A current view of the diagnosis, clinical variants, response to treatment and prognosis of chronic inflammatory demyelinating polyradiculoneuropathy.
We retrospectively analyzed 146 patients fulfilling the European Federation of Neurological Societies and the Peripheral Nerve Society (EFNS/PNS) criteria for definite chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) to (1) evaluate the relevance of these criteria, (2) assess the frequency of CIDP variants, and (3) determine the response to treatment and the prognosis. We found that 75% of these patients fulfilled the main EFNS/PNS clinical and electrophysiological criteria (type I). The remaining patients were diagnosed using laboratory tools as supportive criteria. The common form of CIDP represented 51% of patients. We observed a high frequency of the sensory variant (35% of patients) and the rapid onset form (18%). A positive response to treatment was observed in 87% of patients, with a similar efficacy of prednisone and IVIg. However, in the long term, 40% of treated patients remained dependent on treatment. The IVIg dependency rate was higher than the prednisone or plasma exchange dependency rate (55%, 18%, and 23%, respectively; p = 0.0054). Severe handicap was observed in 24% of patients.
Viala
Karine
K
Fédération de Neurophysiologie Clinique, AP-HP, Groupe Hospitalier Pitié Salpetrière, Paris, France. karine.viala@psl.aphp.fr
Maisonobe
Thierry
T
Stojkovic
Tanya
T
Koutlidis
Régine
R
Ayrignac
Xavier
X
Musset
Lucile
L
Fournier
Emmanuel
E
Léger
Jean-Marc
JM
Bouche
Pierre
P
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005060:Europe; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D009462:Neurology; D017933:Peripheral Nervous System; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000175:diagnosis* / Q000453:epidemiology / Q000503:physiopathology / Q000628:therapy*; D011379:Prognosis; D012189:Retrospective Studies; D012955:Societies, Medical; D012958:Societies, Scientific; D016896:Treatment Outcome; D055815:Young Adult