Case Report : Intrathecal baclofen use in stiff person syndrome
Akshay S. Agrawala, M.D., Rashmi Rikhib Ph.D., Anterpreet Duac, M.D., Mahesh Pattabiramand, Ph.D., Pravardhan Birthid,e, M.D.,
a.Bryan Health, Grand Island, NE
b.University of Nebraska Kearney, Kearney, NE
c.Augusta University, Augusta, NE
d.Tricity Research Center, Grand Island, NE
e.Grand Island Pain Relief Center, Grand Island, NE
Background:
•Rare Autoimmune Disorder: Progressive muscle stiffness, painful spasms, and walking difficulties; often linked to anti-GAD65 antibodies.
•Diagnostic Challenge: Easily misdiagnosed due to overlap with other neuromuscular conditions.
•Limitations of Oral Therapy: Baclofen, diazepam, and tizanidine may give partial relief and cause side effects.
•Intrathecal Baclofen (ITB): Effective in refractory cases, showing significant symptom improvement.
Case:
Patient Background
•57-year-old male with right auricular melanoma
•Previous treatments: partial auriculectomy, sentinel node biopsy, radiotherapy, pembrolizumab
•4 years later: fatigue, dizziness, hand tremors
Disease Progression and Imaging
•Lung nodule → metastatic melanoma confirmed
•PET scan: hypermetabolic activity (lung, subcarinal & cervical nodes, adrenal gland)
•Labs: eosinophilia
•EMG: lower extremity motor neuron/axon injury
Symptom Management (Pre-Diagnosis)
•Symptoms: generalized pain, cramps, dysphagia, fatigue, disrupted sleep
•Medications tried: baclofen, tizanidine, hydrocodone, diazepam, gabapentin, tramadol, prednisone, dantrolene
•Partial relief: diazepam (cramps), gabapentin (neuropathic pain)
•Physical therapy: aquatic therapy unsuccessful
•Oswestry Disability Index (from 0-100): 28 (back; Moderate Disability), 26 (neck; Moderate Disability)
Diagnosis and Targeted Therapy
•Referred to pain management → diagnosed with Stiff Person Syndrome
•Intrathecal baclofen (ITB) pump implanted:
•Start: 75 mcg/day → Titrated to 196 mcg/day
•Outcomes:
•Complete e resolution of lower extremity stiffness
•Reduced pain & opioid requirement
Current Status and Ongoing Issues
•Persistent stiffness & pain: upper extremities, neck, jaw
•Supportive care: stool softeners, ongoing monitoring
•Opioid Risk Tool & CAGE-AID: negative
Discussion:
•Intrathecal baclofen (ITB) offers targeted GABAergic modulation, improving stiffness and spasms in refractory SPS while minimizing systemic side effects (Dalakas, 2009; McKeon et al., 2012; Dalakas, 2020).
•Multidisciplinary management—involving neurology, pain medicine, and physical therapy—is crucial for optimizing outcomes and maintaining functional improvement (Murinson & Guarnaccia, 2008; Rakocevic & Floeter, 2016)
Conclusion:
•Intrathecal baclofen pump therapy is a safe and effective option for patients with refractory stiff person syndrome, providing marked symptom relief and improved quality of life.
•Early diagnosis and multidisciplinary management are essential for optimizing outcomes in this rare and disabling neurological disorder.
References:
•Dalakas MC. Exp Neurol. 2009;217(2):166–168.
•Dalakas MC. Neurotherapeutics. 2020;17(3):1391–1402.
•McKeon A, Robinson MT, McEvoy KM, et al. Arch Neurol. 2012;69(2):230–238.
•Murinson BB, Guarnaccia JB. Neurology. 2008;71(24):1955–1958.
Rakocevic G, Floeter MK. Front Neurol. 2016;7:148.