Rinsho Shinkeigaku (Clinical Neurology)

Brief Clinical Note

A case of cryptogenic new-onset refractory status epilepticus (NORSE) in which cerebrospinal fluid IL-6 was elevated with increased seizure frequency early in the disease: a case report

Ryoji Nakada, M.D.1), Yuka Terasawa, M.D., Ph.D.1), Takeo Sato, M.D.1), Hiroki Takatsu, M.D.1), Nobuyoshi Kaito, M.D., Ph.D.2) and Yasuyuki Iguchi, M.D., Ph.D.1)

1) Department of Neurology, The Jikei University School of Medicine
2) Department of Neurosurgery, The Jikei University School of Medicine

A 25-year-old male presented with clonic seizures three days following a fever. The patient developed status epilepticus and required mechanical ventilation and intravenous anesthesia. The patient's epileptic seizures persisted despite administering intravenous anesthesia and multiple anti-epileptic drugs. The clinical presentation in this case, without pre-existing relevant neurological disorder and an active structural, toxic, or metabolic cause in the acute phase, was compatible with new-onset refractory status epilepticus (NORSE). After immunotherapy, including intravenous methylprednisolone, plasma exchange, and intravenous immunoglobulin therapy, the epileptic discharge on electroencephalogram (EEG) decreased gradually, and mechanical ventilation was discontinued. Neversless the final outcome was poor. The patient's condition was finally diagnosed as cryptogenic NORSE. The IL-6 levels in the cerebrospinal fluid showed a significant increase between day 6 and 11 after onset, during which time there was a rapid escalation in seizure frequency on EEG. Considering this, IL-6 may be involved in the process of seizure exacerbation.
Full Text of this Article in Japanese PDF (1810K)

(CLINICA NEUROL, 63: 843−846, 2023)
key words: new-onset refractory status epilepticus (NORSE), cryptogenic NORSE, status epilepticus, immune therapy, IL-6

(Received: 4-May-23)