Pediatric autoimmune encephalitis

Abstract

Autoimmune (antibody mediated) encephalitis (AE) is emerging as a more common cause of pediatric encephalopathy than previously thought. The autoimmune process may be triggered by an infection, vaccine, or occult neoplasm. In the latter case, onconeural autoantibodies are directed against intracellular neuronal antigens, but a recent heterogeneous group of encephalitic syndromes has been found not to have underlying tumor but is associated with autoantibodies to the neuronal surface or synaptic antigens. Neuropsychiatric symptoms are very common in autoimmune encephalopathy; as a result, affected children may be initially present to psychiatrists. Neurological features are movement disorders, seizures, altered conscious level, and cognitive regression. Hypoventilation and autonomic features may be an aspect. Inflammatory findings in the cerebrospinal fluid may be present but are relatively nonspecific. Magnetic resonance imaging (MRI) may also demonstrate abnormalities that provide clues for diagnosis, particularly on fluid-attenuated inversion recovery or T2-weighted images. AE is well responsive to immune therapy, with prompt diagnosis and treatment strongly beneficial. Patients with paraneoplastic encephalitis are more refractory to treatment compared to those in whom no malignancy is identified. Herein, the authors present an update of literature data on the clinical presentation, laboratory and imaging findings, therapy, and outcomes for the most common autoimmune encephalitides.

Encephalitis diagnosis using metagenomics: application of next generation sequencing for undiagnosed cases

“…The role of autoimmune encephalitis, in some instances triggered by an infection, 60 is beyond the scope of this review. However, since up to 20% of cases of encephalitis are caused by autoimmune disorders, in which the immune system attacks specific host proteins, 21,61 a comprehensive diagnostic service should include antibody mediated, as well as infectious, causes of encephalitis.…”

Electroencephalography Findings in Autoimmune and Neuroinflammation-Related Epilepsies

“…63 Antibodies to GABA A receptors have been associated with more diffuse brain involvement. 64 D2R antibodies have been associated with basal ganglia encephalitis, psychosis, emotional lability, and prominent movement abnormalities. 65 Autoimmune encephalitis associated with autoantibodies targeting intracellular antigens, including antineuronal antibody type-1 (anti-Hu), anti-Ma2 (also known as Ta), and anti-GAD have also been reported.…”

“…Antibodies to GAD are commonly seen in association with type 1 diabetes, other autoimmune epilepsies, cerebellar degeneration, and stiff person syndrome. 64 Unique EEG patterns associated with antibodies against these other neuronal cell-surface antigens, and intracellular antigens have not been reported (►Fig. 8).…”

Pediatric N-methyl-d-aspartate receptor autoimmune encephalitis

“…I have read the review on “Pediatric autoimmune encephalitis,” with interest. [ 1 ] N -methyl- D -aspartate receptor (NMDAR) encephalitis, a newly discovered autoimmune encephalitis with auto-antibodies that target neuronal surface or synaptic antigens, has been identified as a frequent cause of encephalitis among pediatric population. [ 1 ] Through this letter, we tried to highlight some recently available information about this disease in pediatric population.…”

“…As authors pointed out, anti-NMDAR encephalitis presents with a variety of neuropsychiatric symptoms. [ 1 ] A recent case series from a teaching hospital in India reported clinical details of 21 cases from 2010 to 2016, of which 16 were girls, with mean age of the sample between 10 and 11 years. Interestingly, all the children had psychiatric symptoms at clinical presentation, and 47% had it as the first symptom.…”