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One of the reasons I chose psychiatry as a specialty was to address the needs of those who could not be heard, or who were being ignored or demeaned. These included the people who were being written off as crocks, hypochondriacs, whack jobs, and just plain crazy. The examples I can give are legion. Amidst my interests in the past 20 years is the identification, understanding, and treatment of PANDAS. These individuals have been ignored and demeaned consistently.
In the present medical world, it has become very difficult for individuals or small groups to make an impression on corporate and academic medicine. Once upon a time, clusters of case reports and interesting observations would be given credence, voice, and often lead to new discoveries. Not so now.
There was a time when the correspondence section of The New England Journal of Medicine was full of letters from physicians who reported observations and musings. Physicians around the country and around the world responded. Often, this led to the identification of new or refined diagnoses, or new or refined treatments. It was a pre-digital chatroom.
Alas, those days are gone. Nowadays, discoveries can only be made by major academic centers. The same for new or novel treatments. This is why PANDAS has languished in the backwaters, still called by many a sham disease. The idea that independent doctors around the world have anything to offer is ignored. The academics determine what is or is not important. They will decide, when they find it timely, what is and what is not PANDAS. And this is why the internet has become the “letters to the editor” section for most of us.
I have treated childhood, adolescent, and adult PANDAS for almost 20 years. Yet many of my patients and I find the evaluation and treatment of adult PANDAS almost a Sisyphean endeavor. While the disorder is acknowledged in children and adolescents, its occurrence in adults is denied. It is as if it magically goes away at the age of 18 and cannot occur, recur or continue. This is absurd. And to compound the contorted reasoning is the assertion of some academics that PANDAS is very, very rare. Another faulty bit of thinking.
Over the years, I have seen this disorder present in myriad ways. The simple ones are acute onset of new neuropsychiatric symptoms after a sore throat. The more subtle and difficult cases often involve obscure neuropsychiatric symptoms. Some of these are missed, some are ignored. and some are attributed to something else.
The most dramatic are the agitated, aggressive, and violent patients who have outbursts, which they cannot control. This may have been present since childhood or may have begun in adult life. Careful evaluation reveals that they have Tourette’s syndrome. Significantly, the symptoms attenuate dramatically or resolve completely after treatment of the underlying autoimmune disorder. This post is not about the evaluation or treatment of PANDAS. It is about the identification and validation of the problem in adults.
A colleague of mine, and an old friend, TW has two children, now adults, whom I treated for PANDAS about 15 years ago. Both had complete resolution of their symptoms after tonsillectomy. No further treatment was necessary. They have occasional mild flares, which are easily resolved.
My friend has always had a mild facial tic. We assumed it was PANDAS but did nothing about it since it was mild, not a bother, and his tonsils had been removed in childhood. Recently he had a florid and bizarre episode, which is worth reporting. The fascinating cluster of symptoms demonstrated in an adult is a casebook to understand this disease. Simultaneously his misadventures with emergency services and the emergency room illustrate the dismissive treatment offered to such patients.
TW developed a severe sinusitis with concurrent bronchitis. He was treated with repeated courses of various antibiotics, corticosteroids, nasal and pulmonary inhalers, and sinus irrigation. The acute infection subsided but there remained significant sinus inflammation. TW’s facial tic began to worsen from a slight twitch of the upper right lip to marked blepharospasm (blinking) with multiform spasmodic quivering and jerking. The intensity fluctuated. Everything was made worse by cold. Cold drinks or cold air set everything vibrating.
At the same time, he developed hiccups randomly, but always after a cold drink. They would last two to three hours.
Things progressed to stuttering—often severe. This was accompanied by dysarthria (difficulty with articulation), dysphonia (difficulty in production of sounds in the larynx), and severe word-finding problems. TW knew precisely what he wanted to say. He saw the word in his mind, could spell it in his brain, could “say” it in his head, but it would not be expressed.
Contemporaneously, he experienced some strange dizzy spells. After bending over to pick up something or to tie his shoe, when he stood up he felt lightheaded and dizzy. He thought it a peculiar dysautonomia. Variously his blood pressure would be elevated. At other times it would be very low. He queried me about this. I realized that he had Postural Orthostatic Tachycardia Syndrome (POTS), another expression of autoimmune neuropsychiatric illness.
Concurrently with all of this he took a short trip out of town. He experienced a bout of diarrhea that did not resolve. It dragged along for almost a month. It waxed and waned. His stool and flatus were very malodorous. He noted a strange taste in his mouth and his palate felt strangely coated. He was told by his wife that he had an unusual body odor.
The repeated antibiotic treatments had induced a case of colitis. This exacerbated the PANDAS. Now there were two active foci of infection driving the autoimmune disorder.
I received a concerned call from his wife. I knew immediately that all of this was PANDAS. Since I happen to be the go-to guy in this area for the evaluation and treatment of PANDAS I monitored TW. He was bemused but not frightened. He did not anticipate that he would wind up in the hospital emergency room. He made a call to our gastroenterology colleague who worked with me on previous cases of colonic PANDAS but things came to a head before his scheduled appointment.
On a Saturday evening in September after a walk in his neighborhood, hiccups developed. It turned out to be the coolest night of early autumn. Later he told me he had been sleeping with the windows open and no blanket. At 4 a.m. he awoke feeling very strange. His groin felt very cold and he could not feel his testicles. They were not there. His scrotum was tight against his perineum and very cold.
He waited a moment and felt again with the same result. This was something foreign. Quietly he got up and went to his den. He slapped himself a few times to try and make sure that he was not dead or hallucinating. He began to panic thinking he was having a stroke. He decided that he must go to the emergency room.
He tried to tell his wife what was going on but could not talk intelligibly. He called 911 and attempted to make himself understood to the operator. The bizarre speech and the stuttering made it difficult for the 911 operator to understand him.
The ambulance arrived. Speech was still a problem. Most of the paramedics’ questions were answered with shakes or nods of the head. He pointed to his groin but could not make himself understood. His blood pressure was quite elevated. EKG was normal. They ruled out a stroke because his bilateral grip strength was strong and equal and his facial movements symmetrical—if that is how they rule out strokes, they must miss a lot of them!
The paramedics determined TW had high blood pressure. Ignored were his numerous speech issues, multifocal facial tics, and his groin. They told him to call his doctor on Monday. He did not meet criteria for an emergency “run” to the hospital. They would give him a ride if he insisted. His wife insisted. They gave him a slow ride to the hospital and dropped him off outside of the ER at around 5 a.m.
At the entrance desk, he attempted to give information but still could not communicate well. One paramedic did come in and give the nurses information.
His wife arrived. She was on the phone attempting to get their internist, prominent at the hospital, but he was on vacation.
The attending physician enters. She listens as he stutters out the story, often having difficulty with articulation and phonation. Blood pressure is still high. She has heard of PANDAS. However, she has not heard of the groin problem but examines him. She is perplexed and intrigued.
She orders labs, EKG, Chest X-ray, scrotal ultrasound, and a CT of the pelvis and abdomen. Then she calls a urologist who identifies the strange phenomenon.
Retractile Testicles: The testes are drawn up into the inguinal canal and the empty scrotum tightens up flat and smooth. It occurs predominantly in boys but does occur in adult males. The most common cause is exposure to cold. Innervation is the genital branch of the genitofemoral nerve. It is a neuro-motor reflex.
TW asks the ER physician to call me. I give her a very quick summary, make some recommendations, and request to be updated.
The retraction is resolving. All test results are unremarkable thus far. Time for a CT of the abdomen and pelvis with IV contrast. The contrast contains iodine. Iodine is not good for the kidneys.
Over the next several hours in the ER, all of TW’s symptoms have begun to wane, albeit in a staccato manner, with brief flares of all but the retraction. His GI complaints of cramps and diarrhea began to increase.
After the CT scan is completed, TW is told to drink a lot of water. He has had no food or drink for about 20 hours and has had a liter of normal saline drizzled in slowly by IV. Given a jug of ice water—800ccs—and TW drinks it swiftly. He is given another jug and consumes that swiftly as well.
In a few minutes, TW begins to shiver. Then the facial tics go wild. He tries to speak. but it is unintelligible; stammering, dysarthric, dysphonic babbling with continuous word-finding problems.
TW’s bowels were agrumble. About 30 minutes after gulping the ice water, he went to the toilet. There was no formed stool. Just clear liquid. Curiously, the liquid looked and smelled like iodine. Or betadine. The inflamed bowel pulls in fluids to wash out toxins. In this case, it pulled in a lot of iodine. The result was that his antibiotic-induced colitis was resolved. The iodine had sterilized his colon.
After 10 hours, TW is discharged from the hospital and referred back to me and his internist. No new prescriptions. My recommendations for labs and stool cultures were not done.
With resolution of the colitis, there was a rapid diminution in symptoms. No further retraction. The sinus inflammation dwindled but did wax and wane, The tics and speech issues did the same in synchrony with the inflammation.
Clinically, TW did well. He was used to living with tics. Now he had to deal with the sinus inflammation and avoid excessive antibiotic use.
I reviewed the entire course. I thought through the affected neurologic pathways. I traced the nerve distributions of cranial nerves, phrenic nerve and the genital nerves. A fascinating case of PANDAS and POTS in an adult.
What are the takeaways? Well, the general attitude of the medical personnel, across the board, was consistent with the experience that patients with supposed psychiatric issues are given less than short shrift. Were it not that TW is a physician, and that the ER physician communicated with his friends/colleagues, he might have been deemed crazy, shot up with Haldol, and sent home. Or worse, certified to a psychiatric unit somewhere. I have advocated for patients for decades. I am gratified that I was there to advocate for TW.
If PANDAS does not occur in adults, just what the heck did TW experience? Conversion hysteria? Hypochondiasis? Malingering? Munchhausen’s syndrome? Schizophrenia?’ What convoluted explanation can explain this? In medicine, we are obliged to make the most parsimonious diagnosis. Is there one unifying diagnosis? Of course there is. PANDAS/POTS. Physicians must remember: If you do not think of it, you do not look for it. If you do not look for it, you do not find it. PANDAS occurs in adults. It may be new onset or ongoing. Acute, or sub-acute since childhood. This is irrefutable.
The notion that PANDAS is impossible after age 18 is absurd. Those who choose to deny this are either close-minded or gagged. I find the most open-minded free thinkers to be in private practice. They are not enslaved in a corporate or academic center with “best practices” and diagnostic algorithms. If a diagnosis is not coded in their EHR, they cannot consider it. Hence our ER physician made no mention of PANDAS or even an autoimmune disorder in TW’s discharge diagnoses. The systems are vertically integrated and only the top dogs can own a new thought, even if they never thought of it themselves.
In my busy hospital practice, I diagnose PANDAS more than once per month in patients committed involuntarily. Often they have been aggressive, violent, or out of control. Usually, they have a collection of symptoms and diagnoses; most commonly bipolar, borderline, and PTSD. They actually have PANDAS-related Tourette’s. Occasionally there is an acute onset presentation with sudden OCD, panic, eating disorders, tics, and violent outbursts. The same occurs in my private practice. It is an uphill battle to help these people. And it is wearisome to educate a criminal court judge that a patient’s sudden aggression is a symptom of a brain inflammation—particularly so if the court-appointed “expert” psychiatrist has not heard of PANDAS.
Review, if you will, the litany of symptoms experience by TW. All developed in a man of in his late 60s. How about that? He had a smorgasbord of PANDAS/POTS: multiform facial tics; POTS; hiccups; throat clearing; stuttering; dysarthria; dysphonia; word-finding problems; retractile testicles. He had two loci of infection/inflammation to generate the autoimmune response. He had a positive family history of PANDAS.
Here are two final observations that may help understand and treat patients. Firstly, it has been known for decades that corticosteroids are useful in the treatment of central nervous system (CNS) inflammation from cerebritis, encephalitis, vasculitis and the like. PANDAS is an inflammation of the brain. Is there a wonder why corticosteroids are so useful in these cases?
I have two cases in my practice now of adults who become “psychotic” during PANDAS flares. Paranoia, delusions, auditory and visual hallucinations, and explosive violent behavior occur. Anti-psychotic medications, all of which block dopamine do not work and tend to exacerbate all of the symptoms. A burst of corticosteroids with a slow taper resolves all of the psychosis.
Many years ago, I read an obscure paper on “steroid-responsive psychosis.” I have not been able to track it down yet. It described several cases of chronic, intense psychosis, generally schizophreniform in nature, which were not responsive to any antipsychotic pharmacotherapy. Treatment with prednisone cleared the psychoses. The authors were not able to find a source of inflammation such as lupus cerebritis, vasculitis, or anything else. The cases I describe are identical to those referenced in this paper. Might it be that there is a significant subset of patients with chronic, refractory psychotic illnesses who have an autoimmune cerebritis secondary to PANDAS? I think that is the case. To prove this, some blood tests need to be done on these patients. If I am right, it will rock the establishment.
Secondly, TW’s colitis was eradicated by the surge of iodine osmotically sucked into his colon. Might an iodine treatment be a new and useful tool in the treatment of these afflictions? This is a clinical pearl that may have broad applications in the treatment of antibiotic-induced colitis such as the notoriously problematic clostridium difficile.
There is a pertinacious resistance to the acceptance of any new idea or diagnosis in the minds of many in medicine. The various reasons are not the point here. The point is that there are many people afflicted with life-changing illnesses. Often, these illnesses are ill-defined: psychosis; bipolar; borderline; malingering, etc. They seem not to respond to treatment. They are difficult patients.. They do not want to get better. All of the usual tropes.
What if all of these patients had a few blood tests? These tests are inexpensive and common ones. How many cases of PANDAS might be diagnosed? Treatment of these patients might be expensive on the front end. Tonsillectomy at times. IVIG for an uncertain period of time. But what is the calculus of changing the course of chronic, hopeless patients, disabled, non-productive, absorbing huge resources into healthy, productive folks who work, raise families, pay taxes and premiums, and maybe need IVIG or another therapy once a month? To the actuaries and bean counters, I submit that the latter group would turn a lot of loss into a lot of gain.
