Agreement among neurologists on the clinical diagnosis of dystonia at different body sites.
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OBJECTIVE: To study the reliability of the diagnosis of blepharospasm, oromandibular dystonia, cervical dystonia, and writer's cramp among neurologists. METHODS: 12 patients with adult onset focal segmental dystonia were videotaped in a standardised way. The tape was sent to six neurologists who are involved in clinical practice without a specific interest in movement disorders (general neurologists), and to four neurologists expert in movement disorders. The observers had to recognise whether the patients were affected by dystonia and to distinguish among blepharospasm, oromandibular dystonia, cervical dystonia, and writer's cramp. Interobserver reliability was assessed by kappa statistics, and the degree of agreement was classified according to the Landis classification. RESULTS: The 10 neurologists reached slight to moderate agreement on the diagnosis of these four disorders. When the observers were subdivided according with their professional experience in the field, a moderate to perfect agreement on the diagnosis was achieved by specialists in movement disorders, and a fair to moderate agreement by the general neurologists. CONCLUSIONS: Neurologists may have different ability to recognise adult onset focal dystonia, depending on their experience and on the type of dystonia. (+info)
Ovarian granulosa cell tumor presenting as Meigs' syndrome with elevated CA125.
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Herein, a rare case of ovarian granulosa cell tumor, presenting as Meigs' syndrome, with elevated carbohydrate antigen 125 (CA125), is reported. A 69-year-old woman was admitted for the investigation of abdominal fullness and dyspnea. A preoperative examination revealed a huge pelvic tumor and an abdominopelvic magnetic resonance image (MRI) assumed ovarian cancer. A chest computed tomography (CT) scan revealed pleural effusion. A laparotomy confirmed the huge mass to be an ovarian tumor. A total abdominal hysterectomy (TAH), with a bilateral salpingo-oophorectomy (BSO) and partial omentectomy, was performed. Although short-term intrathoracic drainage was required, the hydrothorax and ascites rapidly resolved in the postoperative period. (+info)
Neurophysiological observations on the effects of botulinum toxin treatment in patients with dystonic blepharospasm.
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Botulinum toxin treatment improves dystonic blepharospasm by inducing transient paresis of the orbicularis oculi muscle. It is not known if it also reduces the enhanced brainstem neuronal excitability found in this disorder. We have performed conventional electromyography (EMG) and blink reflex excitability studies on fifteen patients with blepharospasm before and after botulinum toxin treatment. Denervation signs were found with needle EMG in all treated muscles. Amplitude of the facial compound muscle action potential (CMAP) and R1 response was reduced after botulinum toxin injections. In blink reflex excitability studies, the recovery of R2 response was enhanced after treatment even when patients were tested at the time of maximal benefit from botulinum toxin injections. The results suggest that there is little influence of botulinum toxin treatment upon the enhanced excitability of brainstem interneurons in patients with blepharospasm. (+info)
Natural history of treatment of facial dyskinesias with botulinum toxin: a study of 50 consecutive patients over seven years.
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To determine the long-term efficacy of botulinum toxin injections for the treatment of facial dyskinesias we studied 50 consecutive patients with blepharospasm, hemifacial spasm, and Meige syndrome. All received their first injection between September 1983 and June 1984. A total of 520 injections were given; the average number of injections per patient was 10.4 over the seven-year period ending September 1990. Twenty-six (52%) of the patients continued to return for periodic injections, while three patients no longer receive injections since they failed to respond adequately to treatment. Three patients with blepharospasm were in remission and required no further treatment, after a series of six, four, and three injections. Six patients were treated until they died of causes unrelated to facial dyskinesia or its treatment. Six patients are still being treated elsewhere because they could obtain injections closer to their homes. Five of the original 50 patients have been lost to follow-up. A patient with hemifacial spasm had one injection with good result but was not sufficiently bothered by her disease to return for reinjection. Complications were transient, minimal, well tolerated, and did not increase with increased number of injections. (+info)
Primary non-syndromic lymphoedema (Meige disease) is not caused by mutations in FOXC2.
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Oromandibular dystonia consists of prolonged spasms of contraction of the muscles of the mouth and jaw. Primary idiopathic forms and secondary forms exist. Secondary dystonia develops due to environmental factors; some cases of cranial dystonia after dental procedure have been reported, but the causal relationship between these procedures and dystonia remains unclear. Traumatic situations in the mouth, such as poor aligned dentures or multiple teeth extractions may cause an impairment of proprioception of the oral cavity, leading to subsequent development of dystonia. The clinical characteristics of oromandibular dystonia are classified according to the affected muscles. The muscles involved may be the muscles of mastication, muscles of facial expression, or the muscles of the tongue. At present, there is no known cure for OMD. The mainstay of treatment for most focal dystonia is botulinum toxin injections. It is important for the dentist to be familiar with oromandibular dystonia, as it can develop after dental treatment and is often misdiagnosed as a dental problem. (+info)
Brainstem pathology in spasmodic dysphonia.
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