Bibliographic Details
| Title: |
Neurofascin (NF)155- and NF186-Specific T Cell Response in a Patient Developing a Central Pontocerebellar Demyelination after 10 Years of CIDP |
| Authors: |
Juliane Klehmet, Max Staudt, Jan-Markus Diederich, Eberhard Siebert, Edgar Meinl, Lutz Harms, Andreas Meisel |
| Source: |
Frontiers in Neurology, Vol 8 (2017) |
| Publisher Information: |
Frontiers Media S.A., 2017. |
| Publication Year: |
2017 |
| Collection: |
LCC:Neurology. Diseases of the nervous system |
| Subject Terms: |
CIDP, CCPD, neurofascin, neurofascin 155, atypical, Neurology. Diseases of the nervous system, RC346-429 |
| More Details: |
BackgroundInformation and pathobiological understanding about central demyelinating manifestation in patients, who primarily suffer from chronic inflammatory demyelinating polyneuropathy (CIDP), are scarce.MethodsIFN-γ-response as well as antibodies against the (para)nodal antigens neurofascin (NF)155 and NF 186 had been tested by Elispot assay and ELISA before clinical manifestation and at follow-up.Case description and resultsThe patient described here developed a subacute brainstem syndrome more than 10 years after diagnosis of CIDP under low-dose maintenance treatment of intravenous immunoglobulins (IVIG). MRI revealed enhancing right-sided pontocerebellar lesion. CSF examination showed mild pleocytosis and elevated protein, and negative oligoclonal bands. Further diagnostics exclude differential diagnoses such as tuberculoma, sarcoidosis, or metastasis. Specific IFN-γ response against NF155 and NF186 as measured by Elispot assay was elevated before clinical manifestation. NF155 and NF186 antibodies were negative. Escalation of IVIG treatment at 2 g/kg BW followed by 1.4 g/kg BW led to clinical remission albeit to a new asymptomatic central lesion. Follow-up NF155 and NF186-Elispot turned negative.ConclusionThe case reported here with a delayed central manifestation after an initially typical CIDP and NF155 and NF186 T cell responses does not resemble described cases of combined central and peripheral demyelination but may reflect a novel subtype within the great clinical heterogeneity of CIDP. |
| Document Type: |
article |
| File Description: |
electronic resource |
| Language: |
English |
| ISSN: |
1664-2295 |
| Relation: |
http://journal.frontiersin.org/article/10.3389/fneur.2017.00724/full; https://doaj.org/toc/1664-2295 |
| DOI: |
10.3389/fneur.2017.00724 |
| Access URL: |
https://doaj.org/article/85cfc3932d2f48d7860e75a9ab5ef888 |
| Accession Number: |
edsdoj.85cfc3932d2f48d7860e75a9ab5ef888 |
| Database: |
Directory of Open Access Journals |
