基本震颤 - 摇晃场地

三月是National Essential Tremor Awareness Month,一种神经运动障碍,导致手和头部无意的摇动运动。在这个来宾博客中,Ariel Levy和Robert Chen,编辑委员会成员Journal of Clinical Movement Disorders,解释有关该疾病的更多信息。

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A slowly progressive disease causing shaking of the hands and head
A slowly progressive disease causing shaking of the hands and head
Wikipedia (CC-BY)

什么是基本震颤?

Essential tremor is a slowly progressive disease that causes shaking while assuming static positions (postural tremor) and during movements (action tremor). It affects the arms (90%), head (30%), voice (20%) and occasionally trunk and legs (10-15%).

通常,静止没有震颤,震颤并非特定于某些任务,也没有异常的姿势。否则,普通神经检查应是正常的。进展很慢。随着年龄的增长,震颤频率降低,幅度增加,这可能会增加残疾。

通常有较强的家族史,酒精通常会改善震颤。生理(发烧,感染等)和心理压力加剧了病情。

通常有较强的家族史,酒精通常会改善震颤。生理(发烧,感染等)和心理压力加剧了病情。

Although theterm ‘essential’,是在19岁时创造的Thcentury, suggests a benign condition, considerable disability can occur. Tremor can have an impact on eating using a fork or spoon. Drinking a cup of tea or eating soup provides the greatest challenges because the risk of spilling.

与基本震颤一起生活

具有基本震颤的人倾向于用双手抓住杯子,用杯子吃汤,而不是使用勺子或仅使用稻草作为液体。笔迹变得凌乱,可能变得难以辨认。敷料也变得困难,尤其是在衣服或铜管上的纽扣,鞋带和闩锁上。

对于那些需要良好灵巧性来执行工作(例如画家,木工,力学等)的人,震颤尤其令人沮丧。与身体表现相关,与震颤相关的社会污名和患者限制了他们的社交活动。

尽管有一个多世纪的认可基本震颤,但这种情况的原因仍然难以捉摸,也是一个备受争议的话题。必需的经典罪魁祸首是一组名为“中央振荡器”的神经元的功能障碍。

A growing body of literature is implicating the cerebellum and its outflow to other parts of the brain such as the thalamus and the cortex. Classically, tremor was considered the only manifestation of essential tremor.

More recently, it has grown to encompass a syndrome including cognitive problems, olfactory problems, hearing problems, balance disorders and abnormal eye movements.

Yet, more recently, it has grown to encompass a syndrome including cognitive problems, olfactory problems, hearing problems, balance disorders and abnormal eye movements. However, the attribution of these various conditions to a possible ‘essential tremor syndrome’ remains controversial.

Is there any treatment available?

药物是治疗的第一步ET. The American Academy of Neurology haspublished treatment guidelines(updated in 2011) and a recent comprehensivereview of the treatments于2015年出版。

第一线药物是丙酚和原酮,它们具有最强大的临床证据。通常尝试的其他药物是加巴喷丁,托吡酯,氯硝西am和肉毒杆菌毒素注射。

Each of these has side effects limiting their use. Propanolol should not be used in people with asthma, athletes (exercise intolerance) and those with heart conduction disease. Primidone can cause depression, drowsiness, cognitive and behavioral problems.

For those in whom medications have been unsuccessful, interventional approaches are considered. The mainstay for decades was thalamotomy but now thalamic deep brain stimulation has supplanted the former.

这些技术的局限性是只能针对治疗的一侧,通常是最有症状的。

这些技术的局限性是只能针对治疗的一侧,通常是最有症状的。最后,市场上有许多小工具汤匙,碗,改装键盘和软件这允许功能适应震颤。

There has been increased interest in alternative surgical strategies for tremor treatment. Recent studies have assessed treatment with伽玛刀放射外科手术Thalamotomy with some benefit (albeit delayed and requiring multiple treatment cycles).

但是,尚未进行随机试验。另一条研究大道是集中的超声丘脑切开术。最近comparison between deep brain stimulation and focused ultrasoundfound that both are equivalent in terms of disability improvement and quality of life.

There is limited data on the long-term response to treatment. A limitation of these lesion strategies are that only one side can be targeted for treatment, usually the most symptomatic side.

提高我们对未来的理解

遗传学的进步,包括下一代sequencing, paired with an understanding of the associated features of ‘essential tremor syndrome’ will greatly improve our understanding of essential tremor.

研究探索基本震颤中不同大脑区域之间异常联系的研究可能会允许更具体的治疗方法。

同样,研究探索基本震颤中不同大脑区域之间异常联系的研究可能会允许更具体的治疗方法。例如,使用非侵入性脑刺激(例如低频经颅磁刺激)可能会改善震颤,但没有进行大规模的随机临床试验。

However exciting this therapeutic avenue is, (and personal use devices are readily available to purchase on the internet – with little oversight) it is not ready for primetime.

Essential tremor is far from a benign condition. Greater understanding of the causes of the condition with advanced neuroimaging, physiology, genetics and well defined clinical studies have the potential to discover specific treatments for essential tremor and shake up the field.

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8Comments

内森O麦卡斯勒

I’ve had ET since I was 12 and it’s progressive, so it’s gotten worse after my late 30’s and more so after I turned 41. One of the treatments not mentioned above or researched at all is high doses of vitamin D (5000-8000 IU/day). It’s about 30% more effective than propanolol and without the side effects. It’s staggering just how much better my tremor gets and how long this treatment lasts compared to other prescription drugs. I have no idea why this works but someone needs to look into it.

frank long

Have had ET for 20 years, with progessive head and hand (both) tremors. Recently discovered I can reduce hand tremors by initiating a movment, then shifting my focus and attention away while my hand completes the movement. Example: Ordinarily, to type, I focus on a letter, hover a trembling finger over the letter, then tense my finger and force it to press down. Now, to type, say, ABCDE, I start a finger toward A, then shift my eyes and attention toward B while pressing A …and so on. Takes some practice, but have increased my typing speed at least four-fold.
Best wishes to all.
弗兰克·朗(Frank Long),俄勒冈州

Cynthia Volkmann

Are there any tools to help people who have essential tremors to type with accuracy?

Author

The above article has a link to various adapted keyboards and tools for those with tremor.
另一个选择是语音识别软件,这可能非常有用。

Janis Gewartowski

我是一名70岁的女性,一年半前进行了DBS手术。该程序已成功地双侧处理了震颤。双方都没有任何问题。

Lora Ruddick

I definitely have essential tremor. The problem is that I am 79 years old and I’m not sure I can get into any clinical trials. I guarantee you that my quality of life is not good at all and I’ve tried everything that I know of that I can try. The neurologists have no idea what to do. My father had it and my children have it, so it is definitely hereditary. If you can give me any information or help in any way, it would be so greatly appreciated. Thank you for your time.

Admin
Davy Falkner

Dear Lora, although we post blogs on medical topics we do not give medical advice so unfortunately cannot offer assistance in this instance.

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