Encephalopothy

PANDAS is NOT currently classified as encephalitis. However, PANDAS can cause in the acute case histories you’ve read about on this site an Encephalitic-like Sudden Onset.

Dr. Russell C. Dale, Institute of Child Health, University College London, and colleagues have written up groundbreaking work on the nature a form basal ganglia autoimmune encephalitis caused by a virus, Von Economo’s, and its similarities to PANDAS symptoms.

The first commentary with excellent explanation of the basal ganglia: R.C. Dale (2003) Autoimmunity and the Basal Ganglia: New Insights into an Old Disease.

The second study with colleagues is: R.C. Dale, et al (2004) Encephalitis Lethargica Syndrome: 20 New Cases of Evidence of Basal Ganglia Autoimmunity.

Again, typical Encephalitis is different than PANDAS. It is an inflammation caused by a viral or bacterial infection that is present in the brain and physical deterioration is often rapid and severe and it is easily detected via an MRI. This dangerous elevation of symptoms does not seem to occur in PANDAS.

In THE LANCET Neurology article (2011) Autoantibodies associated with diseases of the CNS: new developments and future challenges, neurologists link PANDAS and possible encephalopothy. Specifically look at the chart on page 2 where PANDAS is listed.

Two well-respected Pediatric Neurologists have told me: “It is difficult to prove inflammation because NERVES do not show up in an MRI. The studies have shown some changes in the Virchow Robin Spaces and a Swedo study did show increase volume of basal ganglia. With the brain, we cannot take tissue out and look at under a microscope for inflammation. We must use our own brains to think logically instead.”

One of the Swedo studies the above doctor is referring to is Swedo, et al (2000) – MRI Assessment of Children with OCD or TICS Associated with Streptococcal Infections.

Therefore,

As parents, when we face doctors who will not help us with treatment, it can be helpful to use the words, “an encephalopathic-like episode” has occurred in your child.

Encephalitis can be either caused by:

 1) Viral

2) Bacterial or even

 3) Autoimmune response to a bacteria or virus (as in the rare illness ADEM, Acute Disseminated Encephalomylitis. In ADEM, the white matter of the brain is injured and inflamed).

In PANDAS, the basal ganglia are irritated and sets up a negative autoimmune response to the strep bacteria.

In all fairness, doctors are perplexed with our children because they do not develop severe/rapid mental and physical debilitation – they relapse and remit in their symptoms. Some children respond rapidly to 10 days of antibiotics. Most children require a longer course of antibiotics. Why this is – is not yet clear – though doctors have observed this to be true. PANDAS’ sudden attacks then slow, relapsing-remitting course is NOT how inflammatory brain illnesses are currently thought about by the medical community.

The topic of encephalitis is brought up only to explain that there is precedent for strange and varied psychological behaviors stemming from brain inflammation, especially where the basal ganglia are involved and that a bacterial irritation with ensuing autoimmune responses can be the culprit.

In PANDAS, initially, the episode is a negative autoimmune response in the basal ganglia to the strep bacteria. It is in the basal ganglia area only. It is not in the white or gray matter itself. There is no infection in the brain. **

* * NOTE: Very rarely, it has happened that in a few PANDAS, an MRI has picked up white or gray matter inflammation and the children are in those cases in need of and will receive immediate treatment for encephalitis.

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Attached is a description of encephalitis syndromes where psychological symptoms are nearly identical to that of PANDAS. This is a publication by Oxford University.

Please read this as precedent-only for psychological symptoms, i.e., in Von Economo’s Enceph – for PANDAS-like behaviors, as described by Dale above.

In this viral form of enceph, the basal ganglia are assaulted and the viral infection is also present in the brain. Click HERE for the Oxford Encephalitis Article.

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Basal Ganglia Imaging Study by Dr. Harry Chugani

The rogue white blood cells that cause P.A.N.D.A.S. affect the basil ganglia of the brain. The basil ganglia are several brain structures located in the center of the brain. They function as a communication route to other parts of the brain and are partly responsible for the movement of our body. Other parts of the basal ganglia are involved in memorization, cognitive and emotional processing, and again initiating movement. There is still much to be learned about how this part of our brain functions. It is theorized that P.A.N.D.A.S. causes an inflammation of the basil ganglia. A 1999 study by Ranjit C. Chacko, M.D. showed a connection between OCD and the basil ganglia.

Now, Dr. Harry Chugani at Children’s Hospital of Michigan, is studying the potential use of PET scans as diagnostic tools for P.A.N.D.A.S. Dr Chugani states that the basal ganglia are not hard to detect on an MRI scan. In fact, the basil ganglia are large structures. However, the MRI scan is not useful for detection of abnormal microscopic, neurological functioning in the basal ganglia because MRI scans look at anatomy, i.e., tissue density and damage, water displacement, inflammation of tissue (and not the neurological function of this part of the brain). PET scans, or Positron Emission Tomography scans, may be used instead of, or in addition to, magnetic resonance imaging (MRI) scans. This gives anatomic andfunctional information.

The PET scan works by using PK-11195, a radioactive material, to target the inflammatory cells of the brain.  This chemical is attracted to inflammatory cells and gives information on how the basil ganglia are functioning. Some of the PET scans on P.A.N.D.A.S. children are identifying inflammation in the basal ganglia. This may be useful as the long awaited confirmation of P.A.N.D.A.S. that parents have sought.

Dr Chugani said, “By finding PET scan evidence of abnormality in the basal ganglia, we may now have a biomarker for P.A.N.D.A.S. Indeed, following IVIG in some P.A.N.D.A.S. subjects, the abnormality in basal ganglia has gone away on repeated PET scan. These studies are rather preliminary, on a limited number of subjects, and much more work needs to be done.”

*Please note that Dr. Chugani is not a “PANDAS doctor”.  However this research can really make an impact in the future of diagnosing PANDAS.