CIDP: Pathophysiologyreturn to main page of handbook of neuropathy
In one study, CIDP occurred within a few weeks after an infectious event in 16% of the patients
–Because of the insidious onset, documenting precipitating illnesses or events is very difficult
–Both respiratory and gastrointestinal infections have been cited, but no causative organism has been identified
–With Guillain-Barre syndome, the most common preceding infection is Campylobacter
•The classical picture that suggests CIDP iS the presence of proximal (Shoulder and hip) and distal ( Hand and foot) weakness, with large fiber sensory loss and reduced or absent reflexes.
–Few patients present with classic symtpoms.
•Some authors have suggested subclassifications based on the clinical varied clinical presentation in order to aid in diagnosis and treatment
–Currently these subclasses are not thought to be distinct diseases
•Lewis-Sumner syndrome : (LSS) is a dysimmune peripheral nerve disorder, characterized by a predominantly distal, asymmetric weakness mostly affecting the upper limbs with sensory impairment, and by the presence of multifocal persistent conduction blocks.
Multifocal Motor Neuropathy is an autoimmune disease in which
•Multifocal demyelinating neuropathy with persistent conduction block The chronic motor involvement associated with fasciculations and cramps, mainly in the arms, led, in most patients, to an initial diagnosis of motor neuron disease. In some patients (nine of 24), there was no appreciable muscle atrophy. Tendon reflexes were often absent or weak. The finding of persistent multifocal conduction block confined to motor nerve fibres raises questions about the nature and the importance of this syndrome. Segmental reduction of motor conduction velocity occurred at the site of the block, but significant slowing of motor nerve conduction was not found outside this site. The response to intravenous IVIg treatment seems to be correlated with the absence of amyotrophy. Patients with little or no amyotrophy had an initial and sustained response to IVIg, and did not develop amyotrophy during the follow up study.
•Distal CIDP M-proteins were present in 22% of patients with CIDP. There were no features that distinguished clearly between CIDP patients with or without an M-protein, and nearly all of these patients responded to immunomodulating therapy. In contrast, nearly two-thirds of the patients with DADS neuropathy had immunoglobulinM (IgM) kappa monoclonal gammopathies, and this specific combination predicted a poor response to immunomodulating therapy. Antimyelin-associated glycoprotein (anti-MAG) antibodies were present in 67% of these patients
acquired demyelinating sensory
neuropathy and sensory CIDP. Sensory
CIDP patients had gait ataxia, large
fiber sensory loss, and paresthesias,
and nine had frequent falls. The disease
course was chronic and progressive
(median duration 5 years, range 3 months
to 18 years. Can present with a sensory
ataxia .Sensory CIDP may present as
cryptogenic sensory polyneuropathy with
normal or axonal electrophysiologic