Kernicterus Spectrum Disorders Diagnostic Toolkit: validation using retrospective chart review.

BACKGROUND: Kernicterus Spectrum Disorders (KSDs) result from hyperbilirubinemia-induced brain injury. We developed a Toolkit (KSD-TK) to predict the likelihood of KSDs. This study aims to validate the KSD-TK by comparing it to clinical diagnoses made by the Kernicterus Clinic in the Division of Neurology. METHODS: Through retrospective chart review, we completed a KSD-TK for 37 patients evaluated between 2011 and 2019 using highest bilirubin, newborn risk factors, neonatal exam, follow-up exam, auditory testing, tooth enamel, and MRI brain results. KSD-TK results were compared to the clinical diagnoses given by a kernicterus expert (SS). RESULTS: Of 37 patients, 29 were clinically diagnosed with kernicterus, including 14/14 with KSD-TK scored as "definite", 14/15 "probable", and 1/2 with "possible" kernicterus. None of 6 patients with KSD-TK "not kernicterus" were clinically diagnosed with kernicterus. Combining KSD-TK "definite" and "probable", the KSD-TK has 96.6% sensitivity and 87.5% specificity. Each KSD-TK component had high sensitivity, but only three had specificity ≥0.75: auditory neuropathy spectrum disorder, abnormal movements and/or tone on follow-up exam, and abnormal globus pallidus and/or subthalamic nucleus on MRI. CONCLUSION: The KSD-TK is a promising screening tool for patients at risk for kernicterus. IMPACT: This study provides validation of a Kernicterus Spectrum Disorders (KSDs) Toolkit. The toolkit provides screening criteria for predicting KSD diagnosis. Scores of definite or probable have high sensitivity and specificity for KSDs. Abnormal auditory processing, exam, and MRI were most specific for KSDs.

The Influence of Lifestyle Factors on the Burden of Pediatric Migraine.

There is a gap in patient education and coaching of lifestyle factors related to pediatric migraine, which nurses are in a unique position to fill in order to provide comprehensive care to these patients. In order to help fill this gap, we conducted a targeted review of studies examining migraine and lifestyle factors in children and adolescents. Studies older than 2010, studies examining adults above the age of 18, studies not available in the English language, and secondary sources were excluded from the review. A final sample of 42 studies was included in this review. Lifestyle factors including stress, sleep, obesity, and diet were identified as playing a significant role in increasing the frequency, severity, and duration of migraine attacks in pediatric patients. Based on these findings, a framework is discussed for practical applications of this knowledge by nursing staff working in primary and specialty care clinics.

PARACENTRAL ACUTE MIDDLE MACULOPATHY IN A PATIENT WITH FREQUENT MIGRAINE WITH AURA: A CASE REPORT.

BACKGROUND/PURPOSE: To explore the possible relationship between Paracentral Acute Middle Maculopathy (PAMM) and migraine. Paracentral acute middle maculopathy is a recently described clinical and optical coherence tomography entity involving infarction of the inner nuclear layer secondary to deep retinal capillary ischemia. It presents as a painless paracentral scotoma and often results in permanent visual deficits. Migraine, especially migraine with aura, has been shown to cause structural changes in the retinal microvasculature and to be a risk factor for retinal ischemia. METHODS: A case report and review of the literature. RESULTS: A 39-year-old woman with migraine with visual aura presented with a discrete, monocular, painless "buffalo-shaped" paracentral scotoma, which started during a period of frequent typical visual auras. Her exam and optical coherence tomography were consistent with PAMM. CONCLUSION: We propose that migraine is a risk factor for the development of PAMM. The changes in retinal microvasculature in migraine may increase a patient's susceptibility to retinal ischemia. Other risk factors for retinal ischemia, including diabetes, hypertension, hyperlipidemia, sickle cell disease, and orbital trauma, have been shown to be associated with PAMM. Further research should be conducted to determine whether there is a definite relationship between migraine and PAMM.