Highlights
- •Tumefactive demyelination is any CNS demyelinating lesion that appears tumor-like.
- •Specific imaging characteristics help distinguishing it from tumor or abscess.
- •It can be caused by a variety of disorders, including multiple sclerosis.
- •A paraneoplastic syndrome should always be excluded as a cause.
- •Seminoma is a possible cause of paraneoplastic tumefactive demyelination.
Abstract
Background
Tumefactive demyelination presents as an aggressive, fast progressive focal demyelinating
lesion in the central nervous system, with often devastating clinical outcome if not
acutely treated. Correct and early treatment is threatened by its real diagnostic
challenge.
Case report
We describe a 47-year-old man with tumefactive demyelination and testicular seminoma.
He presented with aphasia, cognitive impairment and right sided weakness of the arm
and lower face. MRI revealed a lesion characteristic of tumefactive demyelination.
Cerebral biopsy confirmed this diagnosis. In addition, a testicular seminoma was diagnosed.
Temporal association of demyelination and malignancy was highly suggestive for a paraneoplastic
syndrome. He responded well to corticosteroid therapy plus orchiectomy, but a behavioral
disturbance remained.
Discussion
A good knowledge of specific imaging characteristics of tumefactive demyelination
can help in early diagnosis. Multiple underlying causes should be considered, including,
but not limited to multiple sclerosis. A paraneoplastic syndrome should not be overlooked.
This is the fifth case in which seminoma is associated with a paraneoplastic tumefactive
demyelination lesion.
Keywords
Abbreviations:
CNS (central nervous system), MS (multiple sclerosis)To read this article in full you will need to make a payment
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References
- Tumefactive demyelinating lesions: a comprehensive review.Mult Scler Relat Disord. 2017 May; 14: 72-79https://doi.org/10.1016/j.msard.2017.04.003
- Paraneoplastic recurrent tumefactive demyelination in a 62-year-old man with metastatic seminoma.Neurol Neuroimmunol. Neuroinflamm. 2018 Dec 13; 6: e527https://doi.org/10.1212/NXI.0000000000000527
- Solitary focal demyelination in the brain as a paraneoplastic disorder.Med. Pediatr Oncol. 1996 Feb; 26: 111-115https://doi.org/10.1002/(SICI)1096-911X(199602)26:2<111::AID-MPO8>3.0.CO;2-O
- Paraneoplastic tumefactive demyelination with underlying combined germ cell cancer.Pract Neurol. 2015 Dec; 15: 451-455https://doi.org/10.1136/practneurol-2015-001146
- Paraneoplastic demyelinating disorder in the brain of a patient with seminoma.J Comput Assist Tomogr. 1998 Jan-Feb; 22: 136-138https://doi.org/10.1097/00004728-199801000-00026
Article info
Publication history
Published online: March 17, 2020
Accepted:
March 15,
2020
Received in revised form:
March 2,
2020
Received:
January 30,
2020
Identification
Copyright
© 2020 Elsevier B.V. All rights reserved.
