Cancer-related syndromes that involve mechanisms other than metastasis or by any of the complications of the cancer such as coagulopathy, stroke, metabolic and nutritional conditions, infections, and side effects of cancer therapy can affect the nervous system and cause disorders called paraneoplastic neurologic disorders (PNDs). In 60% of the patients, these disorders usually precede the cancer that cause them and are usually disabling occurring in 0.5%-1% of all cancer patients, but affect 2%-3% of patients with neuroblastoma or small-cell lung cancer (SCLC) and 30%-50% of patients with thymoma.

The neuronal proteins, also called onconeural antigens, expressed by tumors trigger immune mediated responses that usually mediate most PNDs. Many antibody-associated immune responses have been identified in PNDs of the central nervous system (CNS). The prediction of cancer can be done by the presence of antibodies in the serum and the cerebrospinal fluid (CSF) that react with both neurons and the patient’s tumor. Sometimes the disorders are due to the antibodies released against the antigens expressed on the neuronal cell surface of the CNS or at the neuromuscular junction and not the immune responses mediated by these antigens. These type of responses are treated by immunotherapy rather than the latter. These disorders can occur with or without cancer association. Children and adults both are affected and there is evidence demonstrating the pathogenic role of the antibodies. Some PNDs are thought to be immune mediates but their antigens are unknown. This category involves several syndromes of inflammatory neuropathies and myopathies. Also, many patients with typical PND syndromes are antibody-negative. The majority of the paraneoplastic neurologic syndromes are:

  • Paraneoplastic encephalomyelitis and focal encephalitis with antibodies against intracellular neuronal proteins
  • Encephalitis with antibodies to cell-surface or synaptic proteins
  • Paraneoplastic cerebellar degeneration
  • Paraneoplastic opsoclonus-myoclonus syndrome
  • Paraneoplastic syndromes of the spinal cord
  • Paraneoplastic stiff-person syndrome
  • Paraneoplastic sensory neuronopathy or dorsal root ganglionopathy
  • Paraneoplastic peripheral neuropathies
  • Lambert-Eaton myasthenic syndrome
  • Myasthenia gravis
  • Polymyositis-dermatomyositis
  • Acute-necrotizing myopathy
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The disorders that affect the nervous system and are caused by cancer-related syndromes involving mechanisms other than metastasis or by any of the complications of the cancer such as coagulopathy, stroke, metabolic and nutritional conditions, infections, and side effects of cancer therapy are called paraneoplastic neurologic disorders (PNDs). The majority of the paraneoplastic neurologic syndromes are:

  • Paraneoplastic encephalomyelitis and focal encephalitis with antibodies against intracellular neuronal proteins
  • Pncephalitides with antibodies to cell-surface or synaptic proteins
  • Paraneoplastic cerebellar degeneration
  • Paraneoplastic opsoclonus-myoclonus syndrome
  • Paraneoplastic syndromes of the spinal cord
  • Paraneoplastic stiff-person syndrome
  • Paraneoplastic sensory neuronopathy or dorsal root ganglionopathy
  • Paraneoplastic peripheral neuropathies
  • Lambert-Eaton myasthenicsyndrome
  • Myasthenia gravis
  • Polymyositis-dermatomyositis
  • Acute-necrotizing myopathy
Read more..
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