Nicht-konvulsiver Status epilepticus des Erwachsenen: Typen, Pathophysiologie, Epidemiologie, Ursachen und Diagnose

Zusammenfassung

Der nicht-konvulsive Status epilepticus (NKSE) ist gekennzeichnet durch andauernde elektroklinische epileptische Aktivität oder durch eine Abfolge von epileptischen Anfällen, zwischen denen der Patient nicht zum Vorzustand zurückkehrt, und die ohne eindeutige tonische-klonische Entäußerungen einhergehen. Diese „stille“ Manifestation andauernder klinisch-neurologischer Symptome wie Aphasie, Verwirrung, etc. erschwert das Erkennen eines NKSE; oft ist der wichtigste Diagnoseschritt, überhaupt daran zu denken, dass die vorliegende Klinik auf einem NKSE beruhen könnte. Sicher nachgewiesen und bestätigt werden kann der NKSE nur durch eine unmittelbare EEG-Ableitung. In epidemiologischen Studien scheint der konvulsive Status epilepticus (KSE) häufiger (60 : 40 %) vorzukommen, doch dies ist fraglich, werden doch viele NKSE aufgrund der unspektakulären Manifestation verpasst. Pathophysiologisch spielen sich auf neuronaler Ebene dieselben Phänomene wie beim KSE ab, einfach nur nicht in den primären Motoneuronen. Die permanente Hyperexzitabilität kann die vom NKSE betroffenen Neuronen genauso schädigen. Hingegen fehlen die unmittelbar vital bedrohlichen sekundären pathophysiologischen Folgen des KSE (Lactat-Azidose, respiratorische Erschöpfung, Rhabdomyolyse, etc.) beim NKSE weitgehend. Doch auch dort können autonome Phänomene (Arrhythmien [Kammertachykardie oder Asystolie] und Atmungsprobleme) den Patienten akut lebensgefährlich gefährden. Die Ursachen für einen NKSE sind vielfältig und umfassen bei Patienten mit einer bekannten Epilepsie v. a. Medikationsprobleme (mangelnde Adhärenz, Interaktionen bei Zugabe anderer Medikamente); bei ihnen sowie denjenigen Betroffenen ohne bekannte Epilepsie kommen metabolische, toxische, strukturelle (Tumore, Blutungen, Ischämien), infektiöse, entzündliche und autoimmune Ursachen in Frage. Entsprechend breit sollte die Ursache für einen NKSE abgeklärt werden, da besonders bei den akuten symptomatischen Formen die Behandlung der Ursache mindestens so wichtig ist wie die anfallsbeendende Behandlung des NKSE.

Abstract

Non-convulsive status epilepticus (NCSE) is defined by permanent electroclinical non-convulsive epileptic activity or a series of nonconvulsive seizures without recovery to baseline. This “silent” manifestation of lasting neurological symptoms, like aphasia, confusion, etc., hinders easy recognition of NCSE. The most important diagnostic step often is to think at NCSE at all. NCSE can be confirmed by an immediate EEG recording only. Epidemiological studies show slight preponderance of convulsive status epilepticus (CSE) over NCSE (60 : 40 %); however, this might result from lack of recognition of NCSE because of its very unspectacular manifestation. Regarding pathophysiology, the neuronal mechanisms are identical for both NCSE and CSE, but they spare the primary motor neurons. Permanent hyperexcitability may damage the neurons involved in NCSE the same way as the motor neurons in CSE. However, NCSE is spared from life-threatening secondary pathophysiological sequelae of CSE (lactic acidosis, respiratory exhaustion, rhabdomyolsis, etc.). Nevertheless, autonomic dysregulation (arrhythmias [ventricular tachycardia/asystolia], apneas) may also expose the patient to substantial acute risks. There are myriads of causes for NCSE and they are mainly medication errors (insufficient adherence or addition of new drugs with interactions) in patients with known epilepsy. In these patients and those without known epilepsy, other causes include metabolic, toxic, structural (tumors, hemorrhagies, ischemias), infectious, inflammatory, and autoimmune causes. Thus, an extensive search for the cause of the NCSE is germane because the immediate and correct therapy of the underlying cause, especially of the acute symptomatic forms of NCSE is at least as important as antiictal treatment.

Schlüsselwörter

nicht-konvulsiver Status epilepticus - Koma - Enzephalopathie - Wesensveränderung - Elektroenzephalografie (EEG)

Key words

non-convulsive status epilepticus - coma - encephalopathy - behavioral changes - electroencephalography (EEG)

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