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During adolescence, a minority of adolescents with type 1 diabetes have persistent and serious poor metabolic control. The main cause of poorly controlled diabetes during adolescence seems to be poor adherence to therapy. The reasons are intertwined between social, family, psychological, and other factors. The aim of our qualitative study was to describe the characteristics of adolescents with chronic poorly controlled diabetes and those of their families.We conducted 10 semi-structured interviews with adolescents aged 12-18 years and whose annual average hemoglobin A1c was greater than or equal to 9.5%. Six semi-structured interviews were conducted with parents (either with both or one parent). Interviews were then analyzed according to the comprehensive microanalysis method.We selected three recurrent themes: family life and diabetes, diabetes care and issues, and negative representations of the disease. Family situations were often complex, with limited involvement by fathers and many conflicts regarding medical care. Adolescents were disinvested from day-to-day care although they were aware of the possible long-term complications. Adolescents and their families had a very negative outlook of and experience with diabetes.The study highlighted family issues and difficult disease-related experiences among adolescents with persistent and poorly controlled diabetes. As a part of a comprehensive medical approach, it seems necessary to take into consideration the daily care of patients with diabetes within their unique family dynamic.

ObjectiveTurner Syndrome is associated with several phenotypic conditions associated with a higher risk of subsequent comorbidity. We aimed to evaluate the prevalence of congenital malformations and the occurrence of age-related comorbid conditions and to determine whether the frequencies of congenital and acquired conditions depend on X chromosome gene dosage, as a function of karyotype subgroup.Design and methodsThis national retrospective observational cohort study includes 1501 patients. We evaluated the prevalence of congenital malformations and the cumulative incidence of subsequent specific comorbidities at five-year intervals, from the ages of 10 to 30 years, with stratification by karyotype subgroup: 45,X (n = 549), 45,X/46,isoXq (n = 280), 46,X,r(X)/46,XX (n = 106), 45,X/46,XX (n = 221), presence of Y (n = 87).ResultsMedian age was 9.4 (3.7–13.7) years at first evaluation and 16.8 (11.2–21.4) years at last evaluation. Congenital heart (18.9%) malformations were more frequent in 45,X patients, and congenital renal (17.2%) malformations were more frequent in 45,X, 45,X/46,isoXq and 46,X,r(X)/46,XX patients than in those with 45,X/46,XX mosaicism or a Y chromosome (P ConclusionThese data suggest that X gene chromosome dosage, particularly for Xp genes, contributes to the risk of developing comorbidities.

Appendix - Functional respiratory imaging (FRI) methodology. Detailed description of the FRI methodology and FRI parameters used in the manuscript/study. (PDF 74 kb)

Resume Introduction Le syndrome APECED (auto-immune polyendocrinopathy, candidiasis, ectodermal dystrophy) est une affection rare, de transmission recessive autosomique, liee a des mutations du gene AIRE. Nous en rapportons un cas chez un enfant de trois ans, illustrant sa grande variabilite phenotypique et soulignant l’importance de l’atteinte dermatologique. Observation Un garcon de trois ans etait hospitalise pour une splenomegalie et une alteration de l’etat general conduisant au diagnostic d’hepatite auto-immune. Un avis dermatologique etait demande pour une dermatose evoluant depuis un mois. Il s’agissait d’une eruption generalisee prurigineuse d’evolution fluctuante, d’aspect urticarien. L’examen dermatologique notait egalement des anomalies de la tablette ungueale des 20 ongles, evoluant depuis l’âge de deux ans. Les prelevements mycologiques ungueaux etaient negatifs. Une fibroscopie œso-gastroduodenale mettait en evidence une hypertension portale et une candidose œsophagienne. Dans ce contexte, le diagnostic de syndrome APECED etait suspecte puis confirme par la mise en evidence de deux mutations dans le gene AIRE. Le bilan endocrinien complet realise alors etait strictement normal. Discussion Ce cas clinique illustre la variabilite phenotypique du syndrome APECED. Avant l’apparition de la triade caracteristique : candidose cutaneo-muqueuse recidivante, hypoparathyroidie et insuffisance surrenalienne auto-immune, le diagnostic d’APECED doit etre systematiquement evoque chez le jeune enfant devant toute anomalie cutaneo-phanerienne associee a une candidose cutaneo-muqueuse ou a des manifestations auto-immunes. Conclusion La connaissance des manifestations cutanees du syndrome APECED permet au dermatologue d’en faire le diagnostic precocement, avant l’apparition des manifestations endocriniennes menacant le pronostic vital de l’enfant.

Introduction L’alemtuzumab, anticorps monoclonal anti-CD52 utilise dans la sclerose en plaques de forme remittente, cree une immunodepression, puis une reconstitution immunitaire. Les patients sont a risque de developper des maladies auto-immunes, dont la plus frequente est la maladie de Basedow (MB), survenant chez 16 a 41 % des patients, dont l’evolution est atypique : taux de remission et de passage en hypothyroidie plus importants. Une modification des anticorps anti-recepteur de la TSH (TRAb), devenant bloquants, est suspectee. Observation Patiente de 33 ans sous alemtuzumab depuis 2014, arrete depuis 23 mois. Diagnostic de MB au 7e mois de grossesse (TSH normale au 1er et 2e trimestre) : T4L : 39 pmol/L, T3L : 17 pmol/L, TRAb : 23 UI/L. Enfant ne a 38 semaines sans anomalie clinique. Developpement d’une hyperthyroidie neonatale : TSH Discussion La MB induite par l’alemtuzumab peut survenir jusqu’a 4 ans apres traitement. Observation atypique : debut en seconde partie de grossesse, passage rapide en hypothyroidie chez le nouveau ne, comme decrit chez l’adulte, en l’absence de TRAb bloquants. Il est possible que l’evolution differente de la MB soit secondaire aux modifications immunitaires post-therapeutiques.

Introduction Les macroprolactinomes de l’adolescent sont rares. Le traitement de premiere intention est medical, par les agonistes dopaminergiques. La strategie therapeutique en cas de resistance aux agonistes dopaminergiques a ete peu etudiee. Observation Patient de 11 ans 3 mois presentant un macroprolactinome diagnostique devant l’association cephalees, troubles visuels, signes caverneux. L’IRM montrait un macroadenome hypophysaire de 13 mm, la biologie une hyperprolactinemie a 5447 ng/mL sans insuffisance antehypophysaire. Le patient a ensuite presente une insuffisance gonadotrope necessitant une induction pubertaire par gonadotrophines. Le traitement par cabergoline 0,25 mg par semaine a ete partiellement efficace initialement : diminution de 50 % de la taille de l’adenome et de la prolactinemie en un mois. Puis la prolactinemie a diminue progressivement sous 2 mg de cabergoline par semaine jusqu’a 680 ng/mL a 3 ans de traitement. Le patient a ensuite presente un echappement a la cabergoline malgre l’augmentation de la dose jusqu’a 5 mg par semaine, avec croissance de l’adenome, augmentation de la prolactinemie a 4333 ng/mL, puis apoplexie hypophysaire responsable d’une insuffisance corticotrope et thyreotrope. Il a alors ete opere (Ki67 : 10 %), puis traite par protontherapie 54 Gy 3 mois plus tard sur le residu postoperatoire. La prolactinemie a diminue a 620 ng/mL a 7 mois, alors que le patient etait toujours traite par 2 mg de cabergoline par semaine. L’analyse genetique n’a pas trouve de mutation des genes AIP ou MEN1. Discussion La chirurgie associee a la protontherapie permet de mieux controler les macroprolactinomes resistants aux fortes doses de cabergoline chez l’adolescent.

Le syndrome de Wolfram, du a des mutations du gene WFS1, entraine diverses atteintes : diabete, atrophie optique, surdite, diabete insipide, anomalies urologiques, neurologiques, psychiatriques, hypophysaires, gonadiques, a des degres variables. La puberte et la fonction gonadique sont peu etudiees. Objectif Etudier le developpement pubertaire et la fonction gonadique des patients atteints de syndrome de Wolfram. Methode Etude observationnelle retrospective multicentrique sur 6 CHU. Resultats Douze garcons et 6 filles âges de 21 ± 7 ans etaient inclus. La variabilite phenotypique etait importante, avec une concordance intrafamiliale. Tous les patients avaient un diabete et une atrophie optique, 45,5 % des garcons et 16,6 % des filles avaient un hypogonadisme, peripherique, diagnostique en moyenne a 20,5 ans. Chez les garcons, l’hypogonadisme predominait sur la fonction sertolienne, avec une fonction leydigienne subnormale. Le demarrage pubertaire survenait plus tard qu’en population generale, a 12,8 ans. Chez les garcons presentant un hypogonadisme, il survenait a 13,55 ans, versus 12,43 chez ceux n’en ayant pas. L’acceleration de la vitesse de croissance survenait a 13,8 ans, la cinetique du pic de vitesse de croissance etait identique chez tous nos garcons. Le temps entre le demarrage pubertaire et l’acceleration de la vitesse de croissance etait plus court chez les patients hypogonadiques : 0,2 versus 1,5 ans. Chez les filles, la puberte etait comparable a la population generale. Discussion Chez les garcons, l’insuffisance sertolienne est frequente, precoce, existe avant la puberte, sous-evaluant le diametre testiculaire. Certains patients sont asymptomatiques, la surveillance pubertaire et gonadique sont necessaires, comme la surveillance des autres atteintes.

4MOST is a wide-field, high-multiplex spectroscopic survey facility under development for the VISTA telescope of the European Southern Observatory (ESO). Its main science drivers are in the fields of galactic archeology, high-energy physics, galaxy evolution and cosmology. 4MOST will in particular provide the spectroscopic complements to the large area surveys coming from space missions like Gaia, eROSITA, Euclid, and PLATO and from ground-based facilities like VISTA, VST, DES, LSST and SKA. The 4MOST baseline concept features a 2.5 degree diameter field-of-view with similar to 2400 fibres in the focal surface that are configured by a fibre positioner based on the tilting spine principle. The fibres feed two types of spectrographs; similar to 1600 fibres go to two spectrographs with resolution R> 5000 (lambda similar to 390-930 nm) and similar to 800 fibres to a spectrograph with R> 18,000 (lambda similar to 392-437 nm & 515-572 nm & 605-675 nm). Both types of spectrographs are fixed-configuration, three-channel spectrographs. 4MOST will have an unique operations concept in which 5 year public surveys from both the consortium and the ESO community will be combined and observed in parallel during each exposure, resulting in more than 25 million spectra of targets spread over a large fraction of the southern sky. The 4MOST Facility Simulator (4FS) was developed to demonstrate the feasibility of this observing concept. 4MOST has been accepted for implementation by ESO with operations expected to start by the end of 2020. This paper provides a top-level overview of the 4MOST facility, while other papers in these proceedings provide more detailed descriptions of the instrument concept[1], the instrument requirements development[2], the systems engineering implementation[3], the instrument model[4], the fibre positioner concepts[5], the fibre feed[6], and the spectrographs[7].

New data are presented for the adiabatic compressibility of bovine serum albumen (BSA) over the temperature range 2–85°C and at pH 5, 6, 7 and 8. The relative impact of intrinsic and hydration contributions to the observed compressibility for BSA is estimated and shown to agree very well with general values for globular protein and with previous results for BSA. The compressibility manifests a maximum value in the regions where BSA is most stable, exhibiting a parabolic dependence on temperature with lowest values where it is least stable. These data are obtained with a novel technique that employs a commercial, low cost and automated pulse echo apparatus for the measurement of the speed of sound to an accuracy of 1 m s −1 and a precision of 0.1 m s −1 . This requires the use of relatively high protein concentrations, typically of the order of 10 mg ml −1 upwards. The ultrasound data is analysed using a relation obtained from scattering theory that accounts for the multiple scattering of sound in concentrated systems. The technique has a data acquisition rate of 20 measurements per second and large quantities of high-quality data can be amassed, permitting the automatic scanning of the compressibility of a protein as a function of temperature for example. This makes it particularly appropriate for studying the behaviour of proteins and other large biomolecules in food.

Polyglandular auto-immune syndrome type 1 (PAS-1) or auto-immune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is a rare autosomal recessive disorder linked to auto-immune regulator (AIRE) gene mutations. Herein, we report the case of a 3-year-old boy with APECED emphasizing the wide phenotypic variability and the extent of skin lesions.A 3-year-old boy with a history of auto-immune hepatitis was referred for a generalized pruriginous urticaria-like eruption present for one month. He was born to non-consanguineous parents. Cutaneous examination revealed twenty-nail dystrophy, which had been present since the age of 2 years. Both direct microscopy and culture of nail samples were negative for Candida albicans. Esophagogastroduodenoscopy revealed esophageal candidiasis. A diagnosis of APECED was suspected and subsequently confirmed by molecular analysis of the AIRE gene, which showed two mutations. No other auto-immune endocrinopathies were found.Our case report illustrates the phenotypic variability of APECED with the absence of typical manifestations such as Addison's disease and hypoparathyroidism. APECED should thus be systematically suspected in young children presenting with cutaneous lesions associated with mucocutaneous candidiasis or auto-immune disease, even in the absence of known endocrinopathies.Dermatologists should be aware of this association since early diagnosis of APECED is critical in preventing life-threatening endocrinological crises.

La maladie de Basedow est une thyroidopathie observee chez la femme jeune, pouvant induire des complications materno-fœtales lors de la grossesse. Objectif Evaluer la prise en charge des femmes enceintes avec antecedent de maladie de Basedow, par rapport aux recommandations de l’American Thyroid Association, en vue d’ameliorer leur prise en charge pour diminuer la morbi-mortalite materno-fœtale. Methode Etude retrospective observationnelle sur les femmes ayant accouche au CHU de Besancon entre janvier 2013 et decembre 2015 (8006 accouchements). Resultats Vingt-huit patientes sont incluses : 20 avec un antecedent de Basedow, dont 4 ont recidive lors de la grossesse, et 8 avec un Basedow actif (5 diagnostiques pendant la grossesse et 3 en cours de traitement). Traitement maternel conforme aux recommandations. TSH dans l’objectif. Statut immunologique des TSAb connu chez 55,5 % des patientes. Parmi les patientes positives (73,3 %) : 63,6 % ont un titre d’anticorps inferieur a 5 UI/L, sans complications materno-fœtales et 36,4 % superieur a 5 UI/L, dont une mort fœtale in utero. Pas de surveillance echographique specialisee pour 93 % des patientes avec TSAb positifs. Bilan thyroidien realise a la naissance chez 80 % des nouveau-nes des femmes avec TSAb positifs. Discussion Dans notre serie, 0,35 % de femmes enceintes ont un antecedent ou une maladie de Basedow active. La prise en charge therapeutique est satisfaisante. Neanmoins, il est necessaire d’ameliorer (en ciblant les patientes avec des TSAb superieurs a 5 UI/L), la surveillance biologique des TSAb et echographique chez les meres ainsi que les bilans thyroidiens a la naissance.

Objectif Evaluation du suivi de 30 deficits en GH (DGH) apres un processus de transition structure au CHU de Besancon entre 1988 et 2014. Patients et methode Trente patients traites jusqu’a leur taille finale pour : 16/30 deficits congenitaux (dont 12 syndromes d’interruption de tige), 11/30 tumeurs cerebrales (dont 5 kystes de la poche de Rathke) et 3/30 DGH post-radiotherapie pour leucemie. DGH persistant severe apres reevaluation hypophysaire (10 isoles et 20 deficits combines). Âge moyen au transfert 17,4 (±1,9) ans. Donnees recueillies a la derniere visite en pediatrie puis a 1, 2, 3 et 5 ans : doses de GH, IGF1, DMO, IMC, glycemie et bilan lipidique. Resultats Mediane de suivi : 3 ans Dix-sept sur vingt-cinq (68 %) traites a un an, 14/19 (73,6 %) a 2 ans, 14/16 (87,5 %) a 3 ans et 8/11 (72,7 %) a 5 ans Seuls 5/30 (16,6 %) interrompent le suivi apres un an et 2/25 (23,3 %) apres 2 ans IGF1 dans la cible theorique souhaitee (partie haute de la fourchette normale, soit 0,5 + 2DS) pour 5/25 (31,2 %) a 1 ans, pour 2/19 (14,2 %) a 2 ans et 1/14 (7,1 %) a 3 ans pour des doses moyennes de GH de 1,2 mg/j, 0,9 mg/j et 0,8 mg/j, respectivement (sous reserve de l’observance). DMO (1,165 g/cm 2 ), glycemie et bilan lipidique stable a 3 et 5 ans. Conclusions La plupart des DGH sont traites 5 ans apres la transition soulignant une collaboration active entre equipes pediatrique et adulte. Les taux d’IGF1 non a la cible necessite de discuter une optimisation des posologies et/ou de l’approche motivationnelle.

The 4MOST consortium is currently halfway through a Conceptual Design study for ESO with the aim to develop a wide-field (>3 square degree, goal >5 square degree), high-multiplex (>1500 fibres, goal 3000 fibres) spectroscopic survey facility for an ESO 4m-class telescope (VISTA). 4MOST will run permanently on the telescope to perform a 5 year public survey yielding more than 20 million spectra at resolution R~5000 (��=390-1000 nm) and more than 2 million spectra at R~20,000 (395-456.5 nm & 587-673 nm). The 4MOST design is especially intended to complement three key all-sky, space-based observatories of prime European interest: Gaia, eROSITA and Euclid. Initial design and performance estimates for the wide-field corrector concepts are presented. We consider two fibre positioner concepts, a well-known Phi-Theta system and a new R-Theta concept with a large patrol area. The spectrographs are fixed configuration two-arm spectrographs, with dedicated spectrographs for the high- and low-resolution. A full facility simulator is being developed to guide trade-off decisions regarding the optimal field-of-view, number of fibres needed, and the relative fraction of high-to-low resolution fibres. Mock catalogues with template spectra from seven Design Reference Surveys are simulated to verify the science requirements of 4MOST. The 4MOST consortium aims to deliver the full 4MOST facility by the end of 2018 and start delivering high-level data products for both consortium and ESO community targets a year later with yearly increments., To appear in the proceedings of the SPIE Astronomical Instrumentation + Telescopes conference, Amsterdam, 2012. 15 pages, 16 figures

Data certification and digital signature are a new area of interest and many standards have emerged. Indeed, these technologies offer identification, authentication and non-repudiation capabilities during Internet transactions (emails and e-commerce). However, it appears that both certification and digital signature do not completely answer enterprise data authentication and secured data storage needs. We submit a proposition of an authorities-based architecture to answer these issues. This architecture relies on most of the available standards.

Traitement du syndrome d’hemophagocytose dans l’intolerance aux proteines dibasiques avec lysinurie (IPDL). Introduction L’IPDL est une anomalie hereditaire rare du metabolisme des acides amines dibasiques. Cliniquement, la maladie se manifeste par des symptomes digestifs avec hepatosplenomegalie et retard de croissance. Biologiquement, le diagnostic repose sur une hyperammoniemie avec hyperaminoacidurie dibasique. L’atteinte hematologique peut comporter une cytopenie peripherique principalement par hypersplenisme et les signes biologiques du syndrome d’activation macrophagique. Le mye-logramme peut etre normal ou retrouver une phagocytose particuliere des erythroblasts et des polynucleaires neutrophiles simultanement par des macrophages et des progeniteurs granuleux, specifique de l’IPDL. Cas clinique Il s’agit d’un garcon de 13 ans, dont le diagnostic d’IPDL a ete pose a l’âge de 15 mois sur une volumineuse hepatosplenomegalie avec symptomatologie digestive pour laquelle une nutrition parenterale pauvre en protides a du etre introduite. L’evolution de sa maladie a ete marquee par de multiples episodes de septicemie sur catheter central au cours desquelles l’enfant presentait a chaque fois des pancytopenies mises sur le compte de l’hypersplenisme, spontanement resolutives donc non explorees. Lors de sa derniere septicemie, une hemophagocytose biologique et medullaire specifique telle qu’elle est decrite dans l’IPDL a ete mise en evidence. Cet enfant a ete traite par des corticoides IV 5 jours a 2 mg/kg/j puis per os en decroissance sur 15 jours, associes a de la ciclosporine IV 7 jours a 2,5 mg/kg/j puis per os a 6 mg/kg/j depuis 3 mois toujours en cours de decroissance. L’evolution des signes biologiques de l’hemophagocytose a ete marquee par une normalisation initiale pendant les 20 premiers jours de traitement puis une rechute a l’arret des corticoides. Conclusion Un myelogramme doit etre effectue devant toute pan-cytopenie chez un patient atteint d’une IPDL a la recherche de signes specifiques d’hemophagocytose. Le traitement par ciclosporine et corticoides semble permettre une normalisation des signes biologique de l’hemophagocytose plus rapidement qu’en l’absence de traitement et semble donc interessante surtout dans des contextes infectieux.

Introduction Les formes monogeniques de diabete neonatal non auto-immun (DN) sont caracterisees par de faibles concentrations plasmatiques d'insuline dues a des defauts primitifs des cellules beta pancreatiques. Plusieurs etudes recentes ont montre que des mutations codantes non-synonymes heterozygotes situees dans le gene codant la preproinsuline ( INS ) sont impliquees dans ∼10 % des patients atteints de DN. Le gene INS est regule par de nombreux facteurs de transcription ubiquitaires ou au contraire specifiques de la cellule beta. Nous faisons ici l'hypothese que des mutations homozygotes recessives du promoteur de INS pourraient inactiver ce gene. Materiels et Methodes Le promoteur de INS a ete sequence en deux fragments chez sept patients atteints de DN dont les parents sont consanguins. Des mutations codantes des genes KCNJ11 , ABCC8 et INS , et les anomalies du chromosome 6q24 avaient ete exclues. 350 controles normoglycemiques ont de meme ete sequences. Des experiences cellulaires et moleculaires ont ete realisees apres mutagenese d'un variant identifie a l'etat homozygote, dans un vecteur rapporteur pGL2 contenant le promoteur de INS . Resultats Chez deux patients ayant presente un DN transitoire, nous avons identifie une mutation homozygote c.-331C > G non repertoriee, non presente chez les sujets controles et positionnee sur un motif de fixation des facteurs de transcription GLIS3 et de la famille des KLF. Nous avons montre que la mutation eteignait plus de 90 % de l'activite du promoteur de INS dans la lignee murine de cellules beta INS1. Des etudes d'immunoprecipitation de la chromatine (ChIP) et de retard sur gel vont permettre de determiner quel(s) facteur(s) de transcription KLF et/ou GLIS3 sont impliques dans l'extinction de l'activite du promoteur. Une exploration metabolique des parents porteurs de la mutation heterozygote dans une famille a ete realisee. Conclusion Notre etude montre de facon inedite un nouveau mecanisme implique dans une forme transitoire de diabete tres precoce, via un dysfonctionnement de la regulation transcriptionnelle du gene INS entrainant l'extinction de l'activite de son promoteur.

Introduction le retard pubertaire est un motif frequent de consultation endocrinologique. Il peut s’integrer dans differentes pathologies dont le CDGS de type 1a qui comprend des manifestations neurologiques, cutanees et des atteintes multiviscerales digestives, hepatiques, cardiaques ou renales qui en font la gravite. L’atteinte biologique est quasi constante : cytolyse hepatique moderee, troubles de l’hemostase et troubles hormonaux. Nous reportons ici le cas de Celine nee le 29/06/1978 vue a l’âge de 18 ans et 6 mois pour amenorrhee primaire, retard statural avec retard psychomoteur et dysmorphie (cou court, implantation basse des cheveux, strabisme). Ses ATCD sont marques par une encephalite virale a l’âge de 8 ans et un RCIU (2,740 kg, 45 cm). Le retard statural evolue depuis l’âge de 2 ans a –3DS et le retard ponderal a – 2DS. Elle pese 36,5 kg et mesure 146,5 cm. L’IMC est a 17 kg/m2. Elle est S1P2. L’âge osseux est a 11 ans. Le bilan hormonal montre une IOP : FSH de base a 87 UI/l puis a 114 sous LH-RH, LH de base a 23UI/l puis a 87 sous LH-RH, œstradiol C’est suite au diagnostic de CDGS chez une cousine maternelle de Celine que le diagnostic est evoque. Un deficit majeur en phosphomannomutase et une situation heterozygote composite avec 2 mutations R141H/P113L du gene PMM2 sont finalement trouves confirmant le diagnostic de CDGS 1a. Conclusion ce cas illustre la difficulte diagnostique du CDGS en cas de presentation clinique incomplete. L’IOP associee a un retard mental doit donc faire rechercher le CDGS de type 1a.

Depression and Parkinson disease, two very different conditions at first sight, have much more intricate connections than is usually believed. Depression may be the patient's reaction to Parkinson disease, a condition that is anticipated with anxiety, with good reason as it is often very disabling and has not been significantly prolonged by dopamine therapy. Depression may precede the first signs of Parkinson disease. Pseudoparkinsonian melancholia may be difficult to distinguish from the akinetic form of Parkinson disease. Most of the symptoms of the latter have been encountered in the former. The following features do not occur in depression: astasia with trepidation, festination, monotonous tachyphemia and palilalia, sebaceous hypersecretion, and of course unilateral or frankly asymmetric signs. Parkinson syndrome secondary to depression can be classified with those parkinsonian syndromes that are different from parkinson disease and secondary to a clearcut etiology. In some instances, diagnosis is established by the response to therapy. In the present state of our knowledge, the treatment of depression relies on chemotherapy and sismotherapy and not on dopamine therapy. The management of Parkinson disease rests on dopamine which may be associated with tricylic antidepressants.

Nineteen patients with plexus lesions following radiation therapy were investigated: fifteen with brachial plexus, 4 with lumbar or sacral plexus involvement. Symptoms at onset are usually sensory. Motor disturbances occur either simultaneously or after some delay, their course is generally gradual and unfavourable. Areflexia appears early and was present in every case. Important cutaneous lesions (radiodermitis) and considerable induration of soft tissues were observed in every patient. Diagnosis is a relapse of the mitotic process. Severity of prognosis makes imperative a definite technique of radiation therapy. In all the patients included in this study, dosage had exceded 1,600 rets. Patients were tentatively treated with D-penicillamine, an inhibitor of collagen synthesis.

A 19-year-old woman with migraine under contraceptive therapy had transient right hemiparesis due to a minor lesion in the left hemisphere, which was probably hemorrhagic rather than ischemic. On the left side, angiography showed nearly complete obstruction of the terminal portion of the internal carotid artery, extending to the initial portion of its terminal branches, with an outlined "Moya-Moya" network. On the right side, moderate annular stenosis of the cervical portion of the internal carotid was visible. On subsequent angiographies, done 7 and 19 months later, blood flow was reestablished in the left internal carotid artery as well as in its terminal branches, but with persistent segmental stenosis. Since the stroke, the symptomatology of migrainous attacks has been altered, pointing to a left hemispheric spreading.A 19-year old woman with a migraine who had been taking oral contraceptives (OCs) had transient right hemiparesis due to a minor lesion in the left hemisphere, probably hemorrhagic rather than ischemic. On the left side, angiography showed nearly complete obstruction of the terminal portion of the internal carotid artery, extending to the initial portion of its terminal branches with an outlined "Moya-Moya" network. On the right side, moderate annular stenosis of the cervical portion of the internal carotid was visible. On subsequent angiographies, done 7 and 19 months later, blood flow was reestablished in the left internal carotid artery as well as in its terminal branches, but with persistent segmental stenosis. Since the stroke, the symptomatology of migrainous attacks has been altered, pointing to a left hemispheric spreading. (author's modified)

Neurological and clinical examinations were found to be normal in a woman aged 40 years who had had a single generalized epileptic seizure. Rediological examinations demonstrated a spiral-shaped calcification above and laterally to the left sella turcica. This corresponded to changes in the C 1 segment of the left carotid sinus, which was partly stenosed distally, with left unilateral abnormalities of the circle of Willis and multiple distal stenoses of the left sylvian and vertebro-basilar arteries. Scanning demonstrated that these had been present for a long time, but it is not possible to establish the diagnosis on an etiological basis as no similar radiological findings have been published.

A case of recurrent polyradiculoneuritis in a 54-year-old man is described. It is exceptional because of its lengthy development over a period of 19 years and by its association with a paraprotein of the IgE type without anyother anomal. Corticotherapy had a favourable effect on the clinical symptoms but did not affect the amount of monoclonal immunoglobulin in the serum. The nosological position of this neuropathy and the type of gammapathy are discussed. An immune mechanism seems likely, but no proof of a link between the neuropathy and the gammapathy could be found.

Amantadine has been used since 1969 in the treatment of Parkinson's disease. In 1970, were described the special symptoms noted in the lower limbs due to this drug. The authors, after a review of the various disturbances, have studied 10 cases by Capillaroscopy. They emphasize the interest of the study of this abnormality of the micro-circulation, producing vaso-constriction of the arterioles and venules.

Under the term cisterno-medullary anomalies are included several disorders: bony and nervous malformations, arachnoiditis of the posterior fossa. As they are frequently associated, a thorough investigation, both anatomical and dynamic, is a prerequisite to any therapeutic attempt. Along with causing damage to the neuraxis, these anomalies interfere with the dynamics of the CSF and may lead to the development of a communicating syringomyelia, whatever the theory proposed. The presenting symptoms are varied, and diagnosis should be accordingly suspected. Of fundamental importance are instrumental investigations. A complete evaluation is in most of the cases obtainable with: bone X-rays, air-myelography (or "bulle"), intrathecal ou ventricular radio-isotope scan. Surgery is the only treatment. The aim is both decompression of the neural structures and restoration of a normal CSF dynamic flow. Opening of the posterior fossa is successful in the case of developmental abnormalities, But it seems to prove a failure when the chief anomaly is arachnoiditis. In such cases, ventricular drainage alone may be followed by improvement. It appears from this that the problem is twofold: the technical problem of the drainage, and the pre-operatory diagnosis of a posterior fossa arachnoiditis.

The authors have observed 7 cases of intracranial cavernous haemangioma. A review of the literature shows that the diagnosis is suggested by the occurrence of epileptic seizures, signs of expansive process or meningeal haemorrhage. Computerized tomography displays an area of hyperdensity enhanced by injections of a contrast medium. Surgical excision is mandatory. The post-operative mortality varies with the site of the malformation.

A 54 year old man without pathologic past history but mild hypertension, obesity and gastric ulcer, presented with a syndrome of Wallenberg. He had complained for five days of progressive and diffuse headache. The neurological condition improved initially, but the patient died suddenly two weeks later. Pathological examination showed no significant alteration except for left ventricular enlargement and mild arteriosclerosis. There was a hemodissection (dissecting aneurysm) of the left vertebral artery next to the inferior oliva. It induced a lateral infarct and a limited dorsal infarct at the middle third level of medulla oblongata. Although the location of the arterial changes is usual, their nature is exceptional. The cause of the arterial hemodissection could not be ascertained: fibrous arterial dysplasia, atherosclerosis or congenital abnormalities of internal elastic layer may be discussed. But no definite conclusion can be reached.

A patient diagnosed as having Horton's disease presented a complex neurological picture dominated by sensory-motor neuropathies of all four limbs, one year after the appearance of signs of temporal arteritis. No other etiological factor, apart from the Horton's disease, was discovered, and the causal relationship between this disease and the neuropathy is discussed. The possibility of dysimmunity factors being involved in Horton's disease is raised and the resulting therapeutic implications discussed.

Five patients, four of them with severe or severe generalized myasthenia gravis, were treated by long term orally administrated prednisolone, with following results: one complete remission and two almost complete remissions (in three aged fernale patients), two substantial improvements (one in a male patient, one in a young adult female patient, both thymectomized). The least favourable result was observed in the male patient. Positive results of such treatment were similarly reported by several authors (with an average of 70% complete or almost complete remission, 20% substantial improvement, 7% moderate improvement). These results appear qualitatively superior to those obtained with ACTH, and may be long-lasting. Treatment with prednisolone may be applied to any form of myasthenia gravis, particularly those which do not react to anticholinesterasic agents in moderate dosages. At onset of treatment, patients should be under care of a reanimation unit. Dosage is initially high (60--100 mg daily, secondarily on alternate days), and should be reduced very slowly, once a definite improvement is achieved. The duration of this treatment depends upon the results obtained: it should not last under one year. Associated treatment with anticholinesterasic agents remains disputable, whereas associated thymectomy seems to provide best results.

Case report of a recurring meningioma of the posterior fossa, with pulmonary metastases. Humoral alterations (sedimentation rate, fibrinemia, alkalin phosphatases, sideremia, prothrombin, blood proteins and BSP) paralleled the course of the tumor and may be considered as a para-tumoral syndrome. Pathogenesis is unknown.