Document Detail

Crow-Fukase (POEMS) syndrome: a study of peripheral nerve biopsy in five new cases.
MedLine Citation:
PMID:  12904234     Owner:  NLM     Status:  MEDLINE    
The pathogenesis of Crow-Fukase (POEMS) syndrome is not well known, and in some cases, a definite diagnosis is difficult to establish. Nerve fibers have been studied in about 120 peripheral nerve biopsies (PNBs), and a mixture of axonal and demyelinating lesions were found in most of them. We report five new cases of Crow-Fukase (POEMS) syndrome with ultrastructural examination of their PNBs. In every case, there were features of axonal degeneration and primary demyelination. Interestingly, uncompacted myelin lamellae (UMLs) were present in every case at a percentage of 1-7. The association of UML and Crow-Fukase (POEMS) syndrome was described 20 years ago but was only reported in a few studies and found in 31 of 41 cases. In fact, this association is very significant because apart from Crow-Fukase (POEMS) syndrome, UMLs can only be found with such a frequency in rare cases of Charcot-Marie-Tooth disease type 1B. UML was also reported in acute and chronic inflammatory demyelinating polyneuropathies but at a much lower percentage. Moreover, in our five cases, UML was frequently associated with a decrease in the number of intra-axonal filaments, and this finding raises the problem of relationships between myelin formation and neurofilaments. So far, glomeruloid hemangiomas present in the dermis of some patients are considered as the only specific criteria of Crow-Fukase (POEMS) syndrome, but we think UML can also be regarded as highly suggestive of this entity on condition that a thorough ultrastructural examination of a PNB is performed.
Claude Vital; Anne Vital; Xavier Ferrer; Jean-François Viallard; Jean-Luc Pellegrin; Sandrine Bouillot; Jean-Marc Larrieu; Laurence Lequen; Jean-Louis Larrieu; Christiane Brechenmacher; Klaus G Petry; Alain Lagueny
Related Documents :
8972154 - Alport's syndrome--a case report.
8379034 - Extraordinary urinary frequency syndrome.
19562934 - Concurrent use of cyclophosphamide and prednisolone in childhood nephrotic syndrome in ...
537034 - The börjeson-forssman-lehmann syndrome: a new case.
7540874 - Presumed central nervous system whipple's disease in a child: case report.
6099034 - Synchronous hodgkin's disease in monozygotic twins.
Publication Detail:
Type:  Case Reports; In Vitro; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Journal of the peripheral nervous system : JPNS     Volume:  8     ISSN:  1085-9489     ISO Abbreviation:  J. Peripher. Nerv. Syst.     Publication Date:  2003 Sep 
Date Detail:
Created Date:  2003-08-07     Completed Date:  2003-10-15     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  9704532     Medline TA:  J Peripher Nerv Syst     Country:  United States    
Other Details:
Languages:  eng     Pagination:  136-44     Citation Subset:  IM    
Neuropathology Department, Victor Segalen University, Bordeaux, France.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Antigens, CD / metabolism
Antigens, CD20 / metabolism
Antigens, CD45 / metabolism
Antigens, Differentiation, Myelomonocytic / metabolism
Axons / ultrastructure
Biopsy / methods
Demyelinating Diseases / metabolism,  pathology
Immunohistochemistry / methods
Lymphocytes / metabolism
Microscopy, Electron / instrumentation,  methods
Middle Aged
Myelin Sheath / metabolism,  ultrastructure
POEMS Syndrome / cerebrospinal fluid,  immunology,  pathology*
Peripheral Nerves / immunology,  ultrastructure*
Reg. No./Substance:
0/Antigens, CD; 0/Antigens, CD20; 0/Antigens, Differentiation, Myelomonocytic; 0/CD68 antigen, human; EC, CD45

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

Previous Document:  A study of the yeast cell wall composition and structure in response to growth conditions and mode o...
Next Document:  Comparative dose-dependence study of FK506 on transected mouse sciatic nerve repaired by allograft o...