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CIDP info

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an inflammatory neuropathy, classically characterised by a slowly progressive onset and symmetrical, sensorimotor involvement. However, there are many phenotypic variants, suggesting that CIDP may not be a discrete disease entity but rather a spectrum of related conditions. While the abiding theory of CIDP pathogenesis is that cell-mediated and humoral mechanisms act together in an aberrant immune response to cause damage to peripheral nerves, the relative contributions of T cell and autoantibody responses remain largely undefined. In animal models of spontaneous inflammatory neuropathy, T cell responses to defined myelin antigens are responsible. In other human inflammatory neuropathies, there is evidence of antibody responses to Schwann cell, compact myelin or nodal antigens. In this review, the roles of the cellular and humoral immune systems in the pathogenesis of CIDP will be discussed. In time, it is anticipated that delineation of clinical phenotypes and the underlying disease mechanisms might help guide diagnostic and individualised treatment strategies for CIDP.

There are many phenotypic variants of CIDP. Indeed, CIDP may not be a discrete disease entity but rather a spectrum of discrete albeit related conditions in which immunogenetic variations drive individual phenotypic differences

Typical CIDP involves motor and sensory nerve dysfunction, with motor deficits reported in up to 94% of patients and sensory deficits in up to 89%. However, only 50% of patients with CIDP display the typical phenotype

CIDP

While typical CIDP is characterised by proximal and distal involvement, the distal acquired demyelinating symmetric neuropathy (DADS) variant is restricted to a distal, symmetrical distribution with predominantly sensory symptoms, although there is often electrophysiological evidence of motor involvement. In 50-70% of patients with the clinical picture of DADS phenotype, the cause is a distinctly separate condition in which an IgM paraprotein having antimyelin-associated glycoprotein (anti-MAG) antibody activity is responsible for the pathogenesis. However, the DADS clinical picture may also be caused by a phenotypic variant of CIDP, with considerable overlap with sensory and sensory ataxic CIDP phenotypes
Motor dominant CIDP has been reported, with patients demonstrating relapsing remitting weakness with minor or no sensory electrophysiological features or symptoms. The motor dominant phenotype represents 7-10% of patients with CIDP, with higher rates in patients <20 years age.31 The major differential diagnosis of motor CIDP, particularly the rare instances of focal motor CIDP, is multifocal motor neuropathy (MMN, see below).

Lewis-Sumner syndrome (LSS) or multifocal acquired demyelinating sensory and motor neuropathy (MADSAM) is characterised by asymmetry, presenting as a multifocal multiple mononeuropathy most commonly in the upper limbs. It accounts for 6-15% of CIDP patients. Patients demonstrate abnormal sensory and motor nerve conduction, with multifocal areas of conduction block predominating in one or both upper limbs. The majority of patients eventually develop diffuse, typical CIDP spreading to the other limbs

CIDP info CIDPUSA.org

CIDP info

CIDP

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