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Abstract Details

Decreasing Levels of Neurofilament Light Chain in Relapsing Glial Fibrillary Acidic Protein Astrocytopathy – A Case Report
Autoimmune Neurology
P3 - Poster Session 3 (11:30 AM-12:30 PM)
1-050

We review usage of neurofilament light chain (NfL) as a biomarker for disease activity in glial fibrillary acidic protein astrocytopathy (GFAP-A).

GFAP-A is a monophasic or relapsing immune mediated meningoencephalomyelitis associated with IgG antibodies against GFAP. NfL, a product of axonal breakdown detected in cerebrospinal fluid (CSF) and serum, is an established biomarker of neuroinflammation/neurodegeneration. Elevated CSF and serum NfL (sNfL) were reported during acute GFAP-A.

We describe a case of acute exacerbation of GFAP-A without increased sNfL levels.

A 41-year-old female presented to an outside hospital with generalized weakness, altered mental status, painful spasms, and urinary retention. MRI revealed multiple confluent supratentorial and infratentorial lesions with associated radial perivascular enhancement, and diffuse longitudinal cervical and thoracic cord signal hyperintensity, with edema and patchy enhancement. CSF revealed lymphocytic pleocytosis and elevated protein. Patient was diagnosed and treated for multiple sclerosis with 3 days of high dose intravenous steroids and 300mg of ocrelizumab. Patient then presented to our center with relapsing disease and was diagnosed with CSF confirmed GFAP-A. Patient had significant clinical improvement after plasma exchange and steroid taper, with resolved perivascular enhancement and reduction in parenchymal T2 hyperintensities. sNfl was measured (390 pg/mL, normal <17.3 pg/mL) ~2 months after relapse and steadily declined over 7 months. At 8 months, while on 8mg prednisone, she developed expressive aphasia. Imaging revealed recurrent perivascular enhancement. Despite clinical and radiographic evidence of relapse, sNfL further decreased (31.5 pg/mL). Patient was treated with intravenous steroids, plasma exchange, and rituximab. sNfL 2 months post-treatment was stable.

To our knowledge, no reports exist of declining NfL in acute GFAP-A. These findings suggest NfL levels may not be a reliable marker of disease activity in GFAP-A, possibly representing delayed neurodegeneration after acute inflammatory process. Further studies are needed to understand NfL trajectory throughout this disease course.
Authors/Disclosures
Victoria Dai
PRESENTER 		An immediate family member of Ms. Dai has received personal compensation for serving as an employee of Z-Ply Corporation. An immediate family member of Ms. Dai has received personal compensation for serving as an employee of Empire State Piping. An immediate family member of Ms. Dai has received personal compensation for serving as an employee of Collective Playground Inc.. An immediate family member of Ms. Dai has received personal compensation for serving as an employee of Atlantic Collective Inc.. An immediate family member of Ms. Dai has received personal compensation for serving as an employee of Transperfect. Ms. Dai has or had stock in Lexicon Pharmaceuticals.Ms. Dai has or had stock in Microsoft.Ms. Dai has or had stock in Apple.Ms. Dai has or had stock in Uber. An immediate family member of Ms. Dai has or had stock in Alexion Pharmaceuticals.Ms. Dai has or had stock in Delta Air Lines. An immediate family member of Ms. Dai has or had stock in iShares National Muni Bond ETF.Ms. Dai has or had stock in HCA Healthcare.Ms. Dai has or had stock in NextEra Energy.Ms. Dai has or had stock in Chegg.Ms. Dai has or had stock in IBEX.Ms. Dai has or had stock in Bandwidth.Ms. Dai has or had stock in Illumina.Ms. Dai has or had stock in iShares National Muni Bond ETF.Ms. Dai has or had stock in Etsy.Ms. Dai has or had stock in Equinix.Ms. Dai has or had stock in Tesla.Ms. Dai has or had stock in NIO.Ms. Dai has or had stock in NextCure.Ms. Dai has or had stock in Amgen.Ms. Dai has or had stock in Personalis.Ms. Dai has or had stock in Catalent.Ms. Dai has or had stock in Fiesta Restaurant Group.Ms. Dai has or had stock in Las Vegas Sands.Ms. Dai has or had stock in iShares MSCI Emerging Markets ETF.Ms. Dai has or had stock in NCR Voyix Corporations.Ms. Dai has or had stock in NCR Voyix Corporation.Ms. Dai has or had stock in MidCap Financial.Ms. Dai has or had stock in Surgery Partners.Ms. Dai has or had stock in Acres Commercial Realty.Ms. Dai has or had stock in AT&T.Ms. Dai has or had stock in State Street Energy Select Sector SPDR ETF.Ms. Dai has or had stock in WisdomTree U.S. Efficient Core Fund.Ms. Dai has or had stock in MongoDB.Ms. Dai has or had stock in Vanguard S&P500 ETF.Ms. Dai has or had stock in SPDR S&P 500 ETF Trust. An immediate family member of Ms. Dai has or had stock in Rigetti Computing. An immediate family member of Ms. Dai has or had stock in Robinhood Markets. An immediate family member of Ms. Dai has or had stock in Sandisk. An immediate family member of Ms. Dai has or had stock in Solaredge Technologies. An immediate family member of Ms. Dai has or had stock in SS SPDR SP Biotech ETF. An immediate family member of Ms. Dai has or had stock in Super Micro Computer. An immediate family member of Ms. Dai has or had stock in Taiwan S Manufacturing ADR. An immediate family member of Ms. Dai has or had stock in Terawulf. An immediate family member of Ms. Dai has or had stock in Tesla. An immediate family member of Ms. Dai has or had stock in USA Rare Earth. An immediate family member of Ms. Dai has or had stock in Iren. An immediate family member of Ms. Dai has or had stock in Lumentum Holdings. An immediate family member of Ms. Dai has or had stock in Meta Platforms. An immediate family member of Ms. Dai has or had stock in Micron Technology. An immediate family member of Ms. Dai has or had stock in Microsoft . An immediate family member of Ms. Dai has or had stock in NVDIA. An immediate family member of Ms. Dai has or had stock in OKLO. An immediate family member of Ms. Dai has or had stock in Ondas Holdings. An immediate family member of Ms. Dai has or had stock in Ondas. An immediate family member of Ms. Dai has or had stock in Oracle. An immediate family member of Ms. Dai has or had stock in Palantir . An immediate family member of Ms. Dai has or had stock in Advncd Micro D. An immediate family member of Ms. Dai has or had stock in Arm Hldgs. An immediate family member of Ms. Dai has or had stock in AST Spacemobile. An immediate family member of Ms. Dai has or had stock in Bloom Energy. An immediate family member of Ms. Dai has or had stock in Circle Internet Group. An immediate family member of Ms. Dai has or had stock in Coinbase Global. An immediate family member of Ms. Dai has or had stock in Direxion Daily Semicon. An immediate family member of Ms. Dai has or had stock in Eli Lilly. An immediate family member of Ms. Dai has or had stock in Intel Corp. An immediate family member of Ms. Dai has or had stock in Adobe. An immediate family member of Ms. Dai has or had stock in Advncd Micro D. An immediate family member of Ms. Dai has or had stock in Alibaba Group Holding. An immediate family member of Ms. Dai has or had stock in Alphabet Inc. An immediate family member of Ms. Dai has or had stock in Amazon. An immediate family member of Ms. Dai has or had stock in Amgen. An immediate family member of Ms. Dai has or had stock in Apple. An immediate family member of Ms. Dai has or had stock in Applied Material. An immediate family member of Ms. Dai has or had stock in Applovin. An immediate family member of Ms. Dai has or had stock in Ast Spacemobile.
Elizabeth Verter, MD Dr. Verter has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for EMD Serono. Dr. Verter has received personal compensation in the range of $50,000-$99,999 for serving as a Fellow with National MS Society.