Intention tremor

Intention tremor
Intention tremor
Classification and external resources
ICD-10 G25.2
ICD-9 333.1
MeSH D014202

Intention tremor, also known as cerebellar tremor, is a dyskinetic disorder characterized by a broad, course, and low frequency (below 5 Hz) tremor. The amplitude of an intention tremor increases as an extremity approaches the endpoint of deliberate and visually guided movement (hence the name intention tremor). An intention tremor is usually perpendicular to the direction of movement. When experiencing an intention tremor, one often overshoots or undershoots their target, a condition known as dysmetria.[1][2] Intention tremor is the result of dysfunction of the cerebellum, particularly on the same side as the tremor in the lateral zone, which controls visually guided movements. Depending on the location of cerebellar damage, these tremors can be either unilateral or bilateral.[1]

A variety of causes have been discovered to date, including damage or degradation of the cerebellum due to neurodegenerative diseases, trauma, tumor, stroke, or toxicity. There is currently no established pharmacological treatment, however, some success has been seen using treatments designed for essential tremors.[1][3]

Contents

Causes

Intention tremors are common among individuals with multiple sclerosis. One common symptom of multiple sclerosis is ataxia. This is a lack of coordinated muscle movement is caused by cerebellar lesions characteristic of multiple sclerosis. The disease often destroys physical and cognitive function of individuals. Intention tremors can be a first sign of multiple sclerosis, since loss of deteriorated motor function and sensitivity are often one of the first symptoms of cerebellar lesions.[1][4]

Intention tremors have a variety of other recorded causes as well. These include a variety of neurological disorders, such as stroke, alcoholism, alcohol withdrawal, peripheral neuropathy, Wilson's disease, Creutzfeldt-Jacob disease, Guillain–Barré syndrome and fragile X syndrome, as well as brain tumors, low blood sugar, hyperthyroidism, hypoparathyroidism, insulinoma, normal aging, and traumatic brain injury.[1][5][6][7] Holmes tremor, a rubral or midbrain tremor, is another form of tremor that includes intention tremors, among other symptoms. This disease affects the proximal muscles of the head, shoulders, and neck. Tremors of this disease occur at frequencies of 2–4 Hz or more.[8] Intention tremor is also known to be associated with infections, West Nile Virus, rubella, H. influenza, rabies, and varicella.[1][9] A variety of poisons have been shown to cause intention tremor, including mercury, methyl bromide, and phosphine.[1][10][11] In addition, vitamin deficiencies have been linked to intention tremor, especially deficiency in vitamin E.[12] Pharmacological agents such as anti-arrhythmic drugs, anti-epileptic agents, benzodiazepine, cyclosporine, lithium, neuroleptics, and stimulants have been known to cause intention tremor.[3] Some ordinary activities including ingesting too much caffeine, cigarettes, and alcohol, along with stress, anxiety, fear, anger and fatigue have also been shown to cause intention tremor by negatively effecting the cerebellum, brainstem, or thalamus, as discussed in mechanisms.[5]

Mechanism

Cerebellum
The cerebellum is highlighted in purple

Intention tremors caused by normal, everyday activities, such as stress, anxiety, fear, anger, caffeine, and fatigue do not seem to result from damage to any part of the brain. These tremors instead seem to be a temporary worsening of a small tremor that is present in every human being. These tremors go away with time.[5]

More permanent intention tremors are caused by damage to certain regions of the brain. The most common cause of intention tremors is damage to the cerebellum. The cerebellum is a part of the brain responsible for motor coordination, posture and balance. It is responsible for fine motor movements. When the cerebellum is damaged, a person has difficulty executing a fine motor movement, such as attempting to touch one's nose with one's finger. One of the most common ways for the cerebellum to become damaged is through the development of cerebellar lesions.[13] The most common site for cerebellar lesions that lead to intention tremors has been reported to be the superior cerebellar peduncle, through which all fibers carrying information to the midbrain pass, and the dentate nucleus, which is also responsible for linking the cerebellum to the rest of the brain.[3] Alcohol abuse is one typical cause of this damage to the cerebellum. The alcohol abuse causes degeneration of the anterior vermis of the cerebellum. This leads to an inability to process fine motor movements in the individual and the development of intention tremors. In Multiple Sclerosis, damage occurs due to demyelination and neuron death, which again produces cerebellar lesions and an inability for those neurons to transmit signals.[13] Because of this tight association with damage to the cerebellum, intention tremors are often referred to as cerebellar tremors.[1]

Intention tremors can also be caused as a result of damage to the brainstem or thalamus. Both of these structures are involved in the transmission of information between the cerebellum and the cerebral cortex, as well as between the cerebellum and the spinal cord, and then on to the motor neurons. When these become damaged, the relay system between the cerebellum and the muscle on which it is try to act upon is compromised, resulting in the development of a tremor.[13]

Diagnosis

A working diagnosis is made from a neurological examination and evaluation. Parts of a complete examination include a physical examination, MRI, patient history, and electrophysiological and accelerometric studies. A diagnosis of solely intention tremor can only be made if the tremor is of low frequency (below 5 Hz) and without the presence of any resting tremors.[1] Electrophysiological studies can be useful in determining frequency of the tremor, and accelerometric studies quantify tremor amplitude. MRI is used to locate damage to and degradation of the cerebellum that may be causing the intention tremor. Focal lesions such as neoplasms, tumors, hemorrhages, demyelination, or other damage may be causing dysfunction of the cerebellum and correspondingly the intention tremor.[14]

Physical tests are an easy way to determine the severity of the intention tremor and impairment of physical activity. Common tests that are used to assess intention tremor are the finger-to-nose and heel-to-shin tests. In a finger-to-nose test a physician has the individual touch their nose with their finger while monitoring for irregularity in timing and control of the movement. An individual with intention tremors will have course, side-to-side movements that increase in severity as the finger approaches the nose. Similarly, the heel-to-shin test evaluates intention tremors of the lower extremities. In such a test, the individual places their heel on the top of their opposite knee and is then instructed to slide their heel down their shin to their ankle while being monitored for course and irregular side to side movement as the heel approaches the ankle. Important historical elements to the diagnosis of intention tremor are:

  1. age of onset
  2. mode of onset (sudden or gradual)
  3. anatomical affected sites
  4. rate of progression
  5. exacerbating and remitting factors
  6. alcohol abuse
  7. family history of tremor[15]
  8. current medications[12]

Secondary symptoms commonly observed are dysarthria (a speech disorder characterized by poor articulation and slurred speech), nystagmus (rapid involuntary eye movement, especially rolling of the eyes), gait problems (abnormality in walking), and postural tremor or titubation (to-and-fro movements of the neck and trunk). A postural tremor may also accompany intention tremors.[1][12]

Management

Tiefe Hirnstimulation - Deep Brain Stimulation

It is very difficult to treat an intention tremor. The tremor may disappear for a while after a treatment has been administered, and then return after some time. This situation must be addressed with a different type of treatment. First, individuals will be asked if they use any of the drugs known to cause tremors. If so, they are asked to stop taking the medication and then evaluated after some time to determine if the medication was related to the onset of the tremor. If the tremor persists, treatment that follows may include drug therapy, lifestyle changes, and more invasive forms of treatment, such as surgery and thalamic deep brain stimulation.[14]

Intention tremors are notoriously known to be very difficult to treat with pharmacotherapy and drugs. Although there is no established pharmacological treatment for an intention tremor, several drugs have been found to have positive effects on intention tremors and are used as treatment by many health professionals. Isoniazid, buspirone hydrochloride, glutethimide, carbamazepine, clonazepam, buspirone, topiramate, zofran, propranolol and primidone have all seen moderate results in treating intention tremor and can be prescribed treatments. Isoniazid inhibits γ-aminobutyric acid-aminotransferase, which the first step in enzymatic breakdown of GABA, thus increasing GABA, the major inhibitory neurotransmitter in the central nervous system. This causes a reduction in cerebellar ataxias. Another neurotransmitter targeted by drugs that has been found to alleviate intention tremors is serotonin. The agonist buspirone hydrochloride, which decreases serotonin's function in the central nervous system, has been viewed as an effective treatment of intention tremors.[1]

There are also alternative treatments available. Cannabis has been seen as an effective treatment for individuals with tremors, however there is no scientific data that has been shown to prove this.[16] Also, physical therapy has had great results in reducing tremors, but usually does not cure the tremors. Relaxation techniques, such as meditation, yoga, hypnosis, and biofeedback, are other alternative treatments that have seen some results with tremors. Wearing wrist weights which will weigh down one's hands as they make movements, masking much of the tremor, is a proven home remedy. This is not a true treatment, since wearing the weights does not have any lasting effects when they are not on, however they do help the individual cope with the tremor immediately.[12]

A more radical treatment that is used in individuals who do not respond to drug therapy, physical therapy, or any other treatment listed above, with moderate to severe intention tremors, would be surgical intervention. Deep brain stimulation and surgical lesioning of the thalamic nuclei has been found to be an effective long-term treatment with intention tremors. Deep brain stimulation, treats intention tremors but does not help related diseases or disorders such as dyssynergia and dysmetria.[17] Deep brain stimulation involves the implantation of a device called a brain pacemaker, shown in the picture above. This medical device sends electrical impulses to specific parts of the brain, changing brain activity in a controlled manner. In the case of an intention tremor, the thalamic nuclei is the region of the brain targeted for treatment. This form of treatment causes reversible changes and does not cause any permanent lesions. Since it is reversible, deep brain stimulation is considered fairly safe, and reduction in tremor amplitude is almost guaranteed and sometimes it is even resolved. Some individuals with multiple sclerosis have seen sustained benefits in MS progress.[18] Thalamotomy is another surgical treatment where lesions of the thalamus nucleus are created to disrupt the tremor circuit. Thalamotomy has been used to treat many different forms of tremors, including tremors that arise from trauma, multiple sclerosis, stroke, and those whose cause it unknown. This is a very invasive, high-risk treatment with many negative effects, such as multiple sclerosis worsening, cognitive dysfunction, worsening of dysarthria, and dysphagia. Immediate positive effects are seen in individuals treated with a thalamotomy procedure. However, the tremor often comes back after some time, thus it is not a complete treatment. Thalamotomy is currently in clinical trials to determine the validity of the treatment of intention tremors with all its high risks.[1][19]

Research directions

Current research has focused on finding a pharmacological treatment that is specific for intention tremor. Currently, limited success has been seen in treating intention tremor with drugs effective at treating essential tremor.[12] Clinical trials of levetiracetam, typically used to treat epilepsy, and pramipexole, used to treat resting tremor, have recently been completed to establish their effectiveness in treating kinetic tremor.[20][21] A clinical trial for riluzole, typically used to treat amyotrophic lateral sclerosis, is currently underway at the Sapienza University of Rome to evaluate its effectiveness of treating cerebellar ataxia and kinetic tremor.[22]

History

In 1868, French neurologist Jean-Martin Charcot first characterized the distinction between MS and its resulting intention tremor from resting tremor, characteristic of Parkinson’s disease. Intention tremor became known as part of Charcot's triad which, along with nystagmus and scanning speech, act as strong indications of MS.[23]

External links

References

  1. ^ a b c d e f g h i j k l [1] Seeberger, Lauren. "Cerebellar Tremor-Definition and Treatment." The Colorado Neurological Institute Review. Fall 2005.
  2. ^ [2] National Institute of Neurological Disorders and Stroke. “Tremor Fact Sheet.” Jan 2011. National Institutes of Health.
  3. ^ a b c Bhidayasiri, R (2005). "Differential diagnosis of common tremor syndromes". Postgraduate Medical Journal 81 (962): 756–62. doi:10.1136/pgmj.2005.032979. PMC 1743400. PMID 16344298. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=1743400. 
  4. ^ Deuschl, G.; Wenzelburger, R; Löffler, K; Raethjen, J; Stolze, H (2000). "Essential tremor and cerebellar dysfunction Clinical and kinematic analysis of intention tremor". Brain 123: 1568–80. doi:10.1093/brain/123.8.1568. PMID 10908187. 
  5. ^ a b c [3] Hoch, Daniel MD. “Tremor.” Updated 6/15/2010. U.S. National Library of Medicine.
  6. ^ [4] Hagerman, Paul, Hagerman, Randi. “Fragile X-Associated Tremor/Ataxia Syndrome-An Older Face of the Fragile X Gene.” 2007. In Nature Clinical Practice Neurology. Nature Publishing Group.
  7. ^ [5] Eidelberg, David, Pourfar, Michael. "Tremor." 2007. In The Merck Manuals Online Medical Library.
  8. ^ [6] "Postischemic Holmes Tremor Investigated by FDG- and H2 O-PET." Jan Kassubek, G. Bernhard Landwehrmeyer, Carl H. Lücking, Freimut D. Juengling. The Journal of Radiology, June 2003
  9. ^ Debiasi, Roberta L; Tyler, Kenneth L (2006). "West Nile virus meningoencephalitis". Nature Clinical Practice Neurology 2 (5): 264–75. doi:10.1038/ncpneuro0176. PMID 16932563. 
  10. ^ [7] “Epidemiological Notes and Reports Elemental Mercury Poisoning in a Household – Ohio 1989.” 1990. In Morbidity and Mortality Weekly Report. Centers for Disease Control and Prevention.
  11. ^ [8] “Medical Management Guidelines for Acute Chemical Exposures.” 1992. U.S Department of Human Health Services.
  12. ^ a b c d e Wyne, Kevin T. (2005). "A comprehensive review of tremor". JAAPA : official journal of the American Academy of Physician Assistants 18 (12): 43–50; quiz 57–8. PMID 16392266. http://www.jaapa.com/a-comprehensive-review-of-tremor/article/124526/. 
  13. ^ a b c [9] Purves, Dale et al. Neuroscience. Fourth Edition. 2008. Sinauer Associates, Inc.
  14. ^ a b Chou, Kelvin L. (2004). "Diagnosis and management of the individual with tremor". Medicine and health, Rhode Island 87 (5): 135–8. PMID 15250610. http://med.brown.edu/neurology/articles/kc13504.pdf. 
  15. ^ Walker, H. Kenneth (1990). "The Cerebellum". In Walker HK, Hall WD, Hurst JW. Clinical Methods: The History, Physical, and Laboratory Examinations. Reed Publishing. ISBN 0409950033. [page needed]
  16. ^ Zajicek, John P.; Apostu, Vicentiu I. (2011). "Role of Cannabinoids in Multiple Sclerosis". CNS Drugs 25 (3): 187–201. doi:10.2165/11539000-000000000-00000. PMID 21323391. 
  17. ^ Wishart, H A (2003). "Chronic deep brain stimulation for the treatment of tremor in multiple sclerosis: review and case reports". Journal of Neurology, Neurosurgery & Psychiatry 74: 1392–7. doi:10.1136/jnnp.74.10.1392. 
  18. ^ Karbowniczek, A; Domitrz, I (2011). "Thalamic deep brain stimulation for tremor among multiple sclerosis patients". Neurologia i neurochirurgia polska 45 (1): 11–17. PMID 21384288. 
  19. ^ Benabid, AL; Pollak, P; Gervason, C; Hoffmann, D; Gao, DM; Hommel, M; Perret, JE; De Rougemont, J (1991). "Long-term suppression of tremor by chronic stimulation of the ventral intermediate thalamic nucleus". Lancet 337 (8738): 403–6. doi:10.1016/0140-6736(91)91175-T. PMID 1671433. 
  20. ^ ClinicalTrials.gov NCT00430599 The Effect of Levetiracetam (Keppra) on the Treatment of Tremor in Multiple Sclerosis
  21. ^ ClinicalTrials.gov NCT01100073 Kinetic Tremor in Parkinson's Disease: Its Course Under Pramipexole (Mirapexin®) Treatment and Impact on Quality of Life
  22. ^ ClinicalTrials.gov NCT01104649 Efficacy of Riluzole in Hereditary Cerebellar Ataxia
  23. ^ Orrell, Richard W (2005). "Multiple Sclerosis: The History of a Disease". Journal of the Royal Society of Medicine 98 (6): 289. doi:10.1258/jrsm.98.6.289. PMC 1142241. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=1142241. 

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Look at other dictionaries:

  • intention tremor — n a slow tremor of the extremities that increases on attempted voluntary movement and is observed in certain diseases (as multiple sclerosis) of the nervous system * * * see tremor * * * action t …   Medical dictionary

  • intention tremor — see tremor …   The new mediacal dictionary

  • intention tremor — noun : a slow tremor of the extremities that increases on attempted voluntary movement and is observed in certain diseases (as multiple sclerosis) of the nervous system …   Useful english dictionary

  • intention tremor — noun a trembling of a body part when attempting a precise movement …   English new terms dictionary

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  • tremor — n. a rhythmical and alternating movement that may affect any part of the body. The physiological tremor is a feature of the normal mechanism for maintaining posture. It may be more apparent in states of fatigue or anxiety or when the thyroid… …   The new mediacal dictionary

  • intention — n. 1 (often foll. by to + infin., or of + verbal noun) a thing intended; an aim or purpose (it was not his intention to interfere; have no intention of staying). 2 the act of intending (done without intention). 3 colloq. (usu. in pl.) a person s …   Useful english dictionary

  • Дрожание Интенционное (Intention Tremor) — см. Тремор. Источник: Медицинский словарь …   Медицинские термины

  • Tremor — Als Tremor (lat. tremere „zittern“) wird das unwillkürliche, sich rhythmisch wiederholende Zusammenziehen einander entgegenwirkender Muskelgruppen bezeichnet. Der sogenannte physiologische Tremor von Gesunden ist messbar, allerdings kaum sichtbar …   Deutsch Wikipedia

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