Progressive Quadriparesis and Cancer

A 67-year-old man with a history of cigarette smoking sought care at the emergency department for nonspecific dizziness and fatigue. During evaluation, chest radiography showed a right upper lobe mass, and he subsequently underwent right upper lobectomy. Histologic analysis of resected tissue showed the mass to be small cell lung carcinoma. Progressive myelopathy developed. Adjuvant chemotherapy with carboplatin and etoposide was begun. His neurologic symptoms continued to worsen, with gait imbalance, along with numbness and dysesthesias of the 4 limbs and trunk, with a sensory level at C3-C4. He also reported severe bowel and bladder dysfunction. At his neurologic nadir, he had severe quadriparesis and was wheelchair dependent. Cerebrospinal fluid analysis showed lymphocytic pleocytosis of 9 lymphocytes/µL, erythrocyte count of 2/µL, and normal protein and glucose values. Serum neural autoantibody screening revealed the presence of collapsin-response mediator protein 5–immunoglobulin G antibodies on both tissue-based indirect immunofluorescence assay and Western blot. The patient was diagnosed with paraneoplastic myelopathy. The patient was initially treated acutely with a combination of high-dose oral prednisone and plasmapheresis, without improvement. Subsequently, a combination of intravenous immunoglobulin and rituximab resulted in partial improvement. Soon after treatment discontinuation, his symptoms returned with worsening weakness, numbness, and neuropathic pain. Monthly intravenous immunoglobulin and rituximab were reinitiated, with improvement again noted. Oral corticosteroids, methadone, and high-dose gabapentin were also administered, with mild benefit for neuropathic pain. Follow-up spine magnetic resonance imaging 3 years after symptom onset showed evidence of spinal cord atrophy. At 5-year follow-up he remained in remission from small cell lung cancer but was wheelchair dependent. Paraneoplastic myelopathy is a rare and underrecognized neurologic disorder that most often manifests before cancer detection, Clinical presentation is generally subacute or slowly progressive over months, but acute onset is possible. Cerebrospinal fluid typically shows lymphocytic pleocytosis, as in this case patient.