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The chapter investigates the most relevant legislative transformations affecting Italian regime of public finance in the last 50 years. Moving from the effects caused by the introduction in 2012 of the balanced budgetary rule in the Constitution, it deals with the impact of this rule on both the accounting standards and the local finance.

In and of itself, public debt produces no redistribution to the detriment of future generations. In point of fact, it is the way pertinent resources are spent that brings about such effects. Indeed, under certain conditions, public debt allows the sacrifices imposed on each generation to coincide with the benefits accrued to the generation itself.

The initiatives taken by the Union after the Lisbon Treaty to deal with the economic crisis of recent years, have questioned some of the principles of European economic governance, or at least they have modified the terms of implementation, thus demonstrating their inadequacy in these economic circumstances and how illusory it was to consider some of them as irrevocable fundamental points. However, there are still some aspects that make the changes made so far insufficient: in particular, the central importance attached to budget restraints, the focus on the need to prevent the production of negative externalities by the States, and the sterilization of politics that is enmeshed in a web of rules designed to neutralize conflicts that exist only because Europe is underpinned by an intergovernmental logic.

Introduction We investigated the clinical differences between familial and sporadic frontotemporal dementia (FTD), screening for mutations in known FTD genes. Methods We diagnosed 22 affected individuals belonging to eight families and 43 sporadic cases with FTD in Apulia, Southern Italy, in 2 years. Mutations in common causative FTD genes ( GRN , MAPT , VCP , and TARDBP ) and C9ORF72 expansions were screened. Results Behavioral variant of FTD was the most common clinical subtype (50% and 69% in familial and sporadic cases, respectively). Social conduct impairment/disinhibition, loss of insight, and inflexibility were the most frequent clinical features observed at onset. One new mutation was identified in GRN in family A. Discussion Disease onset in sporadic FTD was more frequently characterized by a clustering of behavioral symptoms with apathy and loss of personal hygiene. Mutations in common causative FTD genes are not a major cause of familial and sporadic FTD in the Southern Italian population.

Her work analyses some modes of urban representation in the French culture of the second half of the eighteenth century. Two types of intellectual operations, description of reality and imagination of a better alterity, are combined to give voice to an increasingly lively critique of eighteenth-century cities, between reformist momentum and utopian spirit. The focus of the work is on the properly architectural urban representation, which was explored by Étienne-Louis Boullée (1728-1799) and Claude-Nicolas Ledoux (1736-1806). Their writings are unique contributions of a specific kind of Enlightenment and give shape to city models created by the union of architectural reform and transformation of the community.

Background: Infections of the central nervous system (CNS) are a difficult diagnostic problem for both clinicians and microbiologists. Various clinical signs, such as encephalitis, myelitis, meningitis, may be associated with herpesviruses. The use of multiplex ‘Herpes Consensus’ polymerase chain reaction (HC-PCR) in association with nested PCR (nPCR), in addition to classical techniques, made it possible to optimise the management of cerebrospinal fluid (CSF) and serum samples from patients affected by these viral diseases of the CNS. Objectives: To test by HC-PCR by nPCR and cell culture the CSF and sera from patients with viral infections of the CNS. Study design: We analysed 320 CFS, 154 serum samples and 11 various samples from 286 patients with clinically suspected encephalitis, meningitis or other diseases of the CNS by HC-PCR, nPCR and traditional investigations (cell culture and serological tests). Results: On molecular analysis with the HC-PCR test, 51 CFS samples (15.9%) were positive for at least one of the six target Herpes viruses: fourteen for Herpes simplex 1 (HSV-1), seven for HSV-2, 12 for Cytomegalovirus (CMV; one of which was from an HIV-positive patient), five for Epstein-Barr virus (EBV; four of which were from HIV-positive patients), three for Varicella-Zoster virus (VZV), five for Human Herpes virus type 6 (HHV-6), three for HSV-1 with HHV-6 co-infection (two cases) and HSV-2 co-infection (one case), and two for HHV-6 with CMV or EBV co-infection (both from patients with immune deficiency). A further 12 samples were positive in nPCR for HHV-7 (8), ADV (1), Enterovirus (1), HSV-1 (1), EBV (1). Of the 154 serum samples, 17 (11.0%) tested positive by HC-PCR for HSV-1 (4), HSV-2 (1), CMV(1), EBV(1), VZV(3) or HHV-6(6), 1 with co-HSV-2/VZV infection. A further five samples tested positive for HHV-7 in nPCR. Culture and tests for antibodies did not supply sufficiently sensitive and specific data. Conclusions: Our laboratory experience shows that herpesviruses play a central aetiological role in viral infections of the CNS. PCR analysis, especially the HC-PCR test, have revolutionised the diagnostic approach to such infections, making possible rapid, specific and highly sensitive baseline screening. In this way, microbiological investigations can lead to prompt diagnosis, which was limited in the past to a very small number of cases.

Composition du jury : Directeur de thèse, Jacques Revel (directeur de thèse) ;Président du jury, Renato Pasta ;Membres du jury, Antoine Lilti et Rolando Minuti (directeur de thèse).Discipline: Histoire et civilisations. Thèse soutenue en cotutelle avec l’Università degli Studi di Firenze (Italie) le 20 mai 2013 à Paris. Résumé : Cette recherche vise à explorer les possibilités de représentation urbaine dans la France de la deuxième moitié du xviiie siècle, et notamment dans la culture archit...

Relazione tenuta al Convegno su “Federalismo ieri oggi e domani” organizzato dalla Facoltà di Scienze Politiche dell’Università degli studi di Urbino “Carlo Bo” in occasione del decennale della Facoltà, Urbino, 21-22 novembre 2003., Studi Urbinati, A - Scienze giuridiche, politiche ed economiche, V. 57, N. 2 (2006)

Cromohs - Cyber Review of Modern Historiography, Vol 17 (2012)

Myotonic dystrophy type 1 (DM1) is caused by a CTG expansion in the myotonic dystrophy protein kinase (DMPK) gene [1]. Although DM1 is a multisystemic disease, the involvement of peripheral nerves is controversial [1]. The development of autoimmune diseases is also largely debated; no co-occurrence of inflammatory neuropathy and Hashimoto thyroiditis (HT) has been reported. We describe the sporadic case of a 67-year-old man presenting with gait disturbance, diffuse muscle weakness progressively worsening in the previous 5 years. Clinical history revealed the diagnosis of bilateral cataract and extrasystolic cardiac arrhythmia. On admission, patient’s ambulation was ataxic and needed unilateral support. Neurological examination revealed myotonic face, slightly nasal speech, weakness and atrophy in neck and facial muscles, diffuse muscle weakness and hypotrophy of the limbs, more prominent in the scapular girdles and in the distal lower limbs. Grip-release myotonia was evocable. Deep tendon reflexes were sluggish; plantar responses were flexor. Vibration sense was markedly impaired, whereas pain and touch sensation was normal. Complete blood count, HgbA1C (4, 3%; normal range: 2.25%) and glucose tolerance test (129 mg/dl 2 h after a 75-g glucose drink; normal range: 140 mg/ dl), liver and kidney function tests, cobalamin, folate, lipid profile, creatine kinase, antigangliosides antibodies and immunofixation were normal. Free T4 was slightly decreased (0.82 ng/dl; normal range: 0.9–1.7 ng/dl), free T3 was normal and TSH (4.3 mUI/l; normal range: 0.3 –3.6 mUI/l), antithyroglobulin (TG) (150.8 UI/l; normal range: 5–100 mUI/l), antithyroperoxidase antibodies (TPO

We assessed the validity of an Italian language version of the Epworth sleepiness scale (ESS). The translated ESS was compared to the multiple sleep latency test (MSLT), considered the gold standard for the diagnosis of excessive daytime sleepiness (EDS). Within the context of a multicentric national study on narcolepsy (Gruppo Italiano Narcolessia Studio Epidemiologico Nazionale, GINSEN) involving 17 Italian sleep centres, we compared the two diagnostic tests on 91 prospectively recruited subjects with suspected EDS (34 with narcolepsy, 16 with obstructive sleep apnea syndrome, 19 with idiopathic hypersomnia, and 22 with other sleep, neurologic or psychiatric disorders). ESS scores were inversely correlated with mean sleep latency values, as measured with MSLT (rho = −0.31, p

Two groups of 10 patients with Parkinson's disease received doses of either 1g acetyl-L-carnitine (ALC) per day for seven days or 2g. The effects of this drug on intermittent luminous stimulation and on nocturnal sleep patterns were studied. In both cases with either dose of ALC the effect was an improvement of the H response, sleep stages and spindling activity. However a further study of the complexity of action of acetyl-L-carnitine is necessary.

Marked adeno-tonsillar hypertrophy is certainly the most common respiratory disturbance found among children. Most particularly obstructive sleep apnea (OSA) leads to serious, at times fatal, consequences on the cardio-respiratory apparatus. Thus opinion in the literature unanimously holds that said syndrome is one precise indication for tonsillectomy and/or adenotomy. The present case study was performed on 19 children, age range 21 months to 6 years, with serious obstructions of the respiratory tract due to tonsillar or adeno-tonsillar hypertrophy (14 cases) or to isolated adenoid hypertrophy (5 cases). The sample was divided into 4 groups, taking into consideration the severity of the nighttime sleep obstruction and the type of obstruction. Polygraphy performed during sleep recorded objective OSA in 10 subjects with tonsillar or adeno-tonsillar hypertrophy, but no case was recorded in any subject with solely adenoid hypertrophy. The severity of the obstruction was likewise shown, judged both on the basis of frequency and duration of the pauses as well as on the cardio-respiratory complications, especially in the smallest children (less than 3 years). Furthermore, in the most serious cases significant hematological alterations were recorded. Tonsillectomy, performed in 9 of the 10 children with OSA, completely resolved the sleep disturbances and improved, or normalized, the broncho-pneumonic picture as well the hematological findings in those cases where preoperative complications were present. Even for those children who did not manifest OSA, tonsillectomy and/or adenotomy led to a more uniform rhythm and quality of their sleep.(ABSTRACT TRUNCATED AT 250 WORDS)

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