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Chronic inflammatory demyelinating polyneuropathy - still a challenging diagnosis

Daniela Leupold, Lenka Schilg-Hafer, Ansgar Felbecker, Dominik Zieglgänsberger, Barbara Tettenborn & Thomas Hundsberger

abstract Background and aims: Chronic inflammatory
demyelinating polyneuropathy (CIDP) is an immunemediated
neuropathy with a highly variable clinical
presentation. Hence, diagnosis is challenging and mostly
relies on clinical suspicion. Various treatment approaches
with different side effects and economical burden are
available.
Methods: We analysed clinical data, diagnostic workup and
treatment of cohort of 18 CIDP patients under intravenous
immunoglobulin therapy (IVIG) from 2003-2014.
Results: We identified 15 men and 3 women with a mean
symptom duration of 32 months before the diagnosis was
made. CIDP was diagnosed in all ages (mean 59 years;
range 18-78). Based on EFNS criteria clinical presentation
was typical in 7 and atypical in 11 patients. Predominantly
distal manifestation (DADS) was found in 1 patient, pure
sensory symptoms in 9 patients and focal involvement of
the brachial plexus in 1 patient. Just half of all patients
fulfilled the electrophysiological criteria of prominent
demyelinating features. Supportive laboratory features such
as elevated CSF protein were found in 15/18 patients. 10
patients received additional immunosuppressive therapy
beside immunoglobulins, most often prednisone (n=9).
Interestingly, the IVIG-dose and therapeutic interval were
highly variable.
Conclusion: In this retrospective cohort we illustrate the
clinical, diagnostic and therapeutic variability in CIDP
patients. Therefore, strict clinical and electrophysiological
criteria of CIDP are less sensitive for daily routine,
especially for atypical forms. Supportive criteria, especially
elevated CSF protein, could be helpful to define the
diagnosis. Difference in IVIG-dose and interval as well as
the use of concomitant immunosuppression were highly
variable which calls for individual treatment approaches
rather than a standard treatment.
   
citation Leupold D, Schilg-Hafer L, Felbecker A, Zieglgänsberger D, Tettenborn B, & Hundsberger T (2016). Chronic inflammatory demyelinating polyneuropathy - still a challenging diagnosis. In European Journal of Neurology (pp. 908): Wiley.
   
type conference paper/poster (Deutsch)
name of conference EAN (Kopenhagen)
date of conference 13-6-2016
title of proceedings European Journal of Neurology
pages 908
publisher Wiley
contact Ansgar Felbecker