Stiff person syndrome: A rare pediatric case in Southern Brazil - Abstract
Introduction: Here we report a rare pediatric case of stiff person syndrome (SPS), an uncommon neurological disorder characterized by persistent muscular rigidity and spasm affecting primarily axial muscles.
Patient description: An 11 year-old boy presented with a 1-year history of axial muscle pain associated with sternocleidomastoid muscle contractions that progressed to bilateral arm muscle contractions. His medical history included febrile seizures and a learning disability. Physical examination revealed generalized bilateral rigidity of the arms and lateral neck. Laboratory test results were normal. Electroneuromyography showed unremitting paravertebral muscular contraction. Clonazepam treatment improved the patient’s functional status and diminished his pain.
Conclusion: Although SPS is rare, pediatricians should be aware of its diagnostic criteria and the potential to treat juvenile SPS with benzodiazepines.