French Society of ENT (SFORL) guidelines. Management of acute Bell's palsy.

AQFThe authors present the guidelines of the French Society of ENT and Head and Neck Surgery (SFORL) regarding the management of Bell's palsy in adults. After a literature review by a multidisciplinary workgroup, guidelines were drawn up based on retrieved articles and group-members' experience, then read over by an independent group to edit the final version. Guidelines were graded A, B, C or "expert opinion" according to decreasing level of evidence. Thorough ENT and neurological clinical examination is recommended in all patients presenting with peripheral facial palsy to confirm diagnosis of Bell's palsy. MRI with gadolinium enhancement should explore the entire course of the facial nerve, if possible within the first month. ENMG should be performed to assess prognosis for recovery. In confirmed Bell's palsy, corticosteroid therapy should be implemented as early as possible (ideally within 72h) at a dose of 1mg/kg/day for 7-10 days. Antiviral therapy should be associated to steroids in patients with severe and early-onset disease and in Ramsay-Hunt syndrome. Isolated antiviral therapy is not recommended. To date, there is no evidence that surgical facial nerve decompression provides benefit.

Focal myositis: A review.

Amongst the heterogeneous group of inflammatory myopathies, focal myositis stands as a rare and benign dysimmune disease. Although it can be associated with root and/or nerve lesions, traumatic muscle lesions and autoimmune diseases, its triggering factors remain poorly understood. Defined as an isolated inflammatory pseudotumour usually restricted to one skeletal muscle, clinical presentation of focal myositis is that of a rapidly growing solitary mass within a single muscle, usually in the lower limbs. Electromyography shows spontaneous activity associated with a myopathic pattern. MRI reveals a contrast enhanced enlarged muscle appearing hyper-intense on FAT-SAT T2 weighted images. Adjacent structures are spared and there are no calcifications. Serum creatine kinase (CK) levels are usually moderately augmented and biological markers of systemic inflammation are absent in most cases. Pathological histological features include marked variation in fibre size, inflammatory infiltrates mostly composed of T CD4+ lymphocytes and macrophages, degenerating/regenerating fibres and interstitial fibrosis. Differential diagnoses are numerous and include myositis of other origin with focal onset. Steroid treatment should be reserved for patients who present with major pain, nerve lesions, associated autoimmune disease, or elevated C reactive protein or CK.

Evaluation of collected outcome measures in axial spondyloarthritis in daily-care rheumatology settings: the experience of the RHEVER network.

OBJECTIVES: Nowadays, the recommended measures for optimal monitoring of axial Spondyloarthritis (ax-SpA) disease activity are either BASDAI and CRP, or ASDAS-CRP. However, there could be a gap between recommendations and daily practice. We aimed to determine the measures collected by rheumatologists in an ax-SpA follow-up visit, and to determine the impact of a meeting (where rheumatologists reached a consensus on the measures to be collected) on the collection of such measures. METHODS: A consensual meeting of a local network of 32 rheumatologists proposed, four months later, to report at least the BASDAI score in the medical file of every ax-SpA patient at every follow-up visit. An independent investigator reviewed the medical files of 10 consecutive patients per rheumatologist, seen twice during the year (e.g. before and after the meeting). The most frequently collected measures were assessed, and then, the frequency of collection before and after the meeting was compared. RESULTS: A total of 456 medical files from 228 patients were reviewed. Treatment (>60%), CRP (51.3%) and total BASDAI (28.5%) were the most reported measures in medical files. Before/After the meeting, the frequencies of collected measures in medical files were 28.5%/51.7%, 51.3%/52.2%, 16.7%/31.6% and 0.9%/6.1% for BASDAI, CRP, BASDAI + CRP and ASDAS, respectively reaching a statistically significance for BASDAI, ASDAS and BASDAI+CRP (p<0.05). CONCLUSIONS: This study revealed a low rate of systematic report of the recommended outcome measures in ax-SpA. However, it suggests that a consensual meeting involving practicing rheumatologists might be relevant to improve the implementation of such recommendations.

Antibodies to clustered acetylcholine receptor: expanding the phenotype.

BACKGROUND AND PURPOSE: To provide a detailed phenotypical description of seronegative patients with generalized myasthenia gravis and antibodies to clustered acetylcholine receptors (AChRs) and to assess their frequency amongst a French seronegative generalized myasthenia gravis (SNMG) population. METHODS: A French SNMG database was created and the sera from the 37 patients included in it were analysed by immunofluorescence of cell-based assays using cotransfection of AChR subunit genes together with rapsyn to densely cluster the AChRs. RESULTS: Sixteen per cent (n = 6) of the SNMG patients were found to have antibodies to clustered AChR. They presented either with early onset MG and thymic hyperplasia, late onset MG and thymic involution, or thymoma associated MG. They responded well to cholinesterase inhibitors and immunosuppressants. CONCLUSIONS: Patients with antibodies to clustered AChR account for a significant proportion of SNMG patients and resemble patients with AChR antibodies detected by standard radio-immunoprecipitation.

[Necrotizing autoimmune myopathies]. / Les myopathies nécrosantes auto-immunes.

Necrotizing autoimmune myopathies are included in the spectrum of inflammatory myopathies, together with polymyosis, dermatopolymyosis and inclusion body myositis, despite the characteristic feature of marked muscular necrosis without inflammatory infiltrates. The clinical presentation is highly variable, often similar to the other inflammatory myopathies. The most common finding is nevertheless the severe form with rhabdomyolysis. The creatine kinase level is elevated (around 10,000IU/l) and electromyography shows myopathic changes with increased spontaneous activities reflecting the importance of the muscular necrosis. Muscle biopsy is required for diagnosis, revealing active necrosis of the muscle fibers without inflammatory invasion by CDA+ or CD8+ T-cells. Deposition of a microvascular membrane attack complex (C5b9) is often noted, whereas the upregulation of MHC class 1 is rarely detected. Signs of endomysial microangiopathy are frequently reported. Necrotizing autoimmune myopathies can be associated with antisignal recognition particle (SRP) antibodies or more rarely with the usual inflammatory myopathy antibodies. Paraneoplasic forms are described but remain exceptional. Lastly, necrotizing autoimmune myopathies, sometimes associated with statin therapy, have been recently described. They are linked with an antibody directed against 3-hydroxy-3-methyglutaryl-coenzyme A. Treatment is based on corticosteroid therapy, immunosuppressive drugs or intravenous immunoglobulins. Response is variable, depending on the clinical form.

[Distal hereditary motor neuropathy]. / Les amyotrophies spinales distales.

INTRODUCTION: Distal hereditary motor neuropathy (dHMN), also known as spinal muscular atrophy, represents a group of clinically and genetically heterogeneous diseases caused by degenerations of spinal motor neurons and leading to distal muscle weakness and wasting. Nerve conduction studies reveal a pure motor axonopathy and needle examination shows chronic denervation. STATE OF ART: dHMN were initially subdivided into seven subtypes according to mode of inheritance, age at onset, and clinical evolution. Recent studies have shown that these subtypes are still heterogeneous at the molecular genetic level and novel clinical and genetic entities have been characterized. To date, mutations in 11 different genes have been identified for autosomal-dominant, autosomal-recessive, and X-linked recessive dHMN. Most of the genes encode protein involved in housekeeping functions, endosomal trafficking, axonal transport, translation synthesis, RNA processing, oxidative stress response and apoptosis. PERSPECTIVES: The pathophysiological mechanisms underlying dHMN seem to be related to the "length-dependent" death of motor neurons of the anterior horn of the spinal cord, likely because their large axons have higher metabolic requirements for maintenance. CONCLUSION: dHMN remain heterogeneous at the clinical and molecular genetic level. The molecular pathomechanisms explaining why mutations in these ubiquitously expressed housekeeping genes result in the selective involvement of spinal motor neurons remain to be unravelled.

Comparison of the efficacy of nonsurgical synovectomy (synoviorthesis) and joint lavage in knee osteoarthritis with effusions.

OBJECTIVE: Radioactive or chemical synovectomy (synoviorthesis) is widely used as local therapy for inflammatory joint disease in France. The objective of this retrospective study was to compare the efficacy of osmic acid or radiation synovectomy with that of joint lavage for the treatment of knee osteoarthritis with effusions. PATIENTS AND METHODS: All study patients met American College of Rheumatology criteria for knee osteoarthritis, which was symptomatic despite conservative therapy including local corticosteroid injections. Fifty-four patients were treated by synoviorthesis (osmic acid, n = 16; yttrium 90, n = 76) and 45 by joint lavage (total 67 lavages). RESULTS: Thirty two per cent of the patients in the synoviorthesis group had a good or excellent outcome after six months. Results were better with yttrium 90 than with osmic acid. Improvements were most marked in patients with chondrocalcinosis. Efficacy was negatively correlated with the femorotibial lesions but not with the patellofemoral lesions. Patients with knee alignment disorders had poorer outcomes. In the joint lavage group, 30% of the knees showed improvements after three months and results were significantly better after three and six months when the lavage was followed by an injection of triamcinolone hexacetonide. No side effects were recorded. CONCLUSION: Our data suggest that chemical or radiation synovectomy or joint lavage followed by injection of a delayed-action steroid may be useful for the treatment of knee osteoarthritis with chronic or recurrent effusions.

[Acute appendicitis in pregnancy, labour, and puerperium]. / Akutni apendicitis u trudnoci, porodu i babinjama.

Four patients with appendicitis in pregnancy and one in puerperium are described. In two pregnant women, in the 12th and the 15th week of pregnancy, the surgery was applied in time and normal deliveries followed. The third patient, after a perforated appendicitis and applied appendectomy, developed the obstruction of the intestines, was operated on again, and then had a spontaneous abortion. The fourth patient, in the 20th week of pregnancy, came to hospital with a diffuse peritonitis following a perforated appendicitis and with a dead fetus. During appendectomy the fetus was removed by using small cesarean section. The fifth patient was surgically treated on the 8th day following delivery for retrocecal perforative appendicitis and diffuse peritonitis. All the patients survived.