Paraneoplastic Syndrome Antibodies

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Contents

Definition

Paraneoplastic syndromes involving the nervous system are theorized to be an immune response against tumor antigens that cross-reacts with the nervous system. Notably, clinically, symptoms tend to be symmetric.

Anti-Hu

Associated with SCLC and Neuroblastoma

1. Subacute Cerebellar Degeneration – destruction of Purkinje cells, gliosis and mild inflammation

2. Limbic Encephalitis – subacute dementia with anteromedial temporal lobe perivascular inflammation and gliosis

3. Subacute Sensory Neuropathy – loss of sensory neurons and inflammation in dorsal root ganglia

4. Sensorimotor Neuropathy – more common than subacute sensory neuropathy, most pronounced in lower extremities

Anti-Tr

Associated with Hodgkins Lymphoma

1. Subacute Cerebellar Degeneration – destruction of Purkinje cells, gliosis and mild inflammation

Anti-Yo

Associated with Breast and Gynecological Cancers

1. Subacute Cerebellar Degeneration – destruction of Purkinje cells, gliosis and mild inflammation

Anti-Ma

Associated with Testicular and other Cancers

1. Limbic Encephalitis – subacute dementia with anteromedial temporal lobe perivascular inflammation and gliosis

Anti-Ri

Associated with Breast Ca and neuroblastoma

1. Opsoclonus-myoclonus

Anti-presynaptic calcium channel

Associated with Eaton-Lambert syndrome

Recoverin

Associated with SCLC: targets photoreceptors leading to retinal degeneration

Amphiphysin

Associated with Breast Cancer: targets spinal interneurons, causing stiff-man syndrome


References

Rosenfeld M, Dalmau J: The clinical spectrum and pathogenesis of paraneoplastic disorders of the central nervous system. Hematol Oncol Clin North Am 15: 1109, 2001.

Rudnicki S, Dalmau J: Paraneoplastic Syndromes of the spinal cord, nerve and muscle. Muscle Nerve 23: 1800, 2000.

Posner J, Dalmau J: Paraneoplastic syndromes of the nervous system. Clin Chem Lab Med 38: 117, 2000.

Kumar, V, Fausto, N, Abbas, A ed. Robbins & Cotran Pathologic Basis of Disease, 7th Ed. Philadelphia: WB Saunders Company, 2004.

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