Akute Immunneuropathie und Optikusneuritis – Übersicht und Fallbericht eines seltenen Überlappungssyndroms

Zusammenfassung

Die vorliegende Arbeit gibt einen Überblick über nosologische, pathomechanistische und therapeutische Aspekte von kranialen Überlappungssyndromen akuter Immunneuropathien. Eine 18-jährige Patientin entwickelte eine bilaterale faziale Parese, akrodistale Parästhesien und Sehstörungen nach einem gastrointestinalen Infekt. Kraft, Koordination und Muskeleigenreflexe der Extremitäten waren regelrecht. Bei Kontrastmittelaufnahme mehrerer Hirnnerven sowie von Konus- und Kaudafasern, zytoalbuminärer Dissoziation im Liquor und Demyelinisierungszeichen in der Elektroneurographie diagnostizierten wir eine akut-inflammatorische demyelinisierende Polyradikuloneuritis mit Neuritis nervi optici. Die Beschwerden der Patientin sprachen gut auf eine Plasmapherese an. Auch bei Patienten mit Hirnnervenparesen und Parästhesien ohne Hyporeflexie oder Extremitätenschwäche sollte an eine akute Immunneuropathie gedacht werden. Kombinationen mit zentraler Affektion wie der Optikusneuritis sind dabei möglich.

Abstract

The present work provides an overview of nosological, pathomechanistic and therapeutic aspects of cranial overlap syndromes of acute immune-mediated neuropathies. An 18-year-old woman developed bilateral facial palsy and hypesthesia, acrodistal paresthesia as well as impaired vision after a gastrointestinal infection. Muscle power and coordination were unaffected and deep tendon reflexes were preserved. Based on contrast enhancement of cranial nerves II, IV, V and VII as well as conus and cauda fibers, albuminocytologic dissociation in the cerebrospinal fluid and electroneurographic signs of demyelination, we diagnosed acute-inflammatory demyelinating polyradiculoneuropathy with optic neuritis. The patient’s complaints responded well to plasmapheresis. Even in patients with cranial nerve palsies and paresthesias without diminished deep tendon reflexes or extremity weakness, one should consider the possibility of acute immune-mediated neuropathy. A coincident central affection such as optic neuritis is possible.

Schlüsselwörter

Inflammatorische Neuropathie - Diplegia facialis - Parästhesie - Polyneuritis cranialis - Zytoalbuminäre Dissoziation

Keywords

Inflammatory neuropathy - paresthesia - albuminocytologic dissociation - bifacial weakness - polyneuritis cranialis

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