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Título

Rituximab therapy in the treatment of Stiff-person Syndrome: a case reports series

RESUMO

Case Report: Stiff-person syndrome (SPS) is characterized by a huge axial and proximal members stiffness associated with spasms. Treatment is basically symptomatic, based on the use of benzodiazepines and baclofen as the main therapeutic interventions. However, there are few studies that describe the benefit of using immunosuppressants in this condition. Case 1: A 30-year-old female patient complained of low back pain radiating to the lower limbs and frequent falling episodes. She presented a claudicating gait, hypertonia of the paravertebral, abdominal and lower limb muscles. After using benzodiazepines, the patient reported a partial improvement of symptoms, but maintained hypertonia and mild pain. Anti-GAD serology showed high titers. With immunoglobulin therapy, ambulation has improved, but falls and painful stiffness persisted. She was then treated with infusions of rituximab every 6 months. After 2 years, she reports improvement of symptoms and life quality, resuming the practices of their activities of daily living. Case 2: A 43-year-old female patient complained of pain in the lower limbs, limiting stiffness and recurrent seizures. She presented hypertonia of paravertebral musculature and high titers of anti-GAD. She had already been unsuccessfully treated with immunoglobulin. During 2 years she was treated with benzodiazepines and presented clinical instability with sporadic seizures and no important recovery from the symptoms. After rituximab therapy with infusions every 6 months, there was a significant clinical and life quality improvement. Case 3: A 46-year-old male patient complained of lumbar and lower limbs pain, limitation of movement, frequent epileptic seizures. He presented hypertonia of the paravertebral muscles and lumbar lordosis. Anti-GAD titers showed elevated values. After unsuccessful immunoglobulin therapy, he underwent infusions of rituximab interspersed over 6 months. There was a recovery of motricity and stiffness and complete cessation of pain, contributing to functional improvement and resumption in the practice of their work activities. Discussion: Most common type of SPS is associated with the presence of anti-GAD impairing the GABA function. Rituximab is a monoclonal antibody against CD20, expressed on the surface of B cells. Its use in SPS is still a recent approach. Conclusion: Rituximab may be a benefit drug option in SPS treatment. However, these reports show partial results, requiring long-term follow-up.

Palavras Chave

Stiff-person syndrome, treatment, rituximab, immunosuppressant