- 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 "Guillain-Barré syndrome in adults: Clinical features and diagnosis"). These include:
●Acute inflammatory demyelinating polyradiculoneuropathy (AIDP)
●Acute motor axonal neuropathy (AMAN)
- So YT. Immune-mediated neuropathies. Continuum (Minneap Minn) 2012; 18:85.
- Lewis RA, Sumner AJ, Brown MJ, Asbury AK. Multifocal demyelinating neuropathy with persistent conduction block. Neurology 1982; 32:958.
- Katz JS, Saperstein DS. Asymmetric Acquired Demyelinating Polyneuropathies: MMN and MADSAM. Curr Treat Options Neurol 2001; 3:119.
- Saperstein DS, Amato AA, Wolfe GI, et al. Multifocal acquired demyelinating sensory and motor neuropathy: the Lewis-Sumner syndrome. Muscle Nerve 1999; 22:560.
- Lewis RA. Chronic inflammatory demyelinating polyneuropathy. Neurol Clin 2007; 25:71.
- Sinnreich M, Klein CJ, Daube JR, et al. Chronic immune sensory polyradiculopathy: a possibly treatable sensory ataxia. Neurology 2004; 63:1662.
- Trip SA, Saifee T, Honan W, et al. Chronic immune sensory polyradiculopathy with cranial and peripheral nerve involvement. J Neurol 2012; 259:1238.
- Finsterer J. Treatment of immune-mediated, dysimmune neuropathies. Acta Neurol Scand 2005; 112:115.
- Alpert JN. GALOP syndrome: case report with 7-year follow-up. South Med J 2004; 97:410.
- Gosselin S, Kyle RA, Dyck PJ. Neuropathy associated with monoclonal gammopathies of undetermined significance. Ann Neurol 1991; 30:54.
- Joint Task Force of the EFNS and the PNS. European Federation of Neurological Societies/Peripheral Nerve Society Guideline on management of paraproteinemic demyelinating neuropathies. Report of a Joint Task Force of the European Federation of Neurological Societies and the Peripheral Nerve Society--first revision. J Peripher Nerv Syst 2010; 15:185.
- Ritz MF, Erne B, Ferracin F, et al. Anti-MAG IgM penetration into myelinated fibers correlates with the extent of myelin widening. Muscle Nerve 1999; 22:1030.
- Kawagashira Y, Koike H, Tomita M, et al. Morphological progression of myelin abnormalities in IgM-monoclonal gammopathy of undetermined significance anti-myelin-associated glycoprotein neuropathy. J Neuropathol Exp Neurol 2010; 69:1143.
- Vital A, Vital C, Julien J, et al. Polyneuropathy associated with IgM monoclonal gammopathy. Immunological and pathological study in 31 patients. Acta Neuropathol 1989; 79:160.
- Shin SC, Robinson-Papp J. Amyloid neuropathies. Mt Sinai J Med 2012; 79:733.
- 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