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Journées SFM 2015 : Myasthénie associée à une myopathie inflammatoire brachiocervicale

Posté : 30 déc. 2015 12:17
par Pboulanger
:hi:

Lu dans le livre des résumés des présentations faites lors du congrès "13éme journées de la Société Française de Myologie SFM" tenu à l'Ecole Normale Supérieure de Lyon les 23-24 & 25 Novembre 2015 http://www.congresjsfm.org/?q=fr/node/15

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Myasthenia gravis associated with brachiocervical inflammatory myopathy

Jessie AOUIZERATE (1,2), Hayet SALHI (3), Jean-Pascal LEFAUCHEUR (4),
Violaine PLANTE-BORDENEUVE (3), François Jérôme AUTHIER (1,2)

1. Reference Centre for Neuromuscular Diseases, Henri Mondor University Hospitals, Créteil, FRANCE
2. UMR INSERM-UPEC U955-Team 10, School of Medicine, Créteil, FRANCE
3. Neurology Department, Henri Mondor University Hospitals, Créteil, FRANCE
4. Neurophysiology Department, Henri Mondor University Hospitals, Créteil, FRANCE


Brachiocervical inflammatory myopathy (BCIM) is a rare condition usually observed in patients with autoimmune disorders and characterized by (i) progressive weakness in the proximal regions of arms and neck; and (ii) inflammation at muscle biopsy (Pestronk et al, Arthritis Rheum 2006 ; 54 : 1687-­‐96).

We report here the case of 62 yrs-­‐old woman who developed dropped head and painful muscle weakness over 6 months in parallel with anorexia and weight loss. Physical examination disclosed muscle deficit prominently affecting upper limbs and cervical paraspinal muscles, diffuse amyotrophy, dysphonia and dysphagia. EMG showed myopathic features and a decrement at 3 Hz stimulation. Biological tests showed increased CK levels (2xN), positive detection of antinuclear (1/1280; nucleolar), anti-­PM/Scl, and anti AChR (0.51mM) autoantibodies. Thorax scan showed mediastinal adenopathy but no thymoma. Muscle biopsy showed active myopathy with predominantly perimysial and perivascular inflammation, nodular B-­‐cell lymphocytic infiltrates and sarcolemmal and endomysial C5b-­‐9 deposition. Outcome was favorable under pyridostigmine, prednisone, azathioprine and polyvalent immunoglobulins.

Myasthenia gravis (MG) was reported in 40% of patients with BCIM. Clinical features of MG may be masked by muscular impairment. The presence of posterior neck weakness or oculomotor signs must prompt the search of MG. Myopathological features, especially the presence of B-­‐cell infiltrates and the pattern of C5b-­‐9 deposition, are suggestive and contribute to the diagnosis. Identifying BCIM is of importance because of clinical and therapeutical implications.