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 7^th 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.