Idiopathic chiasmal neuritis: clinical features and prognosis.

OBJECTIVES: To describe the clinical features of idiopathic chiasmal neuritis in a large cohort of patients and to report their visual and neurologic outcomes. DESIGN: A retrospective medical record review of consecutive patients with chiasmal neuritis at a single institution. Patients with clinical or radiographic evidence of inflammation involving the intraorbital optic nerve and patients with a systemic inflammatory or neoplastic disorder were excluded. RESULTS: Twenty patients were identified (14 female, 6 male; mean age, 37 years). Visual acuity at initial examination ranged from 20/15 to light perception. Progressive visual loss beyond 1 month was documented in 1 patient. Twelve of 15 patients who underwent magnetic resonance imaging demonstrated chiasmal enlargement and/or enhancement; 6 patients had 1 or more white matter lesions. Follow-up time ranged from 2 weeks to 22 years, with a mean of 5.7 years. The final median visual acuity was 20/20 (range, 20/15-20/50) and visual fields were normal or improved. Of 15 patients with a minimum follow-up interval of 1 year, 6 developed multiple sclerosis. CONCLUSIONS: The demographic and clinical features of idiopathic chiasmal neuritis resemble those of idiopathic optic neuritis. Visual prognosis is excellent. In this series, 40% of patients subsequently developed multiple sclerosis.

Current features of infective endocarditis in elderly patients: results of the International Collaboration on Endocarditis Prospective Cohort Study.

BACKGROUND: Elderly patients are emerging as a population at high risk for infective endocarditis (IE). However, adequately sized prospective studies on the features of IE in elderly patients are lacking. METHODS: In this multinational, prospective, observational cohort study within the International Collaboration on Endocarditis, 2759 consecutive patients were enrolled from June 15, 2000, to December 1, 2005; 1056 patients with IE 65 years or older were compared with 1703 patients younger than 65 years. Risk factors, predisposing conditions, origin, clinical features, course, and outcome of IE were comprehensively analyzed. RESULTS: Elderly patients reported more frequently a hospitalization or an invasive procedure before IE onset. Diabetes mellitus and genitourinary and gastrointestinal cancer were the major predisposing conditions. Blood culture yield was higher among elderly patients with IE. The leading causative organism was Staphylococcus aureus, with a higher rate of methicillin resistance. Streptococcus bovis and enterococci were also significantly more prevalent. The clinical presentation of elderly patients with IE was remarkable for lower rates of embolism, immune-mediated phenomena, or septic complications. At both echocardiography and surgery, fewer vegetations and more abscesses were found, and the gain in the diagnostic yield of transesophageal echocardiography was significantly larger. Significantly fewer elderly patients underwent cardiac surgery (38.9% vs 53.5%; P < .001). Elderly patients with IE showed a higher rate of in-hospital death (24.9% vs 12.8%; P < .001), and age older than 65 years was an independent predictor of mortality. CONCLUSIONS: In this large prospective study, increasing age emerges as a major determinant of the clinical characteristics of IE. Lower rates of surgical treatment and high mortality are the most prominent features of elderly patients with IE. Efforts should be made to prevent health care-associated acquisition and improve outcomes in this major subgroup of patients with IE.

Non-classical karyotypic features in relapsed childhood B-cell precursor acute lymphoblastic leukemia.

Karyotype analysis of acute lymphoblastic leukemia (ALL) at diagnosis has provided valuable prognostic markers for treatment stratification. However, reports of cytogenetic studies of relapsed ALL samples are limited. We compared the karyotypes from 436 nonselected B-cell precursor ALL patients at initial diagnosis and of 76 patients at first relapse. We noticed a relative increase of karyotypes that did not fall into the classic ALL cytogenetic subgroups (high hyperdiploidy, t(12;21), t(9;22), 11q23, t(1;19), <45 chromosomes) in a group of 29 patients at relapse (38%) compared to 130 patients at presentation (30%). Non-classical cytogenetic aberrations in these 29 patients were mostly found on chromosomes 1, 2, 7, 9, 13, 14, and 17. We also describe six rare reciprocal translocations, three of which involved 14q32. The most frequent abnormalities were found in 9p (12/29 cases) and were associated with a marked decrease in the duration of the second remission, but not of the probability of 10-year event-free survival after relapse treatment. From 29 patients with non-classical cytogenetic aberrations, only 8 (28%) had been stratified to a high risk-arm on the first treatment protocol, suggesting that this subgroup might benefit from the identification of new prognostic markers in future studies.

Mesenteric venous thrombosis: MDCT features according to the underlying etiology [C-463]

Purpose: To work out certain, well‑defined aetiologies frequently associated with mesenteric venous thrombosis (MVT) in order to predict a typical population at risk, since MVT is nowadays often incidentally detected on cross‑sectional imaging. To demonstrate the MDCT features, frequency and extent of associated bowel ischemia according to the underlying pathology. Methods and Materials: Our electronic database revealed 71 patients (25 women, mean age 55) with thrombosis of the superior and/or inferior mesenteric vein detected by MDCT between 2000 and 2008. Two radiologists jointly reviewed the corresponding MDCT features including intraluminal extension, underlying aetiology and associated bowel ischemia, if present. Results: MVT was associated with carcinoma in 31 (43.7%) patients (pancreas 21.1%, liver 9.9%, others 12.7%). Concomitant inflammation was seen in 15 (21.1%) patients (pancreatitis 11.3%, diverticulitis 4.2%, others 5.6%), whereas coagulation/hematologic disorders were found in 7 (9.9%) patients, liver cirrhosis in 6 (8.5%), mixed/miscellaneous causes in 5 (7%) and still unknown aetiologies in 5 patients (7%). MVT resulted from recent operations in 2 (2.8%) patients. MDCT features of venous bowel ischemia were present in 15 patients (21.1%). 46.5% of MVT were (sub)acute, while 53.5% chronic. The luminal extension was complete in 52.1%, subtotal (50% of lumen) in 22.5% and partial (50% of lumen) in 25.4% of patients, consisting either of blood clots (76.1%) or tumoral tissue (23.9%), the latter mainly due to pancreas adenocarcinoma (76.4%). Conclusion: MDCT features of MVT are seen with a wide range of underlying diseases. Signs of intestinal ischemia are infrequently associated, mostly occurring with coagulation/hematologic disorders (40%).

Optic perineuritis: clinical and radiographic features.

BACKGROUND: Optic perineuritis is an uncommon variety of orbital inflammatory disease that is distinct from demyelinating optic neuritis. OBJECTIVE: To describe the clinical and radiographic features of idiopathic optic perineuritis, with particular emphasis on those features that help to distinguish this condition from optic neuritis. METHODS: We reviewed the medical records of 14 patients with optic perineuritis who were seen in 2 neuro-ophthalmology clinics. RESULTS: Patients ranged in age from 24 to 60 years; 5 were older than 50 years. All patients had visual loss, eye pain, or both. The visual acuity was 20/20 or better in 8 of the 15 eyes. The results of visual field testing were normal in 2 eyes, and a paracentral scotoma or an arcuate defect was seen in 7. Magnetic resonance imaging scans demonstrated circumferential enhancement around the optic nerve, sometimes with intraorbital extension. Response to corticosteroids was dramatic; however, 4 patients had a relapse with lowering of the dose. CONCLUSIONS: In contrast to those with optic neuritis, patients with optic perineuritis are often older at onset and are more likely to show sparing of central vision. Magnetic resonance imaging scans demonstrate enhancement around, rather than within, the optic nerve. Response to corticosteroids is more dramatic than in patients with optic neuritis, and patients are more likely to experience recurrence after stopping treatment.

The Implied Book and the Narrative Text. On a Blind Spot in Narratological Theory - from a Media Studies Perspective

The article is concerned with the formal definition of a largely unnoticed factor in narrative structure. Based on the assumptions that (1) the semantics of a written text depend, among other factors, directly on its visual alignment in space, that (2) the formal structure of a text has to meet that of its spatial presentation and that (3) these assumptions hold true also for narrative texts (which, however, in modern times typically conceal their spatial dimensions by a low-key linear layout), it is argued that, how ever low-key, the expected material shape of a given narrative determines the configuration of its plot by its author. The ,implied book' thus denotes an author's historically assumable, not necessarily conscious idea of how his text, which is still in the process of creation, will be dimensionally presented and under these circumstances visually absorbed. Assuming that an author's knowledge of this later (potentially) substantiated material form influences the composition, the implied book is to be understood as a text-genetically determined, structuring moment of the text. Historically reconstructed, it thus serves the methodical analysis of structural characteristics of a completed text.

Osteoid osteoma-like lesions: MDCT features and treatment by radiofrequency ablation : 46

Purpose: To assess the MDCT features of bone lesions that mimic osteoid osteoma (OO-like lesions) and evaluate their treatment by radiofrequency (RF) ablation. Methods and materials: All percutaneous RF ablations performed between May 2002 and June 2009 for a presumed (clinical and MDCT features) diagnosis of OO were retrospectively reviewed. Per-procedural biopsies were always performed and histopathological diagnoses were noted. The following MDCT features of all bone lesions were assessed by two musculoskeletal radiologists in consensus: skeletal distribution and location within the bone, size, central calcification, surrounding osteosclerosis and periosteal reaction. Clinical success was also evaluated. Results: Eighty patients (54 males, 26 females, mean age 24.1 years, range 5-48) underwent RF ablation. The histopathological diagnoses were: 54 non-contributory biopsies, 16 OO, 10 OO-like lesions (5 chronic osteomyelitis, 3 chondroblastoma, 1 eosinophilic granuloma, 1 fibrous dysplasia). The OO-like lesions were significantly greater in size (p = 0.001) and exhibited trends toward medullary location within the bone, moderate surrounding osteosclerosis and less periosteal reaction, compared to OO. Primary clinical success for OO-like lesions was 100% at 1 month, 85.7% at 6 and 12 months, and 66.7% at 24 months. Secondary success was 100%. Conclusion: Greater size, medullary location within the bone, lesser surrounding osteosclerosis and periosteal reaction on MDCT may help differentiate OO-like lesions from OO. OO-like lesions are safely and successfully treated by RF ablation.

Molecular features of hepatosplenic T-cell lymphoma unravels potential novel therapeutic targets.

The pathogenesis of hepatosplenic T-cell lymphoma (HSTL), a rare entity mostly derived from γδ T cells and usually with a fatal outcome, remains largely unknown. In this study, HSTL samples (7γδ and 2αβ) and the DERL2 HSTL cell line were subjected to combined gene-expression profiling and array-based comparative genomic hybridization. Compared with other T-cell lymphomas, HSTL had a distinct molecular signature irrespective of TCR cell lineage. Compared with peripheral T-cell lymphoma, not otherwise specified and normal γδ T cells, HSTL overexpressed genes encoding NK-cell-associated molecules, oncogenes (FOS and VAV3), the sphingosine-1-phosphatase receptor 5 involved in cell trafficking, and the tyrosine kinase SYK, whereas the tumor-suppressor gene AIM1 (absent in melanoma 1) was among the most down-expressed. We found highly methylated CpG islands of AIM1 in DERL2 cells, and decitabine treatment induced a significant increase in AIM1 transcripts. Syk was present in HSTL cells and DERL2 cells contained phosphorylated Syk and were sensitive to a Syk inhibitor in vitro. Genomic profiles confirmed recurrent isochromosome 7q (n = 6/9) without alterations at the SYK and AIM1 loci. Our results identify a distinct molecular signature for HSTL and highlight oncogenic pathways that offer rationale for exploring new therapeutic options such as Syk inhibitors and demethylating agents.