Paraneoplastic Myelopathy


KEY FACTS

Terminology

  • Paraneoplastic neurologic disorder

  • Antineuronal immune mechanism targeting CNS, occurring in conjunction with cancer

Imaging

  • Longitudinally extensive, symmetric, tract-specific signal changes within spinal cord that often enhance after gadolinium administration in patient with cancer

  • Signal abnormality in ~ 70% of cases

  • Lateral, dorsal columns

  • Central gray matter

Top Differential Diagnoses

  • Cord infarction

  • Neuromyelitis optica

  • B12 deficiency (subacute combined degeneration)

  • Metastatic disease

  • Neurosarcoidosis

Pathology

  • Paraneoplastic disease usually

    • Antibodies against intracellular antigens

    • Older individuals, resistant to treatment

    • Mediated by T-cell mechanisms

  • Most common with lung and breast cancer

  • Most common antibodies causing myelopathy

    • Amphiphysin-IgG

    • Collapsin response-mediator protein-5

Clinical Issues

  • Insidiously progressive myelopathy

  • Often manifesting before detection of underlying cancer (2/3 of patients)

    • Median of 12 months from myelopathy onset to cancer detection

  • Poor prognosis, often becoming wheelchair dependent

Sagittal T1 MR shows multiple metastatic lesions
involving the cervical and upper thoracic vertebral bodies, with marrow conversion from prior radiation therapy
. The cord is mildly expanded.

Sagittal T2 MR in the same patient shows longitudinally extensive high signal within the cervical cord, with mild cord expansion
. This edema pattern is nonspecific, and cord metastases and radiation change should also be considered.

Axial T1 C+ MR in the same patient shows the typical pattern of paraneoplastic myelopathy with focal central tract enhancement
in a symmetrical fashion. This symmetrical tract involvement would not be seen with metastases or radiation change.

Sagittal T1 C+ MR in a patient with a focal cord metastasis
shows the very localized nature of the tumor to be contrasted, with the longitudinally extensive abnormality with paraneoplastic disease of the cord.

TERMINOLOGY

Abbreviations

  • Paraneoplastic neurologic disorder (PND)

Definitions

  • Antineuronal immune mechanism targeting CNS, occurring in conjunction with cancer

IMAGING

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