paraneoplastic myelopathy; paraneoplastic myeloneuropathy
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Etiology
- lung carcinoma (especially small cell lung carcinoma)
- breast carcinoma,
- less frequently
- renal cell carcinoma
- thyroid carcinoma
- ovariancrcimoma
- endometrial carcinoma
- melanoma
Epidemiology
- rare
Pathology
Clinical manifestations
- asymmetric paresthesias (84%)
- neuropathic pain (78%)
- sensory ataxia (69%) with impaired vibration & proprioception
- bladder dysfunction (69%)
- asymmetric weakness (66%)
- concomitant distal or asymmetric hyporeflexia & hyperreflexia (81%)
- due to mixed upper & lower motor neuron involvement when peripheral nerves also affected (myeloneuropathy)
- Babinski response (68%)
- unintentional weight loss >15 pounds (63%)[2]
- symptom onset is typically subacute (52-72%) or insidious
- progressive myelopathy often precedes cancer diagnosis by 12 months (2-44 months)
Laboratory
- elevated CSF protein (>45 mg/dL) most common finding,
- pleocytosis
- oligoclonal bands
Radiology
- MRI of spinal cord
- longitudinally extensive T2 hyperintensities spanning >3 vertebral segments (45-70%)
- symmetric, tract-specific or gray matter-specific signal abnormality
- gadolinium enhancement in the majority of abnormal lesions
- lumbar nerve root enhancement may also be seen (38%)
- whole-body PET-CT for occult malignancy screening
Management
- treatment of the underlying malignancy[3][4]
- immunosuppressive therapy
- IV glucocorticoids, plasma exchange, intravenous immunoglobulin
- escalation to cyclophosphamide or rituximab as needed
prognosis
- generally poor, with significant morbidity[1][5]
- 31% of patients improve with treatment
- 52% become wheelchair-dependent (median time to wheelchair: 9 months)
- 36% unable to ambulate independently at last follow-up
More general terms
References
- ↑ 1.0 1.1 Flanagan EP, McKeon A, Lennon VA Paraneoplastic isolated myelopathy: clinical course and neuroimaging clues. Neurology. 2011 Jun 14;76(24):2089-95. PMID: https://pubmed.ncbi.nlm.nih.gov/21670438 Review.
- ↑ 2.0 2.1 Shah S, Vazquez Do Campo R et al Paraneoplastic Myeloneuropathies: Clinical, Oncologic, and Serologic Accompaniments. Neurology. 2021 Jan 26;96(4):e632-e639. doi:http://dx.doi.org/ 10.1212/WNL.0000000000011218. Epub 2020 Nov 18. PMID: https://pubmed.ncbi.nlm.nih.gov/33208548 PMCID: PMC7905784 Free PMC article. https://pmc.ncbi.nlm.nih.gov/articles/PMC7905784/
- ↑ 3.0 3.1 Darnell RB, Posner JB. Paraneoplastic syndromes involving the nervous system. N Engl J Med. 2003 Oct 16;349(16):1543-54. doi:http://dx.doi.org/ 10.1056/NEJMra023009. PMID: https://pubmed.ncbi.nlm.nih.gov/14561798 Review. https://www.nejm.org/doi/full/10.1056/NEJMra023009
- ↑ 4.0 4.1 Verma N, Jaffer MH, Kolli AS, Mokhtari S. Updates in the Management of Paraneoplastic Syndrome. Semin Neurol. 2024 Feb;44(1):36-46. PMID: https://pubmed.ncbi.nlm.nih.gov/38183975
- ↑ 5.0 5.1 Liu Z, Jiao L, Qiu Z et al Clinical characteristics of patients with paraneoplastic myelopathy. J Neuroimmunol. 2019 May 15;330:136-142. PMID: https://pubmed.ncbi.nlm.nih.gov/30878696 https://www.sciencedirect.com/science/article/abs/pii/S0165572818304776
- ↑ Gilligan M, McGuigan C, McKeon A. Paraneoplastic Neurologic Disorders. Curr Neurol Neurosci Rep. 2023 Mar;23(3):67-82. PMID: https://pubmed.ncbi.nlm.nih.gov/36781586 PMCID: PMC10011323 Free PMC article. Review. https://pmc.ncbi.nlm.nih.gov/articles/PMC10011323/