The etiology of this condition remains obscure, but its resemblance to other inflammatory-demyelinating diseases should be emphasized. The similarities of the histologic changes to those of disseminated encephalomyelitis, noted above, suggest that the two diseases are related forms of the same fundamental process. In fact, cases combining both types of pathologic changes have been described (Fisher et al). It is noteworthy that, among the small number of patients who have recovered from what appeared to be a typical necrotizing hemorrhagic encephalitis, a few have gone on to develop typical MS.
Treatment of Hurst Disease
High-dose intravenous corticosteroids are generally used in the treatment of acute necrotizing hemorrhagic encephalopathy and can produce a favorable result. The use of plasma exchange and intravenous immunoglobulin, as for ADEM, can apparently be successful when instituted early.
This form of brain inflammation, pertinent to the special circumstance of bone marrow transplantation, is included here for lack of a better category with which to align it. Months or years after transplantation, subacute hemiparesis, seizures, behavioral changes, or ataxia arise and may be attributed to PML, a viral infection of the white matter (Chap. 33), or another viral process that is known to occur with circumstances of immunosuppression. The MRI shows white matter lesions that conform to an MS-like periventricular orientation or a more confluent leukoencephalopathy. A patient under our care demonstrated lesions in the splenium of the corpus callosum that extended into the adjacent centrum semiovale (Fig. 35-6). Several reports emphasize a mild vasculitis in the territory of the white matter lesions (Padovan et al). Almost all affected patients have concurrently displayed a tender erythematous, macular rash that is typical of acute graft-versus-host disease. There are also rare, but well-characterized, neuromuscular complications of graft-versus-host disease (Chap. 43).
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