Objective : To re-emphasize that indirect revascularization surgery alone, where multiple burr holes and arachnoid openings are made over both cerebral hemispheres, is beneficial in the treatment of moyamoya disease (MMD) in children. Clinical Presentation : We report a 10-year-old boy who presented with complaints of episodic headache for the last 5 years. At the peak of his headache he had visual disturbances and acute onset weakness of left-sided limbs, recovering within a few minutes. He had no focal neurological deficits. Radiological investigations revealed abnormal findings, demonstrating the features of MMD. Surgical Management : He underwent bilateral multiple burr holes, dural and arachnoid opening over the frontal, parietal and temporal regions of each hemisphere. The elevated periosteal flap was placed in contact with the exposed brain through each burr hole. Results : On 6-months follow-up he had only one episode of transient ischemic attack. Postoperative four vessel angiogram demonstrated excellent cerebral revascularization around the burr hole sites, and single photon emission computerized tomography imaging showed hypoperfusion in the right temporo-occipital area suggestive of an old infarct with no other perfusion defect in the rest of the brain parenchyma. Conclusion : In children with MMD this relatively simple surgical technique is effective and safe, and can be used as the only treatment without supplementary revascularization procedures. This procedure can be done in a single stage on both sides and the number of burr holes made over each hemisphere depends on the extent of the disease.

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Background : Magnesium ion gates the N-methyl-D-aspartate (NMDA) receptor and may protect the brain from NMDA receptor-mediated asphyxial injury. The present study evaluated the neuroprotective role of magnesium in birth asphyxia. Material and Methods : Forty term neonates with severe birth asphyxia were randomized to either the study group or the control group. Neonates in the study group received magnesium sulfate in a dose of 250 mg/kg initially within half an hour of birth followed by 125 mg/kg at 24 and 48 h of birth. Cranial computed tomography (CT) scan and electroencephalography (EEG) were performed for all the babies. Denver II was used for developmental assessment at the age of 6 months. Results : Two babies in each group died of severe hypoxic ischemic encephalopathy. EEG abnormalities occurred in 43.75% of the cases in the control group compared with 31.25% in the study group. CT scan abnormalities were present in 62.5% of the control group compared with 37.5% of the cases in the study group. The Denver II assessment at 6 months revealed that there were five babies that were either abnormal or suspect in the control group compared with three in the study group. Conclusion : Magnesium is well tolerated and does appear to have beneficial effects in babies with severe asphyxia. More data is however needed and a large multicenter trial should be conducted.

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Split notochord syndrome (SNS) is an extremely rare congenital malformation associated with anomalies of the vertebral column, gastrointestinal tract and central nervous system. Twenty cases of SNS associated with dorsal enteric fistula have been reported in literature till date. The present report describes a unique case of SNS associated with lumbosacral meningomyelocele, dorsal neuroenteric fistula and dorsal herniation of right kidney along with vessels. The neonate was well managed by excision of enteric fistula, closure of duramater of meningomyelocele and repair of posterior wall hernia after placement of kidney in renal fossa. This kind of entity is uncommon and not been reported earlier.

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Aim : To study the clinical profile of acute disseminated encephalomyelitis (ADEM) in children. Materials and Methods : All children admitted with ADEM during a period of one and a half years were included in the study. The diagnosis of ADEM was made based on the clinical presentation and suggestive MRI findings. All children were treated with intravenous methyl prednisolone, followed by oral prednisolone and followed up for varying periods up to three and a half years. Results : The sample consisted of 14 children with 11(79%) girls and 3 (21%) boys. The oldest child was 12 years and the youngest was a six-month-old infant. Acute febrile illness preceded the onset of neurological symptoms in 64% of children. The interval between the preceding illness and symptoms of ADEM varied from 7 days to 28 days (mean 12 days). The common presenting symptoms were fever, vomiting, headache, gait disturbance and generalized seizures. Neurological manifestations included altered sensorium, multiple cranial nerve involvement, quadriplegia and paraplegia, dystonia and choreiform movements, nystagmus, bladder involvement (both incontinence and retention), speech defect and double vision. Facial nerve was the most common cranial nerve involved. Psychological manifestations included aggressive behavior, psychotic symptoms and mood changes. One child each had features of acute psychotic episode and depressive episode. All children recovered fully. One child had multiphasic disseminated encephalomyelitis (MDEM) on follow up. Conclusion : Despite the serious neuropsychiatric manifestations, ADEM in children generally has good immediate outcome. Children with ADEM need long-term follow up for cognitive impairments.

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A 15-year-old female presented with seizures, right-sided hemiparesis, hemiatrophy of the right side of the body and mental retardation. MRI brain revealed characteristic features diagnostic of congenital type of cerebral hemiatrophy or Dyke-Davidoff-Masson syndrome.

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Klippel-Trenaunay syndrome and Sturge-Weber syndrome are rare disorders with neurologic and cutaneous signs of vascular origin. Phakomatosis pigmentovascularis represents the association of widespread, aberrant, and persistent nevus flammeus and pigmentary abnormalities. We describe a case with features suggestive of overlap between them. A ten-month-old boy presented with seizures, developmental delay, skin lesions on face, trunk and legs, buphthalmos and right lower limb hypertrophy. CT scan of head showed atrophy of brain and calcification. Our case had overlap of Klippel-Trenaunay syndrome and Sturge-Weber syndrome with phakomatosis pigmentovascularis

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Objective : The minor physical anomaly (MPA) is believed to reflect abnormal development of the CNS. The aim is to find incidence of MPA and its behavioral correlates in Down syndrome and to compare these findings with the other causes of intellectual disability and normal population. Materials and Methods : One-hundred and forty intellectually disabled people attending a tertiary care set-up and from various NGOs are included in the study. The age-matched group from normal population was also studied for comparison. MPA are assessed by using Modified Waldrop scale and behavioral abnormality by Diagnostic assessment scale for severely handicapped (DASH II scale). Results : The Down syndrome group had significantly more MPA than other two groups and most of the MPA is situated in the global head region. There is strong correlation (P < 0.001) between the various grouped items of Modified Waldrop scale. Depression subscale is correlated with anomalies in the hands (P < 0.001), feet and Waldrop total items (P < 0.005). Mania item of DASH II scale is related with anomalies around the eyes (P < 0.001). Self-injurious behavior and total Waldrop score is negatively correlated with global head. Conclusion : Down syndrome group has significantly more MPA and a pattern of correlation between MPA and behavioral abnormalities exists which necessitates a large-scale study.

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To report a rare occurrence of osteoblastoma involving the L4 vertebra in an 8-year-old female child with histological features suggestive of osteoblastoma with secondary aneurysmal changes. The mean age incidence of osteoblastoma is 20.4 years. In our case, a rare presentation of osteoblastoma was seen in the first decade. The child was admitted with a 1-year history of increasing back pain and radiculopathy. The child was evaluated with X-rays, computed tomography scan and magnetic resonance imaging, which indicated involvement of the posterior elements of the 4 ^th lumbar vertebrae. Decompression of the L5 nerve and resection of the tumor was performed. Osteoblastoma is a rare tumor with an incidence of 1% of all tumors and 30-40% of cases involving the spine. Osteoblastoma occurs most commonly in males (M:F, 2.5:1). The most common area of involvement is the cervical spine followed by the lumbar spine. Posterior elements of the vertebrae are commonly involved.

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A nontraumatic spontaneous extradural hematoma, in a fully conscious 10-year-old male child, caused by a solitary eosinophilic granuloma of calvarium presented as a case of localized painful swelling of the head, which rapidly expanded and decreased in size. A plain CT-scan of the head with bone window revealed eroded right parietal bone with subperiosteal debris and extradural hematoma of mixed density. Immediate evacuation of the extradural clot and complete excision of the lesion was performed to prevent the deterioration of the patient and to achieve the histological diagnosis for further management.

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Homocystinuria is an inborn error of amino acid metabolism in which homocystine accumulates in the blood and produces a slowly evolving clinical syndrome. We are presenting a case of a 4-year-old female child who presented to us with stroke and also had megaloblastic anemia. She was diagnosed as having homocystinuria type-1, and she responded to treatment.

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Epidural lipomatosis is a rare entity to cause spinal cord compression and neurological deficits. This is usually associated with excess of steroids in the body either because of endogenous source as in Cushings disease or exogenous intake as in some diseases like systemic lupus erythematosus, in some endocrinopathies or in morbid obesity. But in some cases no cause has been found. Such idiopathic cases of spinal epidural lipomatosis have also been reported. Here, we report a case of idiopathic spinal epidural lipomatosis with relapsing and remitting paraparesis which is quite unusual. Treatment depends upon neurological status of the patient. We operated the patient as he had significant neurological compromise and he improved significantly.

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A 14-year-old male presented with bilateral papilledema, growth retardation and absent secondary sexual characters. He had a past history of fever, headache and fatigue of 6 months duration. The diagnosis of intracranial hypertension (IH) was confirmed by an increased intracranial pressure and normal neuroimaging studies of the brain, except for partial empty sella, prominent perioptic cerebrospinal fluid (CSF) spaces and buckling of optic nerves. Evaluation showed erythrocyte sedimentation rate (ESR) of 150 mm/hr, positive antinuclear antibody (ANA), anti dsDNA and anti ribosomal P protein. Renal biopsy revealed diffuse segmental proliferative lupus nephritis (LN) class IV S (A) confirming the diagnosis of systemic lupus erythematosus (SLE). Treatment of LN with intravenous pulse methyl prednisolone and cyclophosphamide was effective in normalizing the CSF pressure, resulting in express and dramatic resolution of symptomatology. In a case of IH, SLE must be considered. IH, growth retardation and absence of sexual characters may be presenting manifestations of a chronic systemic inflammatory disease like SLE. These manifestations may act as a pointer to associated advanced grades of LN, which can be totally asymptomatic and missed without a renal biopsy.

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Neurological complications have been described with seasonal influenza infection. We report encephalitis manifesting as seizures in a child with confirmed H1N1 infection. Treatment with oseltamivir was started. Child was discharged without any neurological sequelae.

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Brucellosis is an infectious disease, frequently encountered in developing countries. It may involve multiple organ systems of the human body. However, neurobrucellosis is a rare complication of brucellosis. The most frequent events of cranial involvement are meningitis and meningoencephalitis. In the present case, a 10-year-old girl was referred to our clinic with fever, headache, nausea, and vomiting. The patient's blood and cerebrospinal fluid cultures were found positive for brucellosis. Communicating hydrocephalus was also present in the cranial computed tomography as a complication of neurobrucellosis. The patient was successfully treated by external ventricular drainage and triple antibiotic therapy. There was no need to insert a ventriculo-peritoneal shunt.

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Clear cell meningioma is a rare subtype of meningioma seen mainly in pediatric patients. Supratentorial location is an unusual site of occurrence and its natural history and prognosis are not well described in the literature. We present a unique case of a left-sided frontoparietal tumor in a 15-year-old child who was managed successfully with gross total surgical excision and is recurrence-free at 18 months follow-up. A favorable clinical behavior and a longer symptom-free interval can be expected after gross total removal. The patients should be followed after successful surgery and other modalities of therapy should be used for recurrence.

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Developmental malformations of the cortex in neuronal migration disorders result in constellation of findings on radiological scanning. Isolated defects are common but sometimes they occur in varying degrees of combination giving a unique appearance on the imaging studies. We describe a case of neuronal migration disorder in which the computed tomography scan showed the presence of lissencephaly, colpocephaly. and Septal agenesis. These findings make the ventricular system appear in shape of a crown, which we refer to as "CROWN SIGN", first described in neurosurgical literature.

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Meningomyelocoele (MMC) forms one of the commonest forms of neural tube defect (NTD). It commonly affects lumbosacral area. Double or triple MMC has been reported at various sites of the spine. This supports multiple site closure of neural tube. We report a case of double MMC located at back of head, adjacent to each other like twin MMC. To our best knowledge, such defect has never been reported in the literature and raises query of our current understanding of embryogenesis of NTDs.

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The West syndrome (WS) is a characteristic form of epilepsy which usually begins in the first year of life. We describe two female siblings, aged 4 and 2 years, respectively, born from third degree consanguineous parents, with infantile spasms and developmental delay. Epileptic spasms had not a good outcome under antiepileptic drug treatment. Clinical and imaging features were of different severity in both siblings. Routine biochemical tests, metabolic investigations, and chromosomal analysis were normal. We analyzed CDKL5 gene by direct sequences and denaturing high-performance liquid chromatography using Transgenomic WAVE system. Analysis of the CDKL5 gene, which is responsible for female patient with WS, did not show any disease-causing mutation. WS has heterogeneous backgrounds, and may be more than one gene is responsible for its familial forms. In this family, consanguinity is observed in parents, which usually suggests that autosomal recessive inheritance is likely.

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Epidermoid cyst of the quadrigeminal cistern is uncommon, and its presentation as mutism as the main clinical finding with no other neurological finding is very rare. We report a case where the epidermoid cyst presented with progressive symptoms of absolute mutism, which improved significantly following surgery. The possible causes and pathophysiological mechanism of mutism in the lesions of this region are discussed in this paper.

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