P-054 - (Poster #54) Stiff Person Syndrome in the Hospice Setting: A Case Report of Collaborative Symptom Management

The purpose of this work is threefold: 


1. Participants will be able to recognize the clinical features and symptom burden of SPS. 


2. Participants will be able to describe palliative approaches, including continuation of neurologic therapies for symptom relief. 


3. Participants will be able to coordinate interdisciplinary strategies to optimize comfort in patients with rare neurologic conditions at the end of life. 


Methods:

A 57-year-old woman with a history of metastatic colorectal cancer, SPS, and absence seizures was admitted to the hospital with fatigue and melena. At baseline, she was functionally limited but independent, and her SPS was managed with biweekly IVIG and ITB. Her primary symptom burden included episodic muscle cramping and spasms, which were typically exacerbated by stress or illness. The abrupt withdrawal of IVIG or baclofen can lead to recurrence in muscle stiffness, spasms and functional decline. She was also prescribed phenobarbital for seizure control. 

Given her declining performance status and disease progression she was not a candidate for further systemic therapy and she declined radiation for hemostasis, understanding its low potential for benefit. She expressed a clear preference for comfort-focused care but was hesitant to enroll in hospice as hospice typically cannot cover the prohibitive cost of IVIG/ITB - medications that controlled her pain. This concern became the central tension in her end-of-life planning: whether her hospice team could ensure symptom control while adapting to the complex palliative needs of SPS. 

The patient provided verbal consent for use of de-identified case information. 



Results:

Palliative Care collaborated with Neurology, the primary team, and the hospice medical director to create a care plan focused on minimizing discomfort. After discussion, it was agreed that both IVIG and ITB could be continued under the hospice plan of care—not for disease modification, but for symptom palliation. She was also scheduled for a blood transfusion for fatigue management prior to discharge—an intervention that was approved due to her unique needs. 

After discharge, the patient experienced a progression of muscle spasms and rigidity, consistent with the natural course of SPS. Her care team responded by augmenting her regimen with medications that enhance GABAergic transmission. Low-dose diazepam was introduced to provide muscle relaxation and anxiolysis. Levetiracetam was also added, reflecting literature supporting its role in reducing stimulus-sensitive spasms in SPS.  

Though these medications are not commonly covered in standard hospice formularies, their use in this case illustrates how individualized care plans can align with hospice goals while ensuring symptom relief. Environmental modifications complemented pharmacologic strategies. The team reduced exposure to potential spasm triggers by minimizing light, sound, and repositioning. The hospice team, after receiving targeted education about SPS, worked closely with pharmacy and nursing colleagues to ensure timely responses to symptom changes. We ultimately were able to support her comfort, with anticipatory PRN orders for dyspnea, anxiety, and spasms. Her symptoms were well controlled at home, and she avoided emergency department visits or hospital readmission. 


Conclusions/Implications for future research and/or clinical care:

This case underscores the complexity of managing rare neurologic diseases at the end of life. Standard hospice approaches may need adaptation when dealing with conditions like SPS, where painful spasms and rigidity are the primary sources of distress. Disease-specific interventions, including IVIG and ITB, are essential for symptom palliation in SPS, as they modulate the immune system and target the spinal cord to alleviate muscle stiffness and spasms. Additionally, environmental modifications can play a significant role in reducing symptom exacerbations. Likewise, spiritual and psychosocial support are also crucial for addressing the emotional and existential challenges faced by patients with SPS. 

Ongoing research and clinical guidance are vital to refining the management of rare diseases like SPS in the hospice setting. As the understanding of SPS evolves, new treatment strategies—including immunomodulatory therapies and GABAergic agents—hold promise for improving patient outcomes. Furthermore, continued discussion around flexible reimbursement models and equitable access to specialized care will be important to ensure that care remains patient-centered. Ultimately, integrating comfort-focused and disease-specific strategies allows patients with SPS to experience a dignified death, aligned with the core values of high-quality, whole-person palliative care.