The prevalence of Chiari malformation among children with persistent dysphagia.

BACKGROUND: Brain imaging has been utilized as a diagnostic tool in the workup of persistent pediatric dysphagia, yet the indications for imaging and the prevalence of Chiari malformation (CM) have not been established. OBJECTIVE: to evaluate the prevalence of CM anomalies in children who underwent brain magnetic resonance imaging (MRI) for pharyngeal dysphagia and to review the clinical findings in the CM group compared to the non-CM group. METHODS: A retrospective cohort study of children who underwent MRI as part of the workup for the diagnosis of dysphagia in a tertiary care children's hospital between 2010 and 2021. RESULTS: 150 patients were included. The mean age at diagnosis of dysphagia was 1 ± 3.4 years, and the mean age at MRI was 3.5 ± 4.2 years. Common comorbidities in our cohort included prematurity (n = 70, 46.7 %), gastroesophageal reflux (n = 65, 43.3 %), neuromuscular/seizure disorder (n = 53,35.3 %), and underlying syndrome (n = 16, 10.7 %). Abnormal brain findings were seen in 32 (21.3 %) patients, of whom 5 (3.3 %) were diagnosed with CM-I and 4 (2.7 %) patients with tonsillar ectopia. Clinical characteristics and dysphagia severity were similar between patients with CM-I/tonsillar ectopia and patients without tonsillar herniation. CONCLUSIONS: Brain MRI should be pursued as part of the work-up for persistent dysphagia in pediatric patients given the relatively higher prevalence of CM-I. Multi-institutional studies are required to establish the criteria and timing of brain imaging in patients with dysphagia.

Imaging of Inherited Metabolic and Endocrine Disorders.

"Inherited metabolic disorders represent a large group of disorders of which approximately 25% present in neonatal period with acute metabolic decompensation, rapid clinical deterioration, and often nonspecific imaging findings. Neonatal onset signifies the profound severity of the metabolic abnormality compared with cases with later presentation and necessitates rapid diagnosis and urgent therapeutic measures in an attempt to decrease the extent of brain injury and prevent grave neurologic sequela or death. Here, the authors discuss classification and clinical and imaging findings in a spectrum of metabolic and endocrine disorders with neonatal presentation."

Coronal Gradient Echo MRI to Visualize the Zona Incerta for Deep Brain Stimulation Targeting in Parkinson's Disease.

INTRODUCTION: Deep brain stimulation of the zona incerta is effective at treating tremor and other forms of parkinsonism. However, the structure is not well visualized with standard MRI protocols making direct surgical targeting unfeasible and contributing to inconsistent clinical outcomes. In this study, we applied coronal gradient echo MRI to directly visualize the rostral zona incerta in Parkinson's disease patients to improve targeting for deep brain stimulation. METHODS: We conducted a prospective study to optimize and evaluate an MRI sequence to visualize the rostral zona incerta in patients with Parkinson's disease (n = 31) and other movement disorders (n = 13). We performed a contrast-to-noise ratio analysis of specific regions of interest to quantitatively assess visual discrimination of relevant deep brain structures in the optimized MRI sequence. Regions of interest were independently assessed by 2 neuroradiologists, and interrater reliability was assessed. RESULTS: Rostral zona incerta and subthalamic nucleus were well delineated in our 5.5-min MRI sequence, indicated by excellent interrater agreement between neuroradiologists for region-of-interest measurements (>0.90 intraclass coefficient). Mean contrast-to-noise ratio was high for both rostral zona incerta (6.39 ± 3.37) and subthalamic nucleus (17.27 ± 5.61) relative to adjacent white matter. There was no significant difference between mean signal intensities or contrast-to-noise ratio for Parkinson's and non-Parkinson's patients for either structure. DISCUSSION/CONCLUSION: Our optimized coronal gradient echo MRI sequence delineates subcortical structures relevant to traditional and novel deep brain stimulation targets, including the zona incerta, with high contrast-to-noise. Future studies will prospectively apply this sequence to surgical planning and postimplantation outcomes.