Places: Contributions of Conjoint Neuropsychological, Pediatric, Neurologic, and Psychiatric Assessment in the Diagnosis and Behavioral Management of Pediatric Subependymal Heterotopia

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Subependymal heterotopia (SH) is a development brain malformation from abnormal neuronal proliferation and migration resulting in developmental delays and focal childhood epilepsy (Barkovich, 2000). SH results from the interruption of… Click to show full abstract

Subependymal heterotopia (SH) is a development brain malformation from abnormal neuronal proliferation and migration resulting in developmental delays and focal childhood epilepsy (Barkovich, 2000). SH results from the interruption of normal gray matter migration beneath the ependyma of the lateral ventricles. Most males are spontaneously aborted, and although there is some genetic uncertainty, affected females are thought to be X-linked (Xq28) recessive (Mitchell, et.al., 2000). SH prevalence in epileptic children with brain dysgenesis is between 13-20% and manifests in motor, visual-spatial, cognitive, and psychological deficits (Data, et.al., 2018). We present a case study of a seventeen-year-old female who was diagnosed at age eight with ADD and LD by self-report check-lists and achievement tests. Behavioral irregularities and seizure onset lead pediatrics to request neuropsychological testing. Findings revealed cognitive and emotional impairment and consequently further medical evaluation was recommended. Pediatric, neurological, and psychiatric examination, EEG, and MRI confirmed SH along the lateral margin of the right lateral ventricle. Psychometric testing revealed low average FSIQ, PRI, and PSI, impaired arithmetic operations and problem solving, graphomotor deficits, motor speed and executive/planning difficulties, emotional dysregulation, age-inappropriate interpersonal skills, and social-behavioral regression. Neuropsychological assessment provided clear evidence of cognitive impairment beyond the initial diagnoses, suggested lateralized dysfunction, and implicated frontal and subcortical behavioral correlates not explained by other dispositional variables. Comprehensive neuropsychological assessment provided detailed information toward conjoint treatment planning by pediatrics, neurology, psychology, psychiatry, and teachers. Findings provided meaningful diagnostic clarification of SH and furthered clinical understanding of this adolescent’s social, academic, neuropsychological, and psychiatric needs.

Keywords: subependymal heterotopia; normal brain; assessment; brain; brain cells

Journal Title: Archives of Clinical Neuropsychology
Year Published: 2019

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