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Cerebellar ataxia can be caused by neoplasia, toxics (drugs, heavy metals, alcohol), infection, vascular lesions or auto-immune and paraneoplastic pathologies. Neuroimaging must be performed urgently in case of sudden onset and serologies as well as a lumbar puncture should be performed. Several case reports of ataxia associated with COVID-19 have been published, however the underlying pathogenic mechanisms remain unclear. This is a diagnosis of exclusion when other causes are ruled out and when the ataxia appears simultaneously to COVID-19 infection. We lack data on best management, but the prognosis appears mostly favorable with good functional recovery without any specific treatment. This paper describes the case of a patient who developed a cerebellar ataxia as the only neurological manifestation of a SARS-CoV-2 infection.Une ataxie cérébelleuse peut être causée par un processus (para)néoplasique, auto-imun, une exposition toxique, une infection ou une lésion vasculaire. Une imagerie doit être réalisée en urgence devant toute atteinte aiguë et le bilan devrait être complété par des sérologies larges et une ponction lombaire. Plusieurs cas d’ataxie liée au Covid-19 ont été décrits, dont le mécanisme étiopathogénique reste incomplètement élucidé, le diagnostic se faisant plutôt par exclusion lorsque les symptômes apparaissent de manière concomitante à l’infection. Des données manquent sur la prise en charge mais le pronostic semble favorable, avec une bonne récupération fonctionnelle. Cet article décrit le cas d’une patiente ayant présenté une ataxie cérébelleuse comme symptôme neurologique isolé contemporain d’une infection à SARS-CoV-2.

This paper focuses though on the medical management of the chronic ITP, as well as on the controversies generated by it and the therapeutic progress. Even if the idiopathic thrombocytopenic purpura is a rather frequent cause of childhood thrombocytopenia, it remains an exclusion diagnosis to be considered only in cases of persistent isolated thrombocytopenia observed in previously healthy children (other etiology excluded by the physical examination and medical history). Idiopathic thrombocytopenic purpura (ITP) is secondary to the premature platelet lysis by antibodies targeting the platelet membrane glycoproteins. The chronic evolution is defined by a prolongation of symptoms for more than 12 months. The management of chronic ITP remains controversial. Novel treatments are on the spot, such as the rituximab and the thrombopoetin receptors' agonists (the most up-to-date therapies). We present the case of a 10 years old girl admitted in our department for recurrent diffuse purpuric lesions in the context of a chronic idiopathic purpura. The clinical aspects were classical: diffuse purpuric rash, without any other symptoms. An extended check-up was performed: it confirmed the presence of anti-platelets antibodies.

The approval of substantial numbers of targeted biologic therapies (e.g., monoclonal antibodies, fusion proteins) for treatment of inflammatory diseases has positioned these drugs as important to fight chronic disorders such as psoriasis, rheumatoid arthritis and Crohn's disease. One of the concerns raised with the administration of biologic therapies is that because most of them are immunogenic glycoproteins they induce undesirable immune response leading to the generation of specific anti-drug antibodies (ADA). The development of "self" derived protein therapeutics (comprised of human germline sequence), such as recombinant "human" antibodies, helped to reduce the production of ADA but did not avoid all immunogenicity. Reduced efficacy and safety issues such as anaphylaxis or vasculitis accompany the development of ADA. In addition to immune reactions directed against the biologic therapies as a whole, some of them such as anti-TNFα are able to induce auto-immune response, notably antinuclear antibody (ANA). ANA development was associated with induced lupus and in psoriasis it was suggested that it may act as a marker of treatment failure to anti-TNFα. With a focus on psoriasis, this paper makes a current point on the consequences and challenges of the development of anti-drug antibodies and auto-immunity in patients who receive biologic therapies.

The French quality control is organized by the French Health Products Safety Agency. In 2005, the immuno-haematology testing control included the screening of an anti KEL 1 antibody. 17 out of 2639 laboratories (0,64%) answered 'negative screening'. All laboratories received a questionnaire in order to understand the failure. In this paper the authors present the detailed laboratories' responses and failure explanations.

The involvement of serum anti-ovarian autoantibodies (AOA) in ovarian pathology still remains controversial. In some cases of clinically patent ovarian failure, there seems to be a causal relationship between AOA and the ovarian disease. In patients with various organ-specific or systemic autoimmune diseases, or with unexplained, repeated reproductive failure, but otherwise normal ovarian function, it is even more difficult to determine the significance of AOA for several reasons: i) AOA recognize many different antigenic targets in the ovary ii) the antiovarian response may be transient or variable with time iii) the presence of AOA does not imply their aetiopathogenic role in the disease. The present paper reviews the clinical significance of AOA based on their ovarian targets as far as they have been identified until now.

Maternal myasthenia gravis has been associated with the presence of neonatal myasthania and sometimes fetal congenital anomalies. The purpose of this paper is to present an infant with multiple deformations born to a mother with myasthenia gravis. The infant presented with arthrogryposis multiplex and pulmonary hypoplasia. The new born died within the first day of life. Twenty-seven other cases of neonatal myasthenia with arthrogryposis have been reported. Twenty-two of them were stillborn or died. The surviving children needed ventilatory assistance for a long period.

Sjögren's syndrome is an immune exocrinopathy. This paper will deal with the primary Sjogren's syndrome non associated with another rheumatologic disease (polyarthritis or systemic lupus erythematosus). Its prevalence is claimed to increase with age. The main clinical manifestation is salivary and/or ocular dryness due to a lymphocytic infiltration of the salivary or lacrimal glands or both. However, sicca symptoms are very frequent in the elderly, most often iatrogenic. The problem, particularly in this population, is the diagnosis of Sjögren's syndrome. There are yet no consensual criteria, the most used in the literature are the European criteria, published in 1993 and revised in 1996. Some tests, such as the Schirmer's test and non stimulated salivary flow, are not specific over 60 years. The results of other tests are discussed as the focus score on the lip salivary glands biopsy. The diagnosis of Sjögren's syndrome should be envisaged in the elderly, when systemic manifestations are associated with sicca symptoms.

The relevance of detection, quantification and characterisation of antinuclear antibodies and of anti-neutrophil cytoplasm antibodies in diagnosis of connective tissue disorders and of idiopathic vasculitis, is nowadays quite important. Since many years, antibody detection is routinely performed by indirect immunofluorescence tests; their sensitivity is very high so that several diagnoses might be ruled out in the presence of a negative result. These tests are however not specific. During the last years several progress have been realised in the characterisation of the antigens recognized by these antibodies, allowing the development of new techniques for specifically identifying several autoantibodies. It is therefore possible to detect now routinely with specific and sensitive techniques the presence of anti-double stranded DNA, anti-RNP, anti-Sm, anti-Scl-70, anti-SS-A, anti-SS-B, and anti-myeloperoxidase and anti-proteinase 3. The significance of the presence of these antibodies in serum will be discussed in this paper.

In recent years, the clinical spectrum of coeliac disease has changed and forms with mild aspecific symptoms are today frequent. Therefore many infants are submitted to jejunal biopsy in order to exclude coeliac disease or to allow an early diagnosis. This has led to a search for a simple and reliable diagnostic test of coeliac disease in order to limit the use of jejunal biopsy. Recent data suggest that the study of serum antigliadin, antireticulin and antiendomysium antibodies may possibly play the role. In this paper the working group on coeliac disease of the Groupe Francophone de Gastroentérologie et Nutrition Pédiatriques expresses its view on the place of the dosages of these antibodies in the diagnosis and follow up procedures of coeliac disease in infants and children. At the present time, although it allows a simplification of the procedures, these dosages are presently not sufficiently reliable to serve as a substitute of jejunal biopsy.

Scleroderma (systemic sclerosis) is a connective tissue disease characterized by an excessive accumulation of collage in the main body organs. The subsequent progressive fibrosis may result in severe functional impairment of the tissue(s) involved. In this paper, recent advances in the understanding of the disease are reviewed. Particular emphasis is placed on the role played by vascular lesions, inflammatory cell infiltrates, autoimmunity and possibly abnormal secretion of cytokines in the disturbance of connective tissue production. A better knowledge of the pathophysiological process involved in scleroma might lead to the development of new therapeutic approaches.

The present paper describes a new ELISA type immunoenzymatic method for the detection of antibodies directed against nDNA, dDNA, and double stranded RNA (poly A, poly U and poly l, poly C). The antigens are adsorbed on glass beads but as some nucleic acids are difficult to adsorb on glass beads, polylysine was used to bind the antigens to the beads. The beads were saturated with sheep serum to get rid possible unspecific bindings. The sera are diluted before use with PBS containing sheep serum. The rate of antinucleic acid antibodies is obtained by subtraction of OD with antigen from OD without antigen. The technique was applied to human sera from patients with various autoimmune diseases (mostly disseminated lupus erythematosus). There is a rather good correlation between the determinations of anti-nDNA antibodies obtained with the ELISA and with indirect IF on Crithidia Luciliae.

It is a paradox that thyroidology often initiates new biological concepts but lags behind in the characterization of the molecular mechanisms involved, the latter progressing more rapidly in other systems. The question of the TSH receptor is an illustration of this. This paper reviews the main aspects of the biochemistry and functions of the TSH receptor. It is stressed that the classical biochemical approach has failed in the elucidation of the structure of the receptor which should benefit from molecular biology methodologies. Experimental difficulties in the study of the TSH binding function of the receptor are also described. Functional pathways activated by the receptor are discussed, especially within the frame of current researches on the thyroid growth effect of TSH. Another important feature concerning the TSH receptor is its implication in the physiopathology of Graves' disease. Auto-anti-TSH receptor antibodies are the hallmark the disease and are responsible for the hyperfunction of the gland. Characterization of the antigenic epitopes of the receptor and biological effects of the various anti-TSH receptor antibodies, both natural polyclonal and experimental monoclonal, is also an area of active research. It is hoped that understanding of Graves' disease will benefit from improvement in our knowledge of the TSH receptor structure and functions.

131 patients (124 with idiopathic thrombocytopenic purpura) have been investigated for platelet autoantibodies with an antiglobulin consumption assay (ACA) and a direct Coombs test (DCT) using 125I-antiglobulin. The two assays have been simultaneously performed on 165 platelet-suspensions. The results of ACA (expressed as normal or abnormal amounts of platelet bound IgG or PBlgG) and those of DCT (expressed as positive or negative) were concordant in 128 out of 165 cases (77.6 per cent). In 37 of 165 cases (22.4 per cent) the DCT was negative while PBgG was found to be slightly elevated with ACA. There was a significant correlation (r = 0.44) between PBlgG levels and radioactivity index calculated in DCT. The relationship between these data was graphically expressed as a curve constructed on semilog paper meaning that PBlgG levels corresponding to high radioactivity index were more uncertain than those corresponding to lower index. The quantitative expression of DCT would be improved by refering the results to a standard curve and not only to normal platelets. In the case, results of DCT would be probably equivalent to those of ACA.