Disease: PANDAS (Pediatric Autoimmune Neuropsychiatric Disorders Associated With Streptococcal Infections)

    Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS) facts

    • PANDAS is an acronym for "pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections."
    • It is a fairly recently described disorder (1990s).
    • An autoimmune response to a streptococcal infection causes PANDAS.
    • It is diagnosed if there is a history of abruptly developing a number of neuropsychiatric symptoms associated with a strep infection.
    • Medication and cognitive behavioral therapy are the primary treatments for PANDAS.

    What is PANDAS?

    PANDAS is an acronym which refers to "pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections." It is diagnosed clinically after a patient develops a number of behavioral and physical symptoms following a strep throat infection.

    Is PANDAS common?

    PANDAS is a rare condition. It is believed that approximately one in 2,000 children are affected, though the diagnosis may be overlooked due to some of the common symptoms associated with the disease. In fact, there are some researchers who recommend including PANDAS in a category of other disorders which include similar neuropsychiatric symptoms called CANS (childhood acute neuropsychiatric symptoms) or PANS (pediatric acute-onset neuropsychiatric syndrome).

    What is the history of PANDAS?

    PANDAS was described in the late 1990s after investigators identified a subgroup of children who developed a sudden onset of obsessive-compulsive disorder (OCD) symptoms, vocal and motor tics, and other behavioral changes. They identified that there was a common association in these children, and that was identified as a recent or active infection with a beta-hemolytic Streptococcus, the bacteria that causes strep throat. Researchers also identified a number of other infectious agents, known to cause significant auto-inflammatory reactions associated with neuropsychiatric symptoms, including influenza, varicella, and even Borrelia burgdorferi (Lyme disease), but the diagnosis of PANDAS is limited to streptococcal infections.

    What causes PANDAS?

    PANDAS is in part caused by an autoimmune response to a strep infection. Streptococcus is known to be associated with a number of immune-related disorders, including rheumatic fever, scarlet fever, and acute glomerulonephritis (a kidney disorder). The strep molecules are known to hide from the host immune system by mimicking host cells. Ultimately, the immune system recognizes the foreign cells, and the antibodies produced attack the bacterium, and unfortunately, some of the host's own cells due to cross-reactivity. In some situations, these antibodies attack brain cells, causing OCD, tics, and other symptoms frequently observed in PANDAS patients.

    What are symptoms and signs of PANDAS?

    PANDAS symptoms are similar to those of obsessive-compulsive disorder (OCD), attention deficit hyperactivity disorder (ADHD), and motor or verbal tics. Symptoms tend to appear suddenly, and the recovery period is variable. Often the symptoms may worsen during streptococcal infections and improve in between.

    How is PANDAS diagnosed?

    PANDAS is diagnosed clinically. That means that it depends more on history and physical examination rather than other specific studies. The following summarizes the five criteria used to diagnose PANDAS:

    1. Presence of obsessive-compulsive disorder (OCD) and/or a tic disorder, ADHD symptoms, or oppositional behaviors
    2. Abrupt onset and or symptoms vary in intensity
    3. Association with neurological abnormalities, including motor hyperactivity, or abnormal movements, such as choreiform movements (involuntary jerky movements), other combinations of neuropsychiatric symptoms, such as anxiety, emotional lability, bedwetting, or other regressive behaviors (temper tantrums), personality changes and deterioration in math skills and handwriting
    4. Onset of symptoms from age 3 years to puberty
    5. Association with group A beta-hemolytic streptococcal infection (the bacteria that causes strep throat) either by culture or other evidence of infection, such as scarlet fever or by laboratory test evidence

    As mentioned above, there are five criteria used to diagnose the disorder and must include the sudden onset of OCD, tics, ADHD, or a rapid worsening of existing symptoms. To make the diagnosis, one also needs evidence of a recent or active strep infection either by throat culture or by other laboratory confirmative study of that infection (for example, antistreptolysin O or antideoxyribonuclease B antibodies). Along with the clinical diagnosis, it is important to be sure that there is not some other reason for the symptoms, and additional testing might be performed for that reason. In fact, distinguishing PANDAS from Tourette's syndrome (a common tic disorder), OCD, or Sydenham chorea (a movement disorder associated with rheumatic fever also caused by Streptococcus) is not always a simple task.

    What is the treatment for PANDAS?

    PANDAS is treated primarily with cognitive behavioral therapy and medication, depending on the breadth and severity of symptoms. Currently, the primary medical therapy is focused on controlling the OCD symptoms, and therefore selective serotonin reuptake inhibitor (SSRI) medications are frequently used (fluoxetine [Prozac] and others). The other more acute measure in treating PANDAS is treating the strep infection associated with the symptoms. Antibiotics are the treatment of choice to eradicate an active strep throat infection. Your doctor may prescribe penicillin, amoxicillin (Amoxil), azithromycin (Zithromax), or another antibiotic. For severe and refractory symptoms, the use of other treatments aimed at controlling the immune response have been studied and found to be effective. These include the use of intravenous immunoglobulin, steroids, and plasmapheresis (plasma exchange therapy in which blood is withdrawn from an individual and the liquid portion is removed and replaced and the blood is transfused back into the individual). For both treatments, the "offending" antibodies are removed. Steroids are less effective, because, although symptoms decrease during the treatment, they return soon after stopping. More studies are needed to be able to recommend immune-modulating therapies without hesitation.

    What is the prognosis of PANDAS?

    Though some children completely recover from PANDAS, there are some who develop persistent neuropsychiatric symptoms or develop worsening symptoms after each strep infection. It is not currently possible to predict which children will be affected or how severely. The mainstay of therapy is to decrease the OCD, tics, or other behaviors which might interfere with daily life and school.

    What causes PANDAS?

    PANDAS is in part caused by an autoimmune response to a strep infection. Streptococcus is known to be associated with a number of immune-related disorders, including rheumatic fever, scarlet fever, and acute glomerulonephritis (a kidney disorder). The strep molecules are known to hide from the host immune system by mimicking host cells. Ultimately, the immune system recognizes the foreign cells, and the antibodies produced attack the bacterium, and unfortunately, some of the host's own cells due to cross-reactivity. In some situations, these antibodies attack brain cells, causing OCD, tics, and other symptoms frequently observed in PANDAS patients.

    What are symptoms and signs of PANDAS?

    PANDAS symptoms are similar to those of obsessive-compulsive disorder (OCD), attention deficit hyperactivity disorder (ADHD), and motor or verbal tics. Symptoms tend to appear suddenly, and the recovery period is variable. Often the symptoms may worsen during streptococcal infections and improve in between.

    How is PANDAS diagnosed?

    PANDAS is diagnosed clinically. That means that it depends more on history and physical examination rather than other specific studies. The following summarizes the five criteria used to diagnose PANDAS:

    1. Presence of obsessive-compulsive disorder (OCD) and/or a tic disorder, ADHD symptoms, or oppositional behaviors
    2. Abrupt onset and or symptoms vary in intensity
    3. Association with neurological abnormalities, including motor hyperactivity, or abnormal movements, such as choreiform movements (involuntary jerky movements), other combinations of neuropsychiatric symptoms, such as anxiety, emotional lability, bedwetting, or other regressive behaviors (temper tantrums), personality changes and deterioration in math skills and handwriting
    4. Onset of symptoms from age 3 years to puberty
    5. Association with group A beta-hemolytic streptococcal infection (the bacteria that causes strep throat) either by culture or other evidence of infection, such as scarlet fever or by laboratory test evidence

    As mentioned above, there are five criteria used to diagnose the disorder and must include the sudden onset of OCD, tics, ADHD, or a rapid worsening of existing symptoms. To make the diagnosis, one also needs evidence of a recent or active strep infection either by throat culture or by other laboratory confirmative study of that infection (for example, antistreptolysin O or antideoxyribonuclease B antibodies). Along with the clinical diagnosis, it is important to be sure that there is not some other reason for the symptoms, and additional testing might be performed for that reason. In fact, distinguishing PANDAS from Tourette's syndrome (a common tic disorder), OCD, or Sydenham chorea (a movement disorder associated with rheumatic fever also caused by Streptococcus) is not always a simple task.

    What is the treatment for PANDAS?

    PANDAS is treated primarily with cognitive behavioral therapy and medication, depending on the breadth and severity of symptoms. Currently, the primary medical therapy is focused on controlling the OCD symptoms, and therefore selective serotonin reuptake inhibitor (SSRI) medications are frequently used (fluoxetine [Prozac] and others). The other more acute measure in treating PANDAS is treating the strep infection associated with the symptoms. Antibiotics are the treatment of choice to eradicate an active strep throat infection. Your doctor may prescribe penicillin, amoxicillin (Amoxil), azithromycin (Zithromax), or another antibiotic. For severe and refractory symptoms, the use of other treatments aimed at controlling the immune response have been studied and found to be effective. These include the use of intravenous immunoglobulin, steroids, and plasmapheresis (plasma exchange therapy in which blood is withdrawn from an individual and the liquid portion is removed and replaced and the blood is transfused back into the individual). For both treatments, the "offending" antibodies are removed. Steroids are less effective, because, although symptoms decrease during the treatment, they return soon after stopping. More studies are needed to be able to recommend immune-modulating therapies without hesitation.

    What is the prognosis of PANDAS?

    Though some children completely recover from PANDAS, there are some who develop persistent neuropsychiatric symptoms or develop worsening symptoms after each strep infection. It is not currently possible to predict which children will be affected or how severely. The mainstay of therapy is to decrease the OCD, tics, or other behaviors which might interfere with daily life and school.

    Source: http://www.rxlist.com

    PANDAS is treated primarily with cognitive behavioral therapy and medication, depending on the breadth and severity of symptoms. Currently, the primary medical therapy is focused on controlling the OCD symptoms, and therefore selective serotonin reuptake inhibitor (SSRI) medications are frequently used (fluoxetine [Prozac] and others). The other more acute measure in treating PANDAS is treating the strep infection associated with the symptoms. Antibiotics are the treatment of choice to eradicate an active strep throat infection. Your doctor may prescribe penicillin, amoxicillin (Amoxil), azithromycin (Zithromax), or another antibiotic. For severe and refractory symptoms, the use of other treatments aimed at controlling the immune response have been studied and found to be effective. These include the use of intravenous immunoglobulin, steroids, and plasmapheresis (plasma exchange therapy in which blood is withdrawn from an individual and the liquid portion is removed and replaced and the blood is transfused back into the individual). For both treatments, the "offending" antibodies are removed. Steroids are less effective, because, although symptoms decrease during the treatment, they return soon after stopping. More studies are needed to be able to recommend immune-modulating therapies without hesitation.

    Source: http://www.rxlist.com

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