Submitted by: Francisco E. Gomez, MD
Edited by: Niranjan N. Singh, MD
Doneddu PE, Cocito D, Manganelli F, et al. Atypical CIDP: Diagnostic criteria, progression and treatment response. Data from the Italian CIDP Database. J Neurol Neurosurg Psychiatry
. Published Online First: 08 October 2018. doi: 10.1136/jnnp-2018-318714.
In this multi-center retrospective analysis, the authors sought to describe several variants of atypical CIDP as well as their respective diagnostic criteria. These variants are well characterized in the text and include Distal Acquired Demyelinating Syndrome (DADS), pure motor or pure sensory CIDP, Lewis-Sumner Syndrome (MADSAM; Multifocal acquired demyelinating sensory and motor neuropathy) and Focal CIDP. They then applied said diagnostic criteria to a pool of 460 patients (median age 60, 64% men), finding 82% of cases diagnosable as typical CIDP and 18% as atypical (34 DADS, 17 Motor CIDP, 16 sensory CIDP, and 17 with Lewis-Sumner Syndrome). Of interest, they later found that 39% of patients at initial presentation met atypical CIDP criteria, of which 53% progressed to typical CIDP by 5.5 years. Typical CIDP tended to be more disabling. A large proportion of the atypical subtypes of CIDP were treatment responsive.
In this large retrospective review article from a pool of 460 patients, authors have tried to analyze the clinical features and phenotype of typical and atypical CIDP, prognosis and treatment response. Of 1 in 5 patients diagnosed with atypical CIDP at the onset, more than 50% progress to typical CIDP over a span of more than 5 years. DADS and LSS have a less frequent response to intravenous immunoglobulin compared to typical CIDP, raising the possibility of a different underlying pathogenetic mechanism.