Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0013421 (dystonia)
 8,418 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Botulinum toxin (BoNT) has gained widespread use in a variety of neurological conditions. Parkinson's disease is a complex neurodegenerative disorder manifested by motor and non-motor symptoms that can cause significant disability. BoNT has been used to effectively treat a variety of symptoms related to Parkinson's disease. This review will examine the current therapeutic indications of BoNT use in the following disorders related to Parkinson's disease: cervical dystonia, blepharospasm and lid apraxia, focal hand dystonia, foot dystonia, laryngeal dystonia, oromandibular dystonia, camptocormia, hand and jaw tremor, sialorrhea, hyperhidrosis, dysphagia, constipation, and overactive bladder.
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PMID:An update on the use of botulinum toxin therapy in Parkinson's disease. 2540 33

Parkinson`s disease (PD) is the most common neurodegenerative disease and is characterized by tremor, rigidity and akinesia. Diagnosis is clinical in the majority of the patients. Patients with PD may have stooped posture but some of them develop different types of postural and striatal deformities. Usually these deformities are more common in atypical parkinsonian disorders such as progressive supranuclear palsy and multisystem atrophy. But in many studies it has been highlighted that these may also be present in approximately one third of PD patients leading to severe disability. These include antecollis or dropped head, camptocormia, p0 isa syndrome, scoliosis, striatal hands and striatal toes. The pathogenesis of these deformities is a complex combination of central and peripheral influences such as rigidity, dystonia and degenerative skeletal changes. Duration of parkinsonism symptoms is an important risk factor and in majority of the patients these deformities are seen in advanced statge of the disease. The patients with such symptoms may initially respond to dopaminergic medications but if not intervened they may become fixed and difficult to treat. Pain and restriction of movement are most common clinical manifestations and these may mimick symptoms of musculoskeletal disorders like rheumatoid arthritis. Early diagnosis is important as the patients may respond to adjustment in dopaminergic medications. Recent advances such as deep brain stimulation (DBS) and ultrasound guided botulinum toxin injection are helpful in management of these deformities in patients with PD.
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PMID:Postural & striatal deformities in Parkinson`s disease: Are these rare? 2699 7

Camptocormia is a disabling pathological, non-fixed, forward bending of the trunk. The clinical definition using only the bending angle is insufficient; it should include the subjectively perceived inability to stand upright, occurrence of back pain, typical individual complaints, and need for walking aids and compensatory signs (e.g. back-swept wing sign). Due to the heterogeneous etiologies of camptocormia a broad diagnostic approach is necessary. Camptocormia is most frequently encountered in movement disorders (PD and dystonia) and muscles diseases (myositis and myopathy, mainly facio-scapulo-humeral muscular dystrophy (FSHD)). The main diagnostic aim is to discover the etiology by looking for signs of the underlying disease in the neurological examination, EMG, muscle MRI and possibly biopsy. PD and probably myositic camptocormia can be divided into an acute and a chronic stage according to the duration of camptocormia and the findings in the short time inversion recovery (STIR) and T1 sequences of paravertebral muscle MRI. There is no established treatment of camptocormia resulting from any etiology. Case series suggest that deep brain stimulation (DBS) of the subthalamic nucleus (STN-DBS) is effective in the acute but not the chronic stage of PD camptocormia. In chronic stages with degenerated muscles, treatment options are limited to orthoses, walking aids, physiotherapy and pain therapy. In acute myositic camptocormia an escalation strategy with different immunosuppressive drugs is recommended. In dystonic camptocormia, as in dystonia in general, case reports have shown botulinum toxin and DBS of the globus pallidus internus (GPi-DBS) to be effective. Camptocormia in connection with primary myopathies should be treated according to the underlying illness.
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PMID:Pathophysiological Concepts and Treatment of Camptocormia. 2731 57

The therapeutic applications of botulinum toxin (BoNT) have grown manifold since its initial approval in 1989 by the US Food and Drug Administration (FDA) for the treatment of strabismus, blepharospasm, and other facial spasms. Although it is the most potent biologic toxin known to man, long-term studies have established its safety in the treatment of a variety of neurologic and non-neurologic disorders. This review focuses on some novel and uncommon uses of BoNT in the treatment of movement disorders, such as oromandibular dystonia, including bruxism, anterocollis, camptocormia, tremor, tics, tardive and levodopa-induced dyskinesia, and restless legs syndrome. Despite a paucity of randomized controlled trials and lack of FDA approval for these movement disorders, there is growing body of evidence that BoNT provides benefit to patients with these hyperkinetic movement disorders and that BoNT is a safe treatment when used by clinicians skilled in the administration of the drug for these conditions.
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PMID:An update on new and unique uses of botulinum toxin in movement disorders. 2888 28

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

Camptocormia is a rare, involuntary movement disorder, presenting as truncal flexion while standing or walking, and is mainly observed as a feature of Parkinson's disease (PD) and primary dystonia. Deep brain stimulation (DBS) of the globus pallidus internus is effective for refractory camptocormia observed with PD or dystonia. However, the effectiveness of pallidotomy for camptocormia has not been investigated. The authors report the case of a 38-year-old man with anterior truncal bending that developed when he was 36 years old. Prior to the onset of the symptom, he had been taking antipsychotic drugs for schizophrenia. There were no features of PD; the symptom severely interfered with his walking and daily life. He was given anticholinergics, clonazepam, and botulinum toxin injections, which did not result in much success. Because of the patient's unwillingness to undergo implantation of a hardware device, he underwent staged bilateral pallidotomy with complete resolution for a diagnosis of tardive dystonic camptocormia. The Burke-Fahn-Marsden dystonia rating scale subscore for the trunk before and after bilateral pallidotomy was 3 and 0, respectively. No perioperative adverse events were observed. Effects have persisted for 18 months. Bilateral pallidotomy can be a treatment option for medically refractory dystonic camptocormia without the need for device implantation.
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PMID:Staged bilateral pallidotomy for dystonic camptocormia: case report. 3049 97

Botulinum toxin has emerged as an important therapeutic intervention within the realm of movement disorders, especially for focal and generalized dystonias. Botulinum toxin has additionally been used for a variety of symptoms associated with parkinsonism. In this review, we will specifically evaluate use of botulinum toxin in idiopathic Parkinson's disease. We will discuss symptoms including sialorrhea, limb, dystonia, tremor, dyskinesias, freezing of gait, camptocormia, pisa syndrome, urinary dysfunction, constipation, dysphagia, eyelid opening apraxia, and blepharospasm.
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PMID:Use of botulinum toxin in Parkinson's disease. 3057 18

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

Camptocormia is a rare and disabling movement disorder resulting in forward bending of the trunk. Camptocormia has many etiologies, although it is frequently observed patients with in Parkinson's disease and dystonia. Deep brain stimulation(DBS)of the globus pallidus internus(GPi)and subthalamic nucleus effectively treats camptocormia in Parkinson's disease and dystonia patients. Herein, we report a case of dystonic camptocormia induced by repetitive abdominal muscle exercise in which treatment was administered using bilateral GPi-DBS. A 54-year-old woman developed dystonic camptocormia at 53 years of age. Prior to the onset of symptoms, she regularly performed 200 abdominal muscle exercises per day. Oral medications, and botulinum toxin and lidocaine injections, were ineffective. Truncal anterior bending occurred while standing and walking. The patient underwent bilateral GPi-DBS, which instantly and dramatically improved her symptoms. The Burke-Fahn-Marsden dystonia rating scale subscore for the trunk before and after bilateral pallidotomy was 6 and 0, respectively. No perioperative adverse events were observed. Symptomatic relief persisted for 2 years. This case suggest that camptocormia can result from repeated abdominal muscle exercise, and that bilateral GPi-DBS may be a feasible and long-term efficacious procedure for dystonic camptocormia.
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PMID:[Bilateral Pallidal Deep Brain Stimulation for Dystonic Camptocormia Induced by Repetitive Abdominal Muscle Exercise:A Case Report]. 3110 77

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


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