Stiff Person Syndrome: Hidden in Plain Sight

Document Type

Abstract

Location

Virtual

Start Date

24-4-2026 9:00 AM

End Date

24-4-2026 3:00 PM

Description

Case Presentation: We describe a case of a 51-year-old woman with multiple cardiopulmonary comorbidities who was admitted for evaluation of dyspnea and tachycardia noted at her skilled nursing facility. She was found to be in atrial fibrillation with rapid ventricular response, complicated by acute-on-chronic hypoxemic respiratory failure. The patient required non-invasive ventilation and amiodarone infusion, with prompt resolution of her symptoms. Further history obtained during hospitalization revealed several months of progressive generalized stiffness and intermittent, painful spasms most pronounced in her lower extremities, rendering her bedbound and fully dependent for activities of daily living. The physical examination revealed marked hypertonicity, hyperextension, and a reduced range of motion in all extremities, though most severe in the legs. Involuntary muscle contractions were elicited by tactile and auditory stimuli, while cranial nerve function, deep tendon reflexes, and sensation were preserved. Strength testing was limited due to severe hypertonicity. Serologic testing revealed a markedly elevated GAD-65 antibody titer requiring dilution (>25,000 U/mL). Neurology consultation confirmed the diagnosis of SPS, and the patient was initiated on a five-day course of intravenous immunoglobulin (IVIG), with limited improvement, likely reflecting permanent contractures secondary to chronic, untreated disease. Discussion: SPS remains a rare and underrecognized disorder, likely misattributed to generalized debility from chronic illness, aging, or prolonged hospitalization, as occurred in this case. In this patient, timely diagnosis was further obscured by concurrent cardiopulmonary pathology that initially masked the underlying neurologic process. By adding to the limited body of literature on SPS, we aim to heighten clinical suspicion for the disorder in patients with unexplained rigidity or stimulus-induced spasms, as early recognition is key to mitigating long-term disability.

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Apr 24th, 9:00 AM Apr 24th, 3:00 PM

Stiff Person Syndrome: Hidden in Plain Sight

Virtual

Case Presentation: We describe a case of a 51-year-old woman with multiple cardiopulmonary comorbidities who was admitted for evaluation of dyspnea and tachycardia noted at her skilled nursing facility. She was found to be in atrial fibrillation with rapid ventricular response, complicated by acute-on-chronic hypoxemic respiratory failure. The patient required non-invasive ventilation and amiodarone infusion, with prompt resolution of her symptoms. Further history obtained during hospitalization revealed several months of progressive generalized stiffness and intermittent, painful spasms most pronounced in her lower extremities, rendering her bedbound and fully dependent for activities of daily living. The physical examination revealed marked hypertonicity, hyperextension, and a reduced range of motion in all extremities, though most severe in the legs. Involuntary muscle contractions were elicited by tactile and auditory stimuli, while cranial nerve function, deep tendon reflexes, and sensation were preserved. Strength testing was limited due to severe hypertonicity. Serologic testing revealed a markedly elevated GAD-65 antibody titer requiring dilution (>25,000 U/mL). Neurology consultation confirmed the diagnosis of SPS, and the patient was initiated on a five-day course of intravenous immunoglobulin (IVIG), with limited improvement, likely reflecting permanent contractures secondary to chronic, untreated disease. Discussion: SPS remains a rare and underrecognized disorder, likely misattributed to generalized debility from chronic illness, aging, or prolonged hospitalization, as occurred in this case. In this patient, timely diagnosis was further obscured by concurrent cardiopulmonary pathology that initially masked the underlying neurologic process. By adding to the limited body of literature on SPS, we aim to heighten clinical suspicion for the disorder in patients with unexplained rigidity or stimulus-induced spasms, as early recognition is key to mitigating long-term disability.