Clinical features, treatment strategies, and outcomes in hospitalized children with immune-mediated encephalopathies.

McGetrick ME, Varughese NA, Miles DK, Wang CX, McCreary M, Monson NL, Greenberg BM MD. Clinical Features, Treatment Strategies, and Outcomes in Hospitalized Children With Immune-Mediated Encephalopathies. Pediatr Neurol. 2021 Mar;116:20-26. doi: 10.1016/j.pediatrneurol.2020.11.014. Epub 2020 Nov 28. PMID: 33388545.

Abstract

Background: Autoimmune encephalitis (AE) and acute disseminated encephalomyelitis (ADEM) are immune-mediated brain conditions that can cause substantial neurological sequalae. Data describing the clinical characteristics, treatments, and neurological outcomes for these conditions are needed.

Methods: This is a single-center retrospective review of children diagnosed with AE or ADEM over a nine-year period with discharge outcomes measured by the Modified Rankin Score.

Results: Seventy-five patients (23 with ADEM and 52 with AE) were identified. Patients with ADEM had a higher percentage of abnormal magnetic resonance imaging findings (100% vs 60.8%; P < 0.001) and a shorter time from symptom onset to diagnosis (6 vs 14 days; P = 0.024). Oligoclonal bands and serum and cerebrospinal fluid inflammatory indices were higher in patients with AE. Nearly all patients received corticosteroids followed by plasmapheresis or intravenous immunoglobulin, and treatment strategies did not differ significantly between groups. Second-line immune therapies were commonly used in patients with AE. Finally, patients with AE had trends toward longer hospital lengths of stay (21 vs 13 days) and a higher percentage of neurological disability at hospital discharge (59.6% vs 34.8%).

Conclusions: Although patients with ADEM and AE may have similar presenting symptoms, we found significant differences in the frequency of imaging findings, symptom duration, and laboratory and cerebrospinal fluid profiles, which can assist in distinguishing between the diagnoses. Patients in both groups were treated with a combination of immunomodulating therapies, and neurological disability  was common at hospital discharge.      ______________________________________________________________________

The most common forms of immune-related pediatric encephalitis are acute disseminated encephalomyelitis (ADEM) and autoimmune encephalitis (AE). Although these are two distinct disorders, explains UTSW pediatric critical care fellow Molly E. McGetrick, M.D., their presentation -- including disorientation and other signs of altered mental status, seizures, or motor and sensory abnormalities -- is largely the same in children, hindering an accurate diagnosis. In addition, the rarity of AE and ADEM makes amassing data to help distinguish these conditions more difficult.

"Despite their similarities in presentation, patients with AE tend to have a more prolonged and protracted condition that requires more therapies than those with ADEM," McGetrick explains. "Being able to definitively distinguish between these conditions could help doctors guide patients and their families on what to expect."...

McGetrick notes that currently ADEM and AE patients are given similar therapies, including corticosteroids to reduce the body's inflammatory reaction to autoantibodies, intravenous immunoglobulins to bind and neutralize pathologic autoantibodies, or plasmapheresis to remove autoantibodies from the body over a series of sessions. Many times, symptoms for both conditions will resolve with these treatments, but they can take longer for AE and recur in some individuals. The more researchers can learn about the distinguishing characteristics of these conditions, she says, the more they may be able to target specific treatments for each condition, improving the outlook for these patients.

"One of the biggest take-home messages from this study is that we still have a lot to learn about these conditions," McGetrick says. "The more we know, the brighter the future will ultimately be for these patients."

https://www.sciencedaily.com/releases/2021/02/210223121627.htm