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Ailmemts & Remedies

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

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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

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Ailmemts & Remedies

Leigh’s disease

Alternative Name :Subacute Necrotizing Encephalomyelopathy (SNEM)

Definition:
Leigh’s disease is a rare neurometabolic disorder that affects the central nervous system.  This progressive disorder begins in infants between the ages of three months and two years. Rarely, it occurs in teenagers and adults. Leigh’s disease can be caused by mutations in mitochondrial DNA or by deficiencies of an enzyme called pyruvate dehydrogenase. Symptoms of Leigh’s disease usually progress rapidly. The earliest signs may be poor sucking ability,and the loss of head control and motor skills.These symptoms may be accompanied by loss of appetite, vomiting, irritability, continuous crying, and seizures. As the disorder progresses, symptoms may also include generalized weakness, lack of muscle tone, and episodes of lactic acidosis, which can lead to impairment of respiratory and kidney function.
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In Leigh’s disease, genetic mutations in mitochondrial DNA interfere with the energy sources that run cells in an area of the brain that plays a role in motor movements. The primary function of mitochondria is to convert the energy in glucose and fatty acids into a substance called adenosine triphosphate ( ATP). The energy in ATP drives virtually all of a cell’s metabolic functions. Genetic mutations in mitochondrial DNA, therefore, result in a chronic lack of energy in these cells, which in turn affects the central nervous system and causes progressive degeneration of motor functions.
There is also a form of Leigh’s disease (called X-linked Leigh’s disease) which is the result of mutations in a gene that produces another group of substances that are important for cell metabolism. This gene is only found on the X chromosome.

It is named after Denis Archibald Leigh, a British psychiatrist who first described the condition in 1951

Symptoms:
The symptoms of Leigh’s disease usually begin between the ages of 3 months and 2 years. Since the disease affects the central nervous system, symptoms may include:

•poor sucking ability
•difficulty holding up the head
•losing motor skills the infant had such as grasping a rattle and shaking it
•loss of appetite
•vomiting
•irritability
•continuous crying
•seizures
As Leigh’s disease becomes worse over time, the symptoms may include:
•generalized weakness
•lack of muscle tone (hypotonia)
•episodes of lactic acidosis (accumulation of lactic acid in the body and brain) that may impair breathing and kidney function
•heart problems

Causes:
It is an inherited disorder that usually affects infants between the age of three months and two years, but, in rare cases, teenagers and adults as well. In the case of the disease, mutations in mitochondrial DNA (mtDNA) or in nuclear DNA (gene SURF1  and some COX assembly factors) cause degradation of motor skills and eventually death.

Mitochondria are an essential organelle in eukaryotic cells. Their function is to convert the potential energy of glucose, amino acids, and fatty acids into adenosine triphosphate (ATP). Mitochondria carry their own DNA, called mitochondrial DNA [mtDNA]. The information stored in the mtDNA is used to produce several of the enzymes essential to the production of ATP.

Mutations in the mtDNA that cause the mitochondria to fail, to function improperly, a person is at risk for a number of disorders, including Leigh’s disease. In the case of Leigh’s disease, crucial cells in the brain stem have mutated mtDNA, creating poorly functioning mitochondria. This causes a chronic lack of energy in the cells, which, in turn, affects the central nervous system and inhibits motor functions.

Diagnosis:
Diagnosis of Leigh’s disease is based on the symptoms the infant or child has. Tests may show a deficiency of pyruvate dehydrogenase or the presence of lactic acidosis. Individuals with Leigh’s disease may have symmetrical patches of damage in the brain that may be discovered by brain scan. In some individuals, genetic testing may be able to identify the presence of a genetic mutation.

Treatment:
Leigh’s disease is a extremely rare disorder, and there is currently no cure, nor effective treatment. It usually affects infants under two years of age, but, in rarer cases, teenagers and adults as well. A high-fat, low-carbohydrate diet may be recommended. Adults may have puffiness and/or swelling of the eye area and the hands. It is currently treated with thiamin (vitamin B1), but even with treatment, infants rarely live longer than two or three years after the onset of the disease. In cases of older people, the disease takes longer, but is still almost always fatal.

Drug treatments may be needed for epilepsy, movement problems, and cardiac or renal complications.

Prognosis:
The prognosis for individuals with Leigh’s disease is poor. Individuals who lack mitochondrial complex IV activity and those with pyruvate dehydrogenase deficiency tend to have the worst prognosis and die within a few years. Those with partial deficiencies have a better prognosis, and may live to be 6 or 7 years of age. Some have survived to their mid-teenage years.

Resrarch:
The NINDS supports and encourages a broad range of basic and clinical research on neurogenetic disorders such as Leigh’s disease. The goal of this research is to understand what causes these disorders and then to apply these findings to new ways to diagnose, treat, and prevent them.

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/leigh1.shtml
http://rarediseases.about.com/od/mitochondrialdiseases/a/leighsdisease.htm
http://www.ninds.nih.gov/disorders/leighsdisease/leighsdisease.htm
http://en.wikipedia.org/wiki/Leigh’s_disease
http://baby-braden.blogspot.com/2008/10/diagnosis-leighs-disease.html

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Ailmemts & Remedies

Guillain-Barre syndrome

Definition:
Guillain-Barre syndrome is an uncommon disorder in which your body’s immune system attacks your nerves. Weakness and numbness in your extremities are usually the first symptoms. These sensations can quickly spread, eventually paralyzing your whole body.

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The exact cause of Guillain-Barre syndrome is unknown, but it is often preceded by an infectious illness such as a respiratory infection or the stomach flu. Luckily, Guillain-Barre syndrome is relatively rare, affecting only 1 or 2 people per 100,000.

In its most severe form, Guillain-Barre syndrome is a medical emergency requiring hospitalization. There’s no known cure for Guillain-Barre syndrome, but several treatments can ease symptoms and reduce the duration of the illness.

GBS can cause symptoms that last for a few weeks. Most people recover fully from GBS, but some people have permanent nerve damage. In very rare cases, people have died of GBS, usually from difficulty breathing. In the United States, for example, an estimated 3,000 to 6,000 people develop GBS each year on average, whether or not they received a vaccination.

Guillain-Barre affects about 1,500 people every year in the UK, and about 150 develop CIDP. The exact mechanisms that cause the conditions aren’t clear, but about 60 per cent of those affected will have had a throat or intestinal infection, flu or major stress within the previous two weeks. This triggers the immune system, which then attacks the nerves.

It rarely occurs in first-degree relatives, but familial cases have been reported and genetic similarities noted. For example, a study of Japanese people with Guillain-Barre following an intestinal infection with the bacteria Campylobacter jejuni found they were more likely to have a rare version of the gene for an immune system chemical known as tumour necrosis factor.

Symptoms:
Guillain-Barre syndrome often begins with weakness, tingling or loss of sensation starting in your feet and legs and spreading to your upper body and arms. These symptoms may begin — often not causing much notice — in your fingers and toes. In some people, symptoms begin in the arms or even the face. As the disorder progresses, muscle weakness can evolve into paralysis.

Signs and symptoms of Guillain-Barre syndrome may include:

*Prickling, “pins and needles” sensations in your fingers, toes or both
*Weakness or tingling sensations in your legs that spread to your upper body
*Unsteady walking or inability to walk
*Difficulty with eye movement, facial movement, speaking, chewing or swallowing
*Severe pain in your lower back
*Difficulty with bladder control or intestinal functions
*Very slow heart rate or low blood pressure
*Difficulty breathing
.
Most people with Guillain-Barre syndrome experience their most significant weakness within three weeks after symptoms begin. In some cases, signs and symptoms may progress very rapidly with complete paralysis of legs, arms and breathing muscles over the course of a few hours.

The disorder was first described by the French physician Jean Landry in 1859. In 1916, Georges Guillain, Jean Alexandre Barré, and André Strohl diagnosed two soldiers with the illness and discovered the key diagnostic abnormality of increased spinal fluid protein production, but normal cell count.

GBS is also known as acute idiopathic polyradiculoneuritis, acute idiopathic polyneuritis, French polio, Landry’s ascending paralysis and Landry Guillain Barré syndrome.

Canadian neurologist C. Miller Fisher described the variant that bears his name in 1956

Causes:
Many things can cause GBS; about two-thirds of people who develop GBS symptoms do so several days or weeks after they have been sick with diarrhea or a respiratory illness. Infection with the bacterium Campylobacter jejuni is one of the most common risk factors for GBS. People also can develop GBS after having the flu or other infections (such as cytomegalovirus and Epstein Barr virus). On very rare occasions, they may develop GBS in the days or weeks after getting a vaccination.

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Typically, Guillain-Barre develops as an autoimmune reaction following an acute infection. It’s not inherited, although it’s thought that genetic factors may make some people more likely to develop autoimmune conditions.

Risk Factors:
Anyone can develop GBS; however, it is more common among older adults. The incidence of GBS increases with age, and people older than 50 years are at greatest risk for developing GBS.

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Guillain-Barre may be triggered by:

*Most commonly, infection with campylobacter, a type of bacteria often found in undercooked food, especially poultry
*Surgery
*Epstein-Barr virus
*Hodgkin’s disease
*Mononucleosis
*HIV, the virus that causes AIDS
*Rarely, rabies or influenza immunizations

Diagnosis:
The diagnosis of GBS usually depends on findings such as rapid development of muscle paralysis, areflexia, absence of fever, and a likely inciting event. Cerebrospinal fluid analysis (through a lumbar spinal puncture) and electrodiagnostic tests of nerves and muscles (such as nerve conduction studies) are common tests ordered in the diagnosis of GBS.

*cerebrospinal fluid:
Typical CSF findings include albumino-cytological dissociation. As opposed to infectious causes, this is an elevated protein level (100–1000 mg/dL), without an accompanying increased cell count pleocytosis. A sustained increased white blood cell count may indicate an alternative diagnosis such as infection.

.
*Electrodiagnostics
Electromyography (EMG) and nerve conduction study (NCS) may show prolonged distal latencies, conduction slowing, conduction block, and temporal dispersion of compound action potential in demyelinating cases. In primary axonal damage, the findings include reduced amplitude of the action potentials without conduction slowing.

Diagnostic criteria Required:

*Progressive, relatively symmetrical weakness of two or more limbs due to neuropathy
*Areflexia
*Disorder course < 4 weeks
*Exclusion of other causes (see below)

.
Supportive:
*relatively symmetric weakness accompanied by numbness and/or tingling
*mild sensory involvement
*facial nerve or other cranial nerve involvement
*absence of fever
*typical CSF findings obtained from lumbar puncture
*electrophysiologic evidence of demyelination from electromyogram

.
Differential diagnosis:
*acute myelopathies with chronic back pain and sphincter dysfunction
*botulism with early loss of pupillary reactivity and descending paralysis
*diphtheria with early oropharyngeal dysfunction
*Lyme disease polyradiculitis and other tick-borne paralyses
*porphyria with abdominal pain, seizures, psychosis
*vasculitis neuropathy
*poliomyelitis with fever and meningeal signs
*CMV polyradiculitis in immunocompromised patients
*critical illness neuropathy
*myasthenia gravis
*poisonings with organophosphate, poison hemlock, thallium, or arsenic
*intoxication with Karwinskia humboldtiana leaves or seeds
*paresis caused by West Nile virus
*spinal astrocytoma
*motor neurone disease
*West Nile virus can cause severe, potentially fatal neurological illnesses, which include encephalitis, meningitis, Guillain-Barré syndrome, and anterior myelitis.

Treatment :
Supportive care with monitoring of all vital functions is the cornerstone of successful management in the acute patient. Of greatest concern is respiratory failure due to paralysis of the diaphragm. Early intubation should be considered in any patient with a vital capacity (VC) <20 ml/kg, a negative inspiratory force (NIF) that is less negative (i.e., closer to zero) than -25 cmH2O, more than 30% decrease in either VC or NIF within 24 hours, rapid progression of disorder, or autonomic instability.

Once the patient is stabilized, treatment of the underlying condition should be initiated as soon as possible. Either high-dose intravenous immunoglobulins (IVIg) at 400 mg/kg for 5 days or plasmapheresis can be administered, as they are equally effective and a combination of the two is not significantly better than either alone. Therapy is no longer effective two weeks after the first motor symptoms appear, so treatment should be instituted as soon as possible. IVIg is usually used first because of its ease of administration and safety profile, with a total of five daily infusions for a total dose of 2 g/kg body weight (400 mg/kg each day). The use of intravenous immunoglobulins is not without risk, occasionally causing hepatitis, or in rare cases, renal failure if used for longer than five days. Glucocorticoids have not been found to be effective in GBS. If plasmapheresis is chosen, a dose of 40-50 mL/kg plasma exchange (PE) can be administered four times over a week.

Following the acute phase, the patient may also need rehabilitation to regain lost functions. This treatment will focus on improving ADL (activities of daily living) functions such as brushing teeth, washing, and getting dressed. Depending on the local structuring on health care, a team of different therapists and nurses will be established according to patient needs. An occupational therapist can offer equipment (such as wheelchair and special cutlery) to help the patient achieve ADL independence. A physiotherapist would plan a progressive training program and guide the patient to correct functional movement, avoiding harmful compensations which might have a negative effect in the long run. There is also some evidence supporting physiotherapy in helping patients with Guillain–Barré syndrome regain strength, endurance, and gait quality,[23] as well as helping them prevent contractures, bedsores, and cardiopulmonary difficulties. A speech and language therapist would be essential in the patient regaining speaking and swallowing ability if they were intubated and received a tracheostomy. The speech and language therapist would also offer advice to the medical team regarding the swallowing abilities of the patient and would help the patient regain their communication ability pre-dysarthria. There would also be a doctor, nurse and other team members involved, depending on the needs of the patient. This team contribute their knowledge to guide the patient towards his or her goals, and it is important that all goals set by the separate team members are relevant for the patient’s own priorities. After rehabilitation the patient should be able to function in his or her own home and attend necessary training as needed.

Prognosis:
Most of the time recovery starts after the fourth week from the onset of the disorder. Approximately 80% of patients have a complete recovery within a few months to a year, although minor findings may persist, such as areflexia. About 5–10% recover with severe disability, with most of such cases involving severe proximal motor and sensory axonal damage with inability of axonal regeneration. However, this is a grave disorder and despite all improvements in treatment and supportive care, the death rate among patients with this disorder is still about 2–3% even in the best intensive care units. Worldwide, the death rate runs slightly higher (4%), mostly from a lack of availability of life support equipment during the lengthy plateau lasting four to six weeks, and in some cases up to one year, when a ventilator is needed in the worst cases. About 5–10% of patients have one or more late relapses, in which case they are then classified as having chronic inflammatory demyelinating polyneuropathy (CIDP).

Poor prognostic factors include: 1) age, over 40 years, 2) history of preceding diarrheal illness, 3) requiring ventilator support, 4) high anti-GM1 titre and 5) poor upper limb muscle strength

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/guillainbarre1.shtml
http://www.riversideonline.com/health_reference/Nervous-System/DS00413.cfm
http://en.wikipedia.org/wiki/Guillain%E2%80%93Barr%C3%A9_syndrome
http://www.cdc.gov/flu/protect/vaccine/guillainbarre.htm

Guillain-Barré Syndrome

http://nursingcomments.com/tag/guillain-barre-syndrome/

http://www.ami20.com/tag/guillain-barre

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Ailmemts & Remedies

Acoustic neuroma

Other Names : Acoustic neurilemmoma, Acoustic neurinoma, Auditory tumor, Vestibular schwannoma


Definition:

Acoustic neuroma is a non-cancerous tumor that develops on the nerve that connects the ear to the brain.  The neuroma actually arises from cells called Schwann cells that cover the nerve, rather than from the nerve itself, and is therefore correctly called a vestibular schwannoma.

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The tumor usually grows slowly. As it grows, it presses against the hearing and balance nerves. At first, you may have no symptoms or mild symptoms. They can include

*Loss of hearing on one side
*Ringing in ears
*Dizziness and balance problems

Acoustic neuroma can be difficult to diagnose, because the symptoms are similar to those of middle ear problems. Ear exams, hearing tests and scans can show if you have it.

If the tumor stays small, you may only need to have it checked regularly. If you do need treatment, surgery and radiation are options. If the tumors affect both hearing nerves, it is often because of a genetic disorder called neurofibromatosis. The tumor can also eventually cause numbness or paralysis of the face. If it grows large enough, it can press against the brain, becoming life-threatening.


Symptoms
:
Invariably the acoustic neuroma develops only on one side of the head, causing symptoms to occur in that ear. These may include:

*Ringing (tinnitus) in the affected ear
*Vertigo
*Headaches, facial numbness, deterioration of sight and loss of co-ordination are late symptoms
*Hearing loss, usually gradual — although in some cases sudden — and occurring on only one side or more pronounced on one side
*Unsteadiness, loss of balance
*Dizziness (vertigo)
*Facial numbness and weakness

In rare cases, an acoustic neuroma may grow large enough to compress the brainstem and be life-threatening.

Causes:

The cause of acoustic neuromas — tumors on the main nerve leading from your inner ear to your brain (vestibulocochlear nerve) — appears to be a malfunctioning gene on chromosome 22. Normally, this gene produces a protein that helps control the growth of Schwann cells covering the nerves. What makes this gene malfunction isn’t clear. Scientists do know the faulty gene is inherited in about half the cases of neurofibromatosis 2, a rare disorder that typically involves the growth of tumors on the vestibulocochlear nerve on each side of the head (bilateral neuromas).

Most people are between the ages of 40 and 60 when an acoustic neuroma is discovered but why they develop one in the first place is unclear.

Acoustic neuromas may occur sporadically (meaning the cause is unknown), or in some cases occur as part of von Recklinhausen neurofibromatosis, in which case the neuroma may take on one of two forms.

In Neurofibromatosis type I, a schwannoma may sporadically involve the 8th nerve, usually in adult life, but may involve any other cranial nerve or the spinal root. Bilateral acoustic neuromas are rare in this type.
In Neurofibromatosis type II, bilateral acoustic neuromas are the hallmark and typically present before the age of 21. These tumors tend to involve the entire extent of the nerve and show a strong autosomal dominant inheritance. Incidence is about 5 to 10%.

The usual tumor in the adult presents as a solitary tumor, originating in the nerve. It usually arises from the vestibular portion of the 8th nerve, just within the internal auditory canal. As the tumor grows, it usually extends into the posterior fossa to occupy the angle between the cerebellum and the pons (cerebellopontine angle). Because of its position, it may also compress the 5th, 7th, and less often, the 9th and 10th cranial nerves. Later, it may compress the pons and lateral medulla, causing obstruction of the cerebrospinal fluid and increased intracranial pressure.

Schwannomas can occur in relation to other cranial nerves or spinal nerve roots, resulting in radiculopathy or spinal cord compression. Trigeminal neuromas are the second most common form of schwannomas involving cranial nerves. Schwannomas of other cranial nerves are very rare.

Diagnosis:
Signs and symptoms of acoustic neuroma are likely to develop gradually and because hearing loss, tinnitus and problems with balance can be indicators of other middle and inner ear problems, it may be difficult for your doctor to detect the tumor in its early stages. Acoustic neuromas often are found during screening for other conditions.

After asking questions about your symptoms, your doctor will conduct an ear exam and may request the following tests:

*Hearing test (audiometry). During this test conducted by a hearing specialist (audiologist), you wear earphones and hear sounds directed to one ear at a time. The audiologist presents a range of sounds of various tones and asks you to indicate each time you hear the sound. Each tone is repeated at faint levels to find out when you can barely hear. The audiologist will also present various words to determine your hearing ability.

*Brainstem auditory evoked response (BAER). This test checks hearing and neurological functions. Electrodes on your scalp and earlobes capture your brain’s responses to clicking noises you hear through earphones and record the responses on a graph.

*Electronystagmography (ENG). This test evaluates balance (vestibular) function by detecting abnormal rhythmic eye movement (nystagmus) often present with inner ear conditions. The test measures your involuntary eye movements while stressing your balance in various ways.

*Scans. Magnetic resonance imaging (MRI) or computerized tomography (CT) scans of your head can provide images that confirm the presence of an acoustic neuroma.

Treatment :
Acoustic neuroma is a non-cancerous growth, which means it won’t spread to and damage other parts of the body. But it can continue to grow where it is, inside the skull.

It’s important to have it removed because although it grows slowly it can press on the nerves and part of the brain, causing permanent damage. This may result in hearing loss, poor balance and coordination, weakness in the muscles of the face and pain.

When a neuroma is suspected, diagnosis can be confirmed using a CT (computerised tomography) or MRI (magnetic resonance imaging) scan. These can also show the size and position of the tumour.

Most acoustic neuromas are surgically removed, after which many of the symptoms should disappear. This is more likely to be the case when the neuroma is small. Larger neuromas may have done irreversible damage to the brain and nerves before or during surgery.

Many patients have already lost a significant amount of hearing prior to surgery and this is not something that can be reversed although 40 per cent of patients who had tinnitus (ringing in the ears) noticed an improvement in that symptom after surgery.

This is why it’s best to treat an acoustic neuroma sooner rather than later. However, because they’re slow growing, only 1-2mm a year, very small neuromas may initially be just carefully monitored.

Stereotactic Radiotherapy (‘gamma knife’) may also be used to treat an acoustic neuroma.

Risk Factors:
The only known risk factor for acoustic neuroma is having a parent with the rare genetic disorder neurofibromatosis 2, but this accounts for only a minority of cases. A hallmark characteristic of neurofibromatosis 2 is the development of benign tumors on the acoustic nerves on both sides of your head, as well as on other nerves.

Neurofibromatosis 2 is known as an autosomal dominant disorder, meaning the mutation occurs on a nonsex chromosome (autosome) and can be passed on by just one parent (dominant gene). Each child of an affected parent has a 50-50 chance of inheriting it.

Other possible but unconfirmed risk factors for acoustic neuroma include:

*Exposure to loud noise
*Childhood exposure to low-dose radiation of the head and neck
*History of parathyroid adenoma, a benign tumor of the parathyroid glands in the neck
*Heavy use of cellular telephones

Copying & Support:

Dealing with the possibility of hearing loss and facial paralysis and deciding which treatment would be best for you can be quite stressful. Here are some suggestions you may find helpful:

*Educate yourself about acoustic neuroma. The more you know, the better prepared you’ll be to make good choices about treatment. Besides talking to your doctor and your audiologist, you may want to talk to a counselor or medical social worker. Or you may find it helpful to talk to other people who’ve had an acoustic neuroma and learn more about their experiences during treatment and beyond.
*Maintain a strong support system. Family and friends can help you tremendously as you go through this difficult time. Sometimes, though, you may find the concern and understanding of other people with acoustic neuroma especially comforting. Your doctor or a medical social worker may be able to put you in touch with a support group. Or you may find a real or virtual support group through the Acoustic Neuroma Association.

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/acousticneuroma.shtml
http://www.nlm.nih.gov/medlineplus/acousticneuroma.html
http://en.wikipedia.org/wiki/Vestibular_schwannoma
http://www.mayoclinic.com/health/acoustic-neuroma/DS00803

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Mild Memory Loss is Not a Part of Normal Aging

Getting older, in and of itself, is not the cause of so-called “senior moments”. A new study found that even these mild memory lapses are caused by the same brain lesions associated with Alzheimer’s disease and other dementias.
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Researchers found that in the last four to five years of life, people’s memory showed a very rapid decline. Pathologic lesions were found to be related to this rapid decline. The preceding years showed a much more gradual decline that was the actual result of normal aging.

According to Newswise:
“… [R]ecognizing that the earliest changes in memory are related to Alzheimer’s pathology can lead to early diagnosis and will be critical information if a treatment is developed that can alter the pathologic course of the disease.”

Resources:
Newswise September 15, 2010
Neurology September 21, 2010; 75(12):1070-8. Epub 2010 Sep 15

Posted By Dr. Mercola | October 07 2010 | 43,083 views

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