Ministerul Sănătăţii Anexă din 16/09/2010 Intrare In Vigoare

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ANEXA Nr. 20
Ghidul EFNS/Peripheral Nerve Society pentru managementul
neuropatiilor demielinizante paraproteinemice: Raportul Grupului
Comun de Lucru al EFNS şi al Peripheral Nerve Society
R.D.M. Hadden^a, E. Nobile-Orazio^b, C. Sommer^c, A. Hahn^d, I. Illa^e, E. Morra^f, J. Pollard^g, R.A.C. Hughes^h,
P. Bouche^i, D. Cornblath^j, E. Evers^k, C.L. Koski^l, J.M. Leger^m, P. Van den Bergh^n, P. van Doorn^o, I.N. van
Schaik^p
___________
^a Department of Neurology, King's College Hospital, London, UK;
^b Department of Neurological Science, University of Milan, Milan, Italy;
^c Department of Neurology, University of Wurzburg, Wurzburg, Germany;
^d Department of Clinical Neurological Sciences, University of Western Ontario, London, Canada;
^e Department of Neurology, Hospital Sta. Creu i Sant Pau, Barcelona, Spain;
^f Department of Haematology, Niguarda Hospital, Milan, Italy;
^g Department of Medicine, University of Sydney, Sydney, Australia;
^h Department of Clinical Neurosciences, King's College, London School of Medicine, London, UK;
^i Department of Neurophysiology, CHU Pitie-Salpetriere, Paris, France;
^j Department of Neurology, Johns Hopkins University, Baltimore, MD, USA;
^k Lay Member, Guillain-Barre Syndrome Support Group of the UK, Sleaford, UK;
^l Department of Neurology, University of Maryland, Baltimore, MD, USA;
^m Department of Neurology, Faculte de Medecine Pitie-Salpetriere, Paris, France;
^n Department of Neurology, Clinique Universitaire St-Luc, Brussels, Belgium;
^o Department of Neurology, Erasmus Medical Centre, Rott erdam, The Netherlands; and
^p Department of Neurology, Academic Medical Centre, Amsterdam, The Netherlands.
Cuvinte cheie: demielinizare, ghiduri, MAG (glicoproteina asociată mielinei), gamapatie monoclonală, neuropatie,
paraproteină, POEMS, tratament.
GENERALITĂŢI
Neuropatiile asociate cu paraproteine au heterogenitate clinică, neurofiziologică, neuropatologică şi hematologică.
Obiective: Redactarea ghidurilor de consens bazate pe dovezi pentru managementul clinic al pacienţilor cu neuropatie
demielinizantă paraproteinemică. Metode: Căutarea în MEDLINE şi biblioteca Cochrane pentru revizuirea datelor de
evidenţă şi consens despre acest subiect.
RECOMANDĂRI
În absenţa datelor adecvate, recomandările bazate pe evidenţe nu s-au putut stabili, dar au fost emise recomandări de
bună practică medicală: (1) Pacienţii cu NDP trebuie investigaţi pentru o discrazie celulară plasmatică malignă. (2)
Paraproteina este mai probabil să cauzeze neuropatia dacă este IgM, iar anticorpii sunt prezenţi în ser sau la biopsie,
sau dacă fenotipul clinic este de neuropatie senzitivă distală cronică. (3) Pacienţii cu NDP IgM de obicei au afectare
predominant distală şi senzitivă, cu prelungirea latenţelor motorii distale şi frecvent anticorpi antiMAG. (4) NDP IgM
răspunde uneori la imunoterapie. Beneficiul trebuie pus în balanţă cu reacţiile adverse şi progresia de obicei lentă a bolii.
(5) NDP IgG şi IgA nu pot fi diferenţiate de polineuropatia cronică inflamatorie demielinizantă (PCID) din punct de vedere
clinic, electrofiziologic şi al răspunsului terapeutic. (6) Pentru sindromul POEMS trebuie luate în considerare iradierea
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