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

Meige's syndrome is a form of cranial dystonia characterized by the presence of bilateral dystonic spasms of the facial muscles and frequently of other cranial muscles as well. Its most common and disabling manifestation is blepharospasm which can render the patient functionally blind. Several types of orbicularis oculi spasms occur in Meige's syndrome: brief clonic spasms, prolonged dystonic spasms, constant tonic contraction, and "apraxia" of lid opening. In the completed form of the syndrome, blepharospasm is typically associated with lower facial or oromandibular dystonia. Spasms of the neck and limb muscles, generally mild, and action tremor not uncommonly accompany the cranial dystonia. In most patients the cause of the spasms is unknown. This so-called idiopathic or primary form of Meige's syndrome is considered an adult form of adult onset dystonia. Secondary Meige's syndrome can be encountered in the context of several neurodegenerative disorders, chronic administration of neuroleptics, levodopa, or other drugs, and in patients with focal brain lesions. These secondary cases of Meige's syndrome suggest that a dysfunction of the basal ganglia or of the mesencephalic/diencephalic region plays an important role in the pathophysiology of this dystonic syndrome. Recent neurophysiologic studies and postmortem findings in some patients also support the notion that disease of the brain stem contributes to the pathophysiology of orofacial dystonia.
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PMID:Blepharospasm-oromandibular dystonia syndrome (Meige's syndrome): clinical aspects. 327 55

Medical treatment of dystonia usually results in an incomplete response and is frequently unsuccessful. Peripheral surgical therapy is available for some focal dystonias, but may only offer temporary relief and may have unacceptable complications. We have used local injections of botulinum toxin into the appropriate muscles for treatment of disabling focal or segmental dystonia in 93 patients with torticollis, blepharospasm, oromandibular dystonia (OMD), limb dystonia, lingual dystonia, and dystonia adductor dysphonia, in addition to four patients with hemifacial spasm. Significant relief of motor symptoms was seen in 69% of the patients with blepharospasm and 64% of patients with torticollis; 74% of the latter group with pain experience relief. Relief of symptoms was noted in most patients with OMD and limb dystonia, and all with lingual dystonia, dystonic adductor spastic dysphonia, and those with hemifacial spasm. Benefit averaged 2 1/2-3 months initially; however some patients experienced longer relief with subsequent injections. Adverse effects were transient, although 2 patients developed antibodies against the toxin, and we documented evidence for distant effects in others. This approach of chemically weakening contracting muscles in focal dystonia offers many advantages over pharmacotherapy and surgical therapy. Additional experience is needed to explore the proper doses, and potential for long term adverse effects.
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PMID:Localized injections of botulinum toxin for the treatment of focal dystonia and hemifacial spasm. 350 53

The clinical features, differential diagnosis and treatment of unilateral spasms of the jaw and masticatory muscles are discussed and illustrated by eight cases of unilateral jaw spasms of various aetiologies. These include focal dystonia of the jaw, hemimasticatory spasm with and without facial hemiatrophy, paroxysmal events in multiple sclerosis and tetany. Attention is particularly drawn to four cases of unilateral dystonia of the jaw which has not been described before.
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PMID:Focal dystonia of the jaw and the differential diagnosis of unilateral jaw and masticatory spasm. 373 21

Beside the importance of its prehensile function the human hand plays an essential role in the conveyance of expression. Manual gestures support the mimic and confirm the spoken word. Based on the psychofunctional connection between mimic and gesture the hand becomes an outlet for psychogenic disorders. The resultant projection of emotional processes into motor disorders has been a matter of conjecture etiologically. Psychogenic manifestation in isolated motor disorders of the hand is considered to be either a functional nervous disorder or a focal dystonia. In comparison to the conversion phenomena reported up until now, in which flexor muscle units of the hand are involved with increased muscular activity (spasm), the cases presented here are characterized by a functional motor disorder manifested as an isolated paralysis of a selective extensor muscle unit (M. extensor pollicis longus) of the hand. After a multidisciplinary approach including clinical morphology, neurology, EMG and psychiatry the final diagnosis of a psychogenic paralysis of the hand is made using direct electro-stimulation of the neuromuscular unit with a needle electrode under simultaneous elimination of the psychic influence (general anesthesia without muscular relaxation). Through such confirmed motor inactivity the psychic genesis of the clinically existing paralysis is proven. Through immediate introduction of a multimodal therapy including physical and psychiatric treatment psychogenic paralyses of the hand can be restored totally.
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PMID:[Diagnosis and therapy of psychogenic paralyses in the hand region]. 406 16

The descriptive aspects of all types of movement disorders and their related syndromes and terminologies used in the literature are reviewed and described. This comprises the features of (a) movement disorders secondary to neurological diseases affecting the extrapyramidal motor system, such as: athetosis, chorea, dystonia, hemiballismus, myoclonus, tremor, tics and spasm, (b) drug induced movement disorders, such as: akathisia, akinesia, hyperkinesia, dyskinesias, extrapyramidal syndrome, and tardive dyskinesia, and (c) abnormal movements in psychiatric disorders, such as: mannerism, stereotyped behaviour and psychomotor retardation. It is intended to bring about a more comprehensive overview of these movement disorders from a phenomenological perspective, so that clinicians can familiarize with these features for diagnosis. Some general statements are made in regard to some of the characteristics of movement disorders.
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PMID:Clinical features of movement disorders. 662 43

On the basis of histological and histochemical examinations of 100 postmortem observations of bacterial shock, 4 stages of disorders in the microcirculatory bed and in cell elements of organs in this complication are distinguished. The hemodynamic stage consists in redistribution of the blood flow, alternation of spasm and paresis, and vascular dystonia. In the stage of hemorheological disorders congestion is substituted by stasis, sludging of erythrocytes due to pachyemia. As a result of increased permeability of vessel walls, interstitial edema and early changes of parenchymatous cells of organs occur at the enzymatic level. The addition of DIBC syndrome leads to deeper ischemia of organs and formation of necroses, hemorrhages, acute ulcers. As a consequence, organ insufficiency (adrenal, renal, respiratory) develops.
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PMID:[Organ pathology of bacterial shock]. 667 Sep 42

A tonic ambulatory foot response developed one year after clamping of an aneurysm of the contralateral carotid artery in a young adult. No motor deficiency was noted during the postoperative period, but spasm of the carotid bifurcation branches were present on arteriograms. Clinical examination and deep reflexes tests excluded pyramidal lesions. Cortical somatesthetic evoked potential recordings confirmed the absence of sensory disturbances. CT scan suggested the presence of an infarct in the territory of the anterior choroidal artery. The present findings are compared with previous reports. The etiological data and CT scan results in the present case allow reconsideration of the physiopathology of tonic ambulatory foot response in the light of current knowledge of locomotor function in animals. The tonic flexion, an "extension" dystonia of the toes, electively provoked by walking, occurring one year after the neurological accident without any pyramidal signs, could arise from a coordination synkinesis due to lesions limited to the efferent extrapyramidal system.
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PMID:[Isolated tonic ambulatory flexion of the foot (author's transl)]. 733 75

Over recent years botulinum toxin type A has emerged as a safe and effective treatment for a number of previously refractory conditions associated with excessive muscle activity. The list of indications is expanding, but at present it is generally considered to be the treatment of choice for focal dystonias such as blepharospasm, torticollis, laryngeal dystonia, and oromandibular dystonia, as well as hemifacial spasm, strabismus, and some forms of limb spasticity. Carefully targeted intramuscular injections of a small amount of the toxin block the release of acetylcholine at the neuromuscular junction, producing a chemical denervation, with the aim of reducing excessive muscle activity without producing significant functional weakness. In some situations electrophysiological assessment and localisation of the muscles for injection is necessary. Treatment is symptomatic, with effects lasting 3 to 4 months and most patients requiring up to 4 injections per year to maintain the beneficial effect. Appropriate use of the toxin requires both an understanding of the physiological action of the potential muscles involved in each situation, together with a knowledge of the likely dose necessary to reduce muscle activity to the required level. Botulinum toxin represents a major advance in the management of these conditions, many of which responded poorly to previously available forms of therapy.
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PMID:Botulinum toxin in clinical practice. 753 96

A prospective open study of botulinum toxin A treatment for patients with various movement disorders at Siriraj Hospital, Mahidol University was analysed to evaluate its efficacy. The grand total of 900 patients comprised of a) 592 patients (65.78 per cent) with hemifacial spasm; b) 92 patients (10.22 per cent) with occupational cramp; c) 79 patients (8.78 per cent) with blepharospasm and Meige syndrome; d) 72 patients (8.00 per cent) with spasmodic torticollis; e) 19 patients (2.11 per cent) with hemidystonia and generalised dystonia; f) 11 patients (1.22 per cent) with spasmodic dysphonia; g) 10 patients (1.11 per cent) with spastic hemiparesis; and h) 25 patients (2.78 per cent) with miscellaneous group (i.e. tics, Gilles de la Tourette, facial myokimia, benign fasciculation, etc.). The results of treatment for hemifacial spasm were classified as excellent in 486 patients (82.09 per cent), moderate improvement in 60 patients (10.14 per cent), mild improvement in 39 patients (6.59 per cent) and no improvement or worse in 7 patients (1.18 per cent). There were complications of mild transient facial weakness in 50 patients (8.45 per cent) and mild ptosis in 12 patients (2.02 per cent). The effect of botulinum toxin treatment lasted 3-6 months. In occupational cramp and spasmodic torticollis the good response rate was around two-thirds of all patients, whereas, blephalospasm, spasmodic dysphonia, spastic hemiparesis and tics responsed in 79-88 per cent of the patients. Botulinum toxin A injection is thus a simple, safe, and effective out-patient treatment for patients with various kinds of movement disorders but it is a costly therapy.
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PMID:Treatment of various movement disorders with botulinum A toxin injection: an experience of 900 patients. 756 52

Hemifacial spasm is a condition characterized by involuntary, episodic, synchronous contractions of muscles innervated by the facial nerve in one half of the face. The treatment can be medical or neurosurgical, but the most effective is injections of botulinum toxin. 13 patients who received this treatment for hemifacial spasm are described. The treatment of different forms of focal dystonias has been described before in this journal. The aetiology of hemifacial spasm is different from that of focal dystonia. It is probably due to compression of the facial nerve, causing ectopic generation of impulses. Treatment with botulinum toxin is shown to be effective also for this condition, but the dosage and the duration of effect differ from those reported for focal dystonias. This may be due to differences in the pathophysiological mechanisms of the two conditions.
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PMID:[Hemifacial spasms. Causes, clinical findings and treatment]. 757 Apr 93


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