Koki Nagai1, Takanobu Maekawa2, Hiroshi Terashima3, Masaya Kubota3, Akira Ishiguro4. 1. Department of Postgraduate Education and Training, National Center for Child Health and Development, Japan. 2. Department of General Pediatrics and Interdisciplinary Medicine, National Center for Child Health and Development, Japan. 3. Division of Neurology, National Center for Child Health and Development, Japan. 4. Department of Postgraduate Education and Training, National Center for Child Health and Development, Japan. Electronic address: ishiguro-a@ncchd.go.jp.
Abstract
BACKGROUND: General features of anti-glutamic acid decarboxylase (GAD) antibody-associated limbic encephalitis are seizures, cognitive impairment, and imaging findings at the medial temporal lobes. We report a patient affected with remarkably severe anti-GAD antibody-positive encephalitis after hematopoietic stem cell transplantation (HSCT). CASE REPORT: A 5-year-old girl received HSCT due to pineoblastoma. Thirteen months after HSCT, she showed seizure clustering and altered mental status. Her anti-GAD antibody level was high, 65,100 U/mL (reference range < 1.5 U/mL). Her disease was diagnosed as autoimmune encephalitis and she received intravenous immunoglobulin (IVIG) and methylprednisolone. After the therapy, she partially recovered. Encephalitis later relapsed, however, and she showed extremely high anti-GAD antibody, 27 months after HSCT. Although lesions were located in the temporal and occipital lobes by MRI at 5 days after the relapse, very severe whole brain encephalitis was revealed at 13 days after the relapse. Seizures and abnormal encephalogram were resistant to IVIG and methylprednisolone. After plasma exchange, these findings were resolved. MRI revealed diffuse cerebral atrophy, 57 months after the relapse. No relapse has occurred for the past 5 years with decreased anti-GAD antibody after starting bimonthly administration of IVIG. CONCLUSION: This may be the first case of severe and recurrent anti-GAD antibody-associated autoimmune encephalitis after HSCT with specific MRI findings. No relapse has occurred since starting maintenance IVIG.
BACKGROUND: General features of anti-glutamic acid decarboxylase (GAD) antibody-associated limbic encephalitis are seizures, cognitive impairment, and imaging findings at the medial temporal lobes. We report a patient affected with remarkably severe anti-GAD antibody-positive encephalitis after hematopoietic stem cell transplantation (HSCT). CASE REPORT: A 5-year-old girl received HSCT due to pineoblastoma. Thirteen months after HSCT, she showed seizure clustering and altered mental status. Her anti-GAD antibody level was high, 65,100 U/mL (reference range < 1.5 U/mL). Her disease was diagnosed as autoimmune encephalitis and she received intravenous immunoglobulin (IVIG) and methylprednisolone. After the therapy, she partially recovered. Encephalitis later relapsed, however, and she showed extremely high anti-GAD antibody, 27 months after HSCT. Although lesions were located in the temporal and occipital lobes by MRI at 5 days after the relapse, very severe whole brain encephalitis was revealed at 13 days after the relapse. Seizures and abnormal encephalogram were resistant to IVIG and methylprednisolone. After plasma exchange, these findings were resolved. MRI revealed diffuse cerebral atrophy, 57 months after the relapse. No relapse has occurred for the past 5 years with decreased anti-GAD antibody after starting bimonthly administration of IVIG. CONCLUSION: This may be the first case of severe and recurrent anti-GAD antibody-associated autoimmune encephalitis after HSCT with specific MRI findings. No relapse has occurred since starting maintenance IVIG.
Authors: Franz Felix Konen; Philipp Schwenkenbecher; Konstantin Fritz Jendretzky; Stefan Gingele; Lea Grote-Levi; Nora Möhn; Kurt-Wolfram Sühs; Britta Eiz-Vesper; Britta Maecker-Kolhoff; Corinna Trebst; Thomas Skripuletz; Martin W Hümmert Journal: Cells Date: 2022-07-11 Impact factor: 7.666