Tuesday, December 22, 2015

PANS, Lyme and delayed treatment


A lovely 17-year-old young woman presented to my practice about a year ago.  The patient’s mother has a history of Lyme disease and belies that she transmitted Lyme to her daughter in utero. Maybe. The patient suffered with a number of severe infections early in life: pneumonia, age 2 months, periorbital cellulitis age 4 months, chronic tonsillitis age 2 and chronic mycoplasma infection.  Something different also happened at age 6. She experienced an abrupt onset of tics and Tourette’s syndrome. Her pediatrician diagnosed PANDAS and prescribed a course of amoxicillin which seemed to be only somewhat helpful. After 10 days the pediatrician refused to prescribe additional antibiotics because tests for Strep were all negative: rapid strep test, throat culture, ASO titer and aniti-DNAse B.   See a psychiatrist, not PANDAS. Her enlightened pediatrician was aware of PANDAS, pediatric autoimmune neurological disorder associated with streptococcus (grp A, B-hemolytic). The hallmark of the disorder is that it comes on suddenly and is associated with strep. It is an autoimmune condition. Although even this is hotly debated, at least some pediatricians believed PANDAS required long-term antibiotics. The patient's mother always suspected Lyme. PANS (pediatric autoimmune neurological syndrome -- without the strep) was an emerging concept. L:yme and other infections could be alternate culprits. At age 12 the patient experienced transient swelling of a knee and soon thereafter developed severe neuropsychological symptoms which became progressively disabling. My patient developed a strange ailment: confessional OCD, characterized by telling her mother repetitively the same horrible thoughts: “Would I be punished (go to jail) if I drown the cat?” you get the idea. 
The patient subsequently developed frontal lobe symptoms (like pseudo-bulbar dementia). For example, she would burst in laughter or tears, uncontrollably, for no good reason. Despite all, my patient managed to do well in school until 7th grade. Then her grades took a nose dive from As to Ds.

Mom had daughter tested for Lyme:  10/10 IgG bands on the CDC surveillance test. Her pediatrician made little of this. Mom took her daughter to a variety of Lyme doctors who prescribed a parade of antibiotics and even Mepron for possible babesiosis. None of this was helpful.

Shortly before she saw me a neuro-pediatrician told her about a test from Moeculera Lab which measures anti-neuronal antibodies and is a marker for PANS. Mom had the costly test run and the results were resoundingly positive. More than one Lyme doctor refused to look into IVIG. Getting IVIG for PANS is difficult.  Most insurance companies refuse to cover it because the FDA says they can.  Luckily we also found a defect in humoral immunity via pneumonia vaccine challenge and the insurer now agreed to cover the cost of IVIG (not for PANS).

My patient was prescribed a combination of continuous antibiotics and IVIG.

One year later. She is essentially back to normal. No OCD or inappropriate behavior. Getting straight As in school. Well-adjusted and doing great.

Important note. The patient had a tick bite on her ear age 2.  Lyme can be transmitted by a deer tick in a child in a thin skin region, such as the ear (case report at ILADS conference several years ago) in as little as 4 hours.  Early life severe infections may be explained by an immune defect, shown to be present, not Lyme), My patient had normal growth and development and had no learning disorders.

In my, albeit somewhat limited experience, children who acquire Lyme in utero have autism spectrum disorder (Asburger’s) and/or a learning, developmental disorder.  

The Hallmark of PANS is that it hits suddenly: one day your child is normal and the next neuropsychiatric symptoms occur.

My important take away message is that even though many years passed between the onset of OCD etc... and IVIG therapy, the IVIG therapy may still be very effective. 

I currently have a 31-year-old male patient which a very similar tale, disabled and dysfunctional. Because of money problems the Molecular test is still pending.  He has been treated by numerous Lyme doctors over a period of 15 years. Antibiotics alone have never helped. The diagnosis had never been considered – until now.  

2 comments:

Christian said...

Dosing IgG might, in the case seroconversion failure from IgM to IgG in latent or cured infection actually help the immune system to convert permanently. Whether this actually cures any infection or just tilts the immune system in the right direction remains to be answered. I think a whole lot of odd neurological diseases and ailments could benefit tremendously from IgG treatment. But as usual, cost is prohibitive. And doctors, even when granted the resources are extremely reluctant to prescribe it.

http://www.ncbi.nlm.nih.gov/pubmed/11843914

Killing Lyme and Lyme disease is a question of reaching CNS, IgG molecules en masse does just that. A lot (if not all of these patients) seem to have low IgG responses even though a massive borrelia infection was/is present. If not adequate quantities can be reached, killing potential will be limited too, especially in the CNS, even though the CNS synthesizes it's IgG molecules too.

Maybe teasing the body to produce a stronger humoral response would be sufficient too. Thats why stories like "Bee Venom Therapy" fascinates me. I do not believe in the killing effect of melittin even though such an action probably exists, but rather the induced response the body is producing against the invading molecules.

Humoral deficiency or b-cell deficiencies are key,regardless of the patient being infected or not. Most borrelia infected patients seem to cope with the infection just fine (non symptomatic atleast).

valentina said...

Based on what I have read on multiple Lyme and Lyme-related support sites, it has slowly become my steadfast belief that PANS/PANDAS is more resultant of a Bartonella infection than a Lyme one. I am not saying that Lyme cannot create the compendium of neuropsychiatric symptoms that Bartonella can. but it does seem that Bartonella is the more aggressive and destructive of the two infections, and that it is more often associated with more marked multiple neurological and neuropsychiatric issues than Lyme. A combination of the two infections can also result in severe havoc being wreaked on the brain. Bartonella is also known to result in autoimmune encephalitis, often mistaken by (ignorant) mental health professionals for schizophrenia and other forms of mental illness. This usually results from years of untreated Bartonella.