INS NYC 2024 Program

Poster	Poster Session 10 Program Schedule 02/17/2024 09:00 am - 10:15 am Room: Shubert Complex (Posters 1-60) Poster Session 10: Neurodevelopmental \| Congenital Conditions Final Abstract #42 Longitudinal Neuropsychological Assessment of a Patient with Developmental Venous Anomaly and Chronic Steal Syndrome Madison Holmes, Mercer University, Atlanta, United States Susan Lee, Children’s Healthcare of Atlanta, Atlanta, United States Category: Stroke/Cerebrovascular Injury and Disease (Child) Keyword 1: assessment Keyword 2: pediatric neuropsychology Keyword 3: congenital disorders Objective: Developmental venous anomaly (DVA) is the most common congenital brain malformation and is typically asymptomatic. Symptomatic DVAs, predominantly described in case series or cohort studies, may be clinically non-specific or include focal neurological deficit, hemorrhage, seizures, and infarct. Flow-related pathology (stenosis, thrombosis) is often identified in these low-incidence cases of symptomatic DVA. Steal syndrome describes hemodynamic changes leading to altered cerebral blood flow among brain regions, potentially causing hypoperfusion and ischemia. This is described in cases of large vascular malformations (e.g., arteriovenous malformation), but chronic hypoperfusion conditions are uncommon in children (e.g., moyamoya). We present a longitudinal neuropsychological case study across three time points and repeat neuroimaging in a now young-adult male with DVA and a steal syndrome. To our knowledge, this is the first case to describe both neurocognitive and neuroimaging data across development in a pediatric patient with these diagnoses. Participants and Methods: The patient is an 18-year-old bilingual (English proficient) and developmentally right-handed male with a history of DVA, chronic steal syndrome of the left hemisphere, right hemiparesis, and epilepsy. He first presented with right-sided hemiparesis and suspected seizure at age 8. He participated in outpatient rehabilitation for persistent right-sided weakness (8 months post) and was prescribed antiseizure medication following his second seizure (9 months post). Neuroimaging (MRI/MRV) has been generally stable, showing anomalous left hemisphere venous drainage and decreased perfusion; left hemisphere volume loss (mildly increased posterior frontal/temporal); extensive left hemisphere encephalomalacia; basal ganglia, amygdala, and left hippocampus signal abnormality; and right cerebellar gliosis. He underwent comprehensive neuropsychological evaluations at ages 8, 9, and 18 years old. Results: The patient underwent three neuropsychological evaluations over a 10-year period; initial (age 8 and 9) and follow-up (age 18). A downward shift in overall performance was seen at follow-up, especially in language and motor skill. Across time points, the patient showed deficits in basic auditory attention, verbal working memory, verbal and reading fluency, and motor functioning. Language and verbal reasoning skills were broadly intact in initial evaluations, whereas increased deficits in verbal fluency and verbal reasoning abilities were seen at follow-up. Behaviorally, the patient’s expressive language was markedly dysfluent at follow-up. He used his right hand or bimanually completed motor tasks in initial evaluations, but he favored his left hand at follow-up secondary to pronounced right hemiparesis. Lower performance was seen on measures of processing speed, visual memory, executive functioning, and academics at follow-up. Perceptual reasoning, receptive language, and rote verbal learning were generally within age-expectations at initial and follow-up evaluations. Detailed description of evaluation data across time points will be provided. A diagnosis of major neurocognitive disorder was given at follow-up. Conclusions: The chronic and progressive sequelae of this patient’s symptomatic DVA has been demonstrated through longitudinal neuropsychological evaluation and correlates with repeat neuroimaging. Results indicate increasingly widespread deficits and progressive impairment in most left-hemisphere mediated skills. Recommendations for functional independence and transition to adult care are discussed. This case highlights the importance of neurocognitive assessment and regular monitoring for patients with symptomatic DVA.

Poster

02/17/2024
09:00 am - 10:15 am
Room: Shubert Complex (Posters 1-60)

Poster Session 10: Neurodevelopmental | Congenital Conditions

Final Abstract #42

Longitudinal Neuropsychological Assessment of a Patient with Developmental Venous Anomaly and Chronic Steal Syndrome

Madison Holmes, Mercer University, Atlanta, United States
Susan Lee, Children’s Healthcare of Atlanta, Atlanta, United States

Category: Stroke/Cerebrovascular Injury and Disease (Child)

Keyword 1: assessment
Keyword 2: pediatric neuropsychology
Keyword 3: congenital disorders

Objective:

Developmental venous anomaly (DVA) is the most common congenital brain malformation and is typically asymptomatic. Symptomatic DVAs, predominantly described in case series or cohort studies, may be clinically non-specific or include focal neurological deficit, hemorrhage, seizures, and infarct. Flow-related pathology (stenosis, thrombosis) is often identified in these low-incidence cases of symptomatic DVA. Steal syndrome describes hemodynamic changes leading to altered cerebral blood flow among brain regions, potentially causing hypoperfusion and ischemia. This is described in cases of large vascular malformations (e.g., arteriovenous malformation), but chronic hypoperfusion conditions are uncommon in children (e.g., moyamoya). We present a longitudinal neuropsychological case study across three time points and repeat neuroimaging in a now young-adult male with DVA and a steal syndrome. To our knowledge, this is the first case to describe both neurocognitive and neuroimaging data across development in a pediatric patient with these diagnoses.

Participants and Methods:

The patient is an 18-year-old bilingual (English proficient) and developmentally right-handed male with a history of DVA, chronic steal syndrome of the left hemisphere, right hemiparesis, and epilepsy. He first presented with right-sided hemiparesis and suspected seizure at age 8. He participated in outpatient rehabilitation for persistent right-sided weakness (8 months post) and was prescribed antiseizure medication following his second seizure (9 months post). Neuroimaging (MRI/MRV) has been generally stable, showing anomalous left hemisphere venous drainage and decreased perfusion; left hemisphere volume loss (mildly increased posterior frontal/temporal); extensive left hemisphere encephalomalacia; basal ganglia, amygdala, and left hippocampus signal abnormality; and right cerebellar gliosis. He underwent comprehensive neuropsychological evaluations at ages 8, 9, and 18 years old.

Results:

The patient underwent three neuropsychological evaluations over a 10-year period; initial (age 8 and 9) and follow-up (age 18). A downward shift in overall performance was seen at follow-up, especially in language and motor skill. Across time points, the patient showed deficits in basic auditory attention, verbal working memory, verbal and reading fluency, and motor functioning. Language and verbal reasoning skills were broadly intact in initial evaluations, whereas increased deficits in verbal fluency and verbal reasoning abilities were seen at follow-up. Behaviorally, the patient’s expressive language was markedly dysfluent at follow-up. He used his right hand or bimanually completed motor tasks in initial evaluations, but he favored his left hand at follow-up secondary to pronounced right hemiparesis. Lower performance was seen on measures of processing speed, visual memory, executive functioning, and academics at follow-up. Perceptual reasoning, receptive language, and rote verbal learning were generally within age-expectations at initial and follow-up evaluations. Detailed description of evaluation data across time points will be provided. A diagnosis of major neurocognitive disorder was given at follow-up.

Conclusions:

The chronic and progressive sequelae of this patient’s symptomatic DVA has been demonstrated through longitudinal neuropsychological evaluation and correlates with repeat neuroimaging. Results indicate increasingly widespread deficits and progressive impairment in most left-hemisphere mediated skills. Recommendations for functional independence and transition to adult care are discussed. This case highlights the importance of neurocognitive assessment and regular monitoring for patients with symptomatic DVA.