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Progressive multifocal leucoencephalopathy isolated to the brainstem and cerebellum
  1. Denison Alves Pedrosa1,
  2. Luis Filipe de Souza Godoy1,
  3. André Luiz Guimarães de Queiroz1,
  4. Carla Renata Aparecida Vieira Stella2,
  5. Rodrigo B Thomaz1
  1. 1 Hospital Israelita Albert Einstein, São Paulo, São Paulo, Brazil
  2. 2 Faculdade de Ciencias Medicas, Universidade Estadual de Campinas, Campinas, São Paulo, Brazil
  1. Correspondence to Denison Alves Pedrosa, Hospital Israelita Albert Einstein, São Paulo, São Paulo, Brazil; denisonpedrosa{at}hotmail.com

https://doi.org/10.1136/pn-2023-003999

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    Clinical case

    A 27-year-old man, receiving his 27th dose of natalizumab monotherapy for multiple sclerosis (MS), tested positive for the anti-JCV (John Cunningham virus) antibody with a low index (0.23). Five days after his 30th dose, he developed horizontal diplopia. An MR scan of the brain identified a tumefactive lesion in the right middle cerebellar peduncle, with no enhancement, and was interpreted as an MS attack (figure 1). He received two cycles of intravenous methylprednisolone but progressed with dysarthria and tetraplegia. Less than 3 months later, MR scan of the brain showed progressive expansion of the previous lesion, affecting the pons, both the right and left cerebellar peduncles, and the cerebellum (figure 2). Cerebrospinal fluid (CSF) analysis showed a strongly positive JCV PCR (>1 300 000 DNA copies/mL). Pembrolizumab was administered (2 mg/kg body weight intravenously, once) but no clinical response at 6-month follow-up (Expanded Disability Status Scale - EDSS 9.5).

    Figure 1

    MR scan of the brain: (A) axial fluid attenuated inversion recovery (FLAIR) and (B) T2-weighted image, before onset of diplopia showing typical periventricular and deep white matter multiple sclerosis lesions (arrows) and normal right middle …

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    Footnotes

    • Contributors DAP: drafting/revision of the manuscript for content, including medical writing for content; major role in the acquisition of data; study concept or design; and analysis or interpretation of data. LG: drafting/revision of the manuscript for content; major role in the acquisition of data; and analysis or interpretation of data. AQ: drafting/revision of the manuscript for content, including medical writing for content; study concept or design; and analysis or interpretation of data. CRAVS: drafting/revision of the manuscript for content and analysis or interpretation of data. RBT: drafting/revision of the manuscript for content, including medical writing for content; major role in the acquisition of data; study concept or design; and analysis or interpretation of data.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Provenance and peer review Not commissioned; externally peer reviewed by Nicholas Davies, London, UK, and Neil Scolding, Bristol, UK.

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