Abstract
IntroductionPost-infectious autoimmune demyelination of the central nervous system is a rare neurological disorder typically associated with exanthematous viral infections. We report an unusual presentation of the condition and a previously undocumented association with Streptococcus pneumonia meningitis.Case presentationA 50-year-old Caucasian woman presented to our facility with an acute myelopathy three days after discharge following acute Streptococcus pneumoniae meningitis. Imaging studies of the spine ruled out an infective focus and no other lesions were seen within the cord. Diffuse, bilateral white matter lesions were seen within the cerebral hemispheres, and our patient was diagnosed as having a post-infective demyelination syndrome that met the diagnostic criteria for an acute transverse myelitis. Our patient clinically and radiologically improved following treatment with steroids.ConclusionsThe novel association of a Streptococcus pneumoniae infection with post-infectious autoimmune central nervous system demyelination should alert the reader to the potentially causative role of this common organism, and gives insights into the pathogenesis. The unusual dissociation between the clinical presentation and the location of the radiological lesions should also highlight the potential for the condition to mimic the presentation of others, and stimulates debate on the definitions of acute transverse myelitis and acute disseminated encephalomyelitis, and their potential overlap.
Highlights
Post-infectious autoimmune demyelination of the central nervous system is a rare neurological disorder typically associated with exanthematous viral infections
The unusual dissociation between the clinical presentation and the location of the radiological lesions should highlight the potential for the condition to mimic the presentation of others, and stimulates debate on the definitions of acute transverse myelitis and acute disseminated encephalomyelitis, and their potential overlap
Our patient met the Transverse Myelitis Consortium Working Group’s criteria [1] for Acute transverse myelitis (ATM) due to the appropriate clinical presentation, evidence of inflammation within the spinal cord obtained on cerebral spinal fluid (CSF) analysis, and exclusion of other aetiologies; it is worth highlighting that the criteria state that evidence of inflammation on CSF analysis alone, in the absence of radiological features, is sufficient to support the diagnosis
Summary
It appears that this case is unusual for a number of reasons. Firstly, to the best of our knowledge, this is the first reported case in the English literature of an S. pneumoniae infection being associated with postinfective autoimmune CNS demyelination. The precise nature of the lesions is unlikely to be determined; if histological specimens were available, the finding of a perivascular distribution of the demyelination would give a pathological diagnosis of ADEM, lending support to the radiological appearance and suggesting that the absence of encephalopathy is not significant. In the original consensus definition of ADEM, the International Pediatric MS Study Group acknowledged that the absolute requirement for encephalopathy may be overly restrictive, but deemed it necessary in order for sufficient specificity [6] If this were to be the case, the disparity between clinical and radiological localisation of the lesions would still be unusual for ADEM.
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