eMedicine Specialties > Neurology > Neuromuscular Diseases

Stiff Person Syndrome: Differential Diagnoses & Workup

Author: Nancy Theresa Rodgers-Neame, MD, Assistant Professor, Department of Molecular Pharmacology and Physiology, University of South Florida; Director, Florida Comprehensive Epilepsy and Seizure Disorders Program
Contributor Information and Disclosures

Updated: Aug 27, 2009

Differential Diagnoses

Congenital Myopathies
Metabolic Myopathies
Spinal Cord Trauma and Related Diseases

Other Problems to Be Considered

Painful tonic spasms of multiple sclerosis
Hereditary hyperekplexia
Idiopathic hyperekplexia
Cocaine-addicted infant
Startle syndrome
Hereditary startle disease
Stiff encephalomyelitis
Paraneoplastic hypertonic syndrome
Tetanus
Somatoform disorder
Myoclonic seizures
Axial torsion dystonia
Neuromyotonia (Isaac syndrome)
Schwartz-Jampel syndrome

Workup

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

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.

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.14

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.19 In addition, lumbar puncture can add needed information if the patient's presentation is unusual or complex.

More on Stiff Person Syndrome

Overview: Stiff Person Syndrome
Differential Diagnoses & Workup: Stiff Person Syndrome
Treatment & Medication: Stiff Person Syndrome
Follow-up: Stiff Person Syndrome
References
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References

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Further Reading

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Keywords

stiff person syndrome, SPS, stiff man syndrome, SMS, stiff baby syndrome, SBS, hyperekplexia, Moersch-Woltmann syndrome, stiff woman syndrome, stiff limb syndrome, spasticity, GABA, muscle rigidity, axial muscle rigidity, proximal limb muscle rigidity, muscle stiffness, muscle spasm, intermittent rigidity, autoimmune disease, glutamic acid decarboxylase, GAD, GAD antibodies, glycine, spinal motor neurons, motor pathway inhibitor, GABA, motor neurons, motor neuron dysfunction, glutamate, baclofen pump failure, upright posture, posture discomfort, spasm, intermittent spasm, startle syndrome, lumbar lordosis, spastic, stiff encephalomyelitis, paraneoplastic hypertonic syndrome, paraneoplastic syndrome, PNS, myoclonic seizure, seizure

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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; 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.

Medical Editor

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

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Glenn Lopate, MD, Associate Professor, Department of Neurology, Division of Neuromuscular Diseases, Washington University School of Medicine; Chief of Neurology, St Louis ConnectCare, Consulting Staff, 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: Nothing to disclose.

CME Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
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: Nothing to disclose.

Chief Editor

Nicholas Y Lorenzo, MD, Chief Editor, eMedicine Neurology; Consulting Staff, Neurology Specialists and Consultants
Nicholas Y Lorenzo, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Neurology
Disclosure: Nothing to disclose.

 
 
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