Myelinoclastic diffuse sclerosis (Schilder's disease) is immunologically distinct from multiple sclerosis: results from retrospective analysis of 92 lumbar punctures

Journal of Neuroinflammation
S JariusB Wildemann

Abstract

Myelinoclastic diffuse sclerosis (MDS; also termed Schilder's disease) is a rare inflammatory demyelinating disorder of the central nervous system characterised by demyelination of vast areas of the white matter. It is unclear whether MDS is a variant of multiple sclerosis (MS) or a disease entity in its own right. To compare the cerebrospinal fluid (CSF) features of MDS with those of MS. Retrospective analysis of the CSF profile of all patients with MDS reported in the medical literature between 1960 and 2018. The most striking finding was a substantial lack of oligoclonal bands (OCBs) in MDS, which were absent in at least 77% (30/39) of all lumbar punctures (LP) in the total cohort and in 86% in the subgroup of patients with normal very long-chain fatty acid serum ratios (VLCFA). Almost all cases published in the past 15 years were negative for OCBs. These findings are in contrast to MS, in which OCBs are present in up to 98% of cases (p < 0.00001 when compared with reference works in MS; both in adult and in pediatric patients). CSF pleocytosis was absent in at least 79% (46/58) of all LP (p < 0.0001 vs. MS) and in 92% (24/26) of LPs in the VLCFA-tested subgroup. CSF total protein levels were elevated in 56% of all LPs (p < ...Continue Reading

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Citations

Mar 21, 2020·Journal of Computer Assisted Tomography·Ahmed Abdel Khalek Abdel Razek, Nermeen A Elsebaie
Sep 5, 2020·Journal of Neuroinflammation·Sven JariusUNKNOWN in cooperation with the Neuromyelitis Optica Study Group (NEMOS)
Sep 5, 2020·Journal of Neuroinflammation·Sven JariusUNKNOWN in cooperation with the BIOMARKER study group and the Neuromyelitis optica Study Group (NEMOS)
Nov 14, 2020·International Journal of Molecular Sciences·Marina BozikiNikolaos Grigoriadis
May 20, 2021·AJR. American Journal of Roentgenology·Mariko NakayamaToshio Moritani
Aug 24, 2021·Expert Review of Neurotherapeutics·Pedro SánchezTodd A Hardy

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Methods Mentioned

BETA
MDS
biopsy

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