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Paraneoplastic Neurologic Syndromes

Paraneoplastic neurologic syndromes are an extensive group of neurologic disorders that occur in patients with cancer and can affect any part of the nervous system (see below). In most patients the neurologic symptoms develop before the presence of the cancer is known. Because of this and because the paraneoplastic symptoms can mimic other cancer and non-cancer related neurologic problems, the diagnosis of paraneoplastic syndromes is difficult and can be delayed while the patient is being tested for other possible disorders.

However, patients with paraneoplastic syndromes often develop specific serum or spinal fluid antibodies. Detection of these antibodies indicates that the patient’s symptoms are paraneoplastic and can lead to the identification of the tumor at early stage of the disease, when the tumor is curable. The Neuro-oncology team at Penn is recognized as a leader in the diagnosis and treatment of these disorders.

Paraneoplastic Syndromes of the Nervous System:

Syndromes of the brain, brainstem and cerebellum

  • Focal encephalitis
    • Cortical encephalitis
    • Limbic encephalitis
    • Brainstem encephalitis
    • Cerebellar dysfunction
    • Autonomic dysfunction
  • Paraneoplastic cerebellar degeneration
  • Opsoclonus-myoclonus

Syndromes of the spinal cord

  • Subacute necrotizing myelopathy
  • Motor neuron dysfunction
  • Myelitis
  • Stiff-man syndrome

Syndrome of the dorsal root ganglia

  • Sensory neuronopathy

Multiple levels of involvement

  • Encephalomyelitis*, sensory neuronopathy, autonomic dysfunction

Syndromes of the peripheral nerve

  • Chronic and subacute sensorimotor peripheral neuropathy
  • Vasculitis of the nerve and muscle
  • Neuropathy associated with malignant monoclonal gammopathies
  • Peripheral nerve hyperexcitability
  • Autonomic neuropathy

Syndromes of the neuromuscular junction

  • Lambert-Eaton myasthenic syndrome
  • Myasthenia gravis

Syndromes of the muscle

  • Polymyositis/Dermatomyositis
  • Acute necrotizing myopathy

*Includes cortical, limbic or brainstem encephalitis, cerebellar dysfunction, myelitis.

 


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