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)

The results obtained in a retrospective study on clinical and pharmacological aspects of 41 patients suffering craniocervical dystonia (24 with blepharospasm, 17 with torticollis) and 11 with spasm are here presented. Mean age of symptoms onset was 57.4, 43.8 and 55.8 years old respectively; this variable was comparatively higher in females than in males with torticollis. The prevalence of blepharospasm and hemifacial spasm was higher in females. A 38.7% of patients suffering blepharospasm also presented oromandibular dystonia (Meige's syndrome). Other abnormal movements less frequently associated were cephalic tremor, postural hand tremor and larynx dystonia. In three cases with blepharospasm there was family history of Parkinson's disease and in two cases with torticollis there was family history of essential tremor. The mean age of onset was lower in patients with clonic torticollis and the evolution time of symptoms was longer than in those who presented the tonic type. Clonic torticollis were less frequently associated to pain. Trihexyphenidyl (anticholinergic) was the most efficient drug in craniocervical dystonia, and clonazepam in facial hemispasm. In general, as earliest the age of onset was, as better the therapeutical response was.
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PMID:[Craniocervical dystonia and facial hemispasm: clinical and pharmacological characteristics of 52 patients]. 176 88

A case of a mentally retarded patient with sporadic paroxysmal dystonia, unresponsive to anticonvulsant therapy, is described. He had a long-standing history of neuroleptic drug intake. Trihexyphenidyl in a total daily dosage of 20 mg totally suppressed the crises.
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PMID:Paroxysmal dystonia responsive to anticholinergic drugs. 350 80

A total of 15 patients affected by idiopathic dystonia (7 with generalized and 8 with focal or segmental dystonia) were subjected to therapy with bromocriptine at low doses, pimozide and trihexyphenidyl. The symptoms were evaluated by giving a progressive score in relation to the intensity of the dystonic symptom to each of the body segments involved by the dystonia. Bromocriptine did not significantly modify the dystonia. Pimozide showed a slight nonsignificant improvement of the dystonic symptoms. Trihexyphenidyl was effective in the generalized dystonias, in agreement with previous reports in the literature. The variation in the pharmacological results could be due to the diversity of the dystonic syndromes, which comprise cases that are different in age at onset, site of dystonic symptoms, and evolution.
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PMID:Idiopathic dystonia: neuropharmacological study. 618 10

Trihexyphenidyl has been found to be an effective treatment for dystonic movement disorders, improving gross motor function in patients with axial and torsional dystonia, tremors, and myoclonus. In this report, improvements in fine motor control, language, and oral motor skills are described with trihexyphenidyl in an 8-year-old female who developed dystonia after spontaneous bilateral putamenal hemorrhages. No adverse side effects occurred. The mechanism of action of trihexyphenidyl is believed to be in the basal ganglia where it inhibits muscarinic cholinergic receptors and increases the turnover of dopamine.
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PMID:Trihexyphenidyl in posthemorrhagic dystonia: motor and language effects. 1020 32

Trihexyphenidyl (Artane) is a centrally active muscarinic antagonist commonly used to treat patients with generalized dystonia. In a retrospective survey of 22 consecutive children with extrapyramidal cerebral palsy, we evaluated trihexyphenidyl on upper extremity and lower extremity function, expressive language, and drooling. Functional changes were assessed using a parental questionnaire (rating scale 1-5: from 1 = little or no change to 5 = tremendous change, with scores in either a positive or negative direction). Improvements of +4 or +5 were reported in eight children for upper extremity function, in eight children for verbal expressive language, in five for drooling, and in none for lower extremity function. Using bivariate linear regression modeling to investigate variables associated with treatment effects, there was a significant inverse relationship between age at initiation of medication and therapeutic response. Furthermore, beneficial responses were specific to upper-extremity function and expressive language. These results suggest that younger children are more likely to respond to trihexyphenidyl and that primary functional benefits include improved fine motor abilities and expressive language. A prospective masked study with a standardized clinical instrument is needed to confirm these findings.
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PMID:Age-dependent effects of trihexyphenidyl in extrapyramidal cerebral palsy. 1148 97

Cerebral palsy is the main cause of immobility in children. This motor dysfunction is caused by several motor components such as weakness, lack of motor control and muscle hypertonia. Drug treatment, delineated in this review, mainly addresses the latter. Recently, new definitions for clinical features of hypertonia in children were published, assisting the distinction between the two common motor symptoms in cerebral palsy, spasticity and dystonia. The main functional symptoms disrupt functional daily life, dictating the overall approach and the specific drug treatment. There are an increasing number of treatments for this distressing disorder. For general spasticity, treatments provided include Baclofen. If symptoms are local, either dystonia, or spasticity, Botulinum toxin is the revolutionary drug used with significant success and relatively few side effects. For generalized dystonia, a trial of both Dopamine and Trihexyphenidyl should be considered. Cerebral palsy, like other complex disorders, requires individualized decision-making and a team approach. Drug therapy is only one aspect of treatment, yet sometimes it may serve as a window of opportunity to facilitate better motor control.
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PMID:[Drug treatment for children with cerebral palsy]. 1690 Jul 42

Although trihexyphenidyl is used clinically to treat both primary and secondary dystonia in children, limited evidence exists to support its effectiveness, particularly in dystonia secondary to disorders such as cerebral palsy. A prospective, open-label, multicenter pilot trial of high-dose trihexyphenidyl was conducted in 23 children aged 4 to 15 years with cerebral palsy judged to have secondary dystonia impairing function in the dominant upper extremity. All children were given trihexyphenidyl at increasing doses over a 9-week period up to a maximum of 0.75 mg/kg/d. Trihexyphenidyl was subsequently tapered off over the next 5 weeks. Objective motor assessments were performed at baseline, 9 weeks, and 15 weeks. The primary outcome measure was the Melbourne Assessment of Unilateral Upper Limb Function, tested in the dominant arm. Tolerability and safety were monitored closely throughout the trial. Of the 31 children who agreed to participate in the study, 5 failed to meet entry criteria and 3 withdrew due to nonserious adverse events (chorea, drug rash, and hyperactivity). Three children required a dosage reduction because of nonserious adverse events but continued to participate. The 23 children who completed the study showed a significant improvement in arm function at 15 weeks (P = .045) but not at 9 weeks (P = .985). Post hoc analysis showed that a subgroup (n = 10) with hyperkinetic dystonia (excess involuntary movements) worsened at 9 weeks (P = .04) but subsequently returned to baseline following taper of the medicine. The authors conclude that scientific evidence for the clinical use of trihexyphenidyl in cerebral palsy remains equivocal. Trihexyphenidyl may be a safe and effective for treatment for arm dystonia in some children with cerebral palsy if given sufficient time to respond to the medication. Post hoc analyses based on the type of movement disorder suggested that children with hyperkinetic forms of dystonia may worsen. A larger, randomized prospective trial stratified by the presence or absence of hyperkinetic movements is needed to confirm these results.
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PMID:Prospective open-label clinical trial of trihexyphenidyl in children with secondary dystonia due to cerebral palsy. 1865 86

The authors report a patient with dystonia secondary to bilateral lesions of the basal ganglia, who improved dramatically with levodopa. The patient presented at the age of 4 years with progressive dystonia of the lower extremities and right upper extremity. Magnetic resonance imaging (MRI) of the brain showed bilateral hyperintensities of the globus pallidus that remained stable over the years. Despite extensive investigations, the etiology of her basal ganglia lesions remained nebulous. The patient's dystonia responded to Trihexyphenidyl and to tetrabenazine, but these medications needed to be stopped because of side effects. At the age of 12 years, small doses of levodopa-carbidopa were tried and resulted in dramatic improvement of her dystonia. The authors believe that in the pediatric population with secondary dystonias other than Segawa disease, even though this has been reported only rarely to be effective, a therapeutic trial with levodopa should be considered in some instances.
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PMID:A case of secondary dystonia responding to levodopa. 1980 91

In Rett syndrome (RS), acute life-threatening episodes (ALTEs) are usually attributed to epilepsy or autonomic dysfunction but they can represent a movement disorder (MD). We describe three girls with RS who experienced ALTEs from an early age. These were long considered epileptic until video-EEG in Patients 1 and 3 revealed their non-epileptic nature. A primary dystonic mechanism was suspected and Patients 1 and 2 were treated with Trihexyphenidyl with significantly reduced frequency of the ALTEs. Patient 3 died before Trihexyphenidyl was tried. Trihexyphenidyl in RS patients with similar presentations can modify the dystonia and prevent ALTEs.
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PMID:Trihexyphenidyl for acute life-threatening episodes due to a dystonic movement disorder in Rett syndrome. 2006 34

Primary lingual dystonia is a rare condition, especially when it is only induced by speaking. Trihexyphenidyl failed to improve the symptoms. Several case series have demonstrated the effectiveness of botulinum toxin injection for the management of focal lingual movement disorders. Only 1 case of botulinum toxin injection for primary lingual dystonia induced by speaking has been reported, but this treatment has limited effectiveness. Our patient was treated with botulinum toxin using a superficial approach for injection into the tongue with continuing excellent results. Lingual botulinum toxin injection is a fairly simple, safe and viable treatment option for lingual dystonia induced by speaking.
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PMID:Botulinum toxin in the treatment of lingual dystonia induced by speaking. 2480 61


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