“Autism Spectrum Disorders” (ASDs) are neurodevelopment disorders and are characterized by persistent impairments in reciprocal social interaction and communication. Sleep problems in ASD, are a prominent feature that have an impact on social interaction, day to day life, academic achievement, and have been correlated with increased maternal stress and parental sleep disruption. Polysomnography studies of ASD children showed most of their abnormalities related to rapid eye movement (REM) sleep which included decreased quantity, increased undifferentiated sleep, immature organization of eye movements into discrete bursts, decreased time in bed, total sleep time, REM sleep latency, and increased proportion of stage 1 sleep. Implementation of nonpharmacotherapeutic measures such as bedtime routines and sleep-wise approach is the mainstay of behavioral management. Treatment strategies along with limited regulated pharmacotherapy can help improve the quality of life in ASD children and have a beneficial impact on the family. PubMed search was performed for English language articles from January 1995 to January 2015. Following key words: Autism spectrum disorder, sleep disorders and autism, REM sleep and autism, cognitive behavioral therapy, sleep-wise approach, melatonin and ASD were used. Only articles reporting primary data relevant to the above questions were included.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]  [PubMed]

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.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]  [PubMed]

Acute flaccid paralysis (AFP) is a life-threatening clinical entity characterized by weakness in the whole body muscles often accompanied by respiratory and bulbar paralysis. The most common cause is Gullian-Barre syndrome, but infections, spinal cord diseases, neuromuscular diseases such as myasthenia gravis, drugs and toxins, periodic hypokalemic paralysis, electrolyte disturbances, and botulism should be considered as in the differential diagnosis. Human coronaviruses (HCoVs) cause common cold, upper and lower respiratory tract disease, but in the literature presentation with the lower respiratory tract infection and AFP has not been reported previously. In this study, pediatric case admitted with lower respiratory tract infection and AFP, who detected for HCoV 229E and OC43 co-infection by the real-time polymerase chain reaction, has been reported for the first time.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]  [PubMed]

To review the clinical features and current understanding of spina bifida with an emphasis on the Indian Scenario. Selected articles and current English language texts were reviewed. The authors experience was also reviewed and analysed. Spina bifida is a common congenital anomaly encompassing a wide spectrum of neural tube defects.It is broadly classified as spina bifida aperta and occulta. With the prenatal screening, the incidence of aperta is gradually declining, whereas the detection of occulta has increased with the advent of magnetic resonance imaging. Over the years, the understanding of pathophysiology has made a significant changein the management of these anomalies. Early detection and complete correction can significantly reduce the neurological disability. This article is an overview of spina bifida with a special emphasis on Indian scenario.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

Autoimmune (antibody mediated) encephalitis (AE) is emerging as a more common cause of pediatric encephalopathy than previously thought. The autoimmune process may be triggered by an infection, vaccine, or occult neoplasm. In the latter case, onconeural autoantibodies are directed against intracellular neuronal antigens, but a recent heterogeneous group of encephalitic syndromes has been found not to have underlying tumor but is associated with autoantibodies to the neuronal surface or synaptic antigens. Neuropsychiatric symptoms are very common in autoimmune encephalopathy; as a result, affected children may be initially present to psychiatrists. Neurological features are movement disorders, seizures, altered conscious level, and cognitive regression. Hypoventilation and autonomic features may be an aspect. Inflammatory findings in the cerebrospinal fluid may be present but are relatively nonspecific. Magnetic resonance imaging (MRI) may also demonstrate abnormalities that provide clues for diagnosis, particularly on fluid-attenuated inversion recovery or T2-weighted images. AE is well responsive to immune therapy, with prompt diagnosis and treatment strongly beneficial. Patients with paraneoplastic encephalitis are more refractory to treatment compared to those in whom no malignancy is identified. Herein, the authors present an update of literature data on the clinical presentation, laboratory and imaging findings, therapy, and outcomes for the most common autoimmune encephalitides.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]  [PubMed]

Background : Intracranial meningiomas are rare tumors in children accounting for 0.4-4.6% of all primary brain tumors in the age group of 0-18 years. Objective : To retrospectively analyze the epidemiological profile, clinical features, radiological findings, type of excision, histopathological findings, and overall management profile of these patients. Materials and Methods : Eighteen consecutive cases of meningioma in patients under 18 years of age admitted and operated at our institute between the years 1974-2005 were included in this study. Results : The mean age of patient at presentation to our hospital was 12.81 years. The male to female ratio was 1.57:1. The median preoperative duration of symptoms was 1.2 years. An increased incidence was seen in patients with neurofibromatosis. Intraventricular and skull base locations were common. Total tumor excision was achieved in all cases. Conclusion : A higher incidence of atypical and aggressive meningiomas is seen in children. Children with complete resection and a typical benign histology have a good prognosis.

[ABSTRACT]  [FULL TEXT]  [PDF] [CITATIONS]  [PubMed]

Aim: To determine the association between Vitamin D and autism, and the difference in level of Vitamin D in autism children and control. Design: Case-control study conducted between June 2011 and May 2013, among autism at the Hamad Medical Corporation and controls at the School Health Clinics and Primary Health Care Clinics . Subjects and Methods: A total of 254 cases and 254 controls. The Autism Diagnostic Observation Schedule-Generic is a semi-structured, standardized assessment of social interaction, communication, play and imaginative use of materials for individuals suspected of having autism spectrum disorders. Data on clinical manifestations and laboratory, family history, body mass index (BMI) and clinical biochemistry variables including serum 25-hydroxy Vitamin D, calcium, phosphorus and magnesium were obtained. Univariate and multivariate statistical analyzes were performed. Results: Of the total number of 508 children surveyed, 254 of autism and 254 of healthy children were contacted. The mean age (΁ standard deviation, in years) for autism versus control children was 5.51 ΁ 1.58 versus 5.76 ΁ 1.56. There were statistically significant differences between autism and healthy children control subjects with respect to educational level of mother (P = 0.016); occupation of mother (P = 0.005); BMI (P < 0.001); consanguinity (P = 0.015); exposure to sun (P = 0.002) and walking time per day <60 min (P < 0.001). The mean value of Vitamin D in autism children was much lower than the normal value, and there was a significant difference found in the mean values of Vitamin D between autism (18.39 ΁ 8.2 with median 18) and versus control children (21.59 ΁ 8.4) (P < 0.0001) and with median 21 (P = 0.004). Besides mean values of calcium, phosphorous, magnesium, glucose, potassium and alkaline phosphate were statistically significant higher in control healthy children compared to autism children (P < 0.001). Multivariate logistic regression analysis revealed that the mean serum Vitamin D level, calcium, consanguinity, BMI, physical activity, child order, and ferritin, were considered as the main factors associated with autism. Of total 254 of autism children, 14.2% had severe Vitamin D deficiency (<10 ng/ml), 43.7% had moderate insufficient levels (between 10 and 20 ng/ml), 28.3% had mild insufficient levels (between 20 and 30 ng/ml), and only 13.8% of autism had sufficient levels (>30 ng/ml). Similarly, of the total 254 of healthy children 8.3% had severe Vitamin D deficiency (<10 ng/ml), 37% had moderate insufficient levels (between 10 and 20 ng/ml), 37.4% had mild insufficient levels (between 20 and 30 ng/ml), and only 17.3% had sufficient levels (>30 ng/ml). Furthermore, there was statistically significant differences between autism and control subjects with respect to the serum level of Vitamin D (P = 0.023). Conclusion: The present study revealed that Vitamin D deficiency was higher in autism children compared to healthy children and supplementing infants with Vitamin D might be a safe and more effective strategy for reducing the risk of autism.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]  [PubMed]

Central nervous system tuberculosis in children presents commonly as tubercular meningitis, post-tubercular meningitis hydrocephalus, and much more rarely as space-occupying lesions known as tuberculomas. The occurrence of this condition, though previously reported only in the developing world, is now frequently reported in human immunodeficiency virus positive migrants in the western world. The exact pathogenesis of this condition is still incompletely understood, and the mainstay of treatment is chemotherapeutic regimes. Neurosurgical intervention is rarely necessary, and is confined to cases of hydrocephalus after tubercular meningitis and to large tubeculomas with space-occupying effects.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

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.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]  [PubMed]

Craniostenosis is a disease characterized by untimely fusion of cranial sutures resulting in a variety of craniofacial deformities and neurological sequelae due to alteration in cranial volume and restriction of brain growth. This involves vault sutures predominantly, but cranial base is not immune. Association with a variety of syndromes makes the management decision complex. These children need careful evaluation by multiple specialists to have strategic treatment options. Parental counseling is an important and integral part of the treatment. Recent advancements in the surgical techniques and concept of team approach have significantly enhanced the safety and outcome of these children. We had an opportunity of treating 57 children with craniostenosis in the last 15 years at our craniofacial service. Out of them, 40 were nonsyndromic and 17 were syndromic variety. We describe our successful results along with individualized operative technical modifications adopted based on the current understanding of the disease.

[ABSTRACT]  [FULL TEXT]  [PDF] [CITATIONS]  [PubMed]

Background: Timed up and go (TUG) is a quick test used in clinical practice as an outcome measure to assess functional ambulatory mobility or dynamic balance in adults. However, little information is available on TUG test used in cerebral palsy. Hence, the purpose of our study was to assess the intra-rater reliability of TUG test in cerebral palsy children. Aim and Objective: To assess within-session and test-retest reliability after 1 week of TUG test in cerebral palsy children. Setting and Design: It was an a cross-sectional observational study conducted in a neurorehabilitation unit, with 30 cerebral palsy children of 4-12 years, within Gross Motor Function Classification System (GMFCS) level I, II, III, and with an IQ ≥50.The sampling technique used was purposive sampling excluding children with cognitive deficit. Materials and Methods: Subjects performed TUG on three occasions - Initial assessment (time 1), 30 min after initial assessment (time 2), and 1 week after initial assessment (time 3). Three trails were conducted for each of the three occasions. The mean score of three trials was documented as the final score. Within-session and test-retest reliability were analyzed using scores of time 1 and 2, and time 1 and 3, respectively. Statistical Analysis: The documented data were analyzed for within-session and test-retest reliability after 1 week of TUG test by using intraclass correlation coefficient (ICC). Results: Reliability of TUG test was high, with ICC of 0.99 for within-session reliability and 0.99 for test-retest reliability. Conclusion: Intra-rater reliability of TUG test in cerebral palsy children was found to be high.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]  [PubMed]

Background: Split cord malformation (SCM) is a rare condition. With decreasing incidence of neural tube defect (NTD) in the West, the reports of SCM are getting lesser and lesser. However, in India, spinal dysraphism is still a major problem encountered by the neurosurgeons. Objective: Our aim was to analyze 300 patients of SCM for their clinical features, radiological findings and outcome of surgery, which can throw light on the subject to others, who have less scope of finding these cases frequently. Materials and Methods: Over a 16-year period, we encountered 300 cases of SCM at AIIMS. Over the same period, more than 1500 cases of NTD were managed. SCM was noticed in 20% of cases with NTD. Skin stigmata were noted in two-third of the cases, and scoliosis and foot deformity were observed in 50% and 48% cases, respectively. Motor and sensory deficits were observed in 80% and 70% cases, respectively. Commonest site affected was lumbar or dorsolumbar (55% and 23%, respectively). In 3% cases, it was cervical in location. Magnetic resonance imaging (MRI) scan revealed a large number of anomalies like lipoma, neuroenteric cyst, thick filum and dermoid or epidermoid cysts. All the patients were surgically treated. In type I, bony spurs were excised, and in type II, bands tethering the cord were released. Associated anomalies were managed in the same sitting. Patients were followed up from 3 months to 3 years. Results: Overall improvement was noticed in 50% and stabilization in 44% cases and deterioration of neurological status was recorded in 6% cases. However, 50% of those who deteriorated improved to preop status prior to discharge, 7-10 days following surgery. Conclusions: SCM is rare and not many large series are available. We operated 300 cases and noticed a large number of associated anomalies and also multilevel and multisite splits. Improvement or stabilization was noted in 94% and deterioration in 6% cases. We recommended prophylactic surgery for our asymptomatic patients.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

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.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]  [PubMed]

Aim: To study the clinical outcome of shunt surgeries in children with hydrocephalus and evaluate the risk factors for ventriculoperitoneal (VP) shunt failure. Materials and Methods: Patients who underwent VP shunt surgery for hydrocephalus were included. Medical charts, operative reports, imaging studies, and clinical follow-up evaluations were reviewed and analyzed retrospectively. Results: A total of 137 patients with the average age of 20.7 months, range from 1.5 months to 8.5 years at the time of VP shunt surgery were included. The incidence of overall shunt complications was 35.76%; incidence of shunt revision was 27%, shunt blockade 45.94%, shunt infection 16.21%, shunt migration 10.81%, and shunt malfunction due to abdominal pseudocyst 10.81%. The mortality rate was 5.10%. The shunt revisions in the first 6 months after shunt placement was observed in n = 9 (24%). Hydrocephalus was associated with post-tubercular meningitis and intraventricular hemorrhage (IVH) in shunt placement was associated with multiple shunt revisions (n = 13, 35.13%) (n = 5, 45.4%), respectively. Conclusion: The findings of this study indicate that etiology of hydrocephalus, were associated with the shunt survival. Further prospective controlled studies are required to address the observed associations

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]  [PubMed]

Aim: Medulloblastoma is one of the most common posterior fossa tumors in childhood. The treatment-related side effects as well as predictive outcome still remain as a major challenge. The improved understanding of the disease and advances in molecular biology is changing the treatment paradigms from Chang's staging system to molecular risk stratification. However, surgery still remains as an important mainstay of therapy and is formidable. The role of radical surgery has always been a crucial factor in the outcome of these patients, the best survival being reported in patients who had total excision of the tumor and with no metastasis. Patient and Methods: An analysis of 365 patients (age<18 years) of medulloblastoma who underwent treatment at the Seth G.S. Medical College and King Edward VII Memorial hospital (KEM), Mumbai over a 25- year period (1985-2000 and 2001-2010) is presented. The clinical profile, radiological features, pathology and surgical nuances are discussed. Results: The most common age group affected was between 3 and 12 years. 75.3% presented with headaches, vomiting and 63.2% with papilledema. Sitting position was used in majority of cases. A total of 8 patients underwent shunting; all of them were in the postoperative period (5.19%). 92.2% (142 cases) had classical medulloblastoma, 5.1% (8 cases) had desmoplastic variant, 1.9% (3 cases) had anaplastic changes and 0.6% (1 case) had glial differentiation. The 5-year and 10-year progression free survival rate was 73 and 41% for average risk disease while for high risk disease rate it was 34%. The mortality rate was 2%. The quality of life was enhanced in patients who survived 5-10 years after treatment. Conclusion: Surgery for medulloblastoma is formidable. The option of sitting position for medulloblastoma surgery is still viable. A vigilant neuroanesthesiologist and a safe surgery are necessary to achieve a good postoperative result. Radiological characteristics are helpful adjuncts for determining effective surgical strategy. Permanent CSF drainage can be avoided in majority of patients and can be definitively considered in progressive symptomatic hydrocephalus. A safe maximal resection and a good Karnofsky score are paramount to ensure compliance with adjuvant therapy and contribute to an overall survival advantage.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

Context: Brain tumor is one of the most devastating forms of human illness, especially when occurring in the posterior fossa and involving the brainstem. Tumors in the posterior fossa are considered some of the most critical brain lesions. This is primarily due to the limited space within the posterior fossa, as well as the potential involvement of the vital brainstem nuclei. Aims: The aim of this study is to analyze the incidence, clinical features, surgical outcome, complications, and prognosis in a series of 37 pediatric patients with posterior fossa tumors who underwent surgery between September 2012 and January 2015 from the Department of Neurosurgery, King George Hospital, Visakhapatnam (both prospective and retrospective study). Materials and Methods: A series of 37 cases were treated by the Department of Neurosurgery, King George Hospital, between August 2012 and January 2015. Results: Posterior fossa tumors are predominantly seen in children with a peak incidence in the first decade. The most common presenting symptoms are raised intracranial pressure with headache and vomiting. Majority of the tumors are medulloblastomas, ependymomas, and cerebellar astrocytomas. The most common location is the cerebellar vermis, followed by the cerebellar hemispheres, followed by the forth ventricle and then the brainstem.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]  [PubMed]

The fifth edition of the diagnostic and statistical manual of mental disorders (DSM-5) introduced significant changes in the classification of autism spectrum disorders (ASD), including the abolition of the diagnostic subcategories proposed by DSM-IV-Text Revision. DSM-5 describes three levels of increasing severity of ASD. The authors report two explanatory cases with ASD (verbal boys, aged about 7 and a half years, without intellectual disability). According to DSM-5, both cases fall into the lowest severity level of ASD. However, their neuropsychological and neurobehavioral profile varies significantly. While the first boy showed a prevalent impairment of visuoconstructional and visuoperceptual abilities, the second one presented a predominant involvement of verbal functions, with qualitative impairments in communication. A further step forward in the definition and classification of ASD, taking into account both intensity and quality of symptoms, is recommended in order to formulate a reliable prognosis, plan an individualized treatment and monitor the clinical course over time.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]  [PubMed]

Objective: Surgical treatment of retrochiasmatic craniopharyngioma still remains a challenge. While complete removal of the tumor with preservation of the vital neurovascular structures is often the goal of the treatment, there is no optimal surgical approach available to achieve this goal. Transcranial and transsphenoidal microsurgical approaches, commonly used in the past, have considerable technical limitations. The extended endonasal endoscopic surgical route, obtained by removal of tuberculum sellae and planum sphenoidale, offers direct midline access to the retrochiasmatic space and provides excellent visualization of the undersurface of the optic chiasm. In this report, we describe the technical details of the extended endoscopic approach, and review our results using this approach in the surgical management of retrochiasmatic craniopharyngiomas. Methods: Fifteen children, including 9 girls and 6 boys, aged 8 to 15 years underwent surgery using extended endoscopic transsphenoidal approach between 2008 and 2014. Nine patients had a surgical procedure done previously and presented with recurrence of symptoms and regrowth of their residual tumors. Results: A gross total or near total excision was achieved in 10 (66.7%) patients, subtotal resection in 4 (26.7%), and partial removal in 1 (6.7%) patient. Postoperatively, headache improved in 93.3%, vision recovered in 77.3%, and the hormonal levels stabilised in 66.6%. Three patients (20%) developed postoperative CSF leaks which were managed conservatively. Three (20%) patients with diabetes insipidus and 2 (13.3%) with panhypopituitarism required long-term hormonal replacement therapy. Conclusions: Our early experience suggests that the extended endonasal endoscopic approach is a reasonable option for removal of the retrochiasmal craniopharyngiomas. Compared to other surgical approaches, it provides better opportunities for greater tumor removal and visual improvement without any increase in risks.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]  [PubMed]

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.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]  [PubMed]

Infantile tremor syndrome (ITS) is defined by the tetrad of pallor, developmental delay/regression, skin pigmentation, and brown scanty scalp hair. Involuntary movements in the form of tremors supervene in the natural course of the illness in a significant number of cases. The disorder occurs in exclusively breast-fed infants of vegetarian mothers belonging to economically deprived sections of society. Most of the children eventually recover but are frequently left with long-term cognitive and language neurodeficits. ITS continues to be seen in clinical practice, but there has been no consensus on its causation. Last comprehensive review was published in 1987 describing the ITS as a syndrome of unknown or multiple etiologies. Several important papers have been published since then. This review attempts to provide comprehensive and up-to-date information on the subject incorporating recently published studies. In the end, the issue of etiology is objectively re-examined in the light of available evidence some of which has been published in recent years.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]  [PubMed]

Cranial sonography continues to hold an important place in neonatal care. Attributes favorable to sonography that make it almost indispensable for routine care of the newborn includes easy access, low cost, portability, lack of ionizing radiations and exemption from sedation or anaesthesia. Cranial sonography has highest impact in neonates suspected to have meningitis and its complications; perinatal ischemia particularly periventricular leukomalacia (PVL); hydrocephalus resulting from multitude of causes and hemorrhage. Not withstanding this, cranial sonography has yielded results for a repertoire of indications. Approach to cranial sonography involves knowledge of the normal developmental anatomy of brain parenchyma for correct interpretation. Correct technique, taking advantage of multiple sonographic windows and variable frequencies of the ultrasound probes allows a detailed and comprehensive examination of brain parenchyma. In this review, we discuss the technique, normal and variant anatomy as well as disease entities of neonatal cranial sonography.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]  [PubMed]

Background: Anterior encephaloceles are rare conditions. Except for a few places from South East Asia, no large series has been published in the World literature. Materials and Methods: At AIIMS, we have managed 133 cases over a 40-year-period from 1971 to 2010. Frontoethmoidal type was the most frequent, noticed in 104 patients, followed by nasopharyngeal nasal in 12 and orbital encephaloceles in 6 patients. Observation: Ten patients were adults over the age of 18 years and 15 patients were between 5 and 18 years of age. Swelling over the nose was reported in all 104 patients with frontoethmoid type. In nasopharyngeal type, patients presented with respiratory problem. Patients with orbital mass had proptosis, on the side of encephalocele. Computed tomography (CT)/Magnetic resonance imaging (MRI) was performed in 127 patients, which was able to delineate the bone defect and associated brain anomalies. All the patients were subjected to repair of encephalocele. Patients with hypertelorism required orbital osteotomies and correction of deformity. Outcome: There were four deaths, all prior to 2000. No death was encountered in the last 10 years. CSF leak was the commonest postoperative complication, noticed in 24 patients. Overall cosmetic outcome was good.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

Purpose: To examine the effects of task-oriented activities based on neurodevelopmental therapy (TOA-NDT) principles on trunk control, balance, and gross motor function in children with spastic diplegic cerebral palsy (SDCP). Materials and Methods: Forty-four children with SDCP, aged 7–15 years, were recruited to participate in the randomized clinical trial. After random allocation, twenty-two (n = 22) children with SDCP participated in TOA-NDT principles and twenty-two (n = 22) in conventional physiotherapy (CPT) program. Each group underwent the treatment for a duration of 60min per day, 6 days a week for 6 weeks. Gross motor function measure-88 (GMFM-88), postural assessment scale (PAS), pediatric balance scale (PBS), and trunk impairment scale (TIS) were the outcome measures used to document the pre- and post-intervention effect. Results: The mean difference of GMFM-88, PAS, PBS, and TIS was 8.53 (5.84–11.23), 0.90 (5.84–11.23), 4.86 (2.93–6.79), and 1.45 (0.30–2.60), respectively. TOA-NDT group showed improvement in all the outcomes. Conclusion: TOA-NDT principles are more beneficial in improving the trunk control, balance, and gross motor function parameters than CPT.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]  [PubMed]

Increased intracranial pressure (ICP) is associated with worse outcome after traumatic brain injury (TBI). The current guidelines and management strategies are aimed at maintaining adequate cerebral perfusion pressure and treating elevated ICP. Despite controversies, ICP monitoring is important particularly after severe TBI to guide treatment and in developed countries is accepted as a standard of care. We provide a narrative review of the recent evidence for the use of ICP monitoring and management of ICP in pediatric TBI.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]  [PubMed]

Hydrocephalus is a common clinical problem seen in pediatric neurosurgical practice. Hydrocephalus involves dilatation of the cerebral ventricular system with corresponding, compressive effects on the parenchyma. It can be communicative or obstructive types. Congenital, acquired, infective, and secondary hydrocephalus have different clinical features with different modality of treatments. Ventriculoperitoneal shunt is the gold standard of treatment. Endoscopic 3 ^rd ventriculostomy is rapidly gaining prominence as an alternative. Various kinds of hydrocephalus, their pathophysiology, treatment and complications are reviewed.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]