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)

We present a case of 47-year old patient with a rare form of focal dystonia restricted to laryngeal adductors with blepharospasm. Apart from typical symptoms of blepharospasm, the patient had severe problems with articulation in the form of harsh voice, frequently interrupted speech and the sound coming out with a great effort. We applied a transcutaneous botulin toxin therapy to this patient. The toxin was given into thyroarytenoid muscle in transcutaneous injections under control of EMG. Successful clinical effect was achieved with the dose of 80 U of the botulin toxin (Dysport) and lasted 6 months. The treatment was repeated, and the patient has not presented the symptoms of the disease since then for 10 months. We confirm that the botuline toxin transcutaneous injections represent a save and effective treatment for laryngeal adductor dystonia.
Neurol Neurochir Pol
PMID:[A case of laryngeal adductor dystonia treated with transcutaneous injections of botulinum toxin]. 1046 40

64 cases with spasmodic torticollis were observed during 5 years and treated with botulinum toxin (BTX). BTX was injected into dystonic muscles mostly into sternocleidomastoid then--trapezius, and splenius capitis muscle. Improvement (excellent, good and fair) was achieved in 40 patients (62%). Lack of information about 6 patient (9%). Injections were repeated every 3-4 months and in several cases even 1-2 during the year. After several injections atrophy and denervation potentials in EMG were observed in the majority of injected muscles. Neurotic syndromes coexisting with dystonia had worsening influence on therapeutic effects. Adverse events were observed in 5 cases. Treatment with BTX is very simple, easy, harmless and can be administered in outpatients.
Neurol Neurochir Pol
PMID:[Spasmodic torticollis: experience of 5 years with botulinum toxin treatment]. 1046 49

Based on the available literature, we outline the classification criteria of paroxysmal involuntary movements with instant onset and termination. Four types of those movements are currently distinguished: dystonic Mount-Reback paroxysmal choreoathetosis, kinesigenic paroxysmal choreoathetosis, exercise-induced paroxysmal dystonia, and hypnogenic paroxysmal dystonia. We present the clinical characteristics of those entities, as well as diagnostic and therapeutic aspects. An association with epilepsy is emphasised, which seems to predominantly apply to hypnogenic dystonia and kinesigenic paroxysmal choreoathetosis.
Neurol Neurochir Pol
PMID:[Paroxysmal dyskinesis and epilepsy]. 1079 Oct 41

Two cases with blepharospasm followed by Meige syndrome were described. In the first magnetic resonants revealed bilateral hypodensive foci in thalamus. During the few years general dystonia has been developed after several relapses of disease diagnosed as multiple infarcts followed by aphasia, hemiparesis and asynergia. In the other one, who died, loss of neurons in striatum, especially in caudate nuclei was found. It is concluded that blepharospasm could be a syndrome of different origin and only the phase of Meige syndrome.
Pol Merkur Lekarski 2001 Feb
PMID:[Blepharospasm and Meige's syndrome--a contribution to it's pathogenesis]. 1132 May 88

Camptocormia is characterized by pronounced forward flexion of the thoracolumbar spine, which increases while walking and disappears in recumbent position. The clinical spectrum of the described disorders with concomitant camptocormia is heterogenous. It was described for the first time in idiopathic Parkinson's disease in 1999. The pathophysiology of this phenomenon remains unclear but seems to be not related to antiparkinsonian treatment. The authors present the case of a 54 years old woman, with idiopathic Parkinson disease diagnosed 5 years ago. The rapid progression of the disease was associated with good response to Levodopa therapy, although the dose had to be increased up to 1400 mg/d (with peripheral decarboxylase and COMT inhibitor). After 5 years she developed painful spasms of paraspinal muscles which resulted in trunk flexion. The clinical picture resembled the described cases of camptocormia. There was no correlation between the appearance of camptocormia and the regime of levodopa administration (time or dosage). Therefore, one can conclude, that presumably camptocormia is not a form of dystonia of the trunk but, the result of till now unclear other factors (dysfunction in other non-dopaminergic nigrostriatal projections?).
Neurol Neurochir Pol
PMID:[Camptocormia, a rare form of motor system disorders in Parkinson's disease]. 1198 8

Laryngeal dystonia is a focal dystonia occurring more often than it is diagnosed. Adductor type dystonia was described in 5 patients. In 3 cases it manifested itself as blepharospasm, which later developed into Meige's syndrome. The patients were treated with botulinum toxin A injections under EMG control administering 10 i.m. into laryngeal muscles on both sides with good and very good results. There were no serious side effects.
Neurol Neurochir Pol
PMID:[Laryngeal dystonia--personal experience with botulin toxin treatment]. 1205 8

Sensory disturbances (hypoaesthesia in fingertips and tenderness) and movement disorders (tremor, dystonia, muscle spasms, abnormal postures and inability in initiating of movement) were investigated in 80 patients with post-traumatic reflex sympathetic dystrophy (RSD) of the upper extremity. Sensory disturbances were found in 67 patients (84%): most frequently hypoaesthesia in fingertips--in 49, and tenderness of the dorsal aspect of the hand in 24; in six patients both these signs were present simultaneously. Movement disorders were found less frequently: tremor was observed in 12 patients, spasms of the flexor muscles of the forearm and hand in two, postural dystonia in one, and inability initiating of movement in the other one. Sympathetic interruptions reduced movement disturbances in two patients. Dystonia was resistant to various treatment approaches including botulinum toxin injections. Certain hypothesis explaining pathogenic background of motor and sensory disorders in RSD are discussed. The results of this study show that sensory disturbances are frequently observed in RSD, however, they usually disappear after successful treatment of the condition; movement disorders are less frequent, but much more difficult to control. Neurologists are not frequently faced with the problem of neurological disorders after trauma of the limb. Inclusion of RSD in differential diagnosis may improve recognition of the disorder and result in its faster and proper management.
Neurol Neurochir Pol
PMID:[Neurological disorders in post-traumatic algodystrophy of the upper extremity]. 1205 14

This is a rare syndrome, most likely of several genetically determined neurodegenerative disorders with similar pathogenesis. Two forms of the disease are distinguished: familial occurring in about 50% of cases and sporadic with about 15% of cases in which parental consanguinity is found. Clinically, NBIA-1 is characterised by a slow progression of extrapyramidal symptoms and progressive dementia, mostly in children. Relentlessly progressive course is obvious, but the progress may be very slow, taking sometimes several dozen of years. Four subtypes of the disease have been thus far distinguished: early childhood, late childhood, adult onset and with protracted course. The clinical diagnosis of NBIA-1 is only probable because specific abnormalities have not as yet been detected in laboratory investigations. However, NBIA-1 should be suspected, if extrapyramidal symptoms are observed, such as dystonia, choreoathetosis, muscular rigidity, moreover dementia, retinal degeneration and/or optic nerve atrophy and characteristic magnetic resonance imaging (so called "the eye-of-the tiger" sign). At present, only comprehensive symptomatic treatment is possible.
Neurol Neurochir Pol
PMID:[Neurodegeneration with brain iron accumulation, type-I (NBIA-I) (formerly Hallervorden-Spatz, disease). Par I: clinical manifestation and treatment]. 1252 19

Among all the extrapiramidal movement disorders Parkinson's disease (PD) is the one most often submitted to neurosurgical treatment. Technical advances in neurosurgery, neuroimaging and neurophysiology, as well as shortcomings of chronic Levodopa medication (i.e. on/off fluctuations, violent dyskinesia and painful dystonia) have greatly contributed to the renewed interest in the surgical treatment of PD. The attainment of a better understanding of the basal ganglia function and of PD pathophysiology has also encouraged centers to treat Parkinson's disease in recent years. This article presents the current model of PD and the rationale for using GPi, thalamus and STN as target sites in stereotactic surgery.
Neurol Neurochir Pol
PMID:[The currently accepted pathophysiological model underlying surgical management of Parkinson disease]. 1291 Aug 41

Spasmodic dysphonia is a focal laryngeal dystonia. It presents as: adductor spasmodic dysphonia with the strain-strangle voice; abductor spasmodic dysphonia with whispering voice and breathy breaks in connected speech; and adductor respiratory dystonia with paradoxical vocal fold movements and stridor. It is most commonly treated by the intramuscular injection of botulinum toxin. In this article, the authors discuss difficulties in making diagnosis and treatment in patients with laryngeal dystonia concerning their own cases.
Otolaryngol Pol 2003
PMID:[Diagnostic and therapeutic difficulties in laryngeal dystonia]. 1499 22


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