De « l’inéducable » aux « handicaps rares ». Évolution et émergence des catégories politiques en lien avec la structuration du secteur médico-social français

In France, a notion is currently the focus of a number of political debates, that of “rare disabilities”, a notion that does not seem to be used, or to a limited extent, in other countries. The aim of this article is to return to the history of this notion in France: when and why did it appear? What issue and what population does this category designate? To set the issues related to “rare disabilities” in the context of the French disability policy, the first section of the article traces the development of the medico-social sector at the end of the 1940s, on the basis of the notion of educability, superseding that of incurability on one hand, and the categorization of the populations of disabled persons in terms of impairments on the other hand. The second part shows how in this context, the issue of children with several impairments emerges and how, under the impetus of parents and health professionals, distinctions are formed within the category of multiple disabilities between “multi-handicap”, “pluri-handicap” and “polyhandicap”. The third section traces the appearance of the term “rare disabilities” in the context of a political decentralization in 1986, the construction of a new political and administrative category and the fluctuations of its definition, which currently falls short of the challenges raised by this complex form of disability. The fourth section shows how this new category became from 1998 on, a performative and pragmatic category leading to the creation of specific and original modes of care that challenge the traditional institutional approach of disability in a context of reform of the French disability policy. The article is based on an exploratory research that has consisted in an analysis of grey literature (legal texts, associations and working groups reports) concerning the notion of “rare disabilities”.     

Étude comparative de l’efficacité et de la tolérance de l’étidronate et de l’alendronate au cours de l’ostéoporose postménopausique. Influence de l’adjonction d’un traitement hormonal substitutif

Objectives. To compare the efficacy and safety of etidronate and alendronate in patients with postmenopausal osteoporosis and to assess the efficacy of either bisphosphonate in combination with hormone replacement therapy (HRT). Patients and methods. In this pragmatic study, the main efficacy criterion was the mean annual change in bone mineral density (BMD). Patients who had a past or current history of etidronate or alendronate treatment for postmenopausal osteoporosis with at least 18 months follow-up and an evaluation in 1999 were eligible. Recruitment was in an outpatient clinic with a special focus on metabolic bone diseases. Osteoporosis was defined as at least one low-energy fracture or as a lumbar spine or femoral neck BMD decrease to at least 2.5 SD below the mean in young women. HRT was not an exclusion criterion provided treatment duration was longer than one year. Etidronate was given cyclically (14-day courses in a dosage of 400 mg/d separated by 76-day intervals with calcium and vitamin D supplementation) and alendronate was given daily in a dosage of 10 mg/day. Results. Of the 99 patients who met our inclusion criteria, 53 received etidronate (including 23 on HRT) and 46 alendronate (18 on HRT). Repeat BMD measurements were obtained in 88 patients, including 11 who stopped their bisphosphonate therapy within the first year of use because of adverse events. Lumbar spine BMD (mean ±SD) increased significantly both in the etidronate group (+2.1%±0.7%/year) and in the alendronate group (+5.3%±0.9%/year). The increase was significantly greater with alendronate (P<0.01). The lumbar spine BMD increase was largest in the patients on alendronate and HRT (+6.5%±1.4%/year) and was smallest (and nonsignificant) in the patients on etidronate without HRT (+1.2%±0.8%). Femoral neck BMD showed no significant changes in any group. In the intention-to-treat analysis, fractures occurred in 12 etidronate patients (22.6%) and six (13.0%) alendronate patients (nonsignificant). Adverse events requiring bisphosphonate discontinuation before the scheduled date of the follow-up BMD measurement occurred in one patient (1.9%) in the etidronate group (generalized osteomalacia) and in ten patients (21.7%) in the alendronate group (upper or lower gastrointestinal tract symptoms in six and four patients, respectively; P<0.01). Conclusion. Both etidronate and alendronate significantly increased lumbar BMD, but the effect was significantly more marked with alendronate. Conversely, adverse effects, most notably gastrointestinal symptoms, were more common with alendronate, so that premature treatment discontinuation because of adverse events were more common in the alendronate group. Both differences should be taken into account when selecting the best drug for a patient with postmenopausal osteoporosis.     

Étude rétrospective d’une série de 623 tumeurs de la main et du poignet chez l’adulte

ObjectivesEpidemiologic data concerning hand and wrist tumors are still few and sometimes conflicting. In our department, tumor surgeons and hand surgeons manage together those tumors and they have a specific recruitment. This collaboration permitted to make an epidemiologic study of the tumors of the hand operated in the unit.MethodsWe made a retrospective study of all the cases of wrist and hand tumors operated in the unit, from January 1980 to September 2008. Files were collected from diagnostic encoding, letters of consultations and the register of the department of histology.ResultsThe mean age of the population was 48 years (from 16 to 94 years). The series included 623 tumors treated in 620 patients (372 women and 248 men). Tumors concerned soft tissues in 525 cases (84.1%), bone tissue in 70 cases (11.4%) and skin in 28 cases (4.5%). The prevalence of malign tumors was 1.3% in this study.ConclusionsThis series is the third in number of cases and the data are comparable of those from other studies. The prevalence of bone tumors (11.4%) is higher than in others studies (from 1.6 to 6.6%) and the prevalence of skin tumors is lower. Except for skin tumors which recruitment modalities are more variable, this series seems to be a good reflection of the prevalence of the different types of hand and wrist tumors.     

Étude rétrospective du résultat à long terme des neurotomies sélectives périphériques dans le traitement du membre supérieur spastique

Background/ObjectivePeripheral selective neurotomy is commonly used to treat the equinus spastic foot (tibial nerve), but is less frequently used in treating upper limb spasticity, because of the complexity of the articular deformities and the complex innervations of the different muscles. We present our experience and the long-term results of this surgery based on a retrospective series of 22 patients with a disabling spasticity of the upper limb.MethodsBetween 2003 and 2006, neurotomies were performed in 22 patients with disabling spasticity of the upper limb despite optimal medical treatment. Patients were evaluated before and after the surgical procedure. Twelve clinical parameters were studied for describing deformity (resting position and amplitude of each joint), spasticity (Ashworth and Tardieu scores), and the functional impacts of the spasticity.ResultsAt long-term follow-up, all parameters were improved from the surgery, both in terms of spastic symptoms (highly significantly decreased in Ashworth and Tardieu scores) and the deformity of the upper limb (e.g., 60° increase in the extension of the elbow). Pain, active amplitude, and functional impact scores were also statistically significantly improved after surgery. The mean satisfaction index was 7/10 (± 1.6).ConclusionsSelective neurotomy is an effective treatment for patients with a disabling and excessive spasticity in the upper limb. It provides a long-term, objective improvement based on analytical and functional parameters. We emphasize the importance of accurate clinical evaluation and surgical planning. Finally, excessive time to treatment seems to be an important factor for recurrence or incomplete efficiency of the procedure.