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|Year : 2016 | Volume
| Issue : 6 | Page : 1376-1377
The butterfly mystery
Soumya Sharma1, Anu Gupta1, Gulab Soni1, Ravindra K Saran2, Arvind K Srivastava3, Vinod Puri1
1 Department of Neurology, Govind Ballabh Pant Institute of Postgraduate Medical Education and Research, New Delhi, India
2 Department of Pathology, Govind Ballabh Pant Institute of Postgraduate Medical Education and Research, New Delhi, India
3 Department of Neurosurgery, Govind Ballabh Pant Institute of Postgraduate Medical Education and Research, New Delhi, India
|Date of Web Publication||11-Nov-2016|
Dr. Vinod Puri
Department of Neurology, Govind Ballabh Pant Institute of Postgraduate Medical Education and Research, Jawaharlal Nehru Marg, New Delhi - 110 002
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Sharma S, Gupta A, Soni G, Saran RK, Srivastava AK, Puri V. The butterfly mystery. Neurol India 2016;64:1376-7
A 56-year-old woman presented with a 4-week history of headache, vomiting, and progressive bilateral vision loss; and, a 3-week history of left upper/lower limb weakness, altered behavior, and urinary incontinence. On examination, she was confused, had only perception of light in both eyes, papilledema and bi-pyramidal signs (left > right). Gadolinium enhanced magnetic resonance imaging (MRI) of the brain revealed a large peripherally enhancing bi-parietal mass crossing the corpus callosum with mass effect and peripheral diffusion restriction [Figure 1]a-f. Stereotactic biopsy of the lesion revealed active demyelination [Figure 2]a-d. She was given intravenous methylprednisolone 1 gram daily for 5 days followed by oral steroids tapered over 2 months. She had a remarkable improvement in all symptoms with final visual acuity of 6/24 bilaterally.
|Figure 1: Magnetic resonance imaging of the brain. (a and b) T2-weighted and fluid-attenuated inversion recovery axial images show an hyperintense lesion involving corpus callosum; (c) Diffusion weighted images and (d) apparent diffusion coefficient maps show peripheral diffusion restriction. (e and f) Patchy peripheral contrast enhancement seen on T1 post contrast coronal and axial images|
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|Figure 2: Pathology. (a) Luxol Fast Blue stain demonstrates relatively lighter stained area (arrow) corresponding to myelin loss. (b) Hematoxylin and eosin stained sections show a large number of foam cells (arrow). (c) Glial fibrillary acidic protein stain shows reactive astrocytes without nucleomegaly or pleomorphism. (d) neurofilament stain shows both preserved and broken axons|
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Demyelinating pseudotumor can clinically and radiologically mimic a mass lesion. The lesion is usually single and well-circumscribed but may rarely cross the corpus callosum resulting in a “butterfly pattern.”, The differential diagnoses of such a pattern include glioblastoma multiforme, lymphomas with rare reports of progressive multifocal leukoencephalopathy, metastasis, toxoplasmosis, and neuronal ceroid lipofuscinosis presenting similarly.
Certain characteristic MRI features of demyelinating pseudotumor are the presence of a large sized lesion with minimal mass effect, open ring enhancement (incomplete portion on the gray matter side), T2 hypointense rim, peripheral diffusion restriction, and venular enhancement. However, the diagnosis usually comes from a brain biopsy. Eighty percent patients show a significant response to corticosteroids. Thus, demyelination should be considered in the differential diagnosis of such lesions, as it is potentially treatable and carries a relatively favorable prognosis.
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[Figure 1], [Figure 2]