Spontaneous "pneumo-apoplexy" as a presentation of pituitary adenoma.

Cases of spontaneous CSF leak associated with pituitary tumor apoplexy are uncommon in the literature with pneumocephalus or pneumosella being rare, especially spontaneous occurrence of pneumocephalus being extremely rare. We present a case of pituitary macroadenoma apoplexy resulting in spontaneous CSF leak and a large volume of intra-tumoral gas. A 65-year-old female presented with severe headache, profuse rhinorrhea and acute vision loss and was found to have a large sellar and suprasellar lesion with air and hemorrhage with mild peripheral enhancement. The patient underwent trans-sphenoidal tumor resection and repair of skull base for CSF leak with nasoseptal flap placement. Pathology demonstrated a pituitary adenoma exhibiting immunoreactivity for ACTH. To our best knowledge, our case is the first report of features of pituitary tumor apoplexy with hemorrhage and pneumosella, which could be summarized by the term "pneumo-apoplexy".

Practical anatomy of the central skull base region.

The central skull base region represents a complex intersection between the intracranial compartment, the osseous foundation of the skull base, the orbits, the paranasal sinuses, and the suprahyoid neck. A modern radiologic approach to this region should take into account the 3-dimensional complexity of the region as well as the cross-sectional anatomical detail available to today's radiologist. This analytical approach should permit identification of lesional anatomical subsites, establishment of lesional origins, and allow for an anatomy-based differential diagnosis. In this article, we define a practical central skull base region that includes structures that directly affect neuroimaging of this dense landscape. By reframing the boundaries, the central skull base region becomes comprehensive, emphasizing the natural tendency for pathologic processes to involve contiguous anatomical subunits, and underscores the complexity and challenges of this region for neuroimaging specialists.

Lymphopenia in the BB rat model of type 1 diabetes is due to a mutation in a novel immune-associated nucleotide (Ian)-related gene.

The BB (BioBreeding) rat is one of the best models of spontaneous autoimmune diabetes and is used to study non-MHC loci contributing to Type 1 diabetes. Type 1 diabetes in the diabetes-prone BB (BBDP) rat is polygenic, dependent upon mutations at several loci. Iddm1, on chromosome 4, is responsible for a lymphopenia (lyp) phenotype and is essential to diabetes. In this study, we report the positional cloning of the Iddm1/lyp locus. We show that lymphopenia is due to a frameshift deletion in a novel member (Ian5) of the Immune-Associated Nucleotide (IAN)-related gene family, resulting in truncation of a significant portion of the protein. This mutation was absent in 37 other inbred rat strains that are nonlymphopenic and nondiabetic. The IAN gene family, lying within a tight cluster on rat chromosome 4, mouse chromosome 6, and human chromosome 7, is poorly characterized. Some members of the family have been shown to be expressed in mature T cells and switched on during thymic T-cell development, suggesting that Ian5 may be a key factor in T-cell development. The lymphopenia mutation may thus be useful not only to elucidate Type 1 diabetes, but also in the function of the Ian gene family as a whole.