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Journal of Child Neurology

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https://www.readbyqxmd.com/read/29661126/gnao1-mutation-induced-pediatric-dystonic-storm-rescue-with-pallidal-deep-brain-stimulation
#1
C Michael Honey, Armaan K Malhotra, Maja Tarailo-Graovac, Clara D M van Karnebeek, Gabriella Horvath, Adi Sulistyanto
Dystonic storm or status dystonicus is a life-threatening hyperkinetic movement disorder with biochemical alterations due to the excessive muscle contractions. The medical management can require pediatric intensive care unit admission and a combination of medications while the underlying trigger is managed. Severe cases may require general anesthesia and paralytic agents with intubation and may relapse when these drugs are weaned. Deep brain stimulation of the globus pallidum has been reported to terminate dystonic storm in several pediatric cases...
May 2018: Journal of Child Neurology
https://www.readbyqxmd.com/read/29552934/postconcussive-symptoms-after-single-and-repeated-concussions-in-10-to-20-year-olds-a-cross-sectional-study
#2
Temitayo Oyefunmike Oyegbile, Bronson Elizabeth Delasobera, Nassim Zecavati
The objective was to characterize cognitive deficits and postconcussive symptoms in a pediatric population with no concussion, a single concussion, and ≥2 concussions, using a cross-sectional design. Cognitive function and postconcussive symptoms were assessed in participants (age 10-20) with no concussion (n = 1118), single concussion (n = 368), and repeated (≥2) concussions (n = 252). Analyses were adjusted for age and gender. Individuals with ≥2 concussions exhibited more total postconcussive symptoms; more loss of consciousness, amnesia and confusion; more headaches; and poorer cognitive function compared to no concussion and single concussion...
May 2018: Journal of Child Neurology
https://www.readbyqxmd.com/read/29514534/child-neurology-a-fragile-future
#3
Marcio Leyser
No abstract text is available yet for this article.
May 2018: Journal of Child Neurology
https://www.readbyqxmd.com/read/29502465/isolated-optic-nerve-glioma-in-children-with-and-without-neurofibromatosis-retrospective-characterization-and-analysis-of-outcomes
#4
Dima Hamideh, Mary Ellen Hoehn, Julie H Harreld, Paul D Klimo, Amar Gajjar, Ibrahim Qaddoumi
Isolated optic nerve glioma is a rare tumor with no consensus for the best therapeutic approach. Therefore, tumor control and preservation of visual function remain a challenge. In this retrospective study, we describe our experience over 30 years in a single-institutional cohort of children with isolated optic nerve glioma, focusing on treatments and visual outcomes. Seventeen children were followed for a median period of 8 years (range, 2-22 years). Diagnosis was based on typical neuroradiologic findings, and 3 patients had histologic confirmation of their tumors...
May 2018: Journal of Child Neurology
https://www.readbyqxmd.com/read/29575996/soleus-h-reflex-and-its-modulation-with-vibration-in-idiopathic-toe-walkers-and-typically-developing-children
#5
Essi Marttinen Rossi, Päivi Nevalainen, Helena Mäenpää, Leena Lauronen
Idiopathic toe walking is a relatively common developmental condition often leading to secondary problems such as pain and muscle contractures in the lower extremities. The cause of idiopathic toe walking is unknown, which hinders the development of treatment strategies. To test whether children with idiopathic toe walking have functional alterations in their spinal motor circuits, we studied the properties of the soleus H-reflex and its modulation with vibration in 26 idiopathic toe walkers and 16 typically developing children...
April 2018: Journal of Child Neurology
https://www.readbyqxmd.com/read/29575995/large-vessel-arteriopathy-after-cranial-radiation-therapy-in-pediatric-brain-tumor-survivors
#6
Matthew Nordstrom, Erin Felton, Katherine Sear, Benita Tamrazi, Joseph Torkildson, Karen Gauvain, Daphne A Haas-Kogan, Josephine Chen, Benedict Del Buono, Anuradha Banerjee, David Samuel, David Saloner, Bing Tian, Erika Roddy, Christopher Hess, Heather Fullerton, Sabine Mueller
Among childhood cancer survivors, increased stroke risk after cranial radiation therapy may be caused by radiation-induced arteriopathy, but limited data exist to support this hypothesis. Herein, we assess the timing and presence of cerebral arteriopathy identified by magnetic resonance angiography (MRA) after cranial radiation therapy in childhood brain tumor survivors. In a cohort of 115 pediatric brain tumor survivors, we performed chart abstraction and prospective annual follow-up to assess the presence of large vessel cerebral arteriopathy by MRA...
April 2018: Journal of Child Neurology
https://www.readbyqxmd.com/read/29464974/a-comparison-of-spastic-diplegia-in-term-and-preterm-born-children
#7
Prashant Jauhari, Pratibha Singhi, Naveen Sankhyan, Prahbhjot Malhi, Sameer Vyas, Niranjan Khandelwal
This study compared the risk factors and clinical and radiologic profile of children with spastic diplegic cerebral palsy born at term (≥37 weeks) with those born preterm. Children (2-14 years) with cerebral palsy meeting the study criteria for spastic diplegia were enrolled. Antecedent risk factors, clinical profile, and magnetic resonance imaging (MRI) findings were recorded. Spasticity, functional ability, intellectual ability, and social quotient were assessed using standard scales. Ninety-three children met the study inclusion criteria (45 term, 48 preterm)...
April 2018: Journal of Child Neurology
https://www.readbyqxmd.com/read/29451082/the-short-term-placebo-response-in-children-with-attention-deficit-hyperactivity-disorder-adhd
#8
Ayala Cohen, Moran Plonsky-Toder, Emanuel Tirosh
To assess short-term placebo response in 6- to 13-year-old children with ADHD, children who were administered a double-blind placebo-methylphenidate trial, 1 week each, were included in the analysis. Conners' parents and Teacher Rating scales, the Aggregate Neurobehavioral Student Health and Educational Review inventory, and the Matching Familiar Figure Test were employed. A reduction of 30% or more in one or more of the teachers report subscales was observed in 18.8% of the participants. Attention test performance resulted in 58% of children exhibiting reduction in error rates and 36...
April 2018: Journal of Child Neurology
https://www.readbyqxmd.com/read/29451061/neck-tongue-syndrome-an-underrecognized-childhood-onset-cephalalgia
#9
Nicholas M Allen, Hormos S Dafsari, Elizabeth Wraige, Heinz Jungbluth
Neck-tongue syndrome is a rarely reported headache disorder characterized by occipital and/or upper neck pain triggered by sudden rotatory head movement and accompanied by abnormal sensation and/or posture of the ipsilateral tongue. Although onset is thought to be in childhood, most of the limited number of cases reported so far were adults. Here the authors describe 3 cases, 2 girls and 1 boy, with neck-tongue syndrome. In each child additional headache symptoms occurred, headache improved over time in all, spontaneously in 2 and coinciding with gabapentin treatment in the other...
April 2018: Journal of Child Neurology
https://www.readbyqxmd.com/read/29439610/outpatient-procedural-sedation-of-patients-with-autism-spectrum-disorders-for-magnetic-resonance-imaging-of-the-brain-using-propofol
#10
Pradip P Kamat, Marie K Karaga, Benjamin L Wisniewski, Courtney E McCracken, Harold K Simon, Reet Sidhu, Jocelyn R Grunwell
OBJECTIVE: To quantify the number of personnel, time to induce and complete sedation using propofol for outpatient magnetic resonance imaging (MRI) of the brain, and the frequency of serious adverse events (SAEs) in children with autism spectrum disorder (ASD) compared with children without ASD. RESULTS: Baseline characteristics were the same between both groups. Overall sedation success was 99%. Although most children were sedated with ≤3 providers, 10% with ASD needed ≥4 providers (P = ...
April 2018: Journal of Child Neurology
https://www.readbyqxmd.com/read/29421991/association-of-early-onset-spasticity-and-risk-for-cognitive-impairment-with-mutations-at-amino-acid-499-in-spast
#11
Meredith K Gillespie, Peter Humphreys, Hugh J McMillan, Kym M Boycott
Hereditary spastic paraplegia is a phenotypically and genetically heterogeneous group of neurodegenerative disorders characterized by lower extremity weakness and spasticity. Spastic paraplegia 4 (SPG4), caused by heterozygous mutations in the gene SPAST, typically causes a late-onset, uncomplicated form of hereditary spastic paraplegia in affected individuals. Additional clinical features in SPG4 have been reported on occasion, but no genotype-phenotype correlation has been established. Through targeted clinical testing, we identified 2 unrelated female patients with the same de novo p...
April 2018: Journal of Child Neurology
https://www.readbyqxmd.com/read/29421957/neurologic-involvement-in-primary-immunodeficiency-disorders
#12
Mirac Yildirim, Deniz Cagdas Ayvaz, Bahadir Konuskan, Rahsan Gocmen, Ilhan Tezcan, Meral Topcu, Haluk Topaloglu, Banu Anlar
The nervous system may be affected in primary immune deficiency (PID) syndromes through infectious, autoimmune, neoplastic mechanisms, or as a primary feature of the syndrome. However certain neurologic problems do not conform to these etiopathogenetic groups. We retrospectively examined PID patients seen in neurology consultation between 2014 and 2017 in order to determine the spectrum of nervous system involvement. Among patients with confirmed neurologic problems (n = 35), common manifestations were encephalopathy and global developmental/cognitive delay...
April 2018: Journal of Child Neurology
https://www.readbyqxmd.com/read/29433419/changes-in-tactile-function-during-intensive-bimanual-training-in-children-with-unilateral-spastic-cerebral-palsy
#13
Geoffroy Saussez, Marie Van Laethem, Yannick Bleyenheuft
Recently, an intensive bimanual intervention using sensory enriched materials resulted in improved tactile function in children with unilateral spastic cerebral palsy (USCP), raising the question of whether the observed tactile function improvement was due to the sensory enriched environment or the bimanual intervention per se. The present study investigates whether a bimanual intensive intervention improves tactile function. Nineteen children with USCP received 90 hours of bimanual training without enriched environment...
March 2018: Journal of Child Neurology
https://www.readbyqxmd.com/read/29433418/evaluation-of-the-utility-of-a-dietary-therapy-second-opinion-clinic
#14
Angelica R Lackey, Zahava Turner, Courtney A Haney, Anthony C Stanfield, Eric H Kossoff
Fine-tuning ketogenic diets to achieve better seizure control may influence families to seek second opinions. Since 2009, Johns Hopkins Hospital has provided second opinions for children followed at other ketogenic diet centers. We retrospectively reviewed 65 consecutive children seen in this clinic; parents were also sent a 2-page survey. The mean age was 6.6 years and dietary therapy had been used a median 9 months. Seizure reduction >50% was achieved in 65%, including 35% with >90% reduction. Parent questions included how to improve seizure control (65%), ideal diet duration (18%), and confirmation of the plan (11%)...
March 2018: Journal of Child Neurology
https://www.readbyqxmd.com/read/29433417/clinical-findings-and-neurologic-outcome-in-neonatal-encephalopathy-with-white-matter-injury-accompanied-by-rotavirus
#15
Jae Kyun Ku, Young Jin Heo, Keun Soo Lee, Bo Lyun Lee
Our objective was to elucidate the clinical characteristics and neurodevelopmental outcomes in neonatal encephalopathy with characteristic white matter injury as compared with other injury patterns on magnetic resonance diffusion-weighted imaging. We conducted a retrospective study comparing clinical and laboratory findings, and neurologic outcomes between 17 newborns with diffuse lesions in the periventricular white matter and white matter tract (group I) and 22 newborns with other patterns (group II). Stool samples indicated that 16 neonates (94...
March 2018: Journal of Child Neurology
https://www.readbyqxmd.com/read/29366381/an-atypical-rett-syndrome-phenotype-due-to-a-novel-missense-mutation-in-cacna1a
#16
Madison V Epperson, Michael E Haws, Shannon M Standridge, Donald L Gilbert
BACKGROUND: Some typical and atypical Rett syndrome patients lack known genetic mutations. Mutations in the P/Q type calcium channel CACNA1A have been implicated in epileptic encephalopathy, familial hemiplegic migraine, episodic ataxia 2, and spinocerebellar ataxia 6, but not Rett syndrome. Patient Description: The authors describe a female patient with developmental regression and a de novo, likely pathogenic mutation in CACNA1A who meets 3 of 4 main criteria (stereotypic hand movements, loss of purposeful hand movements, gait disturbance), and 6 of 11 supportive criteria (impaired sleep, abnormal tone, vasomotor disturbance, scoliosis, growth retardation, and screaming spells) for atypical Rett syndrome...
March 2018: Journal of Child Neurology
https://www.readbyqxmd.com/read/29366365/early-predictors-and-correlates-of-communication-function-in-children-with-cerebral-palsy
#17
Mary Jo Cooley Hidecker, Jaime Slaughter, Purni Abeysekara, Nhan T Ho, Nancy Dodge, Edward A Hurvitz, Marilyn Seif Workinger, Ray D Kent, Peter Rosenbaum, Madeleine Lenski, Suzette Báez Vanderbeek, Steven DeRoos, Nigel Paneth
Birth characteristics and developmental milestones were evaluated as early predictors/correlates of communication in children with cerebral palsy. The hypothesis was that maternal report of child's age for vocal play and first words would predict current functional communication. A case series of 215 children, 2 to 17 years (mean age = 8.2 years, SD = 3.9) with cerebral palsy was recruited from medical practices in 3 Michigan cities. Early developmental data were collected by maternal interview. The child's Communication Function Classification System (CFCS) level was obtained from parent...
March 2018: Journal of Child Neurology
https://www.readbyqxmd.com/read/29357731/medullary-astrogliosis-in-sudden-infant-death-syndrome-varies-with-sleeping-environment-evidence-for-different-mechanisms-of-death-in-alone-versus-co-sleepers
#18
Jade Spinelli, Roger W Byard, Corinna Van Den Heuvel, Lyndsey E Collins-Praino
Sudden infant death syndrome remains the leading cause of death in infants under 1 year, and underlying pathophysiological mechanisms are poorly understood. The current study investigated the hypothesis that co-sleepers die more rapidly from causes such as suffocation from overlaying by comparing levels of reactive astrogliosis in the medulla of infants who died sleeping alone to those who died co-sleeping. The amount of glial fibrillary acidic protein (GFAP) staining in alone sleepers was significantly higher than shared sleepers in 3 specific areas of the medulla, the inferior vestibular nucleus, the medial vestibular nucleus and the cochlear nucleus...
March 2018: Journal of Child Neurology
https://www.readbyqxmd.com/read/29333903/the-first-report-of-relative-incidence-of-inherited-white-matter-disorders-in-an-asian-country-based-on-an-iranian-bioregistry-system
#19
Mahmoud Reza Ashrafi, Zahra Rezaei, Morteza Heidari, Sedigheh Nikbakht, Reza Azizi Malamiri, Mahmoud Mohammadi, Gholam Reza Zamani, Reza Shervin Badv, Parastoo Rostami, Mojtaba Movahedinia, Mostafa Qorbani, Man Amanat, Ali Reza Tavasoli
Childhood leukodystrophies are a fast-growing field of pediatric neurology practice. Epidemiologic studies on the incidence of these disorders in children show different results. This is the first report of childhood leukodystrophies incidence from Iran. The enrolled patients were recruited from the neurometabolic bioregistry system that was organized in 2010 in the Children's Medical Center, Tehran, Iran. Herein is reported the incidence rate of leukodystrophies in those patients who were residents of 2 big popular provinces near Iran's capital city Tehran, with an average child population of 2 988 800 children...
March 2018: Journal of Child Neurology
https://www.readbyqxmd.com/read/29327642/effects-of-arm-cycling-exercise-in-spinal-muscular-atrophy-type-ii-patients-a-pilot-study
#20
Gamze Bora, Şulenur Subaşı-Yıldız, Ayşe Yeşbek-Kaymaz, Numan Bulut, İpek Alemdaroğlu, Öznur Tunca-Yılmaz, Haluk Topaloğlu, Aynur Ayşe Karaduman, Hayat Erdem-Yurter
Exercise studies in neuromuscular diseases like spinal muscular atrophy (SMA), a devastating disease caused by survival of motor neuron 1 ( SMN1) gene mutations, are drawing attention due to its beneficial effects. In this study, we presented a constructed arm cycling exercise protocol and evaluated the benefits on SMA patients. Five SMA type II patients performed 12 weeks of supervised arm cycling exercise. The physical functions were evaluated together with the SMN2 copy numbers, SMN protein levels, insulin-like growth factor 1(IGF1) and binding protein 3 (IGFBP3) levels...
March 2018: Journal of Child Neurology
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