The Stiff-man Syndrome in Children and Adolescents

Jennifer A. Tracy, Andrew B McKeon

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

The stiff-man syndrome (SMS, also known as stiff-person syndrome) is an autoimmune disorder characterized by stiffness and spasms affecting muscles of limbs and/or axial regions. Rarely reported in childhood and adolescence, SMS may be underrecognized but should be considered in children presenting with a hypertonic or hyperekplectic phenotype. Presentations may include new onset attacks of focal or widespread stiffness and spasms, sometimes precipitated by anxiety, which may lead to falls. The lower extremities and lumbar spine are most commonly affected, but both focal and more extensive "whole-body" phenotypes are recognized. In addition to history and examination findings consistent with SMS, the following are supportive of the diagnosis: a history of autoimmune disease, detection of GAD65 or glycine receptor antibodies in serum or CSF, hyperexcitability on neurophysiological testing, and clinical improvements with benzodiazepines or immunotherapy. Although prognosis varies, most patients improve with treatment, highlighting the importance of early and accurate diagnosis.

Original languageEnglish (US)
Title of host publicationNeuromuscular Disorders of Infancy, Childhood, and Adolescence: A Clinician's Approach
PublisherElsevier Inc.
Pages188-196
Number of pages9
ISBN (Print)9780124171275, 9780124170445
DOIs
StatePublished - Dec 3 2014

Fingerprint

Stiff-Person Syndrome
Spasm
Glycine Receptors
Phenotype
Benzodiazepines
Immunotherapy
Autoimmune Diseases
Early Diagnosis
Lower Extremity
Spine
Extremities
Anxiety
History
Antibodies
Serum
Therapeutics

Keywords

  • Autoimmune
  • Diabetes
  • GAD antibody
  • GAD65 antibody
  • Glycine
  • Stiff child
  • Stiff man
  • Stiff person
  • Thyroid

ASJC Scopus subject areas

  • Medicine(all)
  • Neuroscience(all)

Cite this

Tracy, J. A., & McKeon, A. B. (2014). The Stiff-man Syndrome in Children and Adolescents. In Neuromuscular Disorders of Infancy, Childhood, and Adolescence: A Clinician's Approach (pp. 188-196). Elsevier Inc.. https://doi.org/10.1016/B978-0-12-417044-5.00011-1

The Stiff-man Syndrome in Children and Adolescents. / Tracy, Jennifer A.; McKeon, Andrew B.

Neuromuscular Disorders of Infancy, Childhood, and Adolescence: A Clinician's Approach. Elsevier Inc., 2014. p. 188-196.

Research output: Chapter in Book/Report/Conference proceedingChapter

Tracy, JA & McKeon, AB 2014, The Stiff-man Syndrome in Children and Adolescents. in Neuromuscular Disorders of Infancy, Childhood, and Adolescence: A Clinician's Approach. Elsevier Inc., pp. 188-196. https://doi.org/10.1016/B978-0-12-417044-5.00011-1
Tracy JA, McKeon AB. The Stiff-man Syndrome in Children and Adolescents. In Neuromuscular Disorders of Infancy, Childhood, and Adolescence: A Clinician's Approach. Elsevier Inc. 2014. p. 188-196 https://doi.org/10.1016/B978-0-12-417044-5.00011-1
Tracy, Jennifer A. ; McKeon, Andrew B. / The Stiff-man Syndrome in Children and Adolescents. Neuromuscular Disorders of Infancy, Childhood, and Adolescence: A Clinician's Approach. Elsevier Inc., 2014. pp. 188-196
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