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November 2022; 9 (6) Research ArticleOpen Access

Area Postrema Syndrome in Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy

A Case Series and Literature Review

Bo Deng, Jingguo Wang, Hai Yu, Lei Jin, Yue Qiu, Xiaoni Liu, Pengyu Wang, Xiang Zhang, Xiangjun Chen
First published September 26, 2022, DOI: https://doi.org/10.1212/NXI.0000000000200029
Bo Deng
From the Department of Neurology (B.D., J.W., H.Y., L.J., Y.Q., X.L., P.W., X.Z., X.C.), Huashan Hospital and Insitute of Neurology, Fudan University; National Center for Neurological Disorders (B.D., J.W., H.Y., L.J., Y.Q., X.L., P.W., X.Z., X.C.); and Human Phenome Institute (X.C.), Fudan University, Shanghai, China.
MM
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Jingguo Wang
From the Department of Neurology (B.D., J.W., H.Y., L.J., Y.Q., X.L., P.W., X.Z., X.C.), Huashan Hospital and Insitute of Neurology, Fudan University; National Center for Neurological Disorders (B.D., J.W., H.Y., L.J., Y.Q., X.L., P.W., X.Z., X.C.); and Human Phenome Institute (X.C.), Fudan University, Shanghai, China.
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Hai Yu
From the Department of Neurology (B.D., J.W., H.Y., L.J., Y.Q., X.L., P.W., X.Z., X.C.), Huashan Hospital and Insitute of Neurology, Fudan University; National Center for Neurological Disorders (B.D., J.W., H.Y., L.J., Y.Q., X.L., P.W., X.Z., X.C.); and Human Phenome Institute (X.C.), Fudan University, Shanghai, China.
MM
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Lei Jin
From the Department of Neurology (B.D., J.W., H.Y., L.J., Y.Q., X.L., P.W., X.Z., X.C.), Huashan Hospital and Insitute of Neurology, Fudan University; National Center for Neurological Disorders (B.D., J.W., H.Y., L.J., Y.Q., X.L., P.W., X.Z., X.C.); and Human Phenome Institute (X.C.), Fudan University, Shanghai, China.
MB
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Yue Qiu
From the Department of Neurology (B.D., J.W., H.Y., L.J., Y.Q., X.L., P.W., X.Z., X.C.), Huashan Hospital and Insitute of Neurology, Fudan University; National Center for Neurological Disorders (B.D., J.W., H.Y., L.J., Y.Q., X.L., P.W., X.Z., X.C.); and Human Phenome Institute (X.C.), Fudan University, Shanghai, China.
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Xiaoni Liu
From the Department of Neurology (B.D., J.W., H.Y., L.J., Y.Q., X.L., P.W., X.Z., X.C.), Huashan Hospital and Insitute of Neurology, Fudan University; National Center for Neurological Disorders (B.D., J.W., H.Y., L.J., Y.Q., X.L., P.W., X.Z., X.C.); and Human Phenome Institute (X.C.), Fudan University, Shanghai, China.
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Pengyu Wang
From the Department of Neurology (B.D., J.W., H.Y., L.J., Y.Q., X.L., P.W., X.Z., X.C.), Huashan Hospital and Insitute of Neurology, Fudan University; National Center for Neurological Disorders (B.D., J.W., H.Y., L.J., Y.Q., X.L., P.W., X.Z., X.C.); and Human Phenome Institute (X.C.), Fudan University, Shanghai, China.
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Xiang Zhang
From the Department of Neurology (B.D., J.W., H.Y., L.J., Y.Q., X.L., P.W., X.Z., X.C.), Huashan Hospital and Insitute of Neurology, Fudan University; National Center for Neurological Disorders (B.D., J.W., H.Y., L.J., Y.Q., X.L., P.W., X.Z., X.C.); and Human Phenome Institute (X.C.), Fudan University, Shanghai, China.
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Xiangjun Chen
From the Department of Neurology (B.D., J.W., H.Y., L.J., Y.Q., X.L., P.W., X.Z., X.C.), Huashan Hospital and Insitute of Neurology, Fudan University; National Center for Neurological Disorders (B.D., J.W., H.Y., L.J., Y.Q., X.L., P.W., X.Z., X.C.); and Human Phenome Institute (X.C.), Fudan University, Shanghai, China.
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Full PDF
Citation
Area Postrema Syndrome in Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy
A Case Series and Literature Review
Bo Deng, Jingguo Wang, Hai Yu, Lei Jin, Yue Qiu, Xiaoni Liu, Pengyu Wang, Xiang Zhang, Xiangjun Chen
Neurol Neuroimmunol Neuroinflamm Nov 2022, 9 (6) e200029; DOI: 10.1212/NXI.0000000000200029

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    Figure 1 Flowchart of All Included GFAP-IgG–Positive Cases

    APS = area postrema syndrome; GFAP = glial fibrillary acidic protein.

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    Figure 2 Time Points of APS Onset, Immunotherapy Initiation, and Cessation of APS in Each Glial Fibrillary Acidic Protein-IgG–Positive Case

    The x-axis indicates days from the onset of disease. Each row of the line on the y-axis represents a patient. Symbols of red lightning, green arrows, and dots represent APS onset, immunotherapy initiation, and cessation of APS, respectively. Note that APS occurred at the early stage of disease and was responsive to immunotherapy. APS = area postrema syndrome; Pt = patient.

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    Figure 3 Area Postrema and Dorsal Medulla Lesions in Representative Glial Fibrillary Acidic Protein-IgG–Positive Patients

    Sagittal T2-weighted fluid-attenuated inversion recovery scan shows lesions in the area postrema and multifocal punctate lesions in medulla and cervical spinal cord (A.a [patient 5] and B.a [patient 1]; black arrows). Inset in (A.a) is the axial image (area postrema level), which shows bilateral lesions involving the dorsal medulla (white arrows). Sagittal T1-weighted MRI with gadolinium shows irregular patchy or linear enhancement in the medulla and cervical spinal cord (A.b and B.b; black arrowheads). Inset in (A.b) is the axial image (area postrema level), which shows hazy gadolinium enhancement of bilateral dorsal medulla (white arrowheads). T2-hyperintensity lesions in pons, medulla oblongata, and cervical longitudinally extensive lesions were noted at the initial presentation of patient 4 (C.a). Follow-up MRI at 6 months after immunotherapy reveals remission of most lesions (C.b). Axial images of patient 2 show scattered faint T2-hyperintensity lesions in dorsal medulla oblongata (D.a; black arrows) accompanied by patchy gadolinium enhancement (D.b; black arrowheads).

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