IMAGES IN NEUROLOGY
|Year : 2022 | Volume
| Issue : 6 | Page : 1177-1178
Milky way sign in progressive multifocal leukoencephalopathy
Ayush Agarwal1, Divyani Garg2, Shiny Joy1, Ajay Garg3, Achal K Srivastava1
1 Department of Neurology, All India Institute of Medical Sciences, New Delhi, India
2 Department of Neurology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India
3 Department of Neuroradiology, All India Institute of Medical Sciences, New Delhi, India
|Date of Submission||28-Jul-2022|
|Date of Decision||30-Aug-2022|
|Date of Acceptance||01-Sep-2022|
|Date of Web Publication||3-Dec-2022|
Department of Neuroradiology, All India Institute of Medical Sciences, New Delhi
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Agarwal A, Garg D, Joy S, Garg A, Srivastava AK. Milky way sign in progressive multifocal leukoencephalopathy. Ann Indian Acad Neurol 2022;25:1177-8
A 42-year-old gentleman with no prior comorbidities presented with the sub-acute onset and progressive weakness and numbness over the right half of his body and face over 6 weeks associated with slurring of speech with impaired comprehension. Examination revealed mild sensory aphasia with right sensorimotor hemiplegia, right upper motor neuron facial palsy, and sensory loss over the right half of the face. Magnetic resonance imaging brain revealed multiple well-defined hyperintense lesions in T2 Weighted Imaging (T2-WI) and FLAIR- Fluid Attenuated Inversion Recovery (FLAIR) images in the subcortical and deep white of both cerebral hemispheres with multiple peripheral foci of satellite lesions, a Milky Way appearance. No mass-effect, diffusion restriction, or hemorrhagic foci were seen. Following contrast administration, multiple satellite lesions showed punctate enhancement [Figure 1].
|Figure 1: Axial FLAIR images (a–c) shows multiple well-defined hyperintense lesions in the subcortical and deep white matter of both cerebral hemispheres with multiple peripheral foci of satellite lesions (small arrows), a Milky Way appearance. Post-gadolinium axial T1-WIs (d–f) images show punctate enhancement in multiple satellite lesions|
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His routine investigations were normal. Serum testing for neuromyelitis optica spectrum disorders, myelin oligodendrocyte glycoprotein, anti-nuclear antibody, rheumatoid arthritis, angiotensin-converting enzyme, and other connective tissue disorders was non-revealing. Cerebrospinal fluid (CSF) examination revealed 5 cells (L100), raised protein (112 mg/dl) and normal sugar levels. The remaining CSF examinations were normal. Whole-body positron emission tomography scan revealed areas of hypometabolism in the left fronto-parietal and right parieto-occipital region without evidence of hypermetabolism elsewhere and serum paraneoplastic panel, viral markers, and tumor markers were negative. CSF for John Cunningham virus was also sent, which came positive. He was diagnosed to have immunocompetent progressive multifocal leukoencephalopathy (PML). His CD4 count was 375/mm3. The patient's attendants were counseled about the disease, and he was started on mirtazapine 15 mg daily.
The Milky Way sign indicates multiple punctate contrast-enhancing lesions surrounding the main lesion, which may not be seen without contrast imaging. This is reminiscent of the stars of our galaxy Milky Way which, although individually present, are usually missed in the white band formed by the merging of the light of innumerable stars across the night sky., Although usually seen in PML, it can be seen in other disorders like central nervous system vasculitis, neurosarcoidosis, and Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS). Immunocompetent PML, although rare, has been previously reported and should be considered as a differential in patients presenting with demyelinating lesions.
Written informed consent was taken from the patient
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Wijburg MT, Witte BI, Vennegoor A, Roosendaal SD, Sanchez E, Liu Y, et al
. MRI criteria differentiating asymptomatic PML from new MS lesions during natalizumab pharmacovigilance. J Neurol Neurosurg Psychiatry 20126;87:1138-45.
Hodel J, Darchis C, Outteryck O, Verclytte S, Deramecourt V, Lacour A, et al
. Punctate pattern: A promising imaging marker for the diagnosis of natalizumab-associated PML. Neurology 2016;86:1516-23.
Sinnecker T, Hadisurya J, Schneider-Hohendorf T, Schwab N, Wrede K, Gembruch O, et al
. Extensive immune reconstitution inflammatory syndrome in Fingolimod-associated PML: A case report with 7 Tesla MRI data. BMC Neurol 2019;19:190.
Naess H, Glad S, Storstein A, Rinaldo CH, Mork SJ, Myhr K-M, et al
. Progressive multifocal leucoencephalopathy in an immunocompetent patient with favourable outcome. A case report. BMC Neurol 2010;10:32.