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ObjectiveTo test the hypothesis that myeloneuropathy is a presenting phenotype of paraneoplastic neurologic syndromes we retrospectively reviewed clinical, radiologic, and serologic features of 32 patients with concomitant paraneoplastic spinal cord and peripheral nervous system involvement.MethodsObservational study investigating patients with myeloneuropathy and underlying cancer or onconeural antibody seropositivity.ResultsAmong 32 patients with paraneoplastic myeloneuropathy, 20 (63%) were women with median age 61 years (range 27–84 years). Twenty-six patients (81%) had classified onconeural antibodies (amphiphysin, n = 8; antineuronal nuclear antibody [ANNA] type 1 [anti-Hu], n = 5; collapsin response mediator protein 5 [CRMP5] [anti-CV2], n = 6; Purkinje cell cytoplasmic antibody type 1 [PCA1] [anti-Yo], n = 1; Purkinje cell cytoplasmic antibody type 2 [PCA2], n = 2; kelch-like protein 11 [KLHL11], n = 1; and combinations thereof: ANNA1/CRMP5, n = 1; ANNA1/amphiphysin, n = 1; ANNA3/CRMP5, n = 1). Cancer was confirmed in 25 cases (onconeural antibodies, n = 19; unclassified antibodies, n = 3; no antibodies, n = 3). Paraneoplastic myeloneuropathies had asymmetric paresthesias (84%), neuropathic pain (78%), subacute onset (72%), sensory ataxia (69%), bladder dysfunction (69%), and unintentional weight loss >15 pounds (63%). Neurologic examination demonstrated concomitant distal or asymmetric hyporeflexia and hyperreflexia (81%), impaired vibration and proprioception (69%), Babinski response (68%), and asymmetric weakness (66%). MRI showed longitudinally extensive (45%), tract-specific spinal cord T2 hyperintensities (39%) and lumbar nerve root enhancement (38%). Ten of 28 (36%) were unable to ambulate independently at last follow-up (median 24 months, range 5–133 months). Combined oncologic and immunologic therapy had more favorable modified Rankin Scale scores at post-treatment follow-up compared to those receiving either oncologic or immunologic therapy alone (2 [range 1–4] vs 4 [range 2–6], p < 0.001).ConclusionsParaneoplastic etiologies should be considered in the evaluation of subacute myeloneuropathies. Recognition of key characteristics of paraneoplastic myeloneuropathy may facilitate early tumor diagnosis and initiation of immunosuppressive treatment.

A 49-year-old woman with no relevant medical history presented to an external neurology clinic with progressive hand and foot paresthesia and gait instability for over 6 months. Initially, she reported tingling and shooting electrical-type sensations involving her feet. Within several weeks of the onset of paresthesia, she noted a burning pain sensation involving the left hand. Sensory symptoms continued to worsen and she felt pressure-like sensation around the neck and scalp region. This was shortly followed by right hand numbness and loss of dexterity. Over the next month, she developed progressive gait imbalance and transitioned to a cane for ambulation.

A 20-year-old man presented with vertigo followed by hourly episodes of dysarthria and incoordination lasting 5–20 seconds (video 1). Neurologic examination was normal between episodes. The only brain/spine MRI lesion was in the right superior cerebellar peduncle (figure, A). CSF analysis revealed elevated white blood cells (6/μL [normal, 0–5]) and oligoclonal bands (≥4). Paroxysmal dysarthria–ataxia associated with CNS demyelination was diagnosed and episodes resolved with oral acetazolamide 250 mg twice daily. Subsequent MRI of the brain and spine (figure, B) confirmed multiple sclerosis. Paroxysmal dysarthria–ataxia arises from ephaptic transmission within midbrain or cerebellar multiple sclerosis lesions and may respond to carbamazepine or acetazolamide.1 It should be distinguished from genetic or contactin-associated protein-like-2 (CASPR2) immunoglobulin G–associated episodic ataxia.2