Abstract Details

Title Trident Sign Associated with Glial Fibrillary Acidic Protein (GFAP) Astrocytopathy

Topic Autoimmune Neurology

Presentation(s) P2 - Poster Session 2 (11:45 AM-12:45 PM)

Poster/Presentation
Number 1-008

Objective To report a case of a 67-year-old woman who presented with lower extremity sensorimotor deficits and was found to have a longitudinally extensive transverse myelitis with central and dorsal-subpial enhancement in a “trident sign” pattern due to GFAP astrocytopathy.

Background

The trident sign is a radiological finding on MRI classically described in neurosarcoidosis. The trident sign describes the appearance of central canal enhancement in addition to dorsal subpial enhancement on axial MRI of the spinal cord, giving the appearance of a trident or three-pronged spear. The trident sign has frequently been described with spinal cord involvement of neurosarcoidosis, but it has also been reported with other inflammatory causes of longitudinally extensive transverse myelitis including HTLV-1 infection and neuromyelitis optica spectrum disorder, as well as with diffuse large B-cell lymphoma.

Design/Methods Case report

Results

A 67-year-old woman presented with 4 weeks of progressive, bilateral leg numbness and weakness. Brain MRI demonstrated speckled enhancement in the pons and middle cerebellar peduncles. Spine MRI demonstrated longitudinally extensive T2 hyperintensity with central and dorsal-subpial enhancement in a trident sign appearance. CSF showed 5 nucleated cells/uL (88% lymphocytes, 12% monocytes), 28 red blood cells/uL, glucose 63 mg/dL, and protein 130 mg/dL. CSF GFAP antibodies were positive at 1:32 on an otherwise negative myelopathy panel (Mayo) supporting diagnosis of GFAP astrocytopathy. Serum myelopathy panel (Mayo) and CSF cytokine panel (ARUP), flow cytometry, and cytology were unremarkable, as was whole-body FDG PET. She improved clinically with resolution of MRI abnormalities after intravenous methylprednisolone and plasma exchange. She has been stable for 12 months on maintenance rituximab.

Conclusions

This case demonstrates that the trident sign is not specific to neurosarcoidosis and may be seen in other neuroinflammatory conditions. This case suggests a role for autoantibody testing in the evaluation of a patient with longitudinally extensive transverse myelitis with a trident sign pattern.

Authors/Disclosures
Teri Schreiner, MD, MPH, FAAN (University of Colorado/ Children's Hospital of Colorado) PRESENTER	The institution of Dr. Schreiner has received personal compensation in the range of $500-$4,999 for serving as a Consultant for CDC. Dr. Schreiner has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. The institution of Dr. Schreiner has received research support from Roche Genentech.
Margaret E. Upchurch, MD	The institution of Dr. Upchurch has received research support from National MS Society.
Matthew Doerfler, MD	The institution of Dr. Doerfler has received research support from National MS Society.
Andrew Wolf, MD	The institution of Dr. Wolf has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Genentech. The institution of Dr. Wolf has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for TG Therapeutics. The institution of Dr. Wolf has received personal compensation in the range of $500-$4,999 for serving as a Consultant for EMD Serono. The institution of Dr. Wolf has received research support from Genentech. The institution of Dr. Wolf has received research support from Rocky Mountain MS Center. The institution of Dr. Wolf has received research support from Michael J. Fox Foundation. The institution of Dr. Wolf has received research support from Sanofi. The institution of Dr. Wolf has received research support from Alexion. The institution of Dr. Wolf has received research support from Novartis. The institution of Dr. Wolf has received research support from TG Therapeutics. The institution of Dr. Wolf has received research support from BMS. Dr. Wolf has received publishing royalties from a publication relating to health care. Dr. Wolf has received publishing royalties from a publication relating to health care. Dr. Wolf has received personal compensation in the range of $0-$499 for serving as a Speaker with Rocky Mountain MS Center. Dr. Wolf has a non-compensated relationship as a Board Member with Autoimmune Neurology Research Consortium that is relevant to AAN interests or activities.