Clinical Characteristics of Subependymal Giant Cell Astrocytoma in Tuberous Sclerosis Complex.

Background: This study evaluated the characteristics of subependymal giant cell astrocytoma (SEGA) in patients with tuberous sclerosis complex (TSC) entered into the TuberOus SClerosis registry to increase disease Awareness (TOSCA). Methods: The study was conducted at 170 sites across 31 countries. Data from patients of any age with a documented clinical visit for TSC in the 12 months preceding enrollment or those newly diagnosed with TSC were entered. Results: SEGA were reported in 554 of 2,216 patients (25%). Median age at diagnosis of SEGA was 8 years (range, 18 years. SEGA were symptomatic in 42.1% of patients. Symptoms included increased seizure frequency (15.8%), behavioural disturbance (11.9%), and regression/loss of cognitive skills (9.9%), in addition to those typically associated with increased intracranial pressure. SEGA were significantly more frequent in patients with TSC2 compared to TSC1 variants (33.7 vs. 13.2 %, p < 0.0001). Main treatment modalities included surgery (59.6%) and mammalian target of rapamycin (mTOR) inhibitors (49%). Conclusions: Although SEGA diagnosis and growth typically occurs during childhood, SEGA can occur and grow in both infants and adults

Changing views of cerebral palsy over 35 years: the experience of a center

WOS: 000341411500002PubMed ID: 23692827In this study, it was aimed to evaluate the demographic and clinical characteristics of cerebral palsy (CP) cases over a 35-year period. Findings of 442 patients with CP followed from 1995 to 2006 (Group 2) were compared with 208 patients with CP followed between 1972 and 1994 (Group 1) in the same pediatric neurology division. Ratios of both prematurity (38% vs. 17.7%) and very low birth weight (VLBW) infants (13.8% vs. 1.5%) significantly increased in Group 2. There was also a four-fold increase in cesarean delivery in Group 2 (42.3% vs. 9.6%). A significant increase in the rate of early diagnosis during the first year was also found in this group (56.9% vs. 39.4%). The rate of spastic diparesis cases has significantly increased (33.7% vs. 7.7%), while the rate of spastic tetraparesis cases has significantly decreased (63.5% vs. 37.3%). It was seen that preventable risk factors continue today

Electrophysiologic assessment of spasticity in children using H-reflex

WOS: 000341415400009PubMed ID: 24382533We investigated a possible correlation between Hoffmann's reflex (H-reflex) and the Modified Ashworth Scale (MAS) in children with spasticity. H-reflex latencies, amplitudes (H amplitude), Hmax/Mmax amplitude, and MAS were simultaneously measured in 30 children who had bilateral spasticity on the lower extremities. Children with MAS scores of 1 and + 1 composed Group I (n=11), and children with MAS scores of 2 and 3 composed Group II (n=26) and Group III (n=23), respectively. The H-reflex latencies were significantly shorter and Hmax/Mmax ratios were significantly higher in patients with cerebral palsy than controls irrespective of the degree of the MAS. The H-reflex latencies in patients with MAS of 1 or + 1 were significantly longer than in patients with MAS of 2. Other than between these two groups for H-reflex latencies, no significant differences were revealed among the three different MAS groups for either H-reflex latencies or Hmax/Mmax ratios. There is a positive correlation between spasticity assessed by MAS and H-reflex. We concluded that the H-reflex is a reliable electrophysiologic test for assessment of spasticity in children

The efficacy of nonpharmacologic treatment in children with pharmacoresistant epilepsy

GİRİŞ ve AMAÇ: Epilepsi en yaygın kronik, nörolojik bozukluklardan biridir. Halen epilepsi tedavisinde eski ve yeni çok çeşitli antiepileptik ilaç kullanılmaktadır. Bu antiepileptik ilaçların kullanımına rağmen hastaların yaklaşık %20-30’u başarılı bir şekilde tedavi edilemez. Bu hasta grubuna ilaca dirençli epilepsi hastaları denir. Dirençli epilepsi hastalarında nöbetlerin sıklığı hastanın yaşam kalitesini ve bilişsel fonksiyonlarını olumsuz etkiler. Günümüzde epilepsi cerrahisi uygulamaları ile başarılı sonuçlar elde edilmektedir. Bu çalışmanın amacı; dirençli epilepsili çocuklarda epilepsi cerrahisinin (rezektif/vagal sinir stimülasyonu) etkinliğini değerlendirmekti. YÖNTEM ve GEREÇLER: Gazi Üniversitesi Tıp Fakültesi Çocuk Nöroloji Bilim Dalı Uzun Süreli Video EEG Monitorizasyon Ünitesinde epilepsi tanısı ile izlenen ve epilepsi cerrahi konseyi kararına göre epilepsi cerrahisi uygulanan 62 çocuk hastanın özellikleri incelendi. BULGULAR: Hastalardan 48’ine vagal sinir stimülasyonu (VSS), 14’üne rezektif cerrahi uygulandı, 31’i kız, 31’i erkek ve nöbet başlangıç yaşı 35,2 + 37,0 ay, cerrahi uygulanma yaşı 11,9 + 3,8 yaş, cerrahi öncesi izlem süresi 8,7 + 4,1 yıl idi. Rezektif cerrahi uygulanan hastalardan 12’si halen nöbetsiz iken, vagal sinir stimülasyonu yapılan hastalardan 3’ü nöbetsiz, 1 hastada %90’dan fazla, 7 hastada %75’den fazla, 19 hastada %50’den fazla nöbet kontrolü sağlanmıştır. TARTIŞMA ve SONUÇ: Sonuç olarak; gerek rezektif cerrahi gerekse VSS ilaca dirençli epilepsisi olan çocuk hastalarda uygulanan etkili tedavi yöntemleridir. VSS görece güvenli ve hafif yan etkileri olan bir yöntemdir. Erken dönemde, uygun hastalara uygulanan rezektif epilepsi cerrahisinin mortaliteyi azalttığı görülmüştür. Epilepsi hastalarında uygun cerrahi yöntem ve uygun hasta seçimi ile nöbet kontrolü büyük ölçüde sağlanabilmektedir. Uzun dönem izlenen seriler bize bu tedavi yöntemlerinin etkinliğini ve avantajlarını daha iyi gösterecektir. Daha etkili yöntemler bulunana kadar VSS ve rezektif cerrahi, ilaca dirençli epilepsi hastalarında cazip tedavi yöntemleri olmaya devam edecektir.INTRODUCTION: Epilepsy is one of the most common chronic neurological disorders. Currently, a variety of older and newer AEDs are used in the treatment of epilepsy. Despite the use of these AEDs, about 20-30% of patients are not succesfully treated. This group of patients is called pharmacoresistant epilepsy patients. The frequency of seizures in patients with refractory epilepsy, adversely affects the quality of life and cognitive functions. Today, successful results are obtained from the epilepsy surgery. The purpose of this study was to evaluate the efficacy of epilepsy surgery (resective/VNS) in children with pharmacoresistant epilepsy. METHODS: This study was conducted among 62 children that were followed in Gazi University department of neurology long-term video EEG monitorization unit, with the diagnosis of epilepsy. They underwent the epilepsy surgery with the decision of the epilepsy surgery council. RESULTS: The study included 48 patients that underwent VNS treatment and 14 patients that underwent resective surgery. 31 patient were girls, 31 patients were boys, age at onset of seizures (range) was 35,2 + 37,0 months, age at epilepsy surgery was 11,9 + 3,8 years, time before epilepsy surgery was 8,7 + 4,1 years. 12 patients that underwent resective surgery were seizure free, 3 patients with VNS implantation were seizure free after surgery. Seizure reduction > %90 was achieved in 1 patient, > % 75 in 7 patients and > % 50 in 19 patients. DISCUSSION and CONCLUSION: In conclusion resective surgery and VNS are effective treatment methods in children with pharmacoresistant epilepsy. VNS is relatively safe. In early period, resective epilepsy surgery for appropriate patients reduces the mortality. With appropriate patient and appropriate surgical method selection in patients with epilepsy, seizure control can be achieved largely. Until more effective therapies are developed, VNS and resective surgery will remain attractive alternatives for pharmacoresistant epilepsy

Ratios of Nine Risk Factors in Children With Recurrent Febrile Seizures

WOS: 000281131400006PubMed ID: 20691939Febrile seizures are the most common convulsive disorder of childhood, with a recurrence probability of 33%. The aim of the study was to determine the risk factors for recurrence of febrile seizures in children. In this descriptive, cross-sectional study, nine risk factors of recurrence of febrile seizures were investigated in 259 children with febrile seizures: (1) sex; (2) domicile; (3) income level; (4) family history of febrile seizures; (5) family history of epilepsy; (6) level of fever; (7) duration of fever; (8) type of seizure, simple vs complex; and (9) age at seizure onset. The risk factors were compared for 119 children with isolated febrile seizures (45.9% of the total) and 140 children with two or more febrile seizure recurrences (54.1%). Among the patients with and without recurrent febrile seizures, 32% and 18% were domiciled in nonurban areas, respectively (P = 0.012). There was a family history of febrile seizures in 57% and 44% of cases with and without recurrent febrile seizures, respectively (P = 0.031). According to the logistic regression analysis, a family history of febrile seizures was a risk factor that affected recurrence (P = 0.018; odds ratio OR = 1.933; 95% confidence interval CI = 1.121-3.333). We also found that domicile (P = 0.001) and income (P = 0.013) were risk factors for recurrence. A family history of epilepsy was not a significant risk factor (P = 0.129; OR = 2.110; 95% CI = 0.804-5.539). (C) 2010 by Elsevier Inc. All rights reserved