Acute disseminated encephalomyelitis (ADEM) and Guillain-Barrι syndrome (GBS) are distinct demyelinating disorders that share an autoimmune pathogenesis and prior history of viral infection or vaccination. Our patient is a 10 years with acute flaccid paralysis, quadriparesis (lower limbs affected more than upper limbs), generalized areflexia and urinary retention. He had difficulty in speech and drooling of saliva. He also presented with raised intracranial pressure with papilledema; then bilateral optic neuritis developed during the later course of illness. Based on the temporal association and exclusion of alternative etiologies, diagnosis of the association between ADEM and GBS was made. Electro-diagnosis (electromyography-nerve conduction velocity) and magnetic resonance imaging study supported our diagnosis. He improved remarkably after treatment with intravenous immunoglobulin and intravenous methylprednisolone.

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Recurrent hypersomnias are very rare with two subtypes as Kleine-Levin syndrome and menstruation-related hypersomnia, which is very rarely encountered worldwide. Here, we report a young girl with menstruation-related recurrent hypersomnia, who was misdiagnosed as epilepsy due to co-existing generalized epileptic discharges. The importance of this comorbidity in terms of differential diagnosis of the attacks is discussed.

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Objectives: This study was performed to know the prevalence of primary headache disorders in school going children of central India and to elucidate the effects of various sociodemographic variables like personality or behavior traits, hobbies like TV watching, school life or study pressure in form of school tests, family history of headache, age, sex, body habitus etc., on prevalence of primary headaches in school going children of central India. Materials and Methods: A cross-sectional school-based study was performed on 500 school children (aged 7-14 years) for the duration of 1 year. Potential triggering and aggravating demographic and social variables were investigated based on a diagnosis of International Classification of Headache Disorder-II headache. Results: The prevalence of recurrent headache was found to be 25.5% in Indore. Of the studied population, 15.5% had migraine, 5% had tension-type headache migraine, and 5% had mixed-type headache symptoms suggesting both of above. Overall headaches were found to be more common among girls, but tension-type was more common in boys. Using regression analysis, we found that sensitive personality traits (especially vulnerable children), increasing age, female gender and family history of headache had a statistically significant effect on headaches in children. In addition, mathematic or science test dates and post weekend days in school were found to increase the occurrence of headache in school-going children. Hobbies were found to have a significant effects on headaches. Conclusion: As a common healthcare problem, headache is prevalent among school children. Various sociodemographic factors are known to trigger or aggravate primary headache disorders of school children. Lifestyle-coping strategies are essential for school children.

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Background: Convulsive seizures are the common neurological emergencies in developing regions. Objectives: The aim was to determine the prevalence, causes and outcome of seizures in childhood. Patients and Methods: Participants were children aged 1-5 years old, admitted consecutively with a history of febrile convulsions or were presented seizures with fever during hospitalization, in two pediatric university hospitals. The prospective study covered a period from January to December 2013. At admission, emergency care and resuscitation procedures were provided according to the national guidelines. The history included the number and a parental description of seizures. Children with epilepsy, any central nervous system infections and other disease were excluded. Results: We have recorded 3647 children. Among them, 308 (8.4%) infants had presented with febrile seizures including 174 males and 134 females admitted to both pediatric hospitals (Tokoin University Teaching Hospitals: 206/3070, Campus University Teaching Hospitals: 102/577). Infants from 1 to 3 years age were the most common affected and constituted 65.9% of all patients. The months of September, December and January had recorded the high frequency of admission due to seizures. Regarding the seizures type, generalized tonic-clonic seizures were predominant (46.4%) followed by tonic seizures (17.2%) and status epilepticus in 9%. The etiologies were marked by falciparum malaria (52.3%), and other infections in 47.7%. At discharge, we have noted 11% (34/308) with neurodevelopmental disabilities, 6.7% of epilepsy and 9.7% (30/308) of death. Conclusion: The febrile seizure in child younger 5 years is an indicator of severe malaria in tropical nations. The campaign for "roll back malaria" must continue in developing countries to avoid long-term gross neurological deficits.

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Empty sella syndrome (ESS) is commonly seen in adult and is considered as an infrequent finding in childhood. It may be diagnosed incidentally on imaging in asymptomatic children. However, most of the children with ESS present with features of hypothalamic-pituitary dysfunction. We report a case of ESS in a child with features of failure to thrive as well as hypopituitarism and review the literature briefly on the subject.

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Rare type of calvarial defects seen in patients with neurofibromatosis type-1 (NF1) is presented. The issues of pathogenesis and management are discussed. Two cases of NF1 with skull defects in the region of the lambdoid suture are reported. The possible etiological basis and nature of these type of defects and management issues are discussed. The calvarial skull defects in the lambdoid suture region are rare defects in NF1 patients. The possible reason of the progressive nature of these type of lesions can be the cerebrospinal fluid pulsations behaving like "growing skull fractures," especially when not associated with structural lesions. It leads to progressive enlargement of the small congenital defects in the region of the lambdoid suture and abnormal susceptibility of bones for resorption. For these defects, conservative management is suggested due to its progressive nature and high chances of operative treatment failure.

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Background: Endoscopic procedure has been known as the method of choice for treatment of hydrocephalus with 8.5% complication rate. It seems that good dural closure, reconstructing bone defect and perfect pericranium suturing can decrease the wound complications. Here, we describe the method of minicraniotomy instead of the burr hole in the endoscopic procedure. Materials and Methods: A case-control study regarding the cranial opening for endoscopic surgery was done in 45 patients of <12 months age; 15 patients in case group for minicraniotomy and 30 infants as control group for burr hole. They were followed at least 1-month for complications including cerebrospinal fluid collection, wound dehiscence, wound infection, and meningitis. Result: Patients were between 1 and 11 months. Hydrocephalus (73%) was the most common etiology for endoscopic surgery in this series, followed by the arachnoid cyst (20%). Two patients in the case group and eight in control group developed complications. Meningitis was found in one infant in the control group. Despite less complications in the case group the difference between two cohorts was not statistically significant. Conclusion: Minicraniotomy with providing more space in comparison to burr hole makes dural closure possible. It provides a small bone flap that can be replaced inside the bone defect. In spite of nonsignificant statistical difference between two groups regarding complication rate, we found less wound complications with minicraniotomy. Therefore, we advise this technique for the endoscopic procedure and propose minicraniotomy even in the older population to provide better dural opening, watertight dural closure and reconstructing the bone defect.

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Background: Postoperative cerebral venous infarction (POCVI) is not an uncommon complication in cranial surgeries. However, literature is sparse on the epidemiology and management of postoperative venous infarcts. Aims and Objectives: The aim was to study the incidence and clinico-radiological course of POCVI in patients in a tertiary level neurosurgical unit and compare the outcome between pediatric and adult patients following POCVI. Materials and Methods: In this prospective study carried out over an 8 month period, consecutive patients undergoing elective major cranial surgeries were monitored neurologically and with serial computed tomography (CT) of the head for POCVI in the postoperative period. All patients had at least one CT head done within 24 hours of surgery. Diagnosis of hemorrhagic POCVI was based on the presence of subcortical, multifocal hyperdensities with irregular margins and or low density areas in the perioperative fields. Nonhemorrhagic POCVI was diagnosed if CT showed a localized hypodensity poorly demarcated in the subcortical white matter with/without mass effect, along with the presence of fresh neurological deficits. Observations and Results: A total of 376 patients were enrolled in the study period. Of these, 26 (7%) developed POCVI. The male: female ratio was 1.2:1 and age ranged from 6 to 68 years with 12 (46%) being under the age of 18 years. Sixteen (61%) patients developed hemorrhagic POCVI and 10 (39%) patients developed nonhemorrhagic POCVI. The mean time to POCVI detection was 72 hours (range 24-144 hours). Seventeen (66%) patients were managed conservatively, and nine (34%) patients underwent decompressive craniectomy as an additional procedure for management of POCVI. In five patients (all with hemorrhagic POCVI), the infarction was an incidental finding. Of the 21 patients with symptomatic POCVI, 13 (61.9%) patients improved neurologically and were discharged with residual deficits. Two (9.5%) showed no neurological improvement till discharge, and 6 (28.5%) died during the hospital stay following POCVI. Conclusions: Children constitute a significant population (46% in our study) of the patients who develop POCVI with poor outcome similar to that seen in adult patients.

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Camphor is a pleasant-smelling cyclic ketone with propensity to cause neurologic side-effect, especially seizures. We report a case of 1½-year-old child who after inadvertent consumption of camphor, experienced an episode of generalized tonic clonic seizure. This case highlights the importance of enquiring any intake of material (medicinal or otherwise) in every patient presenting with seizure and notifying presence of typical smell, if any.

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West syndrome is classified according to the underlying etiology into an acquired West syndrome, a congenital/developmental West syndrome, and West syndrome of unknown etiology. Causes of a congenital/developmental West syndrome are extensive and include chromosomal anomalies. We report on a patient carrying a derivative chromosome originating from the reciprocal unbalanced translocation t (8;9) (p11.2;p22) and presenting with macrocephaly, West syndrome, severe mental motor retardation and hypotonia. As far as we know, this is a new chromosomal anomaly associated with West syndrome.

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Suprasellar arachnoid cysts can have varied presentations with signs and symptoms of obstructive hydrocephalus, visual impairment, endocrinal dysfunction, gait ataxia and rarely bobble-head doll movement. The bobble-head doll movement is a rare movement disorder characterized by antero-posterior bobbling of the head and neck on the trunk every 2-3 seconds. We present three cases with bobble-head doll syndrome associated with a large suprasellar arachnoid cyst and obstructive hydrocephalus, which were treated with endoscopic cystoventriculocisternostomy and marsupialization of the cyst.

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Acute disseminated encephalomyelitis (ADEM) is commonly seen after viral and bacterial infections, immunization, and Plasmodium falciparum (PF) malaria. Plasmodium vivax (PV) rarely causes ADEM. We report a 14-year-old female patient who presented with acute onset bilateral cerebellar ataxia and optic neuritis, 2 weeks after recovery from PV. Magnetic resonance imaging showed bilateral cerebellar hyperintensities suggestive of ADEM. No specific viral etiology was found on cerebrospinal fluid examination. Patient responded well to treatment without any sequelae. Thus, PV too is an important cause of ADEM along with PF. Two of the previously reported cases had co-infection with falciparum malaria. The only other two reported cases, as also this patient, are from Asia. A geographical or racial predisposition needs to be evaluated. Also, a possibility of post-PV delayed cerebellar ataxia, which is classically described post-PF infection, may be considered as it may be clinically, radiologically, and prognostically indistinguishable from a milder presentation of ADEM.

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Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is a rare mitochondrial disease. The available studies on MELAS syndrome are limited to evaluation of radiological, audiological, genetic, and neurological findings. Among the various neurological manifestations, speech-language and swallowing manifestations are less discussed in the literature. This report describes the speech-language and swallowing function in an 11-year-old girl with MELAS syndrome. The intervention over a period of 6 months is discussed.

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We present a four-year-old wth ethylmalonic encephalopathy who presented with delayed walking. She had bilateral hyperintense lesions in the basal ganglia. Molecular analysis revealed a homozygous c.3G>T mutation in the ETHE1 gene. She did not have typical findings of the disease including recurrent petechia, chronic diarrhea and acrocyanosis was very subtle and orthostatic. She benefited from riboflavine and Q10 treatments. We suggest that acrocyanosis should be questioned and examined in patients with motor delay.

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Though hemangiomas of the bone are quite common, calvarial (skull) cavernous hemangiomas are relatively rare pathologies. Calvarial hemangiomas are usually small and asymptomatic. However, they may occasionally grow in size to achieve large sizes and can present as a palpable swelling. We present a child with massive temporo-parieto-occipital calvarial cavernous hemangioma, who was managed with a multimodal approach with excellent cosmetic and neurologic outcome.

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Tumefactive multiple sclerosis (MS) is a rare variant of MS characterized by the presence of large demyelinating plaques of more than 2 cm, identified with magnetic resonance imaging (MRI). Distinguishing tumefactive lesions from other etiologies of intracranial space occupying lesions is necessary to avoid the inadvertent intervention. We had a 14-year-old girl who presented to us with two episodes of subacute hemiparesis over a span of 6 months. Her MRI brain showed large lesions, which were hyperintense on T2-weighted/flair images with incomplete ring enhancement open towards the gray matter in postgadolinium images with minimal surrounding edema and mass effect. We treated her as a case of tumefactive demyelination (TD) with steroids after which patient recovered with minimal deficits. TD occurs more commonly in women and young adults and is reported rarely. TD in a young girl with recurrence in such short span causing bilateral hemiparesis has never been reported.

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Guillain-Barre syndrome (GBS) is rarely reported in children with acute lymphoblastic leukemia (ALL) and may be difficult to differentiate from vincristine induced neuropathy. Only few case reports highlighted GBS with ALL. We report a 10-year-old male child who was a diagnosed case of ALL since 3 month on chemotherapy. At 3 ^rd week of chemotherapy, he developed rapidly progressive ascending motor quadriparesis over 2 days. Clinical and electrophysiology revealed acute motor axonal neuropathy (AMAN) variant of GBS. He was treated with intravenous immunoglobulin (2 g/kg) without discontinuing chemotherapy. Complete recovery took 12 weeks despite immunotherapy, and it was corroborating to slow remission. We concluded that AMAN variant is usually present in B-cell type ALL, may be causal for GBS and it takes 6-16 weeks to complete recovery which may correspond to remission of ALL. However, it needs to be studied. We also present a meta-analysis of previously reported cases of GBS in ALL.

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Primary intraosseous or ectopic meningioma of the skull is a rare tumor accounting for about 1% of meningioma. Intradiploic meningioma is an extremely rare type of extraneuraxial meningiomas. Intradiploic meningioma of the orbit is extremely rare, and <8 such cases are reported till date in western literature occurring in the pediatric age group. Here the authors present a case of 16-year-old female, who presented with progressive proptosis, with normal vision and was managed successfully surgically. Clinical features, pathophysiology, and surgical management of this rare entity are discussed in the context of pertinent literature.

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Neurological manifestation as the presenting feature of dengue infection is rare. This is a brief description of five children 5 months to 11 years with presenting features as seizures or altered sensorium. Bleeding manifestations were seen in two. Cerebrospinal fluid examination was normal in all. All were diagnosed as per WHO definition of dengue hemorrhagic fever and managed as per standard protocol. Serology (IgM dengue) or nonstructural protein 1 antigen was positive in all.

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Synchronous intracranial germ cell tumor in the pineal and suprasellar region is rare. They represent only 5-10% of all intracranial germinomas. They are also known by the entity "double midline atypical teratoma" and are common in the second decade of life. We report a case of an 11-year-old girl having dual midline intracranial lesions with obstructive hydrocephalus treated by ventriculo-peritoneal shunt. Diagnosis of germinoma was made on the basis of imaging and elevated beta-human chorionic gonadotropin in cerebrospinal fluid. Radiotherapy was instituted, which resulted in the total disappearance of both the lesions. Clinical expression, diagnosis and management strategies are discussed.

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Angelman syndrome is a neuro-developmental disorder with a genetic basis in maternal genomic imprinting that presents with cognitive and behavioral impairment. The distinction between the presentations of disorders causing developmental delay may be vague in early life, resulting in the potential delay in diagnosis and intervention. We report a case of Angelman syndrome with initial diagnostic confusion with multiple associated pathologies. A holistic evaluation and management of such patients is necessary.

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The aim was to present a rare case of recurrent craniopharyngioma remote from the primary site of origin. A young girl was operated for sellar region craniopharyngioma. For a small residual tumor, she underwent radiotherapy. Follow-up imaging did not reveal any residual tumor or recurrence. Surveillance magnetic resonance imaging after 5 years revealed a recurrence in the right Sylvian fissure. This tumor was totally excised.Recurrence of craniopharyngioma is well-known, but recurrence at a site remote from the original site after radiotherapy is extremely rare. One such case is being presented.

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Osteoma located in the paranasal sinus is a slow growing benign tumor. It commonly occurs in frontal, ethmoid and maxillary sinuses in the order of frequency. Giant osteoma is very rare in the pediatric population, and only nine cases of giant osteoma are reported till date in the form of isolated case reports. Authors report a giant spheno-ethmoidal osteoma in a 14-year-old boy, who presented with progressive visual impairment and proptosis of the left eye for last 1-year. Total excision was carried out using transcranial extradural approach. The postoperative recovery was uneventful and showed remarkable visual recovery along with cosmesis.

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Guillain-Barre syndrome (GBS) is a common cause of acute flaccid paralysis in children. Axonal variants of this disease are rare, and frequently life-threatening or debilitating. The course and outcome of a 17-month-old child with acute flaccid paralysis including severe respiratory involvement are presented. GBS was suspected. Nerve conduction studies demonstrated acute motor-sensory axonal neuropathy including both phrenic nerves. The difficulties with the diagnosis and management of this severe and life-threatening condition are discussed. Significant morbidity is also highlighted. Axonal variants of GBS although rare cause significant morbidity in children. Diagnosis relies solely on accurate neurophysiologic testing and is important because the available treatment options for GBS are frequently ineffective in these variants.

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Spinal intramedullary tubercular abscess itself is a rare entity. Very few cases have been reported. We report a case of a 4-month-old female with a dermal sinus in lower back since birth, intermittent fever for 2 months, acute onset paraparesis and bowel bladder involvement showing an intramedullary contrast enhancing lesion extending from D11 to S2 level with low lying conus, and a subcutaneous tract in lower back at S2 level extending from skin up to the sacral canal on magnetic resonance imaging of the spine. Drainage of abscess and biopsy revealed tubercular infection on histopathology. The patient made a good recovery with anti-tubercular treatment and physiotherapy. The source of tubercular infection could not be established. The baby had received Bacillus Calmette-Guérin vaccination at birth and the possibility of vaccination associated tubercular infection could not be ruled out.

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