[Juvenile dermatomyositis and new autoantibodies: Cases and review]. / Dermatomyosite juvénile et nouveaux auto-anticorps : à propos de quatre observations.

Juvenile dermatomyositis (JDM) is the most common inflammatory myopathy in children. Its diagnosis is usually made on a clinical basis following the criteria of Bohan and Peter (1975). Recently, the presence of myositis-specific autoantibodies (MSAs) have started to be associated with specific outcome in adult patients; the diagnosis and prognosis value of these autoantibodies remains to be identified in children. We report four cases of JDM with MSAs focusing on clinical, biological, and radiological manifestations, and then we describe associated treatment. The cohort comprises four girls with an average age of 8.5 years. The time to diagnosis was 1 week to 4 months. For these patients, the immunologic study found one patient positive for the MDA5 antibody (or CADM 140), one positive for the TIF1γ antibody (or p155/140), and two patients positive for the NXP2 antibody (or p140/MJ). Each patient showed specific and characteristic cutaneous manifestations. For example, the girl positive for the TIF1γ antibody presented the most severe skin disease with urticaria, face edema, and vascularity of the neck and shoulders. However, regarding muscular features, proximal weakness was present in most of the cohort, except for the child positive for the MDA5 antibody, who presented no sign of muscular disease at the beginning with low CK levels. Importantly, acute pancreatitis also affected this patient. Concerning radiological indications, muscular MRI evidenced hyperinflammation, a sign of diffuse myositis, in all these patients. Treatments consisted in corticosteroids together with methotrexate or mycofenolate mofetil associated or not with intravenous immunoglobulin therapy. This report highlights the importance of systematic detection and analysis of MSA in diagnosis and characterization of JDM, and describes a new approach that would allow more focused treatments and be a useful predictor of clinical complications and prognosis in JDM-affected subjects.

[Cochlear implants in children with mental disabilities]. / Implant cochléaire chez l'enfant présentant des troubles envahissants du développement.

INTRODUCTION: Nowadays, profoundly deaf children with associated psychotic disorders can sometimes benefit from a cochlear implant program. These fragile children who have a specific development need a reinforced observation and follow up, and active parental collaboration. METHODS: From a series of 8 children with psychotic disorders implanted between 1999 and 2006, we present 4 representative cases of our practise, which have at least 3 years post implant. RESULTS: The questions that should occur about the conditions of the cochlear implantation are discussed in the light of the results of our observations. No children deteriorated them selves. All of them use their cochlear implant continuously. CONCLUSION: Cochlear implant is a tool in a global approach towards mobilization of communication resources in such children.

[Preliminary study of acoustic and aerodynamic parameters after Tucker frontal anterior laryngectomy]. / Etude préliminaires des paramètres acoustiques et aérodynamiques après laryngectomie frontale antérieure de Tucker.

Currently, not objective method has been demonstrated to be reliable for the evaluation of vocal handicap after partial laryngectomy. Such a tool would allow objective assessment of the post-operative voice and its clinical course as well as a comparison of different surgical techniques in terms of voice quality. We used a EVA device to measure simultaneously acoustic and aerodynamic parameters. We included 23 normal subjects and 34 patients who had undergone a Tucker laryngectomy. All subjects were males. At sustained voice production, acoustic measures of vibration stability (jitter, coefficient of variation in the fundamental frequency, shimmer, coefficient of variation in intensity) and air leak from the glottis (buccal air flow over intensity). The quality of voice in operated patients was judged by a jury of listeners who assigned a global score to voice quality. Results were analyzed to verify the correlation between objective measurements and the jury's score, taken as the reference measurement. The pertinence of the objective measurements was demonstrated for the stability of the frequency and the glottal air leak. Multiple regression analysis demonstrated that total variance of the objective measurements accounted for 84.7% of the scores provided by the jury (R2 = 0.847, p < 0.0001). These satisfactory results emphasize the interest of multiparametric analysis including aerodynamic measurements. These preliminary results led to the hypothesis that objective and subjective measurements could be useful in techniques aimed at improving voice quality (temporal organization of vibratory instability, work on intralaryngeal pressures).