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Abstract Details

MOG-Abs in CSF only: pathological findings support the diagnostic role
Autoimmune Neurology
Autoimmune Neurology Posters (7:00 AM-5:00 PM)
057
to report clinical and pathological data of a patient with anti-myelin oligodendrocyte glycoprotein antibodies (MOG-Abs) detected in CSF only.

serum MOG-Abs define a distinct entity of inflammatory demyelinating diseases, characterised by subacute onset of predominantly optic nerve and/or spinal cord involvement. Recently, we proposed to extend the analysis of MOG-Abs in the CSF of highly suggestive seronegative cases to increase diagnostic sensitivity. However, the exact value of CSF MOG-Abs needs further confirmations.

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a 81-year-old man presented with urinary retention followed by paraplegia and L1 sensory level loss. Spinal cord MRI showed multiple T2 hyperintense lesions in brainstem and spinal cord, a tumefactive central lesion extending from T9 to the conus, homogeneous and diffuse meningeal enhancement along the surface of brainstem and spinal cord together with thickening and enhancement of lumbosacral nerve roots. CSF analysis revealed increased protein content (5.2 g/L), mild lymphocytic pleocytosis (29 cells/μL) and oligoclonal bands (pattern 3). Investigations for infectious and haematological conditions yielded negative results. On autoimmune screening, CSF only MOG-Abs were detected with live cell-based assay. Despite treatment with high-dose intravenous steroids, the clinical picture rapidly deteriorated to upper limbs weakness, multiple cranial nerves involvement, and severe dysautonomia. The patient died 15 days after symptoms onset.

Pathological macroscopic examination showed signs of oedema and necrosis, predominantly involving the brainstem and cervical spinal cord. On microscopic evaluation extensive activated macrophages infiltration was noted on the surface of brainstem and spinal cord. Perivascular and parenchymal lymphocytes (mixed CD20+ and CD3+ with some CD138+ plasma cells) were observed, involving also the leptomeninges.

we herein report the first spinal cord pathological evaluation of a patient with isolated CSF MOG-Abs. Pathological findings, compatible with a subacute inflammatory process, further reinforce the importance of testing MOG-Abs in the CSF of highly suggestive cases.

Authors/Disclosures
Sara Carta, MD (Neurology Unit, University of Verona)
PRESENTER
An immediate family member of Dr. Carta has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for UCB . The institution of Dr. Carta has received research support from MEDEN.
No disclosure on file
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Markus Reindl, PhD (Medical University of Innsbruck) The institution of Dr. Reindl has received research support from Euroimmun. The institution of Dr. Reindl has received research support from Roche.
Romain Marignier, MD, PhD (Lyon University Hospital) Dr. Marignier has received personal compensation in the range of $0-$499 for serving as a Consultant for UCB. Dr. Marignier has received personal compensation in the range of $500-$4,999 for serving as a Consultant for ALEXION. Dr. Marignier has received personal compensation in the range of $500-$4,999 for serving as a Consultant for ROCHE. Dr. Marignier has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Amgen. Dr. Marignier has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for ROCHE.
No disclosure on file
Sara Mariotto, MD, PhD (Neurology Unit, University of Verona) Dr. Mariotto has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Biogen, Sanofi, Alexion, Roche, TSF, Dynamics, UCB, Novartis, Amgen, AAN. The institution of Dr. Mariotto has received research support from Ministero della Salute Italiano. The institution of Dr. Mariotto has received research support from TSF. The institution of Dr. Mariotto has received research support from GJF. The institution of Dr. Mariotto has received research support from Lundbeck. The institution of Dr. Mariotto has received research support from Euroimmun. The institution of Dr. Mariotto has received research support from FISM.