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Query: UMLS:C0013421 (dystonia)
 8,418 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Patients with Parkinson's disease (PD) commonly develop severe spinal deformity, including scoliosis, antecollis, camptocormia, and Pisa syndrome. The etiology of PD-associated spinal deformity is not completely understood and in most cases is likely due to multiple interrelated factors, including central dystonia and focal myopathy. Once spinal deformity has occurred, surgery is often the only modality that can correct the condition, although control of the movement disorder through medication and deep brain stimulation may slow progression. Advances in spinal instrumentation and deformity correction techniques have improved the outcomes of PD spinal deformity patients, though complications and revision surgery rates remain high. Surgical intervention is reserved for individuals who are physiologically healthy and whose condition is refractory to nonoperative management and follows similar treatment principles as other causes of neuromuscular scoliosis/kyphosis. Spinal deformity patients with PD are optimally treated by spinal deformity surgeons who are familiar with the unique needs of PD patients, with vigilant preoperative and postoperative treatment of their movement disorder and bone density.
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PMID:Etiology and Management of Spinal Deformity in Patients With Parkinson's Disease. 3028 78

Postural abnormalities in Parkinson's disease (PD) are considered the rule more than the exception and are disabling complications of the disease. These deformities include camptocormia, antecollis, Pisa syndrome, and scoliosis. Evidence to date suggests that postural deformities have a multifactorial pathophysiology, including muscular rigidity, axial dystonia, weakness due to myopathy, body scheme defects due to centrally impaired proprioception, and structural changes in the spine. Antecollis in parkinsonian disorders refers to a forward flexion of the head and neck. It is usually mild in severity and may be considered part of the stooped posture in patients with PD. Some authors that suggest the term antecollis should only be used in patients with at least a minimum of 45 degrees of thoracolumbar flexion. Neither camptocormia nor Pisa syndrome can be evaluated without taking into account the presence or absence of scoliosis. In this regard, the rotating component of the spine and its behavior in the supine position give important clues for a correct diagnosis. In some cases, X-rays in the standing and supine positions are necessary. The presence of marked camptocormia requires a minimum of flexion in the sagittal plane originating in the thoracolumbar spine greater than 45 degrees, with an almost complete resolution in the supine position. Pisa syndrome requires a minimum of 10 degrees of lateral flexion and is almost completely alleviated by passive mobilization or supine positioning. A certain degree of scoliosis is expected in most parkinsonian patients; therefore, both camptocormia and Pisa syndrome do not generally present as pure syndromes.
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PMID:How Do I Examine Postural Disorders in Parkinson's Disease? 3083 58

Pisa syndrome, defined as dystonia leading to lateral flexion of the spine, is an increasingly recognized complicating factor in the treatment of Parkinson's disease (PD). Symptoms may persist despite medical therapy, or medical therapy may not be tolerated due to adverse effects. Here, we demonstrate the long-term efficacy of deep brain stimulation (DBS) at the globus pallidus internus (GPi) for the treatment of Pisa syndrome. One patient with Pisa syndrome and Parkinson disease underwent bilateral GPi DBS with computed tomography (CT)-and microelectrode-based guidance. Follow-up with neurosurgery and neurology was done over a four-year period. The patient's axial deformity decreased from approximately 45 to 25 degrees, and he reported significant relief from back pain. Bilateral GPi DBS is a safe and effective option for Pisa syndrome in patients with PD.
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PMID:Long-term Outcome of Globus Pallidus Internus Stimulation for Pisa Syndrome. 3089 79

Clinical diagnosis of multiple system atrophy is challenging and many patients with Lewy body disease (i.e. Parkinson's disease or dementia with Lewy bodies) or progressive supranuclear palsy are misdiagnosed as having multiple system atrophy in life. The clinical records of 203 patients with a clinical diagnosis of multiple system atrophy were reviewed to identify diagnostic pitfalls. We also examined 12 features supporting a diagnosis of multiple system atrophy (red flag features: orofacial dystonia, disproportionate antecollis, camptocormia and/or Pisa syndrome, contractures of hands or feet, inspiratory sighs, severe dysphonia, severe dysarthria, snoring, cold hands and feet, pathological laughter and crying, jerky myoclonic postural/action tremor and polyminimyoclonus) and seven disability milestones (frequent falls, use of urinary catheters, wheelchair dependent, unintelligible speech, cognitive impairment, severe dysphagia, residential care). Of 203 cases, 160 (78.8%) were correctly diagnosed in life and had pathologically confirmed multiple system atrophy. The remaining 21.2% (43/203) had alternative pathological diagnoses including Lewy body disease (12.8%; n = 26), progressive supranuclear palsy (6.4%; n = 13), cerebrovascular diseases (1%; n = 2), amyotrophic lateral sclerosis (0.5%; n = 1) and cerebellar degeneration (0.5%; n = 1). More patients with multiple system atrophy developed ataxia, stridor, dysphagia and falls than patients with Lewy body disease; resting tremor, pill-rolling tremor and hallucinations were more frequent in Lewy body disease. Although patients with multiple system atrophy and progressive supranuclear palsy shared several symptoms and signs, ataxia and stridor were more common in multiple system atrophy. Multiple logistic regression analysis revealed increased likelihood of multiple system atrophy versus Lewy body disease and progressive supranuclear palsy if a patient developed orthostatic hypotension or urinary incontinence with the requirement for urinary catheters [multiple system atrophy versus Lewy body disease: odds ratio (OR): 2.0, 95% confidence interval (CI): 1.1-3.7, P = 0.021; multiple system atrophy versus progressive supranuclear palsy: OR: 11.2, 95% CI: 3.2-39.2, P < 0.01]. Furthermore, autonomic dysfunction within the first 3 years from onset can differentiate multiple system atrophy from progressive supranuclear palsy (multiple system atrophy versus progressive supranuclear palsy: OR: 3.4, 95% CI: 1.2-9.7, P = 0.023). Multiple system atrophy patients with predominant parkinsonian signs had a higher number of red flag features than patients with Lewy body disease (OR: 8.8, 95% CI: 3.2-24.2, P < 0.01) and progressive supranuclear palsy (OR: 4.8, 95% CI: 1.7-13.6, P < 0.01). The number of red flag features in multiple system atrophy with predominant cerebellar signs was also higher than in Lewy body disease (OR: 7.0, 95% CI: 2.5-19.5, P < 0.01) and progressive supranuclear palsy (OR: 3.1, 95% CI: 1.1-8.9, P = 0.032). Patients with multiple system atrophy had shorter latency to reach use of urinary catheter and longer latency to residential care than progressive supranuclear palsy patients, whereas patients with Lewy body disease took longer to reach multiple milestones than patients with multiple system atrophy. The present study has highlighted features which should improve the ante-mortem diagnostic accuracy of multiple system atrophy.
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PMID:Improving diagnostic accuracy of multiple system atrophy: a clinicopathological study. 3149 60

Pisa syndrome (PS) is a rare lateral truncal dystonia that is related to dopamine-acetylcholine imbalances, and most cases develop during antipsychotic treatment. Here, we report a case of PS that developed during switching to new antipsychotics and titrating lithium, and PS was aggravated when the lithium dose was increased. Truncal deviation was not relieved with switching back to prior antipsychotics or by discontinuation of all antipsychotics. Pisa syndrome resolved only after discontinuing the lithium treatment. This is the first report of dose-dependent effects of lithium treatment on the aggravation of PS, which may be related to dose-related effects on dopaminergic and cholinergic transmission.
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PMID:Dose-Dependent Effects of Lithium Treatment on the Aggravation of Antipsychotic-Induced Pisa Syndrome. 3238 12


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