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 report two cases of cranial dystonia (blepharospasm) associated with olivopontocerebellar atrophy (OPCA). The pathophysiology of blepharospasm appears to involve an increased excitability of the interneurons of the blink and corneal reflexes. It is hypothesized that blepharospasm associated with OPCA might be due to rostral brainstem lesions disrupting central dopaminergic and cholinergic pathways, resulting in disinhibition of brainstem reflexes or denervation supersensitivity of the facial nuclear complex.
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PMID:Blepharospasm associated with olivopontocerebellar atrophy. 253 Nov 69

Two patients developed difficulties in eyelid opening following long-term neuroleptic treatment of more than 6-8 years. Tardive dyskinesia and dystonia apart from the face were not found in either case. The symptoms fluctuated in their severities on a daily basis and were easily aggravated by various stimuli, e.g., stress, walking, reading and watching television. Electromyographic studies of their faces clearly indicated that the symptoms resulted from spontaneous blepharospasm and were analogous to idiopathic Meige's syndrome. Therefore, the patients' difficulties in opening their eyes were considered to be the so-called drug-induced Meige's syndrome and/or facial tardive dystonia. It must be stressed that this syndrome is extremely distressing to patients and is a severe complication accompanying a long-term neuroleptic treatment.
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PMID:Meige's syndrome during long-term neuroleptic treatment. 257 1

Essential blepharospasm is an idiopathic disorder of progressive involuntary spasms of the orbicularis oculi and upper facial (corrugator, procerus) muscles. Blepharospasm literally means spasm of the eyelids; however, most patients with blepharospasm also have or will develop squeezing in the lower face and neck muscles (Meige's syndrome, orofacial dystonia, or oromandibular dystonia). Some patients develop dystonic, uncontrolled movements in areas outside the facial nerve distribution (segmental cranial dystonia or craniocervical dystonia). Chronic, forceful squeezing by the periocular muscles becomes debilitating for the patient and leads to functional and cosmetic eyelid deformities. Treatment has included a variety of modalities and oral medications that are of limited efficacy. Botulinum-A toxin injections have delivered the best temporary relief from this disorder, while the periorbital myectomy operation has been shown to give the best long-term results.
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PMID:Essential blepharospasm and related dystonias. 268 56

A 16-year-old boy with generalized dystonia had continuous, severe blepharospasm and facial grimacing. Local intradermal injections of botulinum A toxin greatly reduced the spasms and improved function. No side effects were observed. Local botulinum A toxin injections may be useful in the treatment of eyelid and facial spasms in patients with generalized dystonias.
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PMID:Use of botulinum toxin to treat blepharospasm in a 16-year-old with a dystonic syndrome. 271 45

A patient is described who developed frequent blinking and blepharospasm after long-term treatment with trifluoperazine, whose condition improved dramatically after the cessation of the drug. The implications of this for our understanding of the manifestations and natural course of the late-onset side-effects of neuroleptic drugs are discussed. This case further supports the role of dopaminergic mechanisms in the aetiology of Meige's syndrome, which has blepharospasm and oromandibular dystonia as its main manifestations.
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PMID:Blinking-blepharospasm after long-term neuroleptic treatment. 271 47

A 64-year-old woman with blepharospasm, sustained clenching of the jaw, antecollis, and a strained, high-pitched phonation continued chronic trihexyphenidyl therapy despite the lack of any obvious benefit. Abrupt, accidental withdrawal of trihexyphenidyl triggered severe exacerbation of the cranial dystonia associated with inspiratory stridor and acute respiratory difficulties, prompting emergency admission. On indirect laryngoscopy, hyperadduction of the vocal folds was not the cause of the upper airway obstruction. A more likely cause of the inspiratory obstruction appeared to be forward bending of the neck combined with mouth-clenching spasms. Reinstitution of intravenous anticholinergic medication provided relatively prompt relief. We caution against abrupt interruption of anticholinergics in patients with severe segmental cranial dystonia, even in those cases in which no benefit is apparent to observers.
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PMID:Life-threatening cranial dystonia following trihexyphenidyl withdrawal. 281 95

The authors describe 19 patients with severe tardive dyskinesia, 11 of whom had a diagnosis of affective or schizoaffective disorder rather than schizophrenia. Most patients had been receiving long-term neuroleptic treatment with few interruptions and had received only one or two different neuroleptics. Frequent eye blinking was the most prevalent prodromal sign of tardive dyskinesia (in seven patients). Four subtypes of tardive dyskinesia could be distinguished: choreoathetosis, tardive dystonia, blepharospasm, and tardive akathisia. Optimal pharmacotherapy most often consisted of combinations of neuroleptics, lithium carbonate, benzodiazepines, and antiparkinsonian drugs. However, after an average of 62 months, only five patients had markedly improved.
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PMID:Clinical forms of severe tardive dyskinesia. 288 62

Meige's syndrome is characterized by blepharospasm and oromandibular dystonia. Three cases are presented; two were associated with long-term neuroleptic administration. This drug-induced syndrome may be a variant of tardive dystonia, and prompt discontinuation of neuroleptic treatment may be therapeutic.
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PMID:Meige's syndrome associated with neuroleptic treatment. 289 81

Dystonia is a neurologic disorder characterized by abnormal, involuntary movements causing twisting and turning postures; it is postulated to be a disorder of central motor processing. The dystonias, when classified by region of the body involved, have been characterized as focal, segmental, and generalized. Focal dystonia can affect jaw mechanics, leading to forceful contraction of the jaw muscles and resulting in inappropriate deviation of the jaw. Localized injections of botulinum toxin have been used successfully in the management of other focal or segmental dystonias. We have treated 20 oromandibular dystonia patients with botulinum toxin. Six patients had only jaw and tongue involvement; 11 had blepharospasm and jaw involvement; and three had jaw involvement as part of a more generalized dystonia. Five patients had been diagnosed originally and treated as having temporomandibular joint syndrome. All but one of the patients had improvement of their symptoms with the toxin injections. The patients averaged 47% improvement with the injections.
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PMID:Botulinum toxin injection for the treatment of oromandibular dystonia. 291 31

We studied 12 patients with spasmodic dysphonia (SD) and 12 healthy control subjects. The patients, who had no symptomatic involvement of the eyes, were evaluated for increased excitability of blink reflexes, which is characteristic of blepharospasm and generalized dystonia. We measured symptom severity from sound spectrograms of five sentences, including sentence production time, number of pitch phonatory breaks, and percentage of aperiodic phonation. We evoked blink reflexes by electrical and mechanical stimulation, and assessed excitability by obtaining excitability recovery curves and responses to trains of stimuli. Patients and controls differed from each other in test R2 amplitude attenuation across all intervals from 150 to 1,000 msec to electrical and mechanical stimulation. Our results indicate that patients with SD have increased excitability of blink reflexes, which suggests that the dystonia involves not only the larynx but also other anatomical structures.
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PMID:Blink reflex excitability recovery curves in patients with spasmodic dysphonia. 292 83


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