Primary choriocarcinoma of the bilateral basal ganglia presenting in a teenaged male.

Primary intracranial choriocarcinoma (PICCC), a type of germ-cell tumor, is a very rare primary tumor of the central nervous system that generally arises in the pineal or suprasellar region. We present a case of a teenage boy with PICCC of the bilateral basal ganglia, an anatomic site for which we were unable to find the previous reports. We offer discussion of the differential diagnosis, imaging characteristics, and prognosis of PICCC and germ-cell tumors of the basal ganglia, in the hope that it will increase awareness and allow for early detection.

Temporal bone and cranial nerve findings in pontine tegmental cap dysplasia.

INTRODUCTION: Pontine tegmental cap dysplasia (PTCD) is a recently described brain malformation associated with multiple cranial neuropathies, most commonly congenital sensorineural hearing loss. The purpose of this study is to systematically characterize the cranial nerve and temporal bone findings in a cohort of children with this rare condition. METHODS: Sixteen patients with PTCD and diagnostic quality imaging were retrospectively reviewed. All patients had high-resolution MR of the brain and/or internal auditory canals, and seven patients had additional high-resolution CT of the temporal bones. Studies were evaluated by two pediatric neuroradiologists for cranial nerve and temporal bone anomalies. RESULTS: Fifteen of 16 patients (94%) had duplication of one or both internal auditory canals. Of the 24 total duplicated internal auditory canals, all 24 (100%) demonstrated stenosis or atresia of the vestibulocochlear nerve canal, as well as ipsilateral vestibulocochlear nerve aplasia. Of the non-duplicated internal auditory canals, 63% (5/8) were atretic or stenotic. Thirty-eight percent (3/8) were associated with absent vestibulocochlear nerve, and 38% (3/8) demonstrated isolated cochlear nerve aplasia. Twenty-five percent (2/8) demonstrated normal vestibulocochlear nerves, both in the same patient. Fifteen of 16 patients overall (94%) demonstrated bilateral cochlear nerve aplasia. Of the 32 total temporal bones, 4 (13%) demonstrated facial nerve aplasia. Seventy-nine percent (22/28) of facial nerves that were present demonstrated an aberrant origin or course. CONCLUSION: Patients with PTCD have highly characteristic temporal bone and cranial nerve findings on both CT and MR. Recognition of these findings is important for improved diagnosis of this rare disorder, particularly by CT.

Absent cavum septum pellucidum: a review with emphasis on associated commissural abnormalities.

The cavum septum pellucidum (CSP) is an important fetal midline forebrain landmark, and its absence often signifies additional underlying malformations. Frequently detected by prenatal sonography, absence of the CSP requires further imaging with pre- or postnatal MRI to characterize the accompanying abnormalities. This article reviews the developmental anatomy of the CSP and the pivotal role of commissurization in normal development. An understanding of the patterns of commissural abnormalities associated with absence of the CSP can lead to improved characterization of the underlying spectrum of pathology.

Brachial plexus birth palsy: multimodality imaging of spine and shoulder abnormalities in children.

OBJECTIVE. The purpose of this article is to provide a comprehensive overview of the imaging of brachial plexus palsy, including both pathologic conditions of the spine and shoulder and clinical background and management. CONCLUSION. Brachial plexus birth palsy can result in permanent disability and limb deformity. Identifying the lesion type and associated sequelae is important in clinical management aimed at optimizing outcome. The imaging algorithms used are guided by clinical presentation and are designed to assess the extent of injury to guide possible surgical intervention.

Diagnostic imaging of posterior fossa anomalies in the fetus and neonate: part 2, Posterior fossa disorders.

This second portion of a two-part review illustrates examples of posterior fossa disorders detectable on prenatal ultrasound and MRI, with postnatal or pathology correlation where available. These disorders are discussed in the context of an anatomic classification scheme described in Part 1 of this posterior fossa anomaly review. Assessment of the size and formation of the cerebellar hemispheres and vermis is critical. Diagnoses discussed here include arachnoid cyst, Blake's pouch cyst, Dandy-Walker malformation, vermian agenesis, Joubert syndrome, rhombencephalosynapsis, Chiari II malformation, ischemia, and tumors.

Diagnostic imaging of posterior fossa anomalies in the fetus and neonate: part 1, normal anatomy and classification of anomalies.

This article is the first portion of a two-part review that illustrates the normal appearance of the cerebellum and posterior fossa on prenatal ultrasound and MRI and on postnatal diagnostic imaging studies. Classification and terminology of posterior fossa abnormalities in the literature are confusing due to evolution of concepts and sometimes lack of consensus. Accurate classification of posterior fossa anomalies is important for predicting fetal outcome and for appropriate counseling. In Part 1 of this review, prenatal and postnatal imaging techniques for assessing the posterior fossa will be discussed, followed by a discussion of how cerebellar malformations may be classified.

Imaging of pediatric renal transplants and their complications: a pictorial review.

Renal transplantation is the treatment of choice for end-stage renal disease in children. As a technically demanding surgery with complex medical management, it is associated with a number of complications. Anatomic imaging including ultrasonography with color and spectral Doppler and functional assessment with renal perfusion scintigraphy are complementary for the detection and characterization of posttransplant complications. Complications can be characterized by the time of appearance after transplantation (immediate, early, or late) or the anatomic site of origin (perinephric, vascular, urologic, or renal parenchymal). Perinephric fluid collections include hematomas and seromas, abscesses, lymphoceles, and urinomas. Noninfected collections frequently resolve spontaneously but should be monitored to exclude progression. Vascular complications are more prevalent in pediatric patients because of the small vessel caliber and include vascular thrombosis and stenosis. Arteriovenous fistulas and pseudoaneurysms can complicate biopsy and are typically transient. Common urologic complications include urine leak and urinary tract obstruction. Renal perfusion scintigraphy can be invaluable in elucidating the nature of such complications. Renal parenchymal abnormalities include acute tubular necrosis, rejection, and toxic effects of medication. Imaging features of renal parenchymal abnormalities can overlap, and the primary role of imaging is to exclude alternative causes of renal dysfunction. Renal and nonrenal mass lesions are more common in immunosuppressed patients after transplantation. Familiarity with the normal imaging appearance of the renal allograft and the appearances of common complications facilitates accurate diagnosis and timely treatment, with the ultimate goal of increasing graft survival. This goal is particularly crucial in children, given their greater number of projected life years.

Presyrinx in a child with acquired Chiari I malformation.

Spinal cord signal abnormality resulting from alterations in cerebrospinal fluid flow at the craniocervical junction has been termed a presyrinx state. This condition has been described in the adult literature in association with a variety of conditions that cause obstruction to normal cerebrospinal fluid flow. We present a case of presyrinx in a child in the setting of acquired Chiari I malformation caused by lumboperitoneal overshunting. Awareness of the potentially reversible nature of this condition might allow for intervention before irreversible neurological damage has occurred.

Safety and immunogenicity of an AMA-1 malaria vaccine in Malian adults: results of a phase 1 randomized controlled trial.

BACKGROUND: The objective was to evaluate the safety, reactogenicity and immunogenicity of the AMA-1-based blood-stage malaria vaccine FMP2.1/AS02A in adults exposed to seasonal malaria. METHODOLOGY/PRINCIPAL FINDINGS: A phase 1 double blind randomized controlled dose escalation trial was conducted in Bandiagara, Mali, West Africa, a rural town with intense seasonal transmission of Plasmodium falciparum malaria. The malaria vaccine FMP2.1/AS02A is a recombinant protein (FMP2.1) based on apical membrane antigen-1 (AMA-1) from the 3D7 clone of P. falciparum, adjuvanted with AS02A. The comparator vaccine was a cell-culture rabies virus vaccine (RabAvert). Sixty healthy, malaria-experienced adults aged 18-55 y were recruited into 2 cohorts and randomized to receive either a half dose or full dose of the malaria vaccine (FMP2.1 25 microg/AS02A 0.25 mL or FMP2.1 50 microg/AS02A 0.5 mL) or rabies vaccine given in 3 doses at 0, 1 and 2 mo, and were followed for 1 y. Solicited symptoms were assessed for 7 d and unsolicited symptoms for 30 d after each vaccination. Serious adverse events were assessed throughout the study. Titers of anti-AMA-1 antibodies were measured by ELISA and P. falciparum growth inhibition assays were performed on sera collected at pre- and post-vaccination time points. Transient local pain and swelling were common and more frequent in both malaria vaccine dosage groups than in the comparator group. Anti-AMA-1 antibodies increased significantly in both malaria vaccine groups, peaking at nearly 5-fold and more than 6-fold higher than baseline in the half-dose and full-dose groups, respectively. CONCLUSION/SIGNIFICANCE: The FMP2.1/AS02A vaccine had a good safety profile, was well-tolerated, and was highly immunogenic in malaria-exposed adults. This malaria vaccine is being evaluated in Phase 1 and 2 trials in children at this site.