Objective: To analyze immediate and long-term results of lipomeningomyelocele (LMM) repair in asymptomatic patients. Materials and Methods: Seventeen patients of LMM presented to Department of Paediatric Surgery over a period from 2011 to 2015 were evaluated preoperatively by magnetic resonance imaging of whole spine, and pre- and post-operative Ultrasound of kidney, ureter, bladder, and neurosonogram. Surgical procedure involved total excision of lipoma in 15 patients and near total excision in 2 patients. Division of filum terminale could be done in 15 out of 17 patients. Follow-up varied from 1 to 3.5 years (mean 1.9 years). Results: This study included 10 (58.8%) patients of lumbosacral LMM, 5 (29.4%) patients of sacral, and 2 (11.7%) patients of thoracolumbar LMM. About 13 (76.4%) patients were operated before 3 months of age, 2 (23.5%) patients were operated between 3 and 6 months, and two patients were operated between 6 and 11 months. None of the patients had bladder/bowel dysfunction preoperatively. Preoperative lower limb power was normal in all patients. Objective improvement in lower limb motor function was observed in 3 (17.6%) patients and three patients had decreased lower limb power. Two patients developed altered sensations and weakness of lower limb about 2.5-3 years after initial LMM repair. They needed repeat detethering of cord. Two patients had fecal pseudoincontinence, whereas one patient developed constipation. Bowel dysfunction was managed by rectal washouts, and oral laxatives were added if required. One (5.8%) patient of lumbosacral LMM and 1 (5.8%) patient of sacral LMM had urinary incontinence postoperatively. This was managed by clean intermittent catheterization with continuous overnight drainage. Conservative management of bladder and bowel dysfunction was effective in all patients till the last follow-up. Two patients developed hydrocephalus after LMM repair for which low-pressure ventriculoperitoneal shunt was inserted. Wound infection occurred in 1 (5.8%) patient, whereas 7 (41.1%) patients developed seroma in wound which responded to repeated aspirations under aseptic precautions. Conclusion: With total excision of lipoma and division of filum terminale satisfactory outcome for asymptomatic patients of LMM can be achieved. Authors recommend early surgery for LMM even in asymptomatic patients. Patients with residual lipoma and undivided filum terminale should be observed closely for the development of progressive neurological changes.

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Objectives: Triclofos may be a better sedative in view of better palatability and less gastric irritation as compared to chloral hydrate. This study aimed to assess the efficacy of triclofos (a commonly used sedative in India) as a sedative for sleep electroencephalogram (EEG) study in children. Methods: This prospective observational study was carried out in a tertiary care pediatric center. Consecutive children aged 6 months to 5 years referred for sleep EEG evaluation were recruited. Their clinical details were noted in a proforma after an informed consent. After a trial for natural sleep, oral triclofos was administered. Sleep parameters and adverse effects were noted. Results: One-hundred and sixty children were then enrolled. EEG was successfully recorded in 149 (93.1%) children. Median latency of sleep onset was 30 min and median duration of sleep was 90 min. The adverse effects in the following 24 h were mild and included dizziness, irritability, and vomiting. Conclusions: Oral triclofos was found to be an effective sedative for EEG in children with minimal adverse effects.

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A human tail or a caudal appendage is a rare condition with preconceived notions and stigmata. They could be either true tails or "pseudotails" based on their embryology. Clinically, they are considered as a marker of occult spinal dysraphism. We report two such cases with tethering of cord, one of which was associated with a lipomeningomyelocele. These patients are in need of meticulous evaluation and appropriate management.

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Objective: Petit mal epilepsy or absence seizures involve brief, sudden lapses of consciousness and most often occurs in people under age of 20 years. This study was done to find out the most likely significant age affected by petit mal epilepsy and whether they had higher rate of behavioral, educational, and social problems. Materials and Methods: We run tests on total 32 patients (male 16 and female 16) from newborns to 20 years of age. Results: The most affected ages were from 4 to 9 years and both genders were equally affected. They have higher rate of behavioral, educational, and social problems, and most likely recovering ages from the disease were from 15 to 20 years. Conclusion: These findings could contribute in diagnosis and treatment of Petit Mal Epilepsy, as it often misinterpreted as daydreaming or inattention.

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Schizencephaly is an extremely rare developmental birth defect characterized by abnormal slits or clefts in the cerebral hemispheres extending from the lateral ventricle to the cerebral cortex. The margins of the cleft are lined with heterotropic, dysplastic gray matter. The causes of schizencephaly are heterogeneous and include teratogens, prenatal infarction/infections, maternal trauma, or EMX2 mutations. This condition is present at birth and manifests early in life.

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Neurodevelopmental disorders are known to have varied etiology. Among known etiologic causes, congenital rubella syndrome (CRS) is reported to be one of the infections associated with neurodevelopmental disorders. CRS has been reported to be associated with large number of psychiatric manifestation. However, data from developing countries on psychiatric manifestations of CRS are nonexistent. In this report, we present the case of a 7-year-old boy, who presented with mental retardation, atypical autism, and attention deficit hyperactivity disorder. Since birth, the child was found to have congenital cardiac defects and was found to have bilateral profound sensorineural hearing loss since the age of 6 months. Magnetic resonance imaging showed multifocal symmetrical T2/fluid attenuated inversion recovery hyperintensities in bilateral cerebral hemisphere suggestive of sequelae of congenital rubella infection.

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Parry-Romberg syndrome (PRS) is a rare, poorly understood degenerative condition characterized by atrophic changes affecting one side of the face. The cause of these changes remains obscure. Migraine and facial pain such as trigeminal neuralgia are the most common neurological symptoms in this patient group. Sometimes, it causes epilepsy and rarely cerebral hemiatrophy, meningeal thickening, cortical dysgenesis, calcified lesions, aneurysms, and intracranial vascular malformations. Herein, the author reports the first case of PRS with multiple large intracranial cysts producing raised intracranial pressure.

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Metachromatic leukodystrophy (MLD) is caused by insufficiency of arylsulfatase A resulting in impaired myelination. Diffusion magnetic resonance (MR) imaging features of this disease have been rarely reported. We report diffusion MR imaging of MLD in a 12-month-old male who presented with regression of milestones and progressive spasticity.

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Primary cerebellar agenesis is a rare entity. To the best of our knowledge, eleven living cases have been reported till date. Most of these were associated with some degree of motor impairment. We present a case of cerebellar agenesis in a child who presented with cognitive abnormalities leading to poor performance at school. No motor impairment was seen. Among the eleven cases reported earlier, only one case showed lack of motor impairment.

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Japanese encephalitis (JE) is a mosquito borne encephalitis caused by Flavivirus. Neurocysticercosis (NCC) is a parasitic disease of the central nervous system caused by Taenia solium. In this report, we describe the clinical profile, imaging findings, and outcome of two children with JE and coexisting NCC. Eleven and thirteen-year-old boys from the same town of Jharkhand state were brought with history of fever, seizures, altered sensorium, and extrapyramidal symptoms. Dystonia, hypomimia, bradykinesia, and dyskinesia were observed. Meige syndrome observed in one of the children is a novel finding. Magnetic resonance imaging of the brain revealed findings suggestive of JE with cysticercal granulomas. There are few reports of coexistence of JE and NCC in children. Both children were treated with ribavirin, and follow-up imaging had shown significant resolution of signal changes. Both the children had shown marked clinical improvement. Ribavirin was found to beneficial in reducing the morbidity in our patients.

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Atypical teratoid/rhabdoid tumor (AT/RT) is a rare central nervous system neoplasm affecting children, and isolated primary spinal involvement is extremely rare. Authors describe a case of spinal AT/RT in a 5-year-old male child presenting with rapidly progressing quadriparesis diagnosed and managed surgically and medically. Biopsy revealed large, rhabdoid cells with prominent nucleoli in nest and immunohistochemistry further showed loss of integrase integrator 1 expression considered to be gold standard for diagnosis. AT/RT has extremely poor prognosis with median survival being 6 months.

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Aims: Lateral transtemporal approaches are useful for addressing lesions located ventral to the brainstem, especially when the pathologic diagnosis of the tumor dictates that a gross or near total resection improves outcomes. One approach, the presigmoid approach receives little attention in the pediatric population thus far. We sought to characterize morphometric changes, particularly the clival depth and the petroclival Cobb angle, that occur in the temporal bones of children and draw implications about doing a presigmoid approach in children. Settings and Design: This study was a retrospective study performed at John Sealy Hospital, a level-one trauma center that takes care of pediatric injuries as well. Subjects and Methods: We performed a morphometric analysis of noncontrast computed tomography head studies in 96 boys and 67 girls. Central clival depth and petroclival angle were obtained in the axial plane at the level of the internal auditory meatus using the method described by Abdel Aziz et al. Statistical Analysis Used: Descriptive statistics and Student's t-test to compare groups were calculated using Microsoft Excel. Results: We found no gender difference in mean central clival depth or petroclival angle (P = 0.98 and P = 0.61, respectively). However, when we broke our cohort by age into those younger than 9 years of age and those 10 years or older, we found the petroclival angle decreased by 6.2° which was statistically significant (P < 0.000000006). Conclusions: These findings suggest that a presigmoid retrolabyrinthine approach is useful for children 9 years of age and younger as the petroclival angle appears to decrease resulting in a shallower clival depression in these patients.

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Twin nasal dermal sinus with associated intracranial dermoid located in interfalcial region is a very rare occurrence and is reported only in the occipital and temporal regions. However, multiple sinuses located in the canthus and dorsum of nose are not reported till date. Authors report an interesting first case of interfalcial dermoid cyst associated with twin discharging dermal sinuses, who underwent successful surgical repair in the world literature. The authors report the management of an unusual case and the review has been discussed briefly.

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Moyamoya disease (MMD) is a rare cerebrovascular disease characterized by idiopathic occlusion of bilateral internal carotid arteries and the development of characteristic leptomeningeal collateral vessels along anterior or posterior circulation. We present an unusual case of MMD presenting with generalized dystonia as the predominant manifestation.

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Blowout fractures are a common occurrence in traumatic brain injury patients. In pediatric age group, orbital floor fracture is a common occurrence. We report a case of 2-year-old male admitted to trauma center, with penetrating injury to the left eye by the clutch of motorbike which fell on the child. Noncontrast computed tomography scan revealed fracture of the roof of left orbit with left frontal contusion sparing the left eyeball. There was also the continuous leak of brain matter from the left eye which suggested tear of dura mater. Urgent left frontal craniotomy was done with the evacuation of contusion, reconstruction of orbital roof, and duroplasty under general anesthesia.

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Pneumococcal meningitis remains a life-threatening infection, with varied presentations. A 3 month-old-baby with pneumococcal meningitis presented with clusters of seizures evolving into refractory status epilepticus despite standard antibiotic and aggressive anticonvulsant therapy. Progressive illness despite antibiotic initially suggested possible antibiotic resistance and resulted in addition of another antibiotic. Nonresponse to standard treatment and previous history of abscess in the back of neck pointed to some underlying congenital immunodeficiency. Further evaluation showed a deficiency of complement factor C3. This case underlines the need to consider underlying immunodeficiency in cases of refractory status epilepticus due to bacterial meningitis. Gram-staining of cerebrospinal fluid sample showing plenty of Gram-positive bacteria and comparatively fewer pus cells is a clue regarding some underlying immunodeficiency.

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Oculomotor nerve palsy can be due to varied causes that include diabetic neuropathy, myasthenia gravis, brainstem infarction, demyelinating conditions, and cerebral aneurysms. Among the aneurysmal causes of oculomotor nerve palsy, aneurysm of the posterior communicating artery has been observed to be the most common. Pupillary dysfunction is considered to be an important feature of aneurysmal oculomotor nerve paresis. A case of a 7-year-old boy with partial oculomotor nerve palsy with pupillary sparing is being reported here, the etiology of which is tortuous and ectatic distal internal carotid artery. This is a rare cause of oculomotor nerve paresis and to the best of our knowledge has not yet been reported in children. Ischemia rather than compression seems to be the most plausible cause in this case.

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Growing skull fracture is an extremely rare complication of pediatric head injury, especially in infants. Repair of the dural tear early in the course of development of growing skull fracture has been suggested for a better outcome. Surgical repair of large, tense growing skull fractures, especially those in the communication of the ventricles can lead to potentially life-threatening complications. The author reports a rare case of remote intracranial hemorrhage following surgery for large, tense growing skull fracture in a 12-year-old girl and discusses the likely pathogenesis and possible ways to avoid this life-threatening complication.

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