We are presenting a case of a 10-year-old female child who presented with normal development till 5 years of age followed by deterioration in previously acquired language and social skills with stereotypic hand movements suggestive of childhood disintegrative disorder. This case is reported as this condition is very rare.

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Pantothenate kinase-2-associated neurodegeneration (PKAN) is a rare autosomal recessive pediatric neurodegenerative disorder characterized by rigidity, dystonia, impaired postural reflexes, and progressive dementia. On T2-weighted magnetic resonance imaging images, marked low signal intensity is seen in the globus pallidus. This low signal intensity surrounds a central region of high signal intensity in the anteromedial globus pallidus, giving an eye-of-the-tiger appearance.

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Aim: A brain injury results in a temporary or permanent impairment of cognitive, emotional, and/or physical function. Predicting the outcome of pediatric brain injury is difficult. Prognostic instruments are not precise enough to reliably predict individual patient's mortality and long-term functional status. The purpose of this article is to provide a guide to the strengths and limitations of the use of hyperbaric oxygen therapy (HBOT) in treating pediatric patients with severe brain injury. Materials and Methods: We studied total 56 patients of head injury. Out of them 28 received HBOT. Only cases with severe head injury [Glasgow Coma Scale (GCS) < 8] with no other associated injury were included in the study group. After an initial period of resuscitation and conservative management (10-12 days), all were subjected to three sessions of HBOT at 1-week interval. This study group was compared with a control group of similar severity of head injury (GCS < 8). Results: The study and control groups were compared in terms of duration of hospitalization, GCS, disability reduction,and social behavior. Patients who received HBOT were significantly better than the control group on all the parameters with decreased hospital stay, better GCS, and drastic reduction in disability. Conclusion: In children with traumatic brain injury, the addition of HBOT significantly improved outcome and quality of life and reduced the risk of complications.

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Context: Patients with cyanotic congenital heart disease (cCHD) are prone to develop frequent brain abscesses. Surgery for these abscesses is often limited to aspiration under local anesthesia because excision under general anesthesia (GA) is considered a riskier option. Perioperative hemodynamic instability, cyanotic spells, coagulation defects, electrolyte and acid base imbalance, and sudden cardiac arrest are among the major anesthetic concerns. Most of our current knowledge in this area has been gained from a neurosurgical standpoint while there is a paucity of corresponding anesthesia literature. Aims: To highlight the anesthesia issues involved in cCHD children undergoing brain abscess excision under GA. Settings and Design: Retrospective study of our institutional experience over a 5 year period. Materials and Methods: Of all the children with cCHD who underwent brain abscess surgery from January 2005 to December 2009, only 4 were operated under GA. Surgery was done after correcting fever, dehydration, electrolyte imbalance, coagulopathy and acid-base abnormalities, and taking appropriate intraoperative steps to maintain hemodynamic stability and prevent cyanotic spells and arrhythmias. Results: All 4 patients had a successful abscess excision though with varying degrees of intraoperative problems. There was one death, on postoperative day 34, due to septicemia. Conclusions: Brain abscess excision under GA in children of cCHD can be safely carried out with proper planning and attention to detail.

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Objectives: (1) To determine the frequency of abnormal neuroimaging in children with new-onset afebrile and complex febrile seizures; (2) to draw a correlation between Electroencephalogram (EEG) and neuroimaging. Study Design: A hospital-based prospective study. Materials and Methods: A total of 276 children (6 months to 14 years of age), who presented with new-onset afebrile or complex febrile seizures, underwent EEG and neuroimaging [Computed Tomography (CT) and/or Magnetic Resonance Imaging (MRI)]. Results: Generalized seizures constituted the major seizure group in our study - 116/276 (42%) - followed by partial seizures 86/276 (31.2%) and complex febrile seizure in 64/276 (23.2%). Generalized as well as partial seizures were more common in children aged 6-14 years, while complex febrile seizures were predominantly seen in children less than 6 years old. Most of the patients with generalized and partial seizures had EEG abnormalities, while EEG abnormalities were uncommon in patients with complex febrile seizures. A total of 27/276 (9.8%) patients with seizure disorder had abnormal CT scans and this abnormality was more common in patients with partial seizures. CT abnormality was seen more commonly in those patients who had an abnormal EEG. EEG and CT correlation showed that patients with abnormal EEG had higher rates of CT abnormality, ie, 16.1% (25/155). Abnormal MRI was seen in 32/157 (20.4%) of patients; accuracy of picking abnormality by MRI, when EEG was abnormal, was 24.8% (P<0.05). Conclusion: Our findings indicate that clinical examination and EEG results are good indicators for neuroimaging, and these can be used as one of the criteria for ordering neuroimaging in new-onset seizures.

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Iron deficiency anemia is a common pediatric problem affecting up to 25% children worldwide. It has been linked with cerebral venous sinus thrombosis in the literature. We describe a 9-month-old child who had severe iron deficiency anemia and developed acute venous sinus thrombosis associated with minor infection. Treatment with anticoagulation was partially successful with persistent thrombosis after 3 months. We reviewed the current literature highlighting the association of anemia as a risk factor for development of stroke in children.

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Intracranial arachnoid cysts developing in relation to the cerebral hemispheres and middle cranial fossa are usually incidental or asymptomatic. However, most of the clinically active cysts present with seizures because of chronic compression. Presentation as raised intracranial pressure due to cyst rupture into the subdural space is a rare clinical entity. We herein present a case of an asymptomatic arachnoid cyst with rupture into the subdural space bilaterally and presenting as raised intracranial pressure.

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Background: Intensive behavioral intervention programs are recommended for children with autism. However, in resource-constraint settings, such programs are largely inaccessible, and there is an urgent need for development of low-cost interventions. Aim: To evaluate the efficacy of a parent-based behavioral intervention program in Indian autistic children. Materials and Methods: Sixteen children with autistic disorder undergoing the intervention for at least six months were enrolled. The mean development, social, expressive, and receptive language quotients, and the Childhood Autism Rating Scale (CARS) and Autism Behavior Checklist (ABC) scores were compared before and after the intervention. Results: The average duration of therapy was 19.5±11.78 months. There was a significant improvement in the development quotient (P=0.015), social quotient (P=0.004), expressive language quotient (P=0.03), CARS (P=0.001), and ABC (P=0.014) scores. Conclusion: Parent-based behavioral intervention programs have a promising role in management of children with autism in resource-constraint settings.

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Cavernous hemangioma (CH) is a sporadic vascular malformation occurring either as an autosomal dominant condition or as a well-known complication of radiation exposure. Medulloblastoma is a primitive neuroectodermal tumor common in children and currently treated with surgical resection, chemotherapy, and radiotherapy. Neurofibromatosis is the most common single-gene disorder of the central nervous system. Posterior fossa malignant tumors in the context of neurofibromatosis type I (NF1) are very infrequent. This is the first documented case of an unusual metachronous occurrence of non-radiation-induced CH and medulloblastoma in a child with NF1 phenotype. We report the case of a 13-month-old boy with cafι-au-lait skin lesions associated with NF1-like phenotype who underwent surgical resection of a single CH in the temporal lobe due to recurrent seizures. Four years later he presented with signs of raised intracranial pressure associated with a posterior fossa tumor and hydrocephalus, thus requiring gross total resection of the lesion. Histological analysis revealed a medulloblastoma. After being treated with radiotherapy and chemotherapy, he achieved total remission. Six years later a massive recurrence of the tumor was observed and the child eventually died. The interest in this case lies in the rarity of NF1-like phenotype associated with a non-radiation-induced brain CH and medulloblastoma in a child.

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Acute cerebellitis is an inflammatory syndrome occurring most commonly in young children. It is caused by a variety of insults and is usually bilateral. Pseudotumoral hemicerebellitis is an exceptionally rare unilateral presentation of acute cerebellitis mimicking a tumor. Magnetic resonance imaging (MRI) reveals a diffusely swollen cerebellar hemisphere, but with the lack of a well-defined mass, which is hyperintense in T2-weighted images and with pial enhancement in post-contrast images. It typically has a benign course with regression in follow-up scans, thus distinguishing it from a tumor. Recognizing this entity is important because erroneous diagnosis may lead to needless surgical intervention. We present a case of pseudotumoral hemicerebellitis in a 12-year-old boy with coagulopathy, with follow-up MRI depicting hemorrhage, and discuss the pathogenesis.

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Ewing's sarcoma is a primary bone malignancy with the highest incidence in the second decade of life. Although it mostly affects the metaphyseal region of long growing bones, involvement of spine is not very uncommon especially the sacrum. Nonsacral spinal Ewing's sarcoma is rarer and often mimics a benign condition before spreading extensively. They present with neurologic deficits due to spinal cord compression, but acute onset paraplegia has not been previously reported. A high index of clinical suspicion can clinch the diagnosis early in the course of the disease. A prompt intervention is required to keep neurological damage to a minimum, and a correct combination of surgery, chemotherapy, and radiotherapy is required for better long-term patient outcome. We report a 16-year-old female who presented with acute paraplegia and had an excellent postoperative outcome after radical excision of a D9 Ewing's sarcoma.

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Alveolar soft part sarcoma is a rare tumor affecting mainly adolescent and young children. It presents as a slowly growing tumor and is usually overlooked due to lack of symptoms. Early metastasis is a characteristic feature of this tumor and, in a good number of cases, metastasis to the lung or brain is the first manifestation of the disease. In this report, we present a case of alveolar soft part sarcoma predominantly located in the right frontal bone with dural breach and contiguous right frontal lobe involvement in a 17-year-old girl without any evident primary or other secondaries. A brief review of literature is also presented.

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Intramedullary spinal cord tubercular abscess with involvement of whole cord is a rare entity that too with syrinx formation following disseminated meningitis. Accurate diagnosis requires a high index of suspicion with clinical history and imaging features for a favorable outcome. Here-in we present a similar case with tubercular etiology which was also associated with syrinx formation and has not been reported previously in the literature up to the author's knowledge.

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Objective: To evaluate the efficacy and tolerability of levetiracetam (LEV) add-on therapy in children with refractory epilepsy. Materials and Methods: We evaluated 102 children (56 boys, 46 girls, mean age 96.00 ± 31.15 months) with refractory epilepsy. Patients received LEV as add-on therapy. Changes in seizure frequency and adverse events were observed. Follow-up was conducted at least 6 months after treatment. Results: In total, 46 (45.1%) of the 102 children achieved more than 50% seizure frequency reduction, and 16 (15.6%) of these were seizure free. LEV reduced seizure frequency by at least 50% in 58.3% of patients with partial seizures, in 32.0% of patients with primary generalized seizures, and in 17.6% of patients with both partial and generalized seizures. Conclusion: Our results confirm that LEV, as add-on therapy, was effective in reducing seizure frequency in a variety of seizure types but seems most effective for partial-onset seizures. LEV was also well-tolerated in children.

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Japanese encephalitis (JE) and malaria would coexist in the areas where both illnesses are endemic with overlapping clinical pictures, especially in a case of febrile encephalopathy with hepatosplenomegaly. However, there are no published data till date showing concurrent infection of these two agents despite both diseases being coendemic in many areas. We report a case of concurrent infection of JE and mixed plasmodium infection, where the case, initially diagnosed as cerebral malaria did not improve on antimalarials and alternative diagnosis of JEV encephalitis was thought which was confirmed by a serological test. To the best of our knowledge, this is the first case report of concurrent Japanese encephalitis with mixed plasmodium infection. We report a case of 3-year-old male child, who presented with febrile encephalopathy with hepatosplenomegaly. Based on a rapid diagnostic test and peripheral smear examination, a diagnosis of mixed P.Vivax and P.falciparum infection was made and the patient was treated with quinine and doxycycline. However, besides giving antimalarials the patient did not improve and an alternative diagnosis of JE was considered as the patient was from the endemic zone of Japanese encephalitis. Cerebrospinal fluid (CSF) of the patient was sent for a virological study which came out to be positive for JE IgM in CSF, which is confirmatory of JE infection. In a patient with febrile encephalopathy with hepatosplenomegaly especially in areas coendemic for JE and malaria, the possibility of mixed infection should be kept in mind.

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Aseptic meningitis is characterized by noninfective serous inflammation of the meninges. It can occur in a recurrent fashion when associated with dermoid and epidermoid cysts due to rupture of cyst contents into subarachnoid space resulting in aseptic chemical meningitis. Bacterial meningitis in association with these tumors is commonly related to a coexisting dermal sinus tract and the most common organism is Staphylococcus aureus.

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Objective: The objective was to analyze the demography, clinical presentation, and management of spinal intradural schwannomas in pediatric population. Materials and Methods: This retrospective study includes 21 pediatric patients (under 18 years of age) who underwent surgery for spinal intradural schwannomas from January 1998 to April 2008. The medical records were reviewed retrospectively and the information regarding clinical presentation, tumor location, operative findings, and postoperative status and functional outcome were analyzed. Results: A total of 21 patients (14 females and 7 males) were operated for spinal schwannomas. Six patients had associated neurofibromatosis (five were NF I and one was NF II) at presentation. The most common presenting symptom was progressive myelopathy (86%). The tumor location was either cervical or dorsal in 18 cases. All patients underwent surgery. Gross total excision was achieved in 20 cases. The median follow-up was 38 months. All the patients had neurological improvement in both power and bladder symptoms. Conclusion: Pediatric spinal neurofibromas/schwannomas are an uncommon but completely treatable group of tumors. Complete surgical excision gives excellent outcome.

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A rare case of third ventricular choroid plexus papilloma and associated complication of shunt ascites is described. We propose to convert shunt into ventricular drain in such a situation and reassess the requirement of shunt after complete tumor removal.

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Intramedulary dermoid tumors are rare tumors mostly found associated with dermal sinus tracts. Spinal dermoid tumor can occur after myelomeningocele repair. Infected dermoid tumors are reported in patients with dermal sinus tracts as well, but have never been reported subsequent to myelomeningocele surgery. Here, we report a rare association of infected dermoid tumor with tethered cord without dermal sinus tract in a child who had been operated for myelomeningocele during infancy.

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Though primitive neuroectodermal tumor has been considered to arise from a neoplastic transformation of primitive neuroepithelial cells with propensity to involve any part of the central nervous system, a primary intramedullary spinal primitive neuroectodermal tumor is very unusual. The authors describe a case of an 18-year-old female with conus intramedullary tumor diagnosed to be primary spinal primitive neuroectodermal tumor following histopathological examination after surgery. The diagnosis of such a tumor is very crucial as the management strategies for these are relatively unclear and are associated with a poorer outcome compared to the other common intramedullary spinal tumors.

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