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Paraneoplastic autonomic neuropathic syndrome occurring in association with several primary neoplasias, characterized by antibodies directed against a cellular nuclear component expressed in nearly all nervous tissues.

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Paraneoplastic Autonomic Neuropathy; Anti-Hu Syndrome; Denny-Brown Neuronopathy; Subacute Paraneoplastic Sensory Neuropathy; Autoimmune Paraneoplastic Neurologic Syndrome; Paraneoplastic Sensory Neuropathy.

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Clinically a rare disease, but antibodies can be found in up to 10% of pulmonary small cell tumors.

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Acquired autoimmune disease. Not linked to a particular genotype.

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Autoantibodies directed against a nuclear component of nervous system cells called Hu. This antigen is expressed in the central and peripheral nervous systems but can also be expressed by neoplastic cells. It may play a role in nervous system development. Loss of ganglion cells in sacral and lumbar dorsal root ganglia.

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Clinical expression of a subacute mainly sensory neuronopathy, with demonstration of polyclonal anti-Hu antibodies in the plasma. Axons and neuromuscular junctions appear normal on electrophysiologic studies.

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Mainly found in conjunction with pulmonary small cells tumors but may be encountered with other types of tumors. The sensory neuronopathy can be found up to 1 year before clinical discovery of the tumor. Clinically, there is a subacute neuronopathy, sometimes associated with central limbic symptomatology. It may be misdiagnosed as “herpetic encephalitis.” Immunosuppression and plasmapheresis have not shown any benefit.

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Usually, patients present for surgical treatment of the underlying tumor. It is judicious to request a complete neurologic evaluation before and after the procedure to confirm maintenance of neurologic status. In this setting, general anesthesia is preferable, and central neuraxial blockade techniques are not recommended. Look for signs of autonomic dysfunction before induction.

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Anesthetic considerations are those associated with the surgical procedure and not the patient's condition. However, in the presence of neuronopathy, use of anesthetic agent associated with rapid elimination might be appropriate. Usually uneventful recovery.

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No specific implications; however, it is believed that the response to local anesthetics is poorly managed.

Lovblad KO, Boucraut J, Steck AJ: Paraneoplastic subacute sensory Denny-Brown neuronopathy or anti-Hu syndrome: A classical paraneoplastic syndrome. Rev Med Suisse Romande 115:421, 1995.  [PubMed: 7770690]

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