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Chronic dysimmune neuropathy. A subclassification based upon the clinical features of 102 patients.
J Neurol. 2003 Jun; 250(6):714-24.JN

Abstract

The Chronic Dysimmune neuropathies (CDN) are a clinically heterogeneous group of polyneuropathies united by their presumed immune mediated aetiology. At present such neuropathies are classified as Chronic Inflammatory Demyelinating Polyneuropathy (CIDP), Multifocal Motor Neuropathy (MMN) and the Neuropathies in association with serum Paraproteins (Paraproteinaemic Neuropathies). This classification fails to recognise other distinctive syndromes and is limited by heterogeneity within, and overlap between, subgroups. We have refined this clinical subclassification by a review of a consecutive series of 102 unselected patients with CDN referred to a single neurologist. We recognise 6 clinical subtypes of CDN: one sensory ataxic group; three motor-sensory subgroups (chronic motor sensory demyelinating neuropathy, subacute motor sensory demyelinating neuropathy and a multifocal motor sensory neuropathy); and two pure motor subgroups (symmetric pure motor demyelinating neuropathy and multifocal motor neuropathy). This subclassification allows distinct syndromes to be recognised and helps resolve problems of heterogeneity and overlap. Distinction between these subgroups is of immediate practical relevance to patient management. Although steroids are beneficial for most of the subgroups, this is not so for both of the pure motor syndromes which should be treated with intravenous immunoglobulin. Patients with chronic development of Motor Sensory Demyelinating Neuropathy respond less well to steroids than those with a subacute onset. An association was found between elderly patients with Subacute Motor Sensory Demyelinating Neuropathy and carcinomas. Within any clinical subgroup patients behave similarly regardless of the presence of associated paraproteins or nerve specific antibodies.

Authors+Show Affiliations

Department of Clinical Neurology, Radcliffe Infirmary, Oxford OX2 6HE, UK.No affiliation info available

Pub Type(s)

Comparative Study
Journal Article

Language

eng

PubMed ID

12796835

Citation

Busby, Mark, and Michael Donaghy. "Chronic Dysimmune Neuropathy. a Subclassification Based Upon the Clinical Features of 102 Patients." Journal of Neurology, vol. 250, no. 6, 2003, pp. 714-24.
Busby M, Donaghy M. Chronic dysimmune neuropathy. A subclassification based upon the clinical features of 102 patients. J Neurol. 2003;250(6):714-24.
Busby, M., & Donaghy, M. (2003). Chronic dysimmune neuropathy. A subclassification based upon the clinical features of 102 patients. Journal of Neurology, 250(6), 714-24.
Busby M, Donaghy M. Chronic Dysimmune Neuropathy. a Subclassification Based Upon the Clinical Features of 102 Patients. J Neurol. 2003;250(6):714-24. PubMed PMID: 12796835.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Chronic dysimmune neuropathy. A subclassification based upon the clinical features of 102 patients. AU - Busby,Mark, AU - Donaghy,Michael, PY - 2003/6/11/pubmed PY - 2003/8/28/medline PY - 2003/6/11/entrez SP - 714 EP - 24 JF - Journal of neurology JO - J. Neurol. VL - 250 IS - 6 N2 - The Chronic Dysimmune neuropathies (CDN) are a clinically heterogeneous group of polyneuropathies united by their presumed immune mediated aetiology. At present such neuropathies are classified as Chronic Inflammatory Demyelinating Polyneuropathy (CIDP), Multifocal Motor Neuropathy (MMN) and the Neuropathies in association with serum Paraproteins (Paraproteinaemic Neuropathies). This classification fails to recognise other distinctive syndromes and is limited by heterogeneity within, and overlap between, subgroups. We have refined this clinical subclassification by a review of a consecutive series of 102 unselected patients with CDN referred to a single neurologist. We recognise 6 clinical subtypes of CDN: one sensory ataxic group; three motor-sensory subgroups (chronic motor sensory demyelinating neuropathy, subacute motor sensory demyelinating neuropathy and a multifocal motor sensory neuropathy); and two pure motor subgroups (symmetric pure motor demyelinating neuropathy and multifocal motor neuropathy). This subclassification allows distinct syndromes to be recognised and helps resolve problems of heterogeneity and overlap. Distinction between these subgroups is of immediate practical relevance to patient management. Although steroids are beneficial for most of the subgroups, this is not so for both of the pure motor syndromes which should be treated with intravenous immunoglobulin. Patients with chronic development of Motor Sensory Demyelinating Neuropathy respond less well to steroids than those with a subacute onset. An association was found between elderly patients with Subacute Motor Sensory Demyelinating Neuropathy and carcinomas. Within any clinical subgroup patients behave similarly regardless of the presence of associated paraproteins or nerve specific antibodies. SN - 0340-5354 UR - https://www.unboundmedicine.com/medline/citation/12796835/Chronic_dysimmune_neuropathy__A_subclassification_based_upon_the_clinical_features_of_102_patients_ L2 - https://dx.doi.org/10.1007/s00415-003-1068-2 DB - PRIME DP - Unbound Medicine ER -