Immune-mediated conditions affecting the brain, eye and ear (BEE syndromes)

Abstract

The triad of central nervous system symptoms, visual disturbance and hearing impairment is an oft-encountered clinical scenario. A number of immune-mediated diseases should be considered among the differential diagnoses including: Susac syndrome, Cogan syndrome or Vogt-Koyanagi-Harada disease; demyelinating conditions such as multiple sclerosis or neuromyelitis optica spectrum disorder; systemic diseases such as systemic lupus erythematosus, Sjögren syndrome or Behcet disease and granulomatous diseases such as sarcoidosis. In this article, we coin the term ‘BEE syndromes’ to draw attention to the various immune-mediated diseases that affect the brain, eye and ear. We present common disease manifestations and identify key clinical and investigation features.

A treatable cause of vertigo

“…Finally, an MR scan of brain with gadolinium might identify characteristics that suggest the cause, for example, nodular meningeal thickening in neurosarcoidosis or callosal lesions in Susac’s syndrome. In people with possible autoimmune inner-ear disease, clinicians should consider causes of the ‘brain–eye–ear syndrome’ 3 (Table 2). While autoimmune vasculitis cannot be seen in the living ear, it can be seen in the eye.…”

Seltene Erkrankungen des vestibulären Labyrinths: von Zebras, Chamäleons und Wölfen im Schafspelz

“…Dieser entscheidet über weitere Zusatzdiagnostik wie z. B. eine Lumbalpunktion (Nachweis von oligoklonalen Banden, intrathekaler Antikörperproduktion, Antikörper gegen neurotrope Bakterien und Viren, Tumorzellen bei Meningeosis carcinomatosa) und [287,289,290]). Für Details zu den Laborbefunden siehe Tab.…”

“…8 Labordiagnostik bei V.a. autoimmune Innenohrerkrankung (AIED) (nach[284,[287][288][289][290]). Wirkung auch bei normalen Serumspiegeln und noch nach Abschluss der Behandlung entfalten[334].…”

Menière’s disease

“…In Cogan’s syndrome, the characteristic ophthalmological finding is of non-syphilitic interstitial keratitis. 5…”