GABAA receptor encephalitis associated with human parvovirus B19 virus infection

Abstract

Rationale: Human parvovirus B19 (B19) infection can produce a spectrum of clinical syndromes, including neurological manifestations, most notably encephalitis. Although symptoms suggestive of autoimmune disease in patients with B19 infection have been previously described, a clear association of autoimmune encephalitis with B19 infection has yet to be established. Patient concerns: We describe the case of a 6-year-old boy who was hospitalized due to status epilepticus, which evolved to super-refractory status epilepticus that was only mildly responsive to anticonvulsant drugs. Diagnosis: A cerebrospinal fluid study identified slight pleocytosis and B19 positivity. A subsequent autoimmunity cerebrospinal fluid study revealed the presence of anti-γ-aminobutyric acid type A (GABA A ) receptor antibodies. Interventions: After pulse therapy with methylprednisolone and continuous therapy with prednisolone with cyclosporine, the patient experiencing seizure persistence with disordered motor function manifestations and only minor improvement in consciousness, and so, plasmapheresis was performed. With continued immunosuppressive treatments with cyclosporine and prednisolone, the patient's clinical picture showed progressive improvement, with good control of seizures. Although the patient tolerated withdrawal of the anticonvulsant drugs well, he developed seizures when corticosteroid therapy withdrawal was attempted, so was started on azathioprine. Outcomes: After immunosuppressive therapy, the patient evolved with complete remission of symptoms, normal neurological examination and age-appropriate neuropsychomotor development. Lessons: The present case characteristics, together with previous findings, support the hypothesis that autoimmunity may be triggered by extensive antigen release due to degeneration of infected neurons. This case highlights the importance of early clinical suspicion and treatment.

Autoimmune Encephalitis and Other Neurological Syndromes With Rare Neuronal Surface Antibodies in Children: A Systematic Literature Review

“…Twenty-three children with anti-GABAAR autoimmunity were identified ( 20 , 34 – 42 ) (13/23, 56.5% female). Median age at onset was 11 years (mean 9.6, range 2 months – 17 years).…”

Case Report: Overlapping Syndrome of Anti-NMDAR Encephalitis and MOG Inflammatory Demyelinating Disease in a Patient With Human Herpesviruses 7 Infection

“…In the post-solid organ transplant patients, Epstein-Barr Virus (EBV) and NMDAR antibodies have been reported as double positive in rare infected cases (23). Human parvovirus B19 infection has been reported to be associated with GABAA receptor encephalitis (24). Recently, a case of encephalitis associated to immunoreactivity related to SARS-CoV-2 infection has also been reported (25).…”

Actualidades en encefalitis autoinmunes mediadas por anticuerpos en pediatría

“…Estudios en ratones han demostrado que la pérdida de la subunidad β3 provoca convulsiones y déficit de aprendizaje y memoria (Ohkawa et al, 2014;Petit-Pedrol et al, 2014;Spatola et al, 2017;Zhu et al, 2018). La autoinmunidad puede desencadenarse ante la liberación de antígenos debido a la degradación neuronal después de una infección viral del tejido nervioso (Guo et al, 2020;Valle et al, 2021).…”

“…GABA-A se ha identificado en pacientes con esquizofrenia (Shiwaku et al, 2020). Sin embargo, estas afecciones son menos frecuentes en niños, en éstos es más común que se asocie con enfermedades infecciosas: parvovirus B19, herpes simple tipo 1 y 6 (VHS 1 y VHS 6) y herpes Labialis (Guo et al, 2020;Valle et al, 2021) y diferentes enfermedades autoinmunes como encefalitis anti-NMDAR y presencia de anticuerpos contra la enzima descarboxilasa de ácido de glutamato 65 (GAD65) responsable de la canalización de la descarboxilación del glutamato a GABA y CO2 (Daif et al, 2018;Guo et al, 2020;Petit-Pedrol et al, 2014).…”

“…Se presenta cefalea constante o persistente, convulsiones focales con espasmos faciales del lado izquierdo a convulsiones persistentes y generalizadas, disminución del nivel de conciencia, temblores de las extremidades, ataxia, deterioro cognitivo, la mayoría de los síntomas del movimiento o disautonomía se relaciona con la presencia de anticuerpos anti-NMDAR y se ha encontrado anticuerpos también para la proteína-1 inactivada del glioma rica en leucina (LGI1) y proteína asociada anti-contactina 2 (CASPR2), responsables de encefalitis límbica (Guo et al, 2020;Ohkawa et al, 2014;Petit-Pedrol et al, 2014;Sanabria-Sanchinel et al, 2021;Valle et al, 2021).…”