Death following rapidly progressive demyelinating disorder in a…

Multiple sclerosis (MS) is a debilitating, demyelinating disease of the central nervous system, with manifestations ranging from numbness and blindness to paralysis. Typical MS is a slowly progressive demyelinating disease, causing significant morbidity spanning over many years. In contrast, “Marburg’s disease” is a rare variant of MS which demonstrates a malignant monophasic disease progression leading to death within weeks to months. We present a rapidly fatal demyelinating disease with the clinicopathological findings on par with the handful of reported cases of “Marburg’s disease” in the literature. A previously healthy 30-year-old mother of two children was extensively investigated for focal neurological signs succumbing to death 5 weeks after the onset. Antemortem investigations for tuberculosis, autoimmune diseases, and viral studies were negative. Magnetic resonance imaging of the brain showed hyperintense lesions with contrast enhancement compatible with MS. Histopathologic examination confirmed numerous inflammatory and demyelination foci scattered throughout the brain and brain stem predominantly involving the white matter. There were extensive perivascular inflammatory cell cuffs containing lymphocytes admixed with histiocytes. Also, a few foci of vasculitis with fibrinoid necrosis, mediated by lymphocytes and neutrophils were noted associated with parenchymal haemorrhages. Considered a rare variant of MS, the case of Marburg’s disease presented here shows an infrequent association with active vasculitis and haemorrhage, described only a few times in the literature. This wide spectrum of rapidly fatal demyelinating diseases consisting of rare variants with overlapping clinicopathological features makes diagnosis challenging. Therefore, it is important to correlate clinical-radiological and histopathological findings to arrive at an accurate final diagnosis.

Not applicable.

MS:

Multiple sclerosis

GCS:

Glasgow coma scale

pANCA:

Perinuclear antineutrophil cytoplasmic antibodies

cANCA:

Antineutrophil cytoplasmic antibodies

ANA:

Antinuclear antibody

ANF:

Anti-nuclear factor

HIV:

Human immunodeficiency virus

EBV:

Epstein bar virus

HSV:

Herpes simplex virus

CSF:

Cerebrospinal fluid

IgG:

Immunoglobulin G

USS:

Ultrasound scan

NCCT:

Non-contrast computed tomography

CECT:

Contrast-enhanced computed tomography

MRI:

Magnetic resonance imaging

GFAP:

Glial fibrillary acidic protein

Department of Forensic Medicine, University of Peradeniya, Peradeniya, Sri LankaC. U. Wickramasinghe & D. M. G. Fernando

Department of Pathology, University of Peradeniya, Peradeniya, Sri LankaS. Wijetunge & H. R. S. D. Sumanasekara

CW: conception, acquisition, drafting, revising. DF: analysis, revising. SW: histopathological analysis and special stains, revising. SH: drafting and revising. All authors read and approved the final manuscript.

Correspondence to C. U. Wickramasinghe.

Not applicable.

Written informed consent was obtained from the next of kin of the deceased (husband) for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.

The authors declare no competing interests.

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Wickramasinghe, C.U., Fernando, D.M.G., Wijetunge, S. et al. Death following rapidly progressive demyelinating disorder in a young female—a case report. Forensic Sci Med Pathol 19, 86–90 (2023). https://doi.org/10.1007/s12024-022-00564-4

Accepted17 November 2022

Published28 November 2022

Issue DateMarch 2023

DOIhttps://doi.org/10.1007/s12024-022-00564-4