Pediatric cerebrovascular diseases: report of 204 cases
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Etiology
Moyamoya Disease
Moyamoya disease is a cerebrovascular disorder characterized by bilateral stenosis or occlusion of the terminal portions of the internal carotid arteries accompanied by typical net-like collateral vessels in the basal ganglia. Although the etiology of moyamoya disease remains unknown, hereditary and immunogenic as well as hemodynamic factors have been implicated in the underlying mechanism of moyamoya disease. We report two patients with confirmed moyamoya disease and a patient with probable moyamoya disease complicated with Graves' disease. We reviewed the literature and summarized 23 cases of moyamoya disease or probable moyamoya, coexisting with Graves' disease.
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Cerebrovascular disorder
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Moyamoya disease (MMD) is characterized by progressive occlusion of the internal carotid artery or its terminal branches, associated with formation of extensive collateral vessels (moyamoya vessels) at the base of the brain. Whether unilateral moyamoya disease, confirmed by typical angiographic evidence of moyamoya disease unilaterally and normal or equivocal findings contralaterally, is an early form of definite (bilateral) moyamoya disease remains controversial. Inherited or acquired disorders and conditions may present in conjunction with moyamoya disease. This condition is known as quasi-moyamoya disease (quasi-MMD). We attempted to determine the incidence and total patient number of moyamoya disease, unilateral MMD and quasi-MMD, who were treated during 2005 in Japan. Questionnaires were sent to 2,998 departments, which are listed in resident training programs of neurosurgery, neurology and pediatrics. Totally, 1,183 departments replied, and the response rate was 39.5%. The number of annual first-visit patients of MMD, unilateral MMD and quasi-MMD is 571, 118, and 53, respectively. Thus, the number of annual revisit patients of MMD, unilateral MMD and quasi-MMD is 2,064, 214, and 117 respectively. It is estimated that 6,670.9 MMD patient exists in Japan. The incidence rate of MMD, unilateral MMD and quasi-MMD is 1.13, 0.23 and 0.11/100,000, respectively, and the prevalence is 5.22, 0.66 and 0.34/100,000, respectively. This nationwide study revealed the present epidemic status of MMD, unilateral MMD and quasi-MMD.
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Moyamoya Disease
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Interventional radiology
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introduction: Seizure is the most common neurological illness in pediatric population, and its risk is highest in the rst year of life. A comprehensive study regarding etiology, clinical prole is required, hence the current study was undertaken. The objective of this work was to study the etiology, clinical prole of seizures in children aged less than 18 years.Objective: Materials and Methods: A descriptive study of children with seizure was conducted at Neurology outpatient department, and references from pediatrics department. All children with seizures were included. Biochemical and haematological investigations, imaging, and electroencephalogram were performed whenever necessary. Simple descriptive statistics were used to analyse the data in the form of frequencies with percentages as applicable. Out of 65 Pediatric patients who presented with seizures were Febrile seizure – Simple 4.6% (3)Results: ,Atypical 29.2% (19),Fever precipitated seizure 32.3% (21), Seizure disorder with developmental delay 20% (13),Unprovoked 6.1% (4),others (5) Seizure was the commonest neurological condition of children presenting to neurology OPD and referred from pediatrics. FeverConclusion: precipitated seizure being the commonest etiology. The prognosis and outcomes were good but there were prolonged days of hospitalization. Children with unprovoked seizures require brain-imaging studies for better understanding of seizure etiology.
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Pediatric Neurology
Tertiary care
Seizure Disorders
Outpatient clinic
partial seizures
Febrile seizure
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Seizure is the most common neurological illness in pediatric population and its risk is highest in the first year of life. Studies regarding etiology of afebrile seizures worldwide and in India are limited; hence, the current study was undertaken.The objective of this work was to study the etiology of afebrile seizures in infants aged 1 month to 1 year.A retrospective study of afebrile infantile seizure was conducted at pediatric neurology outpatient department between January 2015 and September 2017. All children with first episode of afebrile seizures were included. Biochemical and hematological investigations, imaging, and electroencephalogram were performed whenever necessary. Simple descriptive statistics were used to analyze the data in the form of frequencies with percentages and median as applicable.One hundred twenty-one children with a mean age of 8.1 months were analyzed. Of these children, 58% were males. Positive family history was found in 1.65% children, developmental delay in 70%, and dysmorphism in 8%. Presenting seizure type were generalized (74%), focal (21%), and unknown (6%) onset. Etiology was deducible in 92% cases. Structural etiology was the most common (66%), followed by metabolic (12%), infections (7%), and others (6%). In structural pathology, common causes were perinatal insult (38%) and cerebral malformations (18%). Imaging was done in 98 cases and yield was 83% (85 cases).Etiology was deducible in 92% of children. Seizures due to structural etiology, secondary to perinatal insult followed by metabolic and infections, are important causes. Imaging aids are important in etiological diagnosis. Etiological evaluation should be considered in children following first episode of afebrile seizures, especially in developing countries like India.
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Tertiary care
Pediatric Neurology
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Objective: To investigate the clinical efficacy of superficial temporal artery -middle cerebral artery combined with encephalo-duro-arterio-myo-synangiosis (STA-MCA+EDAMS) and encephalo-duro-arterio-myo-synangiosis (EDAMS) in the treatment of adult moyamoya disease. Methods: The clinical data of 47 adult patients with moyamoya disease who received vascular reconstruction in the Department of Neurosurgery of Taizhou Hospital of Zhejiang Province from January 2014 to January 2018 were retrospectively analyzed. Among them, 21 patients received EDAMS alone (EDAMS group, 14 patients with hemorrhagic moyamoya disease, 7 patients with ischemic moyamoya disease), 26 patients received STA-MCA combined with EDAMS (STA-MCA+EDAMS group, 17 patients with hemorrhagic moyamoya disease, 9 patients with ischemic moyamoya disease). Cerebral hemodynamics at 1 day before surgery and 3 and 6 months after surgery were compared. The clinical efficacy and postoperative complications of the two methods were compared at 3 and 6 months postoperatively in hemorrhagic and ischemic types. Results: For hemorrhagic moyamoya disease, the remission rate (94.1%) at 6 months after surgery in the STA-MCA + EDAMS group was higher than that in the EDAMS group (57.1%), and the difference was statistically significant (P<0.05). The CBF and CBV in the STA-MCA+EDAMS group were higher than those in the EDAMS group at 3 and 6 months after operation, and the MTT and TPP were lower than those in the EDAMS group, but there was no significant difference between the two groups (all P>0.05). For hemorrhagic moyamoya disease and ischemic moyamoya disease, the total incidence of postoperative complications of the two surgical methods was different, but the difference was not statistically significant (both P>0.05). Conclusion: Superficial temporal artery -middle cerebral artery combined with encephalo-duro- arterio-myo-synangiosis (STA-MCA+EDAMS) and encephalo-duro-arterio-myo-synangiosis (EDAMS) can significantly improve neurological function and cerebral hemodynamics in adult moyamoya disease patients with high safety.目的： 探讨颞浅动脉-大脑中动脉搭桥联合脑-硬膜-动脉-颞肌贴敷术（STA-MCA+EDAMS）以及脑-硬膜-动脉-颞肌贴敷术（EDAMS）治疗成人烟雾病的安全性和有效性。 方法： 回顾性分析2014年1月至2018年1月在浙江省台州医院神经外科接受血管重建术治疗的47例成人烟雾病患者的临床资料，其中接受单纯EDAMS者21例（EDAMS组），其中出血型烟雾病14例，缺血型烟雾病7例；接受STA-MCA联合EDAMS者26例（STA-MCA+EDAMS组），其中出血型烟雾病17例，缺血型烟雾病9例。比较2组术前1 d及术后3、6个月的脑血流动力学指标，按出血型和缺血型分别比较2种术式术后3、6个月的临床疗效及术后并发症情况。 结果： 出血型烟雾病中STA-MCA+EDAMS组术后6个月的缓解率为94.1%（16/17），EDAMS组为57.1%（8/14），差异有统计学意义（P<0.05）。STA-MCA+EDAMS组术后3、6个月的脑血流量、脑血容量高于EDAMS组，平均通过时间、达峰时间低于EDAMS组，但差异均无统计学意义（均P>0.05）。不管是出血型烟雾病还是缺血型烟雾病，两种术式术后总并发症发生率差异均无统计学意义（均P>0.05）。 结论： 颞浅动脉-大脑中动脉搭桥联合脑-硬膜-动脉-颞肌贴敷术以及脑-硬膜-动脉-颞肌贴敷术治疗成人烟雾病均可显著改善患者神经功能和脑血流动力学指标，安全性较高。.
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Epilepsy is one of the most frequent diseases of the central nervous system (CNS) in children. It is considered that roughly 50% of epilepsies begin during childhood. The Department of Childrens' Neurology of the Medical Academy of Białystok embraces with medical attention over 500 children with epilepsy from the north-eastern region of Poland. The aim of the study was the analysis of etiology, clinical symptoms and treatment of epilepsy in children, who are under the medical attention of our Department. 392 children aged from 7 months to 18 and 2/12 years were included in the study. In the analysed groups were 46.6% girls and 53.4% boys. 44.4% of the children live in cities and 55.6% in small towns or villages. The etiology of epilepsy was established in 48.7% of the children. The most common etiology of epilepsy were hypoxic-ischemic damages in perinatal period, developmental malformation of the brain, craniocerebral injuries and neuroinfections in the neonatal period. In 50.3% of the children the etiology of epilepsy was unknown. The most common types of seizures were primary and secondary generalised tonic-clonic seizures. 92.6% of the patients received the antiepileptic drugs during the study. Monotherapy was administered in 66.0%, politherapy in 26.5% of the children. The results relating to the etiology and clinical picture of epilepsy in our children are consisted with many dates from literature. It is a favourable observation, that in many children monotherapy is an effective treatment.
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Epilepsy syndromes
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An unverified disease called "Moyamoya Disease" or "Spontaneous occlusion of the circle of Willis" has been recently reported as a disease entity by some Japanese researchers. Since the first report of this disease by Shimizu and the author in 1955, many cases have been reported not only in Japan but in many countries outside Japan. It has been already clarified either clinically or pathologically, that, in the Moyamoya Disease, the most important finding is the basal arterial occlusive change of unknown etiology and the Moyamoya Phenomenon is only nonspecific neuroradiological change as the extraordinary dilated collaterals via the striate arteries, perforators etc. However, the real cause of the arterial obstruction is still obscure in the so-called "true Moyamoya Disease". Further studies will be necessary in order to establish a new clinical entity related to the Moyamoya Disease. However, under existing situations, the Moyamoya Disease must be strictly differentiated from the Moyamoya Phenomenon which can be frequently observed among cases with basal occlusion of known and unknown origin.
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Introduction: Neonatal seizures are significant because most of them are treatable if a specific cause is identified. Prompt diagnosis is important to plan correct treatment. The time of onset of seizures is closely related to the aetiology of seizure Objectives: The objective of the present study is to know the etiology of seizures, the time of onset, relation to the cause and the common types of seizures Methodology: 102 neonates presenting with seizures admitted in the NICU of RMH, Thanjavur Medical College, Thanjavur during January 2014 to august 2014 were taken for study, Detailed antenatal, natal and post natal history were taken and examination of baby done and HIE staged according to Modified Sarnat’s staging. Then relevant investigations were done and etiology of neonatal seizures were diagnosed and staged Results: In the present study, out of 102 neonates studied, 82 were full-term, among these 60 (58.8%) were AGA and 22 (21.6%) were SGA. 19 babies (18.6%) were preterm. Male: Female ratio in our study was 1.5:1. Conclusion: The most common etiology for neonatal seizures is HIE. Onset of seizures during first 3 days of life has significant correlation with HIE as etiology. Subtle seizures is the commonest type of seizures and is difficult to identify. Keywords: Neonatal seizures; Birth asphyxia; HIE; Subtle seizures; Hypoglycemia
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Perinatal asphyxia
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