Search

Resume Introduction La crampe des ecrivains est une dystonie focale dont la prise en charge demeure difficile. Nous rapportons notre experience pendant 14 ans sur une population de 119 patients, d’âge compris entre 18 et 85 ans (âge moyen 43 ans). Methodes Le traitement consistait en injections de toxine botulinique Dysport ® et kinesitherapie. Les patients etaient revus tous les quatre a six mois avec evaluation clinique et video de la crampe analysee par trois observateurs differents et appreciation subjective de l’efficacite du traitement par le patient (score de 1 a 3). Le deficit post-injection eventuel etait aussi quantifie. Resultats Une amelioration de la crampe (score 2 et 3) a ete obtenue chez 61,6 % des patients du groupe traite par toxine et kinesitherapie avec une majorite de patients se declarant moyennement satisfaits (14 patients dans le score 2). Dans le groupe traite par toxine seule, 37,9 % des patients ont ete ameliores (score 2 et 3) avec une majorite tres satisfaite ayant recupere une ecriture normale (18 patients dans le score 3). L’âge n’etait pas un facteur predictif pour la reponse au traitement. Les meilleurs resultats ont ete obtenus par les injections de toxine dans les muscles flexor carpi radialis suivis par le flexor digitorum profundus II et III et le flexor pollicis longus . Soixante et onze pour cent des injections ont entraine un deficit musculaire juge modere et peu invalidant par rapport au benefice du traitement. Vingt-sept patients ont ete suivis pendant plus de deux ans et trois patients du score 3, avec reponse excellente, ont ete suivis pendant 14 ans avec une efficacite tres constante des injections. Si les injections n’etaient pas efficaces la premiere fois, nous les avons reevaluees et adaptees et nous avons considere que le traitement par toxine etait un echec apres trois injections sans aucun benefice. Conclusion Les injections bien ciblees de toxine botulinique restent le traitement de choix dans cette pathologie. La kinesitherapie est utile lorsque les injections de toxine n’ameliorent pas completement l’ecriture. Cela necessite une cooperation etroite entre le medecin injecteur, le kinesitherapeute et le patient.

Poor graft function without signs of graft rejection following allogeneic BMT (allo-BMT) occurs in around 9% of patients. A high incidence of hazardous complications may be encountered, leading to life-threatening situations.We describe three patients who underwent allo-BMT for acute leukemia in first complete remission and untreated myelodysplastic syndrome. The three patients experienced prolonged and profound granulocytopenia, anemia and thrombocytopenia, despite growth factors and transfusions. This was not corrected by donor leukocytes infusion. They received a boost of CD34(+) positively-selected cells from their HLA-identical sibling donors.A rapid improvement of peripheral blood cell counts was observed in both patients who were in full donor chimerism status at time of boost infusion, whereas the patient with mixed chimerism did not show any signs of improvement. Neither patient suffered further exacerbation of GvHD.Allogeneic positively-immunoselected CD34(+) cells can represent an interesting alternative treatment for poor graft function following allo-BMT, in the absence of graft rejection signs.

Summary Background Intermittent interleukin-2 therapy for HIV-1 by continuous intravenous infusion leads to sustained increase of CD4 T cells. This method of administration is, however, inconvenient and has limiting toxic effects. We did a randomised study to compare safety and efficacy of antiviral treatment alone or combined with various interleukin-2 regimens in HIV-1-infected patients. Methods 94 symptom-free patients, naive to antiretroviral treatment, with CD4-T-cell counts of 250–550 cells/μL at baseline were randomly assigned zidovudine and didanosine alone (n=26) or combined with interleukin-2 administered intravenously (12 million IU/day, n=22) or subcutaneously (3 million IU/m 2 twice daily, n=24) for 5 days, or were given polyethylene-glycol-modified (PEG) interleukin-2 (2 million IU/m 2 intravenous bolus, n=22) administered every 2 months from week 2 to week 50 (seven cycles). Safety and immunological and virological results were monitored until week 56. Findings CD4-T-cell count increased to higher than baseline by a mean of 564 cells/μL (subcutaneous group), 676 cells/μL (intravenous group), 105 cells/μL (PEG group), and 55 cells/μL (antiretroviral-therapy group, p=0·0001). 68% and 77% of patients in the subcutaneous and intravenous groups, respectively, achieved an 80% increase of CD4 T cells (p Interpretation Subcutaneous interleukin-2 is a convenient regimen that, as well as intravenous therapy, improves immunological function in HIV-1-infected patients receiving two nucleosides. Larger studies are needed to show whether immunological improvements translate into clinical benefit.

Summary We studied a 53 year old right-handed patient who presented isolated myoclonus of right facial muscles induced exclusively by language. Twitching significantly hindered speaking and reading performance. MRI and CT-scan revealed no brain lesion. Conventional EEG showed a few spike-waves predominantly in the left hemisphere. Spike-waves increased during drowsiness. An EEG-EMG polygraphic study was performed during stimulation tests which included linguistic tasks and non-verbal/non-linguistic tests. Myoclonus was triggered by speaking and writing but not by non-linguistic tasks. The severity of myoclonus was dependent on the complexity of the language task. Back-averaging of right facial EMG bursts failed to show a reliable EEG-EMG correlation. However, the facial reflex myoclonus might have originated from the left rolandicopercular cortex, as it was triggered by complex language activities. Findings in this case are compared with those reported for other forms of reflex seizure and myoclonus.

Summary Objective To describe the outcome of an unselected large series of patients with lymphoblastic lymphoma (LBL) treated in a single institution. Patients and methods Sixty-two patients were treated between 1980 and 1992. Induction chemotherapy (CT) to achieve complete response (CR) was: French Multicenter Acute Lymphoblastic Leukemia (ALL) protocols (38), non-Hodgkin’s Lymphoma (NHL) protocols (20). Thirty patients underwent transplant after achieving CR (allogeneic 12; autologous 18). Results Forty-six patients (74%) achieved CR and 16 (26%) failed to respond. The patients who received an ALL induction had an 89% CR rate, while the CR rate was 52% in patients who received a NHL-like regimen. With a median follow-up of 93 months (range 36–187), the actuarial overall survival (OS) rate for all patients is 49% at five years and 41% at 10 years, and the actuarial event-free survival (EFS) rate is 45% and 37%. OS and EFS in the grafted population are, respectively, 60% and 56% at five years. Our results also show a trend toward a longer OS in allografted group. Conclusions ALL induction therapy is more effective than the NHL-like regimen for augmenting the CR rate. Autologous or allogeneic transplantation should be considered as consolidation therapy in high-risk group patients.

Resume Les alterations infracliniques du systeme nerveux sont frequentes au cours de l'infection par le VIH. Neanmoins, peu d'attention a ete accordee jusqu'ici au systeme nerveux autonome (SNA). Les auteurs ont choisi de rechercher de facon prospective, a l'aide de tests non vulnerants etudiant le SNA cardiaque, les signes d'atteinte fonctionnelle infracliniques du SNA dans une population de 55 sujets VIH +. Parallelement, ils ont realise une etude quantifiee des sensibilites thermique (fibres de petit diametre) et vibratoire (fibres de grand diametre). Leurs resultats ne montrent pas de difference statistiquement significative entre le groupe des sujets infectes et celui des sujets controles ainsi qu'entre les sous-groupes de sujets infectes appartenant aux phases avancees de la maladie par rapport a ceux des phases initiales. Cependant, une analyse individuelle comparant les resultats des seropositifs et ceux du groupe controle indique la presence d'anomalies fonctionnelles du SNA (petites fibres) chez 34,5% des sujets VIH + ainsi qu'une atteinte de frequence comparable pour la perception thermique (petites fibres) et pour la perception vibratoire (grosses fibres). Par ailleurs, les signes de dysautonomie infraclinique sont plus frequents chez les malades des groupes IV des CDC (48,3%) que chez ceux des groupes I, II et III (19%) temoignant ainsi d'une accentuation progressive de la dysautonomie au cours de l'infection.

Resume Les effets du piracetam ont ete evalues par l'EEG quantifie et par des tests psychometriques dans la maladie d'Alzheimer. Les auteurs ont effectue la synthese de deux etudes menees par deux equipes differentes, dans deux centres distincts, sur deux groupes respectivement de 12 (GRI) et 16 (GRII) malades. Pour chaque groupe, les malades ont ete randomises selon la methode du double aveugle en un groupe placebo et un groupe piracetam, recevant soit 9 g/j (groupe I piracetam), soit 2,4 g/j (groupe II piracetam) du produit. Dans les groupes traites, une diminution significative des puissances EEG du 2–6 Hz (groupe I piracetam), 3–5 Hz, 7 Hz (groupe II piracetam), et une augmentation significative du 9–11 Hz (groupe I piracetam) et du 10 et 13 Hz (groupe II piracetam) ont ete observees au bout de 90 jours de traitement. Parmi les tests utilises dans les deux groupes, un seul s'est ameliore de facon significative dans le groupe II. Cependant une bonne correlation est apparue entre la diminution des composantes lentes EEGq et l'amelioration des scores psychometriques dans les deux groupes. L'ensemble de ces donnees suggere que le piracetam a eu un effet sur le niveau attentionnel des malades. La bonne reproductibilite des resultats entre les deux etudes confirme la grande sensibilite de l'EEGq pour mesurer les effets d'un medicament psychotrope comparativement aux tests psychometriques et souligne son interet en tant que marqueur de l'evolution de la maladie.

Twenty six adult patients (pts) with Burkitt's lymphoma (BL) received a pediatric chemotherapy regimen (LMB89), 17/26 have stage IV disease, 10/26 with HIV+. Two pts died of early major toxicity and 24 were evaluable for response. Thirteen entered in complete remission (54 %) and 10/13 are alive in persistant CR. All pts who never had CR (8 PR and 3 failures) died of their lymphoma. LMB89 is efficient in adults with BL.

Plasma selenium (Se) levels and glutathion peroxydase activity (GPX) were measured in 125 HIV positive patients and 25 controls. The mean level values were significantly lower in HIV positive patients (Se : 70 vs 93,5 μg/l ; GPX : 649 vs 912 ui/l ; p = 0,001) but there was no difference according to the CDC staging system. This Se deficiency may lead to further impairment of immune function.

A case of acute erythremia with a chromosomal translocation (8 ; 9) is presented. Response to chemotherapy was poor and the patient died 2 months after the diagnosis. The characteristics and nosological situation of this rare erythroid neoplasia are discussed.

Endothelial markers were investigated in 68 HIV positive patients. We found a significant mean rise in vWF with disease progression, abnormalities in tPA, PAI, and thrombomodulin values without stage correlation, and a protein S deficiency in 26.5% of cases. The mechanism of this endothelial cell dysfunction remains to be elucidated since HIV itself does not infect these cells.

We recently observed 2 cases of vascular thrombosis in HIV positive homosexual men with protein S deficiency and investigated serum protein S concentrations by ELISA in 72 HIV positive patients. Free protein S deficiency was found in 27 % of cases. Its clinical significance and pathophysiology will be discussed.

Summary A total of 109 subjects aged 60 years or more and without any focalized cerebral lesions were studied by E.E.G. and C.T. scan recordings. An original type of planimetric study of the C.T. scan sections was used to obtain numerical ratios of the degree of cerebral atrophy. The planimetric ratios and the E.E.G. data were then studied as a function of age-group and clinical condition. Most of the data obtained was then treated in a computer. Ventricular dilatation is a constant and progressive feature of normal aging, and is therefore correlated with it, whereas it cannot be significantly correlated with senility changes. The E.E.G. shows no precise correlation with age during normal aging but reflects the functional disturbances of senility disorders, especially dementias. There is no correlation between the E.E.G. and C.T. scan results either during normal or pathological aging.

In order to study the activity of phagocytic cells in normal and pathological aging, we compared normal young and aged subjects and patients with Alzheimer's (AD) or Parkinson's (PD) disease. Blood granulocytes and monocytes were separately assayed for ingestion of three different particle species (opsonized zymosan, immunoglobulin-coated sheep red cells (IgG-SRC) and glutaraldehyde-treated sheep red cells (G-SRC)). The superoxide anion production induced by these particles was also measured. All granulocyte responses to zymosan and IgG-SRC were depressed in the three aged groups as compared to young controls. Hence, only functions involving a specific receptor (Fc or C3b receptor) seemed affected. Monocyte activity was slightly decreased in the same groups. No difference was found between AD or PD patients and normal aged subjects. Hence the phagocytic and oxidative defects we found were a consequence of aging.