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In democratic countries, cognitively impaired persons are a substantial and growing group of citizens. Most of them are citizens with dementia. In dementia, cognitive impairment induces a loss of some capacities, resulting in vulnerability and increased need for assistance. Voting by cognitively impaired persons raises any questions about the integrity of the electoral process, the risk of fraud and the respect of their citizenship. In France, the law is not definite about the voting of cognitively impaired persons. An objective assessment for voting capacity may be useful both for professionals in charge of voting organisation and for guardianship judge in order to help him in his decision to remove or keep the voting right of persons placed under guardianship. Assessing the reality of voting by cognitively impaired citizens is necessary to advance respect for their right to vote.

Objective: To assess the effectiveness of providing doctors with written thromboprophylaxis prescription aids based on current recommendations., Design: A prospective trial of specific anticoagulant prescription forms: a 1-day survey before and after the intervention in each centre., Setting: 30 Internal Medicine departments of Assistance Publique-Hôpitaux de Paris., Subjects: All inpatients were included, except those who were either admitted or discharged on the day of the survey, and those receiving curative anticoagulant treatment., Interventions: The study included three parts: (i) a 1-day baseline survey; (ii) over the following 3-month period, departments were randomized into two groups: all practitioners in wards allocated to the intervention group were required to systematically use specific anticoagulant prescription forms, whilst doctors in the control group continued prescribing according to their usual practices and (iii) a 1-day postintervention survey., Main Outcome Measure: The proportion of prescriptions in accordance with the recommendations., Results: 1,469 patients were included. The intervention produced a significant reduction in the frequency of over-prescriptions, from 25% to 14% of the patients who did not meet the guideline criteria (adjusted OR: 0.3; 95% CI: 0.1-0.8). Using specific forms did not improve under-prescription of anticoagulants. A little over 60% of the patients who met guideline criteria for thromboprophylaxis were prescribed anticoagulants in both intervention and control wards, either at baseline or after intervention., Conclusions: Multitargeted interventions using a variety of means and strategies should still be considered to improve prescriptions that may have a significant impact on health expenses and, most importantly, on patients outcomes.

Introduction: Usually, temporal arteritis progresses as a chronic disease., Case Report: The authors report the observation of a 74-year-old woman who presented with two acute flares of temporal arteritis with headache, fever and inflammatory syndrome, which have spontaneously resolved., Discussion: The observations of auto-inflammatory attacks of arteritis disease are rare, but maybe underestimated. The pathophysiology remains unclear.

Nocardia cyriacigeorgica is a recently characterized species within the genus of Nocardia. We report a brain abscess, following a primary pulmonary colonization, due to this species in a human immunodeficiency virus-infected patient. This case confirms that isolation of Nocardia in sputum is associated with a high risk of disseminated infection in immunocompromised patients.

During the first attacks of familial Mediterranean fever, each of the disease symptoms can suggest a series of disorders. When the disease is older, the recurrence of symptoms may simulate some systemic diseases, but mainly suggests familial Mediterranean fever, one of a group of hereditary autoinflammatory diseases. Before the gene for familial Mediterranean fever was identified, various sets of criteria were used for diagnosis. The presence of MEFV mutations confirms the diagnosis, but the clinical criteria still determine who should undergo this genetic testing. The genotype-phenotype correlations add a prognostic dimension to the mutations identified. Genotyping can also lead to the diagnosis of the other autoinflammatory diseases, which constitute the basis of the differential diagnosis of familial Mediterranean fever. The hyperimmunoglobulinemia D syndrome (HIDS) is very similar to familial Mediterranean fever in its recessive transmission and abdominal and articular symptoms. It can be distinguished by the European origin of the patients, the presence of cervical lymph nodes and the increased IgD levels. Of the diseases with dominant transmission, the TNF receptor-associated periodic syndromes (TRAPS) are suggested by periorbital edema and migrating inflammatory cellulitis. Muckle and Wells syndrome is revealed by episodes of fever with urticaria and arthralgia, complicated by deafness and amyloidosis. Mutations in the same gene are responsible for two disorders, both appearing in childhood: familial cold urticaria syndrome (FCUS) and chronic infantile neurocutaneous articular syndrome (CINC). The pathogenesis of familial Mediterranean fever is still unclear. Pyrin/marenostrin, the protein produced by the MEFV gene, appears to hae a physiological antiinflammatory effect that inhibits proinflammatory cytokines. Mutation of the gene may eliminate this feedback mechanism and expose the patient to flares from any inflammatory stimulus, even minimal. Amyloid is produced by the serum amyloid A protein (SAA), and its occurrence is influenced by the type of MEFV mutation, but also the genotype of the gene producing SAA.

The progression of familial Mediterranean fever is marked by the recurrence, at varying intervals, of acute flares that regress spontaneously. Prognosis, which depends on the occurrence of amyloidosis, has been transformed by colchicine treatment. Incidence of amyloidosis is higher in certain ethnic groups (Jews from North Africa, Turks) and depends on by the specific MEFV mutation. Amyloid is composed of clusters of protein strands identical to the AA protein of secondary amyloidosis and infiltrates the walls of all arterioles except those of the central nervous system. The earliest and most consistent localization is in the kidney, where it develops over several years and in 4 stages--preclinical (latency), proteinuric, nephrotic and uremic--before concluding in end-state renal failure. Before the advent of colchicine, dialysis and transplantation, only renal amyloidosis caused clinical manifestations and lethal complications; any amyloidosis at any other sites remained latent. Prolonged survival with hemodialysis and kidney transplantation now leaves time for manifestation of these other localizations, such as infiltration into the intestines causing malabsorption, or potentially lethal cardiac lesions. Treatment of familial Mediterranean fever is based on the continuous administration of colchicine, which at the average dose of 1 to 2 mg per day can prevent flares or at least reduce their frequency or intensity. Systematic use of colchicine also prevents the onset of amyloidosis, even in the rare cases where it cannot prevent flares. These data fully justify the systematic use of colchicine for continuous prophylactic treatment from diagnosis and even after kidney transplantation, to prevent recurrence of the grafted kidney or extension to other organs. The curative efficacy of colchicine on flares is debatable, although several studies report positive results against progression of early amyloidosis.

Familial Mediterranean fever is the best known of the recurrent hereditary autoinflammatory diseases. It predominantly affects subjects of Mediterranean origin, Sephardic Jews in particular. Its gene, MEFV, is located on chromosome 16 and has autosomal recessive transmission, with incomplete penetration. It codes for a protein called pyrin or marenostrin, which is probably involved in the inflammatory process. In most cases, the first episodes appear before the age of 20 years and very rarely after the age of 40. Episodes usually last a few days, although they may extent over several weeks when localized in joints. Fever, occasionally pseudo-malaria, may accompany various symptoms, the most frequent of which are abdominal, articular, pleural or cutaneous. The abdomen is the classic site of this disease, and acute abdominal flares masquerade as abdominal emergencies. Musculoskeletal involvement is revealed by episodes of inflammation of the joints (more often mono- than oligoarthritis) and muscle pain. The flares are usually brief and totally reversible. Flares of thoracic pain corresponding to pleural inflammation and erysipelas-like skin eruptions have been observed. Acute symptoms disappear between flares, but hepatic splenomegaly, swollen lymph nodes or abnormal fundus of the eye may persist. Laboratory findings are typical of nonspecific inflammation, accompanied by moderate hyperleukocytosis during the flares.

Introduction: Simplification of combined antiretroviral therapy in HIV-infected patients is possible, but virological success can be compromised by the development or emergence of resistant viruses., Case: Worsening renal functioning in a patient under successful combination antiretroviral therapy resulted led to the replacement of indinavir by abacavir. Eight weeks later, his viral load rose and he developed a mutant virus resistant to all the nucleoside analogs., Discussion: Our case report illustrates the danger of streamlining combined antiretroviral therapy composed only of nucleoside analogs in patients already successfully treated with nucleoside analogs, by exposing them to the risk of the emergence of a mutant virus.

Calcinosis cutis is common in several connective tissue diseases such as dermatomyositis, scleroderma or lupus erythematous. In dermatomyositis, it is more likely to concern children than adults but it is not exceptional in adults. Several treatments have been used empirically with inconsistent success. We report a case of adult cutis calcinosis associated with dermatomyositis which responded dramatically to treatment with diltiazem.

DIAGNOSTIC DOUBTS: Despite an increased awareness of the clinical features of the disease, adult onset Still's disease (AOSD) remains a diagnosis of exclusion. Many diagnostic criteria have been published, and the most popular are those proposed by Yamaguchi, even though they do not consider the presence of hyperferritinemia or the decrease of it glycosylated fraction, and they include exclusion criteria that are difficult to satisfy. UNPREDICTABLE PROGRESSION: Three evolutive forms have been described: monocyclic, intermittent with articular and/or systemic flares, chronic, usually in the form of chronic polyarthritis. Vital prognosis is sometimes compromised by severe systemic manifestations or the occurrence of amyloidosis, whilst destructive polyarthritis, particularly common in the chronic form, may compromise the function. The aetiology of AOSD remains unknown. THERAPEUTIC UNCERTAINTIES: Treatment is largely empirical in the absence of randomized clinical trials because of the rarity of the disease. Nonsteroidal anti-inflammatory drugs could be used in moderately severe forms of the disease, but they rare rarely sufficient. Around 80% of the patients require corticosteroids. Among the disease modifying drugs, methotrexate still remains the most effective. The role of the new anti-TNF molecules remains to be specified.

A TRIAD OF FEATURES: Adult onset Still's disease (ASD) is an uncommon disorder usually associating high spiking fever, evanescent skin rash constituted of small salmon pink macules, and arthritis. NUMEROUS SYSTEMIC MANIFESTATIONS: A sore throat is common and often misleading. More than 60% of the patients develop mobile and indolent lymph nodes, usually in the cervical area. Liver involvement is common and usually limited to a mild or moderate cytolysis. However, several observations of severe hepatitis have been reported justifying strict monitoring of the liver biology in these patients. Amongst the other numerous systemic manifestations that have been reported, pericarditis is common and sometimes responsible for tamponade, the pulmonary involvement may lead to an acute respiratory distress, and the rare neurological manifestations include aseptic meningitis or cranial nerve palsy. FROM A BIOLOGICAL POINT OF VIEW: The sedimentation rate is consistently elevated and there is usually a marked elevation in the polymorphonuclears. The bacteriological survey is negative as are the immunological tests. An increase in the serum level of IL-18 might be both diagnostic and prognostic. It is the increase of the serum level of ferritin and the marked decrease in its glycosylated fraction below 20% that seem to be of more potent diagnostic value.

Objective: To report our experience on a multicentre series of 32 patients with either typical or atypical Cogan's syndrome, to combine our results with a detailed review of the literature, and to compare the clinical manifestations of typical and atypical Cogan's syndrome., Methods: Patients were identified from a survey conducted with physicians affiliated to the French National Society for Internal Medicine, and were classified into typical or atypical Cogan's syndrome according to the Haynes criteria. Clinical data were collected in a standardized manner. A comprehensive literature review using the Medline database and the reference lists of identified articles was performed., Results: Seventeen patients had typical Cogan's syndrome and 15 had atypical Cogan's syndrome. Apart from non-syphilitic interstitial keratitis, the ocular manifestations of patients with atypical Cogan's syndrome were mainly uveitis and episcleritis. All but one patient presented with Ménière-like syndrome, and at the end of follow-up 11 were deaf and 19 additional patients had developed a significant decrease in auditory acuity. Twenty-five patients (78%) developed systemic manifestations, including aortitis in four. Comparison of typical and atypical Cogan's syndrome showed that some systemic manifestations were more common in atypical Cogan's syndrome, but these differences may be explained by reporting bias in the literature., Conclusion: Differences regarding the associated systemic manifestations of typical and atypical Cogan's syndrome may reflect reporting bias in the literature. However, the diversity of the ocular and audiovestibular manifestations and the acceptable lengthy delay between the two types of involvement in atypical Cogan's syndrome should make one cautious before accepting this diagnosis as the diagnosis may mimic various other systemic diseases.

There is wide evidence for a decreased risk of rheumatoid arthritis in patients with schizophrenia. Nevertheless, very few studies have looked at the risk of schizophrenia in a group of patients with rheumatoid arthritis. We prospectively investigated, with the SCL-90R, 220 consecutive outpatients with rheumatoid arthritis and 196 consecutive outpatients with various medical conditions, half of them suffering from psoriatic arthritis (a medical condition close to rheumatoid arthritis). The SCL-90R appears to be a valuable tool to distinguish patients with schizophrenia from the outpatients of our sample, the former having more "paranoid ideation" (p = 0.004) and more "psychoticism" (p < 0.001) than the latter. The "paranoid ideation" dimension was significantly lower (25% decrease) in the sample of patients with rheumatoid arthritis compared to the combined control group (p = 0.005), ratings under the median value being more frequent in the former group (p = 0.025). Confounding factors might not explain this difference according to the regression logistic analysis performed. As patients with rheumatoid arthritis have a lower score of paranoid ideation than controls in our sample, even after controlling for age, gender and severity of the disease, these data represent further evidence for a decreased risk of schizophrenia in individuals with rheumatoid arthritis.

Introduction: Ankylosing spondylitis is a chronic inflammatory disorder that is rarely associated with severe systemic manifestations., Exegesis: We report the case of a 29-year-old man presenting with an atypical ankylosing spondylitis. The patient presented with a high spiking fever, a marked weight loss, and an unusual increase of acute phase response markers. Such features are rarely associated with ankylosing spondylitis and the review of the few similar reported cases in the literature showed that this presentation is usually associated with a severe disease course, and an asymmetrical joint involvement that is unresponsive to nonsteroidal anti-inflammatory agents., Conclusion: Clinicians should be aware of this unusual presentation of ankylosing spondylitis, which should be included in the list of aetiologies of fever of unknown origin, to avoid useless diagnostic procedures. The severity of the disease course may lead to the consideration of a more aggressive treatment strategy.

Background: Klüver-Bucy syndrome (KBS) is an infrequent condition that includes, in its complete form, often massive amnesia, psychic blindness (so-called visual agnosia), oral tendencies, hypermetamorphosis, changes in emotional behavior and increased sexual activity. Anatomic lesions in KBS involve the temporal lobe cortex bilaterally, and the amygdala as well as temporal white matter., Case Report: A 17-year-old patient treated for herpetic meningoencephalitis developed neuropsychological signs leading to the diagnosis of Klüver-Bucy syndrome., Discussion: KBS can occur in various clinical conditions but herpetic encephalitis is the most frequent cause. KBS may be transient or partially regressive, but severe amnesia that may progress to Korsakoff syndrome is often persistent after herpetic encephalitis. Neuroleptics or carbamazepine can be used for symptomatic treatment.

Background: Facial diplegia is a rare event, most commonly of unknown origin. We report the case of a woman who presented bilateral Bell's palsy a few days after a normal delivery., Case Report: Five days after the delivery of gemellary pregnancy, a 34-year old woman developed complete bilateral facial palsy. No treatment was initially prescribed. She was first seen in our department two weeks after the onset of her illness. The diagnostic work-up was negative and we considered that our patient had bilateral Bell's palsy. Treatment with methylprednisolone and intravenous acyclovir, initiated since admission, have had very limited effect., Discussion: As has already been shown for facial palsy, idiopathic facial diplegia, although exceptional, seems to be more frequent during the last trimester of pregnancy and in the early puerperium. Seven cases have been reported in the literature over the last 30 years. We discuss here the pathophysiology.

Objective: To describe our experience with human immunodeficiency virus (HIV) infected patients receiving protease inhibitor therapy who presented with adhesive capsulitis of the shoulder., Methods: Between July 1996 and December 1999, 8 HIV-infected patients (7 male) treated with protease inhibitors who presented with adhesive capsulitis of the shoulder were retrospectively identified. Diagnosis of adhesive capsulitis relied on clinical features including shoulder pain and both active and passive restricted range of motion (ROM). All available clinical and radiographic data were reviewed., Results: Onset of symptoms was insidious, and at presentation, patients complained of shoulder pain, which was bilateral in 4 of the 8 cases. Physical examination showed global restriction of active and passive ROM of the glenohumeral joint. The mean delay between initiation of HIV protease inhibitors and onset of shoulder pain was 14 months (range 2 to 36). The protease inhibitor therapy always included indinavir. No underlying condition associated with secondary adhesive capsulitis of the shoulder, including shoulder trauma, diabetes mellitus, thyroid disease, pulmonary or cardiac diseases could be identified. In all 8 patients, despite continuation of therapy with indinavir, both shoulder pain and restricted ROM completely resolved, after a mean disease course of 7.4 months., Conclusion: Adhesive capsulitis of shoulder seems to be a new adverse event of HIV protease inhibitor therapy. In all reported cases, patients were treated with indinavir. Further observations will be necessary to confirm adhesive capsulitis as a side effect.

Objectives: Student attendance to lectures in French medical schools is often poor. We surveyed undergraduate medical students in our medical school, repeating a similar survey conducted ten years earlier. The results are presented with the conclusions of the faculty seminar that followed this survey., Methods: A closed item questionnaire was distributed in June, 1998, through the hospital wards where the students were posted. After two reminders, the final response rate was 71% (247/348)., Results: Overall, 71% of the students declared that they never, or only occasionally, attended lectures in the medical school. Reasons included lack of time (75%), the curriculum diverging from the program of the selective examination that gives access to graduate specialization programs (59%), or insufficient practical clinical content (36%); 46% believed that this teaching prepared them to practice family medicine (11% some specially), and 92% that the way it was organized was not compatible with preparing for the selective examination. On the other hand, 75% of the students in the final two years of the curriculum declared that attending regularly special preparation seminars for the selective examination, to succeed at this test (91%), but also to prepare for family practice (25%). Respectively, 75%, 68% and 66% declared that undergraduate medical courses should, ideally, prepare them for the selective examination, but also for the practice of family medicine, and for graduate medical education., Conclusions: These results echoed the difficulties of the faculty of the medical school to reconcile preparing students both for their future medical practice and for the selective examination. Two working groups were asked to identify independently appropriate educational objectives according to each perspective: their conclusions appeared to be quite compatible. Based on these conclusions, institutional objectives were ratified to guide the educational policy of our medical school, including the following: to reinforce the consistency and progressiveness of the curriculum; to promote active and autonomous student learning approaches; to develop faculty teaching skills, curriculum evaluation and recognition of teaching activities.

Background: Livedoid vasculopathy is an uncommon condition that affects young patients. It must be clinically distinguished from cutaneous vasculitis., Case Report: A 27-year-old man presented pain and skin eruptions involving the lower limbs, hands and trunk. The skin eruption predominated on the feet with purpuric lesions producing a livedoid pattern, ulcerations on both ankles and white atrophic scars on the dorsum of the feet. Skin biopsy showed dermic vessel thrombosis without leukocytoclasia. These features favored livedoid vasculopathy rather than vasculitis. Anticardiolipin antibodies were positive. The patient was started on antithrombotic therapy. The skin ulcerations regressed and no recurrence has been observed at 7-months follow-up., Discussion: Livedoid vasculopathy is characterized by painful purpuric lesions that generally occur on the lower limbs, and frequently ulcerate and heal leaving porcelain white atrophic scars (atrophic blanche). The histology evidences a thrombotic process. Livedoid vasculopathy is normally described as occurring as a sole entity, however thera have been reports of an association with anticardiolipin antibodies. This suggest a possible link with antiphospholipid syndrome.