True aneurysm on the posterior meningeal artery associated with a dural arteriovenous fistula: case report.

Background: We report an unusual case of a true dural aneurysm arising from the posterior meningeal artery that fed a symptomatic dural arteriovenous fistula located at the right transverse-sigmoid sinus junction.

Clinical Presentation: A 29-year-old right-handed white woman presented with aneurysmal dilatation of hypertrophied posterior meningeal artery feeding a partially treated dural arteriovenous fistula.

Intervention: The aneurysm, which measured approximately 3 mm in width and 5 mm in length, was located in the intracranial space with a thin-walled dome projecting toward the cerebellum.

Rupture of cerebral myxomatous aneurysm months after resection of the primary cardiac tumor.

Background: The natural history of cerebral aneurysms derived from metastatic spread of cardiac myxomas is not well known, and their management presents many dilemmas.

Methods: Case report and literature review.

Results: An 18-year-old man presented with an intraparenchymal hemorrhage several months after resection of an atrial myxoma.

Bing-Neel syndrome: an illustrative case and a comprehensive review of the published literature.

Waldenstrom's macroglobulinemia (WM) is a chronic lymphoproliferative disorder within the spectrum of lymphoplasmacytic lymphoma characterized by proliferation of plasma cells, small lymphocytes, and plasmacytoid lymphocytes. Central nervous system involvement is very rare (Bing-Neel [BN] syndrome). We present the case of a 62-year-old woman previously diagnosed with WM who presented with Bing-Neel syndrome and review the published literature which consists of only case reports.

Response of an adult patient with pineoblastoma to vorinostat and retinoic acid.

We report the case of an adult patient with pineoblastoma (PBL) who had a complete radiographic response following treatment with vorinostat and retinoic acid. This regimen was used to treat bulky residual tumor that persisted despite radiation therapy (RT) and two cycles of cytotoxic chemotherapy. Vorinostat and retinoic acid were chosen as an alternative to cytotoxic chemotherapy, which our patient was unable to tolerate, based on preclinical data suggesting efficacy of this combination.

High-grade dural arteriovenous fistula simulating a bilateral thalamic neoplasm.

Dural arteriovenous fistulae (dAVF) provide a diagnostic challenge and must be part of a broad differential in pursuit of a difficult diagnosis or unusual presentation. This case report demonstrates an initially misguided diagnosis of bilateral thalamic neoplasm and demonstrates the importance of continued pursuit until the correct diagnosis is obtained. Moreover, to our knowledge, this is the first reported case of a dAVF simulating a bilateral thalamic neoplasm.

Progressive multifocal leukoencephalopathy after rituximab therapy in HIV-negative patients: a report of 57 cases from the Research on Adverse Drug Events and Reports project.

Rituximab improves outcomes for persons with lymphoproliferative disorders and is increasingly used to treat immune-mediated illnesses. Recent reports describe 2 patients with systemic lupus erythematosus and 1 with rheumatoid arthritis who developed progressive multifocal leukoencephalopathy (PML) after rituximab treatment. We reviewed PML case descriptions among patients treated with rituximab from the Food and Drug Administration, the manufacturer, physicians, and a literature review from 1997 to 2008.

Phosphorylation of beta-amyloid precursor protein (APP) cytoplasmic tail facilitates amyloidogenic processing during apoptosis.

Secretion and progressive cerebral accumulation of beta-amyloid peptides (Abeta) derived by endoproteolytic ("amyloidogenic") processing of beta-amyloid precursor protein (APP) represent collectively an early and necessary event in the pathogenesis of Alzheimer's disease. We previously demonstrated that secretion of the neurotoxic species Abeta42 increases during staurosporine-induced apoptosis in undifferentiated PC12 cells, in an endocytosis-dependent manner. In the present study, we tested whether phosphorylation of the APP cytoplasmic-tail is contributory to this apoptosis-related increased Abeta-secretory response.

The endocytotic pathway is required for increased A beta 42 secretion during apoptosis.

Secretion and progressive cerebral accumulation of beta-amyloid peptides (A beta), which derive by endoproteolytic ('amyloidogenic') processing of beta-amyloid precursor protein (APP), are felt to represent collectively an early and necessary event in the pathogenesis of Alzheimer's disease. APP amyloidogenic processing can occur via secretory or endocytotic pathways, but the relative contribution of these pathways to A beta secretion remains to be established. The effect of apoptosis on amyloidogenic processing and A beta secretion similarly is incompletely understood.

Differential expression of cholesterol hydroxylases in Alzheimer's disease.

Cholesterol is eliminated from neurons by oxidization, which generates oxysterols. Cholesterol oxidation is mediated by the enzymes cholesterol 24-hydroxylase (CYP46A1) and cholesterol 27-hydroxylase (CYP27A1). Immunocytochemical studies show that CYP46A1 and CYP27A1 are expressed in neurons and some astrocytes in the normal brain, and CYP27A1 is present in oligodendrocytes.

Surgical management of a ruptured posterior choroidal intraventricular aneurysm associated with moyamoya disease using frameless stereotaxy: case report and review of the literature.

Objective And Importance: Prevention of rebleeding is the most important aspect of the management of hemorrhagic moyamoya disease, because rebleeding causes significant morbidity and mortality.

Clinical Presentation: A 26-year-old male patient with a history of moyamoya disease since the age of 3 years and multiple strokes was in a semicomatose state at presentation. He was found to have intraventricular and periventricular hemorrhages abutting the atrium of the right ventricle.

Presenilins mutated at Asp-257 or Asp-385 restore Pen-2 expression and Nicastrin glycosylation but remain catalytically inactive in the absence of wild type Presenilin.

The Presenilins are part of the gamma-secretase complex that is involved in the regulated intramembrane proteolysis of amyloid precursor protein and other type I integral membrane proteins. Nicastrin, Pen-2, and Aph1 are the other proteins of this complex. The Presenilins probably contribute the catalytic activity to the protease complex.