Stiff Person Syndrome (SPS) is a rare autoimmune neurologic disorder characterized by progressive muscle rigidity and painful spasms, often precipitated by stress, illness, or external stimuli. It primarily affects axial and proximal limb muscles, leading to significant functional impairment and, in severe cases, immobility. SPS most commonly presents in adults between the ages of 30 and 60, with a slight predominance in women, and is frequently misdiagnosed due to overlap with other movement and neuromuscular disorders.

The underlying pathophysiology involves autoantibodies directed against glutamic acid decarboxylase (GAD), resulting in reduced gamma-aminobutyric acid (GABA)–mediated inhibition within the central nervous system. This disruption leads to increased muscle activity and stiffness. Diagnosis is largely clinical, supported by the presence of GAD antibodies and characteristic electromyography findings demonstrating continuous motor unit activity.

Management typically includes immunomodulatory therapies such as intravenous immunoglobulin and symptomatic treatments like benzodiazepines or intrathecal baclofen. However, the use of these interventions in hospice and palliative care settings remains complex and is often guided by goals of care, prognosis, and treatment burden.
This case highlights the importance of interdisciplinary collaboration in managing SPS in the context of advanced illness. Through coordinated efforts between palliative care, neurology, and hospice teams, an individualized approach to symptom management was developed for a patient with coexisting metastatic cancer and SPS. This approach prioritized comfort, functional goals, and quality of life while navigating the challenges of treating a rare neurologic condition in a palliative context.

Stiff Person Syndrome (SPS) is a rare neurologic disorder marked by progressive muscle rigidity and painful spasms, often triggered by stress, illness, or external stimuli. It mainly affects axial and limb muscles, causing functional impairment and sometimes immobility. Most cases occur in adults aged 30–60, with a slight female predominance, and are frequently misdiagnosed as other movement disorders. SPS involves autoantibodies against glutamic acid decarboxylase (GAD), disrupting gamma-aminobutyric acid (GABA)–mediated inhibition and causing uncontrolled muscle activity.1,2 Diagnosis is clinical, supported by GAD antibody testing and electromyography (EMG) findings. Standard treatments include intravenous immunoglobulin (IVIG) and intrathecal baclofen (ITB), though their role in hospice is debated. This case illustrates how collaboration between Palliative Care, Neurology, and Hospice enabled tailored symptom control in a patient with metastatic cancer and SPS.

A 57-year-old woman with metastatic colorectal cancer, Stiff Person Syndrome (SPS), and absence seizures was admitted with fatigue and melena. At baseline, she was functionally limited but independent, with SPS managed by biweekly IVIG and intrathecal baclofen (ITB). Abrupt withdrawal of either can trigger muscle stiffness, spasms, and decline.1 She also used phenobarbital for seizures.
With disease progression and declining status, she was not eligible for further systemic therapy and declined hemostatic radiation. She preferred comfort-focused care but hesitated to enroll in hospice, as coverage rarely includes the costly IVIG/ITB that controlled her pain. This became the central challenge in her end-of-life planning: ensuring symptom control while addressing the unique palliative needs of SPS.

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.

This case highlights the challenges of managing rare neurologic diseases like SPS at the end of life. Standard hospice care may require adaptation when painful spasms and rigidity dominate symptoms. In SPS, interventions such as IVIG and ITB can be essential for palliation, directly targeting immune and spinal cord pathways to reduce stiffness and spasms. Environmental modifications, along with spiritual and psychosocial support, help address both physical and emotional distress.2
Advancing research, updated clinical guidance, and flexible reimbursement models are critical to improve access to disease-specific therapies and ensure patient-centered care. Combining comfort-focused and targeted strategies enables patients with SPS to die with dignity, consistent with the principles of whole-person palliative care.