Unique Characteristics of the Photoparoxysmal Response in Patients With Neuronal Ceroid Lipofuscinosis Type 2: Can EEG Be a Biomarker?
Conclusions:
Photoparoxysmal responses in patients with neuronal ceroid lipofuscinosis type 2 have features which are distinguishing from photoparoxysmal responses seen in other epilepsies. (Source: Journal of Child Neurology)
Source: Journal of Child Neurology - October 11, 2016 Category: Neurology Authors: Albert, D. V., Yin, H., De Los Reyes, E. C., Vidaurre, J. Tags: Original Articles Source Type: research
Mismatch Negativity Recording in Children With Duchenne Muscular Dystrophy: A Preliminary Study Integrating Neurophysiological and Neuropsychological Results
Many studies on Duchenne muscular dystrophy children support the hypothesis of a specific neuropsychological phenotype affecting mostly phonological skills. This prospective study aimed to shed light on the role of phonological abilities. Fourteen Duchenne muscular dystrophy children and 7 healthy children underwent mismatch negativity. Moreover, verbal intelligence, visuospatial attention, immediate verbal memory, working memory, grammar, vocabulary, visuomotor skills, reading, text comprehension, writing, and arithmetic were tested in Duchenne muscular dystrophy children. No significant difference between control and Duc...
Source: Journal of Child Neurology - October 11, 2016 Category: Neurology Authors: Filippini, M., Guerra, A., Negosanti, A., Santi, S., Sarajlija, J., Musti, M. A., Gobbi, G., Lassonde, M., Pini, A. Tags: Original Articles Source Type: research
The Influence of Neurodevelopmental Treatment on Transforming Growth Factor-{beta}1 Levels and Neurological Remodeling in Children With Cerebral Palsy
Neurodevelopmental treatment is an advanced therapeutic approach for the neural rehabilitation of children with cerebral palsy. Cerebral palsy represents a spectrum of neurological disorders primarily affecting gross motor function. The authors investigated the effects of neurodevelopmental treatment on serum levels of transforming growth factor-β1 (TGF-β1), a neuroprotective cytokine, and improvements to motor skills. Serum TGF-β1 levels and total score of the Gross Motor Function Measure–88 (GMFM-88) were significantly higher in children with cerebral palsy who underwent neurodevelopmental treatment ...
Source: Journal of Child Neurology - October 11, 2016 Category: Neurology Authors: Tao, W., Lu, Z., Wen, F. Tags: Original Articles Source Type: research
Metachromatic Leukodystrophy: An Assessment of Disease Burden
In conclusion, a so-far-unreported physical/economic burden in these families is presented. (Source: Journal of Child Neurology)
Source: Journal of Child Neurology - October 11, 2016 Category: Neurology Authors: Eichler, F. S., Cox, T. M., Crombez, E., Dali, C. i., Kohlschütter, A. Tags: Original Articles Source Type: research
Cytokine and Chemokine Expression in CSF May Differentiate Viral and Autoimmune NMDAR Encephalitis in Children
Childhood encephalitis is a potentially devastating condition with significant morbidity and mortality. Researchers currently lack biomarkers for differentiating infectious encephalitis from those with autoimmune causes which may delay adequate treatment. The authors studied the possibility of using cerebrospinal fluid cytokine and chemokine levels for this purpose. Children admitted to hospital care fulfilling criteria for encephalitis were prospectively included. Children who underwent lumbar puncture but were not classified as central nervous system infections served as controls. Cytokine and chemokine levels in the cer...
Source: Journal of Child Neurology - October 11, 2016 Category: Neurology Authors: Ygberg, S., Fowler, A., Wickström, R. Tags: Original Articles Source Type: research
JCN Calendar of Events
(Source: Journal of Child Neurology)
Source: Journal of Child Neurology - September 19, 2016 Category: Neurology Tags: & lt;I gt;JCN lt;/I gt; Calendar of Events Source Type: research
Experimental Therapeutic Trial Design for Pediatric Brain Tumors
Pediatric brain tumors are the leading cause of cancer-related death during childhood. Since the first pediatric brain tumor clinical trials, the field has seen improved outcomes in some, but not all tumor types. In the past few decades, a number of promising new therapeutic agents have emerged, yet only a few of these agents have been incorporated into clinical trials for pediatric brain tumors. In this review, the authors discuss the process of and challenges in pediatric clinical trial design; this will allow for highly efficient and effective clinical trials with appropriate endpoints to ensure rapid and safe investiga...
Source: Journal of Child Neurology - September 19, 2016 Category: Neurology Authors: Bornhorst, M., Hwang, E. I. Tags: Original Articles Source Type: research
Neurological Complications of Pediatric Cancer
Neurologists are often consulted for diagnosis and management of neurologic complications in patients undergoing therapy for cancer. Pediatric patients with cancer, often undergoing the same types of therapy as adults with cancer, may experience different adverse events. The set of neurologic complications in children differs from that in adults and the neurologist must take into account the continuing growth and development of the patient as well as significant differences in primary diagnosis across the population. Correctly recognizing complications and initiating prompt treatment may reduce pain and prevent further pro...
Source: Journal of Child Neurology - September 19, 2016 Category: Neurology Authors: Neil, E. C., Hanmantgad, S., Khakoo, Y. Tags: Original Articles Source Type: research
Neurocutaneous Syndromes and Brain Tumors
The etiology of most childhood cancer remains largely unknown, but is likely attributable to random or induced genetic aberrations in somatic tissue. However, a subset of children develops cancer in the setting of an underlying inheritable condition involving a germline genetic mutation or chromosomal aberration. The term "neurocutaneous syndrome" encompasses a group of multisystem, hereditary disorders that are associated with skin manifestations as well as central and/or peripheral nervous system lesions of variable severity. This review outlines the central nervous system tumors associated with underlying neurocutaneous...
Source: Journal of Child Neurology - September 19, 2016 Category: Neurology Authors: Ullrich, N. J. Tags: Original Articles Source Type: research
Rare Primary Central Nervous System Tumors Encountered in Pediatrics
As part of the special issue on Pediatric Neuro-Oncology, this article will focus on 4 of the rarer tumors in this spectrum, including atypical teratoid rhabdoid tumors, embryonal tumors with multilayered rosettes, choroid plexus tumors, and pleomorphic xanthoastrocytoma. Incidence and current understanding of the molecular pathogenesis of these tumors are discussed, and avenues of therapy both current and prospective are explored. (Source: Journal of Child Neurology)
Source: Journal of Child Neurology - September 19, 2016 Category: Neurology Authors: Kramer, K. Tags: Original Articles Source Type: research
Conventional and Advanced Imaging of Diffuse Intrinsic Pontine Glioma
This article will provide an overview of DIPG and describe the typical imaging findings with a focus on advanced imaging techniques. (Source: Journal of Child Neurology)
Source: Journal of Child Neurology - September 19, 2016 Category: Neurology Authors: Tisnado, J., Young, R., Peck, K. K., Haque, S. Tags: Original Articles Source Type: research
Diffuse Intrinsic Pontine Glioma: Time for Cautious Optimism
This article summarizes the epidemiology, clinical presentation, diagnosis, prognosis, molecular genetics, current treatment, and future therapeutic directions for diffuse intrinsic pontine glioma. (Source: Journal of Child Neurology)
Source: Journal of Child Neurology - September 19, 2016 Category: Neurology Authors: Hennika, T., Becher, O. J. Tags: Original Articles Source Type: research
Pediatric Suprasellar Tumors
The various childhood suprasellar tumors, while pathologically distinct, present similar clinical and surgical challenges as a result of their common anatomic location. These lesions are in close proximity to or may invade the optic nerve and chiasm, pituitary gland and infundibulum, hypothalamus, and third ventricle, leading to presenting features including visual field loss, impairment in visual acuity, endocrine dysfunction, and hydrocephalus. Though many suprasellar lesions are relatively benign in pathology, treatment may be complicated by high surgical morbidity resulting from damage to the hypothalamic-pituitary axi...
Source: Journal of Child Neurology - September 19, 2016 Category: Neurology Authors: McCrea, H. J., George, E., Settler, A., Schwartz, T. H., Greenfield, J. P. Tags: Original Articles Source Type: research
Pediatric Ependymoma
Over the past 150 years since Virchow’s initial characterization of ependymoma, incredible efforts have been made in the classification of these tumors and in the care of pediatric patients with this disease. While the advent of modern neurosurgery and the optimization of radiation have provided significant gains, a more complex but incomplete picture of pediatric ependymomas has begun to form through a combination of international collaborations and detailed genetic and histologic characterizations. This review includes and synthesizes the clinical understanding of pediatric ependymoma and their developing molecular...
Source: Journal of Child Neurology - September 19, 2016 Category: Neurology Authors: Vitanza, N. A., Partap, S. Tags: Original Articles Source Type: research
Medulloblastoma
accounts for nearly 10% of all childhood brain tumors. These tumors occur exclusively in the posterior fossa and have the potential for leptomeningeal spread. Treatment includes a combination of surgery, radiation therapy (in patients >3 years old). Patients >3 years old are stratified based on the volume of postoperative residual tumor and the presence or absence of metastases into "standard risk" and "high risk" categories with long-term survival rates of approximately 85% and 70%, respectively. Outcomes are inferior in infants and children younger than 3 years with exception of those patients with the medulloblas...
Source: Journal of Child Neurology - September 19, 2016 Category: Neurology Authors: Millard, N. E., De Braganca, K. C. Tags: Original Articles Source Type: research
