Paraneoplastic isolated myelopathy: clinical course and neuroimaging clues
| Autor: | Allen J. Aksamit, Marcelo Matiello, Karl N. Krecke, Vanda A. Lennon, Eoin P. Flanagan, B. M. Keegan, Bahram Mokri, Sean J. Pittock, J. Kearns, Brian G. Weinshenker, A. McKeon |
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| Rok vydání: | 2011 |
| Předmět: |
Adult
Male medicine.medical_specialty Gastroenterology Spinal Cord Diseases Myelopathy Internal medicine medicine Carcinoma Humans Pleocytosis Aged medicine.diagnostic_test business.industry Melanoma Thyroid Autoantibody Cancer Magnetic resonance imaging Middle Aged medicine.disease Magnetic Resonance Imaging medicine.anatomical_structure Phenotype Treatment Outcome Spinal Cord Female Neurology (clinical) business Paraneoplastic Syndromes Nervous System |
| Zdroj: | Neurology. 76(24) |
| ISSN: | 1526-632X |
| Popis: | To report the clinical phenotype and outcome of isolated paraneoplastic myelopathy.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).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 (normal4), 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).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. |
| Databáze: | OpenAIRE |
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