Neuromyelitis optica in France: a multicenter study of 125 patients.

BACKGROUND: There have been few epidemiologic studies on neuromyelitis optica (NMO) and none used the recent 2006 diagnostic criteria. Here we describe the clinical, laboratory, MRI, and disability course of NMO in a French cohort of 125 patients. METHODS: We performed an observational, retrospective, multicenter study. Data were collected from September 2007 through August 2008, corresponding to the endpoint of the study. We identified 125 patients fulfilling the 2006 NMO criteria. Selection was made using hospital files and a specific clinical questionnaire for NMO. RESULTS: Mean age at onset was 34.5 years (range 4-66) with a mean disease duration of 10 +/- 7.8 years at the endpoint. The patients were mainly (87%) Caucasian, with a female:male ratio of 3:1. In 90% of cases, the association of optic neuritis, longitudinal extensive myelitis, and a Paty-negative initial brain MRI was sufficient to fulfill the supportive criteria. Eighty-eight percent of patients were treated with immunosuppressive therapies. Median delay from onset to Expanded Disability Status Scale (EDSS) score 4 was 7 years; score 6, 10 years; and score 7, 21 years. The first episode of myelitis was immediately followed by an EDSS score > or = 4 in 37.3% of cases, and a severe residual visual loss was observed in 22% of patients after the first episode of optic neuritis. Multivariate analysis did not reveal any predictors of a poor evolution other than a high number of MRI brain lesions at diagnosis, which were predictive of a residual visual acuity < or = 1/10. CONCLUSIONS: Our demographic data provide new data on disability in patients with neuromyelitis optica, most of whom were receiving treatment.

[Manubrio-sternal arthritis and behcet's disease: report of 3 cases]. / Arthrite manubrio-sternale et syndrome de Behçet: à propos de 3 observations.

Manubrio-sternal joint involvement in arthritis is described and may be evidenced by erosion, reactive sclerosis and ankylosis. Some reports of such involvement appear in the radiological literature in association with psoriatic arthritis and spondylarthropathy. Involvement of the manubrio-sternal joint in Behçet's disease is very uncommon. To our knowledge, only one case has been described. We report a case of manubrio-sternal arthritis in a women with Behçet's disease. Two others cases with suspicion of Behçet's disease are described.

[Spinal cord compression caused by extramedullary hematopoiesis foci in the course of thalassemia]. / Compression médullaire par des foyers d'hématopoïèse extra médullaire au cours de la thalassémie.

BACKGROUND: Extramedullary haematopoiesis is a physiological response to chronic anemia, observed frequently during homozygous thalassemia. It is usually asymptomatic but can be manifested by compression of adjacent organs, particularly the spinal cord. CASE REPORT: A 44-year-old woman diagnosed with thalassaemia intermedia, was admitted for difficulties to walk and sphincter disturbances. Neurologic examination suggested spinal cord compression, which is confirmed by dorso-lumbar resonance magnetic imaging. The histology obtained by laminectomy led to the diagnosis of extramedullary hematopoiesis related to thalassemia. A radiotherapy enabled with good outcome. DISCUSSION: Spinal cord compressions by extramedullary hematopoiesis during thalassemia are uncommon (75 cases in the literature) but can induce severe sequelae if the diagnostic is not rapidly obtained. Magnetic resonance imaging is the gold standard allowing precise diagnosis and spreading of extramedullary hematopoiesis. Radiotherapy and more recently hydroxyurea are the first line treatment. CONCLUSION: This observation recalls that extramedullary hematopoiesis is a differential diagnostic of spinal cord compression in patients with thalassemia. A screening of paravertebral localization of extramedullary hematopoiesis should be performed in high risk thalassemic patients.

[Multiple sclerosis and pregnancy: clinical issues]. / Sclérose en plaques et grossesse: aspects cliniques.

Multiple sclerosis (MS) is the most common chronic disabling neurological disease affecting young women. Paradoxically, our knowledge of the relationship between pregnancy and MS is limited. However, several conclusions emerge from the literature: 1) The rate of relapse in MS decreases during pregnancy, and it rises significantly during the first three months post partum before coming back to its level prior to pregnancy. 2) Although pregnancy and delivery cause changes of the relapse rate, they have no influence on mid and long term residual disability. 3) Breast-feeding and epidural analgesia do not seem to have any deleterious effect on the disease. 4) Lastly, MS does not seem to influence pregnancy, delivery or the child's health. The studies available to date suffer from methodological limitations. They need to be confirmed by prospective studies. This is the purpose of the study entitled "Pregnancy in multiple sclerosis, PRIMS", which has been carried out since 1992 at the European level.

Rate of pregnancy-related relapse in multiple sclerosis. Pregnancy in Multiple Sclerosis Group.

BACKGROUND AND METHODS: Multiple sclerosis often occurs in young women, and the effect of pregnancy on the disease is poorly understood. We studied 254 women with multiple sclerosis during 269 pregnancies in 12 European countries. The women were followed during their pregnancies and for up to 12 months after delivery to determine the rate of relapse per trimester and the score on the Kurtzke Expanded Disability Status Scale (range, 0 to 10, with higher scores indicating more severe disability). The relapse rate in each trimester was compared with the rate during the year before the pregnancy. The effects of epidural analgesia and breast-feeding on the frequency of relapse during the first three months post partum and the disability score at 12 months post partum were also determined. RESULTS: The mean (+/-SD) rate of relapse was 0.7+/-0.9 per woman per year in the year before pregnancy; it was 0.5+/-1.3 during the first trimester (P=0.03 for the comparison with the rate before pregnancy), 0.6+/-1.6 during the second trimester (P=0.17), and 0.2+/-1.0 during the third (P<0.001). The rate increased to 1.2+/-2.0 during the first three months post partum (P<0.001) and then returned to the prepregnancy rate. The mean Kurtzke disability score worsened by 0.7 point during 33 months of follow-up, with no apparent acceleration during the post-partum period. Neither breast-feeding nor epidural analgesia had an adverse effect on the rate of relapse or on the progression of disability in multiple sclerosis. CONCLUSIONS: In women with multiple sclerosis, the rate of relapse declines during pregnancy, especially in the third trimester, and increases during the first three months post partum before returning to the prepregnancy rate.

[A brachial form of motor neuropathy caused by lead poisoning]. / Forme brachiale de neuropathie motrice par intoxication au plomb.

We report the case of a 48-year-old patient who developed acute onset bilateral, peripheral motor dysfunction in the arms and forearms. Clinical history, electromyography and laboratory tests confirmed the diagnosis of peripheral neuropathy caused by lead poisoning from the domestic water supply. The EMG showed axonal alterations. Improvement was observed after treatments with chelating agents and removal of the source of poisoning. This case provides the opportunity to recall the traditional 1889 Dejerine classification of lead-related peripheral neuropathies and is a warning signal that lead poisoning remains a problem in peri-urban areas of developed countries.