TY - JOUR
T1 - Teaching NeuroImages
T2 - Linear Radial Periventricular Enhancement in Glial Fibrillary Acidic Protein Astrocytopathy
AU - Kong, Yongyao
AU - McKeon, Andrew
AU - Koh, Odelia S.Q.
AU - Chiew, Yi Rong
AU - Purohit, Bela
AU - Chin, Chee Fang
AU - Zekeridou, Anastasia
AU - Ng, Adeline S.L.
N1 - Publisher Copyright:
© American Academy of Neurology.
PY - 2023/4/18
Y1 - 2023/4/18
N2 - A 48-year-old woman presented with asymptomatic painless optic disc swelling, seizures, cognitive decline, and weakness over 3 months. She had an upper motor neuron pattern of grade 4/5 weakness in all 4 limbs. Brain MRI revealed striking linear radial perivascular enhancement in the deep white matter1,2 (figure, A and B); MRI spine revealed longitudinally extensive C3-to-conus cord T2 hyperintensity and T1 enhancement2 (figure, C and D). CSF demonstrated glial fibrillary acidic protein (GFAP) pattern by indirect immunofluorescence with GFAPα specificity confirmed by cell-based assay. The patient improved clinically (figure, E and F) with steroid treatment. Linear/radial perivascular enhancement in the deep white matter prompts consideration of a corticosteroid-responsive meningoencephalomyelitis termed autoimmune GFAP astrocytopathy.1
AB - A 48-year-old woman presented with asymptomatic painless optic disc swelling, seizures, cognitive decline, and weakness over 3 months. She had an upper motor neuron pattern of grade 4/5 weakness in all 4 limbs. Brain MRI revealed striking linear radial perivascular enhancement in the deep white matter1,2 (figure, A and B); MRI spine revealed longitudinally extensive C3-to-conus cord T2 hyperintensity and T1 enhancement2 (figure, C and D). CSF demonstrated glial fibrillary acidic protein (GFAP) pattern by indirect immunofluorescence with GFAPα specificity confirmed by cell-based assay. The patient improved clinically (figure, E and F) with steroid treatment. Linear/radial perivascular enhancement in the deep white matter prompts consideration of a corticosteroid-responsive meningoencephalomyelitis termed autoimmune GFAP astrocytopathy.1
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U2 - 10.1212/WNL.0000000000207021
DO - 10.1212/WNL.0000000000207021
M3 - Review article
AN - SCOPUS:85159597838
SN - 0028-3878
VL - 100
SP - S220-S221
JO - Neurology
JF - Neurology
IS - 16
ER -