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The Importance of Rare Subtypes in Diagnosis and Treatment of Peripheral Neuropathy A Review

Brian C. Callaghan, MD, MS1; Raymond S. Price, MD2; Kevin S. Chen, MD3; Eva L. Feldman, MD, PhD1
[+] Author Affiliations
1Department of Neurology, University of Michigan, Ann Arbor
2Department of Neurology, University of Pennsylvania, Philadelphia
3Department of Neurosurgery, University of Michigan, Ann Arbor
JAMA Neurol. 2015;72(12):1510-1518. doi:10.1001/jamaneurol.2015.2347.
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Importance  Peripheral neuropathy is a prevalent condition that usually warrants a thorough history and examination but has limited diagnostic evaluation. However, rare localizations of peripheral neuropathy often require more extensive diagnostic testing and different treatments.

Objective  To describe rare localizations of peripheral neuropathy, including the appropriate diagnostic evaluation and available treatments.

Evidence Review  References were identified from PubMed searches conducted on May 29, 2015, with an emphasis on systematic reviews and randomized clinical trials. Articles were also identified through the use of the authors’ own files. Search terms included common rare neuropathy localizations and their causes, as well as epidemiology, pathophysiology, diagnosis, and treatment.

Findings  Diffuse, nonlength-dependent neuropathies, multiple mononeuropathies, polyradiculopathies, plexopathies, and radiculoplexus neuropathies are rare peripheral neuropathy localizations that often require extensive diagnostic testing. Atypical neuropathy features, such as acute/subacute onset, asymmetry, and/or motor predominant signs, are frequently present. The most common diffuse, nonlength-dependent neuropathies are Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuropathy, multifocal motor neuropathy, and amyotrophic lateral sclerosis. Effective disease-modifying therapies exist for many diffuse, nonlength-dependent neuropathies including Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuropathy, multifocal motor neuropathy, and some paraprotein-associated demyelinating neuropathies. Vasculitic neuropathy (multiple mononeuropathy) also has efficacious treatment options, but definitive evidence of a treatment effect for IgM anti-MAG neuropathy and diabetic amyotrophy (radiculoplexus neuropathy) is lacking.

Conclusions and Relevance  Recognition of rare localizations of peripheral neuropathy is essential given the implications for diagnostic testing and treatment. Electrodiagnostic studies are an important early step in the diagnostic evaluation and provide information on the localization and pathophysiology of nerve injury.

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Figure 1.
Pathophysiology of Peripheral Neuropathy

Nearly every category of disease can manifest within the peripheral nervous system. It is often useful to subdivide the peripheral nervous system into its microanatomical components: neuronal cell bodies within the dorsal root ganglion and spinal cord, interstitial tissues (epineurium, perineurium, and endoneurium), associated vasa nervorum, Schwann cells/myelin sheaths, and axons. Different diseases tend to affect different subsystems of the peripheral nerve, and careful pathologic study along with clinical history may help narrow the differential diagnosis in complex cases.

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Figure 2.
Diagnostic Algorithm for the Evaluation of Atypical Neuropathy

Atypical neuropathy features include nonlength-dependent distribution, acute/subacute onset, asymmetry, and/or motor predominant signs. Electrodiagnostic testing is the first step in further categorizing the peripheral neuropathy subtype and determines further diagnostic evaluation. Abs indicates antibodies; AMA, antimitochondrial antibody; ANCA, antineutrophil cytoplasmic antibody; ANA, antinuclear antibody; ASMA, antismooth muscle; BJS, Bence-Jones proteins; CBC, complete blood count; COMP, comprehensive metabolic panel; CRP, C-reactive protein; CXR, chest x-ray; EMG, electromyogram; ESR, erythrocyte sedimentation rate; FLC, serum free light chain; GM, GM1 ganglioside antibody; HIV, human immunodeficiency virus; HTLV, human T-lymphotrophic virus; IF, immunofixation; MRI, magnetic resonance imaging; NCS, nerve conduction study; RF, rheumatoid factor; SPEP, serum protein electrophoresis; SSA, Sjögren syndrome antigen A; UA, urinalysis; UPEP, urine protein electrophoresis; VEGF, vascular endothelial growth factor; WNV, West Nile virus.

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