Bilateral sudden sensorineural hearing loss as a presenting feature of systemic lupus erythematosus: Case report and brief review of other published cases

International audienceIntroduction: Sudden sensorineural hearing loss is an unusual presenting clinical feature of systemic lupus erythematosus.Case report: We report the case of a young woman who was admitted to hospital for sudden sensorineural hearing loss and hemophagocytic syndrome which was attributed to systemic lupus erythematosus on the basis of specific renal involvement, thrombocytopenia, and consistent autoantibodies. Favorable outcome was obtained on high-dose corticosteroids, and the hearing fully recovered.Discussion: Sudden sensorineural hearing loss in systemic lupus erythematosus is seemingly more frequently associated with severe systemic involvement and antiphospholipid antibodies may be present. Although management remains empirical, the high risk of permanent hearing impairment seems to justify emergency treatment with high-dose corticosteroids. When the clinical and laboratory criteria of antiphospholipid syndrome are met, antiplatelets agents or anticoagulation therapy shall be considered

Brain PET metabolic abnormalities in patients with long-lasting macrophagic myofasciitis

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Distinct interferon signatures stratify inflammatory and dysimmune myopathies

International audienceWhat is already known about this subject? ► among inflammatory/dysimmune myopathies (iDMs), dermatomyositis (DM) is the only associated with type i-interferon signature. ► Most iDMs are associated with myofiber expression of major histocompatibility complex (MHc)-class i. MHc-i is induced by interferons suggesting a possible role for type ii-interferon in iDMs other than DM. What does this study add? ► in this study, we showed that myofiber MHc-ii expression is observed in inclusion body myositis (iBM) and antisynthetase myositis (aSM), but not in DM and necrotizing autoimmune myopathy (naM). ► in accordance with this finding, we showed that iBM and aSM are specifically associated with type-ii iFnγ signature, DM only with type-i iFn signature, and naM with neither type-i nor type-ii iFn signature. How might this impact on clinical practice? ► Distinct iFn signatures allow a more distinct segregation of iDMs and therefore a more accurate diagnosis. ► Deciphering iFn signatures in iDMs will also lead to develop new therapeutic approaches targeting iFns pathways. AbstrAct Objective the role of interferons (iFn) in the pathophysiology of primary inflammatory and dysimmune myopathies (iDM) is increasingly investigated, notably because specific neutralisation approaches may constitute promising therapeutic tracks. in present work we analysed the muscular expression of specific iFnα/β and iFnγ-stimulated genes in patients with various types of iDM. Methods 39 patients with iDM with inclusion body myositis (iBM, n=9), dermatomyositis (DM, n=10), necrotising autoimmune myopathies (naM, n=10) and antisynthetase myositis (aSM, n=10), and 10 controls were included. Quantification of expression levels of iFnγ, iSg15, an iFnα/β-inducible gene and of six iFnγ-inducible genes (gBP2, Hla-DOB, Hla-DPB, ciita, Hla-DrB and Hla-DMB) was performed on muscle biopsy samples. Results DM usually associated with strong type i iFnα/β signature, iBM and aSM with prominent type ii iFnγ signature and naM with neither type i nor type ii iFn signature. immunofluorescence study in aSM and iBM showed myofibre expression of major histocompatibility class 2 (MHc-2) and ciita, confirming the induction of the iFnγ pathway. Furthermore, MHc-2-positive myofibres were observed in close proximity to cD8+ t cells which produce high levels of iFnγ. Conclusion Distinct iFn signatures allow a more distinct segregation of iDMs and myofibre MHc-2 expression is a reliable biomarker of type ii iFn signature

FDG-PET/CT Brain Findings in a Patient With Macrophagic Myofasciitis

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Brain 18 F-FDG PET Metabolic Abnormalities in Patients with Long-Lasting Macrophagic Myofascitis

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Neuropsychological Correlates of Brain Perfusion SPECT in Patients with Macrophagic Myofasciitis

<div><p>Background</p><p>Patients with aluminum hydroxide adjuvant-induced macrophagic myofasciitis (MMF) complain of arthromyalgias, chronic fatigue and cognitive deficits. This study aimed to characterize brain perfusion in these patients.</p><p>Methods</p><p>Brain perfusion SPECT was performed in 76 consecutive patients (aged 49±10 y) followed in the Garches-Necker-Mondor-Hendaye reference center for rare neuromuscular diseases. Images were acquired 30 min after intravenous injection of 925 MBq ^99mTc-ethylcysteinate dimer (ECD) at rest. All patients also underwent a comprehensive battery of neuropsychological tests, within 1.3±5.5 mo from SPECT. Statistical parametric maps (SPM12) were obtained for each test using linear regressions between each performance score and brain perfusion, with adjustment for age, sex, socio-cultural level and time delay between brain SPECT and neuropsychological testing.</p><p>Results</p><p>SPM analysis revealed positive correlation between neuropsychological scores (mostly exploring executive functions) and brain perfusion in the posterior associative cortex, including cuneus/precuneus/occipital lingual areas, the periventricular white matter/corpus callosum, and the cerebellum, while negative correlation was found with amygdalo-hippocampal/entorhinal complexes. A positive correlation was also observed between brain perfusion and the posterior associative cortex when the time elapsed since last vaccine injection was investigated.</p><p>Conclusions</p><p>Brain perfusion SPECT showed a pattern of cortical and subcortical changes in accordance with the MMF-associated cognitive disorder previously described. These results provide a neurobiological substrate for brain dysfunction in aluminum hydroxide adjuvant-induced MMF patients.</p></div

SPM map.

<p>Positive correlation between brain perfusion and dichotic listening (left ear words) normalized scores with adjustment for age, sex, socio-cultural level and time delay between brain SPECT and neuropsychological testing. Linear regression analysis shows diffuse impairment of periventricular areas/corpus callosum. Significant clusters are displayed with T-score values on 2-dimensional projections (glass-brain—left panel) and slices of MRI (right panel) templates in axial, coronal and sagittal orientations. <i>P</i>-value < 0.005 at the voxel level for clusters ≥ 100 contiguous voxels (corrected for cluster volume). L, left; R, right; A, anterior; P, posterior.</p

SPM map.

<p>Positive correlation between brain perfusion and Stroop color and word Z-scores with adjustment for age, sex, socio-cultural level and time delay between brain SPECT and neuropsychological testing. Linear regression analysis shows impairment of posterior associative areas. Significant clusters are displayed with T-score values on 2-dimensional axial, coronal and sagittal orientations (glass-brain—left panel) and projected onto a brain rendered 3D MIP (right panel). P-value < 0.005 at the voxel level for clusters ≥ 100 contiguous voxels (corrected for cluster volume). L, left; R, right; A, anterior; P, posterior.</p