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Smith D.M.,Childrens Hospital of Philadelphia | Smith D.M.,University of Pennsylvania | Vossough A.,Childrens Hospital of PhiladelphiaPA | Vossough A.,University of Pennsylvania | And 6 more authors.
Neurology | Year: 2015

Objective: To describe clinical characteristics, imaging findings, morbidity, and mortality in a single-center cohort of 12 pediatric cavernous sinus thrombosis cases and to review all cases available in recent English literature. Methods: Clinical data and radiographic studies on 12 cases from our institution were analyzed retrospectively. A literature search and review was conducted, with additional cases pooled with the new cohort for an aggregate analysis. Results: Twelve cases of cavernous sinus thrombosis in children from the Children's Hospital of Philadelphia between January 1, 2000, and December 31, 2013, were reviewed. All patients survived to discharge; 3 of 12 (25%) experienced neurologic morbidity. Contrast-enhanced MRI and contrast-enhanced head CT were 100% sensitive for detecting cavernous sinus thrombosis, while noncontrast time-of-flight magnetic resonance venography (TOF MRV) and noncontrast head CT were 0% sensitive. Literature review produced an additional 40 cases, and the aggregate mortality rate was 4 of 52 (8%) and morbidity rate was 10 of 40 (25%). Outcomes did not vary by treatment or with unilateral vs bilateral cavernous sinus involvement. There was a trend toward worse outcomes with fungal infections. Conclusion: Our case series demonstrates low morbidity and mortality with early, aggressive surgical, antimicrobial, and anticoagulation therapies. Although anticoagulation and surgery were not associated with significantly different outcomes, more study is needed. © 2015 American Academy of Neurology.

Lim S.-W.,Seoul National University | Lee H.-E.,Gachon University | Davis M.,Childrens Hospital of PhiladelphiaPA | Park K.,Seoul National University
Neurourology and Urodynamics | Year: 2016

Aims Integrating regular intermittent catheterization (IC) into daily life is essential for good medical outcomes in patients with neurogenic bladders. The goal is to identify long-term IC-related barriers, or difficulties in Korean patients with spinal dysraphism and their parents. Methods The data were prospectively collected using questionnaires from spinal dysraphism patients from two sources: an online community, and those visiting the outpatient clinic of Seoul National university hospital. The questions included were barriers in general and school life, respectively. Also, an open question was included regarding suggestions for school managers or the government in order to overcome identified IC related difficulties. Results A total of 20 patients and 40 parents answered the questionnaire. Common barriers found in general life were related to lack of places, time, or helpers to perform IC. Substantial numbers of adolescent patients also complained that IC could not be adequately performed due to problems related to privacy or lack of understanding at school. However, the number and nature of barriers varied according to the developmental stage and school environments. Almost all IC barriers seemed to be significant in adolescence. Some parents requested that the government provide space and broaden insurance coverage of catheters in order to facilitate IC. Conclusions Various perceived barriers were identified in those who need IC and differences were demonstrated over time. Both dedicated space and time are issues. In addition, patients may benefit from emotional support and enhanced communication with community agencies and government to resolve the problems related with privacy. © 2014 Wiley Periodicals, Inc.

Corben L.A.,Murdoch Childrens Research Institute | Lynch D.,Childrens Hospital of PhiladelphiaPA | Lynch D.,University of Pennsylvania | Pandolfo M.,Free University of Colombia | And 3 more authors.
Orphanet Journal of Rare Diseases | Year: 2014

Friedreich ataxia (FRDA), a multisystem autosomal recessive condition, is the most common inherited ataxia in Caucasians, affecting approximately 1 in 29,000 individuals. The hallmark clinical features of FRDA include progressive afferent and cerebellar ataxia, dysarthria, impaired vibration sense and proprioception, absent tendon reflexes in lower limbs, pyramidal weakness, scoliosis, foot deformity and cardiomyopathy. Despite significant progress in the search for disease modifying agents, the chronic progressive nature of FRDA continues to have a profound impact on the health and well-being of people with FRDA. At present there is no proven treatment that can slow the progression or eventual outcome of this life-shortening condition. Thirty-nine expert clinicians located in Europe, Australia, Canada and USA critically appraised the published evidence related to FRDA clinical care and provided this evidence in a concise manner. Where no published data specific to FRDA existed, recommendations were based on data related to similar conditions and/or expert consensus. There were 146 recommendations developed to ensure best practice in the delivery of health services to people with FRDA. Sixty-two percent of recommendations are based on expert opinion or good practice indicating the paucity of high-level quality clinical studies in this area. Whilst the development of these guidelines provides a critical first step in the provision of appropriate clinical care for people with FRDA, it also highlights the urgency of undertaking high-quality clinical studies that will ensure the delivery of optimum clinical management and intervention for people with FRDA. © 2014 Corben et al.; licensee BioMed Central.

Wingerchuk D.M.,Mayo Medical School | Banwell B.,Childrens Hospital of PhiladelphiaPA | Bennett J.L.,University of Colorado at Denver | Cabre P.,Center Hospitalier University Of Fort Of France | And 15 more authors.
Neurology | Year: 2015

Neuromyelitis optica (NMO) is an inflammatory CNS syndrome distinct from multiple sclerosis (MS) that is associated with serum aquaporin-4 immunoglobulin G antibodies (AQP4-IgG). Prior NMO diagnostic criteria required optic nerve and spinal cord involvement but more restricted or more extensive CNS involvement may occur. The International Panel for NMO Diagnosis (IPND) was convened to develop revised diagnostic criteria using systematic literature reviews and electronic surveys to facilitate consensus. The new nomenclature defines the unifying term NMO spectrum disorders (NMOSD), which is stratified further by serologic testing (NMOSD with or without AQP4-IgG). The core clinical characteristics required for patients with NMOSD with AQP4-IgG include clinical syndromes or MRI findings related to optic nerve, spinal cord, area postrema, other brainstem, diencephalic, or cerebral presentations. More stringent clinical criteria, with additional neuroimaging findings, are required for diagnosis of NMOSD without AQP4-IgG or when serologic testing is unavailable. The IPND also proposed validation strategies and achieved consensus on pediatric NMOSD diagnosis and the concepts of monophasic NMOSD and opticospinal MS. © 2015 American Academy of Neurology.

Arpan I.,University of Florida | Willcocks R.J.,University of Florida | Forbes S.C.,University of Florida | Finkel R.S.,Childrens Hospital of PhiladelphiaPA | And 13 more authors.
Neurology | Year: 2014

Objective: To evaluate the effects of corticosteroids on the lower extremity muscles in boys with Duchenne muscular dystrophy (DMD) using MRI and magnetic resonance spectroscopy (MRS). Methods: Transverse relaxation time (T2) and fat fraction were measured by MRI/MRS in lower extremity muscles of 15 boys with DMD (age 5.0-6.9 years) taking corticosteroids and 15 corticosteroid-naive boys. Subsequently, fat fraction was measured in a subset of these boys at 1 year. Finally, MRI/MRS data were collected from 16 corticosteroid-naive boys with DMD (age 5-8.9 years) at baseline, 3 months, and 6 months. Five boys were treated with corticosteroids after baseline and the remaining 11 served as corticosteroid-naive controls. Results: Cross-sectional comparisons demonstrated lower muscle T2 and less intramuscular (IM) fat deposition in boys with DMD on corticosteroids, suggesting reduced inflammation/damage and fat infiltration with treatment. Boys on corticosteroids demonstrated less increase in IM fat infiltration at 1 year. Finally, T2 by MRI/MRS detected effects of corticosteroids on leg muscles as early as 3 months after drug initiation. Conclusions: These results demonstrate the ability of MRI/MRS to detect therapeutic effects of corticosteroids in reducing inflammatory processes in skeletal muscles of boys with DMD. Our work highlights the potential of MRI/MRS as a biomarker in evaluating therapeutic interventions in DMD.

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