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.

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.

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.

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.

Pediatric Intracranial Hypertension.

Investigators from the Ohio State University, Oregon Health and Science University and Rosalind Franklin School of Medicine examined the presenting manifestations, demographics and treatment strategies in children enrolled in the Intracranial Hypertension Registry (IHR).

Common Neurocutaneous Syndromes.

Neurocutaneous syndromes are a diverse group of neurologic disorders with concurrent skin manifestations. Most neurocutaneous syndromes have a genetic basis and are believed to arise from a defect in the differentiation of the primitive ectoderm. In this regard, the skin can be a window into the central nervous system and can aid in the diagnosis of neurologic disease in children. The cutaneous signs may be subtle, which places great importance on the physical examination skills of clinicians providing primary care to children. Early recognition can help with proper diagnosis, formulating a treatment plan, anticipating potential complications, making appropriate referrals, and offering genetic counseling to families.

Seizure control following palliative resective surgery for intractable epilepsy-a pilot study.

BACKGROUND: Patients with intractable epilepsy who have bilateral epileptic foci may not qualify for curative epilepsy surgery. In some cases palliative resection may be undertaken with a goal to decrease seizure frequency and improve quality of life. Here we present data on the outcome of palliative epilepsy surgery in children. METHODS: We reviewed medical charts of children who underwent palliative resection for intractable epilepsy during the years 1999-2013 at Children's Hospital of Michigan. The palliative intent of resection was declared preoperatively. Outcome was assessed in terms of seizure reduction. RESULTS: There were 18 patients (11 males, median age of surgery was 3.5 years [range 0.5-16 years]). The median duration of follow-up after surgery was 12.5 months (range 6-60 months). Hemispherectomy was the most commonly performed palliative resection (nine patients), followed by lobectomy (six patients), multilobar resection (one patient), and tuberectomy (two patients). Reduction in seizure frequency was observed in 11 patients, with eight patients achieving seizure freedom on antiepileptic drugs and three with >50% reduction in seizure frequency. Transient improvement in seizure frequency occurred in two patients, whereas there was no benefit in five patients. CONCLUSIONS: Beneficial effects of epilepsy surgery may be realized in carefully selected situations wherein the most epileptogenic focus is resected to reduce seizure burden and improve quality of life.

Active comparator-controlled, rater-blinded study of corticotropin-based immunotherapies for opsoclonus-myoclonus syndrome.

To test the efficacy and safety of corticotropin-based immunotherapies in pediatric opsoclonus-myoclonus syndrome, 74 children received corticotropin alone or with intravenous immunoglobulin (groups 1 and 2, active controls); or both with rituximab (group 3) or cyclophosphamide (group 4); or with rituximab plus chemotherapy (group 5) or steroid sparers (group 6). There was 65% improvement in motor severity score across groups (P < .0001), but treatment combinations were more effective than corticotropin alone (P = .0009). Groups 3, 4, and 5 responded better than group 1; groups 3 and 5 responded better than group 2. The response frequency to corticotropin was higher than to prior corticosteroids (P < .0001). Fifty-five percent had adverse events (corticosteroid excess), more so with multiagents (P = .03); and 10% had serious adverse events. This study demonstrates greater efficacy of corticotropin-based multimodal therapy compared with conventional therapy, greater response to corticotropin than corticosteroid-based therapy, and overall tolerability.

The corticospinal tract in Sturge-Weber syndrome: a diffusion tensor tractography study.

OBJECTIVE: To utilize diffusion tensor tractography and evaluate the integrity of the corticospinal tract in children with unilateral Sturge-Weber syndrome (SWS). METHODS: Sixteen children (age: 1.5-12.3 years) with SWS involving one hemisphere and varying degrees of motor deficit, underwent magnetic resonance imaging (MRI) as part of a prospective clinical research study. Diffusion tensor imaging (DTI) was obtained and fiber tracking of the corticospinal tract was performed yielding average FA and ADC values along the pathway. These values were compared between the two hemispheres (affected vs. unaffected) and also correlated with the degree of motor deficits, after correction for age. RESULTS: Corticospinal tract FA values on the side of the affected hemisphere were lower (p=0.008) and ADC values were higher (p=0.011) compared to the normal side. Furthermore, FA and ADC values on the side of the angioma did not show the normal age-related variations, which the contralateral corticospinal pathway values did demonstrate. Although none of the patients had severe hemiparesis, those with moderate motor deficit had increased ADC values, as compared to those with mild (p=0.009) or no motor deficit (p=0.045). CONCLUSION: MRI with DTI shows abnormalities of the corticospinal tract in children with SWS even before severe motor impairment develops. Thus, DTI can be a clinically useful method to evaluate the integrity of the corticospinal tract in young children who are at risk for progressive motor dysfunction.