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)

Extrapyramidal dysfunction is poorly characterized in Rett's syndrome, a neurodegenerative disorder in girls. We studied the motor and behavioral findings in 32 Rett's syndrome patients, 21 months to 30 years old. In addition to the typical stereotyped movements and scoliosis, other motor disturbances included bruxism, sialorrhea, ocular deviations, parkinsonian findings, dystonia, myoclonus, and athetosis. The types of movement disorders seemed to be age-related, with the hyperkinetic disorders occurring in the younger patients and the bradykinetic disorders occurring more frequently in the older patients.
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PMID:Extrapyramidal involvement in Rett's syndrome. 223 45

Rett syndrome, a progressive neurodegenerative disorder described only in female subjects, is manifested by a wide spectrum of behavioral and motor abnormalities. We studied 32 patients with this disorder, ages 30 months to 28 years old, and characterized their extrapyramidal disturbance. The most common motor abnormalities were stereotyped movements and gait disturbance, seen in all patients. Bruxism, oculogyric crises, parkinsonism, and dystonia were also common, but myoclonus and choreoathetosis were seen only infrequently. The hyperkinetic movement disorders tended to dominate in younger patients, while bradykinetic disorders were more evident in the older patients. This study provides evidence that movement disorders seen in Rett syndrome reflect age-related neurodegenerative changes in the basal ganglia.
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PMID:Rett syndrome and associated movement disorders. 238 36

Motor disorders affecting the orofacial musculature include bruxism, chronic orofacial muscle pain affecting the jaw and neck muscles and the involuntary waking period disorders such as orofacial dyskinesia, oral mandibular dystonia, tremor and others. Research at UCLA has touched these and many other areas. Current results have indicated the usefulness of contingent afferent electrical stimulation of the lip to control bruxism; provided information regarding the fatigue, endurance and recovery faculties of the protrusive jaw muscles; explored the issue of chronic muscle hyperactivity inducing headache pain; and worked with botulin toxin as a method to treat orofacial dystonia and dyskinesia.
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PMID:Oral motor disorders in humans. 768 5

Eight cases of diurnal bruxism (DB) secondary to long-term antidopaminergic drug exposure are reported. Five exhibited a grinding pattern, one a clenching form, and two a mixed type. An odontological etiology was absent throughout. EMG recordings disclosed two distinct patterns of muscle activity, one with brief rhythmic, forceful contractions and the other featuring sustained prolonged contractions. Surface EMG and EEG monitoring during a 24-h period confirmed the absence of bruxism during sleep. Several drug trials failed to provide relief. Our findings support DB as a focal tardive dystonia syndrome.
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PMID:Bruxism secondary to chronic antidopaminergic drug exposure. 810 96

To characterize the relationship between bruxism and dystonia, 79 patients (28 men and 51 women) with cranial-cervical dystonia were studied. Sixty-two patients (78.5%), 22 men and 40 women, had bruxism. The mean age at onset of dystonia in patients with bruxism was 52.4 +/- 12.6 years (range 14-80), similar to patients with cranial-cervical dystonia without bruxism. Involuntary oromandibular movements (46 patients) and blepharospasm (34 patients) were the most common initial symptoms among patients with dystonia. About one-fourth of bruxism patients had associated dental problems including TMD (21%) and tooth wear (5%). A majority (58%) of the bruxism patients had diurnal bruxism and 12% had nocturnal bruxism. The bruxism patients were compared to 100 patients with Parkinson's disease (PD), cervical dystonia, cranial dystonia, and normal controls, respectively. The prevalence of bruxism was much higher in the cranial-cervical dystonia patients when compared to normal controls (P < 0.001); however, this difference was not significant between other diseased groups and controls. Medications and botulinum toxin injections, used in the treatment of focal dystonia also provided effective relief of bruxism.
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PMID:Bruxism and cranial-cervical dystonia: is there a relationship? 1065 Apr 7

While chewing and grinding movements have been observed in amphetamine addicts, recognition and management of this problem have rarely been highlighted. Botulinum toxin (BTX) has previously been demonstrated to be effective for bruxism associated with movement disorders, such as cranial-cervical dystonia. However, there is little information on its use in tardive bruxism. Here we report an amphetamine addict who presented with medically intractable bruxism, and discuss its pathophysiology and successful treatment with BTX.
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PMID:Severe amphethamine-induced bruxism: treatment with botulinum toxin. 1258 Aug 70

Tetrabenazine (TBZ) is a catecholamine depletor used for the treatment of a variety of movement disorders. The purpose of this study was to assess the efficacy of TBZ in a retrospective chart review in 3 tertiary care movement disorders centers over long-term treatment. Of 150 patients to whom TBZ was prescribed, 118 were followed up and assessed using the Clinical Global Impression of Change (CGIC), (-3 to +3), a composite grade from a patient and caregiver scale over variable periods. The patients had a variety of hyperkinetic movement disorders including dystonia (generalized and focal: axial, Meige syndrome, torticollis, blepharospasm, bruxism), Huntington disease (HD) or other choreas, tardive dyskinesia (TD) or akathisia, and Tourette syndrome. Mean patient age was 48.8 +/- 18.7 years; 48 were men (40.7%) with a mean disease duration of 93 months. The mean follow-up time was 22 months and the mean TBZ dose was 76.2 +/- 22.5 mg/d (median 75 mg, range 25-175 mg/d). The mean CGIC score was +1 (mild improvement). The group of patients who scored +3 on the CGIC (very good improvement) represented 18.6% (n = 22) of all patients. They had HD or other types of chorea 7.6% (n = 9), facial dystonia/dyskinesia (n = 7, 5.9%), 1 with TD, 2 with trunk dystonia, 2 with Tourette syndrome, and 1 with tardive akathisia. This group had the longest treatment duration and received a mean TBZ dose of 70.5 mg/d (median 75 mg/d) for a mean of 25.4 +/- 21.3 months. The report concludes that TBZ is a moderately effective treatment of a large variety of hyperkinetic movement disorders, with excellent effects in a subgroup with chorea and facial dystonia/dyskinesias.
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PMID:Tetrabenazine treatment in movement disorders. 1560 4

Movement disorders such as Parkinson's disease and Tourette's syndrome, primarily manifest during wakefulness, intrude into sleep. There are some disorders, however, such as periodic limb movements in sleep, restless legs syndrome, paroxysmal nocturnal dystonia, bruxism, and somnambulism, which occur primarily during sleep. The diagnosis and management of these disorders pose a challenge to neuropsychiatric practice, not only because they may be difficult to distinguish from other neuropsychiatric disorders, but also because psychiatric disorders are often co-morbid with them. Study of these disorders is necessary for an understanding of the interaction of sleep and movement, and how disturbance in one may affect the other.
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PMID:Periodic limb movements and other movement disorders in sleep: neuropsychiatric dimensions. 1619 1

Oromandibular dystonia (OMD) is a form of focal dystonia that affects masticatory, lower facial, and lingual muscles. We compared the clinical variables and response to treatment between patients with idiopathic jaw-closure C-OMD (n = 11) and jaw-opening dystonia O-OMD (n = 12) seen in our Movement Disorders clinic over the last 10 years. The co-existence of dystonia in other regions and sensory tricks were significantly more prevalent in O-OMD (P = 0.049 and 0.03, respectively). Male gender, orobuccolingual dyskinesias (facial grimacing, lip biting, tongue dyskinesias, platysma contractions and bruxism) and better response to botulinum toxin injections were more frequent in C-OMD but remained a trend.
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PMID:A comparison of jaw-closing and jaw-opening idiopathic oromandibular dystonia. 1627 95

Amongst all regions of the body, the craniocervical region is the one most frequently affected by dystonia. Whilst blepharospasm--involuntary bilateral eye closure--is produced by spasmodic contractions of the orbicularis oculi muscles, oromandibular dystonia may cause jaw closure with trismus and bruxism, or involuntary jaw opening or deviation, interfering with speaking and chewing. Both forms of dystonia can be effectively treated with botulinum toxin injection. This article summarizes injection techniques in both forms of dystonia and compares doses, potency and efficacy of different commercially available toxins, including Botox, Dysport, Xeomin and Myobloc/NeuroBloc.
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PMID:Botulinum toxin in blepharospasm and oromandibular dystonia: comparing different botulinum toxin preparations. 1641 94


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