Clinical Manifestations

Marinos C. Dalakas, M.D., Principal Author

In the "classic" form, the onset of chronic inflammatory demyelinating polyneuropathy (CIDP) is usually gradual. The disorder is characterized primarily by progressive, usually symmetrical, weakness in the legs and arms that increases for more than 2 months (thereby distinguishing itself from Guillain Barre syndrome (GBS), which reaches its zenith within 2 to 3 weeks, followed by a plateau period and subsequently a gradual, but complete, recovery).

Hyporeflexia, impaired ambulation, sensory loss, often with distal loss of proprioception, and prominent electrophysiological signs of demyelination are evident in CIDP patients.

Limb weakness involving both proximal and distal muscles (Dyck, 1975) is usually symmetrical but in some CIDP patients can begin asymmetrically.

Most CIDP patients experience a chronic progressive course but some, usually younger patients, may have a relapsing and remitting course. Although occasionally patients achieve a sustained remission without the requirement of therapy, and some patients do go into remission after treatment, in most patients, CIDP remains a chronic disorder requiring maintenance therapy indefinitely.

Most CIDP patients who present with motor symptoms only have some sensory abnormality on clinical examination and/or sensory abnormalities on electrodiagnostic testing.  More sensory symptoms and signs may develop as the disorder progresses, but the motor features usually remain predominant.  On the other hand, as many as 15% of CIDP patients may present with only sensory symptoms including tingling, "pins and needles" sensations, and lack of coordination. They may have no clinical weakness, but motor conduction studies reveal conduction slowing in motor nerves as well as in sensory nerves.  While many of the CIDP patients who start with only sensory symptoms develop motor signs within two to three years, many persist with a pure clinical sensory disorder. (van Dijk, 1999)

Tremor may occur in about 10% of patients and become more prominent during subacute worsening of the disorder. (Dalakas 1991; Dalakas 1984) Cranial nerves may be affected in at least 10% of patients, (Dyck et al 1975) but not as often as in GBS. Optic neuritis also may be seen, implying coexisting central demyelination.

CIDP, originally described as a steroid-responsive disorder by Austin (1948), responds to immunotherapies. After a year of successful therapy, nearly 75% of CIDP patients regain a reasonable functional status.  Early diagnosis and treatment can play an important role in increasing the prospects for a good recovery. Death from CIDP is uncommon. (Mendell, 2001)