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Correspondence |

Forme Fruste Manifestations of Chronic Inflammatory Demyelinating Polyradiculoneuropathy

Praful Kelkar, MD
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Copyright 2004 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.

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Arch Neurol. 2004;61(6):984-984. doi:10.1001/archneur.61.6.984-a
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I read the article by Alaedini et al1 with interest. This article reminds the reader of the difficulties in diagnosing chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), especially in those with an atypical or forme fruste manifestation.

The questions of how to document primary demyelination and how extensive these changes should be for it to be considered CIDP have not been resolved. In these 12 patients, it seems that 9 had significantly prolonged or absent F waves, and 3 had conduction block. Thus, 11 of the 12 patients had some features that suggested primary demyelination. American Academy of Neurology ad hoc committee criteria or other criteria can be used to diagnose only about 60% of patients with CIDP.

The finding of modestly elevated IgG antibody levels against gangliosides in these patients is interesting; however, it appears that the other 13 patients without these antibodies had similar clinical and electrophysiological features. Are these antibodies present in patients with "typical" CIDP?

Perhaps multifocal motor neuropathy and multifocal, acquired, demyelinating sensory and motor neuropathy are distinct entities, or maybe not. Rather than adding another eponym to the growing list, as suggested elsewhere by the authors,2 these patients can be considered to represent partial or forme fruste manifestations of CIDP.

The take-home message seems to be that if any electrophysiological finding suggests primary demyelination, even in 1 nerve, one should think of CIDP despite the presence of atypical features. Finally, only 2 of these 12 patients underwent biopsies. Patients with axonal mononeuritis multiplex due to vasculitis or inflammatory vasculopathy typically have multifocal axon loss on biopsy specimens and no features of primary demyelination, although they would also respond to immune therapies.

REFERENCES

Alaedini  A, Sander  HW, Hays  AP, Latov  N. Antiganglioside antibodies in multifocal acquired sensory and motor neuropathy. Arch Neurol. 2003;;60:42-- 46. http://www.ncbi.nlm.nih.gov/htbin-post/Entrez/query?db=m&form=6&Dopt=r&uid=entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=12533087&dopt=Abstract
Sander  HW, Latov  N. Research criteria for defining patients with CIDP. Neurology. 2003;;60(suppl 3, pt 8):S8-- S15. http://www.ncbi.nlm.nih.gov/htbin-post/Entrez/query?db=m&form=6&Dopt=r&uid=entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=12707417&dopt=Abstract

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Alaedini  A, Sander  HW, Hays  AP, Latov  N. Antiganglioside antibodies in multifocal acquired sensory and motor neuropathy. Arch Neurol. 2003;;60:42-- 46. http://www.ncbi.nlm.nih.gov/htbin-post/Entrez/query?db=m&form=6&Dopt=r&uid=entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=12533087&dopt=Abstract
Sander  HW, Latov  N. Research criteria for defining patients with CIDP. Neurology. 2003;;60(suppl 3, pt 8):S8-- S15. http://www.ncbi.nlm.nih.gov/htbin-post/Entrez/query?db=m&form=6&Dopt=r&uid=entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=12707417&dopt=Abstract

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