A-136 Chiari1 Malformation and Syringomyelia: The Role of Neuropsychological Assessment in Treatment and Education Planning

Abstract

Abstract Objective: Chiari 1 malformation (CM1) occurs when the cerebellum bulges through an opening at the base of the skull, putting pressure on the brain and spinal cord. CM1 is frequently related to syringomyelia, characterized by a fluid filled cyst in the spinal cord. CM1 is associated with sensorimotor symptoms such as headache, weakness in the arms, and difficulty walking. This case study will expand upon the existing literature regarding neurodevelopmental impact. Method: In this single case study, a 13-year, 5-month old male with CM1/S was seen for neuropsychological assessment. Diagnosis of CM1/S and foramen magnum decompression with suboccipital craniectomy and C1 laminectomy occurred five months prior. Evaluation was subsequent to concerns regarding executive functioning, literacy skills, and handwriting. Educational disabilities were recognized but not associated with his neurological condition. Results: Assessment revealed average verbal and non-verbal cognitive skills, low average working memory, and extremely low processing speed. Phonological awareness was intact. Phonological memory was extremely low. Rapid naming was below average. Measures of executive functioning and cognitive flexibility suggested weaknesses. Upper extremity fine motor speed and coordination fell in the impaired range. Academically, broad mathematics skills were within the average range while broad reading and writing were low average. Conclusions: The neurodevelopment of children with CM1 and syringomyelia with comorbid neurodevelopmental disorders is not well characterized. Neuropsychological assessment and follow-up is critical for diagnosing disorders, educational planning, and monitoring of overall development. Neuropsychology can play a critical role in facilitating an understanding of the impact of neurological disorders in educational settings.