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Anti-NMDAR encephalitis secondary to acute necrotizing encephalopathy caused by herpes simplex virus infection in infants: Case series. 2023

Jia Zhang, and Jinfeng Liu, and Jianjun Wang, and Jing Gan
Department of Pediatrics, West China Second University Hospital, Sichuan University, Chengdu 610041, China; Key Laboratory of Obstetrics & Gynecologic and Pediatric Diseases and Birth Defects of the Ministry of Education, Sichuan University, Chengdu, Sichuan, China; Key Laboratory of Development and Maternal and Child Diseases of Sichuan Province, China.

To describe the clinical characteristics of anti-NMDAR encephalitis secondary to acute necrotizing encephalopathy caused by herpes simplex virus encephalitis in infants, and aid in its early recognition, diagnosis and treatment. A total of 4 infants were included; all presented with fever, seizures, and progressive disturbances of consciousness and were diagnosed with herpes simplex virus (HSV-1) encephalitis. Cerebrospinal fluid (CSF) protein levels progressively increased, and the head MRI showed necrotizing encephalopathy. There was no significant improvement or recurrence after treatment with acyclovir, dexamethasone, or immunoglobulins. CSF reexamination at 3 weeks to 3 months showed positive anti-NMDAR IgG antibodies and gradual improvement after high-dose methylprednisolone therapy. Infants with ANE associated with HSV can develop secondary anti-NMDAR encephalitis, recognition of which is critical to ensure the appropriate institution of immunotherapy after active CNS infection has been ruled out.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D001927 Brain Diseases Pathologic conditions affecting the BRAIN, which is composed of the intracranial components of the CENTRAL NERVOUS SYSTEM. This includes (but is not limited to) the CEREBRAL CORTEX; intracranial white matter; BASAL GANGLIA; THALAMUS; HYPOTHALAMUS; BRAIN STEM; and CEREBELLUM. Intracranial Central Nervous System Disorders,Brain Disorders,CNS Disorders, Intracranial,Central Nervous System Disorders, Intracranial,Central Nervous System Intracranial Disorders,Encephalon Diseases,Encephalopathy,Intracranial CNS Disorders,Brain Disease,Brain Disorder,CNS Disorder, Intracranial,Encephalon Disease,Encephalopathies,Intracranial CNS Disorder
D006561 Herpes Simplex A group of acute infections caused by herpes simplex virus type 1 or type 2 that is characterized by the development of one or more small fluid-filled vesicles with a raised erythematous base on the skin or mucous membrane. It occurs as a primary infection or recurs due to a reactivation of a latent infection. (Dorland, 27th ed.) Herpes Simplex Virus Infection
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000212 Acyclovir A GUANOSINE analog that acts as an antimetabolite. Viruses are especially susceptible. Used especially against herpes. Acycloguanosine,9-((2-Hydroxyethoxy)methyl)guanine,Aci-Sanorania,Acic,Aciclobeta,Aciclostad,Aciclovir,Aciclovir Alonga,Aciclovir-Sanorania,Acifur,Acipen Solutab,Acivir,Activir,Acyclo-V,Acyclovir Sodium,Antiherpes Creme,Avirax,Cicloferon,Clonorax,Cusiviral,Genvir,Herpetad,Herpofug,Herpotern,Herpoviric,Isavir,Laciken,Mapox,Maynar,Milavir,Opthavir,Supraviran,Viclovir,Vipral,Virax-Puren,Virherpes,Virmen,Virolex,Virupos,Virzin,Wellcome-248U,Zoliparin,Zovirax,Zyclir,aciclovir von ct,Aci Sanorania,Aciclovir Sanorania,Acyclo V,Alonga, Aciclovir,Sodium, Acyclovir,Solutab, Acipen,Virax Puren,ViraxPuren,Wellcome 248U,Wellcome248U
D060426 Anti-N-Methyl-D-Aspartate Receptor Encephalitis Disorder characterized by symptoms of CATATONIA; HYPOVENTILATION; DYSKINESIAS; ENCEPHALITIS; and SEIZURES followed by a reduced CONSCIOUSNESS. It is often followed by a viral-like prodrome. Many cases are self-limiting and respond well to IMMUNOMODULATORY THERAPIES against the NMDA RECEPTORS antibodies. Anti-NMDA Receptor Encephalitis,Anti-NMDAR Encephalitis,Non-paraneoplastic Anti-N-Methyl-D-Aspartate Receptor Encephalitis,Non-paraneoplastic Anti-NMDA Receptor Encephalitis,Non-paraneoplastic Anti-NMDAR Encephalitis,Paraneoplastic Anti-N-Methyl-D-Aspartate Receptor Encephalitis,Paraneoplastic Anti-NMDA Receptor Encephalitis,Paraneoplastic Anti-NMDAR Encephalitis,Anti N Methyl D Aspartate Receptor Encephalitis,Anti NMDA Receptor Encephalitis,Anti NMDAR Encephalitis,Anti-N-Methyl-D-Aspartate Receptor Encephalitides,Anti-NMDA Receptor Encephalitides,Anti-NMDAR Encephalitides,Anti-NMDAR Encephalitides, Non-paraneoplastic,Anti-NMDAR Encephalitides, Paraneoplastic,Anti-NMDAR Encephalitis, Non-paraneoplastic,Anti-NMDAR Encephalitis, Paraneoplastic,Encephalitides, Anti-N-Methyl-D-Aspartate Receptor,Encephalitides, Anti-NMDA Receptor,Encephalitides, Anti-NMDAR,Encephalitides, Non-paraneoplastic Anti-NMDAR,Encephalitides, Paraneoplastic Anti-NMDAR,Encephalitis, Anti-N-Methyl-D-Aspartate Receptor,Encephalitis, Anti-NMDA Receptor,Encephalitis, Anti-NMDAR,Encephalitis, Non-paraneoplastic Anti-NMDAR,Encephalitis, Paraneoplastic Anti-NMDAR,Non paraneoplastic Anti N Methyl D Aspartate Receptor Encephalitis,Non paraneoplastic Anti NMDA Receptor Encephalitis,Non paraneoplastic Anti NMDAR Encephalitis,Non-paraneoplastic Anti-NMDAR Encephalitides,Paraneoplastic Anti N Methyl D Aspartate Receptor Encephalitis,Paraneoplastic Anti NMDA Receptor Encephalitis,Paraneoplastic Anti NMDAR Encephalitis,Paraneoplastic Anti-NMDAR Encephalitides,Receptor Encephalitides, Anti-NMDA,Receptor Encephalitis, Anti-NMDA
D020803 Encephalitis, Herpes Simplex An acute (or rarely chronic) inflammatory process of the brain caused by SIMPLEXVIRUS infections which may be fatal. The majority of infections are caused by human herpesvirus 1 (HERPESVIRUS 1, HUMAN) and less often by human herpesvirus 2 (HERPESVIRUS 2, HUMAN). Clinical manifestations include FEVER; HEADACHE; SEIZURES; HALLUCINATIONS; behavioral alterations; APHASIA; hemiparesis; and COMA. Pathologically, the condition is marked by a hemorrhagic necrosis involving the medial and inferior TEMPORAL LOBE and orbital regions of the FRONTAL LOBE. (From Adams et al., Principles of Neurology, 6th ed, pp751-4) Herpes Simplex Encephalitis,Herpetic Acute Necrotizing Encephalitis,Meningoencephalitis, Herpes Simplex Virus,Acute Necrotizing Encephalitis, Herpetic,Herpes Encephalitis,Herpes Simplex Meningoencephalitis,Herpetic Encephalitis,Herpetic Meningoencephalitis,Encephalitides, Herpes Simplex,Encephalitides, Herpetic,Encephalitis, Herpes,Encephalitis, Herpetic,Herpes Simplex Encephalitides,Herpes Simplex Meningoencephalitides,Herpetic Encephalitides,Herpetic Meningoencephalitides,Meningoencephalitides, Herpes Simplex,Meningoencephalitides, Herpetic,Meningoencephalitis, Herpes Simplex,Meningoencephalitis, Herpetic

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