The spectrum of chronic inflammatory demyelinating polyneuropathy

Francisco T. Rotta, Alyssa T. Sussman, Walter G Bradley, D. Ram Ayyar, Khema R Sharma, Robert T. Shebert

Research output: Contribution to journalArticle

170 Citations (Scopus)

Abstract

Research criteria for the diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) were proposed by an Ad Hoc Subcommittee of the American Academy of Neurology (AAN) in 1991, and since then these criteria have been widely used in clinical studies. We have been impressed by the frequent finding of electrophysiological changes of a demyelinating neuropathy in patients whose clinical presentation does not conform to the usually accepted clinical phenotype of CIDP. To determine the clinical spectrum of CIDP, we conducted a retrospective review of patients of the peripheral electrophysiology laboratory of the University of Miami-Jackson Memorial Medical Center. Diagnostic criteria for acquired demyelination of an individual nerve were adapted from the AAN research criteria for the diagnosis of CIDP (1991). Patients were accepted for inclusion when such evidence was demonstrated in at least one motor nerve or at least two sensory nerves. We then reviewed the clinical phenotype and the underlying etiology of the neuropathy in these cases. Eighty-seven patients, 63 male and 24 female, age of onset 4-84 (mean 49.3) years, met these inclusion criteria. Forty-seven patients (54%) had distinct features outside the usual clinical presentation of CIDP. Of these, 15 (17%) had predominantly distal features, 13 (15%) had exclusively sensory polyneuropathy; seven (8%) had markedly asymmetric disease, seven (8%) had associated CNS demyelination, four (5%) had predominant cranial nerve involvement, and one (1%) had only the restless legs syndrome. An associated medical condition that may have been responsible for the acquired demyelinating neuropathy was present in 60% of the patients. We conclude that spectrum of CIDP is broader than would be indicated by the strict application of the AAN research criteria, and that many of the cases meeting more liberal criteria frequently respond to immunosuppressive therapy. (C) 2000 Elsevier Science B.V.

Original languageEnglish
Pages (from-to)129-139
Number of pages11
JournalJournal of the Neurological Sciences
Volume173
Issue number2
DOIs
StatePublished - Feb 15 2000

Fingerprint

Chronic Inflammatory Demyelinating Polyradiculoneuropathy
Demyelinating Diseases
Neurology
Research
Phenotype
Restless Legs Syndrome
Polyneuropathies
Cranial Nerves
Electrophysiology
Immunosuppressive Agents
Age of Onset

Keywords

  • Demyelination
  • Electrophysiology
  • Immunosuppressive treatment
  • Polyneuropathy

ASJC Scopus subject areas

  • Aging
  • Clinical Neurology
  • Surgery
  • Developmental Neuroscience
  • Neurology
  • Neuroscience(all)

Cite this

The spectrum of chronic inflammatory demyelinating polyneuropathy. / Rotta, Francisco T.; Sussman, Alyssa T.; Bradley, Walter G; Ram Ayyar, D.; Sharma, Khema R; Shebert, Robert T.

In: Journal of the Neurological Sciences, Vol. 173, No. 2, 15.02.2000, p. 129-139.

Research output: Contribution to journalArticle

Rotta, Francisco T. ; Sussman, Alyssa T. ; Bradley, Walter G ; Ram Ayyar, D. ; Sharma, Khema R ; Shebert, Robert T. / The spectrum of chronic inflammatory demyelinating polyneuropathy. In: Journal of the Neurological Sciences. 2000 ; Vol. 173, No. 2. pp. 129-139.
@article{a53a8f327af645228aced6ec79d9babc,
title = "The spectrum of chronic inflammatory demyelinating polyneuropathy",
abstract = "Research criteria for the diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) were proposed by an Ad Hoc Subcommittee of the American Academy of Neurology (AAN) in 1991, and since then these criteria have been widely used in clinical studies. We have been impressed by the frequent finding of electrophysiological changes of a demyelinating neuropathy in patients whose clinical presentation does not conform to the usually accepted clinical phenotype of CIDP. To determine the clinical spectrum of CIDP, we conducted a retrospective review of patients of the peripheral electrophysiology laboratory of the University of Miami-Jackson Memorial Medical Center. Diagnostic criteria for acquired demyelination of an individual nerve were adapted from the AAN research criteria for the diagnosis of CIDP (1991). Patients were accepted for inclusion when such evidence was demonstrated in at least one motor nerve or at least two sensory nerves. We then reviewed the clinical phenotype and the underlying etiology of the neuropathy in these cases. Eighty-seven patients, 63 male and 24 female, age of onset 4-84 (mean 49.3) years, met these inclusion criteria. Forty-seven patients (54{\%}) had distinct features outside the usual clinical presentation of CIDP. Of these, 15 (17{\%}) had predominantly distal features, 13 (15{\%}) had exclusively sensory polyneuropathy; seven (8{\%}) had markedly asymmetric disease, seven (8{\%}) had associated CNS demyelination, four (5{\%}) had predominant cranial nerve involvement, and one (1{\%}) had only the restless legs syndrome. An associated medical condition that may have been responsible for the acquired demyelinating neuropathy was present in 60{\%} of the patients. We conclude that spectrum of CIDP is broader than would be indicated by the strict application of the AAN research criteria, and that many of the cases meeting more liberal criteria frequently respond to immunosuppressive therapy. (C) 2000 Elsevier Science B.V.",
keywords = "Demyelination, Electrophysiology, Immunosuppressive treatment, Polyneuropathy",
author = "Rotta, {Francisco T.} and Sussman, {Alyssa T.} and Bradley, {Walter G} and {Ram Ayyar}, D. and Sharma, {Khema R} and Shebert, {Robert T.}",
year = "2000",
month = "2",
day = "15",
doi = "10.1016/S0022-510X(99)00317-2",
language = "English",
volume = "173",
pages = "129--139",
journal = "Journal of the Neurological Sciences",
issn = "0022-510X",
publisher = "Elsevier",
number = "2",

}

TY - JOUR

T1 - The spectrum of chronic inflammatory demyelinating polyneuropathy

AU - Rotta, Francisco T.

AU - Sussman, Alyssa T.

AU - Bradley, Walter G

AU - Ram Ayyar, D.

AU - Sharma, Khema R

AU - Shebert, Robert T.

PY - 2000/2/15

Y1 - 2000/2/15

N2 - Research criteria for the diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) were proposed by an Ad Hoc Subcommittee of the American Academy of Neurology (AAN) in 1991, and since then these criteria have been widely used in clinical studies. We have been impressed by the frequent finding of electrophysiological changes of a demyelinating neuropathy in patients whose clinical presentation does not conform to the usually accepted clinical phenotype of CIDP. To determine the clinical spectrum of CIDP, we conducted a retrospective review of patients of the peripheral electrophysiology laboratory of the University of Miami-Jackson Memorial Medical Center. Diagnostic criteria for acquired demyelination of an individual nerve were adapted from the AAN research criteria for the diagnosis of CIDP (1991). Patients were accepted for inclusion when such evidence was demonstrated in at least one motor nerve or at least two sensory nerves. We then reviewed the clinical phenotype and the underlying etiology of the neuropathy in these cases. Eighty-seven patients, 63 male and 24 female, age of onset 4-84 (mean 49.3) years, met these inclusion criteria. Forty-seven patients (54%) had distinct features outside the usual clinical presentation of CIDP. Of these, 15 (17%) had predominantly distal features, 13 (15%) had exclusively sensory polyneuropathy; seven (8%) had markedly asymmetric disease, seven (8%) had associated CNS demyelination, four (5%) had predominant cranial nerve involvement, and one (1%) had only the restless legs syndrome. An associated medical condition that may have been responsible for the acquired demyelinating neuropathy was present in 60% of the patients. We conclude that spectrum of CIDP is broader than would be indicated by the strict application of the AAN research criteria, and that many of the cases meeting more liberal criteria frequently respond to immunosuppressive therapy. (C) 2000 Elsevier Science B.V.

AB - Research criteria for the diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) were proposed by an Ad Hoc Subcommittee of the American Academy of Neurology (AAN) in 1991, and since then these criteria have been widely used in clinical studies. We have been impressed by the frequent finding of electrophysiological changes of a demyelinating neuropathy in patients whose clinical presentation does not conform to the usually accepted clinical phenotype of CIDP. To determine the clinical spectrum of CIDP, we conducted a retrospective review of patients of the peripheral electrophysiology laboratory of the University of Miami-Jackson Memorial Medical Center. Diagnostic criteria for acquired demyelination of an individual nerve were adapted from the AAN research criteria for the diagnosis of CIDP (1991). Patients were accepted for inclusion when such evidence was demonstrated in at least one motor nerve or at least two sensory nerves. We then reviewed the clinical phenotype and the underlying etiology of the neuropathy in these cases. Eighty-seven patients, 63 male and 24 female, age of onset 4-84 (mean 49.3) years, met these inclusion criteria. Forty-seven patients (54%) had distinct features outside the usual clinical presentation of CIDP. Of these, 15 (17%) had predominantly distal features, 13 (15%) had exclusively sensory polyneuropathy; seven (8%) had markedly asymmetric disease, seven (8%) had associated CNS demyelination, four (5%) had predominant cranial nerve involvement, and one (1%) had only the restless legs syndrome. An associated medical condition that may have been responsible for the acquired demyelinating neuropathy was present in 60% of the patients. We conclude that spectrum of CIDP is broader than would be indicated by the strict application of the AAN research criteria, and that many of the cases meeting more liberal criteria frequently respond to immunosuppressive therapy. (C) 2000 Elsevier Science B.V.

KW - Demyelination

KW - Electrophysiology

KW - Immunosuppressive treatment

KW - Polyneuropathy

UR - http://www.scopus.com/inward/record.url?scp=0034651624&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0034651624&partnerID=8YFLogxK

U2 - 10.1016/S0022-510X(99)00317-2

DO - 10.1016/S0022-510X(99)00317-2

M3 - Article

VL - 173

SP - 129

EP - 139

JO - Journal of the Neurological Sciences

JF - Journal of the Neurological Sciences

SN - 0022-510X

IS - 2

ER -