title: Paraneoplastic isolated myelopathy: clinical course and neuroimaging clues.
abstract: OBJECTIVE
  To report the clinical phenotype and outcome of isolated paraneoplastic myelopathy.
  
  
  METHODS
  We systematically reviewed clinical, serologic, and MRI data for 31 patients (20 female) who presented with an isolated myelopathy and coexisting cancer: carcinoma (lung, 9; breast, 7; kidney, 2; thyroid, 2; ovary/endometrium, 2), melanoma (2), or other cancer (3), or a paraneoplastic autoantibody with strong cancer association (amphiphysin-immunoglobulin G [IgG], 9; collapsin response-mediator protein 5-IgG, 9; Purkinje-cell cytoplasmic autoantibody type 1, 2; antineuronal nuclear autoantibody [ANNA]-1, 1; ANNA-3, 1).
  
  
  RESULTS
  Of 31 patients who presented with a progressive myelopathy, symptom onset was subacute in 16 (52%). The median age was 62 years. CSF abnormalities included elevated protein (>45 mg/dL), 22; pleocytosis, 15; excess oligoclonal bands (normal <4), 7. MRI cord abnormalities identified in 20 patients were longitudinally extensive (>3 vertebral segments), 14; symmetric tract or gray matter-specific signal abnormality, 15 (enhancing in 13). Myelopathy preceded cancer diagnosis in 18 patients (median interval 12 months; range 2-44). After myelopathy onset, 26 patients underwent oncologic treatment, immunosuppressive treatment (median delay to commencing immunotherapy 9.5 months [range 1-54]), or both; only 8 improved (31%). At last neurologic evaluation (median interval after onset 17 months; range 1-165 months), 16 patients (52%) were wheelchair-dependent (median time from onset to wheelchair 9 months [range 1-21]). Ten patients died after a median of 38 months from symptom onset (range 7-152).
  
  
  CONCLUSIONS
  Symmetric, longitudinally extensive tract or gray matter-specific changes on spinal MRI should raise suspicion for a paraneoplastic myelopathy. Resulting disability is often severe. Only a minority of patients improve with treatment.
authors.affiliation: Mayo Clinic, Department of Neurology, Rochester, MN, USA.
authors.forename: E P
authors.initials: EP
authors.lastname: Flanagan
authors.forename: A
authors.initials: A
authors.lastname: McKeon
authors.forename: V A
authors.initials: VA
authors.lastname: Lennon
authors.forename: J
authors.initials: J
authors.lastname: Kearns
authors.forename: B G
authors.initials: BG
authors.lastname: Weinshenker
authors.forename: K N
authors.initials: KN
authors.lastname: Krecke
authors.forename: M
authors.initials: M
authors.lastname: Matiello
authors.forename: B M
authors.initials: BM
authors.lastname: Keegan
authors.forename: B
authors.initials: B
authors.lastname: Mokri
authors.forename: A J
authors.initials: AJ
authors.lastname: Aksamit
authors.forename: S J
authors.initials: SJ
authors.lastname: Pittock
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D020361:Paraneoplastic Syndromes, Nervous System / Q000473:pathology* / Q000503:physiopathology* / Q000628:therapy; D010641:Phenotype; D013116:Spinal Cord / Q000473:pathology*; D013118:Spinal Cord Diseases / Q000473:pathology* / Q000503:physiopathology* / Q000628:therapy; D016896:Treatment Outcome