In response to the letter by Jackson and Lennox, we have no objections to the use of the term isolated angiopathy of the central nervous system (IACNS) in cases where no histologic tissue specimen was obtained.It should be noted that prior to the publication of our article,1 the literature recommended treatment for IACNS diagnosed by means of angiography with a combination of corticosteroids and cyclophosphamide.2 Our conclusion was that such aggressive treatment is not warranted initially, as most patients will respond to treatment with only corticosteroids. Although Hankey3 concluded from his literature review (note that he had no personal experience) that angiography was normal in 46% of the subset of patients that had both biopsy and angiography, this clearly was not our experience. In all 11 patients referred to us in whom we suspected IACNS, the angiogram was diagnostic for this disease. Perhaps this
Crane R, Kerr LD, Spiera H. Isolated Angiopathy of the Central Nervous System-Reply. Arch Intern Med. 1992;152(10):2145. doi:10.1001/archinte.1992.00400220144033
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