Case report: Progressive multifocal leukoencephalopathy with prominent medullary veins on angiogram

doi:10.1016/S0009-9260(05)82920-4

Clinical Radiology

Volume 49, Issue 1, January 1994, Pages 66-68

https://doi.org/10.1016/S0009-9260(05)82920-4 Get rights and content

A case of progressive multifocal leukoencephalopathy associated with systemic lupus erythematosus is presented. Angiography revealed early filling of prominent medullary veins in the region of the lesion, suggesting active demyelination

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Cited by (13)

COVID-Related Leukoencephalopathy: Unusual MRI Features and Comparability to Delayed Post Hypoxic Ischemic Encephalopathy
2022, Radiology Case Reports
Citation Excerpt :
The MR findings of symmetric and diffuse white matter changes with restricted diffusion matches the recently reported findings in COVID-related leukoencephalopathy, attributing it to delayed post hypoxic injury [4,5]. However, prominent medullary veins is an unusual observation and rarely reported in diffuse leukoencephalopathy [10]. Case 1: A 43 years old COVID positive healthy male presenting with mild symptoms but moderate burden of disease on chest imaging.
COVID-19 related leukoencephalopathy can be multifactorial given the systemic effects of the viral disease. We present couple of cases with typical clinico-imaging stigmata of COVID-19 resulting in severe respiratory insufficiency. MR brain imaging revealed confluent diffuse supratentorial white matter T2 hyperintensity with restricted diffusion during the sub-acute course of the disease. The MR imaging pattern of leukoencephalopathy was non-specific but more comparable to delayed post-hypoxic leukoencephalopathy (DPHL) as also previously reported in COVID-19. Interestingly, T2 imaging showed unusual but peculiar finding of “accentuated medullary veins” in the superficial zones. No dural venous sinus thrombosis or micro-hemorrhages were present to explain “dots and stripes” due to dilated medullary veins. The patho-mechanism of this findings is not clear but may possibly be related to demyelination as DPHL has shown to be a demyelinating process. We present a review of COVID-related leukoencephalopathy with discussion on hypoxia-induced demyelinating process with accentuated medullary veins as possible associated marker.
The emergence of progressive multifocal leukoencephalopathy (PML) in rheumatic diseases
2008, Journal of Autoimmunity
Citation Excerpt :
A 55-year-old man with CNS vasculitis who was placed on imunosuppression and subsequently developed PML (with JC virus detected in the CSF via PCR) was also identified upon literature review [56]. There were 24 patients with SLE found in the literature [56–76] and described by Calabrese et al. [1] that developed PML. All had been on immunosuppressive drugs, and their average age was 43.
Progressive multifocal leukoencephalopathy (PML) is a rare and devastating neurological disease with areas of demyelination in the central nervous system classically associated with profound imunosuppression. PML is caused by reactivation of latent JC virus, leading to the death of myelin-producing oligodendrocytes typically with a rapidly fatal outcome. Once seen primarily in severely immunosuppressed states including lymphoma, solid organ malignancies, and organ transplant recipients, PML became an AIDS-defining illness in the 1980s. PML has now emerged as a catastrophic illness in multiple sclerosis with biologic drug therapy (natalizumab) and reported in rheumatic diseases with and without biologic therapeutic agents. With current and future treatments that suppress and manipulate the immune system, there is risk for severe acute infections and reactivation of latent infections, such as JC virus reactivation leading to PML. It is critical, therefore, to proceed cautiously when immune system modification strategies are being evaluated for fear of unleashing undesirable or even fatal diseases. Fortunately this complication remains a rare event.
Imaging of systemic lupus erythematosus: State of the art
2004, Systemic Lupus Erythematosus, Fourth Edition
Drug-associated progressive multifocal leukoencephalopathy: a clinical, radiological, and cerebrospinal fluid analysis of 326 cases
2016, Journal of Neurology
Progressive multifocal leucoencephalopathy associated with lupus and methotrexate overdose
2008, Lupus
Progressive multifocal leukoencephalopathy in rheumatic diseases: Evolving clinical and pathologic patterns of disease
2007, Arthritis and Rheumatism

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Case reportCase report: Progressive multifocal leukoencephalopathy with prominent medullary veins on angiogram

Progressive multifocal leukoencephalopathy in patients with AIDS: appearance on MR images

Radiology

Progressive multifocal leukoencephalopathy: clinical and radiologic features

Annals of Neurology

MR imaging in progressive multifocal leukoencephalopathy

American Journal of Neuroradiology

Progressive multifocal leukoencephalopathy: presenting as focal mass lesion in the brain

Surgical Neurology

Computed tomography in white-matter disease

Radiology

Case report
Case report: Progressive multifocal leukoencephalopathy with prominent medullary veins on angiogram