Abstract Details

Stiff person syndrome(SPS) is a rare neurologic disorder that is uncommonly associated with a paraneoplastic etiology. The clinical spectrum of SPS is diverse and recognizing syndromes associated with malignancy is critical to pursuing earlier diagnoses in these patients. Additionally, resolution of symptoms in paraneoplastic disorders is supposed to occur following cancer treatment, however, this is not always the case in paraneoplastic SPS.

To provide an overview of characteristics related to paraneoplastic SPS.

Individuals with paraneoplastic SPS seen at Johns Hopkins Hospital from 1997-2021 were identified and demographic, disease characteristics, SPS phenotype, and malignancy data were collected. SPS phenotypes were assigned by review of history and examination.

We identified 9 paraneoplastic SPS out of 242 patients (3.7%). Mean age at SPS symptom onset was 58.9 years and most were women(n=6). Mean time of SPS symptoms developing before cancer was 12.6 months. The SPS phenotypes included 5 classic, 2 SPS-plus, 1  cerebellar, and 1 stiff limb. Most common identified autoantibodies were anti-amphiphysin(n=4), anti-GAD65 (n=4), anti-ANNA-3 (n=1). Associated cancer diagnoses included breast cancer(n=5), small cell lung cancer(n=2), thymoma(n=1), and rectal adenocarcinoma(n=1). One patient died from cancer complications. Of the survivors, 6 exhibit symptoms/signs of SPS despite cancer treatment.

In evaluating patients with a suspected diagnosis of SPS, close monitoring and age-appropriate cancer screening is recommended. This is of utmost importance in those over the age of 50 years and in patients being evaluated within the first few years from their original SPS symptom onset.  Despite treatment of the underlying cancer, some patients with continue to experience long-term SPS symptoms.