As with so many of our Lyme patients, he had no recollection of tick bite or bull’s eye rash. Blood test for Lyme was borderline trending negative, also common in Lyme patients. He grew up in a Lyme endemic area and use to spend a lot of time outdoors.
His symptoms have included: exhaustion, malaise, chills, night sweats, temperature dysregulation, weight loss, poor appetite, trouble sleeping, fragmented sleep, poor endurance, prolonged recovery of exercise, rashes, spots, flushing, blurred vision, double vision, dry eyes, eye pain, tinnitus, dental pain, cough, breathlessness with exertion, air hunger, palpitations and orthostatic symptoms, abdominal pain, nausea, diarrhea, painful swallowing, painful urination, frequent urination, urgency, joint pain, muscle pain, neck pain and back pain, plantar pain, dizziness, vertigo, poor balance, weakness, pins and needles, loss of sensations, depression, anxiety, depersonalization, distractibility, trouble staying on task, confusion, disorientation and getting lost, impaired thinking and brain processing, mixing up words, chemical sensitivity, light and sound sensitivity and food sensitivity. Prominent symptoms include sweats, air hunger and unprovoked bouts of tearfulness.
He gives a “good story” for Lyme, Babesia and Bartonella.
When I first met with him, the family was in crisis. He lives with his mom and step-dad. He was scaring mom to death and “mad” in both senses of the word; there were frantic calls to 911 and hours were spent in ERs. Psychiatric therapies were consistently ineffective. He was the picture of Dr. Jekyll and Mr. Hyde. Rages, with kicking, screaming, throwing inanimate objects and punching through walls occurred regularly. He remembered the episodes poorly. Antipsychotic meds were unhelpful.
He has a prior history of psychiatric illness and a history of 3 psychiatric hospitalizations in his 20s because of similar symptoms and suicidal thinking. I asked some specific questions: “When did it start?” Around age 13-14. “Were you normal before then?” Absolutely, had been an A student, an athlete, well adjusted. “Is there a family history of mental illness?” Some depression. “How did it start; do you remember the first symptom?” Anxiety. “How about OCD?” yes. Thoughts repeat over and over and I can’t control it. “Behavior changes?” Yea. Nervous habits, like chewing finger nails – to the quick.
Then, I asked this question: “Think carefully. Did it start suddenly? Were you normal one day and symptoms appeared abruptly the next day?” Not sure. A lifetime ago. But I think it happened that way.
Then another question: “did you have a lot of infections as a child?” Yes. I had a lot of ear and throat infections. An ENT suggested I get a tonsillectomy at some point.
Lyme test was equivocal. Coinfection testing was negative. There was a positive anti-DNase antibody
Discussion: He has PANS, pediatric acute neuropsychiatric disorder which I believe is a unique form of autoimmune encephalitis. The original designation was PANDAS, pediatric acute neuropsychiatric syndrome associated with Strep. The hallmark of the disorder is that neuropsychiatric symptoms, prototypically OCD and tics appear overnight. A variety of infections can trigger the syndrome, including Lyme and other tickborne pathogens. In this case, Strep may have been an inciting cause. Multiple pathogenic organisms can pile on, making the illness more challenging to treat. I consider the possibility of an immune deficiency issue, previously called CVIDS, chronic variable immune deficiency, now defined more narrowly and called a select IgG subclass deficiency. Demonstration of this is necessary for IVIG approval, the treatment of choice for PANS. I can’t ignore the Strep issue. Perhaps he doses need a tonsillectomy. Strep can hide in folds and crypts of tonsils and he is referred back to his ENT. Based on clinical diagnosis, I believe he suffers with Chronic Lyme disease, Bartonella and Babesia. I always am mindful that my diagnoses are hypothetical and avoid anchoring to a particular diagnosis.
Needing a place to start, I focus on certain key symptoms, although the focus may change over time. He has a lot of constitutional symptoms – malaise, body temperature dysregulation, poor sleep, weight loss, night sweats etc. This tells me he is actively sick. It points to Lyme. It also points to Babesia, the vast and unrecognized epidemic. Night sweats, air hunger and spontaneous crying point in this direction. Bartonella is a frequent trigger for PANS. Specifically, Bartonella is associated with irritability, anger and rage. In addition, pain on the bottom of his feet and neck pain are clues. Bartonella is notorious for making psychiatric symptoms worse in the setting of a multi-organisms-tickborne syndrome.
Treatment. First, I must respond to acute, critical symptoms. Uncontrolled rage is the overarching issue. Even though it is not OCD per say, PANS drugs targeting OCD may work. (incidentally, numerous psychiatrist never got the diagnosis right, most recently a diagnosis of bipolar 2 had been proposed). Although I am not a psychiatrist, I must be familiar with the neurochemistry associated with specific tickborne disorders. I chose two drugs which worked very well. I lucked out. I say this because response to psych drugs, theory notwithstanding, is hard to predict. An old antidepressant, Anafranil and a higher dose of Prozac was highly effective. Psych symptoms quickly receded and became manageable. Anafranil is a tricyclic antidepressant known to work well for OCD, the mechanism not understood. Higher doses of Prozac may also work, and did in this case. This combination was highly effective. I then considered antibiotic therapy. It never ceases to amaze me how many patients previously treated for months and years were never treated with doxycycline. I really don’t think he has tularemia, psittacosis or Brucellosis. But he could have anaplasmosis, ehrlichiosis, rickettsiosis and mycoplasma. Doxycycline treats them all (first or second line). And – it remains one the cornerstone drugs for the treatment of Lyme disease, when combined with other agents. Following my own internal logic, I prescribed doxycycline. He immediately had an exacerbation of psychiatric symptoms. This quickly reverted to baseline when the drug was stopped. I changed to amoxicillin and he did very well. Amoxicillin treats Strep, and, it has poor penetration through the blood brain barrier, a desired goal at that point. He is responding and getting better, week by week. He states, this is the best he has felt in years. Additional antimicrobials will be introduced with great care. I have ordered: brain MRI (negative) brain SPECT pending. Sometimes an MRI may show white matter lesions or other unexpected findings. The SPECT scan is useful and is frequently abnormal in autoimmune encephalitis. The degree of perfusion defects may have some prognostic significance. I must look for an underlying immune deficiency disorder. A history of chronic respiratory infections in childhood is a tip off as well as the presence of persistent, disabling tickborne infection. This requires he receive a Pneumovax (pneumonia vaccine) challenge test. A baseline antibody test of pneumococcal serotype antibodies is obtained; if the baseline test shows little or no immunity to the serotypes, a Pneumovax, (Strep pneumonia killed vaccine) is administered. A repeat test for serotype antibodies is repeated in 4 weeks. If there is little change in a specified number of serotypes, he may qualify for IVIG, based on insurance company bean counters who base approval of IVIG based on limited, specific test results. IVIG is the treatment of choice for PANS, but Mainstream Medicine and insurance companies do not recognize the disease or its proper treatment . Other options, including steroids come in a distant second place. Some readers may be fearful of vaccines. Perhaps they have had bad reaction or heard something in the community . In my experience, the problem vaccines, occasionally associated with autoimmune and other toxic reactions are the DPT, influenza and HPV vaccines. So far, the Pneumonia vaccine has been tolerated OK. To be clear, I am not making a blanket recommendation for or against vaccines. I get a flu vaccine yearly and I recently had Tdap vaccine. A an aside, It is possible the diphtheroid toxins caused by diphtheria bacteria living in your nasopharynx pass into the brain -- and some think these brain toxins may be associated with Alzheimer's. This is supported by published literature. Just a thought.
He is thrilled and happy to share his story.
He is thrilled and happy to share his story.
Following the plan outlined, I hope he will continue to improve.