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1.
The natural history and response to different treatments were assessed in 31 consecutive patients with blepharospasm (BS) and/or oromandibular dystonia (OMD). The mean age at onset was 52.4 years and there was a female preponderance of 2.5 to 1. Ocular symptoms preceded the onset of blepharospasm in more than 50% of the affected patients, whereas psychiatric and dental problems prior to the onset of focal dystonia were found in 10% and 13% of the cases respectively. Dystonia elsewhere, mainly in the craniocervical area, was found in 23% of patients and appeared to follow a somatotopic progression. The first 2–3 years of history were crucial for the spread of dystonia to other face and body parts. When OMD was the first symptom, a lower tendency of dystonia to progress elsewhere was observed. A putative cause was found in 14% of patients who showed clinical and radiographic evidence of basal ganglia or rostral brainstemdiencephalon lesions. The response to different drugs was inconsistent although transient improvement was induced by haloperidol in 6 patients, by L-Dopa plus deprenyl in 3 patients, by trihexyphenidyl in 2 patients and by clonazepam in 2 patients. One, apparently spontaneous, remission was observed. Botulinum A toxin was iniected in the orbicularis oculi of 8 patients affected by BS: moderate to marked improvement lasting 5 to 30 weeks (mean 14.5 weeks) was achieved in all cases; transient ptosis, lasting 1 to 3 weeks, occurred in 3 cases.
Sommario La storia naturale e la prognosi della distonia faciale sono state valutate in una serie di 31 consecutivi pazienti con blefarospasmo (BS) e/o distonia oromandibolare (OMD) (età di esordio: 19–75 anni; durata di malattia: 1–15 anni; rapporto maschi/femmine: 2.5/1). Sintomi oculari precedevano l'insorgenza del BS in oltre il 50% dei pazienti, mentre anomalie dentali e problematiche psichiatriche comparivano come prodromi nel 10% e nel 13% dei casi rispettivamente. La sintomatologia distonica diffondeva, con andamento somatotopico, oltre il distretto cranio faciale nel 23% dei casi. Evidenze cliniche o radiologiche di lesioni dei gangli della base, della parte rostrale del tronco dell'encefalo o del diencefalo erano presenti solo nel 13% dei casi. Un lieve ma transitorio miglioramento della sintomatologia distonica era indotto da aloperidolo in 6 pazienti, da 1-dopa+deprenyl in 3, da triesifenidile in 2 e da clonazepam in 2. Solo 1 paziente andava incontro a remissione apparentemente spontanea della sintomatologia distonica dopo un anno di malattia. L'iniezione di tossina botulinica di tipo A negli orbicolari delle palpebre di 8 pazienti con BS induceva un sensibile miglioramento della sintomatologia distonica che persisteva per 5–30 settimane (media 14.5 settimane) con ridotti effetti collaterali locali (transitoria ptosi in 3 casi).
  相似文献
2.
Abstract: 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.  相似文献
3.
The natural history and response to different treatments were assessed in 31 consecutive patients with blepharospasm (BS) and/or oromandibular dystonia (OMD). The mean age at onset was 52.4 years and there was a female preponderance of 2.5 to 1. Ocular symptoms preceded the onset of blepharospasm in more than 50% of the affected patients, whereas psychiatric and dental problems prior to the onset of focal dystonia were found in 10% and 13% of the cases respectively. Dystonia elsewhere, mainly in the craniocervical area, was found in 23% of patients and appeared to follow a somatotopic progression. The first 2–3 years of history were crucial for the spread of dystonia to other face and body parts. When OMD was the first symptom, a lower tendency of dystonia to progress elsewhere was observed. A putative cause was found in 14% of patients who showed clinical and radiographic evidence of basal ganglia or rostral brainstemdiencephalon lesions. The response to different drugs was inconsistent although transient improvement was induced by haloperidol in 6 patients, by L-Dopa plus deprenyl in 3 patients, by trihexyphenidyl in 2 patients and by clonazepam in 2 patients. One, apparently spontaneous, remission was observed. Botulinum A toxin was iniected in the orbicularis oculi of 8 patients affected by BS: moderate to marked improvement lasting 5 to 30 weeks (mean 14.5 weeks) was achieved in all cases; transient ptosis, lasting 1 to 3 weeks, occurred in 3 cases.
Sommario La storia naturale e la prognosi della distonia faciale sono state valutate in una serie di 31 consecutivi pazienti con blefarospasmo (BS) e/o distonia oromandibolare (OMD) (età di esordio: 19–75 anni; durata di malattia: 1–15 anni; rapporto maschi/femmine: 2.5/1). Sintomi oculari precedevano l'insorgenza del BS in oltre il 50% dei pazienti, mentre anomalie dentali e problematiche psichiatriche comparivano come prodromi nel 10% e nel 13% dei casi rispettivamente. La sintomatologia distonica diffondeva, con andamento somatotopico, oltre il distretto cranio faciale nel 23% dei casi. Evidenze cliniche o radiologiche di lesioni dei gangli della base, della parte rostrale del tronco dell'encefalo o del diencefalo erano presenti solo nel 13% dei casi. Un lieve ma transitorio miglioramento della sintomatologia distonica era indotto da aloperidolo in 6 pazienti, da 1-dopa+deprenyl in 3, da triesifenidile in 2 e da lonazepam in 2. Solo 1 paziente andava incontro a remissione apparentemente spontanea della sintomatologia distonica dopo un anno di malattia. L'iniezione di tossina botulinica di tipo A negli orbicolari delle palpebre di 8 pazienti con BS induceva un sensibile miglioramento della sintomatologia distonica che persisteva per 5–30 settimane (media 14.5 settimane) con ridotti effetti collaterali locali (transitoria ptosi in 3 casi).
  相似文献
4.
Abstract: A botulinum A toxin injection has beneficial effects on patients suffering from facial and cervical spastic disorders. However, its effect almost completely disappears within three months. We have reported a case of a 23-year-old schizophrenic patient with severe neuroleptic-induced Meige's syndrome in whom botulinum toxin treatment exerted a marked effect which lasted more than 15 months after the final injection of botulinum toxin in spite of continuous neuroleptic medication. It is concluded that botulinum can be recommended as a treatment of choice in neuroleptic-induced Meige's syndrome.  相似文献
5.
The spontaneous occurrence of blepharospasm and dystonic movements in face muscles, particularly those of the perioral and mandibular regions, has been named as Meige's disease which was first described by Henry Meige in 1910. We report the case of a woman with Meige's syndrome whose symptoms improved with the use of levetiracetam.  相似文献
6.
We demonstrated the effect of clonazepam (2 mg/day) on Meige syndrome in two schizophrenic patients under continuous treatment with antipsychotic drugs, and changes in the plasma levels of gamma-aminobutyric acid (GABA), homovanillic acid (HVA), and 3-methoxy-4-hydroxyphenylglycol (MHPG) in these cases. The plasma levels of HVA and MHPG during treatment with clonazepam were decreased in the responder, while not changed in the non-responder to clonazepam. A difference between the responder and the non-responder was not found in the plasma GABA levels. These results suggest that hyperactivities of the central dopaminergic and noradrenergic neurones are involved in the pathophysiology of Meige syndrome.  相似文献
7.
目的 观察双侧丘脑底核-脑深部电刺激术( STN - DBS)治疗3例Meige综合征患者的术后长期疗效.方法 3例Meige综合征患者接受双侧脑深部电极刺激术,术中微电极记录确定准确靶点定位,术后进行参数程控.术后1、3、6、12、24个月及最后一次复诊时进行随访,采用BFMDRS评价治疗效果,观察参数变化.结果 3例患者术后症状均得到明显改善,术后1个月时3例患者BFMDRS总评分、运动评分及功能障碍评分均有下降,术后3-6个月3例患者BFMDRS总评分、运动评分及功能障碍评分改善最明显,改善率达到90%以上,6个月后疗效稳定.结论 STN- DBS治疗3例Meige综合征患者效果理想,可以成为治疗Meige综合征的一种选择.  相似文献
8.
目的: Meige综合征是一种特发性累及头面部的肌张力障碍,有时也会影响到颈部。脑深部电刺激(DBS)双侧苍白球内侧部(GPi)治疗肌张力障碍的报道比较多,但治疗Meige综合征的很少。方法对3例双侧GPi-DBS术后的Meige综合征患者进行随访4、6和60个月。所有患者术前术后均采用国际通用的Burke-Fahn-Marsden Dystonia Rating 量表(BFMDRS)评分,包括BFMDRS-Ⅰ(Movement 量表)和BFMDRS-Ⅱ(Disability量表)两个部分。结果3例Meige综合征患者手术后症状均有明显改善,BFMDRS第一部分和第二部分评分的改善率分别为(78±3.1)%(范围75%~83%)和100%。结论双侧GPi-DBS是治疗原发性Meige综合征的一种有效安全的手术方式。  相似文献
9.
目的 探讨立体定向深部脑刺激术( DBS)和脑内核团毁损术在Meige综合征(MS)中的临床应用.方法 2例患者术前均采用Burke - Fahn - Marsden肌张力障碍运动评分(BFMDRS),1例MS患者行双侧苍白球内侧核(GPi) DBS手术治疗,1例患者行单侧GPi射频毁损术治疗.结果 行双侧GPi - DBS患者术后随访1年改善达83.7%(BFMDRS评分由术前的22.5分减少为4分),随访2年改善率达69.1%左右(BFMDRS评分由22.5分减少为6分),疗效较为稳定.1例行立体定向右侧GPi毁损术,术后1周疗效明显,BFMDRS从28分减少到6分,改善达78.6%;术后3个月BFMDRS评分为15分,改善率为46.2%,术后6个月随访,所有症状恢复到术前水平.结论 双侧GPi - DBS对于传统治疗无效的Meige综合征患者是一种安全有效的治疗方法;对于不能接受DBS手术的患者,行苍白球或丘脑毁损手术也是值得推荐的一种方法.  相似文献
10.
目的 研究Meige综合征患者抑郁的发生情况及相关的因素.方法 应用汉密尔顿抑郁量表(HAMD)对62例Meige综合征患者和40例健康体检者进行抑郁评定.结果 62例Meige综合征患者中,有34例(54.8%)伴发抑郁,其中轻度抑郁30例(48.3%),中度抑郁4例(6.5%),无重度抑郁;对照组中4例(10%)伴发抑郁,其中轻度抑郁3例(7.5%),中度抑郁1例(2.5%),无重度抑郁,两组相比较差异有统计学意义(x2=20.91,P<0.001);但是在程度上两组间无差异(x2=0.55,P=0.45);Meige综合征患者抑郁组与非抑郁组HAMD各因子比较显示两组焦虑/躯体化、认知障碍、阻滞、睡眠障碍及绝望感差异均有统计学意义.结论 Meige综合征患者中有较高抑郁的发生率,主要表现为情绪低落、工作和兴趣减退、忧虑、睡眠障碍、迟缓、自卑感等,抑郁的发生与Meige综合征患者病程无关,而与性别相关,女性易发生.  相似文献
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