FAQ - Myoclonic Cerebellar Dyssynergia
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what causes myoclonic arm jerks?


I have been told something is going wrong in my muscles or brain..they are still investigating. Also have messy speech, and slight foot drop on left. This has been going on for 3 years and getting worse, was first told probably young onset parkinson's
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Myoclonus describes a symptom and generally is not a diagnosis of a disease. It refers to sudden, involuntary jerking of a muscle or group of muscles. Myoclonic twitches or jerks usually are caused by sudden muscle contractions, called positive myoclonus, or by muscle relaxation, called negative myoclonus. Myoclonic jerks may occur alone or in sequence, in a pattern or without pattern. They may occur infrequently or many times each minute. Myoclonus sometimes occurs in response to an external event or when a person attempts to make a movement. The twitching cannot be controlled by the person experiencing it.

In its simplest form, myoclonus consists of a muscle twitch followed by relaxation. A hiccup is an example of this type of myoclonus. Other familiar examples of myoclonus are the jerks or "sleep starts" that some people experience while drifting off to sleep. These simple forms of myoclonus occur in normal, healthy persons and cause no difficulties. When more widespread, myoclonus may involve persistent, shock-like contractions in a group of muscles. In some cases, myoclonus begins in one region of the body and spreads to muscles in other areas. More severe cases of myoclonus can distort movement and severely limit a person's ability to eat, talk, or walk. These types of myoclonus may indicate an underlying disorder in the brain or nerves. .

Action myoclonus is characterized by muscular jerking triggered or intensified by voluntary movement or even the intention to move. It may be made worse by attempts at precise, coordinated movements. Action myoclonus is the most disabling form of myoclonus and can affect the arms, legs, face, and even the voice. This type of myoclonus often is caused by brain damage that results from a lack of oxygen and blood flow to the brain when breathing or heartbeat is temporarily stopped.

Cortical reflex myoclonus is thought to be a type of epilepsy that originates in the cerebral cortex - the outer layer, or "gray matter," of the brain, responsible for much of the information processing that takes place in the brain. In this type of myoclonus, jerks usually involve only a few muscles in one part of the body, but jerks involving many muscles also may occur. Cortical reflex myoclonus can be intensified when patients attempt to move in a certain way or perceive a particular sensation.

Essential myoclonus occurs in the absence of epilepsy or other apparent abnormalities in the brain or nerves. It can occur randomly in people with no family history, but it also can appear among members of the same family, indicating that it sometimes may be an inherited disorder. Essential myoclonus tends to be stable without increasing in severity over time. Some scientists speculate that some forms of essential myoclonus may be a type of epilepsy with no known cause.

Palatal myoclonus is a regular, rhythmic contraction of one or both sides of the rear of the roof of the mouth, called the soft palate. These contractions may be accompanied by myoclonus in other muscles, including those in the face, tongue, throat, and diaphragm. The contractions are very rapid, occurring as often as 150 times a minute, and may persist during sleep. The condition usually appears in adults and can last indefinitely. People with palatal myoclonus usually regard it as a minor problem, although some occasionally complain of a "clicking" sound in the ear, a noise made as the muscles in the soft palate contract.

Progressive myoclonus epilepsy (PME) is a group of diseases characterized by myoclonus, epileptic seizures, and other serious symptoms such as trouble walking or speaking. These rare disorders often get worse over time and sometimes are fatal. Studies have identified at least three forms of PME. Lafora body disease is inherited as an autosomal recessive disorder, meaning that the disease occurs only when a child inherits two copies of a defective gene, one from each parent. Lafora body disease is characterized by myoclonus, epileptic seizures, and dementia (progressive loss of memory and other intellectual functions). A second group of PME diseases belonging to the class of cerebral storage diseases usually involves myoclonus, visual problems, dementia, and dystonia (sustained muscle contractions that cause twisting movements or abnormal postures). Another group of PME disorders in the class of system degenerations often is accompanied by action myoclonus, seizures, and problems with balance and walking. Many of these PME diseases begin in childhood or adolescence.

Reticular reflex myoclonus is thought to be a type of generalized epilepsy that originates in the brainstem, the part of the brain that connects to the spinal cord and controls vital functions such as breathing and heartbeat. Myoclonic jerks usually affect the whole body, with muscles on both sides of the body affected simultaneously. In some people, myoclonic jerks occur in only a part of the body, such as the legs, with all the muscles in that part being involved in each jerk. Reticular reflex myoclonus can be triggered by either a voluntary movement or an external stimulus.

Stimulus-sensitive myoclonus is triggered by a variety of external events, including noise, movement, and light. Surprise may increase the sensitivity of the patient.

Sleep myoclonus occurs during the initial phases of sleep, especially at the moment of dropping off to sleep. Some forms appear to be stimulus-sensitive. Some persons with sleep myoclonus are rarely troubled by, or need treatment for, the condition. However, myoclonus may be a symptom in more complex and disturbing sleep disorders, such as restless legs syndrome, and may require treatment by a doctor.

What are the causes of myoclonus?

Myoclonus may develop in response to infection, head or spinal cord injury, stroke, brain tumors, kidney or liver failure, lipid storage disease, chemical or drug poisoning, or other disorders. Prolonged oxygen deprivation to the brain, called hypoxia, may result in posthypoxic myoclonus. Myoclonus can occur by itself, but most often it is one of several symptoms associated with a wide variety of nervous system disorders. For example, myoclonic jerking may develop in patients with multiple sclerosis, Parkinson's disease, Alzheimer's disease, or Creutzfeldt-Jakob disease. Myoclonic jerks commonly occur in persons with epilepsy, a disorder in which the electrical activity in the brain becomes disordered leading to seizures.

http://www.ninds.nih.gov/disorders/myoclonus/detail_myoclonus.htm

For more info, here a few other sites of interest:
http://www.ninds.nih.gov/
http://www.wemove.org/myo/

Hope some of this info helps! Good luck, hope they are able to diagnose you soon so you can get the proper treatment.  (+ info)

Stem Cells can be used to treat Cerebellar degeneration ..is that true ?


we have a patient from Egypt who has a problems in balance , movement and speak due to cerebellar degeneration , we heard that stem cells are now injected inside patient body to recover the cerebellum

we want to know where is a trusted place to go for that
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To be clear, there has been 30 years worth of stem cell treatment using bone stem cells and repairing bones. With a lot of controversy, embryonic stem cells and the related research were thought to be the answer to many medical problems. But now we know that adult stem cells, indeed our own stem cells (without the controversy) can be as useful as embryonic stem cells. Some treatments are close to release, but most are a long way away.

There are some biotech companies (listed in the first link) that may be able to help, or redirect you to a better source.

For further info, try these sites/articles:

http://www.actionbioscience.org/biotech/pecorino2.html

http://www.pnas.org/content/104/8/2997.full

http://www.sciencedaily.com/releases/2007/03/070314153123.htm  (+ info)

what is the prognosis of malignant cerebellar astrocytoma in a child aging 11 years ??


what is the life expetancy in this case??
is the surgery enough for treatment ??
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With well-differentiated astrocytomas, the symptoms may remain static or progress only slowly over a number of years, with a mean survival of more than 5 years. Eventually, however, patients usually have more rapid clinical deterioration that is generally correlated with the appearance of anaplastic features and more rapid growth of the tumor. The prognosis for patients with glioblastoma is very poor. With current treatment, consisting of resection when feasible together with radiotherapy and chemotherapy, the mean length of survival after diagnosis is only 8 to 10 months; fewer than 10% of patients are alive after 2 years. Survival is substantially shorter in older patients.

-random physician  (+ info)

what is a posterior fossa cyst located in the midline to the cerebellar vermis.?


hi shannon i have been diagnosed with a cyst in that same region if you google the term you will see the explaination. Mine is close to my brain which may or may not be operated on. These are benign sacs of fluid could be either ceribral spinal fluid mixed with mucus Anyhow if doctor's choose not to opporate you can live a normal life. depending on the type of cyst you have mine is a arachinoid cyst it can grow and cause hemmoraging if untreated. Hope i answered your question  (+ info)

What can I do for my Chronic Myoclonic Jerks. I have MS also. I am looking for someone with Chronic Myoclonus.


My spasums are spontanious and can be very intense. I have had these for 12 years. Since being diagnosed with MS. If there is ANYONE out there that can help with ANY ideas please contact me. I am always in pain :( Thank you Mary
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see a doctor. he could offer a lot more help than answers  (+ info)

what does a low cerebellar tonsil indicate?


Dear Susan,

I have located the following information regarding low cerebellar tonsil at http://neurosurgery.ucla.edu/body.cfm?id=109&oTopId=482

A-L / Arnold Chiari Malformation
General Information
Chiari malformation is a general term used to describe a condition when the bottom part of the cerebellum (the “tonsils”) dip down into the upper spinal canal.


(Figure legend: MRI image of the base of the brain showing the cerebellar tonsil (outlined by the six arrowheads) below the base of the skull. Note that there is no open connection between the cerebrospinal fluid (CSF) in the cranial (brain) compartment and the spinal compartment).

Commonly, radiologists describe two different types of Chiari malformation (there are actually four, but the other two types are extremely rare).
Chiari I malformation describes low-lying cerebellar tonsils without other congenital brain malformations.
Chiari II malformation is a complex anomaly with skull, dura, brain, spine and spinal cord manifestations, which usually presents in early childhood or in infancy. This disorder is usually associated with the spinal defect myelomeningocele.
What causes a Chiari malformation

In many cases it is congenital (present at birth). Researchers at UCLA discovered that in most cases the compartment of the brain holding the cerebellum (posterior fossa) is smaller than normal.
If the upper certical vertebra (odontoid process of the second cervical vertebra) ascends into the cranial compartment (basilar invagination), the canal at the junction between the brain and spine can narrow.
In rare instances high pressure in the brain compartment can “squeeze” the cerebellar tonsils downward. The condition may be associated with the following conditions:
Pseudotumor cerebr.
Hydrocephalus (typically due to aqueductal stenosis).
Brain tumors in the cerebellum.
Posterior fossa arachnoid cyst.
In other rare instances, abnormally low pressure in the spinal compartment can “draw” the cerebellar tonsils downward. This can occur in association with the following circumstances:
Lumboperitoneal shunt.
Spontaneous intracranial hypotension.
Symptoms

Pain, especially headache in the back of the head, aggravated by coughing and straining.
Weakness is also prominent, especially in the hands when there is associated syringomyelia.
Other symptoms include neck, arm, and leg pain, numbness, loss of temperature sensation, unsteadiness, double vision, slurred speech, trouble swallowing, vomiting and tinnitus (ringing in the ears).
Diagnosis

MRI is the diagnostic test of choice for Chiari I malformation, since it easily shows the tonsillar herniation as well as syringomyelia, which occurs in 20 percent to 30 percent of cases.
An MRI cerebrospinal fluid (CSF) flow study is often helpful for determining the impact of the Chiari malformation.
CT-myelogram for diagnosis of spinal CSF leaks for cases of suspected intracranial hypotension, or syringomyelia in the absence of Chiari malformation.
Treatment

In general, the surgical aim is to reestablish free flow of cerebrospinal fluid back-and-forth from the brain and spinal compartments. One can think of the cerebellar tonsils as a “cork” that is tightly squeezed into the upper spinal canal “bottle.” Depending upon the anatomy and cause of the Chiari malformation, one or more of the following options can be used:
Posterior fossa decompression.
The aim is to remove the back, lower part of the skull bone, plus the back of the first cervical vertebra. This widens the “bottleneck” in order to allow CSF flow around the “cork.” This decompression is often accompanied by a duraplasty, a procedure to expose the coverings over the cerebellum, thereby also widening the fluid space.
Cerebellar tonsillar reduction.
Using microsurgical techniques, the lower part of the tonsils can be shrunk down in size so as to reduce the size of the “cork.”
Removal of the odontoid (part of the second cervical vertebra) in cases in basilar invagination.
Treatment of the pseudotumor cerebri with medications and/or surgery
Endoscopic treatment of hydrocephalus due to aqueductal stenosis by third ventriculostomy.
Placement of a shunt for the treatment of hydrocephalus.
Surgical repair of spinal CSF leak.
Conversion of lumboperitoneal shunt to a ventriculoperitoneal shunt.
UCLA Neurosurgeons are experts in all of these techniques.

Hope this information is helpful to you.  (+ info)

has anyone heard of Cerebellar Atrophy?


I have been diagnosed with this, Very rare and unstudied that I know of
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here is a site i found. hope it helps  (+ info)

What is Cerebellar syndrone? Just been diagnosed?


http://www.diseasesdatabase.com/ddb2218.htm  (+ info)

will i die from herniation of the cerebellar tonsils?


Do you have the Budd-Chiari defect? If not, it's more probable you'll die in a car crash or from a heart attack than from a very uncommon form of death like that.  (+ info)

Myoclonic Seizures, in a 15 year old?


How can I fake a Myoclonic Seizure?
NOTHING STUPID
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Um. Make noticeable jerks in your body like your arms, usually both arms at once and the head goes down, sometimes jsut one arm jerks.. Can I ask why though? I mean.. They really aren't fun when you can't control them. I hate myoclonic seizures, lol. You can't fake them on an EEG so watch what you do if you hook up to an EEG..  (+ info)

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