Paraneoplastic sensory neuronopathy

Edward J Dropcho MD (Dr. Dropcho of Indiana University Medical Center has no relevant financial relationships to disclose.)
Originally released September 2, 1994; last updated May 11, 2016; expires May 11, 2019

Overview

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. The author 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.

Historical note and terminology

In 1948 Denny-Brown reported 2 patients with sensory neuropathy in whom autopsy revealed severe neuronal loss in the dorsal root ganglia and a previously undiagnosed bronchial carcinoma (Denny-Brown 1948). Subsequent reports in the 1950s and 1960s established that in most patients, paraneoplastic sensory neuronopathy is part of a multifocal encephalomyeloneuritis (Croft et al 1965; Henson et al 1965).

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