Frontotemporal dementia with co-occurrence of astrocytic plaques and tufted astrocytes, and severe degeneration of the cerebral white matter: a variant of corticobasal degeneration?

We report two patients who exhibited frontotemporal dementia (FTD) with unusual neuropathological features. The ages of the patients at death were 65 and 67 years, the disease durations were 6 and 5 years, and the clinical diagnoses were Picks disease and corticobasal degeneration (CBD), respectively. At autopsy, both cases exhibited neuropathological findings compatible with those of CBD, including atrophy of the frontal and parietal lobes, neuronal loss and gliosis in the cortical and subcortical regions, and presence of cortical ballooned neurons and astrocytic plaques (APs). In both cases, immunoblotting of insoluble tau exhibited the pattern of selective accumulation of four-repeat tau, a finding that is also compatible with CBD. However, severe degeneration was evident in the frontal and parietal white matter in both cases. Moreover, a striking finding was the widespread presence in the affected cortex of tufted astrocytes (TAs), which are characteristic of progressive supranuclear palsy (PSP). Neither co-occurrence of APs and TAs nor severe degeneration of the cerebral white matter is a feature of either CBD or PSP. No mutations were found in the tau gene in either case. In conclusion, the possibility that these two cases represent a new neuropathological phenotype of non-familial FTD rather than simply a variant of CBD cannot be completely excluded.     

GalNAc-GD1a is localized specifically in ventral spinal roots, but not in dorsal spinal roots

We investigated the localization of GalNAc-GD1a biochemically in the human and bovine peripheral nervous system (PNS). The high-performance thin-layer chromatography (HPTLC)-overlay method with rabbit IgG polyclonal antibody against GalNAc-GD1a (anti-GalNAc-GD1a antibody) revealed expression of GalNAc-GD1a in the ventral spinal nerve roots (VRs) but not in the dorsal spinal nerve roots (DRs) of both species. The amount of GalNAc-GD1a in the human and bovine VRs was 2.22 +/- 0.35 microg/g wet tissue and 7.71 +/- 0.49 microg/g wet tissue, respectively. These results suggest that IgG anti-GalNAc-GD1a antibody may be involved in disturbance of peripheral motor nerves and in the pathogenesis of pure motor neuropathy.     

Different signal intensities between intra- and extracranial components in jugular foramen meningioma: an enigma

BACKGROUND AND PURPOSE: The purpose of this study was to evaluate retrospectively differences in MR signal intensity and contrast enhancement between intra- and extracranial components of jugular foramen meningioma (JFM). METHODS: MR studies of eight patients who underwent surgery for histologically confirmed JFM were reviewed retrospectively. Signal intensity differences between intra- and extracranial components of all eight JFMs on axial T1-, T2-, and postcontrast T1-weighted images were evaluated visually. In six of the eight JFMs, quantitative signal intensity evaluations were also performed by using relative signal intensity ratios of the intra- and extracranial components of JFM to CNS tissue at the same level. Paired t tests were used to evaluate differences in relative signal intensity ratios in each JFM between intra- and extracranial components. RESULTS: Both visual and quantitative signal intensity evaluations revealed that signal intensities of the intracranial component of JFM were significantly higher than those of the extracranial component on T1-, T2-, and postcontrast T1-weighted images. Results of relative signal intensity ratios were 0.89 +/- 0.04 versus 0.77 +/- 0.02 on T1-weighted images (P = .002); 1.66 +/- 0.28 versus 0.88 +/- 0.14 on T2-weighted images (P = .003); and 2.16 +/- 0.29 versus 1.77 +/- 0.26 on postcontrast T1-weighted images (P = .01). CONCLUSION: Intra- and extracranial components of JFM display different signal intensity and enhancement patterns. These differences may be related to histologic composition, and in particular, collagen content.     

Pleural perfusion thermo-chemotherapy under VATS: a new less invasive modality for advanced lung cancer with pleural spread

BACKGROUND: We conducted a trial of a new less invasive, locoregional modality for lung cancer with pleural spread. This study was planned to investigate the feasibility, safety, and pharmacokinetics of pleural perfusion thermochemotherapy (PPTC) under video-assisted thoracoscopic surgery (VATS) and its modified method with a short perfusion time for preventing heat damage to the lung during the procedure. METHODS: Seventeen patients, 59 to 79 years old, underwent surgical resection of the primary lesions and PPTC under VATS without thoracotomy. All had pleural spread with malignant effusion due to lung cancer proven before the treatment. PPTC consists of irrigating the pleural space with 42 degrees C saline solution containing cisplatin (200 mg/m(2)) using a devised circuit. The time for perfusion was two hours in 10 patients (group L), and one hour in 7 patients (group S). RESULTS: All patients successfully completed this treatment with acceptable toxicities. The pharmacokinetic analysis showed that high platinum levels for the regional pleural exposure, which was 20- to 40-fold greater than those for the plasma in both groups. These values were equivalent between the groups, although the levels for the plasma were higher in group L than in group S. Postoperative lung damage was seen in 4 patients with no serious conditions in group L, and none in group S. The median survival for the L and S groups was 17 and 19 months, respectively. CONCLUSIONS: This less invasive modality seems to offer a safe, feasible, and pharmacokinetically advantageous procedure to have excellent local control for lung cancer with pleural spread.     

The usefulness of MRI around the tentorial incisura for determining the mechanism of brain stem injury: a case report

We report a case of isolated brain stem injury caused by the tentorium cerebelli. A 17-year-old male was admitted to our hospital. Thirty minutes before admission, he was struck by another motorcycle while driving his own motorcycle. The Glasgow Coma Scale (GCS) was 10. He had no extremity weakness and his pupils were normal. CT scan showed no evidence of intracranial lesions except for facial bone fractures. His consciousness level improved to GCS 13 at 6 hours after admission. Follow-up CT scan was normal, however MRI 3 days after admission showed a contusion at the left lateral midbrain. He was discharged without any neurological deficits on 6 days after admission. Analysis of the distance between the brain stem and the tentorial margin using MR cisternography showed that the left side was shorter than the right side. We presumed that an isolated lateral brain stem injury was caused by the direct impact of the tentorium cerebelli. Typically the location of this type of lesion is present in the same side as that of impact. However in this case the orientation was opposite to that. We considered that the distance between the brain stem and the tentorial margin affected the mechanism of this lesion.     

Severe Protein-Calorie Malnutrition after Bariatric Procedures

Background: Serious nutritional complications after Roux-en-Y gastric bypass (RYGBP) are infrequent. In a retrospective study of patients operated during a 68-month period, malnutrition was investigated to analyze circumstances associated with nutritional failure. Methods: In 236 consecutive RYGBPs, 11 patients with severe malnutrition were identified (4.7%) with age 45.1 ± 10.6 years (10 females/1 male) and initial BMI 54.6 ± 8.4 kg/m². Results: In these 11 patients, the derangement was diagnosed 17.9 ± 15.8 months after RYGBP, following defined events in 63.6% (gastric stenosis, associated diseases ) or mostly exaggeration of expected symptoms in 36.4% (vomiting without endoscopic abnormalities). BMI then was 31.4 ± 8.6 kg/m² (42.5 ± 9.9% total reduction, or 2.4 ± 2.1% decrease/month), and serum albumin and hemoglobin were 24.0 ± 8.2 g/L and 97.0 ± 23.0 g/L respectively. Edema was present in 45.4% (5/11), hospitalization was required in 54.5% (6/11), and 18.2% (2/11) eventually died. Conclusions: Serious malnutrition was unusual but not exceedingly rare in this series. Exogenous precipitating factors were clearly identified in 63.6% of the patients. Careful clinical and nutritional follow-up is recommended to prevent these uncommon but potentially dangerous complications.     

Abdominal aortic aneurysm associated with crossed renal ectopia without fusion: case report and literature review

Genitourinary anomalies can present a formidable challenge to the vascular surgeon at abdominal aortic reconstruction. We saw a case of crossed renal ectopia without fusion, a rare anomaly, associated with abdominal aortic aneurysm. Because of risk for injury to the kidney during surgery, preoperative evaluation of this anomaly must include computed tomography, angiography, and intravenous pyelography. Preoperative placement of a ureteral catheter may prevent injury to the anomalous ureter. Renal failure of the ectopic kidney during aortic reconstruction can be a serious problem. We used in situ hypothermic perfusion with cold (4 degrees C) Ringer solution for renal protection, and reimplanted the aberrant renal artery. The postoperative course was good, without major complications. The procedure for renal preservation must be selected on the basis of anatomic findings. We review the literature and present the first case of crossed renal ectopia.     

New cell wall targets for antifungal drugs

Fungal infections in humans caused by filamentous moulds and yeasts continue to be serious and often life-threatening, especially among immunocompromised patients. One of the most immediate and unmet medical needs is the identification of new treatment options for invasive aspergillosis infection. The fungal cell wall, as the most obvious difference between human and fungal cells, represents an excellent target for antifungals. Cell wall carbohydrates are polymerized from uridine di-phospho sugars that are synthesized by several biochemical pathways. The pathways for the synthesis of these cell wall precursors represent unexplored targets for new antifungals that have modes of action that are different from currently used therapeutics. This review will focus on the identification of several new therapy targets and on assays to screen for inhibitors of enzymes critical to the synthesis of cell wall precursors.     

Glomerular expression of connective tissue growth factor mRNA in various renal diseases

SUMMARY:   Connective tissue growth factor (CTGF) is a cysteine-rich member of a new family of growth regulators. It is an important factor in the pathogenesis of mesangial matrix accumulation and progressive glomerulosclerosis. The present study was designed to elucidate the role of CTGF in diabetic nephropathy (DN), immunoglobulin A nephropathy (IgA-N), membranous nephropathy (MN), and minimal change nephrotic syndrome (MCNS). We evaluated the expression and localization of CTGF mRNA in surgically excised renal tissue samples from 10 patients with DN, 10 with IgA-N, 10 with MN, 10 with MCNS, and 10 normal human kidney (NHK) tissue samples, by using high-resolution in situ hybridization with digoxigenin-labelled oligonucleotide. To quantify CTGF mRNA expression, we counted all nuclei, and nuclei surrounded by CTGF-positive cytoplasm, in at least 10 randomly selected cross-sections of non-sclerotic glomeruli, and expressed the results as a percentage of total glomerular cells. In all glomeruli, CTGF mRNA was expressed mainly in glomerular intrinsic cells, including glomerular mesangial and epithelial cells and some cells of Bowman's capsule. The percentage of cells positive for CTGF mRNA was significantly higher in DN and IgA-N than in MN, MCNS and NHK. However, there was no significant difference in the percentage of CTGF mRNA-positive cells between DN and IgA-N. Our study indicates that CTGF may play an important role in the development and progression of glomerulosclerosis in DN and IgA-N, which are both accompanied by mesangial matrix expansion and comprise two major causes of end-stage renal failure.