Dilemmas and challenges in treating seronegative autoimmune encephalitis in Indian children

Abstract

Objective:The main objective is to assess the challenges in diagnosis and treatment while managing seronegative cases of autoimmune encephalitis (AIE) in Indian children.Methods:A cohort study of patients with AIE was done where clinical presentations, investigations, management were analyzed and these patients were followed up to assess the evolution of the disease.Results:Nine patients were included in the study. Four patients presented with super-refractory status epilepticus (SRSE). Other presentations were behavioral change, hemiplegia, and autonomic dysfunction. Initial magnetic resonance imaging brain was suggestive of AIE in two patients. Only two were seropositive for cerebrospinal fluid (CSF) autoimmune panel. Five patients responded to the first-line immunotherapy and four required the second-line immunotherapy.Conclusion:The possibility of autoimmune encephalitis should be considered in patients with super-refractory status epilepticus. A large proportion of children with suspected AE may be “seronegative.” A trial of immunotherapy should be given to these children when there is a strong clinical suspicion of autoimmune encephalitis even in the absence of cerebrospinal fluid autoantibodies.

Antibody negative autoimmune encephalitis- Does it differ from definite one?

“…Other studies from India have also supported the use of immunotherapy in seronegative AE. [7] Therefore, in the present paper, we tried to look for any clinical or therapeutic difference in antibody positive (definite) and antibody negative AE.…”

Seronegative Autoimmune Encephalitis

“…Young children and the elderly are vulnerable members of our society whose susceptibility to antibody-mediated illnesses and neurocognitive outcomes depend on the speed of instituting immunotherapy and disease-modifying agents. [ 8 9 ] Use of such scores should be strongly recommended when the tempo of illness is subacute, CSF is cellular, and PET scan is suggestive. [ 7 10 ] However, a larger study in such patients [clinically suggestive of IgLON5 encephalitis but antibody negative] needs to be done to validate these scores and bring them into the standard recommendation.…”

“…[ 8 ] In children, rapid cognitive decline, impairment of memory, refractory seizures, and dyskinesias are suggestive of an autoimmune etiology despite negative antibody results. [ 8 9 ] These scales are invaluable to enhance the yield of diagnosis in patients suspected of autoimmune encephalopathy or epilepsy irrespective of their age. [ 6 7 ]…”

Diagnosis and Management of Suspected Pediatric Autoimmune Encephalitis: A Comprehensive, Multidisciplinary Approach and Review of Literature

“…12,35,41 Additionally, rates of antibody-negative autoimmune encephalitis are around 50%. 8,42 Favorable outcomes are associated with prompt initiation of immunotherapy. 39,[43][44][45] Delays in treatment may be associated with poor treatment response even after immunotherapy is started.…”