Rhombencephalomyelitis due to possible paraneoplastic syndrome associated with Hodgkin's lymphoma

BMJ Case Rep. 2022 Apr 21;15(4):e249089. doi: 10.1136/bcr-2022-249089.

Abstract

We report a unique case of rhombencephalomyelitis with unclear aetiology, diagnosed with Hodgkin's lymphoma (HL) on follow-up.A woman in her 50s was presented with gait difficulty, dysarthria, left Horner's syndrome and left trigeminal sensory loss. MRI of the brain revealed an enhanced T2 and fluid attenuated inversion recovery hyperintense lesion at the cervico-medullary junction, suggesting rhombencephalomyelitis. The initial differential diagnosis included demyelination, other inflammatory conditions like sarcoidosis, Behcet's disease and paraneoplastic syndrome. Cerebrospinal fluid analysis, serum myelin oligodendrocyte glycoprotein, neuromyelitis optica antibodies, vasculitis work-up, onconeural antibody panel and whole-body fluorodeoxyglucose-positron emission tomography scan were all unrevealing, and she was kept under close follow-up. After 18 months, she developed a prolonged fever and was diagnosed with HL. Rhombencephalomyelitis as a HL-associated paraneoplastic syndrome has not been reported in the literature. This case report highlights the importance of serial screening for occult malignancy in patients with suspected paraneoplastic syndrome.

Keywords: Brain stem / cerebellum; Immunology; Neuroimaging; Screening (oncology).

Publication types

  • Case Reports

MeSH terms

  • Female
  • Hodgkin Disease* / complications
  • Hodgkin Disease* / diagnosis
  • Humans
  • Myelin-Oligodendrocyte Glycoprotein
  • Neuromyelitis Optica* / complications
  • Paraneoplastic Syndromes* / complications
  • Paraneoplastic Syndromes* / etiology
  • Positron-Emission Tomography

Substances

  • Myelin-Oligodendrocyte Glycoprotein