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.
Paraneoplastic Autonomic Neuropathy; Anti-Hu Syndrome;
Denny-Brown Neuronopathy; Subacute Paraneoplastic Sensory Neuropathy; Autoimmune Paraneoplastic
Neurologic Syndrome; Paraneoplastic Sensory Neuropathy.
Clinically a rare disease, but antibodies can be found
in up to 10% of pulmonary small cell tumors.
Acquired autoimmune disease. Not linked to a
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.
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
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
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.
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.
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