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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Aim of the Study: The aim of this study is to evaluate the efficacy and safety of levetiracetam (LEV) as first-line treatment of neonatal seizures. Materials and Methods: This study was conducted in patients of Neonatal Intensive Care Unit of Santo Bambino Hospital, University of Catania, Italy, from January to August 2016. A total of 16 neonates with convulsions not associated with major syndromes, which required anticonvulsant therapy, were included and underwent IV LEV at standard doses. Results: All patients responded to treatment, with a variety range of seizure resolution period (from 24 h to 15 days; mean hours: 96 ± 110.95). No patient required a second anticonvulsant therapy. Regarding safety of LEV, no major side-effects were observed. Conclusions: To our knowledge, it is one of the few studies confirming the efficiency of LEV as first-line treatment in seizures of this age group. LEV was effective in resolving seizures and was safely administered in the current study.

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Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of excessive immune activation causing widespread inflammation and tissue destruction leading to multi-organ dysfunction and failure. Making the diagnosis of HLH could be quite challenging due to the broad range of presenting symptoms and their lack of specificity. After ruling out considerations for differential diagnoses, recognizing the most common presenting signs and symptoms of HLH, including neurologic dysfunction, and having a high clinical suspicion for HLH in the setting of inflammatory/demyelinating diseases are important for prompt diagnosis and treatment.

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Encephalocele is a rare lesion, being an embryological mesodermal anomaly which results in a defect in the cranium and dura, associated with herniation of meninges, cerebrospinal fluid, or brain tissues through a defect usually covered by scalp. Surgical management of children with giant occipital encephalocele requires careful attention to pediatric anesthetic and surgical principles. We present a case of a giant occipital encephalocele highlighting the problems encountered in its management.

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Aim and Objective: Cerebral palsy (CP) is the most common motor disability in childhood. This study aimed to describe clinical spectrum, comorbidities, and risk factors associated with CP children. Materials and Methods: This hospital-based observational study was conducted in tertiary level hospital in Jaipur including 180 CP children aged 1–12 years, attending the Paediatric Neurology Outdoor and Child Development Centre. A detailed history of antenatal, natal, and postnatal events taken and thorough examination was performed to stratify children in proper topographical and physiological classification. Results: Mothers of 47.7% CP children were primigravida and 17.7% mothers had anemia during pregnancy. Among natal factors, asphyxia contributed to maximum cases (52.2%). Seizure in postnatal life was the second most common risk factor for CP after asphyxia. Spastic CP (84.4%) was the most common physiological type, and quadriplegia (56.6%) was the most common topographical type observed in this study. Intellectual disability (47.7%) followed by epilepsy (41.6%) was the most common comorbidity. Conclusion: Even with the advancement of health-care system, asphyxia is the most common risk factor, and spastic quadriplegia is the most common type of CP. There is still a need of improving the health facilities to overcome this costly and common neuromotor disability. Widespread knowledge of common risk factors that can predispose to CP can prevent the CP development to some extent and knowledge of clinical spectrum, and comorbidities can improve their targeted treatment which can improve their growth and social participation.

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Cephalhematomas occurring in newborn usually resolve within a month. Rarely, they tend to ossify and present as hard scalp swelling. Unless one is aware of this possibility, this condition may be misinterpreted as bony tumor and cause needless apprehension to parents as well as the treating physician. A suspicion that ossified cephalhematoma (OC) could present in such a manner supported by careful history taking and relevant imaging (X-ray/computed tomography) would help in appropriate evaluation of this benign condition. The management of OC is controversial. Occasionally, they undergo spontaneous remodeling. Those with secondary craniosynostosis and/or disfigurement are treated surgically. Simple ossified lesions with no significant cosmetic issues may be conservatively tackled. We report such a case in a 3-month-old child. The other management options are briefly discussed.

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Solitary plasmacytoma of the bone (SPB) is a rare plasma cell neoplasm representing only about 5% of plasma cell neoplasia. It usually presents as a lytic lesion mainly localized within the axial skeleton. SPB is exceedingly rare in young individuals, and only few cases have been reported so far in patients younger than 20 years of age. In view of rarity of disease, definitive treatment guidelines have not been established. We hereby report a case of SPB involving of lumbar vertebra (L5) in a 12-year-old boy. He was initially treated with antilymphoma therapy and curative radiotherapy considering as primary bone lymphoma. However, he had local recurrence with paraparesis after 9 months which was diagnosed as solitary bone plasmacytoma for which he was treated with decompressive laminectomy and chemotherapy (bortezomib, lenalidomide, and dexamethasone). The purpose of this article is to report a rare case of SPB in a pediatric patient and to review the available literature and treatment options. SPB should be considered in the differential diagnosis of osteolytic bone lesions even in young patients.

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Background: Extent of resection is a very important prognostic marker in most pediatric brain tumors. Intraoperative imaging facilitates resection control. Intraoperative ultrasound (IOUS) is a cost-effective alternative to intraoperative magnetic resonance, but scant literature addresses its utility in this context. Methods: We retrospectively reviewed all pediatric brain tumors operated at our center using navigated three-dimensional ultrasound (US). The utility of the US in resection control was recorded and extent of resection evaluated. Results: IOUS was used in 20 cases (3 for frameless biopsy and 17 for tumor resection control). It was 100% accurate in localizing all tumors and yielded 100% diagnosis in the biopsy cases. Technical limitations precluded its use in 2 of the 17 cases of tumor resection. In the remaining 15, it correctly predicted the residual tumor status in 13 cases (87%). A gross total resection was achieved overall in 12 cases (80%) with postoperative morbidity in only one case. Conclusions: IOUS is a useful tool to localize intracranial tumors and guide the resection reliably. Widespread use can improve its applicability and make it an effective intraoperative imaging tool in pediatric brain tumors.

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Bladder migration and transurethral extrusion is an extremely rare complication of ventriculoperitoneal (VP) shunt. Only eight cases have been reported in the English literature since 1995. We report a case of a 4-year-old boy with cerebral palsy, hydrocephalus, and VP shunted on both sides who presented with a protruded distal VP shunt from his urethral orifice. The patient was reported for having previous shunt extrusion through the anus. The patient was treated on by a multidisciplinary approach, involving a neurosurgeon and urologist. Shunt removal with simple procedure was smoothly achieved without morbidities. He was discharged home in satisfactory condition.

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Osteoblastomas are rare, benign bone tumors mainly arising from the long bones and the posterior vertebral arches. Skull localizations account for approximately 15% of cases. A total amount of thirty cases involving the temporal bone are reported in the literature. Clinical presentation of temporal osteoblastomas often includes local pain and swelling, while 7^th and 8^th cranial nerve impairment is rare. We report the novel finding of increase intracranial pressure syndrome secondary to dominant transverse-sigmoid sinus junction compression caused by a small temporal bone osteoblastoma. Excision of the tumor with the restoration of venous flow in the sigmoid sinus was followed by a prompt clinical improvement. In the management of patients with a venous sinus compression, restoration of venous drainage should be a priority.

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Recent literature data have given emphasis to the relationship between gastrointestinal (GI) disorders and neurologic diseases, underlying a new pathogenic pathway: The so-called “gut–brain axis.” Herein, authors report a case of a 10-month-old male infant, admitted for drug-resistant epilepsy, associated with irritable behavior and GI discomfort, secondary to cow's milk protein allergy. Seizures were described by parents as upward eye movements that were mostly deviated to the right and were associated with slight extension of his neck. They were infrequent at first, but had increased gradually during the course of 3 days (up to 15–20 times/day). No anticonvulsant therapy was effective. Only a cow's milk protein-free diet, accidentally started during a gastroenteritis episode, was effective in stopping seizures. Our case underlines the peculiar vulnerability of the blood–brain barrier under 1 year of age, for which children of this age group experience neurologic manifestations during episodes of systemic inflammation.

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Objective: To observe the effect of L-type calcium channel blocker like nimodipine on morphine's withdrawal when it was administered continuously along with morphine versus a single bolus dose of nimodipine, which was administered at the end of the experiment before the precipitation of withdrawal reaction in morphine-dependent rats. Materials and Methods: Four groups of adult male Wistar rats were rendered morphine dependent by subcutaneous injections of morphine at a dose of 10 mg/kg for 10 days. Nimodipine 10 mg/kg intraperitoneally (ip) administered to one group once daily before morphine administration in the entire experimental period, and another group received nimodipine only once at the end of the experiment as a single bolus dose 2 mg/kg before the administration of naloxone. Naloxone 3 mg/kg was administered ip to all the groups to precipitate withdrawal reactions. The withdrawal reactions were evaluated and scored as per the Gellert and Holtzman global withdrawal rating scale. Results: Nimodipine when administered as a single bolus dose before naloxone administration in morphine-dependant rats reduced the features of withdrawal reactions more effectively than continuous administration of nimodipine along with morphine throughout the experimental period. Conclusion: We discovered that nimodipine helps in attenuating the severity of morphine withdrawal having potential role encountered during pharmacotherapy with morphine management of opioid dependence, well memory, impairement, cell signaling and phosphorylation of neuron.

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Segmentation defects are often seen with congenital atlantoaxial dislocation (AAD) though an associated absence of posterior arch of C2 and butterfly C3 is rare. Apart from rarity, the combination of formation and segmentation defects adds to the management dilemma. We report a case of AAD with assimilated atlas, absent C2 posterior arch, C3 butterfly vertebra with floating posterior elements, and fused C4–C6. The child was managed by C1–C2 fusion alone with immediate symptomatic improvement. The presence of formation defects such as adjacent butterfly vertebra and absent posterior elements does not alter the management of AAD. Fusing the C1–C2 joints appears to be a balanced approach.

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Sandhoff disease is a neurodegenerative disease caused due to deficiency of hexosaminidase (HEX) A and B. A 1-year-old male child presented with regression of milestones, exaggerated startle response, decreased vision, and seizures from 6 months of age. The child had coarse facies without hepatosplenomegaly. Serum levels of β hexosaminidase total (A + B) were low. Genetic testing for Sandhoff disease revealed a homozygous missense variant on HEXB gene. The case is presented to highlight that the absence of hepatosplenomegaly should not restrain in suspecting Sandhoff disease.

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Glutaric aciduria type I (GA I) is an autosomal recessive inborn error of metabolism caused by a deficiency of the enzyme glutaryl-CoA dehydrogenase. This disorder is characterized by progressive dystonia, choreoathetosis, and dyskinesia. It is often misdiagnosed as athetoid cerebral palsy. Laboratory evaluation usually demonstrates increased urinary excretion of gluataric acid and 3-hydroxyglutaric acid. We report a case of a 7-year-old boy presenting with choreoathetosis and dystonia, mimicking as choreoathetoid cerebral palsy. The presence of characteristic neuroimaging and biochemical studies led to the diagnosis of GA I.

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Craniospinal rachischisis is a rare and severe form of neural tube defects (NTDs), which is always fatal. It is characterized by anencephaly accompanied by a bony defect of the spine and exposure of neural tissue. We describe the two patients with ultrasonographic and magnetic resonance imaging appearance of craniospinal rachischisis totalis, detected antenatally at 22 and 25 weeks of gestation, and confirmed after termination of pregnancy. The multifactorial etiology of NTDs, with specific reference to folate deficiency, is discussed with possible role of folate fortification in the Indian context.

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Distal migration of shunt is a very common occurrence. Proximal migration of shunt is rare and possible pathophysiological mechanisms to explain this unusual complication is rarely attempted. A 5-month-old child shunted for posttraumatic hydrocephalus presented 1.5 years later with raised intracranial pressure and seizures. Imaging showed subdural hygroma, partial intracranial migration of shunt/chamber. On endoscopy, choroid plexus was adherent to shunt tip and some pericranial tissue was found in the anchoring suture (intraventricularly displaced). Shunt was retrieved endoscopically and diversion established by endoscopic third ventriculostomy with symptoms free follow-up. Host-related and surgical factors have been postulated. Tug-of-war effect on the anchoring suture and collapsing cortex are the possible mechanisms that explain proximal migration in our case. Three-point fixation of the chamber to pericranium, small burr hole with a smaller durotomy, can prevent shunt migration. Proximal shunt migrations should be dealt with endoscopy so as to avoid complications.

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Intracranial neurenteric cysts are uncommon congenital space-occupying lesions that may be misinterpreted for other more frequent nonneoplastic cysts and cystic tumors. We discuss the imaging findings of this lesion in a 16-year-old female who presented with chronic headaches.

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Lumbosacral lipoma is reported to occur in 4–8 of 100,000 patients. Sixty-six percent of lipomyelomeningocele in young patients have accompanied by hypertrophic filum terminale. It is rare to find two isolated spinal lipomas simultaneously. Embryological origin of dorsal and filar lipomas is different from each other and hence rarer to find them together. Radical resection is now being preferred for better long-term progression-free survival. We report an interesting case of spinal dysraphism in a 4-month-old female child with protruding, nontender, soft, subcutaneous 5 cm × 7 cm mass of the lumbosacral area that had been present since birth. Other anomalies included polydactyly of left hand. Magnetic resonance imaging demonstrated two isolated spinal lipomas, a transitional type and a terminal type filum lipoma with an interval of normal filum between the two. The findings were confirmed at surgery and detethering done along the white plane with neural placode reconstruction as described by Dachling Pang. The child had an uneventful postoperative recovery.

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Proteus syndrome (PS) is an extremely rare and complex disorder. Approximately 200 cases have been reported, and it seems to affect people of all ethnic and racial groups. PS is characterized by segmental overgrowth of multiple tissues and organs including vascular malformations, lipomatous overgrowth, hyperpigmentation, and various types of nevi. We hereby present a 7-year-old boy who presented with seizures and overgrowth of one-half of the body. Although classical physical features have been described, epilepsy and other neurological manifestations are rarely reported features of PS. Early detection of association of epilepsy and hemimegalencephaly with PS can prevent/minimize the neurological complications, disability, morbidity, and mortality.

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Neural tube defects are congenital development anomaly of the central nervous system and usually have relatively more predilection to affect at anterior and posterior neuropore embryological development sites, so usually one or two defects are commonly encountered. However, occurrence of simultaneous multiple neural tube defects is very rare, presence of constellation of five neural defects is extremely rare, and all defects add up together to produce gross neurological deficit. We present an interesting case of a 23-year-old male who presented with history of lower backache and noticed wasting and weakness of lower limbs associated with difficulty in walking for the last 2 years but had no associated sphincter disturbances. He was operated for lumbosacral lipomeningocoele repair at the age of 1 year. He was asymptomatic, following the first surgical intervention. At the current admission, he underwent re-exploration of surgical wound with surgical repair although suffered mild-temporary neurological worsening in the immediate postoperative period. Imaging feature and management of such rare constellation of five embryological anomalies and its significance and brief literature are discussed.

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X-linked lissencephaly with ambiguous genitalia is a rare and recently described syndrome. We report a neonate presenting with the classical features of the syndrome which includes lissencephaly, agenesis of the corpus callosum, intractable epilepsy of neonatal onset, acquired microcephaly, and male genotype with ambiguous genitalia. The baby was managed aggressively with antiepileptics. Early clinical suspicion of the syndrome can prevent future such occurrences in the family through genetic counseling.

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Ocular flutter is an eye movement disorder characterized by purely horizontal rapid saccadic oscillations lasting for a few minutes which stops spontaneously. Postinfectious ocular flutter and truncal ataxia are a rare entity. There are reported cases of opsoclonus myoclonus ataxia in association with dengue virus infection. However, there are no reported cases of parainfectious ocular flutter and truncal ataxia in association with dengue virus infection. Hereby, we report a child with dengue fever who had ocular flutter and truncal ataxia.

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Neurocysticercosis is commonly seen intracranially and its incidence in the spinal cord is very low. Among spine dorsal region is more common due to more blood flow in this region and it is usually associated with lesion at other sites. The intramedullary location is rarer than extramedullary. Hence, solitary intramedullary cervical spine cysticercosis (ICC) is extremely rare entity. Only a handful numbers of cases are reported in the literature. All reported cases are presented with the neurological deficit as spinal canal diameter is very low and any space occupying lesion is not tolerated. We are presenting a 6-year-old girl having solitary ICC with intact neurology, diagnosed by an appreciation of scolex on magnetic resonance imaging and were treated successfully with albendazole. Follow-up was performed by the estimation of Ag-ELISA which was negative after 2 months of completion of treatment and patient was asymptomatic at 2 years of follow-up.

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The selection of a treatment approach for a 2-year-old child with penetrating vertebral and spinal trauma, complicated by meningitis, is presented here. This pathology occurs rather rarely, which complicates the development of a uniform approach for the management of such patients. After surgical treatment of the wound in the lumbar region at L5-S1 level due to trauma, the leakage of a transparent discharge had been observed, the nature of which was unclear; it was considered at this treatment stage to be injury to the right ureter. The secondary surgical treatment of the wound was performed with local flap grafting, external lumbar drainage, and antibacterial therapy. The described treatment approach led to the involution of the main symptoms, and normalization of cerebrospinal fluid indicators resulting in an improvement in the child's condition. The represented supervision certifies that the penetrating wound does not always require laminotomy.

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We present the case of a ten-year old boy diagnosed with a rare syndrome as a complication of a commonly performed Neurosurgical procedure. To our knowledge this association has not been described in the literature and we discuss the plausible aetiology.

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A disorder of infants and children with pathological startle response, features of other system involvement, falls, and stiffness with retained consciousness. It should be differentiated from conditions such as myoclonic epilepsy, psychogenic movement disorder, Isaac syndrome, Schwartz–Jampel syndrome, Gilles de la Tourette, and culture-specific startle syndromes such as jumping Frenchman of Maine. A 5-year-old child symptomatic with repeated falls spontaneously as well as by sound and activities since neonatal period. He was having hyperalert facies, intelligent, cooperative with mild dysmorphism. His investigations were noncontributory except giant somatosensory evoked potentials and skeletal abnormalities. He showed excellent response to clonazepam and no complications on withdrawing the antiepileptic drugs. Proper diagnosis is of great therapeutic relevance and is based on high degree of suspicion.

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Dermoid cysts are rare lesions, particularly in children. Chiari II malformations are seen in patients with myelomeningocele. Here, we present a child with Chiari II malformation who, during a Chiari II decompression, was found to have a dermoid cyst. To the best of our knowledge, this is the first such case ever reported.

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Encephalocranial lipomatosis is a rare disorder that characteristically involves ectomesodermal tissues such as skin, eye, and the central nervous system. Here, we report a 3-year-old girl presented with developmental delay, seizures, limbal dermoid, and weakness of right lower limb. Imaging revealed hemiatrophy, arachnoid cyst, and polymicrogyria. The constellation of clinical finding and imaging leads to the diagnosis.

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Intrasacral meningocele is an unusual congenital lesion. It is an intrathecal cystic formation arising as a protrusion of the arachnoid through a congenitally weak place in the dura mater. We report a case of a 12-year-old child with chronic pain with an intrasacral meningocele identified on magnetic resonance imaging.

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Cerebrospinal fluid (CSF) shunt failure is commonly associated with infection or mechanical obstruction of the shunt system. A 4-year-old male child who had undergone multiple shunt revisions at another hospital for congenital hydrocephalus and later for shunt obstruction, presented with exposed shunt at the supraclavicular region. Shunt revision was performed. The CSF culture showed no growth; however, the histopathological examination of shunt tube showed Aspergillus growth inside the lumen of silicone tube well away from the tip of ventriculoperitoneal shunt. The skin biopsy from the exposed site revealed foreign body giant cell granulomatous reaction. The patient was discharged on postoperative day 6 without any complications. At 3 months follow-up, the patient is doing well. A growth of Aspergillus within the shunt tube prompted us to think of how the hardware can get infected and may remain a source of constant infection.

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