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Stiff person syndrome is a rare condition characterised by prolonged stiffness with superimposed muscle spasms. Immunotherapy relies mainly on intravenous immunoglobulin, steroids and plasma exchange. Azathioprine or rituximab are other possible options. We describe two patients who showed a good clinical response with intravenous immunoglobulin and persistence of the clinical improvement after shifting to equivalent dosage of subcutaneous immunoglobulin. Both patients received a diagnosis of stiff person syndrome based on their clinical symptoms (episodes of stiffness and spasms) and presence of antiglutamic acid decarboxylase. Treatment with intravenous immunoglobulin was started with improvement of symptoms as reported by patients and confirmed also by the spasm frequency scale and modified Ashworth scale. After clinical stabilisation in order to avoid the hospitalisation required for intravenous immunoglobulin treatment a switch to subcutaneous immunoglobulins was made. After one year of follow-up from the switch, the patients show clinical stability. Their scores on the modified Ashworth scale, spasm frequency scale and on the 10 Meter Walking Test were also stable. Subcutaneous formulation of immunoglobulin could be as effective as intravenous immunoglobulin in the maintenance treatment of Stiff person syndrome, although studies involving a larger cohort of patients are needed in order to confirm our anecdotal experience.

An ice crossing is a road constructed on an ice-covered river, organized in the absence of a bridge, as well as in view of the impossibility of a ferry crossing in winter due to the formation of ice cover on water bodies. For most of the regions of Russia, in the cold season, the establishment of negative temperatures is typical, as a result of which ice crossing is a common transport facility. So, in the country annually opens more than 500 crossings, about 20 of which are located in the Perm Krai. Their opening takes place in December - early January, and closing - in late March - early April. The easiest way to arrange crossings is on natural ice, as a result of which their carrying capacity increases as the natural thickness of the ice increases. Because of this, the passing of heavy vehicles is possible only in January – February, which is a significant drawback. Today, there are several ways to artificially increase the carrying capacity, which include reinforcing the ice sheet with geosynthetic material. This method is a new and rather promising direction in road construction due to the reusable use of geomeshs. The article discusses the dependence of changes in the characteristics of the ice cover on the applied geosynthetic material, as well as technological and economic comparison of reinforcement variants. To this end, materials were collected on organized crossings of the Perm Krai, possible types of geomeshs were examined, and calculations were carried out to determine the carrying capacity of the crossing. This reinforcement method contributes to the earlier passage of heavy vehicles at the crossing, and also improves the safety of traffic on it and prolongs its service life.

The article contains the results of research on the development of optimal in terms of time and complexity of work, energy intensity, consumption of materials and cost, the design of the roadbed for use in the construction of oilfield roads in wetlands. Trunk oil and gas pipelines in Western Siberia and north of the European part of Russia began to be built approximately from the mid-1960s. From the very beginning, designers faced serious problems: complex natural and heavy ground and hydro geological conditions, namely, permafrost soils and swamps. The length of the swamp sections is tens and hundreds of meters, and in some cases – tens of kilometers. Road construction in wetlands is a very difficult engineering task. Conditions of excessive moisture, extremely low load-carrying capacity of marsh soils, a characteristic deficiency of quality mineral materials - all this creates obstacles, the overcoming of which is associated with significant material costs. The average cost of road construction through the swamp is 3-5 times higher than in normal conditions. A constructive solution for the roadbed using a layer of non-woven synthetic material in the cage to ensure year-round and uninterrupted movement of technological transport for maintenance of oil facilities was chosen.

Nowadays, the age of most transport facilities in Russian Federation is estimated in many decades. Consequently, there is an urgent need for periodic surveys and monitoring of the state of each structure, its individual structural elements. The main purpose of these measures is to maintain the high performance indicators of the transport structure, the achievement of which is impossible without examination, analysis and evaluation of parts of the structure that are in the aquatic environment and, as a consequence, the occurrence and development of various types and degrees of defects and damages. The most important conditions for ensuring uninterrupted and safe traffic along the bridges of vehicles and pedestrians are: the normative load-carrying capacity of the structure; required clearance of the roadway and sidewalks; compliance with traffic safety requirements for bridges; observance of characteristics and maintenance of the bridge capacity for the period of ice drift and high water; timely performance of work on the care, maintenance and repair of the elements of the bridge. Technically sound structures and their reliability can be ensured if all the parameters of the road category structure and the requirements of regulatory documents are met. The work presents the survey materials of the bridge. Classification of defects by group and class of danger. The results of determining the carrying capacity on the basis of the survey are given.

The conditions of the impact of wind waves on the soil upland slopes of retaining structures and natural banks, which are fastened in the form of reinforced concrete slabs of the covering with deformations, are considered. The results of the analysis of field examinations of the state of reinforced concrete fastenings of slopes and banks of artificial water bodies under the influence of wind waves are presented. A criterion is proposed for assessing the stability of soil slopes and coastal slopes with reinforced concrete fasteners with a broken structure, and the conditions for its applicability are determined.

Article presents a version of textile sand piles application in road construction for road bases arranged on saline soils. Construction of roads in the areas of saline soils occurrence is a complex multidimensional problem, which includes study of soil properties and conditions of their education taking into account specific features of road constructions and features of excavation production technology. In the case of preservation of saline soils in the subgrade it is required to provide stability and reduce the time of accumulation of possible drafts of road the structure. In practice, these conditions can be realized through the use of design and technological solutions, namely, sand piles devices, slots or horizontal surface pillows, precast reinforced concrete piles with a flexible raft of geotextile vertical crushing drainage strips and mounds from polystyrene plates, soil-concrete piles, as well as strengthening the implementation of deep saline soils by mechanical mixing with binders. The calculation of the developed roadbed construction using textile-sand piles to further reduce of cost and timing of the road construction on saline soils was made.

At present, the age of most transport facilities in the Russian Federation estimated by many decades, and most require reconstruction or repair. Consequently, there is an urgent need for periodic surveys and monitoring of the condition of such facilities. The main goal of these measures is to maintain high rates of transport infrastructure efficiency, the achievement of which is impossible without examining, analyzing and evaluating parts of the structure in the aquatic environment, and, as a result, the occurrence and development of various defects and damage. The most important conditions for ensuring uninterrupted and safe traffic on the bridges of vehicles and pedestrians are the regulatory bearing capacity of the structure and cleaning of the roadway and sidewalks. Then compliance with traffic safety requirements on decks and observing the characteristics and maintaining the function of the bridge for the period of ice drift and flooding. Timely execution of works for the care, maintenance, and repair of bridge elements. Technically stable structures and their reliability can be ensured by observing all the parameters of the structure of the road category and the requirements of regulatory documents. The paper presents the materials of the inspection of the bridge after repair — defect classification by groups and hazard classes — the results of determining the carrying capacity of the survey materials given.

Culvert pipes are one of the largest categories of engineering structures on roads of both regional and federal significance. During operation, the structure is affected by static and dynamic loads, for example, from the weight of the dam and passing vehicles, temperature changes, melt, and rainwater is directly flowing through the pipe, streams and small rivers, as well as geological factors - earthquakes, shifts, and vibrations ground. Also, over time, the installed reinforced concrete sections of the conduit have a habit of shifting in horizontal and vertical directions under the influence of various kinds of loads, which leads to the formation of gaps and differences between them. Swings reduce the effective cross-section of the pipe, throughput, and lead to silting. Under flood conditions, the presence of discrepancies between the links of the tube leads to significant erosion, leaving voids in the dam, which is fraught with the collapse of the roadbed and road pavement, and in such situation, it is impossible to talk about the possibility of road operation as a whole. The traditional effective solution to this problem is culvert repair. Identifying the need for repair or reconstruction of the structure is the primary purpose of assessing the technical condition during the professional inspection of culverts.

Road construction machinery is one of the largest and stable sectors of national economy. With the help of road-building machinery, construction, maintenance and repair of highways are carried out. Modern system of road and building machines is a complex of high-performance machines and mechanisms, of large and small capacity and productivity. Expansion of paved roads network of both federal and local values, use of resource-saving technologies, increasing of pace and quality of work, ensuring of reliability and durability of highways have a major impact on the development of road construction machinery. The level of foreign road and construction machinery puts forward complex requirements for compliance with world safety and environmental standards, increasing equipment mobility, expanding areas of effective use and, finally, increasing its capacity and productivity. Article contains approach to the calculation of the operational productivity of asphalt pavers that takes into account technical characteristics and conditions of works production and contributes to the reliability of decisions on organization and management of road maintenance works on urban roads.

Objective : To investigate cortical excitability in patients with corticobasal degeneration (CBD) and to find a reliable diagnostic technique for differentiating CBD from Parkinson's disease (PD). Methods : Using a paired transcranial magnetic stimulation technique, we studied motor cortex excitability at rest in 6 patients with clinically probable CBD, 10 patients with PD, and 10 normal subjects. The recovery cycle of the motor evoked potentials was tested by delivering paired magnetic stimulation over the hand area of the motor cortex at interstimulus intervals (ISIs) from 1 to 17ms. Results : In patients with CBD, paired magnetic stimuli delivered at short ISIs invariably elicited enlarged test MEPs. At ISIs of 1–10ms, the conditioned test MEPs were significantly larger in patients with CBD than in control subjects; and at ISIs of 1, 2, 4, and 6ms ,they were also larger in patients with CBD than in patients with PD. At the other ISIs tested, patients and control subjects had similar amplitude conditioned test responses. Conclusions : Our findings suggest that the unusual clinical manifestations of CBD might arise partly from motor cortex disinhibition. Paired magnetic stimulation could be a useful diagnostic test particularly in the early stages of the disease.

Intracranial hemorrhage is an infrequent but potentially devastating complication associated with the placement of electrodes for deep brain stimulation (DBS). The objective of this retrospective review is to compare the risk of hemorrhage before and after the introduction of image-guided neuronavigation for the implant of electrodes for DBS.We reviewed all DBS implant performed at our Institute between 1998 and 2010. In 63 of the 106 patients, the targeting was based on ventriculography and merge of CT/MRI. After 2006, in the latter 43 procedures, we introduced targeting based on the merging of angio CT and MRI.. In both implant techniques, microelectrode recording (MER) was used to better define the target. All DBS procedures were performed by a single surgeon (M.P.). Patients had postoperative imaging (MRI or CT) 4-24 h following surgery.In the group of patients implanted with the first targeting technique, 3 hematomas occurred and all of them solved with sequelae (one residual weakness and two hemiplegias.) After the introduction of neuronavigator, 2 hemorrhages occurred, one solved without sequelae while the second resulted in epileptic seizures.Although the incidence of hemorrhage occurred before and after the use of neuronavigation is the same, the severity is lower in the neuronavigated procedures. Targeting based on the merging of CT angiography and MRI T1/T2 seemed to increase the safety of the lead placement reducing the risk of sequelae related to bleeding. The use of MER was not found to be correlated with an increased hemorrhage rate.

Homocysteine increases in the acute phase of ischaemic stroke and from the acute to the convalescent phase, suggesting that hyper-homocysteinaemia may be a consequence rather than a causal factor. Therefore we measured homocysteine plasma levels in stroke patients in order to investigate possible correlations of homocysteine with stroke severity and clinical outcome. Further we looked for eventual differences in stroke subtypes. We prospectively studied plasma homocysteine levels in acute stroke patients admitted to the stroke unit of our department. Seven hundred and seventy-five ischaemic stroke patients, 39 cerebral haemorrhages and 421 healthy control subjects have been enrolled. Stroke severity and clinical outcome were measured with the Scandinavian Stroke Scale, the Rankin Scale and the Barthel Index. Stroke severity by linear stepwise regression analysis was not an independent determinant of plasma homocysteine levels. Homocysteine was not correlated with outcome measured by the Barthel Index. Mean plasma homocysteine of both ischaemic and haemorrhagic stroke was significantly higher than controls (p

The 3-month clinical follow-up showed a significant reduction of UPDRS III score in the group with neurophysiological monitoring (-38%, p

We describe the clinical and neuropathological findings of three unrelated autopsy cases of MELAS harboring the A3243G transition in the mitochondrial DNA (mtDNA). Using immunohistochemical techniques, we studied the expression of several subunits of the respiratory chain in various brain regions from the same cases. In all three cases there was a reduced immunocytochemical staining for mtDNA-encoded subunits of the respiratory chain, confirming the presence of a defective mitochondrial protein synthesis in this disease. Mitochondrial abnormalities were mostly confined to multiple areas of different size and shape, in agreement with the focal character of the brain pathology in MELAS, and were most prominent in the cerebral cortex, providing a morphological contribution to the explanation of the cognitive regression of the patients. Immunoreactivity for mtDNA-encoded subunits was reduced in the walls of many pial and intracerebral arterioles of different brain regions but there was no clear correlation between territories of affected vessels and distribution of the histological and immunohistochemical lesions. Cerebral focal lesions in MELAS might have a metabolic nature and several pathogenetic mechanisms might be involved in the genesis of stroke-like episodes when there is a local increased ATP demand.

Twenty-nine young patients with insulin dependent diabetes were studied. Clinical findings, metabolic profile, and neurophysiological data were correlated. Nerve conduction velocity (NCV) and Somato-sensory Evoked Potential (SEPs) were recorded stimulating the median nerve and the posterior tibial nerve bilaterally. High incidence of distal neuropathy was present in our cases plus a significant alteration of the plexus conduction, at the level of brachial plexus and of the cauda. We conclude that even in young diabetic patients the damage can be multifocal, and SEP technique could contribute to a more accurate study of neurological complication in the diabetic disease.

Circumscribed unilateral paralysis of abdominal muscles is a rare clinical feature and has previously been described in diabetic neuropathies and traumatic or non-traumatic compressive neuropathies. The paper describes a case presenting with transversus abdominis muscle paralysis and burning paraesthesia in the anterolateral aspect of the thigh caused by a lateral L2-L3 disc herniation. Abdominal echography and the EMG investigation led to the suspicion of a disc prolapse which was eventually verified by myelography completed with a CT scan. Surgery confirmed L2 root compression by a large calcified herniation in the intervertebral foramen L2-L3. The anatomical principles are recalled to explain the clinical manifestations.

Since a few years the technique of evoked potentials (PE), both visual (VERs) and acoustic (BAERs), of the encephalic trunk has been introduced in the diagnosis of SM. The electrophysiologic data thus obtained are usefully completed by liquor's ones, often allowing a precocious diagnosis of this disease. In this work the authors compare the results of liquor analyses (dosage of the relation IgG/total proteins; electrophoretic research of Oligoclonic Bands) with those of the PE in 28 patients with suspect SM. The high positive percentages of BAERs (78.8%) and of VERs (90%) in certain multiple scleroses, as well as in suspect multiple scleroses (53.6% and 82.1% respectively), prove the high sensibility of these techniques. The positive percentages of liquor analysis appear to be slightly inferior as to those of evoked potentials, even if these differences are not statistically significant. Besides the authors noticed a high degree of accordance between PE and liquor anomalies (in the cases of defined SM the patients with altered liquor presented at least an altered PE).

The possible antiepileptic activity of PS has been tested in a model of generalized epilepsy induced by penicillin in the cat. In acute experiments, topical application to the cortex of PS (from 20 mg/ml to 50 mg/ml) produced a reverse in the polarity of the spike and wave complex and, sometimes, a decrease in the voltage as well as a morphological deformation of s-w bursts. Chronic intraperitoneal administration of PS induced a decrease of the epileptic burst frequency. The effect was already apparent after 14 days and of greater extent after 21 days. Finally the antiepileptic effect of 0,2-0,5 mg/Kg diazepam, given in a single dose during the maximal epileptic activity resulted markedly reinforced in animals chronically treated with PS.

Recent changing in funerary architecture in Italy

Architetture che permettono un approfondimento della visita e un concreto "esperire" il territorio e promuovono una visione diversa dello stesso manufatto, come vero utensile atto alla scoperta dei luoghi

rivolgimenti contemporanei nell'architettura funeraria con speciale attenzione alla emersione delle Case Funerarie in Italia.

presentazione e critica al progetto di Isozaki per la nuova biblioteca comunale di Maranello

nuovi sviluppi architettura cimiteriale tra utopia e speculazione

come si orienta l'urbanistica in città in tempo di crisi.

intervista all'assessore alla urbanistica Patrizia Gabellini nell'ambito della inchiesta che ho curato sulla città di Bologna per Il Giornale dell'Architettura (n100)

descrizione del piano Gabrielli per il Centro Storico di Bologna, durante il Commissarriamento di Anna Maria Cancellieri

inchiesta sulla città di Bologna e sulle più recenti trasformazioni nel suo corpo architettonico e le scelte urbanistiche

1.La concezione nella quale per la gran parte siamo ancora immersi, di un fare architettura come di una operatività artistica di tipo romantico, favorisce l'originalità dell'atto creativo, la provocazione, l'individualità per differenziazione dell'oggetto architettonico ma al contempo scoraggia la didattica del comporre, implicitamente considerando innate capacità individuali o pregresse conoscenze culturali irrinunciabili premesse alla buona capacità di progettare. 2.Una nuova e conseguente reticenza in ambito tecnico-architettonico all'uso della parola "bellezza", ha portato il giudizio sulla architettura ad essere piuttosto di natura prestazionale e normativa, senza considerare però che, se un oggetto può essere giudicato a posteriori in base alla sua rispondenza a parametri quantitativi, viceversa alcun parametro quantitativo sarà mai sufficiente a determinare un oggetto. 3. Riprendere il concetto d’architettura come aspetto dell’arte non significa, rifiutare l’apporto della tecnica e dell’ingegneria. Al contrario le tecniche, le tecnologie, fino al virtuale e alle reti sono la nuova materia plastica a disposizione del progettista che pertanto deve conoscerne le leggi, . Entrando nel dibattito antico se l’architettura sia o non sia un aspetto dell’arte per il fatto di essere contaminata dal corrispondere ad una funzione, occorre ribadire l’essenzialità dell’abitare alla sua definizione, ovvero il carattere intrinseco dell’abitare all’architettura. Per questa via [cfr. S. Bettini; G. Klaus Koenig] si comprende l’architettura come “forma dell’essere”, o come “forma dell’abitare” [cfr. M. Heidegger], la cui materia è, come per la scultura, la pittura e, particolarmente, la musica, l’umano. 4. In ragione di queste considerazioni si elidono le distanze tra l’antico architetto ed il moderno edile.Tanto colui che si pone all’origine del processo determinandone le ragioni compositive (archi – tek-ton), tanto colui che lo intraprende in ragione del suo fine ultimo e concreto (la casa, l’aedesedile) agiscono mescolando due mondi: da un lato quello “delle idee” o delle intime proprie rappresentazioni dell’umano (A) , dall’altro quello della materia, delle tecniche e delle tecnologie attraverso le quali concretizzeranno il proprio prodotto finale (B) che sarà tanto più riuscito quanto corrisponderà essenzialmente ed esistenzialmente all’uomo che ne è dunque la causa finale. 5. Tra il termine (A) ed il termine (B) si snoda dunque il processo compositivo. E’ dunque nella relazione tra MONDO 1 e MONDO 3 [cfr. K. Popper] che interviene la dinamica del processo di composizione architettonica. Se si ritiene che il giudizio estetico intervenga solo sul prodotto finale, del tutto indipendentemente dal processo compositivo che è intervenuto a plasmare l’oggetto, si ricade sostanzialmente in una concezione romantica [cfr.KANT, Critica del Giudizio], per cui educare alla autentica libertà dell’atto compositivo è impossibile ed il solo criterio diviene l’adesione a un canone ideologicamente istituito e assunto come valido e “bello”. Paradossalmente il carattere storico o estemporaneo con il quale ogni scuola di architettura può in questo modo ideologicamente eleggere l’unicità del proprio riferimento ed il carattere assoluto di ciò che si stima come “bellezza”, dimostra al contrario tutta la relatività di un giudizio estetico che riposa, infine, solo in un giudizio di “stile” e non in una valutazione di processo. In altre parole, se le forme della bellezza possono essere molte, ciò mediante cui tali forme si determinano sono processi dalle caratteristiche ricorrenti che individuano processi di coerenza tra il termine (A) e il termine (B), tra Mondo 1 e Mondo 3. 6. Indipendentemente dal linguaggio architettonico, il giudizio estetico si determina dunque già in fase di processo compositivo. Questa concezione permette ed anzi istituisce la necessità di una didattica del progetto non solo in senso tecnico, qua...

The contemporary global conceivement leads “making Architecture” to an artistic work in some way still linked to romantic philosophy. This favors the originality of the creative act, the provocation, implicitly considering innate abilities or previous cultural knowledge essential requisites to a good ability to plan. A new and consequent reserve in the use of the word “BEAUTY” in the technical-architectural area has narrow the judgment on architecture to more strictly quantitative parameters concerning the Energy efficiency, and others performative levels, without considering that, if an object can be judged according to quantitative parameters, viceversa NO quantitative parameter will be ever enough to determinate an object. Reconsidering architecture like a facet of Art does not mean rejecting the contribution of technical and engineering. On the contrary techniques, technologies, and virtual networks are the new plastic material in the availability of the designer, who has to know their laws and potentialities. Entering in the ancient debate whether architecture is or is not part of the good-arts because of its contamination to a specific utility and a proper function, we should underline once more the essentiality of “dwelling” to its definition and to Dwell like the most inner and intrinsic character of Architecture. In this way [cf. S. Bettini, G. Klaus Koenig] we can understand architecture as a "form of being," or "form of living" [cf. M. Heidegger] whise matter is, as sculpture, painting and, especially, music, the human being himself. we can elide now the gap between the ancient architect and the modern building engineer. Both the one who is at the origin of the compositional architectural process, determining its reasons (arché - tek-ton), as the one who embarks on it by reason of its last end (the house, the aedes) do act mixing two worlds: on one hand the one of "ideas" or of their intimate representations of the human being (A), on the other that of matter, techniques and technologies through which they can materialize their final product (B) which will be more successful as much as it will match essentially and existentially to the man for which this process has started for. It is therefore in the relationship between World 1 and World 3 [cf. K. Popper] that intervenes the typical dynamics of the process of architectural composition. If one thinks that the aesthetic judgments intervenes only on the final product of this project, quite apart from the compositional process that intervened in shaping the object, it is inevitable to fall in a substantially romantic conception [cfr.KANT, Critique of Judgement], and educating for an authentic freedom in the act of architectural composition is impossible. The only possible criterion is then to join ideologically a well-established and already accepted rule as valid and "beautiful". If forms of beauty can be many, what through which these forms are determinated by, reveals to be a process characterized by recurrent features that is a consistent processes from the term (A) to the term (B), between World 1 and World 3. Regardless to the architectural “language”or style, the aesthetic evaluation is determined, therefore, already during the process of composition. This idea establishes the necessity of an education to the project, not only in the technical sense, but even in an aesthetic sense, as a way of consistency to the materialization and specification of a concept.

limiti degli attuali sistemi urbanistici per la gestione delle trasformazioni architettoniche e funzionali del territorio di Ravenna

presentazione dell'intervento dell'Architetto Paolo Bedogni sul restauro del Monastero delle Clarisse di Sant'Agata Feltria (PU)

Biografia dell'architetto Bolognese ENZO ZACCHIROLI, in prossimità della sua scomparsa

Articolo breve intorno alle condizioni precarie del PAlazzo Orsi-Marconi a Bologna

INTRODUZIONE AI PROGETTI DI SALE PER IL COMMIATO CHE OGNI MESE SI è CONVENUTO DI PRESENTARE SULLA RIVISTA "OLTRE MAGAZINE" COME RISULTATO DEL LABORATORIO PROGETTUALE INTRAPRESO SULLE FORME CONTEMPORANEE DELL'ARCHITETTURA SACRA.

critica ad ua opera di arredo urbano a Bologna

relazioni tra luogo dell'abitare e valore di appartenenza della cittadinanza

commento al nuovo ingresso della porzione ebraica del cimitero monumentale della Certosa a Bologna

presentazione del piano di valorizzazione museale della città di Bologna ad opera della Fondazione Carisbo

restuaro di una porzione della Sinagoga ebraica di Bologna, anche al fine di attività di ristorazione

note intorno ad una rifunzionalizzazione dello spazio di culto per la città di Bologna, problematiche di resturo e gestione.

la relazione tra processo di composizione architettonica e i mezzi con cui questa si attua nel passaggio da tecniche manuali a quelle digitali

notizia breve in relazione al più grande studentato della città

articolo breve sul restauro condotto dall’arch. Paolo Bedogni sul Monastero di Santa Maria Maddalena di Sant’Agata Feltria

Articolo relativo ai resaturi annunciati alle principali Chiese di Bologna

Introduzione al paesaggio e alle chiese dell'alta valle del Reno nella Diocesi di Bologna