Treatment of epileptic encephalopathy after human herpesvirus 6-induced post-transplantation acute limbic encephalitis with adrenocorticotropic hormone therapy: A case report

Abstract

Recently, several authors have reported cases in which children with human herpesvirus 6-induced post-transplantation acute limbic encephalitis (HHV6-PALE) developed generalized epilepsy and cognitive deterioration. Here, we report an 8-year-old boy who developed HHV6-PALE one month after receiving cord blood transplant for precursor B lymphoblastic leukemia. At the age of 9 years, a series of epileptic spasms started, and his cognitive function deteriorated. An interictal electroencephalogram showed generalized epileptic discharges. The patient was diagnosed with epileptic encephalopathy after HHV6-PALE. Although his condition did not respond to various antiepileptic drugs, ketogenic diets, or immunomodulatory therapies, it improved temporarily after ACTH therapy. Considering that the prognosis of this type of epileptic encephalopathy is devastating, elucidation of the mechanisms responsible for the development of epileptic encephalopathy and development of a new strategy for its management are urgently needed.

Efficacy of long‐term adrenocorticotropic hormone therapy for West syndrome: A retrospective multicenter case series

“…Notably, varied adverse events force termination of ACTH therapy 41 . Previous studies on long‐term therapy have not reported any severe adverse events 23–26,35 . However, we observed nonlethal adverse effects, including height stagnation, hypertension, and irritability.…”

“…41 Previous studies on long-term therapy have not reported any severe adverse events. [23][24][25][26]35 However, we observed nonlethal adverse effects, including height stagnation, hypertension, and irritability. Clinicians who plan to initiate LT-ACTH should pay sufficient attention to these events, as well as give appropriate information to patients' caregivers.…”

“…Based on previous case reports, [23][24][25][26] we defined long-term ACTH therapy (LT-ACTH) as the consecutive combination of daily and weekly injection periods (Figure 1). The daily injection period lasted the first 1-4 weeks, while the weekly injection period involved weekly or biweekly injections for the subsequent 3 months or longer.…”

“…This regimen consisted of 2‐3 weeks of daily ACTH injections followed by 7 months to 1 year of weekly or biweekly injections. This regimen has been employed for various intractable epilepsies 24–26 . Long‐term, weekly ACTH therapy might be a good treatment option for pediatric patients with intractable epilepsy; however, as there has been no relevant large cohort study, and its effectiveness, ideal protocol, patients assumed to be good candidates, and incidence of adverse effects are not known.…”