Paraneoplastic recurrent tumefactive demyelination in a 62-year-old man with metastatic seminoma

Autor: Ilana Hanes, John Woulfe, Pierre R. Bourque, Simon Thebault
Rok vydání: 2018
Předmět:
Zdroj: Neurology® Neuroimmunology & Neuroinflammation
ISSN: 2332-7812
Popis: A 62-year-old man presented with subacutely progressive headache, right-sided weakness, and subtle receptive aphasia. The initial MRI showed a large oval subcortical left frontoparietal lesion, hypertintense on T2- and hypointense on T1-weighted sequences, exerting mass effect on adjacent sulci (figure, A). This lesion, which had a thin rim of peripheral diffusion restriction, and showed only minimal focal enhancement, was favored to represent a low-grade glioma. The patient was admitted to a neurosurgical service, and treated with high-dose dexamethasone, which resulted in partial clinical improvement before open craniotomy and a limited debulking resection. He did well postoperatively and was discharged with only mild right hemisensory loss. Unexpectedly, histopathology showed no evidence of neoplasia. There was demyelination and inflammatory infiltration with a predominance of CD68-immunoreactive foamy reactive macrophages (figure, B). CSF examination (cell counts, protein, and albumin index) was normal, without evidence of oligoclonal bands. The neurology consultant favored a diagnosis of isolated tumefactive demyelinating lesion, lacking MS criteria for clinical or imaging evidence of dissemination in time and space. No further treatment was recommended initially because deficits were minor and improving. The authors thank the University of Oxford Neuroimmunology laboratory for performing immunohistochemistry and live neuronal antibody screens.
Databáze: OpenAIRE