Autoimmunity and inflammation in status epilepticus: from concepts to therapies

Abstract

The understanding of immunological mechanisms underlying some forms of epilepsy and encephalitis has rapidly increased for the last 10 years leading to the concept of status epilepticus of autoimmune origin. Actual treatment recommendations regarding autoimmune status epilepticus are based on retrospective case studies, pathophysiological considerations and experts’ opinion. In addition, there are no clear indicators to predict outcome. In situations where autoimmune mechanisms are suspected in patients with status epilepticus, there is evidence that earlier treatment is related to better outcome. Increased awareness is mandatory to decrease the number of patients with major neurological problems or fatal outcome, which is overall about 50%. We here summarize findings of all pediatric and adult patients reported to date, and review the current state of knowledge in the field of immune therapeutic approaches of status epilepticus.

Keywords::

• autoimmune
• chronic epilepsy
• FIRES
• GABA
• GAD
• Hashimoto encephalitis
• NMDA
• status epilepticus
• VGKC

Financial & competing interests disclosure

Supported by the Swiss National Science Foundation (SPUM grant no 140332 to MS). The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

No writing assistance was utilized in the production of this manuscript.

Key issues

• Patients in refractory or superrefractory status epilepticus with suspected or confirmed autoimmune etiology often need intensive care, antiepileptic and immunomodulatory therapies and follw-up with an interdisciplinary team.

• The understanding of immunological mechanisms of epilepsy is rapidly increasing since the past 10 years. Actual treatment recommendations regarding status epilepticus are based on retrospective case studies, pathophysiological considerations and experts’ opinion.

• There are no clear indicators to predict outcome, which may be good or excellent even after long-lasting superrefractory status epilepticus, justifying long-lasting intensive care and therapies.

• There is evidence that earlier treatment is related to better outcome, and increased awareness is mandatory to decrease the number of patients with major neurological problems or fatal outcome.

Notes