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Autoimmune neuropathies can include Guillain-Barré syndrome (GBS), chronic inflammatory demyelinating polyneuropathy (CIDP), multifocal motor neuropathy (MMN), and IgM paraproteinemic neuropathy, among others. They present with a broad range of symptoms, including slow or insidious onset and progression, asymmetric or multifocal nerve deficits, and selective involvement of motor, sensory, or autonomic nerves. The overlap of symptoms among syndromes often makes the diagnosis difficult. Presumptive diagnosis is based on the patient’s history and clinical signs and findings on physical exam. Initial laboratory testing aims to rule out underlying causes such as infections, metabolic disturbances, and brain tumors. If nothing is uncovered, further evaluation, including advanced imaging, nerve conduction studies, and cerebrospinal fluid (CSF) analysis may be required.
Autoimmune neuropathies can also arise as a condition associated with cancer (paraneoplastic). The detection of an autoantibody in the right clinical setting can provide evidence that the peripheral nerve disturbance is immune mediated and may direct treatment, but autoantibody testing cannot be used as the sole diagnostic tool. Anti-myelin-associated glycoprotein (anti-MAG) antibodies are often present in patients with predominant sensory symptoms, and anti-GM1 antibodies are present in patients with predominant motor nerve symptoms.
Guillain-Barré syndrome
CIDP (*Often confused with GBS, but symptoms continue for longer)
MMN
Guillain–Barré syndrome (GBS) is a rare disorder in which the body’s immune system attacks the nerves. Weakness and tingling in the arms and legs are usually the first symptoms. These sensations can quickly spread, eventually paralyzing the whole body. The exact cause of Guillain-Barré syndrome is unknown, however it is often preceded by an infectious illness such as a respiratory infection or influenza.
GBS occurs in several forms:
Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) is the most common form in North America. The most common sign of AIDP is muscle weakness that starts in the lower part of the body and spreads upward.
Miller Fisher syndrome (MFS), in which paralysis starts in the eyes. MFS is also associated with unsteady gait. MFS occurs in about 5 percent of people with GBS and is more common in Asia.
Acute motor axonal neuropathy (AMAN) and acute motor-sensory axonal neuropathy (AMSAN) are less common in the North America are more frequent in China, Japan and Mexico.