Paraneoplastic neurological syndromes (PNS) are immune-mediated complications of cancer associated with a broad spectrum of clinical manifestations. Optic neuropathy (ON) and myelitis are frequent manifestations of multiple sclerosis (MS) and neuromyelitis optic spectrum disorders (NMOSD) but are considered as non-classical in PNS. Here, we report a case of PNS revealed by simultaneous bilateral ON and myelitis related to multiple co-existing paraneoplastic antibodies (Abs), in the setting of small cell lung cancer (SCLC).
To describe a patient with concomitant bilateral ON and myelitis as revealing neurological manifestations of paraneoplastic autoimmunity.
A 70-year-old man with a 50-pack-year smoking history was admitted because of subacute bilateral and painless vision loss and left lower limb weakness. Visual acuity was 7/10 in the right eye and 3/10 in the left eye. Fundoscopy showed bilateral disc swelling and peripapillary hemorrhages. Brain MRI did not reveal any optic nerve lesion nor lesions suggestive of MS. Spinal cord MRI showed a T2-weighted hyperintensity at the C2 level, with gadolinium enhancement. The visual evoked potentials showed significant bilateral increase of P100 latencies. CSF analysis showed lymphocytic pleocytosis (149 cells/μl), elevated protein concentration (99 mg/dl), and positive CSF-restricted IgG oligoclonal bands. No malignant cells were found in the CSF. Immunologic and infectious workup was unremarkable. Testing for anti-AQP4 and -MOG Abs was negative. Lung cancer was highly suspected on whole body FDG-PET. Transbronchial needle aspiration of hilar lymph nodes revealed SCLC.
The paraneoplastic antibody panel showed a significant signal for anti-Hu Abs in the serum (EUROLINE Neuronal Antigen Profile blots, EUROIMMUN), confirming the diagnosis of PNS. Anti-CV2/CRMP5 and -amphiphysin Abs were also detected, albeit at lower levels. Treatment with intravenous methylprednisolone and plasma exchange resulted in poor clinical improvement. A second malignancy (prostate cancer) was highly suspected but the patient elected to withdraw from further evaluation or treatment and received palliative care at home.
Simultaneous involvement of optic nerves and spinal cord is a rare manifestation of PNS. This presentation should prompt screening for a panel of onconeuronal Abs and for an underlying malignancy, particularly in older adult smokers who are unlikely to have MS or NMOSD.