About Paraneoplastic Neurological Disorders (PND) > Central Nervous System (Brain and Spinal Cord) > Paraneoplastic Stiff-person (or Stiff-man) Syndrome

Paraneoplastic Stiff-person (or Stiff-man) Syndrome

Paraneoplastic Stiff-person (or Stiff-man) syndrome can affect men or women.

Patients develop:

  • muscle stiffness and rigidity,
  • with painful spasms.

These problems usually occur in the muscles of the lower back and legs, but may also affect the arms and neck. In some patients, the initial symptoms can be in one leg, and then progress to affect other parts of the body. The spasms can be triggered or made worse by anxiety, loud noises, or simply by touch or trying to move.

A similar disease may occur and be “non-paraneoplastic” or without an associated cancer. The diagnosis of stiff-person syndrome is usually confirmed with electrical tests such as EMG and nerve conduction studies. Also, seeing how some medications (such as valium) improve the symptoms may help to make the diagnosis. These tests do not clarify whether the disorder is paraneoplastic or not.

When the disorder is paraneoplastic, an antibody called anti-amphiphysin is usually found in the blood and spinal fluid. The tumors more frequently involved are breast and lung cancer.

When the disorder is not paraneoplastic, an antibody called anti-GAD (glutamic acid decarboxylase) is usually found in the serum and spinal fluid of the patient. Anti-GAD antibodies are not fully specific for non-paraneoplastic stiff-person syndrome because they can also be found in some patients with tumors such as thymomas. Anti-GAD antibodies may occur also in patients with some types of epilepsy, cerebellar degeneration, and diabetes, none of them with cancer association.

It is believed that the antibodies against GAD or amphiphysin disturb the function of neurotransmitters (chemicals involved in nerve cell interactions) called GABA and glycine. Therefore, treatments for stiff-person syndrome include:

  • drugs that enhance the function of these neurotransmitters (diazepam, baclofen, sodium valproate, tiagabine, vigabatrin),
  • immunotherapy, and
  • treating the tumor if the disorder is paraneoplastic.

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