Pisa syndrome in Parkinson's disease: An integrated approach from pathophysiology to management

Abstract

Pisa syndrome was first described in 1972 in patients treated with neuroleptics. Since 2003, when it was first reported in patients with Parkinson's disease (PD), Pisa syndrome has progressively drawn the attention of clinicians and researchers. Although emerging evidence has partially clarified its prevalence and pathophysiology, the current debate revolves around diagnostic criteria and assessment and the effectiveness of pharmacological, surgical, and rehabilitative approaches. Contrary to initial thought, Pisa syndrome is common among PD patients, with an estimated prevalence of 8.8% according to a large survey. Furthermore, it is associated with the following specific patient features: more severe motor phenotype, ongoing combined pharmacological treatment with levodopa and dopamine agonists, gait disorders, and such comorbidities as osteoporosis and arthrosis. The present literature on treatment outcomes is scant, and the uneven effectiveness of specific treatments has produced conflicting results. This might be because of the limited knowledge of Pisa syndrome pathophysiology and its variable clinical presentation, which further complicates designing randomized clinical trials on this condition. However, because some forms of Pisa syndrome are potentially reversible, there is growing consensus on the importance of its early recognition and the importance of pharmacological adjustment and rehabilitation. © 2016 International Parkinson and Movement Disorder Society.

Effects of Deep Brain Stimulation on Postural Trunk Deformities: A Systematic Review

“…Axial postural deformities include trunk anteroflexion (camptocormia), lateroflexion (Pisa syndrome), retroflexion (opisthotonus), and neck anteroflexion (anterocollis), which often complicate Parkinson's disease (PD) . The pathogenesis and pathophysiology of these deformities most likely involve multifactorial central and peripheral mechanisms that are potentially different, depending on the underlying disease process (e.g., PD vs. dystonic syndromes).…”

“…The pathogenesis and pathophysiology of these deformities most likely involve multifactorial central and peripheral mechanisms that are potentially different, depending on the underlying disease process (e.g., PD vs. dystonic syndromes). Though these deformities are relatively uncommon, they are difficult to treat and their progression is associated with significant disability, particularly in patients with PD . Reported effects of deep brain stimulation (DBS) on postural trunk deformities have varied from significant worsening to remarkable improvement.…”

Pisa syndrome in Parkinson�s disease: electromyographic quantification of paraspinal and non-paraspinal muscle activity

“…In our recent, multicenter observational study involving 1631 PD patients, we estimated a PS prevalence of 8.8% (Tinazzi et al, 2015). Two different pathophysiological mechanisms for PS have been postulated: (1) a central mechanism arising from a defect in basal ganglia network function that results in dystonic activity and impaired sensorimotor integration (this hypothesis is supported by both experimental animal studies and clinical findings); and (2) a peripheral mechanism due to spinal osteoarticular changes and paraspinal myopathy (Tinazzi et al, 2016). The pathophysiology of PS is largely unknown, and its clinical variability makes the underlying mechanisms difficult to discern and characterize.…”

“…It is a movement disorder characterized by sustained or intermittent involuntary muscle contractions leading to abnormal posture and twisting movements (Albanese et al, 2013). PS can be classified as a generalized and persistent dystonia involving not only the trunk but also the lower limb muscles (Tinazzi et al, 2013;Geroin et al, 2015;Tinazzi et al, 2016). Previous studies documented the presence of dystonia leading to abnormal EMG muscle activity in PS patients (Di Matteo et al, 2011;Tassorelli et al, 2012;Tinazzi et al, 2013).…”

“…Finally, it is worth noting that the muscle activity in PS may change over time, given that PS has different clinical presentations. It can develop in a chronic fashion with subclinical onset and progressive worsening, or ap- © C I C E d i z i o n i I n t e r n a z i o n a l i pear in a short space of time, showing a rapid progression within days or weeks (Tinazzi et al, 2016). In the chronic condition, other musculoskeletal changes (i.e., muscle-tendon retraction, articular ankyloses) might be involved and produce different patterns of muscle activation.…”