Paraneoplastic neurological degenerations: keys to tumour immunity

  title={Paraneoplastic neurological degenerations: keys to tumour immunity},
  author={Matthew L. Albert and Robert B. Darnell},
  journal={Nature Reviews Cancer},
Paraneoplastic neurological degenerations (PNDs) are neurological disorders that develop in patients with cancer. PNDs are triggered by an effective antitumour immune response against neuronal antigens that are expressed in cancer cells, which subsequently develops into autoimmune neurodegenerative disease. Studying patients with PND has offered the opportunity to gain unique insights into mechanisms of tumour immunity and has provided the potential to apply this knowledge to patients with… 

Paraneoplastic syndromes affecting the nervous system.

This work reviews the currently known paraneoplastic neurologic disorders and their pathogenesis and established a model whereby the cancer is believed to initiate the syndrome by expressing a protein antigen normally expressed in the nervous system, leading to anti-tumor immune response followed by autoimmune neurologic symptoms.

A case of follicular lymphoma associated with paraneoplastic cerebellar degeneration.

Cerebellar ataxia was reported which turned out to be paraneoplastic cerebellar degeneration, a subtype of PND that develops Cerebellar symptoms, that was caused by follicular lymphoma.

Cell-Mediated Immune Responses in Paraneoplastic Neurological Syndromes

The link between PNS emergence and antitumor responses may result from more active CTLs and less functional Treg lymphocytes, which often coincide with a less aggressive course of neoplastic disease.

[Paraneoplastic neurological syndromes].

  • A. StorsteinC. Vedeler
  • Medicine
    Tidsskrift for den Norske laegeforening : tidsskrift for praktisk medicin, ny raekke
  • 2009
The cornerstone of therapy is treatment of the underlying tumor, but additional immunosuppressive therapy is often administered, and many, but not all patients harbour onconeural antibodies, which are highly useful diagnostic markers for a paraneoplastic etiology.

Paraneoplastic cerebellar degeneration in ovarian carcinoma: case report with review of immune modulation

A 56-year-old woman with stage IIIC serous ovarian carcinoma diagnosed 3 years prior developed progressive limb ataxia, and serum anti-Yo antibodies were positive, confirming the diagnosis of PCD.

Paraneoplastic Neurologic Syndromes

The effect of immunotherapy in patients with PNS associated with antibodies against the intracellular onconeural antigens is disappointing, and these patients benefit most from early detection and treatment of the underlying tumour.

Case Report: Innate Immune System Challenge Unleashes Paraneoplastic Neurological Autoimmunity

It is hypothesized that a tumor-initiated autoimmune response (evidenced by autoantibody profiles), pre-dating the immune system challenge, was unmasked or amplified in these patients by cytokines released systemically from innate immune cells activated by microbial pathogen-associated molecular patterns (PAMPs).

Paraneoplastic neurological syndromes associated with ovarian tumors

Interestingly, neoplasms associated with PNS are less advanced and metastasize less commonly than those without PNS, suggesting that PNS may be associated with a naturally occurring antitumor response.

Onconeural antibodies as a tool in the diagnosis of malignant tumors and paraneoplastic neurological syndromes

The onconeural antibodies detected in the blood sera of patients with PNS not only are key markers in the diagnosis of PNS and the tumors associated with them but also possess a substantial potential for the serologic diagnosis and prognosis of cancer as a whole.



Onconeural antigens and the paraneoplastic neurologic disorders: at the intersection of cancer, immunity, and the brain.

  • R. Darnell
  • Biology, Medicine
    Proceedings of the National Academy of Sciences of the United States of America
  • 1996
This review considers insights into basic neurobiology, tumor immunology, and autoimmune neuronal degeneration offered by the characterization of the onconeural antigens found in PND patients.

Autoimmune pathogenesis of paraneoplastic neurological syndromes.

Evidence supporting the hypothesis that the underlying tumor and portions of the nervous system share antigens and that an autoimmune response generated against the tumor causes the nervousSystem disorder is presented.

Spontaneous neurological improvement in anti-Hu associated encephalomyelitis.

Spontaneous neurological improvement, although rare, should be considered in the evaluation of therapies for PEM/PSN.

Paraneoplastic neurologic disease antigens: RNA-binding proteins and signaling proteins in neuronal degeneration.

The biology and functions of neuron-specific proteins are reviewed, and a model in which their functions are related to the pathogenesis of autoimmune neurologic disease is discussed.

Paraneoplastic Syndromes of the Nervous System

Experimental animals immunized against a paraneoplastic antigen are partially protected against tumors that express that antigen and low titers of antibody are associated with a better prognosis of the cancer.

Paraneoplastic encephalomyelitis: an update of the effects of the anti-Hu immune response on the nervous system and tumour

This review updates the effects of the HuAb immune response on the nervous system and the tumour of patients with small cell lung cancer and the anti-Hu syndrome.

Paraneoplastic syndromes involving the nervous system.

Paraneoplastic neurologic syndromes are incited by a tumor outside the nervous system that produces a characteristic antigen of the nervous System that damages nerve tissue and inhibits growth of the tumor.

Anti-Hu-associated paraneoplastic encephalomyelitis: analysis of 200 patients.

The predictors of mortality and PEM evolution found in the study may be important in the design of future therapeutic protocols, and emphasize the importance of early diagnosis and treatment of the underlying tumour.

Long-term clinical outcome of paraneoplastic cerebellar degeneration and anti-Yo antibodies

Although paraneoplastic cerebellar degeneration leads to the diagnosis of cancer in 63% of the patients, cancer progression was the cause of death in 52%.

Paraneoplastic cerebellar degeneration: Clinical‐immunological correlations

Four different antineuronal autoantibodies have been identified in 23 of 47 patients with paraneoplastic cerebellar degeneration (PCD), and an APCA was not found in patients with other neurological diseases, with cancer not associated with neurological symptoms, or in normal subjects.