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Palmar hyperhidrosis

Description:
Palmer hyperhidrosis is profuse perspiration (excessive sweating) of the palms.It is one form of focal hyperhidrosis, meaning profuse perspiration affecting one area of the body. Sweaty palms may be accompanied by profuse perspiration of the feet, forehead, ckeeks, armpits (axillae) or be part of general hyperhidrosis (profuse perspiration throughout the body). Hyperhidrosis refers to profuse perspiration beyond the body’s thermoregulatory (temperature control) needs.

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Palmer  hyperhidrosis is a common condition in which the eccrine (sweat) glands of the palms and soles secrete inappropriately large quantities of sweat. The condition may become socially and professionally debilitating. The condition usually is idiopathic  and  it begins in childhood and frequently runs in families.

Symptoms:
The intensity of symptoms may vary among sufferers and trigger factors should be carefully noted. Common symptoms  are :

*Perspiration of the hands can vary from mild clamminess to severe perspiration resulting in dripping sweat.
*Temperature differences of palmar surface compared to surface temperature of other parts of the body may be noted.
*Sloughing (peeling) of skin may be noted in profuse perspiration.
*Episodes of profuse perspiration may be followed by periods of extreme dryness on the palmar surface.
*Hyperhidrosis often starts in puberty, and family history is often reported.

The secondary effects of palmar hyperhidrosis can result in both psychosocial effects as well as difficulty in undertaking certain tasks or handling equipment. Sufferers of palmar hyperhidrosis are often reluctant to partake in socially expected actions like shaking hands or touching loved ones. The embarrassment of dealing with this condition can affect the level of interactivity in both social and work situations. Difficulties with holding objects, gripping equipment or soiling electronic devices like keyboards may affect functioning at work. Daily activities such as writing with a pen or counting cash notes is often difficult.

Causes:
Hyperhidrosis is either primary focal or secondary generalized.

1. Primary Palmar  Hyperhidrosis

Focal palmar hyperhidrosis is usually localized and is referred to as primary (essential, idiopathic), meaning no obvious cause, except strong family predisposition can be found (4,5), and affected persons are otherwise healthy . Sweating on other locations as feet, armpits and face may appear. Primary palmar hyperhidrosis is caused by overactivity of the sympathetic nervous system, primarily triggered by emotional causes including anxiety, nervousness, anger and fear .

There may be a significant reduction in perspiration during sleep or sedation.

2. Secondary Palmar Hyperhidrosis

In secondary palmar hyperhidrosis hands sweat due to an obvious underlying disorder like:

*Infections including local infections, tuberculosis and tinea ugunium.
*Neurological disorders like peripheral autonomic neuropathy
*Frostbite
*Arteriovenous Fistulas
*Acromegaly
*Acrodynia
*Complex Regional Pain Syndromes
*Pachyonychia Congenita
*Primary Hypertrophic osteoarthropathy
*Dyskeratosis Congenita
*Blue rubber-bleb nevus
*Glomus tumor

*Secondary palmar hyperhidrosis as part of generalized hyperhidrosis due to  several  hormonal causes (diabetes, hyperthyroidism, thyrotoxicosis, menstruation, menopause), metabolic disorders, malignant disease (lymphoma, pheochromocitoma), autoimmune disorders (rheumatoid arthritis, systemic lupus erythrematosus), drugs like hypertensive drugs and certain classes of antidepressants (list of medications causing hyperhidrosis), chronic use of alcohol, Parkinson’s disease, neurological disorders (toxic neuropathy), homocystinuria, plasma cell disorders. Detailed list of conditions causing generalyzed hyperhidrosis.

How Sweat Glands Work:
In eccrine glands, the major substance enabling impulse conduction is acetylcholine, and in apocrine glands, they are catecholamines.

Body temperature is controlled by the thermoregulatory center in the hypothalamus and this is influenced not only by  by core body temperature but also by hormones, pyrogens, exercise and emotions.

Diagnosis:
The first step in diagnosing  the  Palmar  hyperhidrosis is to differentiate between generalized and focal hyperhidrosis.

A thorough case taking and medical history is usually sufficient to diagnose palmar hyperhidrosis and any trigger factors (scheduled drugs, narcotics, chronic alcoholism).

Diagnostic criteria for primary focal (including palmar) hyperhidrosis  are:

*Bilateral and relatively symmetric sweating
*Frequency of at least 1 episode per week
*Impairment of daily activities
*Age at onset before 25 years
*Family history
*Cessation of sweating during sleep

Tests may include:
*Hematological studies may be necessary to identify thyroid disorders (thyroid function test for T3 and T4 as well as thyroid antibodies) and diabetes (fasting blood glucose or a glucose tolerance test).

*X-rays and MRI scans will assist for diagnosing tuberculosis, pneumonia and tumors.

*Superficial electroconductivity can be monitored as any hyperhidrosis reduces skin electrical resistance.

*Thermoregulatory sweat test uses moisture-sensitive indicator powder to monitor moisture. Changes in the color of the powder at room temperature will highlight areas of increased perspiration.

Treatment:
Conservative management should be coupled with prescribed treatment by the Doctor to reduce the symptoms.

*Counseling may be effective in managing primary palmar hyperhidrosis in cases of mental-emotional etiology.

*Trigger foods and aggravating factors should be noted if possible and relevant dietary changes should be implemented.

*Effective prevention of secondary palmar hyperhidrosis is difficult with conservative management and drug therapy or surgery may be required.

*Excessive physical activity and extremes of heat may be two trigger factors that should be avoided as far as possible.

*In cases of diabetes, a glucose controlled diet with low glycemic index may improve glucose tolerance which could assist with palmar hyperhidrosis.

*Abstinence from alcohol and narcotics is advisable if it is the causative factor for sweaty palms.

*Stimulants such as caffeine and nicotine may aggravate palmar hypehidrosis and should relevant dietary and lifestyle changes should be implemented.

*Anti-perspirant compounds like aluminum chloride can be applied on the palms to reduce moisture or palmar surfaces. Recent research on an aluminum sesquichlorohydrate foam has shown that it is effective in reducing sweat in palmar hyperhidrosis

Treatment remains a challenge: options include topical and systemic agents, iontophoresis, and botulinum toxin type A injections, with surgical sympathectomy as a last resort. None of the treatments is without limitations or associated complications. Topical aluminum chloride hexahydrate therapy and iontophoresis are simple, safe, and inexpensive therapies; however, continuous application is required because results are often short-lived, and they may be insufficient. Systemic agents such as anticholinergic drugs are tolerated poorly at the dosages required for efficacy and usually are not an option because of their associated toxicity. While botulinum toxin can be used in treatment-resistant cases, numerous painful injections are required, and effects are limited to a few months.

Standard therapeutic protocol may differ among cases of palmar hyperhidrosis depending on medical history and underlying pathology.

*Anticholinergic drugs have a direct effect on the sympathetic nervous system although there are numerous side effects.

*Treatment should be directed at contributing factors.

*Ionophoresis involves the use of electrotherapeutic measures to reduce the activity of sweat glands.

*Botulinum injections at the affected area may be useful for its anticholinergic effects.

*Surgery should be considered if drug therapy proves ineffective. Endoscopic transthoracic sympathectomy involves resection of the sympathetic nerve supply to the affected area. This prevents nerve stimulation of the sweat gland of the palms. However surgery has a host of complications including exacerbating the problem or increasing generalized hyperhidrosis.

Surgical sympathectomy should be reserved for the most severe cases and should be performed only after all other treatments have failed. Although the safety and reliability of treatments for palmoplantar hyperhidrosis have improved dramatically, side effects and compensatory sweating are still common, potentially severe problems.

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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.aafp.org/afp/2004/0301/p1117.html

Causes and Treatment of Palmar Hyperhidrosis – Sweaty Palms/Hands

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Tourette Syndrome

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Alternative Names: Tourette’s syndrome, Tourette’s disorder, Gilles de la Tourette syndrome, GTS or, more commonly, simply Tourette’s or TS

Definition:
Tourette syndrome  is an inherited neuropsychiatric disorder with onset in childhood, characterized by multiple physical (motor) tics and at least one vocal (phonic) tic; these tics characteristically wax and wane. Tourette’s is defined as part of a spectrum of tic disorders, which includes transient and chronic tics.

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Tourette’s was once considered a rare and bizarre syndrome, most often associated with the exclamation of obscene words or socially inappropriate and derogatory remarks (coprolalia), but this symptom is present in only a small minority of people with Tourette’s. Tourette’s is no longer considered a rare condition, but it may not always be correctly identified because most cases are classified as mild. Between 1 and 10 children per 1,000 have Tourette’s; as many as 10 per 1,000 people may have tic disorders, with the more common tics of eye blinking, coughing, throat clearing, sniffing, and facial movements. Tourette’s does not adversely affect intelligence or life expectancy. The severity of the tics decreases for most children as they pass through adolescence, and extreme Tourette’s in adulthood is a rarity. Notable individuals with Tourette’s are found in all walks of life.

Tourette syndrome can be a chronic condition with symptoms lasting a lifetime, most people with the condition experience their worst symptoms in their early teens, with improvement occurring in the late teens and continuing into adulthood.

Clacification
Tics are sudden, repetitive, stereotyped, nonrhythmic movements (motor tics) and utterances (phonic tics) that involve discrete muscle groups.[8] Motor tics are movement-based tics, while phonic tics are involuntary sounds produced by moving air through the nose, mouth, or throat.

Tourette’s is one of several tic disorders, which are classified by the Diagnostic and Statistical Manual of Mental Disorders (DSM) according to type (motor or phonic tics) and duration (transient or chronic). Transient tic disorder consists of multiple motor tics, phonic tics or both, with a duration between four weeks and twelve months. Chronic tic disorder is either single or multiple, motor or phonic tics (but not both), which are present for more than a year. Tourette’s is diagnosed when multiple motor tics, and at least one phonic tic, are present for more than a year. Tic disorders are defined similarly by the World Health Organization (International Statistical Classification of Diseases and Related Health Problems, ICD-10 codes).

Although Tourette’s is the more severe expression of the spectrum of tic disorders, most cases are mild. The severity of symptoms varies widely among people with Tourette’s, and mild cases may be undetected

Symptoms:
Tics — sudden, brief, intermittent movements or sounds — are the hallmark sign of Tourette syndrome. Symptoms range from mild to severe and debilitating.

Tics are classified as either:
*Simple tics, which are sudden, brief and repetitive and involve a limited number of muscle groups

*Complex tics, which are distinct, coordinated patterns of movements involving several muscle groups

Tics involving movement (motor tics) — often facial tics, such as blinking — usually begin before vocal tics do. But the spectrum of tics that people experience is diverse, and there’s no typical case.

Some of the more common tics seen in Tourette syndrome
Motor tics:-

Simple tics:
*Eye blinking
*Head jerking
*Shoulder shrugging
*Eye darting
*Finger flexing
*Sticking the tongue out

Complex tics :
*Touching the nose
*Touching other people
*Smelling objects
*Obscene gestures
*Flapping the arms
*Hopping

Vocal tics:-

Simple tics :
*Hiccuping
*Yelling
*Throat clearing
*Barking

Complex tics :
*Using different voice intonations
*Repeating one’s own words or phrases
*Repeating others’ words or phrases
*Using expletives

Tics can vary in type, frequency and severity over time. They may worsen during periods of stress and anxiety, fatigue, illness, or excitement. They can occur during sleep. You’ll likely experience an urge, called a premonitory urge, before the onset of motor or vocal tics. A premonitory urge is an uncomfortable bodily sensation, such as an itch, a tingle or tension. Expression of the tic brings relief.

Different tics may develop over time. Tourette symptoms are usually at their worst during the teenage years and sometimes improve during the transition to adulthood.

With great effort, some people with Tourette syndrome can sometimes temporarily stop a tic or hold back tics until they find a place where it’s less disruptive to express them.

 

Causes:
The exact cause of Tourette’s is unknown, but it is well established that both genetic and environmental factors are involved. Genetic studies have shown that the overwhelming majority of cases of Tourette’s are inherited, although the exact mode of inheritance is not yet known, and no gene has been identified. In some cases, Tourette’s is sporadic, that is, it is not inherited from parents. In other cases, tics are associated with disorders other than Tourette’s, a phenomenon known as tourettism.

A person with Tourette’s has about a 50% chance of passing the gene(s) to one of his or her children, but Tourette’s is a condition of variable expression and incomplete penetrance. Thus, not everyone who inherits the genetic vulnerability will show symptoms; even close family members may show different severities of symptoms, or no symptoms at all. The gene(s) may express as Tourette’s, as a milder tic disorder (transient or chronic tics), or as obsessive–compulsive symptoms without tics. Only a minority of the children who inherit the gene(s) have symptoms severe enough to require medical attention. Gender appears to have a role in the expression of the genetic vulnerability: males are more likely than females to express tics.

Non-genetic, environmental, infectious, or psychosocial factors—while not causing Tourette’s—can influence its severity. Autoimmune processes may affect tic onset and exacerbation in some cases. In 1998, a team at the US National Institute of Mental Health proposed a hypothesis that both obsessive–compulsive disorder (OCD) and tic disorders may arise in a subset of children as a result of a poststreptococcal autoimmune process. Children who meet five diagnostic criteria are classified, according to the hypothesis, as having Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections (PANDAS).  This contentious hypothesis is the focus of clinical and laboratory research, but remains unproven.

The exact mechanism affecting the inherited vulnerability to Tourette’s has not been established, and the precise etiology is unknown. Tics are believed to result from dysfunction in cortical and subcortical regions, the thalamus, basal ganglia and frontal cortex. Neuroanatomic models implicate failures in circuits connecting the brain’s cortex and subcortex, and imaging techniques implicate the basal ganglia and frontal cortex.

Some forms of OCD may be genetically linked to Tourette’s. A subset of OCD is thought to be etiologically related to Tourette’s and may be a different expression of the same factors that are important for the expression of tics.   The genetic relationship of ADHD to Tourette syndrome, however, has not been fully established

Risk factors
Having a family history of Tourette syndrome or other tic disorders may increase the risk of developing Tourette syndrome.

Complication:
People with Tourette syndrome have a normal life span and often lead a healthy, active life. However, having Tourette syndrome may increase the risk of learning, behavioral and social challenges, which can mar self-image.

In addition, having Tourette syndrome means you’re likely to have other related conditions, such as:

*Attention-deficit/hyperactivity disorder (ADHD)

*Obsessive-compulsive disorder

*Learning disabilities

*Sleep disorders

*Depression

*Anxiety disorders

Diagnosis:
According to the revised fourth edition of the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV-TR), Tourette’s may be diagnosed when a person exhibits both multiple motor and one or more vocal tics (although these do not need to be concurrent) over the period of a year, with no more than three consecutive tic-free months. The previous DSM-IV included a requirement for “marked distress or significant impairment in social, occupational or other important areas of functioning”, but this requirement was removed in the most recent update of the manual, in recognition that clinicians see patients who meet all the other criteria for Tourette’s, but do not have distress or impairment.[44] The onset must have occurred before the age of 18, and cannot be attributed to the “direct physiological effects of a substance or a general medical condition”. Hence, other medical conditions that include tics or tic-like movements—such as autism or other causes of tourettism—must be ruled out before conferring a Tourette’s diagnosis.

There are no specific medical or screening tests that can be used in diagnosing Tourette’s; it is frequently misdiagnosed or underdiagnosed, partly because of the wide expression of severity, ranging from mild (the majority of cases) or moderate, to severe (the rare, but more widely-recognized and publicized cases). Coughing, eye blinking and tics that mimic asthma are commonly misdiagnosed.

The diagnosis is made based on observation of the individual’s symptoms and family history, and after ruling out secondary causes of tic disorders. In patients with a typical onset and a family history of tics or obsessive–compulsive disorder, a basic physical and neurological examination may be sufficient.

There is no requirement that other comorbid conditions (such as ADHD or OCD) be present, but if a physician believes that there may be another condition present that could explain tics, tests may be ordered as necessary to rule out that condition. An example of this is when diagnostic confusion between tics and seizure activity exists, which would call for an EEG, or if there are symptoms that indicate an MRI to rule out brain abnormalities.  TSH levels can be measured to rule out hypothyroidism, which can be a cause of tics. Brain imaging studies are not usually warranted. In teenagers and adults presenting with a sudden onset of tics and other behavioral symptoms, a urine drug screen for cocaine and stimulants might be necessary. If a family history of liver disease is present, serum copper and ceruloplasmin levels can rule out Wilson’s disease. Most cases are diagnosed by merely observing a history of tics.

Secondary causes of tics (not related to inherited Tourette syndrome) are commonly referred to as tourettism. Dystonias, choreas, other genetic conditions, and secondary causes of tics should be ruled out in the differential diagnosis for Tourette syndrome.  Other conditions that may manifest tics or stereotyped movements include developmental disorders, autism spectrum disorders, and stereotypic movement disorder;  Sydenham’s chorea; idiopathic dystonia; and genetic conditions such as Huntington’s disease, neuroacanthocytosis, Hallervorden-Spatz syndrome, Duchenne muscular dystrophy, Wilson’s disease, and tuberous sclerosis. Other possibilities include chromosomal disorders such as Down syndrome, Klinefelter’s syndrome, XYY syndrome and fragile X syndrome. Acquired causes of tics include drug-induced tics, head trauma, encephalitis, stroke, and carbon monoxide poisoning. The symptoms of Lesch-Nyhan syndrome may also be confused with Tourette syndrome. Most of these conditions are rarer than tic disorders, and a thorough history and examination may be enough to rule them out, without medical or screening tests
Treatment:
There’s no cure for Tourette syndrome. Treatment is intended to help control tics that interfere with everyday activities and functioning. When tics aren’t severe, treatment may be unnecessary.

Medications:
No medication is helpful to everyone with Tourette syndrome, none completely eliminates symptoms, and they all have side effects to be weighed against the benefits. However, some medications can be used to help control or minimize tics or to control symptoms of related conditions, such as attention-deficit/hyperactivity disorder (ADHD) or obsessive-compulsive disorder (OCD). These may include:

*Drugs that block or deplete the neurotransmitter dopamine in the brain, such as fluphenazine or pimozide (Orap). Used to control tics, these medications may have side effects such as weight gain and a dulling of the mind.

*Botulinum Toxin Type A (Botox) injections. For simple or vocal tics, an injection into the affected muscle may help relieve the tic.

*Stimulant medications, such as methylphenidate (Concerta, Ritalin, others) and dextroamphetamine (Dexedrine, others). These are used to help increase attention and concentration for people with ADHD.

*Central adrenergic inhibitors, such as clonidine (Catapres) or guanfacine (Tenex). Typically prescribed for high blood pressure, these drugs may help control behavioral symptoms, such as impulse control problems and rage attacks. Side effects may include sleepiness.

*Antidepressants, such as fluoxetine (Prozac, Sarafem, others). These may help control the symptoms of OCD.

Therapies
*Psychotherapy. Psychotherapy can be helpful for two reasons. It can help with accompanying problems, such as ADHD, obsessions, depression and anxiety. Therapy can also help people cope with Tourette syndrome.

*Deep brain stimulation. For debilitating tics that don’t respond to other treatment, deep brain stimulation (DBS) may help. DBS consists of implanting a battery-operated medical device (neurostimulator) in the brain to deliver electrical stimulation to targeted areas that control movement. Further research is needed to determine whether DBS is beneficial for people with Tourette syndrome.

 

Prognosis:
Tourette syndrome is a spectrum disorder—its severity ranges over a spectrum from mild to severe. The majority of cases are mild and require no treatment. In these cases, the impact of symptoms on the individual may be mild, to the extent that casual observers might not know of their condition. The overall prognosis is positive, but a minority of children with Tourette syndrome have severe symptoms that persist into adulthood. A study of 46 subjects at 19 years of age found that the symptoms of 80% had minimum to mild impact on their overall functioning, and that the other 20% experienced at least a moderate impact on their overall functioning. The rare minority of severe cases can inhibit or prevent individuals from holding a job or having a fulfilling social life. In a follow-up study of thirty-one adults with Tourette’s, all patients completed high school, 52% finished at least two years of college, and 71% were full-time employed or were pursuing higher education.

Regardless of symptom severity, individuals with Tourette’s have a normal life span. Although the symptoms may be lifelong and chronic for some, the condition is not degenerative or life-threatening. Intelligence is normal in those with Tourette’s, although there may be learning disabilities. Severity of tics early in life does not predict tic severity in later life, and prognosis is generally favorable, although there is no reliable means of predicting the outcome for a particular individual. The gene or genes associated with Tourette’s have not been identified, and there is no potential “cure”. A higher rate of migraines than the general population and sleep disturbances are reported.

Several studies have demonstrated that the condition in most children improves with maturity. Tics may be at their highest severity at the time that they are diagnosed, and often improve with understanding of the condition by individuals and their families and friends. The statistical age of highest tic severity is typically between eight and twelve, with most individuals experiencing steadily declining tic severity as they pass through adolescence. One study showed no correlation with tic severity and the onset of puberty, in contrast with the popular belief that tics increase at puberty. In many cases, a complete remission of tic symptoms occurs after adolescence. However, a study using videotape to record tics in adults found that, although tics diminished in comparison with childhood, and all measures of tic severity improved by adulthood, 90% of adults still had tics. Half of the adults who considered themselves tic-free still displayed evidence of tics.

It is not uncommon for the parents of affected children to be unaware that they, too, may have had tics as children. Because Tourette’s tends to subside with maturity, and because milder cases of Tourette’s are now more likely to be recognized, the first realization that a parent had tics as a child may not come until their offspring is diagnosed. It is not uncommon for several members of a family to be diagnosed together, as parents bringing children to a physician for an evaluation of tics become aware that they, too, had tics as a child.

Children with Tourette’s may suffer socially if their tics are viewed as “bizarre”. If a child has disabling tics, or tics that interfere with social or academic functioning, supportive psychotherapy or school accommodations can be helpful.  Because comorbid conditions (such as ADHD or OCD) can cause greater impact on overall functioning than tics, a thorough evaluation for comorbidity is called for when symptoms and impairment warrant.

A supportive environment and family generally gives those with Tourette’s the skills to manage the disorder.  People with Tourette’s may learn to camouflage socially inappropriate tics or to channel the energy of their tics into a functional endeavor. Accomplished musicians, athletes, public speakers, and professionals from all walks of life are found among people with Tourette’s. Outcomes in adulthood are associated more with the perceived significance of having severe tics as a child than with the actual severity of the tics. A person who was misunderstood, punished, or teased at home or at school will fare worse than children who enjoyed an understanding and supportive environment

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://en.wikipedia.org/wiki/Tourette_syndrome
http://www.mayoclinic.com/health/tourette-syndrome/DS00541

http://www.sfn.org/index.aspx?pagename=brainBriefings_tourette#full

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Progressive Supranuclear Palsy

Definition:
Progressive supranuclear palsy, also called Steele-Richardson-Olszewski syndrome,(after the Canadian physicians who described it in 1963)  is a brain disorder that causes serious problems with walking, balance and eye movements. Progressive supranuclear palsy results from deterioration of cells in areas of your brain that control movement.
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Progressive supranuclear palsy is a rare disorder that slowly worsens over time. Although it’s not life-threatening itself, progressive supranuclear palsy can lead to life-threatening complications, such as pneumonia and swallowing problems.

Because there’s no cure for progressive supranuclear palsy, treatment focuses on managing and improving the related signs and symptoms.

Males and females are affected approximately equally and there is no racial, geographical or occupational predilection. Approximately 6 people per 100,000 population have PSP.

It has been described as a tauopathy

Symptoms:
The initial symptoms in two-thirds of cases are loss of balance, lunging forward when mobilizing, fast walking, bumping into objects or people, and falls.

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Other common early symptoms are changes in personality, general slowing of movement, and visual symptoms.

Later symptoms and signs are dementia (typically including loss of inhibition and ability to organize information), slurring of speech, difficulty swallowing, and difficulty moving the eyes, particularly in the vertical direction. The latter accounts for some of the falls experienced by these patients as they are unable to look up or down.

Some of the other signs are poor eyelid function, contracture of the facial muscles, a backward tilt of the head with stiffening of the neck muscles, sleep disruption, urinary incontinence and constipation.

The visual symptoms are of particular importance in the diagnosis of this disorder. Notably, the ophthalmoparesis experienced by these patients mainly concerns voluntary eye movement. Patients tend to have difficulty looking down (a downgaze palsy) followed by the addition of an upgaze palsy. Involuntary eye movement, as elicited by Bell’s phenomenon, for instance, may be closer to normal. On close inspection, eye movements called “square-wave jerks” may be visible when the patient fixes at distance. These are fine movements, similar to nystagmus, except that they are not rhythmic in nature. Difficulties with convergence (convergence insufficiency), where the eyes come closer together while focusing on something near, like the pages of a book, is typical. Because the eyes have trouble coming together to focus at short distances, the patient may complain of diplopia (double vision) when reading.

Cardinal manifestations:

*Supranuclear ophthalmoplegia

*Neck dystonia

*Parkinsonism

*Pseudobulbar palsy

*Behavioral and Cognitive impairment

*Imbalance and Difficulties walking

*Frequent Falls

*Stiffness

*Awkward movements

*Falling

*Problems with speech and swallowing

*Dizziness

*Loss of interest in pleasurable activities (apathy)

*Depression and anxiety

*Laughing or crying for no reason

*Forgetfulness

True to its name, the signs and symptoms of progressive supranuclear palsy tend to become progressively worse as the disease advances.

Causes:
We know that the symptoms of PSP are caused by a gradual deterioration of brain cells in a few tiny but important places at the base of the brain, in the region called the brainstem. One of these areas, the substantia nigra, is also affected in Parkinson’s disease, and damage to this region of the brain accounts for the motor symptoms that PSP and Parkinson’s have in common.

Scientists do not know what causes these brain cells to degenerate. There is no evidence that PSP is contagious, and genetic factors have not been implicated. No ethnic or racial groups have been affected more often than any others, and PSP is no more likely to occur in some geographic areas than in others.

There are, however, several theories about PSP’s cause. One possibility is that an unconventional virus-like agent infects the body and takes years or decades to start producing visible effects. Creutzfeldt-Jakob disease is one disease known to be caused by such an agent. Another possibility is that random genetic mutations, of the kind that occur in all of us all the time, happen to occur in particular cells or certain genes, in just the right combination to injure these cells. A third possibility is that there is exposure to some unknown chemical in the food, air, or water which slowly damages certain vulnerable areas of the brain. This theory stems from a clue found on the Pacific island of Guam, where a common neurological disease occurring only there and on a few neighboring islands shares some of the characteristics of PSP, Alzheimer’s disease, Parkinson’s disease, and amyotrophic lateral sclerosis (Lou Gehrig’s disease). Its cause is thought to be a dietary factor or toxic substance found only in that area.

Another possible cause of PSP is cellular damage caused by free radicals, unstable molecules produced continuously by all cells during normal metabolism. Although the body has built-in mechanisms for clearing free radicals from the system, scientists suspect that, under certain circumstances, free radicals can react with and damage other molecules. A great deal of research is directed at understanding the role of free radical damage in human diseases.

Genetics and Causal Factors:
Fewer than 1% of those with PSP have a family member with the same disorder. A variant in the gene for tau protein called the H1 haplotype, located on chromosome 17, has been linked to PSP. Nearly all people with PSP received a copy of that variant from each parent, but this is true of about two-thirds of the general population. Therefore, the H1 haplotype appears to be necessary but not sufficient to cause PSP. Other genes, as well as environmental toxins are being investigated as other possible contributors to the cause of PSP.

Risk Factors:
Risk factors for progressive supranuclear palsy include:

*Age. Progressive supranuclear palsy typically affects people around the age of 60.
*Sex. Men are affected somewhat more often.

Complications :
Complications of progressive supranuclear palsy result primarily from hindered muscle movements. These complications may include:

*Frequent falling, which can lead to head injuries, fractures and other injuries.

*Difficulty focusing your eyes, which also can lead to injuries.

*Problems with reading, driving a car, or other tasks requiring hand-eye coordination.

*Difficulty sleeping.

*Difficulty looking at bright lights.

*Problems swallowing, which can lead to choking or inhaling food or liquid into your airway (aspiration). Aspiration can develop into pneumonia — the most common cause of death in people with progressive supranuclear palsy.

*Impulsive behaviors — for example, standing up without waiting for assistance — which can lead to falls.

Complications from progressive supranuclear palsy may eventually necessitate the use of a feeding tube due to choking hazards. To avoid injuries due to falling, a walker or a wheelchair may also be necessary.

Diagnosis:
Initial complaints in PSP are typically vague and an early diagnosis is always difficult. The primary complaints fall into these categories:

*Symptoms of dysequilibrium, such as unsteady walking or abrupt and unexplained falls without loss of consciousness.

*Visual complaints, including blurred vision, difficulties in looking up or down, double vision, light sensitivity, burning eyes, or other eye trouble.

*Slurred speech.

*Various mental complaints such as slowness of thought, impaired memory, personality changes, and changes in mood.

Progressive Supranuclear Palsy is often misdiagnosed because some of its symptoms are very much like those of Parkinson’s disease, Alzheimer’s disease, and more rare neurodegenerative disorders, such as Creutzfeldt-Jakob disease. In fact, PSP is most often misdiagnosed as Parkinson’s disease early in the course of the illness. Memory problems and personality changes may also lead a physician to mistake PSP for depression, or even attribute symptoms to some form of dementia. The key to establishing the diagnosis of PSP is the identification of early gait instability and difficulty moving the eyes, the hallmark of the disease, as well as ruling out other similar disorders, some of which are treatable.

PSP is frequently misdiagnosed as Parkinson’s disease because of the slowed movements and gait difficulty, or as Alzheimer’s disease because of the behavioral changes. It is one of a number of diseases collectively referred to as Parkinson plus syndromes. A poor response to levodopa along with symmetrial onset can help differentiate this disease from PD.

Difference between PSP &  Parkinson’s Disease:
Both PSP and Parkinson’s disease cause stiffness, movement difficulties, and clumsiness. However, patients with PSP usually stand straight or occasionally even tilt their heads backward (and tend to fall backward), while those with Parkinson’s disease usually bend forward. Problems with speech and swallowing are much more common and severe in PSP than in Parkinson’s disease, and tend to show up earlier in the course of the disease. Both diseases share other features: onset in late middle age, bradykinesia (slow movement), and rigidity of muscles. Tremor, almost universal in Parkinson’s patients, is rare in PSP. Although Parkinson’s patients markedly benefit from the drug levodopa, patients with PSP respond poorly and only transiently to this drug.

Treatment :
There is currently no effective treatment for PSP, although scientists are searching for better ways to manage the disease. In some patients the slowness, stiffness, and balance problems of PSP may respond to antiparkinsonian agents such as levodopa, but the effect is usually temporary. The speech, vision, and swallowing difficulties usually do not respond to any drug treatment.

Another group of drugs that has been of some modest success in PSP are antidepressant medications. The most commonly used of these drugs are fluoxetine (Prozac), amitriptyline (Elavil), and imipramine (Tofranil). The anti-PSP benefit of these drugs seems not to be related to their ability to relieve depression.

Non-drug treatment for PSP can take many forms. Patients frequently use weighted walking aids because of their tendency to fall backward. Bifocals or special glasses called prisms are sometimes prescribed for PSP patients to remedy the difficulty of looking down. Formal physical therapy is of no proven benefit in PSP, but certain exercises can be done to keep the joints limber.

A surgical procedure that may be necessary when there are swallowing disturbances is a gastrostomy. A gastrostomy (or a jejunostomy) is a minimally invasive procedure which is performed when the patient has difficulty swallowing or when severe choking is a definite risk. This surgery involves the placement of a tube through the skin of the abdomen into the stomach (intestine) for feeding purposes. Surgical procedures such as fetal brain cell implantation and pallidotomy, which are being tested as treatments for Parkinson’s disease, are not effective in PSP.

Life Style & Home Remedies:
To minimize the effects of progressive supranuclear palsy, you can take certain steps at home. They may include:

*Eyedrops. Eyedrops may help ease dry eyes that can occur as a result of problems blinking. They can also be helpful for persistent tearing.

*Fall-avoidance aids. Installing grab bars in hallways and bathrooms or using a walker that’s weighted can help you avoid falls. Making home modifications, such as removing scatter rugs or other items that are hard to see without looking downward, also can help with balance and vision problems. When possible, avoid climbing stairs.

Prognosis:
As there is currently no effective treatment or cure for PSP, although some of the symptoms can respond to nonspecific measures. The average age at symptoms onset is 63 and survival from onset averages 7 years with a wide variance

Researches:
Studies to improve the diagnosis of PSP have recently been conducted at the National Institute of Neurological Disorders and Stroke (NINDS). Experiments to find the cause or causes of PSP are currently under way. Therapeutic trials with free radical scavengers (agents that can get rid of potentially harmful free radicals) are being planned for the future.

In addition, there is a great deal of ongoing research on Parkinson’s and Alzheimer’s diseases at the National Institutes of Health and at university medical centers throughout the country. Better understanding of those common related disorders will go a long way toward solving the problem of PSP, just as studying PSP may help shed light on Parkinson’s and Alzheimer’s diseases.

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.healthtouch.com/bin/EContent_HT/showAllLfts.asp?lftname=NINDS156&cid=HT
http://en.wikipedia.org/wiki/Progressive_supranuclear_palsy
http://www.mayoclinic.com/health/progressive-supranuclear-palsy/DS00909
http://www.nlm.nih.gov/medlineplus/progressivesupranuclearpalsy.html

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Ailmemts & Remedies

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

Categories
Ailmemts & Remedies

Joubert syndrome

Alternative Names:  Cerebellar vermis agenesis or Cerebelloparenchymal disorder IV

Definition:
Joubert syndrome is a rare inherited disorder of the brain. It is a genetic birth defect in which the area of the brain that controls balance and coordination is underdeveloped.It is a rare brain malformation characterized by the absence or underdevelopment of the cerebellar vermis – an area of the brain that controls balance and coordination. The most common features of Joubert syndrome in infants include abnormally rapid breathing (hyperpnea), decreased muscle tone (hypotonia), jerky eye movements (oculomotor apraxia), mental retardation, and the inability to coordinate voluntary muscle movements (ataxia).

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An area at the back of the brain which is important for balance and co-ordination, called the cerebellum normally has two interconnected halves or hemispheres. In Joubert syndrome the connection between the two halves, known as the cerebellar vermis, fails to develop properly. As a result, one of the main features of Joubert syndrome is poorly controlled or unsteady movement, known as ataxia.

The severity of the condition varies from child to child, depending on the extent of the abnormalities of the brain. Some children are only mildly affected while others (even within the same family) have severe disabilities.

It occurs in both males and females, in about one in 100,000 births. Joubert syndrome often occurs in a child with no family history of the disorder, but in some children the syndrome appears to be inherited.

Symptoms:
The symptoms of Joubert syndrome are related to the underdevelopment of an area of the brain called the cerebellar vermis, which controls balance and muscle coordination. The symptoms, which may range from mild to severe depending on how much the brain is underdeveloped, may include:

•Periods of abnormally rapid breathing (episodic hyperpnea), which may seem like panting

•jerky eye movements (nystagmus)

•characteristic facial features such as drooping eyelids (ptosis), open mouth with protruding tongue, low-set ears

•mental retardation

•difficulty coordinating voluntary muscle movements (ataxia)
Other birth defects such as extra fingers and toes (polydactyly), heart defects, or cleft lip or palate may be present. Seizures may also occur.

Causes:
Joubert syndrome is a genetic abnormality inherited in an autosomal recessive fashion. This means that if both parents are carriers, there is a 1 in 4 chance that each child will have the disease.

Diagnosis:
The most pronounced symptom in a newborn infant with Joubert syndrome is periods of abnormally rapid breathing, which may be followed by stopping breathing (apnea) for up to one minute. Although these symptoms may occur in other disorders, there are no lung problems in Joubert syndrome, which helps identify it as the cause of the abnormal breathing.

A magnetic resonance imaging (MRI) scan can look for the brain abnormalities that are present in Joubert syndrome and confirm the diagnosis.

Treatment:
There is no cure for Joubert syndrome, so treatment focuses on the symptoms such as breathing problems and to support the child’s development.. Infants with abnormal breathing may have a breathing (apnea) monitor for use at home, especially at night. Physical, occupational, and speech therapy may be helpful for some individuals. Individuals with heart defects, cleft lip or palate, or seizures may require more medical care.

Prognosis:
The prognosis for infants with Joubert syndrome depends on whether or not the cerebellar vermis is partially developed or entirely absent. Some children have a mild form of the disorder, with minimal motor disability and good mental development, while others may have severe motor disability and moderate mental retardation.

Research:
The NINDS supports research on the development of the nervous system and the cerebellum. This research is critical for increasing our understanding of Joubert syndrome, and for developing methods of treatment and prevention. NINDS, in conjunction with the NIH Office of Rare Disorders, sponsored a symposium on Joubert syndrome in 2002. Research priorities for the disorder were outlined at this meeting.

Research has revealed that a number of genetic disorders, not previously thought to be related, may indeed be related as to their root cause. Joubert syndrome is one such disease. It is a member of an emerging class of diseases called cilopathies.

The underlying cause of the ciliopathies may be a dysfunctional molecular mechanism in the primary cilia structures of the cell, organelles which are present in many cellular types throughout the human body. The cilia defects adversely affect “numerous critical developmental signaling pathways” essential to cellular development and thus offer a plausible hypothesis for the often multi-symptom nature of a large set of syndromes and diseases.

Currently recognized ciliopathies include Joubert syndrome, primary ciliary dyskinesia, Bardet-Biedl syndrome, polycystic kidney disease and polycystic liver disease, nephronophthisis, Alstrom syndrome, Meckel-Gruber syndrome and some forms of retinal degeneration.

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/joubert1.shtml
http://www.ninds.nih.gov/disorders/joubert/joubert.htm
http://www.ninds.nih.gov/disorders/joubert/joubert.htmhttp://www.ninds.nih.gov/disorders/joubert/joubert.htm

http://www.joubertfoundation.com/

http://www.health-news-blog.com/blogs/permalinks/6-2007/the-fifth-gene-responsible-for-joubert-syndrome.html

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