Weston Hurst hemorrhagic leukoencephalitis: A novel association with mixed connective tissue disease: uncloaking the “unholy” etiology underneath

Background

Weston Hurst syndrome (WHS), also known as acute hemorrhagic leukoencephalitis (AHLE), has not been previously associated with mixed connective tissue disease (MCTD), a rare rheumatologic disorder.

Case presentation

We report a case of a 35-year-old female who presented with deep coma with features of previously undiagnosed MCTD and responded favorably to intravenous immunoglobulin (IVIG) therapy. On detailed historical enquiry, clinical examination, and investigation, AHLE was diagnosed. Furthermore, our patient showed significant improvement with IVIG administration, even though initial steroid therapy was unsuccessful.

Conclusion

AHLE, an associated novel neurological manifestation of MCTD, can be responsive to IVIG, which makes this case reportable from the diagnostic as well as the therapeutic point of view.