Paraneoplastic neurologic syndromes

Paraneoplastic Encephalomyelitis and Focal Encephalitis The term encephalomyelitis describes an inflammatory process with multifocal involvement of the nervous system, including brain, brainstem, cerebellum, and spinal cord. It is often associated with dorsal root ganglia and autonomic dysfunction. For any given patient, the clinical manifestations are determined by the area or areas predominantly involved, but pathology almost always reveals abnormalities (inflammatory infiltrates, neuronal loss, gliosis) beyond the symptomatic regions. ...

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Encephalitis and Encephalomyelitis: Treatment Most types of paraneoplastic encephalitis and encephalomyelitis respond poorly to treatment. Stabilization of symptoms or partial neurologic improvement may occasionally occur, particularly if there is a satisfactory response of the tumor to treatment. The roles of plasma exchange, IVIg, and immunosuppression have not been established. Approximately 30% of patients with anti-Ma2-associated encephalitis respond to treatment of the tumor (usually a germ-cell neoplasm of the testis) and immunotherapy.

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Paraneoplastic myelitis may present with upper or lower motor neuron symptoms, segmental myoclonus, and rigidity. This syndrome can appear as the presenting manifestation of encephalomyelitis and may be associated with SCLC and serum anti-Hu, anti-CV2/CRMP5, or anti-amphiphysin antibodies. Paraneoplastic myelopathy can also produce several syndromes characterized by prominent muscle stiffness and rigidity.

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Vasculitis of the nerve and muscle causes a painful symmetric or asymmetric distal sensorimotor neuropathy with variable proximal weakness. It predominantly affects elderly men and is associated with an elevated erythrocyte sedimentation rate and increased CSF protein concentration. SCLC and lymphoma are the primary tumors involved. Pathology demonstrates axonal degeneration and T cell infiltrates involving the small vessels of the nerve and muscle. Immunosuppressants (glucocorticoids and cyclophosphamide) often result in neurologic improvement.

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Specific Paraneoplastic Neurologic Syndromes (Table 97-3) Table 97-3 Antibody-Associated Paraneoplastic and Nonparaneoplastic Syndromesa Antibodies Paraneoplastic Syndrome Frequent t Infrequen stic Nonparaneopla Limbic encephalitis Ma2, Hu, CV2/CRMP5, anti-NR1/NR2 of NMDA receptor Tr, VGKC VGKC Cerebellar degeneration Yo, Tr, mGluR1; Gliadin, GAD P/Q VGCC, Hu, MAZ Zic, CV2/CRMP5, Ma1-2 Ri, Hypothalamic, brainstem encephalitis Ma2, Hu P5 CV2/CRM Encephalomye litis Hu, Zic P5, CV2/CRM Ri, amphiphysin Chorea P5 CV2/CRM Opsoclonusmyoclonus Ri Yo, Hu, Ma2, Stif...

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Pathogenesis Most PNDs are mediated by immune responses triggered by neuronal proteins (onconeuronal antigens) expressed by tumors. In PNDs of the central nervous system (CNS), many antibody-associated immune responses have been identified (Table 97-2). These antibodies usually react with the patient's tumor, and their detection in serum or cerebrospinal fluid (CSF) strongly predicts the presence of cancer. The target antigens are usually intracellular proteins with roles in neuronal development and function.

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PND of the Central Nervous System and Dorsal Root Ganglia When symptoms involve brain, spinal cord, or dorsal root ganglia, the suspicion of PND is usually based on a combination of clinical, radiologic, and CSF findings. In these cases, a biopsy of the affected tissue is often difficult to obtain, and although useful to rule out other disorders (e.g., metastasis, infection), neuropathologic findings are not specific for PND. Furthermore, there are no specific radiologic or electrophysiologic tests that are diagnostic of PND. ...

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Neuronal cell-surface antigens can be the target of antibodies in some patients with paraneoplastic encephalitis. A few of these antigens have been identified, including the NR1/NR2 subunits of NMDA receptors (Fig. 97-1) and voltage-gated potassium channels (VGKC). These disorders are more responsive to immunotherapy than those associated with immune responses to intracellular antigens. Figure 97-1 Antibodies to NR1/NR2 subunits of the NMDA receptor in a patient with paraneoplastic encephalitis and ovarian teratoma.

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Harrison's Internal Medicine Chapter 97. Paraneoplastic Neurologic Syndromes Paraneoplastic Neurologic Syndromes: Introduction Paraneoplastic neurologic disorders (PNDs) are cancer-related syndromes that can affect any part of the nervous system (Table 97-1). They are remote effects of cancer, caused by mechanisms other than metastasis or by any of the complications of cancer such as coagulopathy, stroke, metabolic and nutritional conditions, infections, and side effects of cancer therapy. In 60% of patients the neurologic symptoms precede the cancer diagnosis.

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Harrison's Internal Medicine Chapter 96. Paraneoplastic Syndromes: Endocrinologic/Hematologic Paraneoplastic Syndromes: Endocrinologic/Hematologic: Introduction In addition to local tissue invasion and metastasis, neoplastic cells can produce a variety of peptides that that can stimulate hormonal, hematologic, dermatologic, or neurologic responses. Paraneoplastic syndromes refer to the disorders that accompany benign or malignant tumors but are not directly related to mass effects or invasion.

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