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| '''Quick Start:''' ''You can begin to add to or edit text on this wiki doc page by clicking on the edit button above and to the left on this same page. Type in the text you want to add. Once you are done, click the save changes button at the bottom.
| | #redirect[[Stiff person syndrome]] |
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| ''''''
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| ==== Editor-In-Chief: ====
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| Apply to be Editor-In-Chief for this topic: There can be one or more than one Editor-In-Chief. You may also apply to be an Associate Editor-In-Chief of one of the subtopics below. Please email [[mgibson@perfuse.org]] to indicate your interest in serving either as an Editor-In-Chief of the entire topic or as an Associate Editor-In-Chief for a subtopic. Please be sure to attach your CV and or biographical sketch.
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| = Overview =
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| :*Autoimmune disorders with antibodies directed at neurologic targets:
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| ::*SPS: Glutamic Acid Decarboxylase (GAD)
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| ::*Isaac’s syndrome: peripheral nerve Voltage-Gated potassium Channels (VGKC)
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| :::*causes Acquired Neuromyotonia
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| ===== References =====
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| = Epidemiology and Demographics =
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| ===== References =====
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| = Risk Factors =
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| ===== References =====
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| = Screening =
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| ===== References =====
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| = Pathophysiology & Etiology=
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| ===== References =====
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| = Molecular Biology =
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| ===== References =====
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| = Genetics =
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| ===== References =====
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| = Natural History =
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| :*First described in 1956 by Moersch and Woltman
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| :*Characterized by muscle stiffness and rigidity as a result of co-contraction of both agonist and antagonist muscles.
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| :*3 categories of SPS:
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| ::#SPS
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| ::#Progressive encephalomyelitis with rigidity (rapidly progressive disorder, fatal within 16 weeks)
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| ::#Stiff-limb syndrome (asymmetric rigidity and spasms in the distal extremities or face)
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| ===== References =====
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| = Diagnosis =
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| == Differential Diagnosis ==
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| ===== References =====
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| == History and Symptoms ==
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| :*Marked rigidity and painful spasms, particularly in the thoracic and lumbosacral paraspinal, abdominal, and proximal lower extremity muscles
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| :*Onset usually insidious
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| :*Persistent contraction leads to a “board-like” quality
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| :*Fixed thoracolumbar lordosis of spine develops in nearly all cases from co-contraction of abd/paraspinal muscles
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| :*Intermittent painful spasms which may lead to falls due to instability
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| :*10% have generalized seizures or myoclonus
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| :*Spasms are NOT present during sleep
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| ===== References =====
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| == Physical Examination ==
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| === Appearance of the Patient ===
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| === Eyes ===
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| === Ear Nose and Throat ===
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| === Heart ===
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| === Lungs ===
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| === Abdomen ===
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| === Extremities ===
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| === Neurologic ===
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| === Other ===
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| ===== References =====
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| == Laboratory Findings ==
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| === Electrolyte and Biomarker Studies ===
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| =====References=====
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| === Electrocardiogram ===
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| =====References=====
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| === Chest X Ray ===
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| =====References=====
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| === MRI and CT ===
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| Conventional MRI and CT commonly normal
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| =====References=====
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| === Echocardiography or Ultrasound ===
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| =====References=====
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| === Other Imaging Findings ===
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| =====References=====
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| === Other Diagnostic Studies ===
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| ===== References =====
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| = Risk Stratification and Prognosis=
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| ===== References =====
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| = Treatment =
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| == Pharmacotherapy ==
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| === Acute Pharmacotherapies ===
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| :*Benzodiazepines (enhance the affinity of GABA receptors)
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| :*Oral or intrathecal baclofen
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| :*Vigabatrin (irreversible inhibitor of GABA transaminase)
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| :*Valproic Acid, Neurontin
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| :*Injected botulinum toxin benefits some
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| :*Prednisone, plasmapheresis, IVIG all used with some success, but no randomized trials
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| ===== References =====
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| === Chronic Pharmacotherapies ===
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| == Surgery and Device Based Therapy ==
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| === Indications for Surgery ===
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| ===== References =====
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| === Pre-Operative Assessment ===
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| === Post-Operative Management ===
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| === Transplantation ===
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| ===== References =====
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| == Primary Prevention ==
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| == Secondary Prevention ==
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| ===== References =====
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| == Cost-Effectiveness of Therapy ==
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| ===== References =====
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| == Future or Investigational Therapies ==
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| ===== References =====
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| ==Suggested Revisions to the Current Guidelines==
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| == References ==
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| <biblio>
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| </biblio>
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| == Acknowledgements ==
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| The content on this page was first contributed by: Stephen Wiviott, M.D.
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| List of contributors:
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| == Suggested Reading and Key General References ==
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| == Suggested Links and Web Resources ==
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| == For Patients ==
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| [[Category:]
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