Neurológia pre prax 1/2019
Dissociative movement disorder or stiff limb syndrome?
Stiff person syndrome (SPS) is a rare autoimmune disease characterized by progressive fluctuating muscle hypertonia and superimposed painful muscle spasms, resulting in a gait disorder. Its variant – stiff limb syndrome (SLS) – particularly affects limb muscles, with axial muscles being affected substantially less. The disease may respond favourably to immunosuppressive therapy. The video case report presents a female patient examined repeatedly for transient lower limb stiffness. The patient has been suspected to have a functional movement disorder. The disease had progressed gradually and led to loss of self-sufficiency and becoming bedridden. The patient was subsequently found to be positive for anti-glutamic acid decarboxylase antibodies (anti-GAD-Ab), which is characteristic for SPS/SLS. The introduction of immunosuppressive therapy resulted in a significant improvement in the clinical presentation and quality of life of the patient. The video case report of this patient with SLS primarily aims at highlighting the importance of comprehensive patient evaluation and the need to be aware of rare neurological entities.
Keywords: stiff person syndrome, stiff limb syndrome, dissociative gait disorder, anti-GAD antibodies
