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)

Dantrolene sodium has been given to 45 patients suffering from dyskinetic syndromes: 33 were suffering from spastic syndromes, either secondary to cerebral lesions at birth, or to other cerebral lesions, or to cord lesions; 9 were affected by infantile dystonic syndromes; 1 by dystonia muscolorum deformans and the last 2 patients were suffering from parkinsonism. The best dosage schedule was individual and ranged from 50 mg to 300 mg a day. In this range, the majority of the spastic patients showed reduction of spasticity, unrelated with the site of pathology: a slight one in 12 patients, a moderate one in 9 and a marked one in 2. On the contrary, slight improvement has been noticed in only two of the patients suffering from dystonic syndromes. In no case side effects has been noticed. In all patients who underwent slight or moderate improvement only, we tried to obtain better results on spasticity by growing the dosage schedules; but we have always noticed side effects, that is weakness or drowsiness and, sometimes, urinary uncontinence. Moreover 2 patients showed evidence of transitory metabolic side effects. Therefore our experience shows that dantrolene sodium is an useful drug into the therapy of spasticity, even if often a slight of moderate improvement only is achieved. Slow increase in dosage schedule, repeated laboratory controls and alternate periods of treatment and suspended treatment should be observed.
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PMID:[Clinical study of dantrolene sodium in the treatment of spastic and dystonic syndromes]. 102 44

The antiarrhythmic efficacy of the anticonvulsant sodium valproate was studied in 22 patients with ventricular premature contraction in the immediate (10-15 days) and late (2-5 months) periods of therapy. The prolong antiarrhythmic effect of the drug was observed in 66% of the patients in whom it was beneficial in the first days of therapy. The results obtained provide strong evidence for the efficacy of sodium valproate in patients with ventricular premature contraction occurring in the presence of neurocirculatory dystonia.
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PMID:[The anti-arrhythmic efficacy of sodium valproate]. 128 79

This report describes an infant diagnosed aged twenty-five months as having glutaric aciduria Type 1 (GA 1). Initial presentation was with isolated macrocephaly at four months of age. Severe hypertonia, and dystonia, within 24 hours of minor head injury occurred at nineteen months of age. Serial cranial imaging showed subdural fluid collections, and increasing underlying cerebral atrophy, mainly frontal and temporal. Confirmation of the clinical diagnosis required repeated blood and urine analysis by high performance liquid chromatography and gas chromatography/mass spectrometry; diagnosis was later confirmed enzymologically. Treatment with riboflavin, L-carnitine, vigabatrin and baclofen, produced some symptomatic relief; a low protein diet, nitrazepam and sodium valproate appeared of less obvious use. The rationale for these attempts at treatment is discussed. The possible role of quinolinic acid in the genesis of the fronto temporal and striatal atrophy is discussed and measurement of the quinolinate concentration in cerebrospinal fluid (CSF) of this case and age-related controls is presented.
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PMID:Glutaric aciduria type 1 an atypical presentation together with some observations upon treatment and the possible cause of cerebral damage. 149 52

Myoclonic dystonia is a rare disorder that occurs in an hereditary and a sporadic form. The autosomal-dominantly inherited form is responsive to alcohol but not to other drugs. The sporadic form has been relatively resistant to drug treatment. We report a young man with myoclonic dystonia who displayed only little response to alcohol but improved significantly with a combination of sodium valproate for myoclonus and trihexiphenidyl hydrochloride for dystonia. His rehabilitation, however, was confounded by public authorities who thought the patient's appearance was indicative of drug use.
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PMID:Myoclonic dystonia. 159 46

The antiarrhythmic effect of sodium valproate (acidoprol) on the degree of arrhythmic manifestations was examined in 18 patients with neurocirculatory dystonia (NCD) and 19 with coronary heart disease (CHD). A 24-hour ECG monitoring and recording were performed on emotional stress and exercise. The antiarrhythmic effect of the agent was evaluated by the blind method and placebo. The course of its therapy was found to reduce the frequency of ventricular premature contractions in 14 patients with NCD and to substantially limit or eliminate the arrhythmogenic effect of emotional stress and exercise. In CHD, the antiarrhythmic effect of acedoprol was less, it being shown only by 6 patients, but the effect failed to occur during exercise. However, the arrhythmogenic effect of emotional stress in CHD was significantly limited. Thus, the GAMA-ergic, stress-limiting system activator acedoprol has antiarrhythmic activity in cardiac patients, but its activity is drastically pronounced in NCD and less marked in CHD.
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PMID:[First experience in clinical trials of the new domestic drug acediprol used in the treatment of cardiac arrhythmias]. 180 68

Eighty-six patients with acute psychotic exacerbations were treated with fixed dosage regimens of oral fluphenazine up to 10-30 mg/day in randomized, double-blind studies. Dystonic reactions occurred in 33.8% of the subjects at risk. Of these, 58% occurred by the third day, 88% by the fourth day, and 100% by the ninth day of treatment; most occurred later in the interdose interval. Significant predictors of dystonic reactions were higher fluphenazine mg/kg dosage and younger age. There was a trend toward a lower risk of dystonia in patients who received amobarbital sodium for agitation. Results are discussed in relation to possible mechanisms of neuroleptic-induced dystonia.
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PMID:Acute dystonia during fixed-dose neuroleptic treatment. 228 8

The paper provides findings from an examination of 26 patients with neurocirculatory dystonia (NCD) with the hypertensive syndrome, 18 with NCD of the cardiac type (neurocirculatory asthenia), and 10 healthy subjects. Application of original psychological questionnaires made it possible to detect anxiety neurosis in a cardiac variant of NCD and neurosis with marked conversion in a hypertensive variant of NCD. In the latter, there was an increase in Na+/H+ and Li+/Na+ countertransport rates in the erythrocyte membrane, which was typical of essential hypertension. In the former variant, the Na+/H+ countertransport rate was lower in the erythrocyte membrane than that in the controls, whereas no differences were found in Li+/Na+ countertransport rates between the cardiac NCD patients and the controls.
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PMID:[Characteristics of neurotic state and transmembrane transport of sodium in erythrocytes in hypertensive and cardiac variants of neurocirculatory dystonia]. 239 12

A 39-year-old man showed a combination of severe parkinsonism and progressive dystonia following attempted suicide with sodium cyanide. Computed tomography (CT) scan showed bilateral lucencies in the putamen and external globus pallidus. The topography of lesions on CT scan closely correlated with the pathological findings described in a previous report of cyanide-induced parkinsonism. This is the first reported case of cyanide intoxication with delayed-onset dystonia.
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PMID:Clinical and CT scan findings in a case of cyanide intoxication. 273 10

Patients with focal dystonia take advantage of certain cutaneous or proprioceptive sensory inputs to alleviate their symptoms ("sensory trick"). We examined the effects of increasing muscle spindle activity by the tonic vibration reflex maneuver and decreasing it by intramuscular injection of lidocaine. The vibration was applied to the palm or the tendon of forearm muscles in 15 patients with writer's cramp and 15 age-matched normal subjects. In 11 patients, the vibration induced dystonic postures or movements typical of those seen during writing. Normal subjects showed either no response to the vibration or a gradually developing tonic vibration reflex only in the wrist and finger flexors, which produced visible movements with a significantly longer latency (12.5 +/- 6.7 seconds [mean +/- standard deviation]) than what was observed in the patients (2.7 +/- 2.5 seconds, p < 0.0001). Local injection of lidocaine (0.5%, 5-40 ml/muscle) attenuated the tendon reflex with relatively little effect on the M response. Injection into muscles with increased activity produced marked reduction of dystonic movements and significant clinical improvement in 13 patients, whereas injection into the other muscles had no effect. The clinical benefit lasted for 1 to 24 hours after injection. In 13 patients who had additional injections of 10% ethanol, which blocks sodium channels for a longer period than does lidocaine, the duration of action was prolonged to 5 to 21 days. These findings suggest that muscles causing dystonic movements have abnormal sensitivities to vibration at rest and that muscle afferents may play a pivotal role in producing dystonic movements.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Tonic vibration reflex and muscle afferent block in writer's cramp. 765 62

A solution containing S-nitroso-N-acetylpenicillamine (SNAP), a nitric oxide (NO.-releasing compound, was microinjected in doses of 0.25-2 mumol into a lateral ventricle of conscious rats. SNAP produced dose-dependent convulsions similar to those associated with limbic stimulation, such as tonic extension of the hindlimbs and tail, and dystonia of the forepaws. At 2 mumol, SNAP evoked hyperventilation (arterial hypocapnia), arterial hyperglycemia and caused necrotic lesions of periventricular gray (e.g. lateral septal nucleus) and white matter structures. In the caudate nucleus and lateral septal nucleus ipsilateral to injection, SNAP elicited a bipolar metabolic pattern of low glucose metabolism proximal to the ventricle with higher values occurring more distally. In control studies, we proved that the residue of SNAP decomposition, N-acetylpenicillamine disulfide injected intraventricularly (2 mumol), was without physiological, behavioral, or histological effects. Ventricular pretreatment with methylene blue (2 nmol), a putative inhibitor of guanylate cyclase and superoxide generator, suppressed several of the behavioral manifestations of 1 mumol SNAP, such as the forepaw dystonia, squinting, and facial clonus, but was ineffective on the physiological and histological variables affected by the 2 mumol SNAP dose. Another NO. donor, sodium nitroprusside (2 mumol), produced fewer behavioral and cytotoxic effects over a 55-min observation period, but caused more intense and widely distributed metabolic stimulation, especially in commissural and projection white matter tracts. The results are the basis for a conscious rat model using intraventricular injection of nitrocompounds to examine the physiological, behavioral, metabolic and cytotoxic properties of NO. in the brain.
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PMID:Neurotoxicity in conscious rats following intraventricular SNAP, a nitric oxide donor. 796 12


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