Chronic inflammatory demyelinating polyneuropathy

An acquired peripheral neuropathy due to an autoimmune attack of peripheral nerve myelin.

Myelin is the main target of the process, and nerve fibers with the most myelin are the most involved in patients present and weakness, numbness, and sensory ataxia.

The largest nerve fibers are those with the most myelin.

Estimated prevalence 0.5 per 100,000 children and 1-2 per 100,000 adults.

Symmetrical weakness in proximal and distal muscles that progressively increases for more than two months.

Characterized by symmetrical, motor predominant peripheral neuropathy that produces both distal and proximal weakness.

It has a varied course, and presentations include relapsing-remitting,stepwise progressive, or gradually progressive disease.

Large fiber abnormalities, weakness and ataxia, predominate, while small fiber abnormalities, autonomic and pain are less common.

The clinical pattern isn’t like typical peripheral neuropathies that are length dependent with the most distal segments being the most involved.

Inflammatory demyelination manifests as slowed conduction velocities , temporal dispersion, and conduction block on nerve conduction studies ad as segmental demyelination, onion bulb formation, and endoneural inflammatory infiltrates on nerve biopsies.

Associated with impaired sensation, absent or deceased tendon reflexes, an elevated cerebrospinal fluid protein level, demyelinating nerve conduction and signs of demyelination on nerve biopsy specimens.

CIDP can present as various subtypes, the most common being the classical symmetrical polyradiculoneuropathy and the next most common being a localized asymmetrical form, multifocal CIDP.

The course can be relapsing or chronic and progressive.

Diagnosis based on clinical and electrophysiological studies, with CSF examination and nerve biopsy needed to make definitive diagnosis.

Associated with electrical conduction blockdue to segmental demyelination of areas of inflammation as seen on nerve conduction studies.

Associated with high CSF protein.

Typically responds to corticosteroids, which may distinguish from other similar entities.

Idiopathic inflammation of nerves that can occur at any age with a subacute sensorimotor polyneuropathy.

Usually idiopathic but may occur as part of some connective tissue diseases.

Corticosteroids, intravenous gamma globulin and plasma exchange are all effective treatments.

Intravenous immunoglobulin is generally the first line therapy.

If the above agents are not effective immunosuppressive agents are considered.

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