JOURNAL CLUB Diagnosis and treatment of chronic inflammatory demyelinating polyneuropathy Reem A. Amin • N. P. Robertson Ó Springer-Verlag Berlin Heidelberg 2015 Chronic inflammatory demyelinating neuropathy (CIDP) is generally considered to be an immune-mediated neuropa- thy with potential target antigens, although its precise pathogenesis is unclear. Unlike its more acute counterpart, acute inflammatory demyelinating neuropathy, the diag- nosis is necessarily delayed as a result of clinical defini- tions but also the fluctuating clinical manifestations which can evolve over months and years. In addition, as a result of its relative rarity it is not usually definitively diagnosed and treated until patients come into contact with specialist neurology services. However, even within specialist set- tings the diagnosis is often elusive as a result of hetero- geneous clinical manifestations, the patchy nature of the demyelinating pathology and limitations of available diagnostic tests. Despite the limited understanding of disease aetiology, recent studies have underlined the important role of both cellular and humoral components of the immune system in pathogenesis which in turn has informed treatment ap- proaches. Although there remains no overall consensus on the optimum management strategy for CIDP, it is becom- ing clear that individualising therapeutic interventions de- pending on accurate identification of the CIDP variant is important. These include the typical symmetrical motor and sensory polyneuropathy, multifocal acquired de- myelinating sensory and motor neuropathy (MADSAM), pure motor, pure sensory and distal acquired demyelinating symmetric (DADs) neuropathy, all of which may demon- strate variation in response to intravenous im- munoglobulins, plasma exchange (PE) and other immunosuppressive therapies possibly as a result of dif- ferent underlying pathological mechanisms. In this month’s Journal Club, we review three papers focusing on different aspects of diagnosis and treatment in CIDP. The first paper explores patterns of magnetic reso- nance neurography and the distribution of nerve hypertro- phy in CIDP variants. The second paper reviews different electrophysiological profiles and treatment response in typical and atypical forms, whilst the third investigates predictors of intravenous immunoglobulin (IVIG) respon- siveness in patients with CIDP. Reconstruction magnetic resonance neurography in chronic inflammatory demyelinating polyneuropathy Magnetic resonance imaging (MRI) has gained increasing credibility in the evaluation of peripheral neuropathies. In particular, it is able to define the distribution of nerve hypertrophy that may provide objective information to aid disease classification. The primary objective of this study was to evaluate the patterns and distribution of nerve en- largement in CIDP and to investigate whether there is pattern variation amongst CIDP subtypes. Sixty-five patients (33 CIDP patients and 32 disease controls) were examined using a magnetic resonance neurography with three-dimensional reconstruction of short-tau inversion recovery (STIR) images to visualise from the nerve root to the peripheral nerve trunk in the upper arms. Median age of the patients studied was 47 with median disease duration of 7.2 years. Twenty-seven cases of typical CIDP and 6 with MADSAM were included. MADSAM was defined as typical mononeuropathy multiplex or asymmetric weak- ness manifesting as differences in muscle strength by one or more Medical Research Council (MRC) scale in the R. A. Amin Á N. P. Robertson (&) Department of Neurology, Institute of Psychological Medicine and Clinical Neuroscience, Cardiff University, Cardiff, UK e-mail: robertsonnp@cardiff.ac.uk 123 J Neurol DOI 10.1007/s00415-015-7667-x