An Unusual Cause of Chronic Headache in an Adolescent Boy: A Case Report.

Spontaneous intracranial hypotension (SIH) is an under-diagnosed cause of headache in children and adolescents. SIH results from cerebrospinal fluid (CSF) leak due to breach in the dura mater and the etiology for dural breach is often diverse. We report an adolescent boy who presented with chronic episodic headache that later progressed to daily headache. There was a typical history of worsening of headache on upright position and relief of headache on lying down. He was treated with migraine prophylaxis in another hospital but there was no response. Marfanoid features and brisk deep tendon reflexes were observed on clinical examination. Brain magnetic resonance imaging (MRI) revealed sagging of the brain stem, pachymeningeal enhancement, and tonsillar herniation. MRI of spine myelogram confirmed multiple levels of CSF leak. He was initially managed with supportive measures and fluoroscopic-guided fibrin glue injection. Although child remained symptom-free for the next 6 months, he again developed headache. MRI and computed tomography spine myelogram revealed a meningeal diverticulum in the lumbar spine. He was managed with an autologous epidural blood patch and he has been well since then. In this report, we highlight the clinical and radiological pointers to the presence of SIH in children with recurrent headache.

Reliability of Imaging-Based Diagnosis of Lateral Ventricular Masses in Children.

OBJECTIVE: We studied the accuracy of the radiologic diagnosis of lateral ventricular masses in children (<20 years of age). METHODS: In this retrospective study, data were collected from children with lateral ventricular masses managed in our unit between 2001 and 2016. There were 43 patients (26 boys and 17 girls; mean age, 12.1 years; range, 8 months to 20 years). Magnetic resonance imaging was available for 36 patients, whereas in 7 patients only a contrast-enhanced computed tomography scan was available. The images were read independently by 3 radiologists, who were blinded to the pathology. Two differential diagnoses were offered for each patient. The agreement between the 3 radiologists was calculated using the Fleiss κ statistic. RESULTS: The common pathologic diagnoses were subependymal giant cell astrocytoma (SEGA) (20.9%), low-grade astrocytoma (16.3%), high-grade astrocytoma (9.3%), choroid plexus papilloma (9.3%), and meningioma (9.3%). The sensitivity, specificity, and accuracy of the radiologic diagnoses were 62%, 96.7%, and 94.2%, respectively. Low-grade tumors such as low-grade gliomas, SEGAs, choroid plexus papillomas, and meningiomas were diagnosed with a high level of accuracy. High-grade gliomas, choroid plexus carcinomas, and other malignant neoplasms were difficult to diagnose on imaging. Cavernous angiomas were also difficult to diagnose. There was only fair agreement between the 3 radiologists (Fleiss κ = 0.24). CONCLUSIONS: The pathologic spectrum of lateral ventricular tumors in children is wide, and identifying the pathology on imaging is difficult for malignant tumors. Benign tumors such as SEGAs, low-grade astrocytomas, and choroid plexus papillomas are relatively easier to diagnose. There is significant interobserver variability in the radiologic diagnosis of these tumors.

Management and Outcome in Patients with Advanced Juvenile Nasopharyngeal Angiofibroma.

Objective To report the management outcome in a series of patients with advanced juvenile nasopharyngeal angiofibroma (JNA). Design Retrospective study. Setting Tertiary care teaching hospital. Participants Forty-five patients classified as Radkowski stage IIIA or IIIB who presented to us over the past 10 years. Main Outcome Measures Surgical approaches used and disease free outcomes in patients with advanced JNA. Results Surgical access for the extracranial component included open (41.9%) and expanded endonasal approaches (58.1%). Craniotomy (16.3%), endoscopy-assisted open approach (7%), or expanded endonasal approach (20.9%) was performed to excise the skull base or intracranial component. Follow up ranged from 4 to 96 months (mean, 20.3 months). Of 35 patients who underwent imaging at the first postoperative follow up, 25 (71.4%) had negative scans. Three symptomatic patients with residual disease underwent endoscopic excision and had negative scans thereafter. Of two others who had radiation therapy, one was disease free and the other lost to follow up. Five others had stable, residual disease. Three patients (8.6%) with recurrent disease underwent surgical excision, of whom two had minimal, stable residual disease. At the last follow-up, 27 (77.1%) patients had negative scans, and 7 (20%) had stable residual disease with one (2.9%) patient lost to follow-up. Conclusions Advanced JNA may be successfully treated in most cases with expanded endonasal/endoscopy assisted ± craniotomy approach after appropriate preoperative evaluation. At follow-up, only symptomatic patients or those with enlarging residue require treatment; periodic imaging surveillance is adequate for those with stable disease.

Role of Noninvasive Imaging of Cerebral Arterial System in Ischemic Stroke: Comparison of Transcranial Color-coded Doppler Sonography with Magnetic Resonance Angiography.

AIM: To determine the accuracy of transcranial color-coded Doppler sonography (TCCS) in the evaluation of cerebral arterial system in patients with ischemic stroke attending a tertiary care hospital in South India. OBJECTIVES: (1) To describe the topographical distribution of atherosclerotic lesions in the cerebral circulation in patients presenting with ischemic stroke from the Indian subcontinent and (2) to determine the accuracy of TCCS for detection and quantification of intracranial stenoses in various segments of the intracerebral arterial system in comparison with magnetic resonance angiography (MRA). MATERIALS AND METHODS: The demographic profile and risk factors of consecutive patients who presented to neurology outpatient department with cerebral ischemia and scheduled for MRA were determined. These patients had undergone neck Doppler, TCCS, and MRA. The agreement between the MRA and TCCS was assessed using kappa statistics. The sensitivity, specificity, and positive and negative predictive values of TCCS as compared to MRA were calculated. RESULTS: Ninety patients were included in the final analysis. Intracranial atherosclerosis was found in 35.6% of cases. The agreement between TCCS and MRA in detecting lesions for the different arterial segments in the intracranial circulation was 0.83 for anterior cerebral artery (ACA), 0.66 for M1 segment of middle cerebral artery (MCA), 0.45 for M2 segment of MCA, 0.86 for terminal internal carotid artery (TICA), 0.46 for posterior cerebral artery (PCA), and 0.81 for vertebral artery (VA). The sensitivity for the detection of hemodynamically significant arterial lesions in different vascular segments was 100%, 70%, 33.3%, 90.9%, 33.3%, and 72.7% for ACA, M1, M2, TICA, PCA, and VA, respectively. CONCLUSION: Intracranial atherosclerosis was found to be the predominant distribution of cerebral atherosclerosis. TCCS is a safe method for evaluation of proximal basal cerebral arteries in the intracranial circulation with relatively better sensitivity in the anterior circulation.

Pediatric Balint's Syndrome Variant: A Possible Diagnosis in Children.

Balint's syndrome is well described in adults, but not in children. It is caused by bilateral posterior parietal lobe damage and comprises a triad of simultanagnosia (inability to simultaneously see more than a small number of items), optic ataxia (impaired visual guidance of movement of the limbs and body), and apraxia of gaze (inability to volitionally direct gaze despite the requisite motor substrate) often associated with homonymous lower visual field loss. We, here, describe five children (four males, one female; mean age 7.4 years, [range 4-11 years]; birth weight ≤ 2.5 kg; four were born ≤ 36 weeks of gestational age and one at 40 weeks) who presented to the Cerebral Visual Impairment Clinic at a tertiary care center in South India with clinical features remarkably consistent with the above description. In all children neuroimaging showed bilateral parietooccipital gliosis with regional white matter volume loss and focal callosal thinning, consistent with perinatal hypoxic ischemic encephalopathy and possible neonatal hypoglycemia.

Proximal Interruption of the Pulmonary Artery: A Case Series.

We present a few cases of Proximal Interruption of the Pulmonary Artery, an uncommon developmental anomaly associated with congenital heart disease. The cases had varied clinical presentations. Chest radiograph showed a hypoplastic lung with an ipsilateral small hilum on the side of the interruption and hyperinflation of the contralateral lung. Contrast CT confirmed the diagnosis, demonstrating non-visualization of the left or right pulmonary artery, and other related findings.

Tuberculosis--the great mimicker.

Tuberculosis is an immense health problem in the developing world, and it remains a health care challenge in the developed world. It can affect virtually any organ system in the body. Diagnosis of tuberculosis is often difficult. Many patients with tuberculosis present with nonspecific symptoms, negative purified protein derivative skin test result, and negative findings on culture specimens. Cross-sectional imaging with ultrasound, multidetector computed tomography, and magnetic resonance imaging plays an important role in the diagnosis of tuberculosis. Tuberculosis demonstrates a variety of radiologic features depending on the organ involved and can mimic a number of other disease entities. Cross-sectional imaging alone is insufficient in reaching a conclusive diagnosis. Tuberculosis is a great mimicker as its radiologic manifestations can simulate numerous other diseases across the body systems. However, recognition and understanding of the common and uncommon radiologic manifestations of tuberculosis should alert considering tuberculosis in the high-risk population and correct clinical setting to enable appropriate treatment.

Leflunomide with low-dose everolimus for treatment of Kaposi's sarcoma in a renal allograft recipient.

Current treatment of Kaposi's sarcoma is reduction of immunosuppression with or without addition of mammalian target of rapamycin inhibitors (mTORi). Akt signalling plays a central role in oncogenesis of Kaposi's sarcoma. We describe a case of multifocal Kaposi's sarcoma in a renal allograft recipient, which showed unsatisfactory early response to immunosuppression reduction along with everolimus therapy but completely resolved after adding leflunomide. mTORi impair Kaposi's sarcoma oncogenesis by inhibiting mTOR downstream from the Akt signalling. Leflunomide inhibits Akt phosphorylation. This synergistic effect may be beneficial in treatment of Kaposi sarcoma and needs to be explored in trials.