Nausea and Vomiting as Initial Manifestations of Pediatric NMOSD.

Intractable nausea and vomiting are commonly attributed to gastrointestinal (GI) conditions but can sometimes be a symptom of an underlying central nervous system disease. One potentially overlooked neurologic cause of intractable nausea and vomiting that is refractory to antiemetics is area postrema syndrome (APS). APS is a condition characterized by lesions of the dorsal caudal medulla and is considered a core clinical feature of neuromyelitis optica spectrum disorder (NMOSD). APS is present in up to 30% of patients ultimately diagnosed with NMOSD and can be the first presenting symptom of NMOSD in 12% of patients, as our case illustrates. Importantly, APS is highly responsive to immunotherapy. We present the case of a 14-year-old female with a history of migraines who presented to the emergency department multiple times for persistent nausea, vomiting, and hiccups. Multiple GI diagnoses were considered until she developed additional neurologic symptoms that prompted further workup and revealed the final diagnosis of NMOSD-APS. We posit that NMOSD-APS should be considered in the differential diagnosis for patients with intractable nausea and vomiting, especially in patients with a negative GI workup result and poor response to antiemetics.

Child Neurology and Neurodevelopmental Disabilities Program Directors' Opinions on Preference Signaling in the 2023-2024 National Resident Matching Program Match: A Survey.

BACKGROUND: Changes in residency recruitment have significantly altered how programs and applicants evaluate each other including virtual interviews, discontinuation of the United States Medical Licensing Exam Step 2 Clinical Skills exam, and transition of United States Medical Licensing Exam Step 1 to pass-fail scoring. To improve program-applicant fit, the Electronic Residency Application Service introduced supplemental application features including geographic preference, program signaling, and the opportunity to highlight impactful and meaningful experiences. We sought to evaluate child neurology (CN) and neurodevelopmental disabilities (NDD) program director's (PD) opinions regarding these changes. METHODS: A 10-question anonymous survey was sent to CN (n=75) and NDD (n=8) PDs. The questions centered on PDs' opinions regarding components of the supplemental application, having a standard application review period and in-person recruitment activities. Answer choices to the questions were all close-ended. Respondents could select questions to complete. RESULTS: Thirty-eight CN residency PDs (49%) and 4 NDD residency PDs (50%) responded to the survey. Among CN PDs, there was strong support for use of the supplemental application questions and for the use of 3 program signals per applicant. Most PDs supported a standardized application review period prior to programs sending interview offers; however, there was no consensus on the appropriate length of time. Nearly half agreed with virtual-only interviews, and 62% agreed with the option of in-person second-look visits. CONCLUSIONS: CN PDs generally support many of the recent or proposed changes to residency recruitment. The impact of these changes on recruitment will be a topic of future investigation.

Pearls & Oy-sters: Leber Hereditary Optic Neuropathy-Plus Masquerading as Neuromyelitis Optica Spectrum Disorder in a 2-Year-Old Child.

"Leber hereditary optic neuropathy (LHON-Plus)" is a phenotype of LHON that is characterized by extraocular neurologic manifestations, which may be the first manifestations of the disease.

Submandibular Gland Invasion by Oral Cavity Cancers: A Systematic Review.

OBJECTIVE: The submandibular gland (SMG) is typically included in level I neck dissection specimens despite limited data demonstrating SMG invasion. The main objective of this article is to determine the rate and pathways of SMG invasion by squamous cell carcinoma of the oral cavity and oropharynx. DATA SOURCES: A systematic review of relevant studies was performed, evaluating articles identified via the PubMed, Cochrane, and Medline databases. REVIEW METHODS: Descriptive features of primary tumors, primary treatment modalities, the rate and pathway of SMG invasion, and survival outcomes, if present, were reported following the PRISMA guidelines. RESULTS: The initial literature search yielded 273 articles, of which 17 met inclusion criteria. A total of 2306 patients with 2792 SMG resections were analyzed. Fifty-eight resections (2.0%) were revealed to have tumor involvement. Among patients with SMG tumor involvement, the most common invasion pathway was direct SMG invasion by primary tumor (43 of 58, 74.1%). The second-most common mode of SMG invasion was from involved adjacent lymph nodes (10 of 58, 17.2%). Only 3 SMG resections out of 2792 (0.1%) had isolated metastatic parenchyma without evidence of direct tumor invasion or invasion by involved lymph nodes. CONCLUSION: Given this rarity of SMG involvement, preservation of SMG might be feasible in selected patient population. However, additional studies need to examine the functionality of preserved SMGs among patients who receive postoperative adjuvant radiation therapy.

Conditional Disabled-1 Deletion in Mice Alters Hippocampal Neurogenesis and Reduces Seizure Threshold.

Many animal models of temporal lobe epilepsy (TLE) exhibit altered neurogenesis arising from progenitors within the dentate gyrus subgranular zone (SGZ). Aberrant integration of new neurons into the existing circuit is thought to contribute to epileptogenesis. In particular, adult-born neurons that exhibit ectopic migration and hilar basal dendrites (HBDs) are suggested to be pro-epileptogenic. Loss of reelin signaling may contribute to these morphological changes in patients with epilepsy. We previously demonstrated that conditional deletion of the reelin adaptor protein, disabled-1 (Dab1), from postnatal mouse SGZ progenitors generated dentate granule cells (DGCs) with abnormal dendritic development and ectopic placement. To determine whether the early postnatal loss of reelin signaling is epileptogenic, we conditionally deleted Dab1 in neural progenitors and their progeny on postnatal days 7-8 and performed chronic video-EEG recordings 8-10 weeks later. Dab1-deficient mice did not have spontaneous seizures but exhibited interictal epileptiform abnormalities and a significantly reduced latency to pilocarpine-induced status epilepticus. After chemoconvulsant treatment, over 90% of mice deficient for Dab1 developed generalized motor convulsions with tonic-clonic movements, rearing, and falling compared to <20% of wild-type mice. Recombination efficiency, measured by Cre reporter expression, inversely correlated with time to the first sustained seizure. These pro-epileptogenic changes were associated with decreased neurogenesis and increased numbers of hilar ectopic DGCs. Interestingly, neurons co-expressing the Cre reporter comprised a fraction of these hilar ectopic DGCs cells, suggesting a non-cell autonomous effect for the loss of reelin signaling. We also noted a dispersion of the CA1 pyramidal layer, likely due to hypomorphic effects of the conditional Dab1 allele, but this abnormality did not correlate with seizure susceptibility. These findings suggest that the misplacement or reduction of postnatally-generated DGCs contributes to aberrant circuit development and hyperexcitability, but aberrant neurogenesis after conditional Dab1 deletion alone is not sufficient to produce spontaneous seizures.