Background: Information and pathobiological understanding about central demyelinating manifestation in patients, who primarily suffer from chronic inflammatory demyelinating polyneuropathy (CIDP), are scarce. Methods: IFN-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 results: The 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. Conclusion: The 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. Introduction Multiple sclerosis (MS) and chronic inflammatory demyelinating polyneuropathy (CIDP) are both demyelinating disorders of the nervous system with unknown underlying pathomechanisms. Whereas MS is restricted to the central nervous system, CIDP comprises demyelination of the peripheral nervous system. There are few reports in the literature about patients suffering from both, CNS and PNS manifestation in similar extent (1, 2). However, only little is known about central manifestations other than cranial nerve involvement over the course of disease in patients, who primarily suffer from CIDP. Due to its heterogeneous manifestation, different autoimmune targets are likely to be relevant in CIDP. T cell responses have been shown to be involved in its pathogenesis (3, 4).
Klehmet, J., Staudt, M., Diederich, J. M., Siebert, E., Meinl, E., Harms, L., & Meisel, A. (2017). Neurofascin (NF)155- and NF186-specific T Cell response in a patient developing a central pontocerebellar demyelination after 10 Years of CIDP. Frontiers in Neurology, 8(DEC). https://doi.org/10.3389/fneur.2017.00724