A 63-year-old woman had development of ascending paresthesias with lancinating and stabbing pain in all extremities. The symptoms began asymmetrically in the left leg and progressed to involve the right leg and both hands. She noticed progressive bilateral leg weakness, gait instability, and new numbness in the left middle back. She had recurrent falls and required the use of a cane and eventually a walker. She had 18.2 kg of weight loss. She had no autonomic symptoms or bladder or bowel dysfunction.
Examination 1 year after symptom onset showed an emaciated, ill-appearing woman. On neurologic examination, she had marked gait ataxia with profound proprioceptive loss in all extremities, asymmetrically reduced pinprick sensation distal to the left thigh, right knee, and both forearms, as well as bilateral middle thoracic dermatomes. She had bilateral foot drop and mild weakness in bilateral hamstring and intrinsic hand muscles. Her deep tendon reflexes were globally absent. Toes were mute to plantar stimulation.
Magnetic resonance imaging of the entire spine demonstrated subtle increased T2 signal in the posterior columns extending along cervical and thoracic segments and patchy gadolinium enhancement of the cervical and lumbosacral nerve roots. Electrodiagnostic testing demonstrated a length-dependent axonal sensorimotor peripheral neuropathy. Cerebrospinal fluid analysis showed an increased protein concentration, 9 cerebrospinal fluid-restricted oligoclonal bands, increased immunoglobulin G index and synthesis rate, and normal blood cell count. Paraneoplastic antibody evaluation showed high titers of collapsin-response mediator protein 5-immunoglobulin G autoantibodies in the serum and cerebrospinal fluid. Whole-body 18F-fludeoxyglucose–positron emission tomography indicated multiple hypermetabolic mediastinal and hilar lymph nodes. Biopsy of a mediastinal lymph node was consistent with small cell lung carcinoma.
The patient was diagnosed with collapsin-response mediator protein 5- immunoglobulin G paraneoplastic myeloneuropathy associated with small cell carcinoma of the lung.
The patient received a trial of intravenous methylprednisolone followed by chemotherapy, along with chest and prophylactic brain radiotherapy. She achieved a short period of clinical stabilization and mild improvement in gait and limb ataxia. Subsequently, her ataxia and bilateral leg weakness worsened. Her clinical condition rapidly deteriorated, and she became severely malnourished and bedridden, dying 12 weeks later, 2 years after symptom onset. Collapsin-response mediator protein 5- immunoglobulin G antibodies have been associated with asymmetric, painful polyradiculoneuropathy with coexisting myelopathy, often in the setting of thymoma or small cell lung cancer.