Hereditary neurological tumor syndromes: clues to glioma oncogenesis?

Hereditary Neuroendocrine Tumor Syndromes

Neuroendocrine tumours (NETs) are rare and heterogeneous neoplasms with variable bio‐ logical behaviour. The estimated incidence of NETs is about 1-5 cases/100,000/year. The most recent data show a progressive increase of the incidence in the last years and a high increase of their prevalence and survival [1]. NETs can be sporadic or can arise in complex hereditary endocrine disorders such as M...

Spectrum of neurological syndromes associated with glutamic acid decarboxylase antibodies: diagnostic clues for this association.

The association of high levels of autoantibodies to glutamic acid decarboxylase (GAD-ab) and stiff-person syndrome (SPS) is well known. However, the full spectrum of neurological syndromes associated with GAD-ab is not well established. In addition, these patients usually present type 1 diabetes mellitus (DM1) that could justify the presence of high GAD-ab levels. To clarify these issues, we re...

Introduction to oncogenesis by RNA tumor viruses.

Pediatric hereditary autoinflammatory syndromes.

OBJECTIVE To describe the most prevalent pediatric hereditary autoinflammatory syndromes. SOURCES A review of the literature including relevant references from the PubMed and SciELO was carried out using the keywords autoinflammatory syndromes and child. SUMMARY OF THE FINDINGS The hereditary autoinflammatory syndromes are caused by monogenic defects of innate immunity and are classified as...

Hereditary periodic fever syndromes.

The hereditary periodic fevers are a group of Mendelian disorders characterized by seemingly unprovoked fever and localized inflammation. Recent data indicate that these illnesses represent inborn errors in the regulation of innate immunity. Pyrin, the protein mutated in familial Mediterranean fever, defines an N-terminal domain found in a large family of proteins involved in inflammation and a...