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Parkinson’s Disease

Alternative Names : Parkinson disease, Parkinson’s, idiopathic parkinsonism, primary parkinsonism, PD, or paralysis agitans

Definition:
Parkinson’s disease is the second most common neurodegenerative disorder and the most common movement disorder. It is characterized by progressive loss of muscle control, which leads to trembling of the limbs and head while at rest, stiffness, slowness, and impaired balance. As symptoms worsen, it may become difficult to walk, talk, and complete simple tasks.
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Parkinson’s disease is a progressive disorder of the nervous system that affects movement. It develops gradually, often starting with a barely noticeable tremor in just one hand. But while tremor may be the most well-known sign of Parkinson’s disease, the disorder also commonly causes a slowing or freezing of movement. Many people with Parkinson’s disease live long productive lives, whereas others become disabled much more quickly. Premature death is usually due to complications such as falling-related injuries or pneumonia.

Friends and family may notice that your face shows little or no expression and your arms don’t swing when you walk. Speech often becomes soft and mumbling. Parkinson’s symptoms tend to worsen as the disease progresses.

While there is no cure for Parkinson’s disease, many different types of medicines can treat its symptoms. In some cases,  doctor may suggest surgery.

In the United States, about 1 million people are affected by Parkinson’s disease and worldwide about 5 million. Most individuals who develop Parkinson’s disease are 60 years of age or older. Parkinson’s disease occurs in approximately 1% of individuals aged 60 years and in about 4% of those aged 80 years. Since overall life expectancy is rising, the number of individuals with Parkinson’s disease will increase in the future. Adult-onset Parkinson’s disease is most common, but early-onset Parkinson’s disease (onset between 21-40 years), and juvenile-onset Parkinson’s disease (onset before age 21) also exist.

Descriptions of Parkinson’s disease date back as far as 5000 BC. Around that time, an ancient Indian civilization called the disorder Kampavata and treated it with the seeds of a plant containing therapeutic levels of what is today known as levodopa. Parkinson’s disease was named after the British doctor James Parkinson, who in 1817 first described the disorder in great detail as “shaking palsy.”

Symptoms:
The symptoms of Parkinson’s disease can vary from person to person. Early signs may be subtle and can go unnoticed. Symptoms typically begin on one side of the body and usually remain worse on that side even after symptoms begin to affect both sides.

Parkinson’s signs and symptoms may include:

*Tremor. The characteristic shaking associated with Parkinson’s disease often begins in a hand. A back-and-forth rubbing of your thumb and forefinger, known as pill-rolling, is common, and may occur when your hand is at rest. However, not everyone experiences tremors.

*Slowed motion (bradykinesia). Over time, Parkinson’s disease may reduce your ability to initiate voluntary movement. This may make even the simplest tasks difficult and time-consuming. When you walk, your steps may become short and shuffling. Or your feet may freeze to the floor, making it hard to take the first step.

*Rigid muscles. Muscle stiffness can occur in any part of your body. Sometimes the stiffness can be so severe that it limits the range of your movements and causes pain. People may first notice this sign when you no longer swing your arms when you’re walking.

*Impaired posture and balance. Your posture may become stooped as a result of Parkinson’s disease. Balance problems also may occur, although this is usually in the later stages of the disease.

*Loss of automatic movements. Blinking, smiling and swinging your arms when you walk are all unconscious acts that are a normal part of being human. In Parkinson’s disease, these acts tend to be diminished and even lost. Some people may develop a fixed staring expression and unblinking eyes. Others may no longer gesture or seem animated when they speak.

*Speech changes. Many people with Parkinson’s disease have problems with speech. You may speak more softly, rapidly or in a monotone, sometimes slurring or repeating words, or hesitating before speaking.

*Dementia. In the later stages of Parkinson’s disease, some people develop problems with memory and mental clarity. Alzheimer’s drugs appear to alleviate some of these symptoms to a mild degree.

Causes:
The exact cause of Parkinson’s disease is unknown, but several factors appear to play a role, including:

*Genes. Researchers have found specific genetic mutations that likely play a role in Parkinson’s disease. In addition, scientists suspect that many more changes in genes — whether inherited or caused by an environmental exposure — may be responsible for Parkinson’s disease.

*Environmental triggers. Exposure to toxins or certain viruses may trigger Parkinson’s signs and symptoms.In addition, numerous changes are found in the brains of people with Parkinson’s disease. The role of these factors in the development of the disease, if any, isn’t clear, however. These changes include:

*A lack of dopamine. A substance called dopamine acts as a messenger between two brain areas – the substantia nigra and the corpus striatum – to produce smooth, controlled movements. Most of the movement-related symptoms of Parkinson’s disease are caused by a lack of dopamine due to the loss of dopamine-producing cells in the substantia nigra. When the amount of dopamine is too low, communication between the substantia nigra and corpus striatum becomes ineffective, and movement becomes impaired; the greater the loss of dopamine, the worse the movement-related symptoms. Other cells in the brain also degenerate to some degree and may contribute to non-movement related symptoms of Parkinson’s disease.

Although it is well known that lack of dopamine causes the motor symptoms of Parkinson’s disease, it is not clear why the dopamine-producing brain cells deteriorate. Genetic and pathological studies have revealed that various dysfunctional cellular processes, inflammation, and stress can all contribute to cell damage. In addition, abnormal clumps called Lewy bodies, which contain the protein alpha-synuclein, are found in many brain cells of individuals with Parkinson’s disease. The function of these clumps in regards to Parkinson’s disease is not understood. In general, scientists suspect that dopamine loss is due to a combination of genetic and environmental factors.

*Low norepinephrine levels. People with Parkinson’s disease also have damage to the nerve endings that make another important chemical messenger called norepinephrine. Norepinephrine plays a role in regulating the autonomic nervous system, which controls automatic functions, such as blood pressure regulation.

*The presence of Lewy bodies. Unusual protein clumps called Lewy bodies are found in the brains of many people with Parkinson’s disease. How they got there and what type of damage, if any, Lewy bodies might cause is still unknown.

Risk Factors:
Risk factors for Parkinson’s disease are:

*Age : Age is the largest risk factor for the development and progression of Parkinson’s disease. Most people who develop Parkinson’s disease are older than 60 years years of age.Young adults rarely experience Parkinson’s disease. It ordinarily begins in middle or late life, and the risk continues to increase with age.

*Heredity : Having a close relative with Parkinson’s increases the chances that you’ll also develop the disease, A small number of individuals are at increased risk because of a family history of the disorder. Although your risk is still no more than about 4 to 6 percent.

*Sex: Men are more likely to develop Parkinson’s disease than women are.Men are affected about 1.5 to 2 times more often than women.

*Exposure to toxins: Ongoing exposure to herbicides and pesticides puts you at slightly increased risk of Parkinson’s.Head trauma, illness, or exposure to environmental toxins such as pesticides and herbicides may be a risk factor.
Complications:
Parkinson’s disease is often accompanied by these additional problems:

*Depression:  Depression is common in people with Parkinson’s disease. Receiving treatment for depression can make it easier to handle the other challenges of Parkinson’s disease.

*Sleep problems:  People with Parkinson’s disease often have trouble falling asleep and may wake up frequently throughout the night. They may also experience sudden sleep onset, called sleep attacks, during the day.

*Difficulty chewing and swallowing:  The muscles you use to swallow may be affected in the later stages of the disease, making eating more difficult.

*Urinary problems:  Parkinson’s disease may cause either urinary incontinence or urine retention. Certain medications used to treat Parkinson’s also can make it difficult to urinate.

*Constipation: Many people with Parkinson’s disease develop constipation because the digestive tract works more slowly. Constipation may also be a side effect of medications used to treat the disease.

*Sexual dysfunction:  Some people with Parkinson’s disease may notice a decrease in sexual desire. This may stem from a combination of psychological and physical factors, or it may be the result of physical factors alone.Medications for Parkinson’s disease also may cause a number of complications, including involuntary twitching or jerking movements of the arms or legs, hallucinations, sleepiness, and a drop in blood pressure when standing up.

Diagnosis:
A physician will diagnose Parkinson’s disease from the medical history and a neurological examination.  There is no lab test that will clearly identify the disease, but brain scans are sometimes used to rule out disorders that could give rise to similar symptoms. Patients may be given levodopa and resulting relief of motor impairment tends to confirm diagnosis. The finding of Lewy bodies in the midbrain on autopsy is usually considered proof that the patient suffered from Parkinson’s disease. The progress of the illness over time may reveal it is not Parkinson’s disease, and some authorities recommend that the diagnosis be periodically reviewed.

Other causes that can secondarily produce a parkinsonian syndrome are Alzheimer’s disease, multiple cerebral infarction and drug-induced parkinsonism.  Parkinson plus syndromes such as progressive supranuclear palsy and multiple system atrophy must be ruled out.  Anti-Parkinson’s medications are typically less effective at controlling symptoms in Parkinson plus syndromes. Faster progression rates, early cognitive dysfunction or postural instability, minimal tremor or symmetry at onset may indicate a Parkinson plus disease rather than PD itself.  Genetic forms are usually classified as PD, although the terms familial Parkinson’s disease and familial parkinsonism are used for disease entities with an autosomal dominant or recessive pattern of inheritance.

Medical organizations have created diagnostic criteria to ease and standardize the diagnostic process, especially in the early stages of the disease. The most widely known criteria come from the UK Parkinson’s Disease Society Brain Bank and the US National Institute of Neurological Disorders and Stroke. The PD Society Brain Bank criteria require slowness of movement (bradykinesia) plus either rigidity, resting tremor, or postural instability. Other possible causes for these symptoms need to be ruled out. Finally, three or more of the following features are required during onset or evolution: unilateral onset, tremor at rest, progression in time, asymmetry of motor symptoms, response to levodopa for at least five years, clinical course of at least ten years and appearance of dyskinesias induced by the intake of excessive levodopa. Accuracy of diagnostic criteria evaluated at autopsy is 75–90%, with specialists such as neurologists having the highest rates.

Computed tomography (CT) and magnetic resonance imaging (MRI) brain scans of people with PD usually appear normal.  These techniques are nevertheless useful to rule out other diseases that can be secondary causes of parkinsonism, such as basal ganglia tumors, vascular pathology and hydrocephalus.  A specific technique of MRI, diffusion MRI, has been reported to be useful at discriminating between typical and atypical parkinsonism, although its exact diagnostic value is still under investigation. Dopaminergic function in the basal ganglia can be measured with different PET and SPECT radiotracers. Examples are ioflupane (123I) (trade name DaTSCAN) and iometopane (Dopascan) for SPECT or fludeoxyglucose (18F) for PET. A pattern of reduced dopaminergic activity in the basal ganglia can aid in diagnosing PD

Treatment :
There’s no cure for Parkinson’s disease although new research is just starting to suggest that some drugs already used for the condition do have some effect in holding back progression of the disease.

A lot can be done to relieve symptoms, especially in the early stages, by replacing the missing dopamine in the brain. This can be done very effectively with a drug called levodopa – a synthetic chemical that’s converted into dopamine in the brain. However, there can be severe side-effects with prolonged usage.

Because of these problems, doctors usually try to delay using levodopa, especially in younger people. Instead, they use other drugs that boost dopamine activity or mimic its effects, known as dopamine agonists. These drugs also have side-effects and doses have to be carefully tailored to each patient’s needs.

Another option for people with more advanced Parkinson’s is injections of a drug called apomorphine which can ‘rescue’ people from sudden ‘off’ periods (episodes of greatly reduced mobility).

This drug can also be given as a continuous infusion for those with severe movement fluctuations and reduces the dose of levodopa that a person requires.

Occupational therapists and physiotherapists help people manage their condition by assisting with movement and providing advice on how to maintain independence in everyday life. Speech and language therapists help with communication or swallowing difficulties.

Deep brain stimulation is a form of surgery that can be used to treat some of the symptoms of Parkinson’s. A wire with four electrodes at its tip is implanted in one of four target sites in the brain. Then a small unit, which generates electrical signals for the stimulation, is implanted into the person’s chest. When the stimulation is switched on, electrical signals are sent to the brain to stop or reduce the symptoms of Parkinson’s. It’s not suitable for everyone with Parkinson’s, but can provide significant improvement in symptoms and quality of life.

In the future, gene therapy and stem cell therapy may hold some possibility of more effective treatment of Parkinson’s disease.

YOU MAY CLICK & SEE  : Parkinson’s disease ‘may start in gut’

Lifestyle and home remedies:
If you’ve received a diagnosis of Parkinson’s disease, you’ll need to work closely with your doctor to find a treatment plan that offers you the greatest relief from symptoms with the fewest side effects. Certain lifestyle changes also may help make living with Parkinson’s disease easier.

Healthy eating
Eat a nutritionally balanced diet that contains plenty of fruits, vegetables and whole grains. These foods are high in fiber, which is important for helping prevent the constipation that is common in Parkinson’s disease. A balanced diet also provides nutrients, such as omega-3 fatty acids, that may be beneficial for people with Parkinson’s disease.

If you take a fiber supplement, such as psyllium powder, Metamucil or Citrucel, be sure to introduce it gradually and drink plenty of fluids daily. Otherwise, your constipation may become worse. If you find that fiber helps your symptoms, use it on a regular basis for the best results.

Walking with care
Parkinson’s disease can disturb your sense of balance, making it difficult to walk with a normal gait.

These suggestions may help:

*Try not to move too quickly.
*Aim for your heel to strike the floor first when you’re walking.
*If you notice yourself shuffling, stop and check your posture. It’s best to stand up straight.

Avoiding falls
In the later stages of the disease, you may fall more easily. In fact, you may be thrown off balance by just a small push or bump.

The following suggestions may help:

*Don’t pivot your body over your feet while turning. Instead, make a U-turn.
*Don’t lean or reach. Keep your center of gravity over your feet.
*Don’t carry things while walking.
*Avoid walking backward.

Dressing
Dressing can be the most frustrating of all activities for someone with Parkinson’s disease. The loss of fine motor control makes it hard to button and zip clothes, and even to step into a pair of pants. An occupational therapist can point out techniques that make daily activities easier.

These suggestions also may help:

*Allow plenty of time so that you don’t feel rushed.
*Lay clothes nearby.
*Choose clothes that you can slip on easily, such as sweat pants, simple dresses or pants with elastic waistbands.
*Use fabric fasteners, such as Velcro, instead of buttons.

Alternative Medications:
Forms of alternative medicine that may help people with Parkinson’s include:

*Coenzyme Q10. People with Parkinson’s disease tend to have low levels of coenzyme Q10, and some research has suggested it may be beneficial. However, subsequent research hasn’t confirmed this benefit. You can buy coenzyme Q10 without a prescription in drugstores and natural food stores. Talk with your doctor before taking this supplement to ensure that it won’t interfere with any medication you may be taking.

*Massage. Massage therapy can reduce muscle tension and promote relaxation, which may be especially helpful to people experiencing muscle rigidity associated with Parkinson’s disease. These services, however, are rarely covered by health insurance.

*Tai chi. An ancient form of Chinese exercise, tai chi employs slow, flowing motions that help improve flexibility and balance. Several forms of tai chi are tailored for people of any age or physical condition.

*Yoga. Yoga is another type of exercise that increases flexibility and balance. Most poses can be modified, depending on your physical abilities.

Prognosis:
PD invariably progresses with time. Motor symptoms, if not treated, advance aggressively in the early stages of the disease and more slowly later. Untreated, individuals are expected to lose independent ambulation after an average of eight years and be bedridden after ten years.  However, it is uncommon to find untreated people nowadays. Medication has improved the prognosis of motor symptoms, while at the same time it is a new source of disability because of the undesired effects of levodopa after years of use.   In people taking levodopa, the progression time of symptoms to a stage of high dependency from caregivers may be over 15 years.  However, it is hard to predict what course the disease will take for a given individual. Age is the best predictor of disease progression. The rate of motor decline is greater in those with less impairment at the time of diagnosis, while cognitive impairment is more frequent in those who are over 70 years of age at symptom onset.

Since current therapies improve motor symptoms, disability at present is mainly related to non-motor features of the disease.Nevertheless, the relationship between disease progression and disability is not linear. Disability is initially related to motor symptoms. As the disease advances, disability is more related to motor symptoms that do not respond adequately to medication, such as swallowing/speech difficulties, and gait/balance problems; and also to motor complications, which appear in up to 50% of individuals after 5 years of levodopa usage. Finally, after ten years most people with the disease have autonomic disturbances, sleep problems, mood alterations and cognitive decline. All of these symptoms, especially cognitive decline, greatly increase disability.

The life expectancy of people with PD is reduced. Mortality ratios are around twice those of unaffected people. Cognitive decline and dementia, old age at onset, a more advanced disease state and presence of swallowing problems are all mortality risk factors. On the other hand a disease pattern mainly characterized by tremor as opposed to rigidity predicts an improved survival. Death from aspiration pneumonia is twice as common in individuals with PD as in the healthy population

Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose

Resources:
http://www.bbc.co.uk/health/physical_health/conditions/parkinsons1.shtml
http://en.wikipedia.org/wiki/Parkinson’s_disease
http://www.medicinenet.com/parkinsons_disease/article.htm
http://en.wikipedia.org/wiki/Parkinson’s_disease
http://www.mayoclinic.com/health/parkinsons-disease/DS00295

Essential Tremor

Alternative Names:  Tremor – essential; Familial tremor; Tremor – familial

Definition:
Essential tremor is a disorder of the nervous system that causes a rhythmic shaking. Essential tremor (ET) goes by many names (benign essential tremor and familial tremor being two others), but regardless of the name it is characterized by a shaking in primarily the hands and arms, but it can be found in the jaw and throat and even more rarely the legs. ET is a widely varying disease that can affect many body parts and can vary in its intensity. Some people have a minor tremor in their hands, while others will have a highly noticeable tremor that affects the afflicted’s quality of life and can be mistaken for Parkinson’s Disease by the lay person. Many diseases have a tremor associated with them, but what sets ET apart from other diseases is its lack of any symptom outside of the tremor. Most people will never be diagnosed as having ET, and even when diagnosed there are few treatments available. Essential tremor is inherited by more people on a year by year basis than any other movement disease, this being because it is a simple dominant autosomal trait……...click & see

click to see the picture

Although usually not a dangerous condition, essential tremor worsens over time and can be severe in some people.Essential tremor can occur at any age but is most common in older adults……....you may click  to read more

Symptoms:
The tremor is usually most obvious in the hands, but may affect the arms, head, eyelids, or other muscles. The tremor rarely affects the legs or feet. People with essential tremor may have trouble holding or using small objects such as silverware or a pen.

The shaking usually involves small, rapid movements — more than 5 times a second.

Specific symptoms may include:

*Begin gradually

*Worsen with movement

*Usually occur in the hands first, affecting one hand or both hands

*Are aggravated by emotional stress, fatigue, caffeine or extremes of temperature

*Head nodding (Can include a “yes-yes” or “no-no” motion of the head)

*Shaking or quivering sound to the voice if the tremor affects the voice box

*Difficulty writing, drawing, drinking from a cup, or using tools if the tremor affects the hands

The tremors may:

*Occur when you move (action-related tremor), and may be less noticeable with rest

*Come and go, but generally get worse as you age

*Get worse with stress, caffeine, and certain medications

*Not affect both sides of the body the same way

Essential tremor vs. Parkinson’s disease

Many people associate tremors with Parkinson’s disease, but the two conditions differ in key ways:

*When tremors occur. Essential tremor of the hands typically occurs when you use your hands. Tremors from Parkinson’s are most prominent when your hands are at your sides or resting in your lap.

*Associated conditions. Essential tremor doesn’t cause other health problems, whereas Parkinson’s is associated with a stooped posture, slow movement and a shuffling gait. However, people with essential tremor may sometimes develop other neurological signs and symptoms — such as an unsteady gait (ataxia).

*Parts of body affected. Essential tremor can involve your hands, head, voice and legs. Tremors from Parkinson’s typically affect your hands but not your head or voice.

Causes:
Essential tremor is the most common type of tremor. In general, tremors occur when there is a problem with the nerves supplying certain muscles. However, everyone has some essential tremor but the movements are usually so small that they can’t be seen.

About half of essential tremor cases appear to occur because of a genetic mutation. This is referred to as familial tremor. What causes essential tremor in people without a known genetic mutation isn’t clear.

Some research suggests that the cerebellum, the part of the brain that controls muscles movements, does not work correctly in patients with essential tremor.

Noticeable essential tremors can be seen at any age but are most common in people older than 65.

Essential tremor can also occur with other neurological conditions, including dystonia, parkinsonism, and certain inherited nerve conditions such as Charcot-Marie-Tooth disease.

If an essential tremor occurs in more than one member of a family, it is called a familial tremor. This type of essential tremor is passed down through families (inherited), which suggests that genes play a role in its cause.

Familial tremor is usually a dominant trait, which means that you only need to get the gene from one parent to develop the disorder. It usually starts in early middle age, but may be seen in people who are older or younger.

Risk Factors:
There are two known risk factors for essential tremor:

*Genetic mutation. The inherited variety of essential tremor is an autosomal dominant disorder, which means that a defective gene from just one parent is needed to pass on the condition. If you have a parent with a genetic mutation for essential tremor, you have a 50 percent chance of developing the disorder yourself.

*Age. Essential tremor is more common in middle age and older.

Complications:
Essential tremor is not life-threatening, but symptoms often worsen over time. If the tremors become severe, you may find it difficult to:

*Hold a cup or glass without spilling

*Eat normally

*Put on makeup or shave

*Talk, if your voice box or tongue is affected

*Write — handwriting may become increasingly large, shaky and illegible

Diagnosis:

Your doctor can make the diagnosis by performing a physical exam and asking questions about your medical and personal history.

A physical exam will show shaking with movement, usually small movements that are faster than 5 times per second. There are usually no problems with coordination or mental function.

Further tests may be needed to rule out other reasons for the tremors. Other causes of tremors may include:

•Alcohol withdrawal
•Cigarette smoking
•Hyperthyroidism
•Pheochromocytoma
•Too much caffeine
•Use of certain medications
•Wilson’s disease
Blood tests and imaging studies (such as a CT scan of the head, brain MRI, and x-rays) are usually normal.
The most common way to diagnose ET is by having a patient draw the Archimedes Spiral, which is shown immediately below.

If there is a shakiness detected while drawing the spiral, ET can be diagnosed assuming there are no neurological or biological reasons to be found. Below is a picture of what the Archimedes Spiral looks like when drawn by a person with a relatively severe tremor:

Treatment:
Treatment may not be necessary unless the tremors interfere with your daily activities or cause embarrassment.

When diagnosed with ET there are several courses of action depending on the severity of the tremor.
Medicines may help relieve symptoms. How well medicines work depend on the individual patient.

Two medications used to treat tremors include:

•Propranolol, a drug that blocks the action of stimulating substances called neurotransmitters, particularly those related to adrenaline
•Primidone, an antiseizure drug that also controls the function of some neurotransmitters
The drugs can have significant side effects.

Side effects of propranolol include:

•Fatigue
•Nose stuffiness
•Shortness of breath (people with asthma should not use this drug)
•Slow heart beat
Side effects of primidone include:

•Drowsiness
•Difficulty concentrating
•Nausea
•Problems with walking, balance, and coordination
Other medications that may reduce tremors include:

•Antiseizure drugs such as gabapentin and topiramate
•Mild tranquilizers such as alprazolam or clonazepam
•Blood pressure drugs called calcium-channel blockers such as flunarizine and nimodipine
Botox injections, given in the hand, have been used to reduce tremors by weakening local muscles.

In severe cases, surgery to implant a stimulating device in the brain may be an option.

Prognosis:

An essential tremor is not a dangerous condition, but some patients find the tremors annoying and embarrassing. In some cases, it may be dramatic enough to interfere with work, eating, or drinking.

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Prevention:
Stress and caffeine can make tremors worse. Avoid caffeinated drinks such as coffee, tea, and soda, and other stimulants. Exercise and counseling to reduce emotional stress may also help.

Alcoholic beverages in small quantities may decrease tremors but can lead to alcohol dependence and alcohol abuse, especially if you have a family history of such problems. How alcohol helps relieve tremors is unknown.


Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose

Resources:
http://wiki.ggc.usg.edu/mediawiki/index.php/Essential_Tremor
http://www.mayoclinic.com/health/essential-tremor/DS00367
http://www.nlm.nih.gov/medlineplus/ency/article/000762.htm

Ataxia

Definition:
Ataxia (from Greek , meaning “lack of order”) is a neurological sign and symptom consisting of gross lack of coordination of muscle movements. Ataxia is a non-specific clinical manifestation implying dysfunction of parts of the nervous system that coordinate movement, such as the cerebellum. Several possible causes exist for these patterns of neurological dysfunction. The term “dystaxia” is rarely used as a synonym.

A sign of an underlying condition, ataxia can affect your movements, your speech, your eye movements and your ability to swallow.
………………….click & see
Persistent ataxia usually results from damage to your cerebellum — the part of your brain that controls muscle coordination. Many conditions may cause ataxia, including alcohol abuse, stroke, tumor, cerebral palsy and multiple sclerosis. It’s also possible to inherit a defective gene that may cause one of many ataxia variants.

Types of ataxia:-

#Cerebellar ataxia:
The term cerebellar ataxia is employed to indicate ataxia due to dysfunction of the cerebellum. This causes a variety of elementary neurological deficits, such as antagonist hypotonia, asynergy, dysmetria, dyschronometria, and dysdiadochokinesia. How and where these abnormalities manifest depend on which cerebellar structures are lesioned, and whether the lesion is bilateral or unilateral.

*Dysfunction of the vestibulocerebellum impairs balance and control of eye movements. This presents with postural instability, in which the person tends to separate the feet on standing to gain a wider base and avoid oscillations (especially posterior-anterior ones); instability is therefore worsened when standing with the feet together (irrespective of whether the eyes are open or closed: this is a negative Romberg’s test, or more accurately, denotes the inability to carry out the test as the individual is unstable even with open eyes).

*Dysfunction of the spinocerebellum presents with a wide-based “drunken sailor” gait, characterised by uncertain start and stop, lateral deviations, and unequal steps. This part of the cerebellum regulates body and limb movements.

*Dysfunction of the cerebrocerebellum presents with disturbances in carrying out voluntary, planned movements, including intention tremor (coarse trembling, accentuated over the execution of voluntary movements, possibly involving the head and eyes as well as the limbs and torso), peculiar writing abnormalities (large, unequal letters, irregular underlining), and a peculiar pattern of dysarthria (slurred speech, sometimes characterised by explosive variations in voice intensity despite a regular rhythm).

#Sensory ataxia:
The term sensory ataxia is employed to indicate ataxia due to loss of proprioception (sensitivity to joint and body part position), which generally depends on dysfunction of the dorsal columns of the spinal cord, since they carry proprioceptive information up to the brain; in some cases, the cause may instead be dysfunction of the various brain parts that receive that information, including the cerebellum, thalamus, and parietal lobes. Sensory ataxia presents with an unsteady “stomping” gait with heavy heel strikes, as well as postural instability that is characteristically worsened when the lack of proprioceptive input cannot be compensated by visual input, such as in poorly lit environments. Doctors can evidence this during physical examination by having the patient stand with his / her feet together and eyes shut, which will cause the patient’s instability to markedly worsen, producing wide oscillations and possibly a fall (this is called a positive Romberg’s test). Worsening of the finger-pointing test with the eyes closed is another feature of sensory ataxia. Also, when the patient is standing with arms and hands extended toward the examiner, if the eyes are closed, the patient’s finger will tend to “fall down” and be restored to the horizontal extended position by sudden extensor contractions (“ataxic hand”).

#Vestibular ataxia:
The term vestibular ataxia is employed to indicate ataxia due to dysfunction of the vestibular system, which in acute and unilateral cases is associated with prominent vertigo, nausea and vomiting. In slow-onset, chronic bilateral cases of vestibular dysfunction, these characteristic manifestations may be absent, and dysequilibrium may be the sole presentation.

Symptoms:-
Symptoms may vary depending on the severity and type of ataxia, of which there are many. If the ataxia is caused by an injury or another health condition, symptoms may emerge at any age, and may well improve and eventually disappear.

Initial ataxia symptoms usually include:
*Poor limb coordination.
*Dysarthria – slurred and slow speech that is difficult to produce. The patient may also have difficulties controlling volume, rhythm, and pitch.

If the ataxia advances other symptoms may also appear:

*Swallowing difficulties, which may sometimes result in choking or coughing.
*Facial expressions become less apparent.
*Tremors – parts of the body may shake or tremble unintentionally.
*Nystagmus – involuntary rapid rhythmic repetitious eye movement. Movements may be vertical, horizontal, or circular.
*Pes cavus – a foot with too high an arch.
*Cold feet – because of a lack of muscle activity.
*Problems with balance.
*Walking difficulties – in severe cases the patient may need a wheelchair.
*Vision problems.
*Hearing problems.
*Depression – as a result of having to live and cope with the symptoms.

Cerebellar ataxias:
Cerebellar ataxia early onset usually emerges between the ages of 4 and 26. Late onset ataxia generally appears after the patient is 20 years old. Late-onset ataxias usually present less severe symptoms, compared to early-onset ataxia.

Ataxia telangiectasia symptoms generally include:
*Small veins appear around the corner of the eyes, cheeks and ears.
*Physical and sexual development is usually delayed.

Friedreich’s ataxia symptoms generally include:
*The spine curves sideways (scoliosis).
*The heart muscle becomes weaker (cardiomyopathy).
*Diabetes.

For patients whose symptoms are caused by injury or illness, symptoms often improve over time, and eventually go away completely.

When to see a doctor:-
If you aren’t aware of having a condition that causes ataxia, such as multiple sclerosis, see your doctor if you:

*Lose balance
*Lose muscle coordination in a hand, arm or leg
*Have difficulty walking
*Slur your speech
*Have difficulty swallowing

Causes:-
The three types of ataxia have overlapping causes, and can therefore either coexist or occur in isolation.

Focal lesions
Any type of focal lesion of the central nervous system (such as stroke, brain tumour, multiple sclerosis) will cause the type of ataxia corresponding to the site of the lesion: cerebellar if in the cerebellum, sensory if in the dorsal spinal cord (and rarely in the thalamus or parietal lobe), vestibular if in the vestibular system (including the vestibular areas of the cerebral cortex).

Exogenous substances
Exogenous substances that cause ataxia mainly do so because they have a depressant effect on central nervous system function. The most common example is ethanol, which is capable of causing reversible cerebellar and vestibular ataxia. Other examples include various prescription drugs (e.g. most antiepileptic drugs have cerebellar ataxia as a possible adverse effect), marijuana ingestion[2] and various other recreational drugs (e.g. ketamine, PCP or dextromethorphan, all of which are NMDA receptor antagonists that produce a dissociative state at high doses).

Vitamin B12 deficiency
Vitamin B12 deficiency may cause, among several neurological abnormalities, overlapping cerebellar and sensory ataxia.

Causes of isolated sensory ataxia

Peripheral neuropathies may cause generalised or localised sensory ataxia (e.g. a limb only) depending on the extent of the neuropathic involvement. Spinal disorders of various types may cause sensory ataxia from the lesioned level below, when they involve the dorsal columns.

Non-hereditary cerebellar degeneration
Non-hereditary causes of cerebellar degeneration include chronic ethanol abuse, paraneoplastic cerebellar degeneration, high altitude cerebral oedema, coeliac disease, normal pressure hydrocephalus and cerebellitis.

Hereditary ataxias
Ataxia may depend on hereditary disorders consisting of degeneration of the cerebellum and/or of the spine; most cases feature both to some extent, and therefore present with overlapping cerebellar and sensory ataxia, even though one is often more evident than the other. Hereditary disorders causing ataxia include autosomal dominant ones such as spinocerebellar ataxia, episodic ataxia, and dentatorubropallidoluysian atrophy, as well as autosomal recessive disorders such as Friedreich’s ataxia (sensory and cerebellar, with the former predominating) and Niemann Pick disease, ataxia-telangiectasia (sensory and cerebellar, with the latter predominating), and abetalipoproteinaemia. An example of X-linked ataxic condition is the rare fragile X-associated tremor/ataxia syndrome.

GeneReview/NIH/UW entry on Hereditary Ataxia Overview

Arnold-Chiari Malformation
Arnold-Chiari malformation is a malformation of the brain. It consists of a downward displacement of the cerebellar tonsils and the medulla through the foramen magnum, sometimes causing hydrocephalus as a result of obstruction of CSF (cerebrospinal fluid) outflow.

Diagnosis:-
Besides conducting a physical exam and a neurological exam, including checking your memory and concentration, vision, hearing, balance, coordination and reflexes, your doctor may request these laboratory tests:

#Blood tests. Certain blood tests can confirm or exclude the suspected condition. A sample of your blood will be drawn from your arm through a needle. You may be required to fast from midnight the night before your appointment until after your blood is drawn. The specific tests will depend on the suspected cause, but most likely will include a complete blood count (CBC), which helps evaluate your overall health and detect a range of disorders, including infection and heavy metal poisoning.

#Urine tests. Examining a sample of your urine under a microscope (urinalysis) may suggest certain systemic abnormalities that can be related to some forms of ataxia. If your doctor suspects Wilson’s disease, you may be asked for a 24-hour urine collection to help determine the amount of copper in your system.

#Imaging studies. A computerized tomography (CT) scan or magnetic resonance imaging (MRI) of your brain may help determine potential causes.

#Genetic testing. Your doctor may recommend genetic testing to determine whether you or your child has the gene mutation that causes one of the hereditary ataxic conditions. Gene tests are available for many but not all of the hereditary ataxias.

Treatments:-
There’s no treatment specifically for ataxia and it is not curable  but a great deal can be done to ease symptoms and improve the quality of life of the patient. Treatment for coordination and balance problems usually involves the use of adaptive devices which help the patient attain as much independence as possible. These may include the use of a cane (walking stick), crutches, walker or a wheelchair. Symptoms such as tremor, stiffness, spasticity, sleep disorders, muscle weakness, depression (or frustration, sadness and anger) may be addressed with targeted physical therapy, speech therapy, medications and counseling.

Occupational therapy – the occupational therapist can help the patient manage better around the house and work. This may involve some home adaptations, wheelchair assessments, and making the kitchen more practical for the patient.

#Speech therapy – the speech therapist can help with swallowing, coughing, choking and speech problems. If speech becomes very difficult the speech therapist can help the patient learn how to use speech aids.

#Orthopedic care – this can help patients with curvature of the spine (scoliosis).

Physical therapy (physiotherapy) – a physical therapist (physiotherapist) can help maintain strength and improve mobility.

#Physical therapy to help you build strength and enhance your mobility
#Occupational therapy to help you with daily living tasks, such as feeding yourself
#Speech therapy to improve speech and aid swallowing

*Counseling – patients with ataxia commonly become frustrated and depressed; this usually results from having to cope with some symptoms which affect physical mobility and coordination. Talking to a well qualified counselor, such as a psychotherapist can help.

*Supplements and nutrition – some patients with ataxia have very low levels of vitamin E and require supplements and/or a special diet. As sensitivity to gluten is more common among ataxia patients, a gluten-free diet also helps.

*Medication – some patients with ataxia telangiectasia are prescribed gamma-globulin injections to boost their immune systems. There are also drugs for muscle spasms and uncontrollable eye movements.

Coping and support:-
The challenges you face when living with ataxia, such as loss of independence, or having a child with the condition, may make you feel alone or lead to depression and anxiety. Talking to a counselor or therapist may lessen your sense of isolation and help you cope. Or you may find encouragement and understanding in a support group, either for ataxia or for your underlying condition, such as cancer or multiple sclerosis.

Although support groups aren’t for everyone, they can be good sources of information. Group members often know about the latest treatments and tend to share their own experiences. If you’re interested, your doctor may be able to recommend a group in your area.

Other uses of the term:-
The term “ataxia” is sometimes used in a broader sense to indicate lack of coordination in some physiological process. Examples include optic ataxia (lack of coordination between visual inputs and hand movements, resulting in inability to reach and grab objects, usually part of Balint’s syndrome, but can be seen in isolation with injuries to the superior parietal lobule, as it represents a disconnection between visual-association cortex and the frontal premotor and motor cortex), and ataxic respiration (lack of coordination in respiratory movements, usually due to dysfunction of the respiratory centres in the medulla oblongata).

You may Click to see:->  Related articles from newspapers, magazines, and more

Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose.

Resources:
http://www.mayoclinic.com/health/ataxia/DS00910
http://en.wikipedia.org/wiki/Ataxia
http://www.medicalnewstoday.com/articles/162368.php

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Tremor (When the muscles refuse to obey)

At 40 plus, just at the peak of a successful career, the sudden onset of tremor can be devastating. Careers nosedive as the young executive, although with intelligence undiminished, is unable to speak lucidly. The handwriting has little spidery spikes and is illegible. The head constantly moves in a side-to-side motion, a  yes-yes no-no  see saw oscillation that sends confused signals to the bemused bystander. Eventually, the involuntary to and fro motion affects other muscle groups in the arms, legs and trunk. Gait is affected and becomes unsteady and lurching. Speech becomes tremulous with an up and down intonation as the vocal cords get affected. Even daily tasks like dressing and eating become difficult to perform. Worse still, typing and computer keyboard coordination become impossible. And once rapid button-pressing skills are compromised, life in the 21st century becomes impracticable.

Parkinsons disease  is the diagnosis that leaps to the mind. However, all tremors are not Parkinsons. Parkinsonism occurs later, around the age of 60 years. The tremor is typical and is described as  pill rolling . The face is mask-like and expressionless.

A young person is more likely to have hereditary essential tremor. This is inherited as an autosomal dominant condition (if one parent has tremor the offspring has a 50 per cent chance of inheriting it). It affects around 0.4-3 per cent of the population (both male and female) around the age of 40 years.

Any malfunction of the areas of the brain that control movement can cause tremor. This can be caused by infectious diseases like meningitis or encephalitis, stroke, traumatic brain injury, tumours and neurodegenerative diseases. Tremor can also be brought on by low blood sugar and a hyper functioning thyroid gland.

However, not all tremors are sinister. Standing for a long time in a particular position may cause the legs to shake. This tremor is normal and disappears if the person sits down.

Sometimes a person may complain of tremor and yet nothing may be grossly visible. This fine physiological or normal tremor can be proved by asking the person to hold a small, lighted torch and focus it on a wall. The light shakes from side to side. This kind of tremor is increased by anxiety and fear but disappears at rest and when the person is calm.

Alcohol can provoke or normalise tremor, depending on whether it is due to excessive consumption or withdrawal.

Tremors caused by an underlying medical condition spontaneously disappear once the condition is removed. Appropriate treatment depends on accurate diagnosis of the cause.

Symptomatic drug therapy is available for several forms of tremor. Parkinsonian tremor can be treated with a combination of levodopa, other dopamine-like drugs and anticholinergic medication. Unfortunately, the response decreases over time so the dosage has to be increased or more drugs added.

Essential tremor may be treated with beta blockers and primidone, an anticonvulsant drug. The response is variable.

Caffeine in coffee, tea and cola drinks, nicotine in cigarettes, and alcohol behave as tremor  triggers . Eliminating them from the diet controls all kinds of tremor.

Sometimes, the tremor can become so uncontrolled that the person expends all his or her energy. Food intake cannot keep pace and the person becomes cachexic and moribund. If the response to medication is also inadequate, surgical intervention may help. These procedures are usually performed only when the tremor is severe and does not respond to drugs.

The thalamus is the part of the brain that is responsible for most tremors. Implantable electrodes can be used to send high-frequency electrical signals to this region. A hand-held magnet can be used to turn on and turn off a pulse generator that is surgically implanted under the skin. This temporarily disables the tremor. The batteries in the generator last about five years and have to be replaced surgically. This procedure can be performed for both Parkinsonian and essential tremors.

If this is not practical, in severe cases the thalamus can be electrically ablated with brain surgery. This permanently cures the tremor without disrupting sensations or voluntary control of the muscles.

Tremor is debilitating and depressing for the patient. The caregiver also has a difficult time trying to cope with the uncoordinated and uncontrolled motor activity of a person whose muscles simply refuse to obey commands. Physical therapy helps to reduce the tremor. A qualified physiotherapist can work with the patient to improve coordination, muscle strength, control and functional skills. Control in a tremulous limb can be regained to some extent by bracing the limb and regularly exercising using weights and splints. Some traditional forms of exercise like yoga and Taichi are also beneficial. They may help to retard the progress of the disease if started in the early stages in conjunction with medication.

Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.

Written by:Dr Gita Mathai is a paediatrician with a family practice at Vellore. Questions on health issues may be emailed to her at yourhealthgm@yahoo.co.in