A 15-year-old boy presented with coarse tremors of right hand and dysarthric speech. Neurologic examination demonstrated Kayser-Fleischer rings and dystonic tremor of the right hand. Serum ceruloplasmin and urine copper studies established the diagnosis of Wilson's disease. Brain MRI showed bilateral T2 hyperintensity involving putamen, thalami, and brainstem. Involvement of brainstem revealed the characteristic "double panda sign."

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Anterior sacral meningocele (ASM) is a rare form of spinal dysraphism in children. Usually asymptomatic, it can present as constipation, urinary problems or rarely neurological symptoms. High index of suspicion with careful clinical examination is necessary to make early diagnosis. Magnetic resonance imaging is the investigation of choice. We describe a successfully managed young child with ASM associated with rib and vertebral defects.

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Aim: The long term outcomes of congenital hydrocephalus are still not clearly known despite it being a common clinical condition. Several clinical, radiological factors were correlated to predict the functional outcomes. This study aimed to correlate the clinical, radiological parameters with the regional functional outcomes of the brain. Materials and Methods: Children with congenital hydrocephalus were divided into Group A with hydrocephalus alone and Group B hydrocephalus with spina bifida. Ventriculoperitoneal shunt surgery was performed by the same surgeon. CT scans and neuropsychological assessments were performed before and serially after the shunt. The clinical and the radiological findings were correlated with the developmental levels during the follow-up. Results: There were 25 children in Group A and 15 children in Group B; 72% in Group A and 93% in Group B were less than 6 months of age at the time of treatment. Forty percent in Group A and 92% in Group B had the signs of hydrocephalus at admission. Cerebrospinal fluid (CSF) diversion results in the reduction in ventricular dilatation and corresponding increase in the cortical mantle thickness. The ventricular size and the cortical mantle thickness were measured serially and correlated with the development in the neuropsychological function. In this study, 80% in Group B reached near normal development in comparison to 33% in Group A. We have noticed a significant correlation in the increase in the regional cortical mantle thickness with corresponding improvement in the functional development. This clearly ratifies the improvement in the frontal and parietal areas having their distinctive effect on the functional development of the child. Conclusion: Early CSF diversion and timely intervention seems to benefit functional recovery. It is interesting to note that reconstitution of cortical mantle in different areas of the brain showing corresponding improvement in their respective areas. Large ventricles (head circumference more than 50 cm) recurrent subdural collections and repeated shunt obstructions have a bad influence on the long-term outcome. Unlike the previous belief the children with myelomeningocele can have equal benefit in terms of neuropsychological development after the shunt surgery.

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Neurocysticercosis is the most common parasitic infection of the central nervous system caused by larvae of Taenia solium. Spinal cysticercosis is an uncommon site of cysticercal infection, and isolated intramedullary involvement is even rarer. We present a case of 10-year-old girl who presented with gradual onset paraparesis with sensory loss and bowel and bladder incontinence. Magnetic resonance imaging (MRI) of spine revealed a cystic lesion with mural nodule (scolex) which was diagnostic for cysticercosis. Patient was treated with antihelminthic, which led to marked clinico-radiological improvement.

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Attention-deficit hyperactivity disorder (ADHD/ADD) is a neurobehavioral disorder of childhood onset characterized by severe, developmentally inappropriate motor hyperactivity, inattention, and impulsiveness that result in impairment in more than one setting. It affects the home, school, and community life of 39% of school-going children worldwide. There is increasing recognition that ADHD symptoms and clinically defined disorder can persist into adult life and are associated with later drug and alcohol misuse and social and work difficulties. Added to that is the extreme variability of the disorder over time, within the same individual, between individuals, and across different circumstances. Treatment with stimulants and nonstimulants has proven effective in different subgroups, with the effectiveness of specific agents most likely related to the primary neurotransmitter involved. However, stimulants with a short duration of action have been problematic for some patients. Parent training and cognitive behavioral therapies represent the most widely adjunct psychosocial interventions to pharmacotherapy.

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Ophthalmoplegic migraine (OM) is characterized by recurrent attacks of headache with paresis of ocular cranial nerves. Previously, it was classified as a variant of migraine, but recently, International Headache Classification (IHCD-II) has reclassified OM to the category of neuralgia. Presently, OM is considered a type of recurrent demyelinating cranial neuropathy. We report an adolescent girl with OM, who had been treated with steroid and showed dramatic improvement.

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Joubert syndrome (JS) is a rare autosomal recessive disorder with key finding of cerebellar vermis hypoplasia with a complex brainstem malformation that comprises the molar tooth sign on axial magnetic resonance images. This syndrome is difficult to diagnose clinically because of its variable phenotype. The exact diagnosis is often not made for several years after birth. This report shows that with the availability of magnetic resonance imaging (MRI), especially in developing countries like India, it is quite feasible to make an early diagnosis which may positively affect the subsequent management and outcome. We present a case of JS in a 7-month-old girl who presented to the pediatric outpatient clinic with developmental delay and abnormal eye movements. MRI showed molar tooth configuration of superior cerebellar peduncles, the fourth ventricle shaped like a bat wing and hypoplasia of the vermis which resulted in median approach of the two cerebellar hemispheres.

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Acute disseminated encephalomyelitis (ADEM) is an inflammatory immune-mediated disorder which is more common in pediatric patients. The clinical setting is characterized by a rapid onset of encephalopathy and multifocal neurological features. Acute hemorrhagic encephalomyelitis (AHEM) is considered a rare form of ADEM. This report shows a 2-year-old patient who presented with the classical features of ADEM and after 8 weeks developed severe neurological worsening. The second magnetic resonance image (MRI) showed hemorrhagic lesions. Differences in prognosis between ADEM and AHEM justify the investigation of AHEM whenever a patient has neurological recrudescence in a known patient of ADEM.

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Hemifacial spasm is a disorder of the seventh cranial nerve, which is characterized by irregular, involuntary and recurrent tonic and clonic contractions of the ipsilateral facial expression muscles. This disorder affects mainly adults, and there are few cases reported in childhood. The main etiologies are vascular problems, although tumors are an important cause of hemifacial spasm via a direct or an indirect mass effect. We report a 6-year-old girl who presented with right hemifacial spasm. Magnetic resonance imaging showed a lesion in the cerebellopontine angle, extending from the midbrain to the pons with a slight mass effect on the fourth ventricle. The histological examination revealed a pilocytic astrocytoma.

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Megalencephalic leukoencephalopathy with subcortical cysts is an inherited autosomal recessive disorder with characteristic MRI features and a variable but mild clinical course. Frontal and temporal subcortical cysts are the diagnostic hallmark. It usually presents with pyramidal and cerebellar signs. Megalencephaly is usually detected early. Seizures may be present but are usually easily controlled. It has been reported commonly from a certain ethnicity of northern Indian origin, but its presence is global. We encountered four patients and describe the clinical and radiological features of these patients. Seizures though reported to be uncommon were seen in all our patients. Neuropsychiatric features have not been described as presentation so far but one of our patients had moderately severe depression. All the patients were diagnosed by MRI features and they responded well to symptomatic treatment.

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Esthesioneuroblastoma (ENB) presenting simultaneously with proptosis and bilateral neck metastasis is a very rare presentation. ENB is a rare tumor arising from the olfactory epithelium of the nasal vault which frequently invades the cranial base, cranial vault and orbit. ENB has a bimodal age distribution between 11 and 20 years and between 51 and 60 years. ENB accounts for approximately 1 to 5% of intranasal cancers and no consensus has been reached yet regarding the treatment of this tumor. We are reporting a 17-year-old male patient who presented with right eye proptosis with loss of vision and bilateral neck metastasis. Contrast enhanced computed tomography (CECT) and magnetic resonance imaging (MRI) was done which showed Kadish stage C tumor. Nasal biopsy was done to make the diagnosis and the patient was subjected to radiotherapy. Tumor responded to radiotherapy and both the primary lesion and the cervical lymph node metastasis disappeared. The purpose of this study is to report the rare presentation of proptosis along with bilateral cervical lymph node metastasis in this rare sinonasal tumor.

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The role of endoscopic third ventriculostomy (ETV) is getting more popular for all types of hydrocephalus. It has several advantages and is also being considered for malfunctioning of ventriculo-peritoneal shunt. A 16-year-old child had fourteen shunt revisions in his life. He was eventually treated with ETV with successful result. Repeated shunt failure can be an additional indication of ETV.

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Pneumocephalus is a rare condition, characterized by the presence of gas in the cranial cavity, resulting from trauma, tumors and surgical or diagnostic procedures. Intracranial infection, without any predisposing factor like trauma or surgical intervention as a cause of pneumocephalus is relatively uncommon. While, intracranial infections by gas producing organisms as a cause of pneumocephalus are well known, a spontaneous intracranial infection caused by Staphylococcus aureus causing pneumocephalus is little known. We report here a child who developed pneumocephalus following staphylococcal lung infection with meningitis and eventually showed complete recovery. Meningitis should be considered as a possible cause of pneumocephalus in absence of trauma and surgical intervention.

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Dysraphisms involving cervical region are very rare and there are very few series describing their follow-up in literature. Here, we report a 6-year-old boy who underwent postnatal "cosmetic" repair of posterior cervical cystic lesion and presented to us with a large recurrence with syringohydromyelia and tethering. Tethered cord should be suspected in the presence of meningocele and intact neurology. Treatment protocols of such complicated cervical spinal dysraphisms should include intradural exploration and detethering, with an aim to prevent neurological deterioration in future.

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The authors here have reported a rare case of a child with a complex spina bifida with two different levels of split cord malformation (SCM) type 1 and single level type 2, a non terminal myelocystocele, coccygeal dermal sinus, bifid fatty filum and hydrocephalus, which substantiates the neuroenteric canal theory and have further tried to highlight the importance of complete Magnetic resonance imaging (MRI) screening of the whole spine and brain with SCM to rule out other associated conditions. The patient was admitted with a leaking myelocystocele with bilateral lower limb weakness. MRI of whole spine with screening of brain was done. Patient underwent 5 operations in the same sitting- (According to classification given by Mahapatra et al.) removal of SCM type 1a at D7-8; removal of SCM type1c at L2-3; removal of SCM type 2 at D10; repair of non terminal myelocystocele at D6-D10; low pressure ventriculoperitoneal shunt on right side with excision of dermal coccygeal sinus; and, excision of bifid fatty filum. The clinicoradiological findings in our patient further substantiate the multiple accessory neuroenteric canal theory in the development of composite type of SCM. The physical and neurological signs of SCM and nonterminal myelocystocele should prompt the neurosurgeon to consider performing the screening MRI of whole spine with brain to rule out other composite types of SCM and hydrocephalus.

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Diabetes insipidus (DI) associated with a thickened pituitary stalk is a diagnostic challenge in the pediatric population. Langerhans Cell Histiocytosis (LCH) is a rare cause of this entity. A 4-year-old male child presented with central DI of 1-year duration, associated with a thickened pituitary stalk. The etiology for the same remained elusive as the patient had no other manifestation to suggest LCH. A year later, the patient developed a left frontal scalp swelling. Neuroradiology demonstrated multiple punched out osteolytic lesions in both the frontal bones. The infundibulum was thickened and showed post-contrast enhancement. Histology and immunohistochemistry (IHC) of the biopsy specimen confirmed LCH. The child was administered chemotherapy according to LCH protocol, which resulted in 33% reduction in the size of the skull lesions. The DI was controlled with medical management. The present case highlights the need for serial follow-up and magnetic resonance (MR) imaging that led to a diagnosis of LCH. The clinical presentation and management of central DI and a thickened pituitary stalk is presented and the relevant literature is discussed.

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Neurofibromatosis is one of the phakomatosis (neurodermatoses) which are genetically determined hamartomatous or neoplastic diseases of the skin and nervous system. It is not a single entity, but a group of heterogeneous multisystemic neurocutaneous disorders involving both neuroectodermal and mesenchymal derivatives. Plexiform neurofibroma, a histopathologic variant of neurofibroma, is a hallmark of neurofibromatosis type I (NF-I). It is pathognomonic of NF-I and may be the first sign of neurofibromatosis. It often appears within the first 2 years of life or before adolescence and occurs in only 5% of NF-I cases. We present a case of intraosseous plexiform neurofibroma of the maxilla in a 2-year old female, which is rare, along with the oral manifestations and clinicopathological characteristics. Because NF-I is one of the most common genetic disorders and oral manifestations are common, knowledge of the variability of presentation in children is necessary for prompt diagnosis.

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Infantile hemiplegia is one of the clinical forms of cerebral palsy that refers to impaired motor function of one half of the body owing to contralateral brain damage due to prenatal, perinatal and postnatal causes amongst which vascular lesion is the most common causative factor. We report here the effects of constraint-induced movement therapy in a five-year-old female child with infantile hemiplegia on improvement of upper extremity motor skills.

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Vein of Galen Aneurysm malformation is one of the most difficult intracranial vascular lesions to manage. The difficulty in management of its shunts is aggravated by the hemodynamic effect and shunting reflected in the growing brain of infants and children. At present, the therapeutic options are widened by the opening of a new horizon in Intervention Neuroradiology. We present a series of two case reports of these malformations treated using the endovascular method, at our institution.

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Ewing's sarcoma causing unilateral proptosis along with bifrontal extradural infiltration in a child is an unusual presentation. A female patient presented with features of painless proptosis of the left eye with visual deterioration. Her radiology revealed an infiltrating intraorbital, extraconal tumor with intracranial bifrontal extradural extension causing mass effect. Total excision of the intraorbital and intracranial part of the tumor along with postoperative chemo- and radiotherapy brought about a substantial relief. The clinicoradiological presentation and management of this rare entity are discussed.

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Background: Glioma is a deadly neurological tumor. For modern management of glioma, glioma vaccinotherapy is the new concept. Materials and Methods: Based on present biomedical technique, the identification of T-cell epitopes via MHC mapping can help clarify the inter-relationship of tumor and immune system. This process can be performed using advanced immunoinformatics technique. Results: Here, the author performs an immunoinformatics analysis to find alternative epitopes for glioma-related antigen, EPHA2. Conclusion: After complete manipulation on EPHA2 molecules, the five best epitopes were derived.

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Epilepsy is the most common neurological disease worldwide and is second only to stroke in causing neurological morbidity. Neuroimaging plays a very important role in the diagnosis and treatment of patients with epilepsy. This review article highlights the specific role of various imaging modalities in patients with epilepsy, and their practical applications in the management of epileptic patients.

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