[Adamantiadis-Beh  et syndrome: XXXX angiography and choroid ischemia].

@entity1 @entity32 @entity2194 @entity66 @entity1333 @entity7809 @entity262 @entity3193 @entity74 @entity432 @entity941 @entity18773 @entity65 @entity4816
BACKGROUND: Adamantiadis-Beh  et's disease is a chronically progressing @entity66 . The underlying disease mechanism is an obliterative @entity1333 of unknown etiology. Main clinical symptoms are @entity7809 and intraocular @entity32 . Additionally, cutaneous, @entity65 manifestations may be observed. Diagnosis is based on clinical features since currently no specific laboratory tests or pathognomonic histopathological features are available. Ocular changes may provide important diagnostic clues to the @entity262 . @entity1 AND METHODS: In a retrospective fashion 196 @entity1 of the Department of Ophthalmology of the University of Erlangen-N  rnberg between 1988 and 1998 with @entity4816 seen by fluorescence angiography were evaluated according to the diagnostic criteria of the "Beh  et's Disease Research Committee of Japan". RESULTS: Among 196 @entity1 there were 12 @entity1 with Adamantiadis-Beh  et's disease. Apart from @entity4816 , angiographic features included capillary dropout in 64% (9/14), swelling of the optic disc in 79% (11/14) and irregularly delayed @entity18773 of choriocapillaris in the early phase of fluorescence angiography in 43% (6/14) of eyes. There was no statistically significant relationship between severity of the @entity74 and the activity of the @entity2194 . CONCLUSION: Apart from @entity4816 , 43% of eyes in @entity1 with Adamantiadis-Beh  et's disease presented a delayed @entity3193 in fluorescence angiography as a sign of @entity432 . We observed leakage of @entity941 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-Beh  et's disease.
