Microangiopathy of the brain, retina, and ear: improvement without immunosuppressive therapy.

Abstract

We describe a pregnant young woman with branch retinal arteriolar occlusions, encephalopathy, and hearing loss in whom we demonstrated a patent foramen ovale. She improved while receiving anticoagulants and no immunosuppressive therapy. The microangiopathic syndrome of retinopathy, encephalopathy, and deafness may be due to a disturbance of coagulation and/or microembolism. (Stroke 1991;22:933-937) S ince 1973 several reports have described a "microangiopathy" causing retinopathy, encephalopathy, and deafness in young women.

Skin involvement in Susac's syndrome

“…Brain biopsy can show microinfarctions, endothelial thickening and/or damage and a mild perivascular lymphocytic infiltration [8]. Patients sometimes spontaneously improve [9,10]. Yet, permanent deafness, blindness or dementia may persist [11].…”

Susac's syndrome: A case of simultaneous development of all three components of the triad

“…Since the disease has nonspecific clinical manifestations, diagnosis is difficult and often delayed [2][3][4][5][6][7][8][9]. The clinical patterns are variable such as headache, dementia, hemiplegia, seizures, cranial palsies, hearing loss, vertigo, tinnitus and diminution or loss of vision [10][11][12].…”

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

“…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.…”

“…The syndromehas the nature of a self-limiting course, although therapeutic trials are usually required to prevent exacerbation, and for the purpose of avoiding complicating permanent deficits. To dater hyperbaric oxygen (12,19), dextran (9), plasmapheresis (10, 1 1), high-doses of intravenous immunoglobulin (10), or various kinds of drugs such as corticosteroid (1, 3, 6, 7, ll, 15, 19), cyclophosphamide (3, 7, 10, ll), warfarin (8), aspirin (8,9,13), and nimodipine (13) have been used in treatment. Our review of the literature indicates that early treatment with corticosteroids seems to be the most useful to prevent the progression of the disease.…”