Pediatric Stiff-Person Spectrum Disorders

  • Pichet Termsarasab
  • Thananan Thammongkolchai
  • Bashar Katirji


Pediatric stiff-person spectrum disorders (SPSD) are even much less common than adult SPSD. Data on pediatric SPSD are very limited. The phenotypes described in pediatric SPSD are similar to adults SPSD, most commonly the classic stiff-person syndrome phenotype. The stiff-limb syndrome and progressive encephalomyelitis with rigidity and myoclonus phenotypes have also been reported in children. Pediatric SPSD are associated with anti-glutamic acid decarboxylase 65 (anti-GAD 65) and anti-glycine receptor antibodies. Differential diagnoses include hereditary hyperekplexia (aka stiff-baby syndrome), disorders manifesting with dystonia or spasticity, tonic seizures, as well as tetanus. Aggressive treatment with immunotherapies is often used in the pediatric population. Future research on SPSD dedicated to pediatric populations is warranted.


Pediatric Stiff-person syndrome Stiff-person spectrum disorders Hyperekplexia Stiff-baby syndrome Stiffness Tetanus 


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© Springer Nature Switzerland AG 2020

Authors and Affiliations

  • Pichet Termsarasab
    • 1
  • Thananan Thammongkolchai
    • 1
  • Bashar Katirji
    • 2
  1. 1.Division of NeurologyFaculty of Medicine Ramathibodi Hospital, Mahidol UniversityBangkokThailand
  2. 2.Department of NeurologyUniversity Hospitals Cleveland Medical Center, Case Western Reserve UniversityClevelandUSA

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