Inflammatory polyneuropathy

Inflammatory polyneuropathy covers a range of syndromes, both acute and chronic. Guillain-Barre syndrome is the best-known acute form, while chronic inflammatory demyelinating polyradiculoneuropathy is the classic chronic variant.

Clinical and experimental findings suggest that humoral factors, such as anti-peripheral nerve antibodies and cytokines, may be implicated in the immunopathogenesis of Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Inflammatory substances generated include interleukin-6; increased IL-6 release is associated with autoantibody production in a number of immune-mediated and neoplastic disorders.

Chronic

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is the best-known variant of this uncommon disease. It has several variants:

• predominantly sensory symptoms
• distal symmetric disorder (DADS)
• multifocal sensorimotor neuropathy or sensorimotor mononeuropathy multiplex with prominent conduction block (also known as Lewis-Sumner neuropathy)
• CIDP with associated CNS demyelination or with other systemic disorders

It is differentiated from:

• demyelinating neuropathies associated with immunoglobulin M (IgM) paraproteins, including those with anti–myelin-associated glycoprotein (MAG) antibodies
• POEMS syndrome of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes
• multifocal motor neuropathy

A slowly progressive autoimmune demyelinating disease of peripheral nerves and nerve roots. Clinical manifestations include weakness and sensory loss in the extremities and enlargement of peripheral nerves. The course may be relapsing-remitting or demonstrate a step-wise progression. Protein is usually elevated in the spinal fluid and cranial nerves are typically spared. Guillain-Barre syndrome features a relatively rapid progression of disease which distinguishes it from this condition.

Intravenous immune globulin (IVIG) has been reported to improve quality of life. Patient response to IVIG, however, is influenced by a single nucleotide polymorphism in the transient axonal glycoprotein 1 gene (TAG-1)

Guillain-Barre syndrome

An acute inflammatory autoimmune neuritis caused by T cell- mediated cellular immune response directed towards peripheral myelin. Demyelination occurs in peripheral nerves and nerve roots. The process is often preceded by a viral or bacterial infection, surgery, immunization, lymphoma, or exposure to toxins. Common clinical manifestations include progressive weakness, loss of sensation, and loss of deep tendon reflexes. Weakness of respiratory muscles and autonomic dysfunction may occur.