We have used a PCR-based technology to study the V185 and V1317 repertoire of T-cell populations in HLA-DR2 multiple sclerosis (MS) patients. We have found that the five MS DR2 patients studied present, at the moment of diagnosis and prior to any treatment, a marked expansion of a CD4+ T-cell population bearing Ve ¶35-J.81. The characterization of T cells involved in MS is crucial to the description of the disease. Several strategies have been used to identify possible biases in the T-cell repertoire of MS patients. They include flow cytometry analysis, extensive cell cloning, and determination of the nucleotide sequences of the T-cell receptor (TcR) of T cells either present at the site of the brain lesions or proliferating on brain autoantigens. T cells reacting against myelin basic protein (MBP) are found both in the CNS and in the peripheral blood lymphocytes (PBLs). A recent review of the sequences of the TcR of MBP-reactive cells reported the absence of an association of specific VP3 and Jo3 with MS (4). However, restriction at the major histocompatibility complex was not taken into account in this review. In view of the association of MS with HLA-DR2, VP3 usage and complementarity-determining region 3 (CDR3) diversity of the TcR could be considered in their relationship with the DR type of the patients (5, 6). The techniques mentioned above tell which V,3 and which CDR3 sequence is used in the sample studied. However, they cannot tell if a T-cell clone characterized by the distinctive 1 chain of its TcR is expanded in vivo. To determine if a T-cell population is expanded in an MS patient, we have used a new PCR-based technology that generates a "snapshot" of the T-cell populations at the clonal or oligoclonal level and that tells, on the basis of VP, Jo3, and CDR3 length, which population of T cells is expanded at the moment the sample is taken (7).We have restricted our study of the T-cell repertoire in MS to genetically defined MS patients (patients and controls were all HLA-DR2). Also, to avoid the consequences of treatment on the diversity of the T-cell repertoire, we have recruited HLA-DR2 MS patients at the moment of diagnosis and prior to any treatment. In view of the large number of signals that could be generated from a unique blood sample (7) not all V1-J1 combinations could be studied. Several V,B segments other than V135 and Vf17 are used by autoreactive peripheral T cells in MS DR2 patients (8). However, the T cells infiltrating the CNS in DR2 MS patients have been found to be enriched in V,5.2/5.3+ T cells (5) and their main targets are CNS self proteins: MBP, proteolipid protein, and myelin oligodendrocyte glycoprotein (9, 10). Also, some authors have reported that T-cell clones responding to MBP use predominantly VP35.2/5.3 (6). Finally, DR2-restricted T-cell clones that proliferate with the peptide 84-102 of MBP are predominantly V,B17+ (11). We have therefore restricted the present study to the V,B5+ and V/317+ repertoires.We (12). The patients were 40 years old on the average a...

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anisms of the disease.Five of the 15 patients initially treated with steroids with or without other immunosuppressors deteriorated during the treatment.'^^^^ Although these data may suggest that immunosuppressor therapy is not effective in this disorder, the rapid improvement of patient 2 i n our study with intravenous immunoglobulins would support another explanation; a s in rapidly progressive myasthenia gravis, the standard oral immunosuppressor therapy does not act fast enough to prevent the usually subacute progression of the disorder. In a previous patient, the treatment with immunoglobulins did not change the progression of the disease, but the patient was reported in an abstract and the dose of immunoglobulins and the time when the treatment was given are not known.7In conclusion, the good response of patient 2 in our study to high-dose intravenous immunoglobulins suggest that this treatment should be tried in other patients with this syndrome. References 1. Susac JO, Hardimann JM, Selhorst JB. Microangiopathy of the brain and retina. Neurology 1979;29:313-316. 2. Susac JO. Susac's syndrome: the triad of microangiopathy of the brain and retina with hearing loss in young women. Neurology 1994;44:591-593. 3. Kaminska EA, Sadler M, Sangalang V, Hoskinmott A, Silverberg D. Microangiopathic syndrome of encephalopathy, retinal vessel occlusion, and hearing loss [abstract]. Can J Neurol Sci 1990;17:241. 4. Monteiro MLR, Swanson RA, Coppeto JR, Cuneo RA, DeArmond SJ, Prusiner SB. A microangiopathic syndrome of encephalopathy, hearing loss, and retinal arteriolar occlusions. Neurology 1985;35:1113-1121. 5. Bogousslavsky J, Gaio J-M, Caplan LR, et al. Encephalopathy, deafness and blindness in young women: a distinct retinocochleocerebral arteriopathy? J Neurol Neurosurg Psychiatry 1989;52:43-46. 6. Petty GW, Yanagihara T, Bartleson JD, Younge BR, Mokri B. Retinoeochleocerebral vasculopathy [abstract]. Ann Neurol 1991;30:245. 7. Manor RS, Ouaknine L, Ouaknine G. Susac-RED syndrome [abstract].

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