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Journal of Neuroinflammation Feb 2019Myelinoclastic diffuse sclerosis (MDS; also termed Schilder's disease) is a rare inflammatory demyelinating disorder of the central nervous system characterised by...
BACKGROUND
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.
OBJECTIVE
To compare the cerebrospinal fluid (CSF) features of MDS with those of MS.
METHODS
Retrospective analysis of the CSF profile of all patients with MDS reported in the medical literature between 1960 and 2018.
RESULTS
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 < 0.0001 vs. MS) and in 63% of LPs in the VLCFA-tested subgroup and were often higher than in typical MS (> 100 mg/dL in 13/22; up to 220 mg/dL). EBV serum antibodies, which are present in virtually all patients with MS, and the so-called MRZ (measles/rubella/zoster) reaction, a highly specific marker of MS, were absent in all of the few patients tested. In addition, we discuss further differences between MS and MDS, taking into account also Schilder's original comprehensive case description from 1912.
CONCLUSION
In the majority of patients diagnosed with MDS, CSF features differ significantly from those typically found in MS and are more similar to those previously reported in patients with myelin oligodendrocyte glycoprotein-immunoglobulin G (IgG)-positive encephalomyelitis, aquaporin-4-IgG-positive neuromyelitis optica spectrum disorders or Baló's concentric sclerosis. Our data suggest that MDS and MS are immunopathologically distinct entities in the majority of cases.
Topics: Adolescent; Adult; Age of Onset; Aged; Biomarkers; Child; Child, Preschool; Diffuse Cerebral Sclerosis of Schilder; Female; Humans; Infant; Male; Middle Aged; Multiple Sclerosis; Oligoclonal Bands; Retrospective Studies; Young Adult
PubMed: 30819213
DOI: 10.1186/s12974-019-1425-4 -
Journal of Infection and Public Health 2019Leptospirosis is the most widely spread zoonosis and Leptospirosis Associated Acute Kidney Injury (LAKI) is common and fatal if not properly and swiftly treated. The aim...
Leptospirosis is the most widely spread zoonosis and Leptospirosis Associated Acute Kidney Injury (LAKI) is common and fatal if not properly and swiftly treated. The aim of this review is to evaluate the mortality of LAKI and to identify the risk factors for its development. An electronic search was performed to identify the studies included LAKI patients series. Only studies which investigated mortality or risk factors for LAKI development in adults were included. Twenty-three studies with 24 patients series were included in the final analysis and included 1698 patients. The median series mortality was 10.05% (range 0-33.3%) with a total of 223 death. Only four studies identified the independent risk factors for LAKI development which were oliguria, jaundice, arrhythmia, crackles, elevated direct bilirubin level, elevated activated prothrombin time, hyperbilirubinemia and leukocytosis. Although the mortality of LAKI is high, its predictors are not studied enough in literature.
Topics: Acute Kidney Injury; Adolescent; Adult; Aged; Child; Female; Humans; Leptospirosis; Male; Middle Aged; Risk Factors; Survival Analysis; Young Adult
PubMed: 31281106
DOI: 10.1016/j.jiph.2019.06.014