Mikrozystisches Makulaödem bei Optikusatrophie: eine Fallserie Microcystic Macular Edema in Optic Nerve Atrophy: A Case Series.

Background:Microcystic macular edema can occur after optic neuropathies of various etiologies, and is easily demonstrated by OCT. We report a cohort of patients with microcystic macular edema. Patients and Methods: All patients with optic neuropathy and microcystic macular edema were enrolled. Demographics, visual function, retinal angiographies and OCT parameters were studied. Results: Nineteen patients (23 eyes) exhibited microcystic macular edema: 10 men/9 women, aged 17-91 years. Etiologies of optic nerve atrophy were compressive (5), inflammatory (4), glaucoma (3), ischemic (3), trauma (2), degenerative (1), and hereditary (1). Median visual acuity was 4/10 (NLP-12/10). Fluorescein angiography showed no leakage. Topography of the microcystic macular edema correlated with near infrared images but with visual field defects in only 26 %. OCT parameters were all abnormal. Conclusions: Microcystic macular edema is a non-specific manifestation from an optic neuropathy of any etiology. The precise mechanism leading to microcystic macular edema remains unknown but trans-synaptic retrograde degeneration with Müller cells dysfunction is likely. Zusammenfassung Hintergrund: Das mikrozystische Makulaödem kann im Rahmen einer Optikusatrophie jeglicher Ätiologie auftreten und ist leicht mit dem OCT zu erkennen. Wir berichten über eine Patientenkohorte mit mikrozystischem Makulaödem. Patienten und Methoden: Alle Patienten mit einer Optikusneuropathie und einem mikrozystischen Makulaödem wurden in diese Studie eingeschlossen. Die Demografie, die Sehfunktion, die Netzhautangiografie und die OCT-Parameter wurden untersucht. Ergebnisse: Neunzehn Patienten (23 Augen) hatten ein mikrozystisches Makulaödem: 10 Männer/9 Frauen im Alter von 17 bis 91 Jahren. Die Ursachen der Optikusatrophie waren Kompressionen (5), Entzündungen (4), Glaukom (3), Ischämien (3), Traumata (2), Degenerationen (1) und genetisch (1). Der mittlere Visus war 0,4 (keine Lichtwahrnehmung 1,2). In der Fluoreszenzangiografie fand sich keine Leckage. Das OCT des mikrozystischen Makulaödems korrelierte immer mit den Infrarot-Bildern (Nahaufnahme), jedoch nur in 26 % mit den Gesichtsfelddefekten. Alle OCT-Parameter waren abnormal. Schlussfolgerungen: Das mikrozystische Makulaödem ist eine unspezifische Manifestation einer Optikusneuropathie jeglicher Ätiologie. Der genaue Mechanismus, der zu einem mikrozystischen Makulaödem führt, ist unbekannt, eine trans-synaptische retrograde Degeneration mit Dysfunktion der Müller-Zellen ist jedoch wahrscheinlich.

Transient ocular motor nerve palsies associated with presumed cranial nerve schwannomas.

BACKGROUND: Cranial nerve schwannomas are radiologically characterized by nodular cranial nerve enhancement on magnetic resonance imaging (MRI). Schwannomas typically present with gradually progressive symptoms, but isolated reports have suggested that schwannomas may cause fluctuating symptoms as well. METHODS: This is a report of ten cases of presumed cranial nerve schwannoma that presented with transient or recurring ocular motor nerve deficits. RESULTS: Schwannomas of the third, fourth, and fifth nerves resulted in fluctuating deficits of all 3 ocular motor nerves. Persistent nodular cranial nerve enhancement was present on sequential MRI studies. Several episodes of transient oculomotor (III) deficts were associated with headaches, mimicking ophthalmoplegic migraine. CONCLUSIONS: Cranial nerve schwannomas may result in relapsing and remitting cranial nerve symptoms.

Reducción del vector de características de reconocimiento facial

In this present work, we are proposing a characteristics reduction system for a facial biometric identification system, using transformed domains such as discrete cosine transformed (DCT) and discrete wavelets transformed (DWT) as parameterization; and Support Vector Machines (SVM) and Neural Network (NN) as classifiers. The size reduction has been done with Principal Component Analysis (PCA) and with Independent Component Analysis (ICA). This system presents a similar success results for both DWT-SVM system and DWT-PCA-SVM system, about 98%. The computational load is improved on training mode due to the decreasing of input’s size and less complexity of the classifier.

Peripheral Nerve Repair: Multimodal Comparison of the Long-Term Regenerative Potential of Adipose Tissue-Derived Cells in a Biodegradable Conduit.

Tissue engineering is a popular topic in peripheral nerve repair. Combining a nerve conduit with supporting adipose-derived cells could offer an opportunity to prevent time-consuming Schwann cell culture or the use of an autograft with its donor site morbidity and eventually improve clinical outcome. The aim of this study was to provide a broad overview over promising transplantable cells under equal experimental conditions over a long-term period. A 10-mm gap in the sciatic nerve of female Sprague-Dawley rats (7 groups of 7 animals, 8 weeks old) was bridged through a biodegradable fibrin conduit filled with rat adipose-derived stem cells (rASCs), differentiated rASCs (drASCs), human (h)ASCs from the superficial and deep abdominal layer, human stromal vascular fraction (SVF), or rat Schwann cells, respectively. As a control, we resutured a nerve segment as an autograft. Long-term evaluation was carried out after 12 weeks comprising walking track, morphometric, and MRI analyses. The sciatic functional index was calculated. Cross sections of the nerve, proximal, distal, and in between the two sutures, were analyzed for re-/myelination and axon count. Gastrocnemius muscle weights were compared. MRI proved biodegradation of the conduit. Differentiated rat ASCs performed significantly better than undifferentiated rASCs with less muscle atrophy and superior functional results. Superficial hASCs supported regeneration better than deep hASCs, in line with published in vitro data. The best regeneration potential was achieved by the drASC group when compared with other adipose tissue-derived cells. Considering the ease of procedure from harvesting to transplanting, we conclude that comparison of promising cells for nerve regeneration revealed that particularly differentiated ASCs could be a clinically translatable route toward new methods to enhance peripheral nerve repair.

Lower incisor dentoalveolar compensation and symphysis dimensions among Class I and III malocclusion patients with different facial vertical skeletal patterns

Objective: To compare lower incisor dentoalveolar compensation and mandible symphysis morphology among Class I and Class III malocclusion patients with different facial vertical skeletal patterns. Materials and Methods: Lower incisor extrusion and inclination, as well as buccal (LA) and lingual (LP) cortex depth, and mandibular symphysis height (LH) were measured in 107 lateral cephalometric x-rays of adult patients without prior orthodontic treatment. In addition, malocclusion type (Class I or III) and facial vertical skeletal pattern were considered. Through a principal component analysis (PCA) related variables were reduced. Simple regression equation and multivariate analyses of variance were also used. Results: Incisor mandibular plane angle (P < .001) and extrusion (P  =  .03) values showed significant differences between the sagittal malocclusion groups. Variations in the mandibular plane have a negative correlation with LA (Class I P  =  .03 and Class III P  =  .01) and a positive correlation with LH (Class I P  =  .01 and Class III P  =  .02) in both groups. Within the Class III group, there was a negative correlation between the mandibular plane and LP (P  =  .02). PCA showed that the tendency toward a long face causes the symphysis to elongate and narrow. In Class III, alveolar narrowing is also found in normal faces. Conclusions: Vertical facial pattern is a significant factor in mandibular symphysis alveolar morphology and lower incisor positioning, both for Class I and Class III patients. Short-faced Class III patients have a widened alveolar bone. However, for long-faced and normal-faced Class III, natural compensation elongates the symphysis and influences lower incisor position.

Persistence of tracer in the application site -a potential confounding factor in nerve regeneration studies

Selective reinnervation of peripheral targets after nerve injury might be assessed by injecting a first tracer in a target before nerve injury to label the original neuronal population, and applying a second tracer after the regeneration period to label the regenerated population. However, altered uptake of tracer, fading, and cell death may interfere with the results. Furthermore, if the first tracer injected remains in the target tissue, available for 're-uptake' by misdirected regenerating axons, which originally innervated another region, then the identification of the original population would be confused. With the aim of studying this problem, the sciatic nerve of adult rats was sectioned and sutured. After 3 days, to allow the distal axon to degenerate avoiding immediate retrograde transport, one of the dyes: Fast Blue (FB), Fluoro-Gold (FG) or Diamidino Yellow (DY), was injected into the tibial branch of the sciatic nerve, or in the skin of one of the denervated digits. Rats survived 2-3 months. The results showed labelled dorsal root ganglion (DRG) cells and motoneurones, indicating that late re-uptake of a first tracer occurs. This phenomenon must be considered when the model of sequential labelling is used for studying the accuracy of peripheral reinnervation.

Collagen (NeuraGen®) nerve conduits and stem cells for peripheral nerve gap repair

Le système nerveux périphérique est responsable de la transmission des impulses motrices, ainsi que de la réception des afférences sensorielles. Les lésions traumatiques des nerfs périphériques conduisent à une impotence fonctionnelle qui peut être dévastant, notamment chez les travailleurs manuels,. La récupération fonctionnelle est donc le but principal dans chirurgie des nerfs périphériques. Malheureusement, une suture directe des moignons nerveux est souvent impossible dans le contexte des traumatismes complexes qui surviennent lors des accidents. La suture nerveuse par interposition d'autogreffe reste le gold standard dans la pratique chirurgicale mais nécessite le sacrifice d'un nerf donneur, avec dysesthésie et possibles douleurs neuropathiques conséquentes. Alternativement, des guides tubulaires pour les nerfs peuvent être utilisées si le gap nerveux est inférieur à 3 cm. Plusieurs guides résorbables en collagène sont approuve en Europe et aux Etas Unis (FDA). Dans cette étude, des conduits de collagène ont été associe a des cellules régénératives (cellules souches adultes) comme stratégie supplémentaire de régénération. Une fois testé le rapport des cellules avec le biomatériau (NeuraGen® nerve guides) in vitro, une étude in vivo dans le rat a été effectuée. Les différents groupes de conduits ont été supplémentés respectivement avec Schwann cells (SC); avec cellules souches adultes dérivées de la moelle épinière, différentiées en cellules "Schwann-like" (dMSC); avec cellules souches adultes dérivées de la graisse, différentiées en cellules "Schwann-like" (dASC). Un groupe de conduits avec du milieu de culture sans cellules a été utilisé comme group control. Les conduits ont été utilisés pour combler un gap de 1cm dans un model de section totale du nerf sciatique chez le rat. Deux semaines post implantation, une analyse immuno-histochimique a été effectuée pour évaluer la régénération axonales et l'infiltration de cellules de Schwann au niveau du conduit. Les cellules ont montré une adhérence efficace aux parois de collagène. En particulier, les cellules de Schwann ont montré une amélioration significative au niveau du sprouting distale. Par contre, aucune différence significative n'a été remarquée entre les groupes pour le sprouting axonale proximal. De plus, si les cellules souches ont montré un pattern de sprouting diffus, les cellules de Schwann ont par contre garanti un cône de croissance typique, associé a une affinité remarquable pour les parois de collagène. NeuraGen® guides pourraient donc être un moyen adapté a l'association avec la thérapie cellulaire en raison de la bonne adhérence des cellules au biomatériau. Des modifications de surface dans le but d'améliorer la performance neurotrophique cellulaire in vivo (e.g. peptides de matrice extracellulaire) pourront être utilisées dans des applications futures.

Comparative study of two needle models in terms of deflection during inferior alveolar nerve block

Objectives: The purpose of this study is to determine the possible differences in deflection between two needles of same length and external gauge but with different internal gauges during truncal block of the inferior alveolar nerve. The initial working hypothesis was that greater deflection may be expected with larger internal gauge needles. Study design: Four clinicians subjected 346 patients to inferior alveolar nerve block and infiltrating anesthesia of the buccal nerve trajectory for the surgical or conventional extraction of the lower third molar. A nonautoaspirating syringe system with 2 types of needle was used: a standard 27-gauge x 35-mm needle with an internal gauge of 0.215 mm or an XL Monoprotect® 27-gauge x 35-mm needle with an internal gauge of 0.265 mm. The following information was systematically recorded for each patient: needle type, gender, anesthetic technique (direct or indirect truncal block) and the number of bone contacts during the procedure, the patient-extraction side, the practitioner performing the technique, and blood aspiration (either positive or negative). Results: 346 needles were used in total. 190 were standard needles (27-gauge x 35-mm needle with an internal gauge of 0.215 mm) and 156 were XL Monoprotect®. Incidence of deflection was observed in 49.1% of cases (170 needles) where 94 were standard needles and 76 XL Monoprotect®. Needle torsion ranged from 0º and 6º. Conclusions: No significant differences were recorded in terms of deflection and internal gauge, operator, patient-extraction side, the anesthetic technique involved and the number of bone contacts during the procedure

Estimations of topographically correct regeneration to nerve branches and skin after peripheral nerve injury and repair.

Peripheral nerve injury is typically associated with long-term disturbances in sensory localization, despite nerve repair and regeneration. Here, we investigate the extent of correct reinnervation by back-labeling neuronal soma with fluorescent tracers applied in the target area before and after sciatic nerve injury and repair in the rat. The subpopulations of sensory or motor neurons that had regenerated their axons to either the tibial branch or the skin of the third hindlimb digit were calculated from the number of cell bodies labeled by the first and/or second tracer. Compared to the normal control side, 81% of the sensory and 66% of the motor tibial nerve cells regenerated their axons back to this nerve, while 22% of the afferent cells from the third digit reinnervated this digit. Corresponding percentages based on quantification of the surviving population on the experimental side showed 91%, 87%, and 56%, respectively. The results show that nerve injury followed by nerve repair by epineurial suture results in a high but variable amount of topographically correct regeneration, and that proportionally more neurons regenerate into the correct proximal nerve branch than into the correct innervation territory in the skin

NBAS mutations cause a multisystem disorder involving bone, connective tissue, liver, immune system, and retina.

We report two unrelated patients with a multisystem disease involving liver, eye, immune system, connective tissue, and bone, caused by biallelic mutations in the neuroblastoma amplified sequence (NBAS) gene. Both presented as infants with recurrent episodes triggered by fever with vomiting, dehydration, and elevated transaminases. They had frequent infections, hypogammaglobulinemia, reduced natural killer cells, and the Pelger-Huët anomaly of their granulocytes. Their facial features were similar with a pointed chin and proptosis; loose skin and reduced subcutaneous fat gave them a progeroid appearance. Skeletal features included short stature, slender bones, epiphyseal dysplasia with multiple phalangeal pseudo-epiphyses, and small C1-C2 vertebrae causing cervical instability and myelopathy. Retinal dystrophy and optic atrophy were present in one patient. NBAS is a component of the synthaxin-18 complex and is involved in nonsense-mediated mRNA decay control. Putative loss-of-function mutations in NBAS are already known to cause disease in humans. A specific founder mutation has been associated with short stature, optic nerve atrophy and Pelger-Huët anomaly of granulocytes (SOPH) in the Siberian Yakut population. A more recent report associates NBAS mutations with recurrent acute liver failure in infancy in a group of patients of European descent. Our observations indicate that the phenotypic spectrum of NBAS deficiency is wider than previously known and includes skeletal, hepatic, metabolic, and immunologic aspects. Early recognition of the skeletal phenotype is important for preventive management of cervical instability. © 2015 Wiley Periodicals, Inc.

Comparison of acute non-visual bright light responses in patients with optic nerve disease, glaucoma and healthy controls.

This study examined the effect of optic nerve disease, hence retinal ganglion cell loss, on non-visual functions related to melanopsin signalling. Test subjects were patients with bilateral visual loss and optic atrophy from either hereditary optic neuropathy (n = 11) or glaucoma (n = 11). We measured melatonin suppression, subjective sleepiness and cognitive functions in response to bright light exposure in the evening. We also quantified the post-illumination pupil response to a blue light stimulus. All results were compared to age-matched controls (n = 22). Both groups of patients showed similar melatonin suppression when compared to their controls. Greater melatonin suppression was intra-individually correlated to larger post-illumination pupil response in patients and controls. Only the glaucoma patients demonstrated a relative attenuation of their pupil response. In addition, they were sleepier with slower reaction times during nocturnal light exposure. In conclusion, glaucomatous, but not hereditary, optic neuropathy is associated with reduced acute light effects. At mild to moderate stages of disease, this is detected only in the pupil function and not in responses conveyed via the retinohypothalamic tract such as melatonin suppression.