Stiff Person Syndrome Workup

  • Author: Nancy Theresa Rodgers-Neame, MD; Chief Editor: Nicholas Lorenzo, MD   more...
 
Updated: Feb 6, 2012
 

Laboratory Studies

  • Obtain the following laboratory tests and interpret the results as outlined in Pathophysiology:
    • Hemoglobin A1C: This is obtained because of association with diabetes mellitus.
    • Complete blood count: An association with pernicious anemia has been reported.
    • Comprehensive metabolic profile
    • Thyroid-stimulating hormone: Thyroiditis is sometimes associated.
  • Special tests
    • Anti-GAD antibodies
    • Anti–pancreatic islet cell antibodies
    • Anti-amphiphysin antibodies
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Imaging Studies

  • MRI or CT scanning of the brain is only indicated if cortical or corticospinal tract signs are present on examination, for example, frontal lobe signs, increased reflexes, clonus, or abnormal plantar reflexes.
  • Chest CT may also be indicated. Several individual case studies have reported thymoma in stiff person syndrome.
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Other Tests

  • Electromyography (EMG): Characteristic continuous motor unit activity with normal morphology is especially prominent in the paraspinal muscles. Myotonic potentials are absent. Activity resolves with sleep and abates with benzodiazepines (diazepam). Simultaneous continuous motor activity is noted in opposing muscles.
  • Electroencephalography: EEG is indicated when episodic or paroxysmal stiffness occurs or signs of cortical abnormalities are present on examination. Rare cases of stiff person syndrome with associated refractory partial epilepsy have been reported.[15]
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Procedures

Lumbar puncture and associated CSF studies should be obtained in patients with a presentation that is consistent with stiff person syndrome to rule out other etiologies. Oligoclonal bands can be observed in approximately two thirds of patients with antibody-positive stiff person syndrome.[20] In addition, lumbar puncture can add needed information if the patient's presentation is unusual or complex.

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Contributor Information and Disclosures
Author

Nancy Theresa Rodgers-Neame, MD  Assistant Professor, Department of Molecular Pharmacology and Physiology, University of South Florida College of Medicine; Director, Florida Comprehensive Epilepsy and Seizure Disorders Program

Nancy Theresa Rodgers-Neame, MD is a member of the following medical societies: American Academy of Neurology, American Clinical Neurophysiology Society, American Epilepsy Society, American Medical Women's Association, Society for Neuroscience, and Southern Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Paul E Barkhaus, MD  Professor, Department of Neurology, Medical College of Wisconsin; Director of Neuromuscular Diseases, Milwaukee Veterans Affairs Medical Center

Paul E Barkhaus, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and American Neurological Association

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Glenn Lopate, MD  Associate Professor, Department of Neurology, Division of Neuromuscular Diseases, Washington University School of Medicine; Director of Neurology Clinic, St Louis ConnectCare; Consulting Staff, Department of Neurology, Barnes-Jewish Hospital

Glenn Lopate, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and Phi Beta Kappa

Disclosure: Baxter Grant/research funds Other; Amgen Grant/research funds None

Selim R Benbadis, MD  Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, Tampa General Hospital, University of South Florida College of Medicine

Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association

Disclosure: UCB Pharma Honoraria Speaking, consulting; Lundbeck Honoraria Speaking, consulting; Cyberonics Honoraria Speaking, consulting; Glaxo Smith Kline Honoraria Speaking, consulting; Pfizer Honoraria Speaking, consulting; Sleepmed/DigiTrace Honoraria Speaking, consulting

Chief Editor

Nicholas Lorenzo, MD  Consulting Staff, Neurology Specialists and Consultants

Nicholas Lorenzo, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, and American College of Physician Executives

Disclosure: Nothing to disclose.

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