1/67. Atypical and typical cranial dystonia following dental procedures.It is generally recognized that focal dystonia of the limbs or cervical region and blepharospasm sometimes follow, and in these cases may be caused or triggered by, peripheral injury. However, the association between peripheral injury and lower cranial dystonia is rare. We report eight cases who developed cranial dystonia within hours to months following a dental procedure. One group of five cases, all women, developed atypical dystonia associated with painful paresthesias at the site of dystonia. Two of these five cases had fixed jaw-deviating dystonia, whereas the remaining three had additional tremor and spread of their dystonia to involve the tongue in all three, and the lips and neck in two cases. These five patients are reminiscent of cases of limb causalgia-dystonia syndrome, which occurs after minor peripheral trauma and can spread. The remaining three cases developed more typical cranial dystonia following the dental procedure. There was no family history of dystonia or prior use of neuroleptics in any of the patients. The close association in time and location of the procedure and onset of symptoms suggests that the onset of the dystonia may have been caused by the dental intervention, but whether there is a causal relationship between the dental intervention and the development of the dyskinesias requires further epidemiologic studies.- - - - - - - - - - ranking = 1keywords = hour (Clic here for more details about this article) |
2/67. Rapid-onset dystonia-parkinsonism: a clinical and genetic analysis of a new kindred.BACKGROUND: Rapid-onset dystonia-parkinsonism (RDP) is an autosomal dominant disorder linked to chromosome 19q13 that is characterized by sudden onset of primarily bulbar and upper limb dystonia with parkinsonism. methods: The authors evaluated 12 individuals from three generations of an Irish family and obtained detailed medical records on a deceased member. The authors describe the clinical, psychiatric, and genetic features of the affected individuals. RESULTS: Five of eight affected members developed sudden-onset (several hours to days) dystonia with postural instability. Four of the five also had bulbar symptoms. Two have stable focal or segmental limb dystonia. One has intermittent hemidystonia with dysarthria that comes on abruptly in times of stress or anxiety. Three had a history of profound difficulty socializing, and at presentation two developed depression. Three patients had a trial of dopamine agonists without benefit. Genetic analysis suggests linkage to chromosome 19 with lod score of 2.1 at zero recombination. CONCLUSION: This is the third reported family with chromosome 19q13 rapid-onset dystonia-parkinsonism. Psychiatric morbidity appeared common in affected members of this family and may be part of the RDP phenotype.- - - - - - - - - - ranking = 1keywords = hour (Clic here for more details about this article) |
3/67. Pallidal and thalamic neurostimulation in severe tardive dystonia.A 70 year old woman presented with a 6 year history of medically refractory severe tardive dystonia. After informed consent, a bilateral stereotactic electrode placement targeting the ventral intermediate thalamic nucleus (VIM) and the globus pallidus internus (GPi) was performed. After bilateral stimulation of the GPi, the patient showed a clear and stable improvement of the painful dystonic syndrome within hours. Stimulation of the VIM did not improve the hyperkinetic movements and simultaneous stimulation of both the GPi and the VIM did not result in any additional benefit. The possible pathophysiological mechanisms are discussed.- - - - - - - - - - ranking = 1keywords = hour (Clic here for more details about this article) |
4/67. bupropion-induced acute dystonia.OBJECTIVE: To report a case of acute dystonia consisting of neck stiffness, trismus, and unilateral temporomandibular joint (TMJ) pain and subluxation secondary to an increase in sustained-release (SR) bupropion. CASE SUMMARY: A 44-year-old white man with a history of chronic low-back pain and tension headaches, taking no other medications, was started on bupropion SR 150 mg once a day for depression. The dosage was increased to 150 mg SR twice a day and eventually augmented with buspirone 15 mg 3 times a day. He developed bilateral trismus, inability to rotate his head laterally, and spontaneous left TMJ subluxation. Symptoms recessed with discontinuation of both medications and failed to reappear with a trial of buspirone 15 mg 3 times a day alone. A retrial of bupropion alone evidenced no adverse effects at a dosage of 150 mg SR once a day. However, when the dosage was increased to 150 mg SR twice a day, the patient reexperienced initial signs of neck stiffness, jaw muscle tightness, and left TMJ subluxation within 24-48 hours. Reduction of the bupropion dosage to 150 mg SR once daily stopped the symptoms; the patient has continued at this dosage without adverse effects for > 1 year. DISCUSSION: Medication-induced focal dystonias usually present with dramatic head (most frequently oral-buccal) and neck muscle spasm with occasional jaw clenching, bruxism, and TMJ syndrome. In this case, the rapid onset of neck and jaw symptoms within 24-48 hours of an increase of bupropion SR from 150 mg once a day to 150 mg twice a day suggest that the patient may have been sensitized by an initial trial of bupropion and buspirone, or by the increased dose of bupropion alone. Both agents are reported to interact with both the dopaminergic and serotonergic systems. Although buspirone has been implicated in inducing acute dystonia, it did not do so in this case when used alone at a dose of 45 mg a day. During a second trial of bupropion SR 150 mg a day, neck and jaw symptoms recurred within 24-48 hours of increasing the dose to 150 mg SR twice a day. The symptoms receded when the bupropion dose was returned to 150 mg SR once a day, suggesting a dose-response relationship. The Naranjo probability scale indicated that this untoward reaction was probable. CONCLUSIONS: This case suggests that selected patients may experience dose-related acute dystonic adverse reactions to bupropion with or without buspirone augmentation. Dystonias, which usually follow administration of antipsychotics, have been linked to acute dopamine depletion and basal ganglion-derived gamma synchronization dysfunction. Acute dystonia symptoms may begin within hours of starting or changing antipsychotic drug dosage; however, 90% of symptoms are observed during the first 3-5 days of starting or increasing dosage. To the best of our knowledge, there have been no reports of bupropion-induced dystonia.- - - - - - - - - - ranking = 4keywords = hour (Clic here for more details about this article) |
5/67. Post-streptococcal autoimmune neuropsychiatric disease presenting as paroxysmal dystonic choreoathetosis.Paroxysmal dystonic choreoathetosis (PDC) is an episodic, non-kinesogenic, extrapyramidal movement disorder. It is postulated that PDC is an ion channel disorder. We describe a sporadic case of paroxysmal dystonic choreoathetosis occurring after streptococcal pharyngitis. The episodes were characterized by abrupt-onset dystonic posturing, choreoathetosis, visual hallucinations and behavioral disturbance. Each episode lasted between 10 minutes and 4 hours, and occurred up to 4 times per day. In between attacks, examination was normal. The episodes waxed and waned in frequency during a 6-month illness. magnetic resonance imaging of the brain was normal. Post-streptococcal neuropsychiatric disease has a proposed autoimmune etiology, which is supported by the presence of serum antibasal ganglia antibodies. Western immunoblotting of this case's serum demonstrated antibody binding to a basal ganglia antigens of molecular weight 80 kDa and 95 kDa. immunohistochemistry examination demonstrated specific antibody binding to large striatal neurones. We propose that autoantibodies produced in post-streptococcal neuropsychiatric disease cause alteration in neurotransmission, possibly secondary to ion channel binding.- - - - - - - - - - ranking = 1keywords = hour (Clic here for more details about this article) |
6/67. fever-induced dystonia.The etiology of dystonia in children is often elusive. We report two children with fever-induced dystonia, an association which has not been previously reported. Both children had episodes of lower extremity dystonia with inversion of the feet and dorsiflexion of the great toes. Events began at 2 years of age, were preceded by febrile illness, and lasted for up to 24 hours. In both children, one of the parents had a history of similar but milder symptoms in childhood. Between spells the children were neurologically normal. Multiple investigations, including metabolic and imaging studies, were normal and therapies were ineffective. As the children aged the dystonic events occurred less frequently. This dystonic syndrome is a previously unrecognized benign episodic movement disorder of childhood.- - - - - - - - - - ranking = 1keywords = hour (Clic here for more details about this article) |
7/67. Spinocerebellar ataxia type 3 presenting as an L-DOPA responsive dystonia phenotype in a Chinese family.The clinical spectrum of spinocerebellar ataxia 3 (SCA 3) disease is wide and varied. We describe a Chinese patient with a mutation at the SCA 3 locus with clinical features of levodopa-responsive dystonia. The family history was suggestive of being autosomally dominant. levodopa responsiveness though rare has been described in families with features of parkinsonism. Noteworthy is the relatively late onset of disease (>40 years) possibly explained by the low number of affected alleles at 59, the usual range being from 62 to 86, with the lowest recorded number at 56. This expands the wide and varied phenotypic manifestations of SCA 3, and highlights the observation that features suggestive of levodopa-responsive dystonia (DRD) such as focal dystonia, gait difficulty with diurnal fluctuation of symptoms, and a marked response to low doses of levodopa can be presenting features of SCA 3. SCA 3 should be considered a differential diagnosis in adult patients who present with DRD phenotype and with a positive family history.- - - - - - - - - - ranking = 32804.464511376keywords = diurnal (Clic here for more details about this article) |
8/67. life-threatening respiratory failure due to cranial dystonia after dental procedure in a patient with multiple system atrophy.We report on a woman with a an 8-year history of multiple system atrophy with predominance of parkinsonism who developed jaw-locking oromandibular dystonia within hours after insertion of ill-fitting dentures. dystonia spread rapidly to involve other facial muscles and the larynx causing stridor with respiratory failure necessitating crush intubation.- - - - - - - - - - ranking = 1keywords = hour (Clic here for more details about this article) |
9/67. Delayed onset of oculogyric crisis and torticollis with intramuscular haloperidol.OBJECTIVE: To report a case of delayed-onset dystonic reactions, oculogyric crisis (OGC), and torticollis after treatment with intramuscular haloperidol lactate injection. CASE SUMMARY: A 22-year-old Mexican American woman received intramuscular haloperidol lactate 7.5 mg followed 4 hours later by 10 mg. Twenty-six hours after the first injection, the patient reported that she was unable to lower her gaze and that her neck was stiff. She was immediately given intramuscular benztropine 2 mg; there was a nearly complete remission of symptoms within 15 minutes of treatment. An objective causality assessment revealed a probable relationship between the OGC/torticollis and haloperidol therapy. DISCUSSION: Dystonic reactions have been reported in 10-60% of patients treated with neuroleptic medication, most commonly when patients just start or increase the dose of the drug. The highest frequency of dystonic reactions has occurred in patients receiving high-potency neuroleptics. It has also been suggested that haloperidol-induced dystonic reactions are a result of the toxic metabolites of that agent. CONCLUSIONS: OGC and torticollis reactions may occur 12-24 hours after treatment with a high-potency neuroleptic, even in the absence of symptoms of extrapyramidal side effects (EPSEs). The delayed dystonic reaction may begin suddenly (no early EPSE symptomatology).- - - - - - - - - - ranking = 3keywords = hour (Clic here for more details about this article) |
10/67. Hereditary progressive dystonia with diurnal fluctuation (Segawa's syndrome)--an unusual case.A young girl with hereditary progressive dystonia with diurnal fluctuation or Segawa's syndrome, completely handicapped and confined to a wheelchair between the age of 5 and 9, revealed an unusually slow response to levodopa. The ability to walk returned only after 12 to 14 months of treatment. Apart from peculiarities of behaviour due to longstanding immobility and ensuing parental overprotection, there were no psychological or mental abnormalities. Other organic diseases were ruled out. A series of regular follow-ups over the course of 7 years was performed. While residual and irregular day-to-day variation of functional capacities almost disappeared after conversion to a preparation with a decarboxylase inhibitor, some mild neurological abnormalities and a slight choreic hyperkinesia persisted with doses providing functional results. The patient today leads an almost normal teenage life and has performed well in school.- - - - - - - - - - ranking = 164022.32255688keywords = diurnal (Clic here for more details about this article) |
| | Next -> |