Paraneoplastic Neurologic Syndromes

Published on 04/03/2015 by admin

Filed under Hematology, Oncology and Palliative Medicine

Last modified 04/03/2015

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Chapter 39

Paraneoplastic Neurologic Syndromes

Summary of Key Points

• The paraneoplastic neurologic syndromes include an extensive group of disorders that can affect any part of the central or peripheral nervous system.

• Evidence indicates that many of these syndromes have an immunopathogenesis.

• Patients with paraneoplastic neurologic syndromes may have serum and cerebrospinal fluid antibodies that are highly specific for the presence of a cancer. The detection of these antibodies serves as a marker of paraneoplasia. Other antibodies associate with specific neurologic syndromes that occur with or without cancer. In these cases, the antibodies serve as markers for the neurologic syndrome but do not distinguish between a paraneoplastic or nonparaneoplastic etiology.

• Antibodies directly mediate some disorders such as the Lambert-Eaton myasthenic syndrome, myasthenia gravis, and neuromyotonia and likely mediate other disorders such as anti-N-methyl-d-aspartate receptor encephalitis. In these disorders the antibodies target neuronal cell surface proteins (e.g., receptors and ion channels), and immunotherapy along with tumor treatment often result in substantial neurologic improvement.

• For paraneoplastic neurologic syndromes in which T-cell mechanisms are predominant, the associated antibodies target intracellular neuronal proteins. In this group of disorders, the response to therapy is, in general, disappointing. The physician’s main concern should be to rule out other diagnostic entities and to uncover the presence of the associated neoplasm. For these syndromes the treatment approach should be aimed at the tumor, because stabilization and, less often, improvement of neurologic symptoms after tumor treatment have been reported for almost all syndromes. In a few cases, depending on the syndrome and whether the patient is in the early stages of the neurologic disease, treatment with immunosuppression may have some effect on the paraneoplastic neurologic syndrome.

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