Anti-N-methyl-D-aspartate receptor encephalitis with concurrent human herpes virus-6A deoxyribonucleic acid detection: An autopsy case.
N-methyl-d-aspartate (NMDA)
autoimmune diseases
encephalitis
human herpes virus-6 (HHV-6)
neuropathology
Journal
Neuropathology : official journal of the Japanese Society of Neuropathology
ISSN: 1440-1789
Titre abrégé: Neuropathology
Pays: Australia
ID NLM: 9606526
Informations de publication
Date de publication:
Jun 2023
Jun 2023
Historique:
revised:
22
10
2022
received:
17
08
2022
accepted:
25
10
2022
medline:
5
6
2023
pubmed:
10
11
2022
entrez:
9
11
2022
Statut:
ppublish
Résumé
We report an autopsy case of anti-N-methyl-D-aspartate (NMDA) receptor (NMDAR) encephalitis with concurrent human herpes virus-6 (HHV-6) A deoxyribonucleic acid (DNA) detection in cerebrospinal fluid (CSF). A 38-year-old previously healthy Japanese man presented with a generalized seizure. Brain magnetic resonance imaging (MRI) findings were unremarkable, but CSF revealed pleocytosis. On Day 11, HHV-6 DNA was detected in CSF, and IgG antibodies against the NR1 subunit of the NMDAR (GluN1) were subsequently detected. Since HHV-6 encephalitis was initially suspected, the patient was treated with foscarnet and ganciclovir, but the HHV-6A copy number increased from 200 (Day 22) to 2000 copies/mL (Day 47), and the therapy was ineffective. As typical symptoms of anti-NMDAR encephalitis developed, we changed the patient's treatment to combat anti-NMDAR encephalitis. He was repeatedly treated with first-line immunotherapy, and GluN1 antibody titer decreased. He was not treated with second-line immunotherapy because of recurrent infections; he died on Day 310. Postmortem examinations did not show systemic tumors. Microscopic examination of the brain revealed only severe neuronal rarefaction in the hippocampal cornu ammonis (CA) 3-4 areas with gliosis. Early initiation of aggressive immunotherapy may be required in a refractory case of anti-NMDAR encephalitis, even with HHV-6A DNA detection, because the significance of this concurrent detection in autoimmune encephalitis remains unclear.
Types de publication
Case Reports
Langues
eng
Sous-ensembles de citation
IM
Pagination
257-261Subventions
Organisme : Japan Agency for Medical Research and Development
ID : JP21wm0425019
Organisme : Japan Agency for Medical Research and Development
ID : JP22fk0108637
Organisme : Japan Society for the Promotion of Science
ID : 16H06277
Organisme : Japan Society for the Promotion of Science
ID : 21K06417
Organisme : National Center of Neurology and Psychiatry
Informations de copyright
© 2022 Japanese Society of Neuropathology.
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