555 research outputs found
Metalloproteinase alterations in the bone marrow of ALS patients
No full textAmyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease, nowadays considered as suitable candidate for autologous stem therapy with bone marrow (BM). A careful characterization of BM stem cell (SC) compartment is mandatory before its extensive application to clinic. Indeed, widespread systemic involvement has been recently advocated given that non-neuronal neighboring cells actively influence the pathological neuronal loss. We therefore investigated BM samples from 21 ALS patients and reported normal hematopoietic biological properties while an atypical behavior and impaired SC capabilities affected only the mesenchymal compartment. Moreover, by quantitative real-time approach, we observed altered Collagen IV and Metalloproteinase-9 levels in patients' derived mesenchymal stem cells (MSCs). Widespread metalloproteinase (MMPs) and their tissue inhibitor (TIMPs) alterations were established by multiplex ELISA analysis, demonstrating diffuse enzymatic variations in MSC compartment. Since MMPs act as fundamental effectors of extra-cellular matrix remodeling and stem cell mobilization, their modifications in ALS may influence reparative mechanisms effective in counteracting the pathology. In conclusion, ALS is further confirmed to be a systemic disease, not restricted to the nervous system, but affecting also the BM stromal compartment, even in sporadic cases. Therefore, therapeutic implantation of autologous BM derived SC in ALS patients needs to be carefully reevaluated
Functional alteration of bone marrow mesenchymal stem cells of Amyotrophic Lateral Sclerosis Patients
No full textIntracerebral haemorrhage, a possible presentation in Churg-Strauss syndrome: case report and review of the literature
No full textChurg-Strauss syndrome (CSS) is a rare systemic vasculitis, almost invariably accompanied by asthma, nasal polyposis, paranasal sinus abnormalities, and increased peripheral blood eosinophil count. Neurological involvement as peripheral neuropathy is a common feature, whereas cerebral involvement is extremely rare. Herein, we report the case of a young man who presented with sudden onset of right-side emiparesis and aphasia, whose head CT scan showed the presence of large haemorrhage in the left striatum nucleus involving part of the temporal lobe. Based on clinical and laboratory findings (asthma, eosinophilia > 10%, paranasal sinus abnormalities and mononeuritis multiplex) a diagnosis of CSS was made. Cerebral angiography resulted normal, excluding the presence of vascular malformations or signs of vessel abnormalities. Pharmacotherapy with (intravenous and afterwards oral) corticosteroid and immunosuppressors (cyclophosphamide and then azathioprine) was initiated. The outcome was good with neurological follow-up showing a nearly complete recover. Our case points out that intracerebral haemorrhage can be, despite rare, a presenting feature of CSS. Previously reported patients affected by cerebral haemorrhage and CSS are summarized and briefly reviewed
Ariprazole in the treatment of Huntington's Disease. A case series.
No full textObjectives: The aim of the study was to describe the effects of aripiprazole, a new atypical antipsychotic drug that acts as a partial dopamine agonist on motor, behavioral and cognitive functions in patients with genetically confirmed Huntington's disease (HD). Methods and results: Three HD patients were evaluated for Unified Huntington Disease Rating Scale part I and II and Beck Depression Inventory at baseline, after two months and one-year treatment. Aripiprazole effectively controlled involuntary movements and psychiatric symptoms, with effects on cognitive functions. Conclusions: Our case reports suggest that aripiprazole is well tolerated, remarkably improving some of the motor and behavioral symptoms in patients affected by HD. Randomized, controlled, long-term studies are warranted
Oligoclonal bands in the cerebrospinal fluid of amyotrophic lateral sclerosis patients with disease-associated mutations
No full textIn amyotrophic lateral sclerosis (ALS) cerebrospinal fluid (CSF) analysis is usually performed to exclude inflammatory processes of the central nervous system. Although in a small subset of patients an intrathecal synthesis of IgG is detectable, usually there is no clear explanation for this evidence. This study investigates the occurrence of oligoclonal bands (OCBs) in the CSF of a large series of ALS patients, attempting a correlation with genotype data. CSF was collected from 259 ALS patients. CSF parameters were measured according to standard procedures, and detection of OCBs performed by isoelectric focusing. The patients were screened for mutations in SOD1, FUS, TARDBP, ANG, OPTN, and C9ORF72. We observed the presence of OCBs in the CSF of 9/259 ALS patients (3.5 %), and of disease-associated mutations in 12 cases. OCBs were significantly more frequent in mutation carriers compared to the remaining cohort (3/12 vs 6/247; p < 0.01). Among patients with OCBs, two patients had the TARDBP p.A382T mutation (one of which in homozygous state), and one the ANG p.P-4S variant. Both patients carrying the p.A382T mutation had an atypical phenotype, one of them manifesting signs suggestive of a cerebellar involvement, and the other presenting neuroradiological findings suggestive of an inflammatory disorder of the central nervous system. Our results suggest that ALS patients with OCBs may harbor mutations in disease-causing genes. We speculate that mutations in both TARDBP and ANG genes may disrupt the blood-brain barrier (BBB), promoting local immune responses and neuroinflammation. The role of mutant TARDBP and ANG genes on BBB integrity of ALS patients warrants further investigation
Modificazioni dei livelli di Brain-Derived Neurotrophic Factor (BDNF) nel siero di pazienti affetti da Malattia di Huntington
No full textBiological characterization of hematopoietic and mesenchymal stem cell compartments in sporadic Amyotrophic Lateral Sclerosis
No full textProblem periodyzacji literatury łacińskiej doby cesarstwa rzymskiego (I–II wiek n.e.)
No full textThe article deals with the problem of periodization of Latin literature at the time of the Roman Empire, especially in the first two centuries AD. Having assumed the cyclical nature of phenomena in the field of culture and literature, the author proposes to identify three successive historical-literary phases: [a] the early-imperial period (14–68 AD); [b] the Flavian or liberal period (69–117 AD); [c] the archaizing or Frontonian period (117–192 AD). The scrupulous analysis of the epic, lyric and dramatic works, written in Latin in the first and second centuries AD, clearly documents that the individual literary periods stand in clear opposition to the preceding and following phases. They contain dominant trends, current movements and literary phenomena, distinct from the ones which precede and follow. Ten main differences or similarities between the historical-literary periods are carefully presented in three attached tables (Nos. 1–3).Artykuł podejmuje problem periodyzacji literatury rzymskiej doby cesarstwa rzymskiego, skupiając się na dwóch pierwszych wiekach n.e. Autor, zakładając cykliczność zjawisk w dziedzinie kultury i literatury, proponuje wydzielenie trzech faz historycznoliterackich: [a] okres wczesnoimperialny (14–68 n.e.); [b] okres flawiański lub liberalny (69–117 n.e.); [c] okres archaizujący lub frontoniański (117–192 n.e.). Skrupulatna analiza dzieł epickich, lirycznych i dramatycznych, napisanych po łacinie w pierwszym i drugim stuleciu n.e., jasno dokumentuje że wyróżnione okresy literackie stoją w wyraźnej opozycji do faz poprzedzających i po nich następujących. Występują w nich dominujące prądy i zjawiska literackie, odmienne niż w fazie poprzedzającej i bezpośrednio po nich następującej. Trzy załączone tabele prezentują dziesiątkę głównych odrębności lub podobieństw pomiędzy trzema analizowanymi okresami literarckimi
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