The underlying pathological substrates of localization-related epilepsy are varied. In children, the foremost among these are the malformative disorders of cortical development of which focal cortical dysplasia (FCD) is the most prominent. Other conditions include tuberous sclerosis, Sturge-Weber syndrome, vascular malformations, ischemic lesions and epilepsy-associated tumors. As in adults, medial temporal sclerosis is also a common histopathological finding. Resective surgery for extratemporal lesions is now the treatment of choice as more accurate identification of lesion with modern imaging and electrophysiological techniques is possible and a good surgical outcome is seen in most cases. This review describes the common causal histopathological substrates of epilepsy in children.

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Spinal injuries in the pediatric population are relatively rare. Hence there is not enough knowledge, experience and exposure amongst pediatric neurosurgeons about spinal injuries in children. They have to rely on general spinal or pediatric orthopedic colleagues for a comprehensive management of spine and spinal cord trauma. In addition, the advances in spinal instrumentation techniques and vast array of implantable devices for spinal stabilization add to the complexity of the problem. It is imperative that a pediatric neurosurgeon should be aware of the mechanics of spinal injury and recent advances in the management strategy of pediatric spinal injuries.

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Hyponatremia in acute CNS diseases is often attributed to the Syndrome of Inappropriate Antidiuretic Hormone Secretion (SIADH). Other causes may be in operation and may be overlooked. Aims: The objective of the study was to determine the etiology, evaluate treatment modalites and assess the outcome in children with an underlying acute neurologic disease who were hyponatremic. All these children were admitted to the Intensive Care Unit (ICU). Methods and Materials: This is descriptive hospital based retrospective chart review. Clinical indices of hydration, serum and urine sodium and osmolality were used in children to determine the cause of hyponatremia. In such of those who were hyponatremic, the cause of hyponatremia, treatment and outcome were assessed. Management of hyponatremia depended on etiology and severity of symptoms. Symptomatic patients had serum sodium raised by 3-5 mEq/l in order to control symptoms, following which a more gradual correction was carried out. Children with SIADH were fluid restricted while those with hyponatremic dehydration and Cerebral Salt Wasting (CSW) received supplemental saline and fluids. Results: Out of 1371 Pediatric Intensive Care Unit (PICU) admissions over a 30-month period, 385 (28%) had primary CNS disorders and of these, 58 were hyponatremic. The causes were SIADH in 19 (33%), hyponatremic dehydration in 16 (28%), drug-induced hyponatremia in 13 (22%) and CSW in 10 (17%) patients. About 10 of the 58 hyponatremic patients expired. All deaths were due to the severity of the underlying neurological condition. About 3 patients were hyponatremic at the time of death. Conclusion: The etiology of hyponatremia in acute CNS disease is multifactorial, and is not always due to SIADH. Careful evaluation and targeted therapy is required for the optimal management of these children

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Objective: To determine the relative contributions of clinical, radiological and histopatholgical predictors of survival in children with medulloblastoma (MB) and to compare it with their adult counterparts. Materials and Methods: Retrospective case record analyses of 79 children (<16 y) operated after Jan. 1990, who have completed at least 5 y of follow-up. The following variables were assessed by bivariate analysis: age, CT scan location of the lesion, brainstem invasion, extent of excision, histological subtype. Statistical analysis was performed using Chi-square test, Fischers test and Student's t test. Results: Near-total to total excision could be achieved in 59 (74.6%) cases. Twenty-three patients (29.11%) required CSF diversion procedures. Histopathology revealed features of classical medulloblastoma in 63.2%, thermoplastic variant in 11% and glial differentiation in 25.3% of cases. Postoperative mutism was seen in 14 (17.72%) patients. All patients received adjuvant therapy. On follow-up, 34 patients were found to have posterior fossa recurrence and four patients were re-operated. An additional 17% of patients were found to have either spinal or supratentorial metastasis on follow-up. The overall 5-year recurrence-free survival rate was 19 (24.05%). Mortality was recorded in 23 patients and nearly 29 patients who were severely disabled on follow-up were referred to terminal care centres. Conclusion: In spite of recent advances in management, children with medulloblastoma still carry a poor prognosis. We observed poor outcome in children below 7 y of age. Vermian location had a better outcome in adults but not in children. Desmoplastic variant was observed to be a significant prognostic factor in paediatric, group while brain stem invasion carried poor prognosis for both.

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Temporal lobe epilepsy is a fairly homogenous syndrome in adults, with hippocampal sclerosis being the commonest etiology. In children, temporal lobe epilepsy is more commonly due to cortical dysplasia or tumors. The semiology and electrophysiology of temporal lobe seizures in children are distinct from adults and have age-dependent variations. The first-line treatment option in children includes antiepileptic drugs. Ketogenic diet and surgery are therapeutic options in refractory pediatric temporal lobe epilepsy.

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The epilepsies are socially handicapping disorders and even a single seizure occurring in certain circumstances may have disastrous effect. The impact of epilepsy on every aspect of both the lives of the child and family is significant. Issues such as mental retardation, subtle neuropsychologic disturbances, cognitive problems, behavioral problems and learning difficulties are major factors affecting the children with epilepsy. Drug treatment is the major form of therapy for a vast majority of children with epilepsy. The choice of antiepileptic drugs (AEDs) in a child depends on the type of seizures, syndrome diagnosis, age of the patient and its consequent adverse effects. The metabolism of AED also differs according to the pediatric age group, requiring different dose regimens. Here, a brief review of AED used in the pediatric age group is presented.

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Background: The authors report a unique concomitant occurrence of mental retardation, hydrocephalus due to aqueductal stenosis, a freely floating atlas (having anterior, posterior and transverse atlantoaxial dislocation [AAD]) consequent to an orthotopic os odontoideum and stenosis of atlantal ring. There were no features of coexisting chondroskeletal dysplasias or other systemic diseases. To the best of the authors' knowledge, this is the first reported case of its type in the literature. Case Report: This 17-year-old girl with delayed milestones had enlarging head with vomiting at 1 year of age. Her CT scan revealed hydrocephalus with aqueductal stenosis, and she underwent a ventriculoperitoneal shunt. She subsequently had progressive quadriparesis. A minor fall led to transient unconsciousness and aggravation of her symptoms. Radiology of the craniovertebral junction revealed os odontoideum with anterior, posterior and transverse C1-2 subluxation and atlantal ring stenosis with marked cervical compression. A C1 laminectomy with occipitocervical contoured rod fusion with onlay autologous bone graft under guidance of intraoperative image intensifier was performed. Conclusion: The concomitant presence of hydrocephalus due to aqueductal stenosis, mental retardation and congenital stenosis of the atlantal ring points towards a congenital origin for the os odontoideum. The free floating atlantal ring on the axis led to anteroposterior and transverse AAD, necessitating intubation and occipitocervical stabilization in absolutely neutral position of the neck since both flexion as well as extension movements would have been deleterious. Congenital stenosis of atlas is an extremely rare entity; it contributed to cervical canal compromise even in neutral position of the cervical spine when the AAD had been adequately reduced, requiring an additional C1 laminectomy. The simultaneous presence of all these anomalies merited unique management considerations.

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Primary thalamic tumors are rare and bilateral thalamic tumors are even rarer. The incidence, clinical manifestations, natural history and prognosis of primary bilateral thalamic gliomas (PBTT) remain relatively obscure. In this article, four cases of bilateral thalamic gliomas are discussed and the available literature is reviewed. We conclude that primary bilateral thalamic tumors are distinct lesions, as proven by their specific neuroradiological and metabolic properties, unresponsiveness to radiotherapy and chemotherapy as well as a rapidly fatal clinical evolution. Early diagnosis and prompt therapy may delay the devastating effects of this tumor.

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The authors describe their experience with endoscopic ventriculo-cysto-cisternostomy and placement of cisterno-cysto-venticulo peritoneal shunt for suprasellar arachnoid cyst for a child with bobble head doll movements. The abnormal head movements improved and follow up imaging showed reduction in the cyst and ventricular size. The benefits of minimally invasive endoscopic ventriculocystocisternostomy with endoscopic guided placement of cisterno-cysto-ventriculo peritoneal shunt is been discussed.

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The objective of the multimodality presurgical evaluation in patients with refractory epilepsy is to establish sufficient concordance among the various investigations. There should be maximum overlap in the epileptogenic zone, the irritative zone, the ictal onset zone, the functional deficit zone and the symptomatogenic zone. The ictal and interictal electroencephalogram measures the localization of epileptiform discharges, which should be concordant with structural abnormalities noted on MRI brain and functional abnormalities in the form of a zone of hypometabolism on fluorodeoxyglucose positron emission tomography, interictal single photon emission computerized tomography (SPECT) or hyperperfusion of the epileptogenic zone on ictal SPECT for a good surgical outcome. There should be no conflicting data from any of these studies, neuropsychological evaluation or seizure semiology.

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Split cord malformations (SCM) are increasingly being recognized as one of the causes of tethered cord syndrome. Renaming them as SCMs has reduced the conflict in terminology, though the pathogenesis of these complex anomalies still remains controversial. The diagnosis has become easy and certain with the advent of magnetic resonance imaging (MRI). It is necessary to screen the entire spine in order to recognize all the associated anomalies and to plan the management strategies. Aim: To study the role of prophylactic surgery in patients with SCM. Settings and Design: Retrospective clinical analysis. Methods and Material: In a series of 217 children treated with spinal dysraphism from 1994 to 2004 at Manipal Institute for Neurological disorders (MIND), 38 had SCMs and the clinical presentation and surgical outcome was retrospectively analyzed. Statistical analysis used Results: 34 children had isolated SCM, four children had other complex congenital anomalies. 23 children [type 1-18 (47%) and type 2-20 (53%)] were operated prophylactically. They maintained neurological stability after surgery. Among the children with pre-existing neurological deficits, stability was achieved in all and significant improvement in only 8%. Outcome was better in isolated SCM in compression to complex ones. Conclusion: We recommend surgery before the onset of neurological deficits. Surgical results are excellent with good microsurgical technique

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This case report is presented with the aim of discussing differential diagnosis of CNS demyelinating diseases in children below 15 years of age. The various diagnoses range from acute disseminated demyelinating disease to multiple sclerosis. In between the two extremes, we have multiphasic demyelinating encephalomyelitis. We had discussed the role of oligoclonal band in cerebrospinal fluid, auto antibodies and magnetic resonance imaging to differentiate these diagnoses.

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Cerebellar glioblastoma multiforme (GBM) is a highly malignant brain tumour, which is exceedingly rare and such tumour presenting as cerebellopontine angle (CPA) mass is even rarer. We here discuss the case of a 15-year-old girl who had cerebellar GBM presenting as CPA mass that resembled meningioma on CT scan and was managed successfully with minimal problems.

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Aims and Objectives: To review the clinical features and current understanding of the biology and management of pediatric ependymoma, critically analysing the different treatment modalities. Materials and Methods: The MEDLINE database, bibliographies of selected articles, and current English-language texts on the subject were reviewed. A Pubmed search was made with keywords pediatric, intracranial, ependymoma, surgery, chemotherapy, and radiotherapy. Most recent articles and also significant older articles having all above said words were selected and their results were compared in detail. Results: Almost all articles stress the complete or near total resection of the tumor at first surgery followed by radiotherapy in patients older than 3 years of age and chemotherapy in younger children. Conformal radiation therapy (CRT) is a technique which has promising results. Conclusion: Local tumor control is single most important prognostic factor. This is best achieved through gross total tumor resection wherever possible. Radiotherapy should be offered to all patients (>3-years age) with focused dose (CRT) to tumor bed. Chemotherapy with the current agents does not appear to hold much promise. However, it may be useful in the context of providing the surgeon with an opportunity to do further surgery on a tumor that is less vascularized.

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'White cerebellum sign' is a classic yet uncommon radiological finding in severe pediatric head injury. We present a case of a 7-year-old child who had this finding on computed tomography. We also discuss the possible etiogenesis and review the relevant literature.

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Patients with multiple congenital abnormalities and mental retardation are the most frequent patient group who are referred to a genetic clinic. Specific diagnosis for these patients will provide a better understanding of the possible reasons of pathogenesis, thereby providing more true information to families on recurrence risk, prognosis, possible treatment options and prenatal diagnosis. With the aim of finding out the etiology of the genetic diseases, 4659 patients who were classified into mental retardation or multiple congenital anomaly and mental retardation (MR or MCA/MR) group who were referred to Istanbul University, Departments of Medical Genetics of Cerrahpasa Medical Faculty and Istanbul Medical Faculty in between 1985 and 2005 were analyzed retrospectively and a two step study was generated: first step involved the etiologic classification of MR or MCA/MR group and the second evaluation of the factors that help in finding out the etiologies such as age at first observation, number and periods of observations and completion of laboratory tests. In the first part of the study, etiologic classification of the patients with MR or MCA/MR who were referred to genetic clinics of the two medical faculty of Istanbul University were mentioned. 2847 patients have had an etiological diagnosis (61.10%): from these patients, 1541 out of them had a chromosomal abnormality (33.07%), 555 were known single gene mutations (11.91%), 20 were recognized syndromes (00.42%), 567 were sequences (12.16%), 6 were associations (00.12%), 29 had spectrums (00.62%), 98 had structural abnormality of CNS (2.10%), and finally, 31 suffered from prematurity and its complications, toxic drugs, infections and hypoxic ischemic encephalopathy. This study will be the one of the biggest studies in Turkey and also around the world, evaluating the most frequent patient group referred to genetic clinics: MCA or MR.

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Intracranial aneurysms in childhood account for 1-2% of intracranial aneurysms.[1],[2] These aneurysms have unique characteristics that make them different from those in adults. These differences are evident in their epidemiology, location, clinical spectrum, association with trauma and infection, complications and outcome.

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This review focuses on the present status of inter-ictal surface EEG, simultaneous ictal video-EEG and intracranial EEG in the evaluation of children for epilepsy surgery. This is considered in the context of high-end structural and functional neuroimaging available today. Initially the concept of the epileptogenic zone and its different components are discussed so that it is easier to understand how the EEG helps in localization. Methods of maximizing the yield of the EEG are emphasized. Abnormalities in the inter-ictal and ictal EEG are discussed in some detail. Special emphasis is given to the seizure semiology and how this helps in identifying which lobe is primarily involved. Limitations of surface EEG are then detailed, with special emphasis on young infants. Finally the discussion turns to intracranial EEG. This is used intra-operatively in one stage epilepsy surgery and more definitively extra-operatively in two stage surgery not only to define the epileptogenic zone but also to identify critical cortex.

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Epilepsy and cognition have a multi-tiered reciprocal relationship. Alteration in cognitive abilities and performance may occur in tandem with persistent seizures in a patient with epilepsy. Age at onset, type of seizures, frequency of seizures, types of underlying epilepsy syndrome, and the underlying pathological brain substrate driving epilepsy may all have variable and independent effects on cognition. Therapeutic intervention with anti-epileptic drugs (AEDs) variably modulates cognitive abilities in a patient with epilepsy. Pathological substrate specific effects can compound the potential negative effects of AEDs on cognition. In this review all these aspects are addressed with an analysis of relevant evidence from peer-reviewed publications.

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The supraorbital 'keyhole' craniotomy with an eyebrow incision is a versatile minimally invasive keyhole procedure for anterior and midline skull-base lesions. Experience with this technique for craniopharyngioma is presented, describing the surgical procedure with a brief literature review.

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A three and a half year old boy was diagnosed to have acute lymphoblastic leukemia in September 2002 and was treated with induction chemotherapy. He also received prophylactic cranial irradiation (12 Gy) and intrathecal methotrexate. In November 2005, at the age of 7 years, he developed right focal seizures and was diagnosed to have a glioblastoma in the left parietooccipital region while the leukemia was in remission. The possibilities are the glioma may have been radiation- and / or chemotherapy-induced.

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Although definititions of refractory epilepsy vary, about 40% of prevalent cases of epilepsy are not controlled by anti-epileptic drugs. A substantial proportion of this population requires palliative therapy since only a minority are candidates for epilepsy surgery. Drug therapy can be optimised after accurate classification of the epilepsy. Monotherapy is often as effective as polytherapy with fewer adverse effects. Depression and CNS adverse effects significantly impact quality of life and must be systematically screened for and treated. The ketogenic diet and vagal nerve stimulation provide substantial seizure control in a significant number of cases and may be used synergistically. Deep brain stimulation is another promising modality.

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A retrospective review of 41 children diagnosed with medulloblastoma in Birmingham (United Kingdom) between 1992-2002 presented. The age of the patients ranged from 2 weeks to 14.1 years (mean 5.89 years) and the mean follow-up was 3.8 years. Children less than 3 years of age fared worse than those over 3 years. The 5-year survival with total and subtotal tumor excision was 61.3 and 40% respectively. Those children who received radiotherapy following surgery had an improved survival of 68% at 5 years as compared to 18% in those who did not receive irradiation. With regards to chemotherapy, the 5-year survival rates were 57% in those who received it and 52% in those who did not. The overall survival was 62% at 5 years for those patients who had postoperative combined radiotherapy and chemotherapy and only 48% for the rest, who received no adjuvant therapy. Age of the patient has a definite influence on overall survival. We found significant statistical difference in survival between patients with total tumor resection and those with subtotal resection. The 5-year survival rate was not directly affected by tumor location, hydrocephalus or ventriculo-peritoneal shunting. Postoperative irradiation and chemotherapy are clearly associated with improved survival.

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Bilateral symmetric calcification involving striatum pallidum with or without deposits in the dentate nucleus, thalamus and white matter is commonly referred to as Fahr's syndrome. Symptoms of the disorder may include deterioration of motor function, spasticity, spastic paralysis, dysarthria, dementia, seizures, headache and athetosis. The clinical and imaging abnormalities are restricted to the central nervous system (CNS). We report an unusual association of Fahr's syndrome with polyarticular juvenile idiopathic arthritis in a girl.

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Pyogenic liver abscess occurring as a complication of ventriculoperitoneal shunt is of rare occurrence. The authors report an unusual occurrence of a pyogenic liver abscess in a 10-year-old child complicating the insertion of a ventriculoperitoneal shunt. It was successfully treated by intravenous antibiotics following shunt removal.

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