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Résumé Introduction Le syndrome de l’homme raide est une pathologie neurologique rare associant une rigidité fluctuante du tronc et des membres et des spasmes musculaires douloureux. Les variants cliniques incluent le syndrome des membres raides où la rigidité ne touche qu’un des membres et l’encéphalomyélite progressive avec rigidité et myoclonies ( progressive encephalomyelitis with rigidity and myoclonus [PERM]), caractérisée par l’association d’une raideur et de spasmes myocloniques avec des signes focaux. Observation Nous rapportons un cas de PERM, chez une patiente âgée de 73 ans, initialement explorée pour une altération de l’état général assortie de douleurs abdominales. Conclusion De par ses présentations cliniques diverses et parfois non neurologiques, le diagnostic de PERM est souvent fait tardivement. La recherche d’anticorps antineuropile dans le sérum et le LCR, et l’aspect de spasmes à l’électromyogramme sans atteinte myogène caractérisent cette pathologie neurologique atypique. Introduction Stiff-person syndrome is rare neurological disease, associating trunk rigidity and painful muscular spasms. A clinical variant of stiff person syndrome is the progressive encephalomyelitis with rigidity and myoclonus (PERM), which includes neurological cognitive disturbances. Case report We report a 73-year-old woman initially addressed for abdominal pain, anorexia and severe weight-loss, for whom diagnosis of PERM was made. Conclusion Because of its various clinical presentations, sometimes without evidence for neurological disease, the diagnosis of PERM is delayed. The presence of antineuropile antibodies associated with muscular spasms at electromyogram are strong evidence for this diagnosis. [ABSTRACT FROM AUTHOR]

Abstract: Links between Takayasu''s arteritis (TA) and tuberculosis are discussed in the literature. We report the case of a Caucasian woman who was first seen for a regressive fever, associated with a normal clinical and chest and abdominal CT-scan examination. A minor granulomatous hepatitis was documented. She had no symptoms for the following four years. A second episode of persisting fever led to the diagnosis of simultaneous occurrence of diffuse TA and severe disseminated tuberculosis. Both affections were treated and the patient was still in good health after three years of follow-up. Simultaneous occurrence of both diseases in our observation supports evidence for a relationship between those two granulomatous diseases. [Copyright &y& Elsevier]

Introduction. – Ictero-hemorrhagic leptospirosis is an endemic disease in France. Weil''s disease, a form of leptospirosis, is well known. Fort Bragg fever is characterized by a constant pretibial papular lesion. First described in the USA, this non icteric form of leptospirosis is usually benign. We report the first French case of a mixed form of leptospirosis.Exegesis. – A 52-year-old man living in South East France suffered from fever and myalgias associated with a pretibial papular lesion. A severe icterus appeared and permitted a diagnosis of leptospirosis.Conclusion. – Our case recalls the clinical presentation of Fort Bragg fever, which is recognized through its inflammatory pretibial lesion associated or not with icterus. [Copyright &y& Elsevier]

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Introduction. – Palpation of soft tissues constitutes part of the physical examination when faced with a patient with fever of unknown origin. Our case highlights the difficulty of diagnosis when the tumor still remains impalpable ten months after onset of fever and severe biological inflammatory process.Exegesis. – A 49-year-old woman was admitted for fever of unknown origin associated with important biological inflammatory process. In view of her past medical history of breast carcinoma, a search for a relapse was performed and remained negative. Despite multiple investigations and repeated physical examinations, no diagnosis was obtained. Finally, [(18)F] fluorodoexyglucose positron emission tomography led to a diagnosis showing a high fixation located in the right thigh, related to a myxoid liposarcoma after surgical resection.Conclusion. – Dramatic regression of fever and biological inflammatory process after surgical treatment illustrates the concept of “small tumor and fever of unknown origin”, an exceptional entity but which physicians should be aware of. The role of new technical imaging with [(18)F] fluorodoexyglucose positron emission tomography has however to be clarified in the evaluation of fever of unknown origin. [Copyright &y& Elsevier]

Résumé: Introduction. – La fièvre des safaris ou fièvre de primo-invasion bilharzienne, décrite initialement au Japon sous le nom de fièvre de Katayama, est une réaction immunoallergique toxémique liée à la présence dans les vaisseaux de l’organisme de cercaires infectantes. Nous rapportons le cas collectif de sept jeunes touristes en vacances au Mali. Exégèse. – Une jeune fille de 18 ans présente une fièvre céphalalgique au retour du pays Dogon (au sud du Mali). Après avoir éliminé un paludisme et devant la notion d’une baignade en eau douce suivie de prurit, une fièvre des safaris est évoquée et confirmée. L’alerte a alors été donnée auprès des autres membres du groupe. Ainsi tous ont pu être traités afin d’éviter le passage à la chronicité de cette bilharziose. Conclusion. – Cette observation rappelle que le risque bilharzien occasionné par une baignade en eau douce en zone d’endémie est une réalité. La prophylaxie repose sur l’éducation des voyageurs. La fièvre des safaris, lorsqu’elle survient, est un vrai signal d’alerte car correctement interprétée, permet d’éviter le passage vers une bilharziose chronique. [Copyright 2004 Elsevier]

Purpose. – Prothrombin gene mutation G20210A (factor II) is, in frequency, the second genetic polymorphism involved in venous thrombosis. We report a retrospective studies on 38 patients issued from our medical department, all heterozygous for the factor II mutation and a literature review.Methods. – We have studied 38 patients, all heterozygous for the factor II mutation, selected through a population of 516 tested patients issued from our medical department from 1997 to 2002. The research was performed face with history of thrombotic or obstetrical events, angiopathy or familial screening.Results. – Twenty out of thirty-eight patients have at least one episode of venous thrombosis: superficial thromboses, deep thromboses and/or pulmonary embolism. One case of cerebral thrombophlebitis is observed. Venous thrombotic risk factors are associated in 12 cases (60%). Four out of thrity-eight patients have one episode of arterial thrombosis: cardiovascular, peripheral or cerebral. Arterial thrombotic risk factors are associated in all cases. Median age of the first venous thrombosis is earlier than the one of arterial thrombosis (39.11 versus 49.25 years).Conclusion. – Our studies confirms the interest to search the prothrombin gene mutation when faced with a venous thrombotic event (deep vein thrombosis and/or pulmonary embolism) with or without acquired risk factors. Its involvement in thrombotic arterial disease is still a matter of debate. Data concerning its involvement in systemic diseases and angiopathies (thromboangeitis obliterans, Raynaud''s phenomenon and migraine) are still needed. Mechanisms of thromboses could be an increase of prothrombin plasma level with high thrombin synthesis. [Copyright &y& Elsevier]

Purpose. – Gamma/delta T lymphocytes constitute a singular population due to their particular antigenic recognition and their localization inside the epithelium. Their functions are complementary to those of the alpha/beta T-cells and they are involved in the defense and regulation of the immune system. Their role in human diseases is not very well understood and the aim of our study was to analyze a population of patients with a peripheral gamma/delta T-cell lymphocytosis.Methods. – The study included 55 patients, recruited from 1997 to 2000, with a peripheral gamma/delta T lymphocytosis (defined by a proportion of gamma/delta T-cells of over 10% of total peripheral T lymphocytes). Analysis of the lymphocyte population was obtained by cytometry after peripheral blood sampling.Results. – Three main groups of diseases were observed: infectious diseases (viral infections and tuberculosis), inflammatory diseases (sarcoidosis and autoimmune diseases) and blood diseases (monoclonal gammopathies and hemopathies). Persistence of gamma/delta T lymphocytosis was dependent on the underlying disease (transitional when associated with an infectious disease and lasting when associated with sarcoidosis). The rest of the immunophenotyping analysis was usually normal.Conclusion. – Our results confirm the data published in the literature concerning the role of the gamma/delta T lymphocytes in infectious, inflammatory and autoimmune diseases and neoplasias. These data are in agreement with the cytotoxic and regular functions of these lymphocytes. [ABSTRACT FROM AUTHOR]

A neurocognitive phenotype of post-COVID-19 infection has recently been described that is characterized by a lack of awareness of memory impairment (i.e., anosognosia), altered functional connectivity in the brain's default mode and limbic networks, and an elevated monocyte count. However, the relationship between these cognitive and brain functional connectivity alterations in the chronic phase with the level of cytokines during the acute phase has yet to be identified. Determine whether acute cytokine type and levels is associated with anosognosia and functional patterns of brain connectivity 6–9 months after infection. We analyzed the predictive value of the concentration of acute cytokines (IL-1RA, IL-1β, IL-6, IL-8, IFNγ, G-CSF, GM-CSF) (cytokine panel by multiplex immunoassay) in the plasma of 39 patients (mean age 59 yrs, 38–78) in relation to their anosognosia scores for memory deficits via stepwise linear regression. Then, associations between the different cytokines and brain functional connectivity patterns were analyzed by MRI and multivariate partial least squares correlations for the whole group. Stepwise regression modeling allowed us to show that acute TNFα levels predicted (R2 = 0.145; β = −0.38; p =.017) and were associated (r = −0.587; p <.001) with scores of anosognosia for memory deficits observed 6–9 months post-infection. Finally, high TNFα levels were associated with hippocampal, temporal pole, accumbens nucleus, amygdala, and cerebellum connectivity. Increased plasma TNFα levels in the acute phase of COVID-19 predict the presence of long-term anosognosia scores and changes in limbic system functional connectivity. • TNFα predicts anosognosia scores for memory deficits. • TNFα accumulation is associated with decreased limbic functional connectivity. • COVID-19 affects cognitive processes related to self-awareness and memory. [ABSTRACT FROM AUTHOR]

Résumé Introduction Le rituximab est un anticorps monoclonal anti-CD20 souvent bien toléré. Pourtant, un syndrome immuno-allergique sévère mais rare associant fièvre, frissons et thrombopénie peut apparaître peu après la perfusion de rituximab. Observation Nous rapportons le cas d’une thrombopénie aiguë sévère induite au rituximab d’évolution favorable chez un patient ayant une leucémie lymphoïde chronique et discutons les mécanismes sous-jacents possibles. Conclusion Malgré la potentielle sévérité initiale de la thrombopénie induite au rituximab dans la leucémie lymphoïde chronique, la chimiothérapie ne devrait pas être interrompue, la tolérance peut s’améliorer avec le contrôle de la maladie hématologique. Introduction Rituximab is a chimeric anti-CD20 monoclonal antibody generally well tolerated. However, a severe but rare rituximab-related immune-toxic syndrome, associating fever, chills and thrombocytopenia can occur shortly after the infusion. Case report We report a case of severe acute rituximab-induced thrombocytopenia with favorable outcome in a patient with chronic lymphocytic leukemia and discuss the possible underlying mechanisms. Conclusion Despite the potential initial severity of rituximab-induced thrombocytopenia in CLL, chemotherapy should not be discontinued; tolerance might increase as the hematologic disorder is controlled. [ABSTRACT FROM AUTHOR]

Résumé: Propos: L’atteinte aortique dans la maladie de Horton est sous-estimée en raison de son caractère souvent asymptomatique. Les particularités de la maladie avec atteinte aortique sont mal connues et le lien entre l’aortite et les complications vasculaires de la maladie n’est pas démontré. L’objectif principal de cette étude était de comparer les caractéristiques cliniques, biologiques et évolutives d’une série de patients consécutifs atteints de maladie de Horton en fonction de la présence ou non d’une aortite scannographique au diagnostic. L’objectif secondaire était d’évaluer l’apport de la TEP-TDM par rapport à l’angioscanner dans le dépistage de l’atteinte inflammatoire de l’aorte et ses branches, le suivi et la prise en charge thérapeutique des patients. Méthodes: Nous présentons une étude rétrospective portant sur 63 patients présentant une maladie de Horton, diagnostiqués dans les services de médecine interne du CHU de Marseille sur une période allant de 2005 à 2011. Tous les patients inclus présentaient au moins trois des cinq critères de l’ACR et avaient eu un angioscanner thoracoabdominopelvien au diagnostic. Le diagnostic d’aortite était retenu lorsque l’angioscanner objectivait la présence d’un épaississement pariétal aortique circonférentiel, régulier et homogène et supérieur à 3mm d’épaisseur. Résultats: Vingt-six patients sur 63 (41,3 %) avaient une aortite authentifiée par angioscanner. L’âge au diagnostic était significativement plus jeune dans le groupe avec aortite par rapport au groupe indemne d’aortite (66,8 vs 73,8 ans ; p =0,002). Les dorsalgies-lombalgies d’allure inflammatoire, les signes d’atteinte vasculaire aux membres supérieurs, des marqueurs inflammatoires plus élevés ainsi que des lésions ectasiques ou anévrismales de l’aorte étaient significativement associés à la présence de l’aortite. Vingt patients ont bénéficié de façon concomitante d’un angioscanner et d’une TEP-TDM. Aucun diagnostic d’aortite n’a été porté par la TEP-TDM alors que l’angioscanner était normal. Sous corticothérapie, la régression de l’aortite sur l’angioscanner était notée dans les six mois chez tous les patients alors qu’une fixation du 18FDG à la TEP-TDM persistait dans 80 % des cas à six mois et dans deux tiers des cas à un an, sans influencer le traitement. Conclusion: Cette série montre qu’il n’y a pas de caractéristiques spécifiques de l’atteinte inflammatoire de l’aorte et ses branches, justifiant ainsi son dépistage systématique par angioscanner. L’aortite semble être associée au développement de complications vasculaires, comme en témoignent la fréquence des lésions ectasiantes et anévrismales et un cas de dissection aortique précoce observé dans le groupe des patients avec aortite. Au-delà de sa signification pronostique, la fréquence de cette atteinte pourrait en faire une véritable aide au diagnostic de la maladie. Enfin, la place de la TEP-TDM reste à préciser tant pour le dépistage de l’atteinte vasculaire que dans le suivi thérapeutique. [ABSTRACT FROM AUTHOR]

Abstract: Introduction: Ischemic cholangitis in intensive care unit is a recently reported liver disease in patients who have had a prolonged mechanical ventilation and vasopressive drug support for multiple organ deficiency. Prognosis is usually poor and the only life-saving therapy is liver transplantation despite ursodesoxycholic acid treatment. Case report: We report a 63-year-old man who presented with a sclerosis cholangitis after a month in intensive care unit, effectively treated with fenofibrate and ursodesoxycholic acid. Recent reports underline fenofibrate efficacy in the treatment of primary biliary cirrhosis, especially in association with ursodesoxycholic acid. This treatment has prevented liver transplantation for our patient with a correct quality of life. Conclusion: The addition of fibrate to ursodesoxycholic acid improves persistent cholestasis in sclerosing cholangitis. [Copyright &y& Elsevier]