Unusual case of enlarged choroidal vessels in neurofibromatosis type 1 observed with near-infrared reflectance and spectral domain optical coherence tomography.

ABSTRACT: The cardinal diagnostic signs of neurofibromatosis type 1 (NF1) are Lisch nodules of the iris and optic pathway gliomas. Retinal mi-crovascular alterations have been described but with uncertain signi-ficance. Choroidal nodules, detectable with near-infrared reflectance (NIR) imaging, are present in most cases and have been proposed as a new diagnostic criterion. Recently, a study reported the presence of unusual dilated choroidal vessels, visible through NIR examination. We report a case of a 65-year-old patient with NF1. Best-corrected visual acuity was 20/20 with a refractive error of +2.75 diopters in both eyes. Anterior segment examination revealed Lisch nodules in both eyes. At NIR imaging the patient presented typical choroidal alterations in both eyes. No retinal vessel anomalies were detected. The patient presented enlarged choroidal vessels in the left eye, first detected by NIR and then analyzed through enhanced depth imaging spectral domain optical coherence tomography (EDI-SDOCT). These vessels extended from the choroidal-scleral junction to the outer border of the retinal pigment epithelium/Bruch's layer. The choriocapillaris layer was absent above the dilated vessels. The presence of enlarged choroidal vessels may be considered as a novel distinctive ophthalmologic aspect of NF1, but further studies are necessary.

Isolation of bacterial microbiota associated to honey bees and evaluation of potential biocontrol agents of Varroa destructor.

The honey bee parasitic mite Varroa destructor is one of the main causes of depopulation of bee colonies. Bacterial symbionts associated to honey bees are known to produce a variety of bioactive molecules that have been suggested to play a protective role against honey bee pathogens. We hypothesised that among these bacteria, those colonising the external body of honey bees, and therefore able to survive and reproduce in the hive environment outside the insect gut, may be good candidate biocontrol agents to be tested against V. destructor. The aim of this study was to isolate bacterial species from healthy honey bees and dead varroa mites and to evaluate the potential miticidal effect of their spent medium containing both bacterial metabolites and viable cells, with the final objective of finding a long-lasting solution for mite control. 61 bacterial strains belonging to the Firmicutes, Proteobacteria and Actinobacteria phyla were isolated from the surface of foragers, nurse bees and larvae collected in 10 different apiaries. The most common species was Lactobacillus kunkeei (62%). Growth capability of a selection of isolates was observed at 30 and 34 °C with 1% and 20% glucose and fructose. Laboratory bioassays were conducted by spraying mites with six-day-grown bacterial cultures containing 107 cfu/ml of four strains of L. kunkeei, Bacillus thuringiensis, Bifidobacterium asteroides and an Acetobacteraceae bacterium. The effect of each strain on varroa survival was tested independently. The first three caused 95-100% mortality of mites in 3 days, indicating a potential role as natural antagonists towards varroa. The mediation of pH of the bacterial cultures did not appear to be determinant in mite inhibition, suggesting the involvement of other modes of action against varroa. The exploitation of honey bee microbiota for controlling one of the major threats for honey bee health may be a promising approach deserving further investigation.

Secondary Metabolites Produced by Honey Bee-Associated Bacteria for Apiary Health: Potential Activity of Platynecine.

Secondary metabolites of bacteria associated with honey bees were evaluated as part of an investigation on their potentiality for apiary health. Low molecular weight compounds released into culture filtrates by the four bacterial isolates taken from surface of healthy honey bees were analyzed using time-of-flight mass spectrometry. Only one low molecular weight compound was found in the culture filtrate of each isolate. Bacillus thuringiensis, Bifidobacterium asteroides and Acetobacteraceae bacterium, released into culture filtrates platynecine, a pyrrolizidine alkaloid of plant origin, which, until now, had never been reported as produced by bacteria. Lactobacillus kunkeei produced a 3,5-dinitropyridine, of unknown biological action never associated so far to bacteria. The highest relative concentration of platynecine was detected in B. thuringiensis (100%), B. asteroides and A. bacterium showed a concentration above 50% and below 25% that concentration. An in vitro assay on the potential for controlling the parasitic mite Varroa destructor by the culture filtrates of the three platynecine-producing bacteria was performed. Varroa mite mortality was proportional to the platynecine relative concentration into culture filtrate. Although miticidal activity of B. thuringiensis might be associated to other toxic proteins produced by this species, B. asteroides toxicity toward V. destructor along with the other findings of this study support the hypothesis that platynecine plays a direct or an indirect role in controlling varroa. Findings of this study suggest that secondary metabolites released by honey bee-associated bacteria represent a source of natural compounds to be considered in the challenge for counteracting the colony decline.

The term diplegia should be enhanced. Part II: contribution to validation of the new rehabilitation oriented classification.

Recent proposals of classification for cerebral palsy (CP), mainly revised for epidemiological purposes, suggest to abandon the use of the term diplegia. Conversely, in this paper data are presented to support the proposal to maintain the distinction between spastic tetraplegia and diplegia, and to subdivide this latter according to four main clinical patterns of walking observable in these children. This proposal of classification was validated by testing a group of 467 subjects with CP, of whom 213 with diplegia and 115 with tetraplegia, consecutively admitted between January 2005 and December 2006 to two national reference centers for this disability. The results were compared with findings obtained by other methods of classifying gross and fine motor function and associated disorders. The subjects with tetraplegia strongly differ from those of diplegia, both for motor functions and for other disabilities. The four main walking patterns of spastic diplegia were easily recognizable and observers were able to assign most of the subjects to one form of the classification. Significant correlations between walking forms of diplegia and distribution of Gross Motor Function Classification System (GMFCS) levels were found. Some of the forms significantly differ also for fine motor and mental disability. These findings suggest that in clinical practice the category of diplegia not only can be kept as a separate form of CP, but it may be enhanced, through the identification of different subcategories of children, divided according to their walking patterns.

[Control strategies against Varroa mites]. / Strategie di lotta alla varroatosi delle api.

In many cases, the easiest way to control a pest or a pathogen is to resort to chemical treatments. In the short period this option is often the most convenient economically and usually does not require refined knowledge of the biology of the pest. Many efforts have been directed to develop chemical treatments against Varroa destructor Anderson & Trueman. Nowadays, product based on organic acids, essential oils, pyrethroids, organophosphate, and formamidine are available. Resistance is serious problem in the chemotherapy of parasites and pathogens of the honeybee. High-level resistance to several active substances (the pyrethroids acrinathrin, flumethrin, fluvalinate; the organophosphate coumaphos; the formamidine amitraz) was detected with laboratory assays in different varroa populations and caused failure of the control in the field. At the end of the '80s, products based on pyrethroids, very effective against the mite, but without any appreciable side effect on bees, became available. Resistance to fluvalinate in Italy and later in other countries caused serious damage to beekeeping; early detection was crucial to reduce losses. The problem of developing suitable treatments was difficult in the case of the varroa mite because most substances active against V. destructor have unacceptable side effects on bees. The idea that the mite could be controlled indefinitely with these strategies began to gain ground.

Epidemiological study of myasthenia gravis in the province of Reggio Emilia, Italy.

We carried out a retrospective incidence, prevalence and mortality survey of myasthenia gravis in the province of Reggio Emilia in Northern Italy. Based on 49 patients, the mean incidence per year for the period 1980 through 1994 was 7.8 per 1,000,000. On 31 December 1994 the prevalence rate was 117.5 per 1,000,000 for all patients, either active or recovered (50 cases in a population of 427,493) and 103.4 per 1,000,000 for the active disease. In the 15-year period 1980-1994 the average mortality rate was 1.0 per 1,000,000 per year. The average age at onset was 44.6 +/- 21.0, and the average age at the time of prevalence determination was 51.1 +/- 19.6 for the active disease. At the time of diagnosis, 21 patients (36.8%) were classed in group I according to Osserman's criteria, 31 in group II (54.4%), (19 in group II-A and 12 in group II-B), and the other 5 (8.8%) in group III. Of all the prevalence cases, 6 (12%) were in remission without therapy and 6 with therapy, while most of the others 16 (32%) were classed in group I, 15 (30%) in group II, and 1 (2%) in group III. Thymectomy was performed in 20 patients (35.1%), 12 (21%) had thymoma (malignant in 4 cases), 6 had thymic hyperplasia while in two patients thymic histology was normal. The relation the grade of Osserman's scale at the time of incidence and the presence of thymoma were significant. Higher grades of Osserman's scale were associated were malignant thymoma. Furthermore the relationship between thymectomy and the grade of Osserman's scale at the date of prevalence was significant for the presence of lower grades of Osserman's scale in the patients submitted to thymectomy.

Immunohistochemical study of merosin-negative congenital muscular dystrophy: laminin alpha 2 deficiency in skin biopsy.

We studied the immunohistochemical expression of laminin subunits alpha 2, alpha 1, beta 1 in muscle and skin biopsy samples from three patients with congenital muscular dystrophy (CMD), and from ten control patients investigated for various neuromuscular disorders. Merosin alpha 2 chain was not detectable in the basement membrane of muscle fibers, or in the nerve endings, cutaneous nerves, and corium in the skin of the CMD patients, whereas it was clearly expressed in the skin biopsy samples from control patients, especially in the nerve endings of the arrector pili muscles. Laminin alpha 1 chain was expressed in the corium, in the muscle fiber membranes of arrector pili muscles and in cutaneous nerve fibers, perineurium and blood vessels in controls and in CMD patients. Laminin beta 1 chain was faintly expressed in the corium, and a diffuse labeling was detected on arrector pili muscle with enhanced expression at nerve endings, intracutaneous nerves and capillaries, with similar findings in all biopsy specimens. For merosin-negative CMD patients, skin biopsy may provide a diagnostic alternative to muscle biopsy since merosin deficiency can be demonstrated in the skin neural structures, and in particular in the nerve endings of the arrector pili smooth muscles.

Performance of left brain-damaged patients on imitation of single movements and motor sequences. Frontal and parietal-injured patients compared.

The aim of this study was to investigate the relation of apraxia to the sequential features of the motor task required and to the intra-hemispheric locus of lesion. A single movement and a multiple movement imitation test were given to 60 control patients and 60 left brain-damaged patients, among which patients with frontal and parietal lesion were identified, based on CT scan evidence. Both groups performed the tasks using the left limb. On either test left brain-damaged patients scored poorer than controls and parietal patients were significantly more impaired not only than controls, but also than frontal patients. Seventy five per cent of them performed lower than the poorest control patient. In comparison, the severity and the frequency of the motor deficit following frontal damage was much lower. In no case was there a significant difference between the discriminating power of the single movement test and of the sequence test. These findings suggest that the left parietal lobe has a leading role in motor planning and that the control it exerts over the motor cortex of the right hemisphere does not necessarily involve pathways running through the left premotor area.