CMT and central nervous system

[Guest guest]

Hi Larry,

 

I thought you might find this article interesting.  It discusses the

similiarities between Multiple Sclerosis and CMT.  It points out that there are

cases where CMT has affected the central nervous system and there are cases

where MS has affected the peripheral system. 

 

(Note - In other research, I found that there are certain CMT subtypes which are

more likely than others to have central nervous system involvement, i.e. CMT1X.)

 

Anyway...here's the article discussing similiarities of CMT and MS:

 

http://www.ncbi.nlm.nih.gov/pubmed/15530551?itool=EntrezSystem2.PEntrez.Pubmed.P\

ubmed_ResultsPanel.Pubmed_RVDocSum & ordinalpos=18

 

Ultrastructural and immunohistochemical similarities of two distinct entities;

multiple sclerosis and hereditary motor sensory neuropathy.

Acar G, Tanriover G, Demir N, Kayisli UA, Sati GL, Yaba A, Idiman E, Demir R.

Department of Neurology, Medical School, Dokuz Eylül University, Izmir, Turkey.

In the present study, we present the ultrastructural and immunohistochemical

properties of the sural nerves of two patients, one of whom was diagnosed as

having multiple sclerosis with involvement of the peripheral nervous system

(PNS), and the other as having hereditary motor sensory neuropathy type-I with

involvement of the central nervous system (CNS). Expression of several

extracellular matrix (ECM) proteins (fibronectin, laminin, and collagen

type-IV), intermediate filaments (vimentin) and S-100 protein (marker for the

axon-Schwann cell interface) was investigated by means of immunohistochemical

methods. In addition, the tissue samples were evaluated ultrastructurally.

Immunohistochemical staining revealed increased expression of the ECM molecules

mentioned above in relation with the sural nerves of the patients. We

hypothesize that this enhanced expression is due to Schwann cell-axon

interactions. Vimentin expression was different in Schwann cells

and S-100 immunostaining was decreased near the Schwann cell-axon interface.

Myelin fragmentation, axon vacuolization, onion bulbs, tomoculous formation,

axonal degeneration were found to occur. These results suggest that there is

active ECM reorganization in the sural nerve of these patients, and some

ultrastructural changes are similar in the damaged axonal organization and in

Schwann cells although the changes are not completely the same in the two

patients. In conclusion, our study demonstrates that there is an association

between the demyelinization process in the CNS and the PNS even though they are

affected by different mechanisms.