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To the Editor:
I read with interest the report of a lethal capillary leak syndrome (CLS) after a single administration of interferon beta-1b.1 It clearly points out the difficulty in diagnosing MS without oligoclonal IgG-bands in CSF. Monoclonal gammopathy of unknown significance (MGUS) should have prompted further investigation of possible causes for the presenting disease and MRI findings, which might have suggested MS.2 As reported white matter lesions were found, autopsy findings described disseminated infarctions, probably also in gray matter, and there was no evidence of primary demyelination. This is an impressive example of the imprecision of MRI in identifying MS. Another link in the fatal misdiagnosis might have been the partial recovery after methylprednisolone therapy.
The patient had MGUS of the IgG kappa type and obviously acquired C1-inhibitor (C1-INH) deficiency. There was no history of angioedema …
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