Slowly progressive hemiparesis in childhood as a consequence of Rasmussen encephalitis without or with delayed-onset seizures

Five young children developed slowly progressive hemiparesis as the initial manifestation of Rasmussen encephalitis (RE). Three have remained seizure free over an observational period of 1.3–1.9 years. In the remaining two patients, seizures occurred after 0.5 and 0.6 years respectively. We suggest that RE might be presently underdiagnosed and should be suspected in cases of new onset hemiparesis. In this series, three out of five patients showed oligoclonal bands on examination of cerebrospinal fluid (CSF) which represented additional diagnostic hints towards an immune-mediated condition. According to recently published formal diagnostic criteria, evidence of progressive cerebral hemiatrophy or bioptic identification of RE-typical inflammation confirms the diagnosis in such cases. Long-term immunotherapy is recommended in order to prevent further tissue loss and functional decline.     

Value of digital volume tomography in patients with conductive hearing loss

Digital volume tomography (DVT) is an extension of panoramic tomography. With this diagnostic technique, characterized by high resolution, a narrow section width (0.125 mm) and three-dimensional display, small pathological processes can be well visualized. Twenty-five patients with the history of a progressive hearing loss were examined with DVT (Accu-I-tomo, Morita, Japan). The results were compared with pre- and intraoperative findings to evaluate the diagnostic value of DVT in cases of erosion of the ossicular chain. With high resolution and artifact-free demonstration of the middle ear and the ossicular chain, it was possible to define its continuity preoperatively by DVT in all 25 cases. An intact ossicular chain was found by DVT in 13 cases and was later confirmed by surgery. The predicted erosion of the ossicles was verified in 12 patients, and a tympanoplasty type III was performed. Digital volume tomography is an excellent technique to examine the middle ear cleft and inner ear, and expands the application of diagnostic possibilities in the lateral skull base. Therefore, improvement in preoperative diagnosis is achieved along with more accurate planning of the surgical procedure. Digital volume tomography delivers a small radiation dose with a high resolution and a low purchase price for the equipment.     

Utilization of preoperative cerebrospinal fluid drain in skull base surgery.

OBJECTIVE: Retrospectively assess the efficacy of lumbar cerebrospinal fluid (CSF) drainage placed preoperatively in skull base operations in decreasing the incidence of postoperative CSF fistula. METHODS: A retrospective review of 150 patients undergoing a posterior fossa craniotomy from 1989 to 2000 was conducted. Patients were divided into those receiving preoperative lumbar drains and those that did not. The rates of postoperative CSF leakage were compared between the two groups. Patient data were analyzed to determine if there were other comorbidities affecting the postoperative incidence of CSF leakage such as smoking, diabetes, or hypertension. RESULTS: Between 1989 and 1994, 25/72 (35%) patients with no preoperative lumbar drain had a postoperative CSF leak. From 1995 to 2000, 9/78 (12%) patients with a preoperative lumbar drain had a CSF leak. This was a 23% decreased incidence of postoperative CSF leakage and a significant decrease in the probability (p < 0.001) of CSF leakage in patients treated with a preoperative lumbar drain. The comorbidities of diabetes, smoking, or hypertension did not increase the probability of a CSF leak (p = 0.43). CONCLUSIONS: A preoperatively placed lumbar drain can significantly lower the rate of postoperative CSF leakage after skull base surgery. The drain is a well-tolerated adjunct to dural closure and helps increase surgical exposure of the posterior fossa. The comorbidities of diabetes, smoking, or hypertension do not contribute to an increased rate of CSF leakage.dagger Lyal Leibrock M.D., F.A.C.S. is Deceased.     

Fibroblasts from recurrent fibrotic overgrowths reveal high rate of proliferation in vitro - findings from the study of hereditary and idiopathic gingival fibromatosis

ABSTRACT

Purpose: Investigate the content of fibrotic fibrils in gingival tissue and the proliferation of fibroblasts collected from recurrent and non-recurrent hereditary gingival fibromatosis (HGF) and idiopathic gingival fibromatosis (IGF).

Methods: Gingival biopsies were collected from HGF (n = 3) and IGF (n = 3) donors with recurrent and non-recurrent gingival overgrowths and from a control group (Ctrl, n = 3). Hematoxylin staining was performed to evaluate the histomorphology of gingival tissue. Heidenhain’s AZAN trichrome staining served for visualization of fibrotic fibrils in gingiva. Quantitative analysis of the content of fibrotic fibrils in gingival tissue was performed using a polarized light microscope. Proliferation was evaluated at 24 h, 48 h, and 72 h in fibroblast cultures using a cell proliferation ELISA assay based on 5-bromo-2?-deoxyuridine (BrdU).

Results: Numerous blood vessels and fibroblasts were observed in recurrent overgrowths, whereas moderate blood vessels and moderate to scanty fibroblasts were detected in non-recurrent overgrowths. Heidenhain’s staining revealed numerous collagen fibers in both recurrent and non-recurrent overgrowths. Quantitative analysis in a polarizing microscope showed significant accumulation of fibrotic fibrils exclusively in the overgrowths with the recurrence. In all time-points, increased proliferation of cells from all recurrent overgrowths was observed, but not from overgrowths which do not reoccur.

Conclusions: The study revealed that recurrent gingival overgrowths consist of highly fibrotic and dense connective tissue with numerous blood vessels and abundant fibroblasts. We also demonstrated that unlike fibroblasts derived from overgrowths, which did not present recurrence, fibroblasts derived from highly fibrotic and recurrent overgrowths maintain high rate of proliferation in vitro.     

真空紫外辐照对加成型硅橡胶光学性能的影响

考察了加成型硅橡胶在真空环境中经1000ESH紫外辐照后的性能变化。结果表明,辐照后材料均出现发黄的现象,光学透过率大幅度 下降,同时加入硅酸钾包覆后制备的热控涂层反射率下降。经原位测试与离位测试,发现加成型硅橡胶在两种不同条件下测得的结果差异较小,而在有机硅橡胶中加入ZnO后原位与离位测试结果则差异明显,表现出明显的漂白作用。     

High hydrostatic pressure effects onSpirostomum ambiguum

The effects of hydrostatic pressure on the ciliateSpirostomum ambiguum, when the organism is uncalcified in the early stage of culture and when it is calcified after periods of time are described. Uncalcified cells are sensitive to pressure, becoming quiescent at 250 atm, distorting at 300 atm and disrupting at 550 atm. In contrast, the mature calcified animals show no changes at 550 atm. and small temporary increases in diameter only during 2 hr of treatment at 1000 atm.

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Zusammenfassung Es wird der Einfluß von hydrostatischem Druck auf die CiliatenSpirostomum ambiguum beschrieben, einerseits auf nicht verkalkte, im frühen Stadium der Kultur stehende Infusionen, anderseits auf verkalkte, längere Zeit gewachsene Mikroorganismen. Nicht verkalkte Zellen sind druckempfindlich; bei 250 Atm. verlieren sie ihre Mobilität, bei 300 Atm. verändern sie ihre Form und bei 550 Atm. platzen sie. Im Gegensatz dazu weisen die ausgewachsenen verkalkten Tiere bei 550 Atm. keine Veränderungen auf; nach Einwirkung von 1000 Atm. während 2 Std zeigen sie nur eine kleine vorübergehende Zunahme ihres Durchmessers.

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Résumé Les effets de la pression hydrostatique ont été étudiés surSpirostomum ambiguum cilié, à un stade précoce du culture, lorsqu'il n'est pas calcifié et plus tardivement lorsqu'il est calcifié. Les cellules non calcifiées sont sensibles à la pression. Elles deviennent inertes à 250 atm.; elles changent de forme à 30 0atm. et elles éclatent à 550 atm. Par contre, les organismes calcifiés adultes ne sont pas altérés à 550 atm. et ne présentent qu'une faible augmentation transitoire de diamètre qu'au bout de 2 heures de traitement à 1000 atm.

     

Highly selective inhibition of butyrylcholinesterase by a novel melatonin–tacrine heterodimers

Novel inhibitors of cholinesterases, especially butyrylcholinesterase (BuChE), were obtained by coupling melatonin–tacrine heterodimers via the carbamate bond. Compounds 14a‐i possessed potent cholinesterase inhibitory activity (with IC₅₀ values as low as 1.18 nm for acetylcholinesterase (AChE) and 0.24 nm for butyrylcholinesterase (BuChE)). These heterodimers exhibit selectivity toward BuChE, being from 4‐ to 256‐fold more active toward BuChE than AChE, but still acting as better AChE inhibitors than tacrine 4 .     

Routine diagnostics for neural antibodies,clinical correlates,treatment and functional outcome

Objective

To determine frequencies, interlaboratory reproducibility, clinical ratings, and prognostic implications of neural antibodies in a routine laboratory setting in patients with suspected neuropsychiatric autoimmune conditions.

Methods

Earliest available samples from 10,919 patients were tested for a broad panel of neural antibodies. Sera that reacted with leucine-rich glioma-inactivated protein 1 (LGI1), contactin-associated protein-2 (CASPR2), or the voltage-gated potassium channel (VGKC) complex were retested for LGI1 and CASPR2 antibodies by another laboratory. Physicians in charge of patients with positive antibody results retrospectively reported on clinical, treatment, and outcome parameters.

Results

Positive results were obtained for 576 patients (5.3%). Median disease duration was 6 months (interquartile range 0.6–46 months). In most patients, antibodies were detected both in CSF and serum. However, in 16 (28%) patients with N-methyl-d-aspartate receptor (NMDAR) antibodies, this diagnosis could be made only in cerebrospinal fluid (CSF). The two laboratories agreed largely on LGI1 and CASPR2 antibody diagnoses (κ = 0.95). The clinicians (413 responses, 71.7%) rated two-thirds of the antibody-positive patients as autoimmune. Antibodies against the α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR), NMDAR (CSF or high serum titer), γ-aminobutyric acid-B receptor (GABABR), and LGI1 had ≥ 90% positive ratings, whereas antibodies against the glycine receptor, VGKC complex, or otherwise unspecified neuropil had ≤ 40% positive ratings. Of the patients with surface antibodies, 64% improved after ≥ 3 months, mostly with ≥ 1 immunotherapy intervention.

Conclusions

This novel approach starting from routine diagnostics in a dedicated laboratory provides reliable and useful results with therapeutic implications. Counseling should consider clinical presentation, demographic features, and antibody titers of the individual patient.

     

Facing Antibiotic Resistance: Staphylococcus aureus Phages as a Medical Tool

Staphylococcus aureus is a common and often virulent pathogen in humans. This bacterium is widespread, being present on the skin and in the nose of healthy people. Staphylococcus aureus can cause infections with severe outcomes ranging from pustules to sepsis and death. The introduction of antibiotics led to a general belief that the problem of bacterial infections would be solved. Nonetheless, pathogens including staphylococci have evolved mechanisms of drug resistance. Among current attempts to address this problem, phage therapy offers a promising alternative to combat staphylococcal infections. Here, we present an overview of current knowledge on staphylococcal infections and bacteriophages able to kill Staphylococcus, including experimental studies and available data on their clinical use.