Das Adamantiadis-Behçet-Syndrom: Fluoreszenzangiographie und choroidale Minderperfusion*

Abstract

Apart from retinal vasculitis, 43% of eyes in patients with Adamantiadis-Behçet's disease presented a delayed choroidal filling in fluorescence angiography as a sign of choroidal involvement of occlusive vasculitis. We observed leakage of fluorescein from the optic disc, which could be due to a secondary inflammation of the ciliary circulation. Inflammatory involvement predominantly of choroidal vessels, as visualized in fluorescence angiography, may be a diagnostic lead in Adamantiadis-Behçet's disease.

Ocular Manifestation of Non-Parenchymal Neuro-Behçet Syndrome

“…We postulate that the persistent occipital headache was a result of the thrombosis, which produced increased intracranial pressure. The clinical findings supported by fluorescein angiography of bilateral optic disc edema associated with retinal, as well as optic disc vascular leakage unequivocally documented relapsing background inflammation in our case [5,6]. Dalteparin and a steroid were administered to treat the transverse sinus thrombosis and to complement the anti-inflammatory effect of Colchicine.…”

“…Unremitting headaches and new visual impairment in a patient with rheumatic syndrome, in our case Behçetʼs disease, are imperative leads to in-depth analysis of the intraocular and cerebral blood supply. Fluorescein angiography, which is a method of choice for following ophthalmic-Behçet [5], depicts sharp estimates of the evolving ocular angiitis, while in our experience it also provided important diagnostic clues for the systemic disease. MR imaging is invaluable in Neuro-Behçet syndrome alone.…”

Epidemiologie der Augenbeteiligung beim Morbus Adamantiades-Behçet

“…Hierbei stand die Entwicklung einer Katarakt bei 37% der Augen im Vordergrund. Andere Komplikationen, die wir beobachteten, waren die Entwicklung chorioretinaler Narben (36%), der Verschluss retinaler Gefä-ße (29%), eine Makulopathie (27%) und in 9% ein chronisches Glaukom, wobei bei den Letzteren die Zahlen in der Literatur zwischen 1 und 43% schwanken [8,16,19,[25][26][27].…”

A Historical Review of Early Descriptions of Adamantiades-Behçet's Disease

“…Recurrent oral and genital aphthous ulcers, hypopyon-iritis, sterile pleuritis (one male and one female patient) Knapp (1938/39) Basel Recurrent mucocutaneous lesions, hypopyon-iritis, neurologic manifestation (one female patient) Rossochowitz and Metze, 1970), Jebejian from Syria (Jebejian, 1979), Daghfous from Tunisia (Daghfous et al, 1980), Theoharis from France (Theoharis et al, 1988), Panayi from the U.K. (Choy et al, 1993), Wang from Japan (Wang et al, 1997), Freitas from Portugal (Freitas et al, 1998), Wenkel, Glosemeyer, and Stadler from Germany (Krist et al, 2000;Glosemeyer et al, 2001), Foster from the U.S.A. (Afshari et al, 2001), among others, who got access to the work of both authors, added the name of Adamantiades to that of Behc Ëet. The term ``Adamantiades±Behc Ëet's disease'' honors both ®rst describers of the several manifestations constituting an autonomous disease in modern times and is therefore advisable for naming this disorder.…”