Microangiopathy with encephalopathy, hearing loss and retinal arteriolar occlusions: Two new cases

Treatment of Susac syndrome with gamma globulin and corticosteroids

“…However, occasional reports describe the arteriolar microangiopathy that we observed, including adventitial thickening, endothelial swelling and an accompanying perivascular mononuclear inflammatory infiltrate [2]. The prominent staining of basement membrane and reticulin is consistent with electron microscopic studies showing thickening basement membrane [6].…”

Treatment of Susac's syndrome

“…At least 11 reports on SS have included brain biopsy results [1,5,[6][7][8][9][10][11][12][13][14]. Most have described small foci of necrosis (microinfarcts) within the cerebral cortex and white matter, with loss of neurons, axons, and myelin.…”

Susac's Syndrome: Beneficial Effects of Corticosteroid Therapy in a Japanese Case.

“…Varying degrees of headache and of the fnigraine type, or a non-throbbing nature, are recognized as prodromal symptoms. Ten previous brain biopsies (2,5,15) showed findings of microinfarctions, or perivascular inflammatory infiltrates of small vessels mimicking active small vessel angitis without fibrinoid necrosis. In addition to histopathologic characteristics, the presence of steroid responsive multiple lesions with enhancement on MRIshare a similar pattern to that of isolated central nervous system angiopathy (20).…”

“…From an epidemiological aspect, this syndrome is a very rare disorder (1), with all previously reported cases being Caucasians (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19). A recent review (16), however, indicates that this syndromeis more common than previously thought.…”