- Dale Lange, MD
Dale Lange, MD
- Professor of Neurology
- Weill College of Medicine at Cornell University
- Jessica Robinson-Papp, MD
Jessica Robinson-Papp, MD
- Assistant Professor
- Icahn School of Medicine at Mount Sinai
INTRODUCTION AND DEFINITION
Polyneuropathy may be defined broadly as the dysfunction of many or all nerves. It is useful to classify polyneuropathies into two general categories based on pathophysiology: axonal and demyelinating. Axonal neuropathies cause symptoms and signs related to axon loss, while demyelinating neuropathies produce abnormalities based on the lack of appropriate interaction of Schwann cells with axons. The distinction between axonal and demyelinating polyneuropathies is clinically important because it guides the differential diagnosis, and ultimately the approach to treatment. Axonal neuropathies may be caused by a broad spectrum of systemic illnesses. The differential diagnosis of demyelinating neuropathies includes immune-mediated, toxic, and hereditary etiologies. In this article we specifically address the immune-mediated polyneuropathies, with emphasis on the demyelinating neuropathies.
Immune-mediated neuropathies can be divided into acute and chronic forms.
The acute immune-mediated polyneuropathies are classified under the eponym Guillain-Barré syndrome (GBS), a heterogeneous condition with several variant forms (see "Clinical features and diagnosis of Guillain-Barré syndrome in adults"). These include:
●Acute inflammatory demyelinating polyradiculoneuropathy (AIDP)
●Acute motor axonal neuropathy (AMAN)
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- INTRODUCTION AND DEFINITION
- GUILLAIN-BARRÉ SYNDROME
- CHRONIC INFLAMMATORY DEMYELINATING POLYNEUROPATHY
- CIDP VARIANTS
- Multifocal acquired demyelinating sensory and motor neuropathy
- Chronic immune sensory polyradiculopathy
- Sensory predominant CIDP
- Distal acquired demyelinating symmetric neuropathy
- Gait ataxia with late onset polyneuropathy
- Multifocal motor neuropathy
- SYSTEMIC DISORDERS ASSOCIATED WITH IMMUNE-MEDIATED NEUROPATHIES
- Monoclonal gammopathy of undetermined significance
- - POEMS syndrome
- Neuropathy with IgM gammopathy
- - Waldenström macroglobulinemia
- - Mixed cryoglobulinemia
- Primary amyloidosis
- Other paraneoplastic neuropathies
- Neuropathy with systemic autoimmune disease
- Neuropathy with other systemic diseases