Paraneoplastic sensory neuronopathy

Introduction
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By Edward J Dropcho MD

Paraneoplastic sensory neuronopathy is a rare but potentially devastating complication of small cell lung carcinoma or, less often, other systemic neoplasms. In most patients the sensory neuronopathy is part of a multifocal encephalomyeloneuritis. The disorder is believed to arise from an autoimmune response directed against onconeural antigen(s) shared by tumor cells and primary sensory neurons in the dorsal root ganglia. Early identification of this syndrome and treatment of the underlying tumor can improve the likelihood of neurologic recovery, although most patients are severely and permanently disabled. Dr. Dropcho, from the Indiana University Medical Center, summarizes the clinical presentation, autoimmune features, and treatment options for patients with paraneoplastic sensory neuronopathy.

Key Points

  • Paraneoplastic sensory neuronopathy (dorsal root ganglionitis) is most often associated with small-cell lung carcinoma and is usually the presenting feature of the tumor.
  • Paraneoplastic sensory neuronopathy generally occurs as part of a multifocal encephalomyelitis or encephalomyeloneuritis and less often as an isolated clinical syndrome.
  • Paraneoplastic sensory neuronopathy is often disabling due to painful dysesthesias, profound loss of proprioception, and sensory gait ataxia.
  • Most patients with paraneoplastic sensory neuronopathy and small-cell lung cancer do not show significant neurologic improvement despite successful tumor treatment and/or immunosuppressive therapy, though there are some notable exceptions.

In This Article

Introduction
Historical note and nomenclature
Clinical manifestations
Clinical vignette
Etiology
Pathogenesis and pathophysiology
Epidemiology
Prevention
Differential diagnosis
Diagnostic workup
Prognosis and complications
Management
References cited
Contributors