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.

SUMMARY 37 children presenting with West's syndrome were studied by computerized tomography scanning. An abnormality consisting of diffuse cerebral atrophy, predominating in the fronto-temporal region, was demonstrated in 30 cases. The abnormality was sometimes associated with calcification (tuberous sclerosis) or malformation (agenesis of the corpus callosum). The aetiology of this atrophy and its relationship to clinical and electro-encephalographic signs is discussed. Computerized tomography scanning is recommended for children with West's syndrome in order to distinguish between primary and secondary cases, and for greater prognostic accuracy. RESUME Etude tomodensitometrique du syndrome de West 37 enfants avec syndrome de West ont ete etudies par la tomodensitometrie; celle-ci a revele dans 81 pour cent des cas une anomalie, representee le plus souvent par une atrophie cerebrale diffuse a predominance fronto-temporale, quelquefois associee a des calcifications (sclerose tubereuse) ou malformations (agenesie du corps calleux). L'eetiologie de cette atrophie tres particuliere et ses rapports avec l'importance des signes cliniques et eeg sont discutes. Un examen tomodensitometrique des enfants presentant un syndrome de West est recommande pour distinguer les cas primitifs des cas secondaires et donc pour mieux apprecier le pronostic de cette encephalopathie epileptogene. ZUSAMMENFUNG Komputertomographie bei der Untersuchung des West Syndrom Bei 37 Kindern mit einem West Syndrom wurde eine Komputertomographie durchge-fuhrt. In 30 Fallen stellte sich eine Fehlbildung in Form einer diffusen Hirnatrophie dar, die im Bereich der Frontalregion am ausgepragtesten war. Zusatzlich zu dieser Fehlbildung fand man in einigen Fallen Verkalkungen (tuberose Hirnsklerose) oder andere Missbildungen (Agenesie des Corpus callosum). Eine Diskussion uber die Aetiologie dieser Atrophie und ihre klinischen und elektro-encephalographischen Merkmale schliesst sich an. Die Komputertomographie wird als Untersuchung bei Kindern mit einem West Syndrom empfohlen, um zwischen sekundaren und primaren Fallen zu unterscheiden und um eien genauere Prognose stellen zu konnen.

The aim of this retrospective analysis was to assess the benefit of reduced-intensity conditioning allo SCT (RIC allo-SCT) in a cohort of 32 relapsed multiple myeloma (MM) patients. A total of 19 patients had an HLA-identical sibling donor ('donor' group), while 13 patients had no donor ('no-donor' group). There were no significant differences between these two groups as for prognosis risk factors. Eighteen patients from the 'donor' group could actually proceed to RIC allo-SCT. With a median follow-up of 36 (range, 21-60) months, six patients died from transplant-related toxicity (cumulative incidence, 33% (95% CI, 11-55%)). Only 4 patients from the 18 transplanted patients (22%; 95% CI, 7-48%) progressed after RIC allo-SCT, as compared to 12 (86%; 95% CI, 56-98%; P=0.0003) among the nontransplanted patients. In an 'intention-to-treat' analysis, the Kaplan-Meier estimate of PFS was significantly higher in the 'donor' group as compared to the 'no-donor' group (P=0.01; 46 versus 8% at 3 years). There was no difference in terms of overall survival. However, in multivariate analysis, actual performance of RIC allo-SCT was associated with better PFS (relative risk, 0.35; 95% CI, 0.15-0.82; P=0.01). These data suggest a potential benefit for RIC allo-SCT in the management of relapsed MM warranting further prospective investigations.

HIV lipodystrophy syndrome, characterized by a significant excess of visceral adiposity and a reduced subcutaneous fat mass in association with insulin resistance and dyslipidemia, still affects the majority of antiretroviral-treated HIV-infected patients. The therapeutic management of this syndrome has not yet been well established. Benfluorex is known to decrease insulin resistance with no side effects on lactate levels in HIV-negative patients.We conducted an open-label study of benfluorex (150 mg, 2-3 times a day) that was prescribed for 60 HIV-infected patients who were diagnosed with glucose metabolism abnormalities by oral glucose tolerance test (OGTT); 47 of these patients had visceral fat accumulation measured by computed tomography (VAT). Median follow-up was 12 months (interquartile range [IQR] = 6-12 months). The great majority of patients (90%) were treated with at least triple therapy (in 70% the therapy included at least one PI), with a nonsignificant change over the study period.Added to antiretroviral therapy, benfluorex improved OGTT in 47/60 cases, including total normalization in 34/60 without lactate concentration modification. A trend toward a decrease in VAT distribution was observed (p =.06). No significant difference was observed in subcutaneous fat distribution, although an increase in subcutaneous thigh adipose tissue was observed in 17/47 (36.2%) cases and 6 patients (12.7%) presented both subcutaneous fat increase and VAT decrease.

Evaluation of the response to therapy is important for optimal selection of treatment strategy in patients with Hodgkin's disease (HD). Refractory disease requires intensive high-dose chemotherapy, whereas unnecessary treatment should be avoided in patients in complete remission. The purpose of this study was to evaluate the contribution of gallium-67 scintigraphy in predicting the clinical outcome in patients with HD and mediastinal involvement on the basis of scan results at the end of chemotherapy. Seventy-four patients with HD and mediastinal involvement were retrospectively investigated with 67Ga scintigraphy 72 h after injection of 220 MBq 67Ga citrate (planar and single-photon emission tomographic studies) following the completion of chemotherapy. At the same time, they all underwent computed tomography (CT). Patients were followed up for an average of 63 months (range 28–124 months). The disease status was newly diagnosed disease in 64 of the patients and relapse in 10. Systemic symptoms were absent (A) in 34 cases and present (B) in 40 cases. Forty-one patients had stage I or II disease and 33 patients had stage III or IV disease. Twenty-two patients had bulky disease on initial diagnosis. At the end of chemotherapy, all 74 patients showed regression of the mass by more than 50% (50%–100%) on CT. Patients were divided into two groups according to the positivity or negativity of the gallium scan after chemotherapy: 61 patients had negative and 13 patients had positive gallium scans. In the gallium-negative group, 19.7% of the patients relapsed and 91.8% were alive at the end of the follow-up. Relapse occurred in 20% of the patients with residual mass and in 19.6% of the patients without residual mass. In the gallium-positive group, 84.6% of the patients had recurrent disease and 61.5% were alive after intensive chemotherapy. There was a statistically significant difference in overall survival between patients with positive and patients with negative gallium results (P=0.0034). Disease-free survival differed significantly between patients with positive and patients with negative gallium scans at the end of chemotherapy (P

This paper investigates physicians' judgements about adherence to antiretroviral treatment (ART) among patients who have been HIV-infected through injecting drug use (IDU). Comparisons were made between data collected from physicians at enrollment (January 1996 to January 1998) of a prospective cohort study (MANIF 2000) and self-declarations of 196 HIV-infected injecting drug users (IDUs) who have been prescribed ART. The likelihood of being perceived as 'adherent' by physicians was higher for women, patients of 30 years of age or older, with biological markers indicative of a healthier status, and who were perceived as 'free of injecting behaviour' and not in drug maintenance treatment. Although the proportion of non-adherent patients was similar in physicians' assessment (26.0%) and patients' self-declarations (27.0%), a strong discordance occurred: 60.4% of patients self-reporting non-adherence to ART (80.0% for those receiving a protease inhibitor) were classified as adherent by their prescribing physicians. The study suggests that a priori judgements based on clinical experience but also on social stereotypes interfere with physicians' assessment, and that physicians' decisions to initiate complex treatment regimens may further induce optimistic biases and an underestimation of the problems faced by IDU patients to adequately adhere to them.

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.

16 patients with low-grade lymphoid malignancies and bone marrow involvement were transplanted with selected CD34 positive Peripheral Blood Progenitor Cell (PBSC) prepared from autologous aphereses.All but one patients were mobilized with a combination of chemotherapy (including high-dose cyclophosphamide and VP16 or adriamycin, aracytin with cysplatyl) and recombinant human Granulocyte Colony-Stimulating Factor (rhG-CSF).A median of 3 (range, 1 to 9) aphereses yielded 15.35 x 10(6) CD34+ cells/kg (range, 4.45 to 70.88). A median of 5.01 x 10(6) adsorbed CD34+ cells/kg (range 2.01 to 24.13) was obtained after selection (median purity: 86%; range, 59-99%). The CD34 PBSC were infused one day after either one of two conditioning regimens: 11 patients received the association of cyclophosphamide (120 mg/kg) and TBI (8Gy), and 5 patients received the BEAM regimen. No recombinant hematopoietic growth factor was used after cell reinfusion. Median days to 0.5 x 10(9)/l neutrophils and 50 x 10(9)/l platelets were 13 (range, 9 to 18) and 16 (range, 11 to 35), respectively. The median number of red blood cell (RBC) unit transfusions was 4 (range, 0 to 10). The median number of platelet transfusions was 3.5 (range, 0 to 8). No individual received backup PBSC, nor required platelet transfusion beyond 3 months post-transplant.This study confirms the feasability of using blood CD34 cells to support hematopoietic recovery after myelo-suppressive or myelo-ablative regimens, in patients with low-grade NHL.

Background The long-term effectiveness of potent three-drug antiretroviral regimens for the treatment of human immunodeficiency virus type 1 (HIV-1) infection is limited by problems related to compliance and tolerability. We investigated whether two-drug maintenance therapy would suppress viral replication after a three-month period of aggressive triple-drug induction therapy. Methods A total of 378 HIV-1–infected adults who had not received previous antiretroviral treatment received three months of induction therapy consisting of 300 mg of zidovudine every 12 hours, 150 mg of lamivudine every 12 hours, and 800 mg of indinavir every 8 hours. The 279 patients in whom the plasma HIV-1 RNA titer fell below 500 copies per milliliter after two months of triple-drug therapy, and who completed the induction phase, were randomly assigned at month 3 to one of the following three open-label maintenance regimens: zidovudine, lamivudine, and indinavir; zidovudine and lamivudine; or zidovudine and indinavir. The prima...

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.

PURPOSE To demonstrate the feasibility and efficacy of six ambulatory high-dose sequential chemotherapy courses that include three intensified cycles supported by stem-cell infusion in high-risk and high-intermediate-risk untreated non-Hodgkin's lymphoma (NHL) patients. PATIENTS AND METHODS A pilot nonrandomized study included 20 untreated patients aged less than 60 years with aggressive histologically identified NHL and two or three adverse-prognosis criteria (International Index). Patients received an ambulatory regimen with high-dose chemotherapy supported by granulocyte colony-stimulating factor (G-CSF) and repeated peripheral-blood stem-cell (PBSC) infusion. The median age was 39 years (range, 20 to 59), with 13 men and seven women. Chemotherapy consisted of one cycle every 21 days for a total of six cycles. The first three cycles (A1, A2, and A3) consisted of cyclophosphamide (Cy) 3,000 mg/m2, doxorubicin (Doxo) 75 mg/m2, and vincristine 2 mg (plus corticosteroids). The last three cycles (B4, B5, and B6) consisted of the same drug combination plus etoposide 300 mg/m2 and cisplatin 100 mg/m2. For an expected duration of 18 weeks, the projected dose-intensity was 25 mg/m2/wk for Doxo and 1,000 mg/m2/wk for Cy. G-CSF 300 micrograms was administered from day 6 following each cycle until neutrophil reconstitution. Two aphereses were performed at approximately day 13 after each A cycle, and PBSCs were injected at day 4 of each B cycle. Radiotherapy on tumor masses > or = 5 cm was scheduled after completion of the last cycle. RESULTS The median duration of grade 4 neutropenia was 1 day (range, 0 to 7) for each A cycle and 4 days (range, 1 to 10) for each B cycle (P = .02). The median duration of grade 4 thrombopenia was 0 days (range, 0 to 8) for each A cycle and 6 days (range, 1 to 21) for each B cycle (P < .001). Hospitalization for febrile neutropenia was required for 18% and 44% of patients during cycles A and B, respectively (P < .01). Only three patients did not complete the protocol: one due to emergency surgery after cycle B4, one who died after cycle B5 from interstitial pneumonia, and one with delayed hematologic reconstitution after cycle B4. Chemotherapy delivery was optimal (median actual relative dose-intensity, 97%; range, 66 to 100). The median total dose administered over 18 weeks was 18,000 mg Cy (range, 12,000 to 18,000), 450 mg Doxo (range, 300 to 450), 900 mg etoposide (range, 300 to 900), and 300 mg cisplatin (range, 100 to 300). Evaluation of response after six courses showed 13 complete remissions ([CRs] 65%), four partial remissions (PRs), two nonresponses (NRs), and one toxic death. With a median follow-up period of 25 months (range, 16 to 43), 15 patients are alive, with 12 in continuous first CR; five patients relapsed (four of four PRs and one of 13 CRs). Two-year survival and failure-free survival (FFS) rates are 73% and 56%, respectively. The disease-free survival (DFS) rate for the CRs is 86%. CONCLUSION PBSC support contributes to the feasibility of first-line, very-high-dose, ambulatory chemotherapy delivery in poor-risk NHL and is associated with a high rate of remission and FFS.

High-dose chemotherapy (HDC) supported by autologous transplantation of blood stem cells (BSC) is used increasingly for patients with poor-risk malignancies. We report our experience with 93 consecutive patients who were mobilized with recombinant human granulocyte colony-stimulating factor (rhG-CSF) alone. They received a fixed dose of G-CSF for 5 or 6 days, and BSC were collected by leukapheresis. Aphereses were evaluated for MNC, CD34+ cells, and CFU-GM counts and cryopreserved. All patients received a conditioning regimen without TBI. Engraftment was assessed as the first of 2 consecutive days on which patients achieved 0.5 and 1 x 10(9)/L neutrophils and an unsupported platelet count of 25 x 10(9)/L. Multivariate analysis was performed to study patients and graft characteristics that could influence reconstitution. The G-CSF priming regimen was well tolerated and allowed collection of BSC for all patients, 66% of them achieving >3 x 10(6)/kg CD34+ cells, and 86% achieving >10 x 10(4) CFU-GM/kg. The numbers of collected CD34 and CFU-GM cells were highly correlated. The number of courses of chemotherapy prior to collection, a diagnosis of breast cancer, the use of rhG-CSF posttransplant, and the numbers of CFU-GM and CD34+ cells reinfused were correlated with hematologic recovery. In a multivariate analysis, however, the number of CD34+ cells was the only factor independently influencing both granulocyte and platelet recovery. Patients who received at least 3 x 10(6)/kg CD34+ cells achieved granulocyte reconstitution on day 11 after reinfusion (range 8-15) and an unsupported platelet count of 25 x 10(9)/l on day 14 (range 12-180), significantly earlier than patients who received fewer cells (p < 0.001). In addition, G-CSF administration postreinfusion independently enhanced granulocyte reconstitution but not platelet recovery. In conclusion, CD34+ cell number appears to be the only factor predicting both granulocyte and platelet reconstitution. Based on this study, the collection of a minimal number of 3 x 10(6)/kg CD34+ cells appears desirable.

Between 1986 and 1993 visceral leishmaniasis (VL) was diagnosed in 50 adult patients with human immunodeficiency virus type 1 (HIV-1) infection (8 females, 42 males: 31 intravenous drug users, 11 homosexual or bisexual men, 6 heterosexual individuals, 2 blood recipients) from 5 hospital centres in southern France. Diagnosis of VL was by demonstration of Leishmania and isolation of promastigotes by culture in Novy-McNeal-Nicolle medium. Leishmania isolates were identified by their isoenzyme profile in 28 patients. All the patients were immunocompromised when VL was diagnosed. Their median CD4 cell count was 25 × 106 (0–200). However, only 21 patients (42%) fulfilled the 1987 CDC criteria for the acquired immune deficiency syndrome before VL developed. Fever (84%), splenomegaly (56%), hepatomegaly (34%), and pancytopenia (62%) were the most common presenting features. Clinical signs were lacking in 10% of patients. Anti-leishmanial antibodies were detected by indirect immunofluorescence or enzyme-linked immunosorbent assay in 26 47 cases (55%). Combining these techniques with Western blotting (WB) gave a positivity rate of 95%. Amastigotes were demonstrated in bone marrow aspirates in 47 cases (94%). Unusual sites for parasites were found in 17 patients (34%), mainly in the digestive tract but also skin and lung. Viscerotropic L. infantum zymodeme MON-1 was characterized in 86% of cases. Dermotropic zymodemes MON-24, MON-29, MON-33, and a previously undescribed zymodeme MON-183, were isolated from 4 patients. The response rate to pentavalent antimony was 50% and to amphotericin B 100%, but clinical relapses were noted in both groups. In endemic areas, VL should be considered as a possible opportunistic infection in HIV-infected patients. WB would be a valuable tool for diagnosis of VL in these 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.

Writer's cramp is a focal dystonia; treatment remains disappointing. We report our 14-year experience with a population of 119 patients aged between 18 and 85 years (average age 43 years).Treatment was based on botulinum toxin injections (Dysport) and physiotherapy. Patients were reviewed every four to six months with clinical and video evaluation by three different observers and subjective analysis of the treatment efficiency by the patient (score of 1 to 3). The post-injection deficit, if present, was also quantified.In the group treated with toxin and physiotherapy, cramps improved (score 2 and 3) in 61.6% of patients; a majority of patients (n=14) reported they were moderately satisfied (score 2). In the group treated with toxin alone, 37.9% of patients were improved (score 2 and 3) with a majority (n=18) very satisfied reporting normal writing (score 3). Age was not a predictor of therapeutic response. Good results were obtained with injections of the flexor carpi radialis followed by flexor digitorum profundus II and III and the flexor pollicis longus. Seventy-one per cent of injections caused moderate muscle weakness, minimally disabling compared to the benefit of injections. Twenty-seven patients were followed for more than two years and three patients, who had achieved score 3 with excellent response, were followed for 14 years with very efficient repeated injections. If the injections were not effective the first time, we re-assessed the situation and adjusted the injections; we considered that toxin treatment was unsuccessful after three injections without benefit.The choice treatment for writer's cramp remains well-targeted injections of botulinum toxin. Physiotherapy is useful when the toxin injections are ineffective in completely improving writing. This requires close cooperation between the injector, the physiotherapist and the patient.

We previously reported a beneficial effect of benfluorex (BFL) on oral glucose tolerance test (OGTT) and visceral fat mass in an open-label study conducted in 60 HIV-infected patients. The objective of this study was to assess whether administration of BFL compared to placebo (PBO) improves insulin resistance (IR) in HIV+ patients with HAART- induced lipodystrophy.22 HIV-infected patients with IR or impaired glucose tolerance were double-blind randomly assigned to receive BFL 3 tablets/day or PBO for 24 weeks. Efficacy assessments included OGTT, abdominal computed tomography, and the measurement of fasting lipids.Change of median insulin AUC was -53.0 microIU/mL (IQR, -126.0 to -12.7) in the BFL group vs. +33.6 microIU/mL (IQR, 7.0 to 115.6) (p = .01) in PBO group. Weight decreased significantly in the BFL group (-2 kg +/- 2.6; IQR, -6.8 to 2.0) compared to the PBO group (0.8 kg +/- 1.7; IQR, -2.0 to 0.5) (p = .02). No significant changes in visceral or subcutaneous fat mass and plasma lipid level were observed between the two groups.Added to antiretroviral therapy, a 6-month therapy with BFL improved insulin sensitivity but is not sufficient to reduce significantly visceral fat mass.

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.

International audience; This study present a Case-control nested analysis of cervical squamous intraepithelial lesions (SIL) in a cohort of 423 HIV-infected women with registered Pap smears between 1991 and 2004. Data on Pap smear results, CDC HIV classification, CD4 cell count and antiretroviral therapy were prospectively collected. Pap smears were classified using the Bethesda classification. Women had a median of 3 Pap smears registered in the database. The first Pap smear was registered ´1996 for 57.7%. Two hundred and twenty women were diagnosed with SIL at least once, mainly at their first screening. Compared to women free of SIL and cancer at all Pap-smear, they were younger, had lower CD4 cell count and were more likely included ´1996 and less likely treated with HAART. These differences persisted in multivariate analysis. Among the 142 women with secondary pap smears after SIL diagnosis, 43 (30.3%) worsen their Pap smear results. No specific characteristics could be identified for these patients compared to those who did not. Finally 21 women were diagnosed with cervix cancer, 3 at first Pap smear and the others after a previous SIL diagnosis in a median delay of 3 years. These results reflect the change in gynecologic management of HIV-infected women after HAART introduction but also underline the necessity to maintain a regular gynecologic screening for HIV-infected women.

Chronic lymphocytic leukemia (CLL) is the most common leukaemia in the Western world. Recent advancement in the aetiology, pathophysiology and the development of new therapeutics tools have significantly modified the current management of CLL.The cellular origin of CLL is still unknown. The current main hypothesis will be first briefly described. This review will then focus on the newly defined prognostic factors and the development and use of new drugs for the treatment of CLL. To describe the modern and practical management of CLL, we will compare classical and new prognostic markers. Then, we will discuss the various therapeutic options including chemotherapy and immunotherapy (monoclonal antibodies, allogenic transplantation), and define their current respective indications.These new diagnostic and prognostic markers will allow the characterization of new prognostic subgroups of patients. This will lead to a targeted and individualized therapeutic approach. We will present the first results of clinical trials and the on-going studies conducted in this disease.

This retrospective and longitudinal study evaluated the long-term hepatic tolerance of a nelfinavir (NFV)-antiretroviral combined regimen in 82 patients of the HCV-HIV Cohort of CISIH-Sud of Marseilles. Follow-up data (liver enzyme levels, CD4 cell count, HIV viral load, and metabolic parameters) of patients treated with NFV on inclusion or during the follow-up of the cohort were analyzed under treatment over 24 months. Comparisons were performed with X2 or Kruskal-Wallis tests. At baseline (n = 82), the median exposure to NFV was 4.1 months; 58 patients received NFV combined with NRTI and 24 with NNRTI. The median CD4 cell count was 337/mm3 [interquartile range (IR): 216-480) and 39.7% had an undetectable HIV RNA level. Qualitative HCV PCR was positive in 91% of the patients and 19/51 patients with liver biopsy were F3-F4. Median alanine and aspartate aminotransferase (ALAT, ASAT), gamma-glutamyltransferase (GT), and alkaline phosphatase (ALP) were 46 UI/liter (IR: 36-76), 55 UI/liter (IR: 32-97), 97 UI/liter (IR: 50-194), and 88 UI/liter (IR: 72-104), respectively, with 76% of the patients with ALAT/ASAT grade2. Median follow-up was 23 months (IR: 13.8-37). No significant difference was observed in the distribution of ALAT, ASAT, GT, and ALP as well as of ALAT/ASAT grades over the 24-month study period. Patients treated with NFV + NNRTI had significantly higher GT and ALP levels at baseline with no significant increase during follow-up. Cholesterol, triglyceride, and glycemia distributions remained stable over time. In conclusion, this study showed a good hepatic and metabolic tolerance of a long-term NFV-combined regimen in HIV-HCV coinfected patients.

We have examined 9 healthy volunteers and 63 HIV-patients (16 asymptomatic patients and 47 patients with clinical AIDS-dementia complex, ADC) by magnetic resonance spectroscopy (MRS) and imaging (MRI) on a Siemens Magnetom SP63 (1.5 T). Proton MRS of the brain was performed at 63 MHz using the PRESS sequence (echo time = 135 ms, TR = 1.6 s). Four main results have been found: (1) HIV-related encephalopathy induces significant modifications of brain metabolism analyzed by MRS and the most sensitive metabolic parameter is the N-acetyl-aspartate/Choline ratio, (2) the correlation between MRS and MRI is good in 75% of patients, (3) in 4 of the 16 neuro-asymptomatic patients (i.e. 25%) a metabolic encephalopathy was found while MRI was still normal, and (4) MR spectra describe 3 different pathological metabolic patterns in the brain of HIV patients. Two patterns might correspond to the two entities of HIV-induced lesions i.e. HIV encephalitis and HIV-related progressive leukoencephalopathy.

Extramedullary (EM) localizations at diagnosis or during the course of multiple myeloma (MM) are rare. We conducted a large retrospective study to more accurately describe the clinical and laboratory features of this entity, and the outcome of these manifestations. The charts of 19 eligible patients out of 432 patients with MM were retrieved from the hematology department of the Institut Paoli-Calmettes Cancer Center. Median age was 61 (range: 39-79) with a female/male sex ratio of 8/11. Ten patients were found to have EM and extraosseous tumor at the time of MM diagnosis, and nine patients developed EM tumor during the course of the disease. Neither the stage of the disease, the LDH level, or the type of immunoglobulin (Ig) was found to be associated with the development of EM disease. Patients who developed EM tumor during the course of MM had a lower serum Ig and a higher monoclonal Bence-Jones proteinuria at the diagnosis of MM than patients who presented with EM tumor at diagnosis. Multiple sites were usually involved. Resistance to chemotherapy was frequent and response to thalidomide was poor. Eight out of the 19 patients responded to high-dose chemotherapy. The remaining 11 patients progressed while on therapy. With a median follow-up of 13 months (range: 2-65), six patients are alive, four patients are in partial remission and two patients in present progressive disease. In conclusion, EM tumors are a rare manifestation of MM, with a cumulative incidence of 4.6% of MM. Multiple sites are usually involved. The response to chemotherapy is very poor with a very low response rate to thalidomide. The prognosis is very poor, especially when the diagnosis of EM tumor is concurrent with the diagnosis of MM.

The use of reduced intensity conditioning (RIC) regimens for allogeneic stem cell transplantation (allo-SCT) can result in a significant decrease in early procedure-related toxicity in patients not eligible for standard myeloablative regimens. However, acute graft-versus-host disease (aGVHD) remains a matter of concern after RIC allo-SCT, and its incidence might be expected to be higher in elderly and high-risk patients. This report investigated mycophenolate mofetil (MMF) and cyclosporin A (CsA) combination (n=14) in comparison to CsA alone (n=20) for GVHD prophylaxis in cancer patients aged over 50 years (27 haematological malignancies and seven solid tumours) receiving an HLA-identical sibling antithymocyte-globulin (ATG)-based RIC allo-SCT. Baseline demographic characteristics and risk factors for aGVHD were comparable between both groups. Although MMF administration was not associated with any significant toxicity, the cumulative incidence of any form of GVHD was comparable between both groups (cumulative incidence of grade II-IV aGVHD, 50% (95% CI, 28-72%) for CsA alone, as compared to 64% (95% CI, 39-89%) to CsA and MMF, P=NS), suggesting that adjunction of MMF to CsA is feasible, but does not translate towards a significant reduction of aGVHD, at least in the context ATG-based RIC allo-SCT.

To evaluate tolerance and efficacy of an open-label interferon-ribavirin treatment and their determinants in 62 HCV-HIV coinfected patients in routine followup.Patients received at least 6 and up to 12 months of combination interferon alpha-2b (peg or not) plus ribavirin. Determinants of therapeutic success were estimated by a multivariate logistic regression.Five patients stopped the study, 4 were lost to follow-up, and 53 participated in the entire therapeutic protocol. Among these 53, the end-of-treatment results showed complete clearance of HCV-RNA in 17 (32%). A sustained virologic response (SVR) after 6 or 9 months was observed in 9 (17%) patients, 3 relapsed, and data were not available for 5. Genotype 3a (odds ratio [OR] = 14.4; confidence interval [CI] = 1.84-110.3) favored SVR and treatment with protease inhibitor (PI) therapeutic resistance (OR = 14.4; CI = 1.01-200); as well, a higher fibrosis score tended to increase resistance (p =.11). Adverse events were reported by 24/53 patients (45.3%).HCV therapy associating interferon and ribavirin in HCV-HIV coinfected patients is well accepted even if tolerance is moderate. Treatment permitted SVR in at least 17% of the cases. This is likely when patients initiate treatment at the early fibrosis stage and are infected with genotype 3a. The potential interaction with PI therapy should be explored.

A 57-year-old man with acute myeloid leukemia (AML) French-American-British (FAB) 4 developed disseminated invasive cerebral and pulmonary aspergillosis during postinduction aplasia. According to international consensus, infection was categorized as probable (two host factors: deep neutropenia for >10 days and refractory fever for >96 h; major clinical criteria of lower respiratory tract and CNS invasive fungal infection; positive results for galactomannan antigen in three blood samples). After the failure of standard amphotericin-based therapy, the spectacular regression of multifocal brain and lung lesions was rapidly achieved under a caspofungin acetate/voriconazole combination. Further permanent caspofungin maintenance with voriconazole added during aplasia periods permitted two consolidation courses and autograft-based intensification without any delay.

DFT (fronto-temporal dementia) was diagnosed in 33 patients (17 males and 16 females), mean age 74.9 years, using the criteria from Neary et al. (1998). The severity of the disease was evaluated based on the presence or absence of 4 clinical groups of signs (behavioral, affective, neurological signs and language) allowing the calculation of a global clinical ratio on 20. A CT-scan and a Single Photon Emission Computed Tomography (SPECT) scan using Tc99m-ECD with quantification of brain perfusion were then performed. This study confirmed the classical clinical forms of DFT: a psycho-affective form (21%), where negative symptoms are predominant, and a behavioral disinhibited form (9%). Most of our cases (40%) corresponded to a global form. Finally we also identified a fourth clinical form: cognitive form because of predominance of language disturbances (30%). Concerning SPECT, we found 4 topographic forms: frontal global (46%), frontal superior (24%), frontal inferior (15%) and frontal unilateral (12%). In the frontal inferior form, behavioral disturbances were correlated to the intensity of frontal inferior hypoperfusion, corresponding to an orbito-frontal dysfunction. In the frontal superior form, psycho-affective disturbances (apathy) were correlated with frontal superior hypoperfusion (anterior cingulum and superior dorso-lateral cortex). Finally, in global and frontal inferior SPECT forms, language disturbances were correlated with a frontal inferior hypoperfusion (prefrontal and inferior left frontal cortex).

Subclinical impairment of the peripheral nervous system (PNS) is frequent during HIV infection. Nevertheless, little attention has been given to the autonomic nervous system (ANS). We undertook a prospective study of the ANS, exploring 55 HIV+ subjects with non-invasive functional tests for the cardiac ANS. We also made a quantitative study of thermic (small fibers) and vibratory (large fibers) sensibilities. The results do not show any statistically significant difference between HIV+ subjects and control subjects or between HIV+ subjects of groups I, II and III and those of group IV of the CDC classification. However, an individual analysis comparing the results of the infected subjects with those of the control group indicated the presence of functional abnormalities of the ANS (small fibers) in 34.5% of HIV+ patients with an equally frequent impairment of the thermic perception (small fibers) and of the vibratory perception (large fibers). Moreover, the signs of subclinical dysautonomia were more frequent in group IV (48.3%) than in groups I, II and III (19%) showing a progressive accentuation of the troubles during the course of the infection.

The authors report two cases of hemichorea-hemiballism revealing a toxoplasmic abscess in the subthalamic nucleus during AIDS. Despite the great frequency of this opportunistic infection such cases are exceptional. This rarity seems to be explained by the frequent association of other lesions of the basal ganglia which may prevent the movement disorders. Nevertheless an hemichorea-hemiballism in young people must suggest a cerebral toxoplasmosis. Finally a symptomatic treatment is often necessary; in these cases the use of sodium valproate must be considered, this drug being able to induce a clinical improvement.

Approaches using reduced conditioning regimens have been developed to obtain minimal procedure-related toxicity. Such novel therapeutic options are being explored with good preliminary results concerning feasibility and engraftment. However, many aspects remain under-evaluated and few data are available about immune and dendritic cell (DC) reconstitution after these highly immunosuppressive regimens. We present here our data in 20 patients receiving allogeneic bone marrow transplantation (allo-BMT) using a reduced preparative regimen. We evaluated in the first 3 months following allo-BMT, several immunological parameters including DC subsets, and compared these to historical results obtained in a group of myeloablative allo-BMT patients. We found an early recovery of leukocytes, CD8+ and NK lymphocytes. We also found a trend towards an improved B cell recovery. These results are somewhat in contrast to the altered immune recovery observed in the myeloablative setting. In addition, we found a significant early circulating DC recovery. Circulating blood DCs were also found to be of full donor origin as assessed by FISH in sex-mismatched pairs. Nevertheless, naive CD4 + CD45RA + T cells were found to be profoundly reduced following such regimens.Collectively, these data further enhance the overall benefits of reduced intensity regimens and the need for a stringent biological monitoring for assessment of the potential advantages of reduced intensity allo-BMT in comparison with conventional allo-BMT.

We describe the follow-up of a cohort of 255 Alzheimer's disease (AD) patients (81 males, 174 females) treated by tacrine during 4 years. We performed the survey of hepatic, cholinergic and general tolérance. Drug efficacy was measured by MMS examination on weeks 0, 18, 30, 52, 104, 156 and 208. A total of 190 patients (74.5 percent) were dropped out of this study, 75 (29 percent) for adverse events. We found 85 hepatic (33 percent), 79 cholinergic (31 percent), 31 (12 percent) neuropsychiatric and 72 general (28 percent) side effects. In term of drug efficacy we observed a global decline of 2.5 MMS points during the first year and 2 MMS points between W52 and W156. Tacrine's symptomatic efficacy, defined as the number of patients improved or stabilized at W30, was present in 50 patients (46 percent) among the 109 patients reaching W30. The intensity of symptomatic efficacy was expressed by a 2.7 MMS points increase in 37 patients improved on W30. The long term effects of Tacrine, measured by the MMS score at one year, showed a positive impact as the MMS was 2.5 points above the expected score in non treated AD patients. This study raises the practical problem of optimal cholinesterase inhibitors use in AD and the theoretical question of long term action of cholinesterase inhibitors on cerebral lesions of AD.

The incidence of human immunodeficiency virus (HIV)-Leishmania coinfections in France was estimated on the basis of the French Hospital Database on HIV, and risk factors for the occurrence of visceral leishmaniasis (VL) were analyzed by a multivariate Cox model. VL was diagnosed in 165 of 55,626 HIV-infected patients followed since 1992. The incidence of VL decreased from 11.6 ± 1.2 per 10,000 persons-years before 1996 to 6.3 ± 0.7 per 10,000 persons-years after 1996, the year when highly active antiretroviral therapy (HAART) was initiated in France. The relative hazard (RH) for development of VL was higher in (1) intravenous drug users versus other transmission groups (RH = 1.56; 95% CI, 1.13-2.15), (2) patients living in southern France versus those living in northern France (RH = 3.36; 95% CI, 2.44 -4.61), and (3) patients who had a CD4 cell count of ≤50/mm 3 during their follow-up versus those who did not (RH = 6.45; 95% CI, 4.27-9.75) but was lower in (4) patients who received antiretroviral therapy including ≥3 drugs versus those who did not (RH = 0.41; 95% CI, 0.26-0.65). We found a significant decrease in the incidence of HIV-Leishmania coinfections after 1996, associated with the introduction of HAART in France.

Fifty-two patients at various stages of human immunodeficiency virus (HIV) infection who had one or several epileptic seizures in the course of that disease were retrospectively studied from 1985 to 1990. Thirty-five percent of these patients were in overt clinical AIDS at the time of the seizure(s). AIDS was revealed by a seizure in 2 cases. Generalized seizures were observed in 71 percent of the patients, and partial seizures in 29 percent. Electroencephalograms showed signs of brain irritation in only 19 percent of the cases. The cause of epileptic seizure(s) could be determined in 36 patients: cerebral toxoplasmosis in 23 cases; progressive multifocal leucoencephalitis in 2 cases; HIV encephalopathy in 3 cases; iatrogenic cause in 4 cases; meningoencephalitis in 3 cases and neurosyphilis in 1 case. No cause other than HIV infection was found in 16 patients. These findings confirm those of previous studies. In about one-third of AIDS patients epileptic seizures are the only clinical manifestation of viral central nervous system infection.

Summary: Purpose: To describe a particular form of absence epilepsy (AE) characterized by an atypical EEG pattern (fast rhythmic discharges) during sleep and an unfavorable course with the persistence of seizures at a late age and drug resistance. Methods: We reviewed the medical files of 31 patients diagnosed with AE at our institution been 1995 and 1999 for whom an EEG during sleep had been recorded. Patients with fast rhythmic discharges during sleep were selected for the study. Results: Five patients showed the usual criteria of childhood absence epilepsy (CAE) or juvenile absence epilepsy (JAE), but with fast discharges of rhythmic spikes (10–15 Hz) during sleep, such as those typically observed in Lennox–Gastaut syndrome. Four patients were of adult age at the time of the last examination. Intellectual capacities were “borderline” in each case, with visible social and learning handicaps. Absences were the initial seizure event in all patients, but four patients developed generalized tonic–clonic seizures during the course of the disease. Treatment using a combination of antiabsence drugs such as valproate and lamotrigine led to only marginal improvement. Conclusions: These findings illustrate the utility of sleep EEG in detecting the fast rhythmic discharges that are markers of drug resistance in AE and suggest the existence of transitional forms of AE that are intermediate between idiopathic and cryptogenic/symptomatic generalized epilepsies.

Thymoma is a tumour originating in the epithelial cells of the thymus, associated with several immunologic disorders. The association of thymoma with systemic lupus erythematosus has rarely been described. We report two cases of this association.Description of two cases and a review of the literature. Mr T. was 41 years old when the diagnosis of thymoma and lupus was made. The thymectomy did not influence the evolution of his lupus. Mrs G. had been treated because of a lupus for 8 years prior to developing a thymoma. One year later she presented with erythroblastopenia, which was only sensitive to cyclosporin.The association between lupus and thymoma has been reported in 36 cases in the literature. Thymoma is benign in 59% of the cases. The clinical presentation of lupus is nonspecific except for age, median 48 years, and sex ratio, 4:3. The clinical outcome of the lupus is not influenced by the thymectomy. Thymoma may precede lupus with a delay of several years or it may be diagnosed concurrently or several years later. This association is not accidental, though the pathogenic link between these conditions remains unknown. One could suppose that the decrease of the thymic function in the course of thymoma could enhance the expression of autoreactive T lymphocytes as well as the activation of B cells. Patients should be followed after thymectomy because autoimmune diseases, particularly lupus, may develop belatedly. On the other hand, thymoma may be suspected mainly when lupus occurs in patients around 50 years of age.

Background: Aggressive non-Hodgkin's lymphomas (NHL) in patients under the age of 60 have a very poor prognosis when the international prognostic index (IPI) is high, with an age-adjusted (Aa)-IPI score at 3. In such patients, conventional chemotherapy results in a low complete response (CR) rate of 46%, a five-year survival and disease-free survival (DFS) of 32% and 58%, respectively. For this report we have analyzed whether front-line high-dose chemotherapy could influence the outcome of this group of patients. Patients and methods: From 1992 onwards we conducted two pilot clinical trials of intensive sequential chemotherapy ( ISC) with growth factors and blood stem cell support as initial treatment in 62 poor-risk patients with aggressive NHL. Of these patients, 33 were considered to be a high-risk group based on the Aa-IPI. Results: The median age was 42 years (range 21-60). The treatment was completed in 88% of patients, 86% receiving greater than 75% or more of the projected dose-intensity. Twenty patients (61%) achieved a CR. At a median follow-up of 48 months (range 26-86), the estimated five-year survival and DFS was 51% (95% confidence interval (CI): 34%-68%) and 70% (95% CI: 50%-90%), respectively. Conclusion: These results suggest that primary treatment using high-dose therapy supported by both growth factors and peripheral blood stem cells can cure up to 50% of high-risk patients with malignant lymphomas.

We have studied 95 HIV seropositive patients (77 males and 18 females; mean age: 31 years): 67 had no neurological symptoms or signs, 28 had various neurological symptoms and signs. This study included a full multimodal evoked potentials (MEP) assessment: visual evoked potentials by flash and reversal checkerboard; brainstem auditory evoked potentials; somatosensory evoked potentials obtained by stimulation of the median nerve. Patient evaluation further included: electroencephalography, electromyography with measurement of conduction velocities and neuroimaging (brain CT scan and/or MRI). We found abnormal MEP for all modalities. The prevalence of abnormal results was high in neurological symptomatic patients; in non neurological ones, the changes tended to be more frequent with the progression of the HIV infection. Whatever the stage of the disease, the various modes were equally affected. MEP were abnormal in 54.7 p. 100 of the cases: in 41.8 p. 100 (28/67) of patient without neurological signs (in 4/12 of fully asymptomatic subjects, 11/34 ARC patients and 13/21 AIDS patients) vs 85.7 p. 100 of neurological symptomatic patients. In neurological asymptomatic patients, a similar proportion of abnormal MEP was found in asymptomatic and ARC patients, while the evolution into AIDS was associated with a higher prevalence of abnormal MEP. In the latter group, MEP changes were nearly as frequent as in neurological symptomatic patients. Comparison between MEP and other electrophysiological procedures (EEG, EMG) and with neuroimaging techniques (CT Scan, MRI) showed the high sensitivity of the MEP technique at all stages of the disease. EMG was a sensitive method and complementary to MEP. The EEG and neuroimaging techniques showed abnormalities principally at the neurological symptomatic stage. Previous studies could not be properly compared.(ABSTRACT TRUNCATED AT 250 WORDS)

We report a case which illustrates the fact that an exacerbation of Charcot Marie Tooth disease, while rare, is possible during pregnancy. Moreover our case suggests the possibility of a positive effect of corticosteroids on such a complication, with an improvement of clinical symptoms as well as of electrophysiological results.

Intracranial lipomas are congenital malformations composed of mature adipocytes. They are usually located in the midline, particularly in the pericallosal region, a hemispheric location accounting for only 3 to 7% of cases. Review of the literature found 21 previous cases of hemispheric lipoma. Although hemispheric cerebral lipomas are rare, association with epilepsy appears to be frequent. We have recently studied two patients in whom epilepsy was the first clinical manifestation of hemispheric cerebral lipoma in the sylvian region. The patients presented with simple motor partial seizures as the first manifestation of the lesion. Neurological examination was normal. MRI disclosed in both cases a lesion involving the sylvian fissure with characteristics of the lipid signal. MRI also demonstrated abnormalities involving the cerebral cortex in the vicinity of the lesion (pachygyria-like aspect). Partial excision of the lesion was achieved in one patient but was followed by a worsening of seizures and neurological condition (hemiparesis). According to the literature, the prognosis for epilepsy in patients with hemispheric lipoma appears good. Several other arguments support non-surgical management: the lesion is benign and can be identified with a high degree of certainty by imaging; surgery is technically difficult due to adherence to adjacent vascular and cerebral structures and hypervascularity; location near functional brain tissue increases the risk of postoperative sequelae. In addition, mechanisms of epilepsy probably involve vascular and cortical dysplasic abnormalities. In consideration of the complexity of the lesion, hemispheric lipomas are more appropriately classified with localized cortical malformations rather than as simple extracerebral malformations.

To assess adherence to highly active antiretroviral therapies (HAART) in a cohort of French patients infected by HIV through injection drug use (IDU), and the impact on adherence of buprenorphine ambulatory drug maintenance treatment (DMT) which has been widely introduced since 1996.Adherence assessment at first visit after initiation of HAART in the MANIF2000 cohort study.Patient's face-to-face and self-administered questionnaires. Univariate and logistic regression adjusted odds ratios (OR) to compare characteristics of non-adherent versus adherent patients.Of the 164 patients, 34.8% took less than 80% of the prescribed HAART doses during the previous week. Decrease in viral load titres after initiation of HAART was significantly lower among non-adherent patients. After adjustment by logistic regression, non-adherence was associated with younger age, alcohol consumption, frequency of negative life-events during the prior 6 months and active drug use. However, IDU in buprenorphine DMT reached higher levels of adherence (78.1%) than ex-IDU (65.5%), although this difference did not reach statistical significance.Prescription of buprenorphine DMT may increase adherence to HAART among HIV-infected opiate-dependent patients. Reducing the negative impact of stressful life-events through psychosocial interventions should be considered, even for those who have stopped using drugs.

The rationale for immune control of cancer is now better defined via the immunovirology of transforming viruses, definition of human tumor antigens recognized by T-lymphocytes, and cellular and humoral components of the anticancer response. Nonetheless tumors can escape from immune surveillance. To better define immunomodulation strategies, we describe some of the various strategies developed by transformed cells to evade the immune response.Both the lack of specific tumor antigen and down-regulation of major histocompatibility complex (MHC) molecule expression hamper recognition of neoplastic cells by T-lymphocytes. In presence of defective expression of ligands for the T-cell co-stimulatory receptors, tumor recognition may lead to the development of tolerance instead of specific cytotoxic activity. Tumor cell counter-attack against effector T-cells has also been described, using either inhibitory cytokines (IL-10), apoptosis induction (via Fas signalling), functional inactivation (disruption of normal CD40/CD40 ligand interactions), or induction of anergy.Despite the many different mechanisms of tumor escape, the immune system has developed efficient counter-attacks. For instance, natural killer cells may detect and destroy tumor cells that lack class 1 MHC molecules and thus escape from specific T-lymphocyte cytolysis. Moreover, immunogenicity can be restored, at least in vitro, by different means such as tumor cell stimulation by cytokines or CD40, suggesting that therapeutic strategies will soon be developed in order to stimulate an efficient antitumoral immune response.

In several experimental models, status epilepticus (SE) leads to secondary brain hyperexcitability and epileptogenesis. In humans, such phenomena have been rarely demonstrated, particularly in cases of SE involving the neocortical structures. We report a 36 year old woman that presented partial SE in May 1991 involving the right cerebral hemisphere. The patient was then treated in the intensive care unit with artificial ventilation and anesthesia by pentobarbital and clometiazole. MRI showed transient right parietal and temporal posterior cortical hyperintensity. The cause of SE was not determined. Three months later, the patient developed partial complex seizures with aura characterized by vertigo, nausea and auditory hallucination. Ictal video/EEG recording showed a clear right temporal posterior onset of the discharges. We speculate that status epilepticus created the lesions which subsequently caused the focal chronic epilepsy.

Rationale: Cortical dysplasia (CD) designates a diverse group of malformations resulting from one or more abnormalities in the development of the cerebral cortex. The clinical manifestations of CD are varied, probably depending on the type, location and extent of CD. Epilepsy is a potential late manifestation of any cortical malformation. To our knowledge, however, no study has focused specifically on late onset of epilepsy in patients with localized CD. Material and Methods: We studied patients with localized CD confirmed by MRI. Patients were divided into 2 groups according to age at onset of epilepsy. Group 1 included patients in whom the first seizure occurred up to the age of 12 (early-onset group) and group 2 included patients in whom the first seizure occurred after the age of 12 (late-onset group). The two groups were compared with regard to the type of CD, clinical findings and EEG findings. Results: Thirty-three patients with various forms of CD were studied. Onset of epilepsy occurred in adolescence or adulthood in 9 cases (37%). In 6 of these (17% overall), the first seizure occurred in adulthood. CD were posterior bilateral pachygyria (1), unilateral polymicrogyria (3), focal dysplasia with subcortical gray matter heterotopia (1), perisylvian bilateral polymicrogyria (1), bioccipital polymicrogyria (1) and bilateral nodular periventricular gray matter heterotopia (2). The incidence of neurological signs was lower in the late-onset group. Mental retardation was moderate or absent, thus allowing a fairly normal lifestyle. All patients presented partial seizures with a lower incidence of drug resistance (p < 0.01). EEG demonstrated preservation of background activity and absence of diffuse or multifocal abnormalities. Conclusion: Onset of epilepsy with various forms of CD may be delayed until adolescence or adulthood. Prognosis of epilepsy is usually more favorable in these cases.

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.94 symptom-free patients, naïve to antiretroviral treatment, with CD4-T-cell counts of 250-550 cells/microL 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/m2 twice daily, n=24) for 5 days, or were given polyethylene-glycol-modified (PEG) interleukin-2 (2 million IU/m2 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.CD4-T-cell count increased to higher than baseline by a mean of 564 cells/microL (subcutaneous group), 676 cells/microL (intravenous group), 105 cells/microL (PEG group), and 55 cells/microL (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 (p0.001). In these two groups, 50% of patients restored a CD4/CD8-T-cell ratio of more than 1. The groups did not differ significantly for changes in plasma HIV-1 RNA loads throughout the study. The duration of common side-effects of interleukin-2 was shorter in the subcutaneous group, which enabled outpatient treatment. Naïve and memory CD4 T cells, CD28 expression on CD4 and CD8 T cells, and restoration of in-vitro proliferative response to mitogens and recall antigens increased in the intravenous and subcutaneous groups.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.

Myalgia can occur in active drug addicts as well as subjects undergoing withdrawal with methadone. HIV infection can also cause painful muscle complications.

We report an autopsy case of a malformed fetus with thymic hypoplasia. The autopsy was performed after therapeutic termination in the second trimester of pregnancy. The HIV-1 infected mother had received 3 antiviral agents and treatment for opportunistic infections.