Epilepsy with continuous spike-waves during slow-wave sleep (CSWS) is often characterized by a severe cognitive and behavioral impairment. Symptomatic cases also include patients with an early-onset hydrocephalus, but in literature detailed neuropsychological data on these subjects are not available. We describe the results of serial cognitive assessments in a girl with shunted early-onset hydrocephalus, followed by partial epilepsy complicated with CSWS at 4 years 10 months, in which a dramatic cognitive and behavioral deterioration occurred few months after CSWS onset. Adrenocorticotropic hormone treatment improved both clinical and electroencephalogram picture, but an impairment of visual perception, visual-motor coordination and executive functions persisted after CSWS disappearance. We hypothesize, in this case, an involvement of right occipital-parietal lobe and prefrontal lobe.

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Neonatal hypoxic-ischemic encephalopathy (HIE) is a devastating condition that may result in death or severe neurologic deficits in children. Neuroimaging with cranial ultrasound (US), computed tomography and magnetic resonance imaging are valuable tools in the workup of patients with HIE. The pattern of brain injury depends on the severity and duration of hypoxia and degree of brain maturation. Mild to moderate HI injury results in periventricular leukomalacia and germinal matrix bleed in preterm neonates, and parasagittal watershed infarcts in full-term neonates. Severe HI injury involves deep gray matter in both term and preterm infants. Treatment of HIE is largely supportive. The current article reviews the etiopathophysiology and clinical manifestations of HIE, role of imaging in the evaluation of the condition, patterns of brain injury in term and preterm neonates, the treatment and the prognosis.

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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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The glucose transport protein type 1 (GLUT1) deficit causes a chronic brain energy failure. The classic phenotype of GLUT1 deficiency syndrome is characterized by: Mild to severe motor delay and mental retardation; infantile-onset epilepsy; head growth deceleration; movement disorders (ataxia, dystonia, spasticity); and non-epileptic paroxysmal events (intermittent ataxia, periodic confusion, recurrent headaches). During last years the classic phenotype of this syndrome, as originally reported, has expanded. We report the atypical phenotype of a boy with GLUT1 deficiency syndrome, characterized by mild mental retardation and drug-resistant absence seizures with onset at the age of 6 years, without movement disorders nor decrease of head circumference. A prompt diagnosis of this disorder is mandatory since the ketogenic diet might represent an effective treatment.

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Introduction: Levetiracetam (LEV) is an effective antiepileptic drug also used in childhood and adolescence. Literature data regarding the long-term effects of LEV in childhood epilepsy and based on extensive neuropsychological evaluations using standardized tools are still scanty. Our study aimed to address this topic. Materials and Methods: We studied 10 patients with epilepsy characterized by focal or generalized seizures (4 boys, 6 girls; mean age: 10 years 8 months; range: 6 years 2 months - 16 years 2 months), treated with adjunctive LEV during a follow-up of 12 months. In 6 patients electroencephalogram (EEG) showed continuous spike and waves during sleep. Using standardized tools, we performed seriated assessments of cognitive and behavioral functioning in relation to seizure and EEG outcome. Results: Six patients completed the trial after 12 months of treatment; 1 patient dropped out of the study after 9 months, 3 patients after 6 months. Adjunctive LEV was effective on seizures in 3/10 patients and on EEG in 2/10 patients, and was well tolerated in all examined cases. Overall, no worsening of cognitive or behavioral functions has been detected during the period of the study; even at 6 and 12 months from baseline, an improvement in patients' abstract reasoning has been found, that was not related to seizure or EEG outcome. Conclusions: In our population of children and adolescents, LEV had no adverse cognitive or behavioral effects, short- or long-term. We found an improvement of abstract reasoning, regardless of seizure and EEG outcome.

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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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Background: Electrical status epilepticus in slow wave sleep (ESES) is a rare, age-related, self-limited disorder characterized as epilepsy with different seizure types, neuropsychological impairment in the form of global or selective regression of cognitive functions, motor impairment, and typical electroencephalographic (EEG) findings of continuous epileptic activity occupying 85% of nonrapid eye movement sleep. Aims: The aim is to examine the clinical and electrophysiological findings and treatment modalities of children with ESES and to evaluate the outcome of the disorder. Materials and Methods: Fourteen patients with a diagnosis of electrical status epilepticus during slow wave sleep and followed-up at least 2 years were included. Statistical Analysis: Pearson correlation test was used in the study. Results: Among the 14 patients, eight of them had normal mental development before ESES. Twelve of the patients mentioned cognitive impairment and decline in school performance during ESES. After ESES, seven patients had mental retardation in different severity. One of these patients was diagnosed with benign partial epilepsy of childhood with centrotemporal spikes and had normal intelligence quotient level prior to ESES. The diagnosis of ESES was made after newly occurred different seizure types in four of the patients while two of the previously known epileptic patients presented with only severe psychiatric impairment. Valproic acid and carbamazepine were the mostly frequently used drugs before the onset of ESES. After at least 2 years of follow-up, seven patients were seizure free, but still taking antiepileptic treatment. Five patients were seizure free, while two of them had ongoing seizures despite antiepileptic therapy. Conclusion: ESES should be kept in mind in children with unexplained regression or stagnation of development associated with seizures or not. Sleep EEGs should be performed for timely diagnosis, proper treatment and prevention of permanent cognitive impairment.

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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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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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Hypotonia in a newborn presents a diagnostic challenge for clinicians. It is an important clinical feature that may indicate an underlying systemic illness or neurological problem at the level of the central or peripheral nervous system. It is important to know the different presentations of hypotonia and to have the knowledge of the diagnostic work up which requires multidisciplinary assessment and input and the prognostic implications of these disorders. This review article presents a structured approach highlighting initial assessment, examination, and management of a neonate with generalized hypotonia.

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“Autism Spectrum Disorders” (ASDs) are neurodevelopment disorders and are characterized by persistent impairments in reciprocal social interaction and communication. Sleep problems in ASD, are a prominent feature that have an impact on social interaction, day to day life, academic achievement, and have been correlated with increased maternal stress and parental sleep disruption. Polysomnography studies of ASD children showed most of their abnormalities related to rapid eye movement (REM) sleep which included decreased quantity, increased undifferentiated sleep, immature organization of eye movements into discrete bursts, decreased time in bed, total sleep time, REM sleep latency, and increased proportion of stage 1 sleep. Implementation of nonpharmacotherapeutic measures such as bedtime routines and sleep-wise approach is the mainstay of behavioral management. Treatment strategies along with limited regulated pharmacotherapy can help improve the quality of life in ASD children and have a beneficial impact on the family. PubMed search was performed for English language articles from January 1995 to January 2015. Following key words: Autism spectrum disorder, sleep disorders and autism, REM sleep and autism, cognitive behavioral therapy, sleep-wise approach, melatonin and ASD were used. Only articles reporting primary data relevant to the above questions were included.

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To review the clinical features and current understanding of spina bifida with an emphasis on the Indian Scenario. Selected articles and current English language texts were reviewed. The authors experience was also reviewed and analysed. Spina bifida is a common congenital anomaly encompassing a wide spectrum of neural tube defects.It is broadly classified as spina bifida aperta and occulta. With the prenatal screening, the incidence of aperta is gradually declining, whereas the detection of occulta has increased with the advent of magnetic resonance imaging. Over the years, the understanding of pathophysiology has made a significant changein the management of these anomalies. Early detection and complete correction can significantly reduce the neurological disability. This article is an overview of spina bifida with a special emphasis on Indian scenario.

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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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Epilepsy is the most common neurological disease worldwide and is second only to stroke in causing neurological morbidity. Neuroimaging plays a very important role in the diagnosis and treatment of patients with epilepsy. This review article highlights the specific role of various imaging modalities in patients with epilepsy, and their practical applications in the management of epileptic patients.

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Objective : To compare the efficacy and side effects of 3% hypertonic saline and mannitol in the management of raised intracranial pressure in children. Design : Prospective randomized study. Setting : Pediatric intensive care unit (PICU) in a tertiary care hospital. Subject : 200 patients with raised intracranial pressure. Materials and Methods : Patients were randomized into two statistically comparable groups; Group A (n = 98) was treated with mannitol while Group B (n = 100) was treated with 3% hypertonic saline. Group C (n = 2) included those members of Group A in whom serum osmolality ≥320 mosmol/kg and were then treated with 3% hypertonic saline. Both Drugs were given at a loading dose of 5 ml/kg stat followed by 2 ml/kg in every 6 h(both have same osmolarity) for two days in their respective groups. Besides monitoring, blood pressure (NIBP), mean arterial pressure (pre and post 30 min of drug), serum sodium, chloride and osmolality were measured. Intracranial pressure was assessed indirectly by measuring mean arterial ressure "MAP". Student paired 't' test was applied. Results : Decrease in MAP was highly significant (P<0.001) at 0 h in males 0,6 h in females, and moderately significant at 12,36 h in females and significant(P<0.05) at 6,24,42 h in males of Group B. Decrease in coma hours was a highly significant finding (P<0.001) in Group B. In Group B, serum sodium and chloride increased significantly but remained within acceptable limits. There was no difference in osmolality and mortality (fisher Z). Conclusion : Mannitol has several side effects, 3% hypertonic saline is a safe and effective alternative in managing cerebral edema.

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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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Purpose: To examine the effects of task-oriented activities based on neurodevelopmental therapy (TOA-NDT) principles on trunk control, balance, and gross motor function in children with spastic diplegic cerebral palsy (SDCP). Materials and Methods: Forty-four children with SDCP, aged 7–15 years, were recruited to participate in the randomized clinical trial. After random allocation, twenty-two (n = 22) children with SDCP participated in TOA-NDT principles and twenty-two (n = 22) in conventional physiotherapy (CPT) program. Each group underwent the treatment for a duration of 60min per day, 6 days a week for 6 weeks. Gross motor function measure-88 (GMFM-88), postural assessment scale (PAS), pediatric balance scale (PBS), and trunk impairment scale (TIS) were the outcome measures used to document the pre- and post-intervention effect. Results: The mean difference of GMFM-88, PAS, PBS, and TIS was 8.53 (5.84–11.23), 0.90 (5.84–11.23), 4.86 (2.93–6.79), and 1.45 (0.30–2.60), respectively. TOA-NDT group showed improvement in all the outcomes. Conclusion: TOA-NDT principles are more beneficial in improving the trunk control, balance, and gross motor function parameters than CPT.

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Acute muscle pain and walking difficulty are symptoms compatible with both benign and severe degenerative diseases. As a consequence, in some cases invasive tests and hospitalizations are improperly scheduled. We report the case of a 7-year-old child suffering from acute calf pain and abnormal gait following flu-like symptoms. A review of the literature will be helpful to better define differential diagnosis in cases of muscle pain in children. d0aily physical examination and urine dipstick are sufficient to confirm the diagnosis of benign acute childhood myositis (BACM) during the acute phase, to promptly detect severe complications and to rule out degenerative diseases. Children with BACM do not require hospitalization, medical interventions or long-term follow-up.

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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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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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Background: Diverse tumors in the posterior third ventricular region (TPTVR) frequently occur in children. A decade's experience with pediatric TPTVR is presented, focusing on the Indian perspective. Materials and Methods: 25 children (age range: 3-18 years; mean age: 13.32 years; presentation range: 7 days-2.5 years) had clinico-radiological assessment with contrast computed tomography (CT) and magnetic resonance imaging (MRI). The ventricular/lumbar cerebrospinal fluid (CSF) alpha feto protein (AFP)/beta human chorionic gonadotrophin (HCG) estimation was done when radiological suspicion of a germ cell tumor was present. Extent of resection was deemed partial when some tumor mass remained at the end of surgery, near total when <10% was retained over vital neurovascular structures, and total when complete resection was attained. Results: Operations included infratentorial supracerebellar approach (n = 12), occipito-transtentorial approach (n = 2), endoscopic biopsy and third ventriculostomy (n = 1), frontal parasagittal craniotomy, interhemispheric transcallosal subchoroidal approach (n = 2), middle temporal gyrus transcortical transventricular approach (n = 1), fronto-temporo-zygomatic combined transylvian and subtemporal approach (n = 1) and right ventriculoperitoneal shunt and stereotactic biopsy (n = 1). Only CSF diversion was performed for five patients with a small TPTVR. CSF diversion was required in 12 (48%) patients. Tumor pathology included pinealoblastoma (n = 4; one with pineocytic differentiation), nongerminomatous germ cell tumor (NGGCT; n = 3), germinoma (n = 3), pilocytic astrocytoma (n = 2), epidermoid (n = 3) and primitive neuroectodermal tumor (PNET), fibrillary astrocytoma, glioblastoma, teratoma, and meningioma (n = 1, respectively). A patient with neurocysticercosis was diagnosed solely on MRI (four did not undergo biopsy). Fractionated radiotherapy was administered in 13 patients with primary pineal tumors, PNET, NGGCT, fibrillary astrocytoma and glioblastoma. Extent of excision was total in 10 (40%), near total in 5 (20%), partial in 3 (12%) and a biopsy in 2 (8%) patients. Conclusions: Histopathologic characterization of TPTVR is essential prior to their further management. Benign lesions often have a good prognosis following gross total surgical resection. Pure germinomas are highly susceptible to radiotherapy. NGGCTs often have malignant components that require adjuvant therapy following surgery. The advancements in microsurgical techniques have led to gratifying perioperative results in these deep-seated lesions.

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Increased intracranial pressure (ICP) is associated with worse outcome after traumatic brain injury (TBI). The current guidelines and management strategies are aimed at maintaining adequate cerebral perfusion pressure and treating elevated ICP. Despite controversies, ICP monitoring is important particularly after severe TBI to guide treatment and in developed countries is accepted as a standard of care. We provide a narrative review of the recent evidence for the use of ICP monitoring and management of ICP in pediatric TBI.

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Central nervous system tuberculosis in children presents commonly as tubercular meningitis, post-tubercular meningitis hydrocephalus, and much more rarely as space-occupying lesions known as tuberculomas. The occurrence of this condition, though previously reported only in the developing world, is now frequently reported in human immunodeficiency virus positive migrants in the western world. The exact pathogenesis of this condition is still incompletely understood, and the mainstay of treatment is chemotherapeutic regimes. Neurosurgical intervention is rarely necessary, and is confined to cases of hydrocephalus after tubercular meningitis and to large tubeculomas with space-occupying effects.

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Autoimmune (antibody mediated) encephalitis (AE) is emerging as a more common cause of pediatric encephalopathy than previously thought. The autoimmune process may be triggered by an infection, vaccine, or occult neoplasm. In the latter case, onconeural autoantibodies are directed against intracellular neuronal antigens, but a recent heterogeneous group of encephalitic syndromes has been found not to have underlying tumor but is associated with autoantibodies to the neuronal surface or synaptic antigens. Neuropsychiatric symptoms are very common in autoimmune encephalopathy; as a result, affected children may be initially present to psychiatrists. Neurological features are movement disorders, seizures, altered conscious level, and cognitive regression. Hypoventilation and autonomic features may be an aspect. Inflammatory findings in the cerebrospinal fluid may be present but are relatively nonspecific. Magnetic resonance imaging (MRI) may also demonstrate abnormalities that provide clues for diagnosis, particularly on fluid-attenuated inversion recovery or T2-weighted images. AE is well responsive to immune therapy, with prompt diagnosis and treatment strongly beneficial. Patients with paraneoplastic encephalitis are more refractory to treatment compared to those in whom no malignancy is identified. Herein, the authors present an update of literature data on the clinical presentation, laboratory and imaging findings, therapy, and outcomes for the most common autoimmune encephalitides.

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