Stiff Person Syndrome in the Hospice Patient: A Case Report and Discussion

Introduction

Stiff Person Syndrome (SPS) is a rare autoimmune-mediated neurological disorder that presents significant challenges in palliative and end-of-life care. Characterized by muscle stiffness, episodic spasms, and exaggerated startle responses to sensory stimuli, SPS can lead to profound disability and distress. ¹ The pathophysiology of SPS is linked to autoantibodies—most commonly against glutamic acid decarboxylase (GAD), which impairs GABAergic inhibition within the central nervous system. Treatment is largely symptomatic and includes immunomodulatory therapies like intravenous immunoglobulin (IVIG), intrathecal baclofen (ITB), and gamma-aminobutyric acid (GABA)-enhancing agents such as benzodiazepines and anticonvulsants. ¹

As SPS is a chronic and progressive disorder without a curative option, management becomes increasingly complex when layered atop life-limiting illness. In the hospice setting, where emphasis is placed on comfort rather than disease modification, continued use of neurological treatments may raise concerns about cost, scope, and philosophical alignment with end-of-life care goals. Yet, for conditions like SPS, withdrawal of key therapies may precipitate painful spasms, functional decline, or emotional distress—counter to the very aims of palliative care.

This case report illustrates the challenges and opportunities involved in caring for a patient with SPS and metastatic cancer at the end of life. It underscores how interdisciplinary collaboration, flexible thinking, and individualized planning can support symptom control, support preservation of dignity, and align care with the patient’s values—even when treatments fall outside conventional hospice protocols.

Case Description

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, requiring increasing assistance with activities of daily living, 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. She was also prescribed phenobarbital for seizure control.

She was not a candidate for further disease-directed therapies for her malignancy and declined radiation for hemostasis because she felt the burdens of further radiation for hemostasis outweighed the perceived benefit. ² She expressed a clear preference for comfort-focused care but was hesitant to enroll in hospice due to concern that her muscle spasms and pain would not be adequately managed without her usual neurological medications. Particularly given that the abrupt withdrawal of IVIG or baclofen can lead to recurrence in muscle stiffness, spasms, and functional decline. ¹ This concern became the central tension in her end-of-life planning: whether her hospice team could continue management of symptoms with the successful therapy she had been receiving.

Palliative Care collaborated with Neurology, the primary team, and the hospice medical director to create a care plan focused on minimizing discomfort in alignment with the hospice philosophy. 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. ³ Though these medications are not commonly emphasized in standard hospice formularies, their targeted use in this case illustrates how individualized care plans can align with hospice goals while focusing on symptom relief.

Environmental modifications complemented pharmacologic strategies. The team reduced exposure to potential spasm triggers by minimizing light and 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. An interdisciplinary approach supported her comfort, with anticipatory as-needed orders for dyspnea, anxiety, and spasms. She remained at home with well-controlled symptoms for the final three weeks of her life, underscoring that continuation of IVIG and ITB provided meaningful comfort during her last days. Her symptoms were well controlled at home, and she avoided emergency department visits or hospital readmission.

Discussion

SPS is a rare, progressive neurological disorder characterized by muscle rigidity, painful spasms, and hypersensitivity to external stimuli.^4,5 The condition predominantly affects the axial muscles and limbs, leading to functional impairment and, in severe cases, immobility. SPS is most commonly diagnosed in adults between the ages of 30 and 60, with a female predominance. It can be initially misdiagnosed as other movement disorders due to its similar presentation. ⁶ The underlying pathophysiology involves autoantibodies against the enzyme GAD, which disrupts GABA-mediated inhibitory signaling in the central nervous system. ⁴ This leads to the uncontrolled muscle activity characteristic of the disease.^1,6 The diagnosis of SPS is primarily clinical and confirmed by the presence of GAD antibodies and characteristic findings on electromyography, although diagnosis can be challenging, especially in the early stages. ¹ The disease course is progressive, with patients experiencing exacerbations triggered by stress, illness, or injury. Over time, this results in significant disability and a reduced quality of life. ⁵

As there is no cure for SPS, treatment is primarily aimed at controlling symptoms and improving function. The cornerstone of pharmacologic therapy includes immunomodulatory treatments such as IVIG and ITB. IVIG works by modulating the immune response and can provide relief by reducing the severity of muscle stiffness and spasms, particularly in GAD-positive patients. ⁷ ITB, a GABA-B agonist, is effective in alleviating spasticity by directly targeting the spinal cord, where the GABAergic deficiency occurs. This therapy is especially valuable for patients who do not respond to oral medications, as it provides localized relief while minimizing systemic side effects.^6,8 Other pharmacologic agents, such as benzodiazepines (e.g., diazepam) and anticonvulsants (e.g., levetiracetam), are commonly used to address anxiety, muscle relaxation, and spasms. ⁹

Non-pharmacologic interventions are also essential in the management of SPS. Environmental modifications, such as reducing sensory stimuli (e.g., dimming lights, minimizing noise), can significantly reduce the frequency of spasms triggered by external stimuli.^1,5,10 In this case, the palliative care team worked closely with the patient to identify and eliminate potential triggers. Repositioning and physical therapy are key in preventing contractures and maintaining comfort as the disease progresses.

Additionally, interdisciplinary collaboration is critical in creating a holistic care plan that addresses not only the physical symptoms but also the psychological and emotional challenges that patients with SPS face, especially as they approach the end of life. Despite the potential benefits of hospice, patients with neurological diseases—including neurodegenerative conditions—are significantly less likely to utilize hospice services at the end of life. ¹¹ This underutilization may stem from prognostic uncertainty, symptom complexity, and concerns about whether hospice can adequately address neurological needs. ¹¹

Social work and spiritual care are important components in providing emotional support and facilitating decision-making, helping patients and families navigate complex issues related to the disease and its prognosis.^5,12 While cost and standardization are important considerations, this case also reflects the foundational philosophy of hospice, which is to provide individualized care that prioritizes comfort and dignity at the end of life. A tailored care plan that incorporates both pharmacologic and nonpharmacologic interventions is essential to supporting comfort for patients with complex conditions such as SPS.

Conclusion

This case of a patient with SPS receiving hospice care underscores the complexity of managing rare neurological 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, such as 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.^7,9 Additionally, environmental modifications—such as minimizing sensory stimuli—can play a significant role in reducing symptom exacerbations. ⁶ Alongside pharmacologic treatments, spiritual and psychosocial support are crucial for addressing the emotional and existential challenges faced by patients with SPS, helping them and their families find peace and meaning during this vulnerable time.^5,9

Ongoing research and clinical guidance are vital to refining the management of rare diseases like SPS in the hospice setting. Current hospice reimbursement structures often prioritize standardized, familiar, and low-cost interventions, which can inadvertently exclude patients with rare or complex conditions who require specialized therapies. Without reform, these limitations risk perpetuating inequities in end-of-life care. Addressing this challenge will require advocacy, policy innovation, and research into sustainable models that support individualized, high-quality care for all patients, regardless of diagnosis.