Paraneoplastic sensory neuronopathy

Hamza Malek MD (Dr. Malek of Northwestern University Feinberg School of Medicine has no financial relationships to disclose.)
Jinny O Tavee MD (

Dr. Tavee of Northwestern University Feinberg School of Medicine has no relevant financial relationships to disclose.

)
Rimas V Lukas MD, editor. (

Dr. Lukas of Northwestern University Feinberg School of Medicine received honorariums from AbbVie as a guest speaker, from NewLink Genetics and Reneuron for consulting work, and from Monetris as an advisory board member.

)
Originally released September 2, 1994; last updated September 1, 2018; expires September 1, 2021

Overview

Paraneoplastic sensory neuronopathy is a rare but potentially devastating complication of systemic neoplasms, in particular small cell lung carcinoma. In some 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 authors summarize 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 may occur as part of a multifocal encephalomyeloneuritis or may be 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).

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