Sobre els valors bioclimàtics en la rehabilitació de l'arquitectura tradicional de la Mediterrània

Els autors reflexionen sobre la riquesa i les possibilitats que ofereix l'arquitectura tradicional de la conca mediterrània, una forma de construir que està desapareixent però que, paradoxalment, acumula una gran saviesa des d'una perspectiva sostenibilista.Peer ReviewedPostprint (published version

Antibody-Mediated Encephalitis

Antibody-mediated encephalitides constitute a group of inflammatory brain diseases that are characterized by prominent neuropsychiatric symptoms and are associated with antibodies against neuronal cell-surface proteins, ion channels, or receptors (Table 1).1 Common clinical features include a change in behavior, psychosis, seizures, memory and cognitive deficits, abnormal movements, dysautonomia, and a decreased level of consciousness. There are, however, no systemic manifestations other than autonomic dysfunction, and this group of diseases is separable from traditional autoimmune disorders such as systemic lupus erythematosus, which may affect the nervous system. Also separate from this group of antibody-mediated encephalitides are several disorders, some of which are paraneoplastic, such as cerebellar degeneration,2 neuromyelitis optica,3 and stiff-person spectrum diseases,4 that are associated with antibodies against neuronal or glial cell-surface antigens but that are rarely associated with the aforementioned symptoms. The antibody-mediated encephalitides occur in person

Tractament dels gliomes malignes: estat actual*

Els gliomes malignes (astrocitoma anaplàstic i glioblastoma multiforme)són els tumors dels sistema nerviós més freqüents després dels meningiomes. La seva incidència, l'ocurrència en l'edat mitjana de vida, així com la considerable morbiditat que produeixen fan dels gliomes malignes un problema mèdic i social important. El tractament actual dels gliomes malignes no és, ni de bon tros, óptim. En les dues últimes dècades el seu pronòstic s'ha intentat millorar mitjançant el tractament combinat amb cirurgia, radioteràpia (RT) i quimioteràpia. L'eficàcia dels diferents tractaments ha estat avaluada en treballs clínics randomitzats i ben dissenyats...

Central nervous system neuronal surface antibody associated syndromes: review and guidelines for recognition

The concept of antibody mediated CNS disorders is relatively recent. The classical CNS paraneoplastic neurological syndromes are thought to be T cell mediated, and the onconeural antibodies merely biomarkers for the presence of the tumour. Thus it was thought that antibodies rarely, if ever, cause CNS disease. Over the past 10 years, identification of autoimmune forms of encephalitis with antibodies against neuronal surface antigens, particularly the voltage gated potassium channel complex proteins or the glutamate N-methyl-D-aspartate receptor, have shown that CNS disorders, often without associated tumours, can be antibody mediated and benefit from immunomodulatory therapies. The clinical spectrum of these diseases is not yet fully explored, there may be others yet to be discovered and some types of more common disorders (eg, epilepsy or psychosis) may prove to have an autoimmune basis. Here, the known conditions associated with neuronal surface antibodies are briefly reviewed, some general aspects of these syndromes are considered and guidelines that could help in the recognition of further disorders are suggested

Macroglobulinemia de Waldenström

La malaltia de Waldenström és una entitat poc freqüent, de la qual hi ha poques sèries, i que es defineix com una neoplàsia limfoproliferativa amb secreció monoclonal de macroglobulines. Ambdós criteris poden matisar-se, encara que les manifestacions clíniques predominants solen referir-se al component secretor, el qual necessàriament ha de pertànyer al grup d'immunoglobulines M, amb exclusió d'altres entitats similars amb components monoclonals d'altra naturalesa..

Clinical features of seronegative, but CSF antibody-positive, anti-NMDA receptor encephalitis

To determine the frequency of anti-NMDA receptor encephalitis without detectable serum NMDAR antibodies and to compare the clinical features of these patients with those with NMDAR antibodies in serum and CSF.This is a retrospective assessment of serum antibody status and clinical features of 489 patients with anti-NMDAR encephalitis, defined by the presence of NMDAR antibodies in the CSF, and available paired serum/CSF samples examined at Hospital Clínic-Institut d'Investigacions Biomèdiques August Pi I Sunyer, Barcelona, between January 2007 and December 2017. NMDAR antibodies were determined with rat brain immunostaining, in-house cell-based assay (CBA), and a commercial CBA. Patients were considered seronegative if NMDAR antibodies were undetectable with the 3 indicated techniques.Serum NMDAR antibodies were not detected in 75 of 489 (15%) patients. Compared with the 414 seropositive patients, the seronegative were older (23.5 years [interquartile range (IQR): 17-43] vs 20.5 [IQR: 14-31]; p < 0.0001) and less frequently women (39 [52%] vs 313 [76%]; p < 0.001) and had less tumors (6 [9%] vs 128 [32%]; p < 0.001). In multivariate analysis, older age at diagnosis (odds ratio [OR]: 1.35 [per decade]; 95% confidence interval [CI]: 1.10-1.67), absence of tumor (OR: 0.14; 95% CI: 0.05-0.43), and less need for intensive care unit admission (OR: 0.35; 95% CI: 0.18-0.69) were independent variables associated with the absence of serum NMDAR antibodies. Time to diagnosis, treatment with immunotherapy, relapses, and outcome were similar in seronegative and seropositive patients.NMDAR antibodies are not detected in the serum of 15% of the patients with anti-NMDAR encephalitis. These patients appear to be older and have milder neurologic symptoms with less frequency of tumors.Copyright © 2020 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology

Au sujet des valeurs bioclimatiques dans la réhabilitation de l'architecture traditionnelle méditerranéenne

Els autors han incorporat l'article en els tres idiomes en els quals ha sortit publicat, francès, anglès i castellà.L’architecture traditionnelle du bassin méditerranéen révèle aujourd’hui encore une extraordinaire richesse. Elle est le fruit et le miroir de sociétés habituées aux échanges intenses, c’est donc naturellement et grâce à ces derniers qu’elle a pris forme lentement. Il est important de souligner que cette architecture disparaît peu à peu dans la mesure où elle répondait, à l’origine, à une logique que l’on pourrait qualifier de préindustrielle, où les changements se faisaient peu à peu, où les formes de l’architecture se distillaient avec le temps et où les techniques de construction étaient transmises de génération en génération, d’un père constructeur à un fils également constructeur (les « maîtres » ou « maalem » en arabe). Les sociétés qui habitent le bassin méditerranéen ont expérimenté de profonds changements depuis l’arrivée de l’industrialisation, point de départ de ce que l’on appelle aujourd’hui la mondialisation. Les communautés qui ont construit et habité cette architecture ont disparu, se sont éteintes, et d’autres perspectives l’animent aujourd’hui (les migrations, la conversion en tant que résidence secondaire, la ghettoïsation, la gentrification, la perte de la valeur immobilière face à la construction de nouveaux immeubles à leur emplacement, etc.1). Le regard empreint de mélancolie et de romantisme qu’elle nous inspire nous fait oublier que ses habitants doivent pouvoir la transformer pour l’adapter aux nécessités mais aussi aux rêves et aspirations de notre époque. Nous tenterons ici de dévoiler la richesse de cette architecture du point de vue bioclimatique et de réfléchir aux possibilités de la réhabiliter en tirant profit des possibilités qu’elle offre et en lui apportant le soin et le respect qu’elle mérite.Postprint (published version

Carbonic anhydrase-related protein VIII antibodies and paraneoplastic cerebellar degeneration

Paraneoplastic cerebellar degeneration (PCD) is a common cause of subacute cerebellar ataxia caused by widespread loss of the Purkinje cells of the cerebellum. PCD diagnosis can be confirmed by detection of onconeural antibodies that may also indicate the underlying tumour type 1. Most onconeural antibodies associated with PCD recognize intracellular antigens in Purkinje cells 2. These antigens are supposed to induce, besides the antibody synthesis, an antigen‐specific cytotoxic T‐cell attack that probably is responsible of the Purkinje cell death and limited response to treatment 3, 4. The most common onconeural antibody in PCD is the Yo antibody that associates with breast and ovarian cancer 4. In a previous case report, we identified a new antibody, targeting the intracellular Purkinje cell protein carbonic anhydrase‐related protein VIII (CARP VIII), in a patient with PCD and melanoma 5. We provide further evidence for the association of CARP VIII antibodies with PCD by demonstrating a second patient with these antibodies, who had an ovarian adenocarcinoma and developed cerebellar ataxia

The Diagnostic Value of Onconeural Antibodies Depends on How They Are Tested

Detection of onconeural antibodies is important because establishes a definitive diagnosis of paraneoplastic neurological syndrome (PNS). The recommended method for diagnosis of onconeural antibodies is by immunohistochemistry on rodent brain sections and confirmation of results by immunoblot. However, in many diagnostic laboratories samples are only tested with commercial line blots. In this study we inquired whether this change in diagnostic methodology (line blot alone vs. combined immunohistochemistry and line blot) would affect the results. Among 439 samples examined by immunohistochemistry and a commercial line blot (Euroimmun, Lübeck, Germany) 96 (22%) were positive by line blot, and their clinical information was reviewed. Onconeural antibodies were detected by both assays in 46/96 (48%) patients (concordant group) whereas 50 (52%) were only positive by line blot (discordant group). In the concordant group 42/46 (91%) patients had a definite diagnosis of PNS whereas in the discordant group only 4/50 (8%) had PNS (p < 0.00001). None of the 14 patients with ZIC4 antibodies and 1/13 (8%) with Yo antibodies demonstrated only by line blot had PNS. These findings show a robust diagnostic value of combined diagnostic techniques, and both should be used to demonstrate onconeural antibodies, If antibody testing is performed only with line blot assay, positive bands should be confirmed by rodent brain immunohistochemistry. For ZIC4 or Yo antibody testing, line blot positivity with negative immunohistochemistry has no diagnostic significance, and for the rest of onconeural antibodies the predictive diagnostic value is low

Antibody repertoire in paraneoplastic cerebellar degeneration and small cell lung cancer

The goal of this study is to determine whether patients with paraneoplastic cerebellar degeneration (PCD) and small-cell lung cancer (SCLC) have a specific repertoire of antibodies, if SOX1 antibodies (SOX1-ab) can predict the presence of SCLC, and if antibodies to cell surface antigens occur in this syndrome. Antibody analysis was done using immunohistochemistry on rat brain, immunoblot with recombinant antigens, screening of cDNA expression libraries, and immunolabeling of live neurons in 39 patients with PCD and SCLC. VGCC-ab were measured by RIA, and SOX1-ab, Hu-ab, and ZIC4-ab by immunoblot. Lambert-Eaton myastenic syndrome (LEMS) was present in 10 of 23 patients with electrophysiological studies. At least one antibody was detected in 72% of patients. The individual frequencies were: 49% SOX1-ab, 44% VGCC-ab, 31% Hu-ab, and 13% ZIC4-ab. SOX1-ab occurred in 76% of patients with VGCC-ab and 27% of those without VGCC-ab (p = 0.0036). SOX1-ab were not found in 39 patients with sporadic late-onset cerebellar ataxia, 23 with cerebellar ataxia and glutamic acid decarboxylase antibodies, and 73 with PCD and cancer types other than SCLC (31 without onconeural antibodies, 25 with Yo-ab , 17 with Tr-ab). Five patients (13%) had antibodies against unknown neuronal cell surface antigens but none of them improved with immunotherapy. One serum immunoreacted against the axon initial segment of neurons and another serum against ELKS1, a protein highly expressed in the cerebellum that interacts with the beta4-subunit of the VGCC. In conclusion, 72% of patients with PCD and SCLC had one or more antibodies that indicate the presence of this tumor. In these patients, VGCC-ab and SOX1-ab occur tightly associated. SOX1-ab are predictors of SCLC in ataxia patients with a specificity of 100% and sensitivity of 49%. Unlike limbic encephalitis with SCLC, antibodies to cell surface antigens other than VGCC-ab, are infrequent and do not predict response to treatment