CIDPUSA.org Autoimmune diseases

Histologically, the nerves in cases with CIDP show segmental demyelination and remyelination and thinning of myelin sheaths, and the nerves often have an onion bulb appearance, features present in many of the chronic polyneuropathies. Mononuclear cell infiltrates may be present in the endoneurium. There may be a preferential involvement of the motor nerves, which may account for negative sural nerve biopsies in some patients.

Most cases of CIDP are idiopathic, but it can be associated with a variety of disorders including diabetes, HIV infection, celiac disease (gluten-sensitive enteropathy), melanoma, Sjogren's syndrome, lymphoma, IgM gammopathy (polyclonal or monoclonal), or with hepatitis. Patients with chronic polyneuropathies often have elevated antiganglioside and/or antisulfatide IgM antibodies. The peripheral nerves are most commonly involved in patients with CIDP. Biopsy of the sural nerve may help confirm the diagnosis. In the spine, CIDP affects the cauda equina and lumbar nerve roots more commonly than the cervical nerve roots. The thoracic nerve roots and cranial nerves may also be affected but are less commonly involved than in AIDP

Patients with CIDP classically present with a history of a chronic, progressive, or relapsing disorder with muscle weakness and often sensory loss. Patients with sensory neuropathy have predominant involvement of large-fiber nerves. This is usually a polyradicular condition with symmetric or asymmetric involvement of proximal and distal muscles in the lower extremity. Neurogenic pain is not a common initial manifestation of CIDP, but pain may occur as the disease evolves. Rarely, neurogenic pain is the initial manifestation of CIDP. Neuropathic pain is very common in patients with small-fiber peripheral neuropathies, such as patients with diabetic neuropathy or amyloidosis-related neuropathy. Patients may present with symptoms similar to multiple sclerosis.

Classically, GBS symptoms progress within the first few weeks and are maximal at 4 weeks from onset of the disease, and thereafter the disorder is usually monophasic. In CIDP, symptoms usually progress for at least 2 months, and thereafter the disorder may be monophasic, relapsing-remitting, or steadily progressive. In practice, however, it may be difficult clinically to differentiate CIDP from progressive GBS, especially in the early stages of the disease. The major electromyographic (EMG) finding in CIDP is slow nerve conduction similar to that seen with demyelinating disorders in general. A more detailed summary of the EMG findings of CIDP can be found elsewhere. Patients with mild cases of CIDP tend to recover with complete or near complete regaining of function. Severe cases have a chronic, progressive, relapsing-remitting illness. Rarely, CIDP can be fatal.

Sensory ganglioneuropathies , which involve the dorsal root ganglia, represent a subgroup of polyneuropathies that may occur with paraneoplastic subacute sensory neuronopathy (SSN), Sjogren's syndrome, Miller-Fisher syndrome, Bickerstaff's brainstem encephalitis, inherited ganglioneuropathies (Friedreich's ataxia and other inherited cerebellar ataxias), and with certain drugs (pyridoxine, vincristine, taxane, and cisplatin). There is a predisposition to developing chemotherapy-induced neuropathy in patients who are diabetic, chronic alcoholics, and in patients with inherited neuropathies.

Paraneoplastic neuropathy (PPN) is rarely encountered in routine practice but may produce clinical manifestations and an MR imaging appearance identical to GBS or CIDP. Indeed, PPN may be considered to be a subtype of CIDP. There are other paraneoplastic syndromes that affect the nervous system, including paraneoplastic encephalomyelitis, paraneoplastic cerebellar degeneration, cancer-associated retinopathy, and paraneoplastic opsoclonus-myoclonus-ataxia syndrome. Paraneoplastic neuropathy commonly involves the dorsal root ganglia, but the spinal cauda equina, nerve roots, and peripheral nerves may be involved as well 

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