@entity17 as a variant of XXXX : clinical, electrophysiological, laboratory features and response to treatment in 10 cases.

@entity1 @entity17 @entity1581 @entity16 @entity465 @entity2646 @entity400 @entity1778 @entity1200 @entity1893
BACKGROUND AND PURPOSE: Some @entity1 within the spectrum of @entity2646 ( @entity2646 ) have distal acquired demyelinating symmetric (DADS) @entity16 , usually associated with anti-myelin-associated-glycoprotein (MAG) IgM @entity1581 . The aim of this retrospective study was to investigate @entity1 with @entity17 without anti-MAG antibodies, and study their response to immunotherapy. METHODS: @entity1 were selected on the basis of (i) 'Definite @entity2646 ' according to the EFNS/PNS Guideline criteria, (ii) The presence of disproportionately prolonged motor latencies resulting in a @entity465 ( @entity465 )  <= 0.25 in at least two motor nerves and (iii) The absence of anti-MAG antibodies on ELISA. Response to immunotherapy was defined as persistent improvement by at least one point on the INCAT disability score. RESULTS: Data from 146 @entity2646 @entity1 were analysed, and 10 @entity1 were included. Six had clinically pure @entity16 , and four had @entity16 . Ataxia was present in nine @entity1 , generalized areflexia in seven and postural @entity400 in two. Five of the 10 @entity1 had abnormal sensory potentials only in the upper limbs. An associated condition was found in nine @entity1 : two chronic @entity1778 , four IgG @entity1581 (one associated with @entity1893 ) and two IgM @entity1581 of unknown significance. @entity1 were mostly improved with intravenous immunoglobulin (IVIg), corticosteroids, plasma exchanges, or a combination thereof. CONCLUSION: @entity17 without anti-MAG antibodies is more likely to be considered a variant of @entity2646 . In addition, such @entity1 should be systematically investigated for an associated @entity1200 .
