Eosinophilic Meningitis: Eleven-Year Experience at Texas Children's Hospital.

Eosinophilic meningitis can be caused by various etiologies and is reported mostly in tropical climates. The diagnosis is rare in the continental US, presenting challenges for management. Following a case of pediatric eosinophilic meningitis, we reviewed our 11-year experience with this diagnosis at a large US children's hospital.

Low iron storage and mild anemia in postural tachycardia syndrome in adolescents.

OBJECTIVE: We reported low iron storage in neurally mediated syncope (NMS). While reduced red cell mass indicative of anemia has been reported in POTS, iron indices and hemoglobin (Hb) data were not reported. We investigated whether POTS, like NMS, is associated with low iron storage and anemia. METHODS: Thirty two children evaluated in 2007 and 2008 for probable POTS by a standing or tilt test or both at Texas Children's Hospital were included in a retrospective study. We measured serum ferritin (SF) and Hb values. We defined iron deficiency as SF < 12 µg/L, low iron storage as SF ≤ 25 µg/L, anemia as low Hb values for age and sex, and POTS as ≥2 symptoms of orthostatic intolerance >3 months and increased HR of >30 BPM or HR of >120 BPM within 10 min of standing or 70° tilt. RESULTS: Twenty four children had POTS, ages 12-18 years, 17 (71 %) were females. Value range (median) of SF 2-289 µg/L (25), Hb 11.5-14.6 (12.5) in females and 12-15.9 g/L (13.6) in males. Patients with POTS, when compared with normal US pediatric population had higher prevalence of low iron storage (50 vs. 14 %), iron deficiency (25 % of teenage girls vs. 9 %, and 16 % of teenage boys vs. 1 %), and anemia (18 % of teenage girls vs. 1.5 %, and 43 % of teenage boys vs. 0.1 %). INTERPRETATION: Low iron storage and mild anemia are associated with POTS suggesting that low iron storage is a potentially pathophysiologic factor in both POTS and NMS.

Neurocognitive Profiles in Adolescents With Postural Tachycardia Syndrome and Perceived Brain Fog: A Preliminary Report.

BACKGROUND: Postural tachycardia syndrome (POTS) is associated with complaints of cognitive and emotional difficulties that may contribute to severe functional disability. For high-achieving adolescents, these symptoms can result in decreased participation in school and extracurricular activities. There are very limited data comparing subjective symptom reports to neurocognitive profiles in adolescents presenting with POTS, "brain fog," and cognitive difficulties. METHODS: A review of medical records and neuropsychological data was conducted for six adolescents diagnosed with POTS at a pediatric neurology clinic. All patients had frequent symptoms of orthostatic intolerance for more than three months. There was heart rate increase of ≥40 beats per minute (bpm) within 10 minutes of active standing or head-up tilt test in five patients and 36 bpm in one patient, who was diagnosed with probable POTS. All were referred for neuropsychological evaluations due to reported debilitating cognitive problems and an inability to function in a regular academic setting. Patients underwent a six-hour neuropsychological evaluation utilizing standardized measures of cognitive and emotional functioning. Clinically reported symptoms included fatigue, poor concentration, and memory impairment as well as "brain fog." RESULTS: Subjective complaints differed from patients' performance on standardized neuropsychological measures. Patients performed in the average to superior range across measures of general intelligence, verbal and working memory, processing speed, and sustained attention. CONCLUSIONS: Further research is needed to elucidate the basis for perceived "brain fog" and cognitive impairment in POTS, such as better understanding of patient and parental perceptions of initial medical symptoms and diagnosis as well as symptom amplification due to biopsychosocial processes.

Management of Extensive Central Nervous System Cladophialophora bantiana Infection in a 9-Year-Old Child.

BACKGROUND: Pediatric central nervous system (CNS) phaeohyphomycosis is a rare invasive fungal infection associated with high mortality. METHODS: We describe a child with progressive neurologic symptoms whose ultimate diagnosis was Cladophialophora bantiana -associated CNS phaeohyphomycosis. We discuss her clinical presentation, medical and surgical management and review the current literature. RESULTS: A 9-year-old female presented with acute onset of headaches, ophthalmoplegia and ataxia. Initial infectious work-up was negative, including serial fungal cerebrospinal fluid cultures. Over 2 months, she experienced progressive cognitive and motor declines, and imaging revealed worsening meningitis, ventriculitis and cerebritis. Ultimately, Cladophialophora was detected by plasma metagenomic next-generation sequencing (mNGS). Fourth ventricle fluid sampling confirmed the diagnosis of C. bantiana infection. Given the extent of her disease, complete surgical resection was not feasible. She required multiple surgical debridement procedures and prolonged antifungal therapy, including the instillation of intraventricular amphotericin B. With aggressive surgical and medical management, despite her continued neurologic deficits, she remains alive 3 years after her initial diagnosis. To our knowledge, this is one of a few published pediatric cases of CNS phaeohyphomycosis and the first with the causative pathogen identified by plasma mNGS. CONCLUSION: CNS phaeohyphomycosis is a serious, life-threatening infection. The preferred management includes a combination of surgical resection and antifungal therapy. In cases complicated by refractory ventriculitis, intraventricular antifungal therapy can be considered as adjuvant therapy. Direct sampling of the CNS for pathogen identification and susceptibility testing is the gold standard for diagnosis; however, the use of plasma mNGS may expedite the diagnosis.

Low iron storage in children and adolescents with neurally mediated syncope.

OBJECTIVE: To investigate whether neurally mediated syncope (NMS) is associated with low iron storage or serum ferritin (SF). STUDY DESIGN: 206 children evaluated between 2000 and 2004 for probable syncope at a tertiary care Pediatric Neurology Clinic were included in a retrospective study. Serum ferritin (SF), iron, total iron binding capacity, and hemoglobin were measured prospectively after initial history taking and physical examination, along with other diagnostic testing. We defined iron deficiency (ID) as SF <12 microg/L, and low iron storage as SF

Pediatric Disorders of Orthostatic Intolerance.

Orthostatic intolerance (OI), having difficulty tolerating an upright posture because of symptoms or signs that abate when returned to supine, is common in pediatrics. For example, ∼40% of people faint during their lives, half of whom faint during adolescence, and the peak age for first faint is 15 years. Because of this, we describe the most common forms of OI in pediatrics and distinguish between chronic and acute OI. These common forms of OI include initial orthostatic hypotension (which is a frequently seen benign condition in youngsters), true orthostatic hypotension (both neurogenic and nonneurogenic), vasovagal syncope, and postural tachycardia syndrome. We also describe the influences of chronic bed rest and rapid weight loss as aggravating factors and causes of OI. Presenting signs and symptoms are discussed as well as patient evaluation and testing modalities. Putative causes of OI, such as gravitational and exercise deconditioning, immune-mediated disease, mast cell activation, and central hypovolemia, are described as well as frequent comorbidities, such as joint hypermobility, anxiety, and gastrointestinal issues. The medical management of OI is considered, which includes both nonpharmacologic and pharmacologic approaches. Finally, we discuss the prognosis and long-term implications of OI and indicate future directions for research and patient management.

Migraine and recurrent epistaxis in children.

Recurrent epistaxis is a common pediatric problem with uncertain etiology in most cases. We observed frequent complaints, or history of epistaxis in children with migraine. The aim of this study was to determine whether there is an association between epistaxis and migraine in children. A detailed questionnaire was used to conduct a study of 45 consecutive patients, ages 6-11 years, with migraine, diagnosed according to the 1997 proposed pediatric revisions to the International Headache Society criteria; the patients were evaluated in our Pediatric Neurology Clinic. Control subjects consisted of 64 children without recurrent headaches, matched as a group for age and sex, and drawn as a convenient sample from two general pediatric practices and an elementary school. Sixteen (36%) of 45 patients with migraine had epistaxis as compared with 7 (11%) of 64 control subjects (odds ratio = 4.5; 95% confidence interval 1.6-12.1; P = 0.002). Epistaxis began an average of 3 years before migraine with similar characteristics to idiopathic epistaxis in habitual nose-bleeders, such as onset in early childhood, high incidence in sleep, and family history of epistaxis. This study demonstrates a significant association between migraine and recurrent epistaxis in children. Recurrent epistaxis increased the odds of migraine more than fourfold. Moreover, these data raise the question of whether epistaxis may represent a precursor to childhood migraine. The two disorders may share a common pathogenesis, and a prospective, longitudinal study is required to define further the relationship between them.

Neurodevelopmental outcome after extreme prematurity: a review of the literature.

BACKGROUND: Advances in obstetric and neonatal medical care and assisted reproductive technology have increased the rates of preterm birth, decreased preterm mortality rates, and lowered the limit of viability. However, morbidity in survivors, including neurodevelopmental disabilities, has increased, especially in extremely preterm infants born at ≤25 weeks' gestation. A better understanding of the prevalence and patterns of adverse neurodevelopmental outcomes in extremely preterm infants is important for patient care, counseling of families, and research. METHODS: The PubMed and Ovid Medline databases were searched for full text articles published between 1999 and 2013 in English that reported neurodevelopmental outcomes after extreme prematurity, and a review of identified relevant cohort studies was performed. RESULTS: Extreme prematurity of 22 to 25 weeks' gestation is associated with an overall high mortality of ≥50%. High rates (17% to 59%) of severe neurodevelopmental disabilities occur among survivors on short-term follow-up. The rates of surviving unimpaired or minimally impaired are 6% to 20% for live-born infants at ≤25 weeks' gestation and <5% for infants born at 22 and 23 weeks' gestation. Long-term adverse outcomes after extreme prematurity include intellectual disability (5% to 36%), cerebral palsy (9% to 18%), blindness (0.7% to 9%), and deafness (2% to 4%). Milder degrees of disability involving cognition, behavior, and learning are increasingly recognized among older preterm children, teens, and young adults. CONCLUSIONS: Infants who are born at ≤25 weeks' gestation, especially those born at 22 and 23 weeks' gestation, have a very low likelihood of surviving little or no impairment. Nearly half of surviving extremely premature infants have significant neurodevelopmental disabilities on short- and long-term follow-up. Instituting early intervention programs, providing family support, and establishing special educational school programs can pay high dividends and lead to brighter futures and, hence, help improve neurodevelopmental outcome of preterm infants.

Postural tachycardia syndrome in children and adolescents.

Postural tachycardia syndrome is a chronic condition with frequent symptoms of orthostatic intolerance or sympathetic activation and excessive tachycardia while standing, without significant hypotension. Orthostatic symptoms include dizziness, lightheadedness, blurring of vision, near faints, weakness in legs, poor concentration, nausea, and headaches. Somatic symptoms include fatigue, sleep disorder, widespread pain, abdominal pain, and menstrual irregularities. Psychological problems may overlap with physical complaints. This review discusses the normal physiology of orthostatic change, different pathophysiological mechanisms of postural tachycardia syndrome, including hypovolemia, venous pooling, autonomic neuropathy, and hyperadrenergic responses. In addition, an outline for management tailored to the patient's clinical syndrome is presented, along with concluding thoughts on future research needs.