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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.

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.

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.

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.

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.

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.

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).

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.

We report our first 100 cases of Alzheimer's (AD) patients treated with tacrine (Cognex) for a period of one year. At the beginning of treatment the mean Mini-Mental-Status (MMS) score was 15.1. To date 71 patients are still under treatment (12 for more than 12 months). Forty-three instances of side-effects were observed, of which 31 involved hepatic side-effects with an increase in ALATIN (normal value) (6 cases3N), the mean date of appearance was 10.4 +/- 6.8 weeks, there were 16 cholinergic side-effects (nausea, vomiting, diarrhoea), plus 4 neurologic and 2 cutaneous side-effects. These side-effects led to the arrest of the treatment in 19 cases (16 for hepatic toxicity). Treatment was reattempted after interruption in 13 cases; successfully in 3 instances only. The measure of tacrine efficacy was based on 52 MMS score re-evaluations in week 18: there was an increase of the MMS score in 22 cases (3.3 points +/- 2.5), a stabilisation in 11 cases and a decrease in 19 cases (3.3 points +/- 2.2.). In week 30, the MMS scores (35 patients) increased in 9 cases (3.6 points +/- 2.4), stabilized in 5 cases and decreased in 21 cases (3.9 points +/- 3.3). At week 52, only 28 per cent of the patients were considered as either improved or stabilized. We conclude that there is a necessity for close follow-up of tacrine-treated patients, and that globally at 8 months there is an improvement or a stabilization in 40 per cent of patients and long term (at one year) a stabilizing effect on AD patients.

We report two cases of "frontal lobe dementia", patho-anatomically defined by non-specific as neuronal loss, gliosis and spongosis. The review of the literature permit to discuss the clinical picture of "fronto-temporal dementia" in regard to neuropathological basis. Further, the boundaries with Pick's disease, Kraepelin disease, subcortical gliosis and the "prions diseases" are considered. Without more precise nosological status, tacking in count the great variability of the clinical signs, we can concluded that, at present time, the term of frontal type dementia with "non-specific histological features", that underlines the neuropathological characteristics is the most appropriate to define this clinico-pathological entity.

We have analyzed the cerebrospinal fluid (CSF) of 19 patients with multiple sclerosis (MS), 12 patients with degenerative dementia and 17 control patients using in vitro high resolution proton magnetic resonance spectroscopy (MRS) at 400 MHz. The CSF metabolic profile is slightly modified in MS patients (increased lactate and fructose concentrations, decreased creatinine and phenylalanine concentrations) and is not correlated with the intensity of the intrathecal inflammation. Proton MRS of CSF does not differentiate relapsing-remitting MS and primary progressive MS. We have not detected any specific abnormal resonance in native or lyophilized CSF. The CSF metabolic profile of demented patients is much more altered (increased concentration of lactate, pyruvate, alanine, lysine, valine, leucine-isoleucine, tyrosine, glutamine) and is in agreement with a brain oxidative metabolism impairment as already described in Alzheimer's disease. Unassigned abnormal but non specific or constant resonances have been detected on MR spectra of demented patients. CSF inositol concentration is also increased in the CSF of patients with Alzheimer's disease. In vitro high resolution proton MRS of the CSF constitutes a new and original way to explore CSF for the differential and/or early diagnosis of dementias, as a complement to in vivo proton cerebral MRS.

A prospective randomized study was conducted comparing the efficacy and toxicity of two anthracyclines for the treatment of patients with acute myeloid leukemia (AML) between 55 and 75 years. A total of 220 patients were randomized to receive as induction chemotherapy cytosine arabinoside (Ara-C: 100 mg/m2/day; continuous infusion for 7 days) combined with either daunorubicin (DNR: 50 mg/m2/day, i.v. bolus for 3 days) (n=108) or idarubicin (IDA: 8 mg/m2/day, i.v. bolus for 5 days) (n=112). The complete remission (CR) rate was similar (P=0.296) after IDA (76/112; 68%) and DNR (66/108; 61%) (P=0.3). For patients aged 55-65, the CR rate was significantly higher after IDA (39/47; 83%) than after DNR (29/50; 58%) (P=0.007). Persistent leukemia was more frequent after DNR (26/108) than after IDA (13/112; P=0.015). Hematological and extra-hematological toxicities were similar. The CR patients were given a consolidation course of chemotherapy with Ara-C: 50 mg/m2/12 h, subcutaneously for 5 days, combined with either DNR:30 mg m2/day, i.v. bolus for 3 days or IDA:8 mg/m2/day i.v. bolus for 3 days according to the initial randomization, and then received a continuous maintenance treatment for 2 years. The survival and disease-free survival (DFS) were similar in both groups; there was no difference in the risk of relapse. However, there was a trend for a longer event-free survival (EFS) in the IDA group than for the DNR patients (P=0.07). Our results seem to indicate that IDA is probably more efficient than DNR for AML patients between 55 and 75 years, and confirm the data published in other studies comparing prospectively IDA and DNR in adults.

The first cases of Hodgkin's disease (HD) associated with HIV infection were reported in 1984. Since then, short series of seropositive patients suffering from HD have been published. In order to identify the characteristics, treatment response and outcome of HIV-associated Hodgkin's disease (HIV-HD), the data of HIV-HD patients recorded between 1987 and 1989 were analysed and compared with those of primary HD patient and with those of HIV-associated non-Hodgkin's lymphoma (HIV-NHL), registered during the same period.The 45 cases of HD collected by the French registry of HIV-associated tumors between January 1987 and December 1989 were included in this study. All patients were clinically staged according to the Ann Arbor system. To compare HIV-HD characteristics with those of primary HD, we used a cohort of 407 patients with clinical stages (CS) IA to IVB, who were enrolled between September 1981 and August 1988 in a multicentric clinical trial. To identify the relationship between HIV-HD and the course of HIV infection we studied, when available, the routes of infection, initial CD4 cell count at the moment of HD diagnostic as well as the CDC class of HIV infection and compared these data with the same parameters observed in 142 HIV-NHL enrolled in the registry during the same period.HIV-HD is characterized by an increase in mixed-cellularity histology (49%), with a predominance of advanced stages (75%) and B symptoms (80%). A unique observation is made regarding mediastinal involvement, present in only 13% of HIV-HD (71% in primary HD). The HIV-HD/HIV-NHL ratio was significantly higher in intravenous drug abusers than in male homosexuals. Median CD4 cell count was 306/microliters at HIV-HD diagnosis, and only 11% of the cases were preceded by an AIDS manifestation. With standard therapy, 79% of the patients achieved complete remission, but hematological and infectious complications were very frequent. The progression to AIDS rate was 94% at two years and opportunistic infections were the most frequent cause of death. Overall two-year survival was 41% with 71% for patients with initial CD 4 cell counts higher than 300/microliter and 0% for those with CD4 cell counts lower than 300/microliter (P0.01).HIV-HD has a particular clinico-pathological profile when compared to primary HD, with a predominance of mixed-cellularity type, a high frequency of advanced stages and a high proportion of patients without mediastinal involvement. Moreover, HIV-HD seems to occur preferentially in the group of subjects infected by needle sharing. Standard HD therapy seems to be efficient but excessively toxic.

Local injections of botulinum toxin constitute the only truly effective treatment of certain abnormal movements and focal dystonias. The authors describe its indications and report on their personal experience. One hundred and seventeen patients were treated: 48 for blepharospasm, 46 for hemifacial spasm and 23 for spasmodic torticollis. The results were evaluated by means of a score taking into account the effectiveness of treatment, the duration of this effectiveness, the side-effects, if any, observed, and the course of the neurological disorder after several series of injections. The results were good or excellent in 91 percent of patients with hemifacial spasm and 79 percent of patients with blepharospasm. Spasmodic torticollis was much improved in 35 percent of the cases and less, but satisfactorily, improved in 48 percent. In this disease, the muscles which antagonize those responsible for the dystonia must absolutely be re-educated.

Q fever is caused by Coxiella burnetii, a strictly intracellular bacterium that lives within the phagolysosome of infected cells. We report here five cases of Q fever in patients with cancer. Three of them had a solid tumor, one had a B cell lymphoma, and one had chronic myeloid leukemia. One patient had acute Q fever, and the four others had chronic Q fever endocarditis. Two patients with endocarditis had no previous history of valvulopathy. C. burnetii was isolated from the valves of two patients. One of the patients with endocarditis died. Patients with cancer who have unexplained fever and live in areas in which C. burnetii is endemic should undergo serological testing for infection with this microorganism.

One study was performed in 12 patients with presenile Alzheimer's disease (group I), the other one in 16 patients with mild senile dementia of Alzheimer type (group II). In each study, patients were divided into two randomized parallel groups, one receiving placebo, the other piracetam (9 g daily in group I piracetam and 2.4 g daily in group II piracetam) during three months, piracetam induced a decrease in EEG power on the 2-6 Hz range (group I piracetam), 3-5 Hz and 7 Hz (group II piracetam) and an increase of EEG power in the 9-11 Hz range (group I piracetam) and in the 10 Hz and 13 Hz frequencies (group II piracetam). There was also a significant improvement in the Trail Making Test part A in group II piracetam. Correlations between decreased EEG low frequency components and improvement in some psychometric tests were found significant in the two groups. It seems that the main effect of piracetam was to induce increased alertness. The same results were found in both studies; the good reproducibility suggests that EEG spectral analysis is a reliable tool in the assessment of psychotropic drug effects.

Recently published encouraging data concerning the value of anticardiolipin antibody assays in patients with HIV infection, both for prognostic purposes and to diagnose pneumocystosis, have prompted us to evaluate these assays in a series of 116 patients. We were unable to demonstrate any correlation between the levels of these antibodies and the clinical stage of the infection or the presence of thrombocytopenia. Varying levels of anticardiolipin antibodies were found in 29.3 percent of our patients. More frequent positivity and higher levels seemed to be associated only with the development of cerebral toxoplasmosis. The value and the possible physiopathological role of these antibodies in patients with HIV infection should be reconsidered.

Between 1 January 1980 and 31 July 1988, 62 patients with chronic lymphocytic leukaemia (CLL) or malignant non-Hodgkin's lymphoma (NHL) were splenectomized for splenomegaly and presumed hypersplenism. All patients except one had splenomegaly (mean(s.d.) weight 1585 (872) g, range 150–4300 g) and 34 had massive splenomegaly (> 1500 g). Forty-nine patients had platelet counts < 100 × 109/l and 16 patients had anaemia with haemoglobin levels < 10 g/dl. White cell counts were < 3×109/l in six NHL patients. Fifteen patients had bicytopenia, and three NHL patients had tricytopenia. The selected group of 62 patients underwent splenectomy largely because of failure to respond to medical therapy (39 patients) or inability to tolerate or start adequate chemotherapy because of very low blood counts (11 patients). There was one postoperative death, and a 29 per cent morbidity rate. The response rate was 89 per cent in the first month after splenectomy and 39 patients (63 per cent) had a continuing complete response with a median follow-up of 26 months (range 3–96 months). Twelve patients (10 with CLL) received no further therapy after splenectomy. Seven patients failed to respond and 15 relapsed after splenectomy. These 22 patients could be distinguished on the basis of: (1) lower average preoperative platelet counts (P < 0·007), postoperative platelet counts (P < 0·001), and postoperative rise in platelets (P < 0·004); (2) lower average spleen weight (P < 0·052); (3) preoperative chemotherapy (P < 0·044). However preoperative and postoperative platelet counts were the only two variables selected by stepwise regression analysis (P < 0·05 and P < 0·01, respectively). Bone marrow failure did not preclude complete response after splenectomy. Long-term survivors emerged from the group of patients with continuing complete response. Of the seven patients who failed to respond, five died with a median survival of 4 months, and of the 15 patients who relapsed after splenectomy, 13 died, with a median survival of 6 months after relapse and 18 months after splenectomy. Thus, splenectomy may be an effective palliation for both CLL and NHL patients with splenomegaly and hypersplenism.

Ninety-two elderly patients (ages 50-70) with "de novo" acute myeloid leukemia were given induction chemotherapy consisting of aclacinomycin-A (ACLA) (100 mg/m2/day x 3) and cytosine arabinoside (ARA-C) (100 mg/m2 day, continuous infusion, 7 days). Fifty-one patients (55%) achieved complete remission (CR), 8 patients exhibited drug resistance and 33 patients died during chemotherapy or aplasia. Three patients had severe cardiac toxicity. The only prognostic factor significantly affecting CR was the initial leukocyte count. After consolidation using ACLA (80 mg/m2/day x 2) and ARA-C (100 mg/m2/day x 5), 47 CR patients were randomly assigned to 2 different treatment arms: 23 patients (Group A) received intensive sequential chemotherapy consisting of 4 monthly courses of 8 different drugs while 24 other patients (Group B) were given ACLA and ARA-C at regular intervals, associated with continuous chemotherapy consisting of 6-mercaptopurine, methotrexate and androgens. The probability of disease-free survival at 2 years was significantly higher (33 +/- 22%) for Group B patients than for Group A (13 +/- 16%) (P less than 0.05). We conclude that continuous maintenance chemotherapy may be useful in increasing the number of long-term survivors, at least in the elderly who have not received very intense consolidation chemotherapy following CR.

The aim of this prospective study was to assess the frequency of gastroduodenal involvement during initial staging of extradigestive non Hodgkin's lymphoma. One hundred and one patients (64 men and 37 women; mean age 57.7; range 22-77 years) underwent an upper digestive endoscopy with multiple biopsies before any treatment. The origin of lymphoma was nodal in 76 cases and extranodal in 25 cases. Biopsy specimens were taken from all gross lesions; when the mucosa was considered as normal, biopsy specimens were obtained from the corpus, the antrum, and the duodenum. Endoscopy was normal in 37 patients; nevertheless in 4/37 patients, biopsies revealed lymphoma infiltration. In 64 patients, endoscopy showed macroscopic anomalies: biopsies were negative in 47 patients and showed lymphoma involvement in 17 patients. The involvement site was the corpus of the stomach (11 cases), the antrum (9 cases), and the duodenum (9 cases); lesions were multifocal in 10/21 patients. The association of several types of lesions was noticed in 5 patients. Lesions included volcano-like craters (8 cases), large folds of brain-like appearance (4 cases), tumors greater than 3 cm (3 cases), gastric ulcers (3 cases), erosive gastritis (3 cases), and erythematous duodenitis (2 cases). Overall, 21 of 101 patients (20.8 percent) had positive biopsies, 17 with endoscopic lesions and 4 without. Only 5/21 patients presented upper gastrointestinal symptoms. Staging of the lymphoma changed to Stage IV in 11 patients (4 stage I, 4 stage II and 3 stage III) (10.9 percent) after gastrointestinal involvement was documented. Gastroduodenal involvement is not related to initial site (nodal or extranodal), stage of extension or grade of malignancy of the lymphoma.

Summary. Membrane-bound monoester lipase (MEL) activity was measured by a radiochemical assay in intact red blood cells (RBC) from 77 patients with various blood disorders. MEL activity levels in their RBC were compared with levels in normal subjects. The increase in MEL activity was greatest in RBC of regenerative anaemias (N=8) and blastic crisis of chronic granulocytic leukaemias (7) whereas a lesser, although significant, increase was found in acute non-lymphoblastic leukaemias (14). MEL activity was markedly decreased in RBC of erythroleukaemias (N=5) and decreased to a lesser extent in acute lymphoblastic leukaemias (10) and in chronic myeloproliferative disorders other than chronic granulocytic leukaemias (8). MEL activity levels were roughly correlated with reticulocyte counts, but the relationship was not of predictive value in individual cases. Further exploration of the possible diagnostic or prognostic implications of these variations in enzyme levels seems warranted. A monoester lipase (MEL) activity has been recently identified and characterized as a membrane-bound enzyme in red blood cell (RBC) from rats (Arnaud et al, 1979) and human subjects (Boyer et al, 1981). No attempt has been made so far to search for abnormalities of this enzyme. We have therefore measured MEL activity in RBC of 77 patients with various blood disorders.

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.

SUMMARY Approximately three-quarters of epileptic patients seen in consultation by specialists can easily be assigned to one of the diagnostic categories of the International League against Epilepsy. When patient age is taken into account, such a distribution shows that partial epilepsy is more common than generalized epilepsy (62% versus 38%) in patients of all ages, especially in those over 15 years of age (78% versus 22%). In contrast, generalized epilepsy is more frequent in subjects under 15 than partial epilepsy (55% versus 45%). In this younger age group the rarity of partial epilepsy is primarily due to the infrequency of temporal lobe epilepsy (21% of cases, as opposed to 56% in subjects over 15), whereas both primary and secondary generalized epilepsy contribute to the frequency of generalized epilepsy in this group. RESUME II est aise de repartir les trois quarts environ des Epileptiques d'une consultation specialisee entre les differentes categories d'epilepsie qui ont ete adoptees par la Ligue Internationale contre 1'epilepsie. Compte tenu de 1'age des sujets, cette repartition fait apparaftre que les epilepsies partielles sont plus frequentes que les epilepsies generalisees (62% contre 38%) pour 1'ensemble de la population et, surtout, pour la partie de cette population comportant des sujets ages de plus de 15 ans (78% contre 22%). Chez les sujets ages de moins de 15 ans ce sont, au contraire, les Epilepsies generalises qui sont plus frequentes que les epilepsies partielles (55% contre 45%); chez ces sujets, la rarete des Epilepsies partielles tient surtout a la rarete des epilepsies du lobe temporal (21% des cas contre 56% chez les sujets ages de plus de 15 ans), tandis que la frequence des epilepsies gen6ralis6es concerne aussi bien les epilepsies generalisees primaires que les epilepsies general-isees secondaires. RESUMEN Aproximadamente las tres cuartas partes de los enfermos epilepticos vistos en consulta pueden, facilmente, incluirse en una de las categorfas diagnosticas de la Liga International contra la Epilepsia. Cuando se considera la edad del paciente se observa que la epilepsia partial es mas comun que la epilepsia generalizada (62% versus 38%) en los enfermos de todas las edades y especialmente en los que sobrepasan los 15 anos, de edad (78% versus 22%). En sujetos por debajo de los 15 anos, por el contrario, la epilepsia generalizada es mas frecuente que la partial (55% versus 45%). En este grupo de enfermos mas jovenes la rareza de epilepsia partial es fundamentalmente debida a la infrecuencia de epilepsia temporal (21% de los casos, en contraste con 56% en sujetos may ores de 15 anos) mientras que la epilepsia generalizada primaria y secundaria contribuye a la frecuencia de epilepsia generalizada en este grupo. ZUSAMMENFASSUNG Annahernd 3/4 der anfallskranken Patienten in der Sprechstunde von Spezialisten konnen leicht einer der diagnostischen Kategorien zugeordnet werden, die von der Internationalen Liga gegen epilepsie herausgegeben wurden. Unter Berucksichtigung des Alters der Patienten zeigt eine solche Verteilung, dass Partial-epilepsie haufiger ist als die generalisierte epilepsie (62% gegenuber 38%). Das trifft fur Patienten aller Altersgruppen zu, besonders solcheuiber 15 Jahren (78% gegenuber 22%). Im Gegensatz hierzu ziegt sich, dass bei Patienten unter 15 Jahren die generalisierte epilepsie haufiger als die Partialepilepsie auftritt (55% gegenuber 45%). In dieser jiingeren Altersgruppe ist die Seltenheit der Partialepilepsie in erster Linie auf die Seltenheit der Temporallappenepilepsie zu beziehen (21% der FaTle gegenuber 56% bei Patientenuiber 15 Jahren). Demgegenuber tragen sowohl die primar als auch die sekundar generalisierten Epilepsien zur Gesamthaufigkeit der generalisierten Epilepsien in dieser Altersgruppe bei.

The authors report 7 cases of nerve injury during venous puncture for blood sample withdrawal or intravenous injection of infusion. pain unrelieved by the usual analgesic drugs and sensorimotor deficiency are regular features which regress more or less rapidly and completely. Although less common than nerve injuries consecutive to intramuscular injections, these complications deserve to be described in view of their therapeutic and legal consequences.

On the basis of 88 patients examined with CAT, the writers consider the merits of this method of investigation in a study of endocranial complications in blood diseases. Interest in CAT is related in great part to its innoccuousness (making it possible to extend its application to often seriously ill patients) and the precision of the images which are obtained. This precision makes it possible to visualize lesions which at times can not be detected with other methods. A great part of present knowledge on endocranial complications in blood diseases is based on autopsy findings. With CAT, there is definitely the possibility of performing anatomical studies during the patient's lifetime, thus providing a better understanding of the characteristics and frequency of these complications. This is already made clear by this preliminary study, particularly in the area of acute leukemia and Hodgkin's disease.

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 its, 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.

A trial of maintenance chemotherapy of nonlymphoblastic acute leukemia led to a comparison of two groups of patients in complete remission. Group 1 (14 patients) received only monthly reinduction chemotherapy. Group 2 (17 patients) received identical chemotherapy together with weekly immunotherapy combining BCG and irradiated leukemic cells. While the duration of the first complete remission was unmodified, the overall survival time and, above all, survival after the first relapse were prolonged in group 2 chemoimmunotherapy. These results were all the more marked when a homogeneous group of patients having received the same induction chemotherapy were considered.

Between January 1, 1980, and July 31, 1985, 168 patients were treated at our institution for chronic lymphocytic leukemia (CLL). Splenectomy was performed on 32 (23 men and 9 women). The mean age of these surgical patients was 59.4 years (range, 43-75). Twenty-four patients had received chemotherapy and/or corticotherapy prior to surgery and 1 of these 24 also underwent splenic radiotherapy. The interval between diagnosis and splenectomy ranged from 1 to 108 months (mean, 33.6 months +/- 28.6). Before splenectomy, 9 patients had platelet counts greater than 100,000/mm3 (3 with normal counts), 18 had counts between 50,000 and 100,000/mm3, and 5 had less than 50,000/mm3. Seven patients had anemia with hemoglobin levels less than 10 g/dl (3 associated with thrombocytopenia); in the other 25 patients, hemoglobin levels were between 10 and 11 g/dl in 12 (9 associated with thrombocytopenia) and greater than 11 g/dl in 13. All patients but one had splenomegaly (mean spleen weight 1780 g +/- 938.7 range, 160-4300 g). One postoperative death was recorded. Postoperative morbidity occurred in 7 of 31 cases; severe infection was never observed. The average duration of hospitalization was 15 +/- 4.7 days (range, 9-28). Twenty-two of the 23 patients with thrombocytopenia less than 100,000/mm3 had complete remission after splenectomy. One had a partial remission that was later completed by means of drug therapy. Two patients relapsed within 6 months and one after 32 months. All but two cases of anemia responded to splenectomy; one patient relapsed. In 15 of 31 surviving patients, further treatment was required within a few weeks or months after surgery. Five patients died at 8, 17, 22, 26, and 35 months, respectively. The other 26 patients are alive and well with a mean follow-up of 34 months (range, 11-71 months). Splenectomy may be an effective treatment for CLL patients with splenomegaly, thrombocytopenia, and/or anemia.

Vascular accidents of the cerebellum are traditionally considered to be very difficult to diagnose, and as spontaneously following a fatal course without neurosurgical decompressive intervention. Such views are undoubtedly due to the fact that usually only the most serious vascular accidents of the cerebellum are diagnosed prior to surgical intervention or autopsy. Following 12 observations (2 hematomas and 5 infarcts) using computerized tomography, which facilitates the diagnosis of less severe forms, the authors suggest that some changes are necessary in the classic view of these disorders. From a clinical point of view, they point out the consistency of a cerebellar syndrome in their series and the subtlety of disturbances of consciousness. They also stress the possibility, verified in 10 these 12 cases, of a favourable outcome with medical treatment alone. The CT scan not only facilitates the diagnosis of vascular accidents of cerebellum, but can distinguish between hematomas and infarcts (the incidence of the latter has certainly been underestimated until now). Furthermore, the CT scan allows direct clinico-anatomical surveillance of the lesion, and equally importantly of its possible obstructing effects on the ventricular system.

The authors described 10 cases of scapulo-peroneal amyotrophy of spinal origin of the Stark-Kaeser type. The main characteristics are a frequently dominant autosomal heredity, onset in the muscles of the legs, scapulp-peroneal weakness and amyotrophy with extension sometimes to the bulbar muscles, areflexia without sensory disturbances, an electromyogram of the neurogenic type with a normal conduction rate, neurogenic histological aspects with normal peripheral nerve. It does not usually result in severe disability. This syndrome is related to the scapulo-peroneal or facio-scapulo-peroneal myopathies and the scapulo-peroneal neuropathies. The problem of the boundaries between these disorders and their association does not alter the fact that the concept of spinal amyotrophy of the Stark-Kaeser type should be kept as a reference group.

Clinical and electrophysiological data in two cases of amyotrophy of parietal origin (Silverstein's syndrome) are reported. Some parietal lesions provoke amyotrophy, usually with a sensory deficit but sometimes isolated, of rapid onset and affecting mainly the upper limb extremity. In the two cases reported, a spontaneous electromyographic activity was present with fibrillation and positive sharp waves, an unusual finding in central nervous system diseases. Despite its lack of specificity an activity of this type is observed mainly with peripheral axonal lesions. The physiopathology of Silverstein's syndrome is entirely hypothetical. The decreased transsynaptic trophic influence of central on peripheral cells could result in a reduced trophic effect of peripheral nerves on muscles, and the spontaneous activity could be related to muscle cell membrane instability.

Two cases of CLL terminating in Richter's syndrome and in blastic crisis raise the problem of the physiopathological signification of these unusual complications. Richter's syndrome is described as a "histiocytic" lymphoma or a Hodgkin's disease supervening on a CLL. It does not seem that these two pictures have actually to be distinguished since clinical and haematological aspects are not different, and histopathologic studies show always atypical aspects with difficulty of an accurate diagnosis. Cases of blastic crisis of CLL are very uncommon. They correspond to the transformation of a CLL in acute lymphoblastic leukaemia. These two terminal complications of CLL suggest they may be interpreted as two pictures of the transformation of the same initial B cellular clone. This hypothesis, highly probable for the Richter's syndrome, has been actually demonstrated for the acute transformation of CLL.

Vindesine is a semisynthetic vinca alkaloïd. The short plasma half-life suggested that continuous infusion can improve the therapeutic results by maintaining a constant plasma level. Twenty one patients (acute lymphoblastic leukemia = 5 cases; blastic crisis of chronic myelocytic leukemia = 6 cases; lymphomas 10 cases) received 5-day Vindesine infusion (0,7 mg/m2/d). All these patients had previously several treatments and were resistant to usual chemotherapies including other vinca alkaloids (76% of cases). Eighteen patients were evaluable for response: 13 (72%) had partial responses or minor regressions. Toxicity was not important. Continuous 5-day infusion of Vindesine might be used widely in patients with blood diseases.

Dose-effect relationships of high-dose melphalan were evaluated in 37 patients with measurable relapsed or refractory acute leukemias. Thirteen patients (Group 1) received 70-100 mg/m2 of melphalan without marrow rescue and 24 patients (Group 2) received 140-180 mg/m2 of melphalan followed by marrow transplantation. Patients in both groups were comparable with respect to age, sex, diagnosis, and status of the leukemia. The complete remission rate was 23% in Group 1 versus 75% in Group 2 (P less than 0.01). Hematological recovery of remission patients was not statistically different in either group. Nonhematological toxicity was comparable in the two dose ranges examined. These results demonstrate the existence of a dose-response effect of high-dose melphalan regimens in relapsed acute leukemias, without marked increases in nonhematological toxicity with these doses.

Seven patients with cancer presented a sensory peripheral neuropathy induced by cisplatinum. This drug was used alone in 1 case and, in 6 other cases it was associated with drugs without any toxicity for the peripheral nervous system. Every patient had an electromyogram and motor and sensory nerve conduction studies. A sural nerve biopsy was performed in 5 cases for light and electron microscopic studies as well as for teasing and quantitative studies. Electromyograms and motor nerve conductions were normal. Sensory nerve conductions were slowed with very low amplitude sensory action potentials. Such results suggested axonal changes. Nerve biopsies showed typical axonopathic changes with secondary demyelination. Morphometric studies confirmed a loss of myelinated fibers affecting the large fibers in all cases, according to the slowed sensory nerve conductions. This study confirmed that the cisplatinum-induced neuropathy is a new form of toxic distal axonal neuropathy. The hypothesis of a primary demyelination of peripheral nerves, which has been proposed, could not be retained.

Following post-operative occlusion of the right internal carotid artery, a 65-year old man developed abnormal left involuntary movements which appeared in upright position or at exercise and disappeared in sitting or supine positions. The clinical and electroencephalographic features of these movements could have been epileptic. However, this case together with data from the literature suggest that transient ischemic episodes in the carotid territory may be expressed as involuntary movements occurring in certain positions, betraying a severe and abrupt fall in cerebral blood flow.

The coexistence of a myeloma and Paget's disease in the same patient is only rarely reported in the published literature. The frequency of such an association was studied when reviewing 3 cases observed by the authors: an IgG myeloma discovered following a spasmodic paraplegia in a patient with Paget's disease; a patient who was found to have a myeloma, a polynuclear neutrophil leukemia, and Paget's disease; and an IgG myeloma in a patient with probable localized Paget's disease. The incidence of the association myeloma-Paget's disease is much lower than that of sarcomatous degeneration. It should be determined precisely, however, as our future conception of the reciprocal relationship between the two diseases is greatly dependent on this information. Furthermore, the very few cases reported, and their lack of homogenicity, are the reasons why most authors feel that the association of myeloma and Paget's disease as pure coincidence. Many new cases, however, have been recently reported. The problem of the significance of the association of Paget's disease and macroglobulinemia or other monoclonal gammapathies has still to be solved.

One hundred patients suffering from haematological diseases were explored by whole body scanning. The diagnosis was a lymphoma in the majority of cases (77). Analytical study of the images obtained was made on the basis of the various sections made systematically in the protocol of the study. Scanning proved to be of particular value in the thorax in the study of pleural and juxta-pleural lesions. In the abdomen, study of the liver, spleen and retro-peritoneal space benefited from the use of this means of investigation. Supra-mesocolic sections were felt to be of the greatest value, making it possible to demonstrate lymphadenopathies not revealed by lymphography. Scanning, perfectly harmless, provides a valuable contribution in determining the extent of lymphoma spread and facilitates the therapeutic surveillance of haematological disorders.

One hundred two patients submitted to intensive chemotherapy were included in a randomized study with cross over comparing for the second course two anti-emetics: tetracosactide (D1: 3 mg, D2 and D3: 2 mg/d) and methylprednisolone (D1: 240 mg, D2 and D3: 160 mg/d). Most patients presented with malignant lymphoma. All patients experienced nausea and emesis during first course of chemotherapy. Results were similar in both groups, respectively for tetracosactide and methylprednisolone: no nausea 37 versus 40%, less than 3 emesis 69% versus 73%. Secondary effects were observed in 5 and 8% of cases. Tetracosactide with a schedule of 7 mg for 3 days gives the same results than methylprednisolone 560 mg for 3 days in chemotherapy induced nausea and emesis prevention.

The authors review the literautre and then proceed to analyse a series of 16 cases of Gougerot Sjögren Syndrome. The electromyogram (EMG) was abnormal on only 2 occasions. Histologically inflammatory aspects could be seen on 4 occasions with perifascicular atrophy in one case. Atrophy of II fibres was present in 7 cases together with signs of disorganization of the mitochondrial reticulum and images of mitochondrial aggregates. Under the electron microscope minor anomalies were particularly visiole and the internal structure was normal as often as not. Overall it is possible to distinguish forms of a subacute polymyositis type (4 cases), and a pauci-inflammatory myalgic type proximal to the scappular predominance (4 cases), in which the mitochondrial anomalies are commonest. Another "infraclinical" group shows no other frankly pathological changes. Finally in 3 cases the "dry" syndrome is strictly free of any muscular attack.

A trial of immunotherapy in the maintenance treatment of non-lymphoblastic acute leukaemias led to a comparison of 2 groups of patients in complete remission. Group C (14 patients) received only monthly reinduction chemotherapy. Group C + I (17 patients) received identical chemotherapy together with weekly immunotherapy combining BCG and irradiated leukaemic cells. Whilst the duration of the first remission was unmodified, the overall survival and above all survival after the first recurrence were prolonged in group C + I. These results were all the more marked when a homogeneous patients having received the same induction chemotherapy is considered.

Cyclic cytidine 3':5'-monophosphate (cyclic CMP), cyclic guanosine 3':5'-monophosphate (cyclic GMP), and cyclic adenosine 3':5'-monophosphate (cyclic AMP) contents of leukocytes and urines of leukemic patients have been investigated. We have studied four types of leukemia: acute myeloblastic leukemia; chronic myelocytic leukemia; acute lymphoblastic leukemia; and chronic lymphocytic leukemia. As controls, the cyclic nucleotide content of leukocytes and urines of healthy volunteers and patients with solid tumors selected for their normal hemogram has been determined. It has also been measured in phytohemagglutinin-stimulated lymphocytes. Our data show that: (a) the concentration of cyclic CMP is always lower than that of cyclic GMP or cyclic AMP; (b) in urines, the concentrations of the three nucleotides are higher in patients than in healthy volunteers, the greatest differences being observed between the cyclic CMP concentrations of acute leukemia patients and controls; and (c) in white blood cells, cyclic AMP concentration is lower in leukemic than in normal cells. The cyclic GMP concentration is the same everywhere except in monoblastic cells and leukocytes from solid tumor patients. High cyclic CMP levels are associated only with acute leukemia, whether myeloblastic, monoblastic, or lymphoblastic, a fact which suggests that cyclic CMP could be a biochemical marker of hematopoietic stem cell malignancy.

Amyotrophy of cerebral origin was analyzed in 12 hemiplegics by means of comparative histological, histoenzymological, histographic and biochemical analysis of biopsies carried out in symmetrical zones. In the 6 cases in which atrophy predominated on the paralyzed side, it occurred early, the deficiency affecting the upper limb in particular and the sensory disturbance being irregular. Attempted numeration of the motor units remaining, according to the "incremential" method of stimulation, suggests a numerical reduction on the hemipelgic side. Despite reservations and general criticisms of this method, its comparative value, although approximate, must be acknowledged. However, although amyotrophy presumes a transsynaptic change in trophic function to have taken place in the peripheral neurone, neuronal depopulation--if one accepts it--cannot be other than functional.