Description

European Federation of Neurological Societies and the Peripheral Nerve Society reported criteria for the diagnosis of Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP). One element involves the clinical features.


One of the following:

(1) typical CIDP

(2) atypical CIDP

 

Typical CIDP - all of the following:

(1) chronically progressive, stepwise or recurrent symmetric proximal and distal weakness and sensory dysfunction of all extremities

(2) develops over >= 2 months

(3) variable cranial nerve involvement

(4) absent or reduced tendon reflexes in all extremities

 

Atypical CIDP - one of the following:

(1) predominantly distal (distal acquired demyelinating symmetric)

(2) asymmetric (multifocal acquired demyelinating sensory or motor neuropathy, MADSAM)

(3) focal (brachial plexus, lumbosacral plexus, >=1 nerve in one extremity)

(4) pure motor

(5) purely sensory (including chronic immune sensory polyradiculopathy)

 

In atypical CIDP the tendon reflexes may be normal in unaffected limbs.

 

Exclusion criteria:

(1) infection: Borrelia burgdorferi, diphtheria

(2) drug or toxin exposure causing neuropathy

(3) hereditary demyelinating neuropathy

(4) prominent sphincter disturbance

(5) multifocal motor neuropathy

(6) IgM monoclonal gammopathy with antibodies to myelin-associated glycoproteins

(7) other causes of demyelinating neuropathy (POEMS, osteosclerotic myeloma, diabetes, non-diabetic lumbosacral radiculoplexus neuropathy, PNS lymphoma, amyloidosis)


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