Neurocutaneous Disorders in Pregnancy.

Importance: Neurocutaneous disorders have significant implications for care of the pregnant patient. As neurocutaneous disorders are uncommon, obstetricians may be unfamiliar with these disorders and with recommendations for appropriate care of this population. Objective: This review aims to summarize existing literature on the interaction between neurocutaneous disorders and pregnancy and to provide a guide for physicians caring for an affected patient. Evidence Acquisition: A PubMed, MEDLINE, and Google Scholar search was carried out with a broad range of combinations of the medical subject headings (MeSH) terms "pregnancy," "Sturge -Weber," "Neurofibromatosis Type 1," "neurofibromatosis type 2," "von Hippel Lindau," "Tuberous Sclerosis," "neurocutaneous disorder," "treatment," "congenital malformations," "neurodevelopmental defects," "miscarriage," "breastfeeding," "autoimmune," "pathophysiology," and "management." References of included articles were searched to identify any articles that may have been missed after the above method was used. Results: Neurocutaneous disorders are associated with increased pregnancy-associated maternal and fetal/neonatal morbidity, largely surrounding hypertensive disorders, epilepsy, and medication exposure. Some features of neurocutaneous disorders may be worsened or accelerated by pregnancy. Neurocutaneous disorders can often be diagnosed prenatally. Therefore, directed assessment should be offered to affected individuals with a personal or family history of a neurocutaneous disorder. Conclusion and Relevance: Patients affected by neurocutaneous disorders who are pregnant or planning for future pregnancy should be carefully followed by a multidisciplinary team, which could include maternal-fetal medicine, neurology, and anesthesia, as well as other relevant subspecialists. Additional research is required regarding optimal counseling and management of these patients.

Hereditary Neuropathy With Liability to Pressure Palsy Detected During the Use of Recreational Drugs.

Background: Nitrous oxide (N2O) has been an increasingly popular recreational drug over the past few years. Abuse is associated with severe neurological complications and even fatal outcomes. Purpose: Here we present a case of chronic nitric oxide abuse in a teenager presenting with rapidly progressive mixed sensory and motor polyneuropathy. Results: The initial diagnostic workup excluded electrolyte derangement, heavy metal intoxication, autoimmune neuropathy, myopathy, hematological disorders, and thyroid disease. On further questioning, patient reported 8-months of inhalation of nitrous oxide, commonly known as "whippets". Subsequent tests revealed low Vitamin B12 and elevated homocysteine level. Eventual genetic test demonstrated a heterozygous deletion in the gene that encodes the peripheral myelin protein 22 (PMP22), consistent with a diagnosis of Hereditary Neuropathy with Liability to Pressure Palsies (HNPP). Conclusion: The association of neurologic and genetic findings with the timeline of nitrous oxide inhalation suggests a multifactorial etiology of her symptoms, with the N2O acting as a trigger to the axonal degeneration and demyelination detected on electrodiagnostic studies.

Pediatric Resident Confidence in Assessing Neurological Cases: A Nationwide Survey.

BACKGROUND: A relative shortage of pediatric neurologists in proportion to estimated neurological disorders often results in general pediatricians evaluating and treating children with complex neurological conditions. Dedicated rotations in pediatric neurology are not mandated during medical school or pediatric residency. We evaluated the perceptions of a large cohort of pediatric residents and program directors (PDs) regarding child neurology training. METHODS: Using an online tool, surveys were sent to pediatric residents and pediatric and pediatric neurology PDs. RESULTS: Response rates were 41% from pediatric residency programs, yielding 538 resident responses; 31% from pediatric PDs; and 62% from pediatric neurology PDs. Only 27% of the surveyed residents reported completing a neurology rotation during residency, 89% of whom expressed a subjective improvement in confidence with neurological assessments. Factors affecting comfort with eliciting a neurological history included exposure to a neurology rotation during residency, year of training, duration of neurology rotation in medical school, and inpatient exposure to neurological patients, whereas those associated with examination additionally included program size and postresidency plans. Overall, 80% of surveyed residents, 78% of pediatric PDs, and 96% of pediatric neurology PDs acknowledged the potential value of a mandatory pediatric neurology rotation during residency. CONCLUSION: We suggest that a mandatory pediatric neurology rotation will boost the confidence of current and future pediatric trainees in assessing common neurological conditions of childhood.

Yield of Neurodiagnostic Testing in Children Presenting to a Pediatric Emergency Department With Altered Mental Status.

BACKGROUND: Emergency department (ED) visits for altered mental status (AMS) in children are common. Neuroimaging is often performed to ascertain etiology, but its utility has not been well studied. Our objective is to describe the yield of neuroimaging studies in children who present to an ED with AMS. METHODS: We performed a retrospective chart review of children 0-18 years of age, presenting to our PED between 2018 and 2021 with AMS. We abstracted patient demographics, physical examination, neuroimaging and EEG results, and final diagnosis. Neuroimaging and EEG studies were classified as normal or abnormal. Abnormal studies were categorized as clinically important and contributory: abnormalities that were clinically important and contributed to the etiology, clinically important but noncontributory: abnormalities that were clinically significant but did not explain the etiology, and incidental: abnormalities that were not clinically significant. RESULTS: We analyzed 371 patients. The most common etiology of AMS was toxicologic (188, 51%) with neurologic causes (n = 50, 13.5%) accounting for a minority. Neuroimaging was performed in one-half (169, 45.5%) and abnormalities were noted in 44 (26%) studies. Abnormalities were clinically important and contributed to the etiologic diagnosis of AMS in 15/169 (8.9%), clinically important and noncontributory in 18/169 (10.7%), and incidental in 11/169 (6.5%). EEG was performed in 65 patients (17.5%), of which 17 (26%) were abnormal with only one being clinically important and contributory. CONCLUSIONS: Though neuroimaging was performed in approximately one half of the cohort, it was contributory in a minority. Similarly, diagnostic utility of EEG in children with AMS was low.

An Atypical Radiologic Presentation of Right Sixth Lateral Rectus Palsy: A Case Report.

Isolated ocular palsies are often associated with a benign process in the pediatric population but early diagnosis is critical to exclude any serious pathology. In this case, a six-year-old female with no significant past medical history presented with isolated right-eye medial deviation. The patient reported right-eye medial deviation for the past several weeks and associated double vision, but denied any pain with eye movements, other cranial nerve changes, or headaches. This case highlights the key radiologic finding which may ultimately allow for a leading diagnosis and inform further management in cases of isolated ocular nerve palsy.

Neurological Effects of COVID-19 in Children.

The COVID-19 pandemic has spread rapidly across the world in 2020, affecting both adults and, to a lesser extent, children. In this article, the authors describe the neurologic manifestations of COVID-19 in children, including the epidemiology, pathogenesis, clinical features, laboratory and imaging findings, and treatment options. The management of patients with concomitant neuroimmunologic disorders and drug interactions between medications used to treat COVID-19 and other neurologic disorders (especially immune-modifying drugs) is also discussed.

Children With Complex Febrile Seizures: Is Hospital Admission Necessary?

Children with complex febrile seizure (CFS) are often hospitalized for concerns for serious bacterial infection (SBI) or seizure recurrence. We describe the yield of diagnostic studies and seizure recurrence during hospitalization in CFS children. We performed a retrospective review of 372 visits in 350 developmentally normal children aged 6 to 60 months between 2011 and 2016 for CFS. Majority of patients were male (200; 57.1%), with a mean age of 19.8 ± 11.3 months. Active seizures were noted in 42 (11.3%), status epilepticus in 35 (9.4%) while 97 (26.1%) had a seizure in the pediatric emergency department. The distribution of SBI was as follows: bacteremia (3; 1.1%), urinary tract infection (7; 3.7%), pneumonia (15; 6.8%), and bacterial meningitis (0; 0%). Electroencephalography (EEG) abnormality was rare (7/158; 4.4%). Seizure recurrence during hospitalization was uncommon (19; 5.1%). Logistic regression analysis did not reveal any predictors for seizure recurrence. The seizure recurrence rate during hospitalization, EEG yield, and SBI incidence was low in children with CFS.

Factors Predisposing to Post Dural Puncture Headache in Children.

BACKGROUND: Knowledge of the factors that predispose to postdural puncture headache in children may help reduce the occurrence of this complication. MATERIALS AND METHODS: A retrospective cohort study of children who presented to the study institution between 2010 and 2018 was conducted. Children were divided into 2 groups: those who experienced postdural puncture headache and those who did not. The 2 groups were compared with respect to certain demographic, technical, and personnel-related factors. Only children who had opening pressure documented during the procedure were included in the core study group. RESULTS: In univariate analysis, children aged ≥10 years, female gender, children with higher body mass index, standard blinded lumbar puncture procedure, use of sedation, higher opening pressure, and presence of pseudotumor cerebri increased the probability of postdural puncture headache. In multivariable logistic regression analysis, presence of pseudotumor cerebri was the only factor that attained statistical significance when the opening pressure was measured and documented. CONCLUSIONS: The risk factors for postdural puncture headache in a pediatric cohort varied from risk factors that are classically implicated in adults.

Effectiveness of Standard Combination Therapy in Pediatric Migraine.

BACKGROUND: A combination of parenteral medications (often referred to as standard combination therapy) is frequently used in the treatment of acute migraine in the pediatric emergency department (PED). The primary aim of this study was to evaluate the two-hour, 24-hour, and seven-day impact of one such regimen on pain in children who present to the PED. Standard combination therapy for purposes of our study is defined as a bolus of intravenous saline, and a combination of intravenous ketorolac, prochlorperazine, and diphenhydramine. METHODS: This prospective observational study included 120 children between the ages seven and 18 years who presented to the PED with migraine, whose parents could read and understand the consent form in English, and who were treated with standard combination therapy. The primary outcome measure for this study was the change in severity of pain as noted by the child using the Faces Pain Scale-Revised. We analyzed normally distributed continuous variables by mean and standard deviation, whereas non-normally distributed continuous variables are reported by median and interquartile range. RESULTS: Nonparametric Friedman testing on the entire cohort (n = 120) noted that there was a statistically significant change in the Faces pain scale from before administration of standard combination therapy to the two-hour, 24-hour, and one-week time point with a reduction in pain score of 87.5%, 100%, and 50%, respectively, at the three time points. CONCLUSIONS: This study noted moderate relief of pain after administration of standard combination therapy, which persisted at one-week after administration.

Reversible Cerebral Vasoconstriction Syndrome and Sickle Cell Disease: A Case Report.

Reversible cerebral vasoconstriction syndrome (RCVS), is rare in the pediatric population and is characterized by severe headaches and other neurologic symptoms. We present a case of RCVS occurring concomitantly with posterior reversible encephalopathy syndrome in an 8-year-old African American child with sickle cell disease (HbSS). Imaging studies including computed tomography, magnetic resonance imaging and cerebral angiography of the brain showed acute hemorrhagic stroke and a beaded appearance of peripheral cerebral vessels. In this report, we focus on the typical features of RCVS and discuss the underlying risk factors that may increase the risk in patients with HbSS disease.

Quality of Life in Multiple Sclerosis.

Investigators from Karolinska Institute in Stockholm, Sweden report on their findings comparing quality of life (QoL) measures in both pediatric-onset multiple sclerosis (POMS) and adult-onset multiple sclerosis (AOM).

Diagnosing Pseudotumor Cerebri: An Age-based Approach.

Investigators from Hillel-Yaffe, Carmel, and Bnai Zion Medical Centers in Israel studied the comparative clinical presentations and predisposing factors for idiopathic intracranial hypertension (IIH) across age groups.

Rapid Sequence MRI Protocol in the Evaluation of Pediatric Brain Attacks.

BACKGROUND: The objective of our study was to evaluate the clinical utility of rapid sequence magnetic resonance imaging (MRI) utilizing diffusion-weighted imaging and fluid-attenuated inversion recovery sequences in children with acute ischemic strokes and nonstroke brain attacks. METHODS: We performed a retrospective chart review of patients aged one month to 25 years for whom a pediatric stroke clinical pathway was activated. Diffusion-weighted imaging and fluid-attenuated inversion recovery were obtained followed by a complete MRI. Imaging was interpreted by a pediatric radiologist and the study neurologist. We collected information regarding patient demographics, neuroimaging results, and final diagnosis. RESULTS: The Pediatric Stroke Clinical Pathway was activated for 59 patients of whom 52 were included for analysis. The majority of patients were female (n = 29, 55.8%) and African American (n = 32, 61.5%), with a median age of 12 years (interquartile range 9, 16). Six patients had an ischemic stroke. Seizures, migraines, and psychosomatic disorders (each with n = 7; 13.5%) were the most common nonstroke diagnoses. Diffusion-weighted imaging was more sensitive (100% [55.0% to 100%] versus 80 % [32% to 99%]) and specific (73.9% [68% to 74%] versus 37.2% [32% to 39%]) compared with fluid-attenuated inversion recovery in identification of an ischemic stroke. However, fluid-attenuated inversion recovery was useful in the identification of inflammatory and metabolic disorders. CONCLUSION: Rapid sequence MRI can be utilized as a screening imaging modality in children with suspected brain attacks in cases where there may be delays in obtaining full sequence brain imaging.

Management of Childhood Migraine by Headache Specialist vs Non-Headache Specialists.

OBJECTIVE: This study aims to compare the management practices of a headache specialist with non-headache specialists in the treatment of children with migraine. The use of appropriate rescue medications and prophylactic agents, application of neuroimaging, and short-term outcomes are compared in children treated by the two groups of physicians. METHODS: A retrospective cohort study was conducted by utilizing the electronic medical records of children 3-18 years of age with migraine, who were evaluated at a tertiary care children's hospital from 2016 to 2018. RESULTS: Of the 849 patients who met the study criteria, 469 children were classified as having chronic migraine or high-frequency episodic migraine and were followed-up on at least 1 occasion by the neurologists. Imaging was obtained in 66.5% of all children with migraine. The headache specialist used 5-HT agonists ("triptans") for migraine management in 56.7% (76/135) of cases compared to non-headache specialists who prescribed them in 28.7% (96/334) of cases (P < .001). Of the children with chronic migraine, the headache specialist evaluated 135 patients while the non-headache specialists treated 334 children. Non-headache specialists prescribed prophylaxis in the form of natural supplements more frequently (63.8% of cases) compared to the headache specialist (38.5% of children) (P < .001). Moreover, prophylaxis with prescription drugs was utilized more often by headache specialist (66.7%) than non-headache specialists (37.4%) (P < .001). CONCLUSIONS: Imaging appears to be commonly recommended by both headache specialists and non-headache specialists in children with migraine. The headache specialist was more likely to use triptans as rescue medications for pediatric migraine. Outcomes in the short-term were not statistically different whether children were being managed by the headache specialist or the non-headache specialists.

Utility of Neuroimaging in Children Presenting to a Pediatric Emergency Department With Ataxia.

OBJECTIVES: The aim of this study was to evaluate the utility of neuroimaging in children who present to the pediatric emergency department with acute-/subacute-onset ataxia. Neuroimaging is performed in many children with ataxia to rule out serious intracranial pathology. There is, however, limited evidence to support such practice. METHODS: This was a retrospective review of electronic medical records of children who presented to the emergency department with ataxia between 2007 and 2013. Patient demographics, historical features, physical examination findings, laboratory results, and neuroimaging results were collected. Neuroimaging studies that were classified as abnormal by a neuroradiologist were further reviewed and classified by the study neurologist as clinically significant or not. RESULTS: The records of 141 subjects were analyzed. The most common causes of ataxia were infectious/postinfectious (36.2%) and ingestion (15.6%). Neuroimaging was performed in 104 children (73.8%). Neuroimaging was abnormal in 63 children (60.6%). However, these abnormalities were clinically significant in only 14 children (13.5%). Focal neurological findings were noted in 12 of 14 children (85.7%) with clinically significant neuroimaging. CONCLUSIONS: Clinically significant neuroimaging was noted in a minority of children who presented with acute/subacute ataxia. The majority of patients with clinically significant neuroimaging had focal neurological findings on examination. Neuroimaging may not be required in all children presenting to the ED with acute ataxia, but further large-scale studies are needed to validate these findings and identify a subset of patients with ataxia in whom imaging can be deferred.