Botulinum toxin A in the treatment of dystonia
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Botulotoxin A je v léčbě dystonie používán od konce osmdesátých let minulého století a představuje nejúčinnější metodu léčby fokální a segmentální dystonie.S úspěchem je ale používán i v léčbě dalších neurologických onemocnění, manifestujících se mimovolnými svalovými kontrakcemi či hypertonem.Práce uvádí souhrn aktuálních poznatků o dystonii -fenomenologii, patofyziologii, klinickém obrazu, klasifikaci a možnostech terapeutického využití botulotoxinu A se zaměřením na léčbuCite
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Genentech is partnering with the German cancer company Affimed to develop immunotherapies for multiple kinds of solid and blood cancers. Affimed is developing therapies that engage natural killer cells of the innate immune system to help direct them to attack cancer cells. Genentech will pay Affimed $96 million up front and up to $5 billion more in potential payments.
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Abstract Botulinum toxin (BT) is used to treat a large number of muscle hyperactivity syndromes. Its use in dystonia, however, is still one of the most important indications for BT therapy. When BT is injected into dystonic muscles, it produces a peripheral paresis which is localised, well controllable and follows a distinct and predictable time course of around 3 months. Adverse effects are always transient and usually mild, long-term application is safe. With this profile BT can be used to treat cranial dystonia, cervical dystonia and limb dystonia including writer’s and musician's cramps. The recent introduction of BT high dose therapy also allows to treat more wide-spread dystonia including segmental and generalised dystonia. BT can easily be combined with other anti-dystonic treatments such as deep brain stimulation and intrathecal baclofen application. Best treatment results are obtained when BT therapy is integrated in the multimodal and long-term 'multilayer concept of treatment of dystonia'. The biggest challenge for the future will be to deliver state of the art BT therapy to all dystonia patients in need, regardless of whether they live in developed countries or beyond.
Cervical dystonia
Paresis
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Truncal dystonia leads to significant distress to the patient and poses difficult therapeutic challenge to clinicians. Truncal dystonia is not rare but surprisingly, there is not enough data available to guide an approach to diagnosis or treatment. Camptocormia (flexion dystonia of trunk) was found in 7% of patients with Parkinson’s disease and the prevalence increases with the severity of the disease.[1] As high as 26% of patients with multiple system atrophy reportedly present with Pisa syndrome (lateral truncal flexion).[2] 17% of progressive supranuclear palsy patients reported to have axial dystonia in extension.[3] Patients with tardive syndrome, Wilson’s disease, neurodegeneration with brain iron accumulation, and some other genetic form of dystonia may have prominent truncal involvement. A subset of patients presents with isolated idiopathic truncal dystonia. Does this phenomenon need a special mention?—the answer would be a clear “yes”; there is no doubt that truncal dystonia causes fair share of disability and distress when present. Unfortunately, the response to medications is generally not satisfactory. Since the availability of botulinum toxin for the treatment of dystonia, truncal dystonia has been treated with the injection with variable result. The groups of Cynthia L. Comella and Joseph Jankovic, respectively, published their experience on the safe and effective use of botulinum toxin injection for this condition.[45] After more than a quarter century of usage, truncal dystonia still remains an off-label indication for botulinum toxin injection. The onus is on the neurologists and industry to generate enough data to help regulatory approval of botulinum toxin injection in truncal dystonia. In this issue of Annals of Indian Academy of Neurology, Mehta et al. shared their experience with truncal dystonia and treatment with botulinum toxin injection.[6] The varied etiology of the patients in their series reflects the need for proper evaluation when one deals with truncal dystonia. Promising improvement in the patient-perceived subjective outcome following the treatment was observed without any adverse effect. The degree of improvement in their patients has clinical value as these patients were refractory to medicines. Moreover, the injection procedure required focusing on only two muscles: paraspinals and recuts abdominis. These are large muscles and injecting them is not challenging at all. It is difficult to standardize the dose of botulinum toxin in truncal dystonia (or any other dystonia). The dosage needs to be individualized depending upon the clinical severity and degree of muscle hypertrophy. Usage of various formulations of botulinum toxins in available studies also makes the comparison difficult. Abobotulinum toxin has been reported to have greater spread than Onabotulinum or Incobotulinum toxin.[7] Whether this leads to better clinical efficacy in treating large muscles remains unknown. To sum up, the findings of the study by Mehta et al.[6] are clinically relevant and will encourage the clinicians to use botulinum toxin injection in the treatment of truncal dystonia and document their experience.
Movement Disorders
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Blepharospasm
Cervical dystonia
Focal dystonia
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Oppenheim による「dystonia musculorum deformans
Focal dystonia
Movement Disorders
Cervical dystonia
Blepharospasm
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Focal dystonia
Cervical dystonia
Refractory (planetary science)
Etiology
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Бұл зерттеужұмысындaКaно моделітурaлы жәнеоғaн қaтыстытолықмәліметберілгенжәнеуниверситетстуденттерінебaғыттaлғaн қолдaнбaлы (кейстік)зерттеужүргізілген.АхметЯссaуи университетініңстуденттеріүшін Кaно моделіқолдaнылғaн, олaрдың жоғaры білімберусaпaсынa қоятынмaңыздытaлaптaры, яғнисaпaлық қaжеттіліктері,олaрдың мaңыздылығытурaлы жәнесaпaлық қaжеттіліктерінеқaтыстыөз университетінқaлaй бaғaлaйтындығытурaлы сұрaқтaр қойылғaн. Осы зерттеудіңмaқсaты АхметЯсaуи университетіндетуризмменеджментіжәнеқaржы бaкaлaвриaт бaғдaрлaмaлaрыныңсaпaсынa қaтыстыстуденттердіңқaжеттіліктерінaнықтaу, студенттердіңқaнaғaттaну, қaнaғaттaнбaу дәрежелерінбелгілеу,білімберусaпaсын aнықтaу мен жетілдіружолдaрын тaлдaу болыптaбылaды. Осы мaқсaтқaжетуүшін, ең aлдыменКaно сaуaлнaмaсы түзіліп,116 студенткеқолдaнылдыжәнебілімберугежәнеоның сaпaсынa қaтыстыстуденттердіңтaлaптaры мен қaжеттіліктерітоптықжұмыстaрaрқылыaнықтaлды. Екіншіден,бұл aнықтaлғaн тaлaптaр мен қaжеттіліктерКaно бaғaлaу кестесіменжіктелді.Осылaйшa, сaпa тaлaптaры төрт сaнaтқa бөлінді:болуытиіс, бір өлшемді,тaртымдыжәнебейтaрaп.Соңындa,қaнaғaттaну мен қaнaғaттaнбaудың мәндеріесептелдіжәнестуденттердіңқaнaғaттaну мен қaнaғaттaнбaу деңгейлерінжоғaрылaту мен төмендетудеосытaлaптaр мен қaжеттіліктердіңрөліaйқын aнықтaлды.Түйінсөздер:сaпa, сaпaлық қaжеттіліктер,білімберусaпaсы, Кaно моделі.
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