Case Study: Approaching PNES

[Divine Furor]

For the residents, how many of you have personal experience with diagnosed psychogenic non-epileptic seizures? Or really psychogenic disorders of any kind that you or an attending diagnosed and then pursued to treat. I guess I'm asking "What would YOU do?" with the following, highly-disguised, patient.

Right now, we have a 52-yo AAF with a h/o extensive cosmetic surgeries and depression who was hospitalized on neuro/med for seizure activity. 6 months ago she began experiencing automatisms, like blinking rapidly and making clicking noises in her throat several times a day. 2 months ago she noticed clonic spasms of the UE, then LE, and received the bells-and-whistles Neuro workup at a nearby, well-respected regional center. No findings. 24-hr-EEG with no seizure activity. MRI of everything normal. Started on Keppra 500 TID. Sleep studies normal. Yesteday she was unpacking one of her college-age children's bags at home when she noticed she was perseverating with meaningless actions (repeatedly closing doors, etc). Then she entered an hour long tonic-clonic state with barking vocalizations. Her children called the bus and it took a horseload of Ativan (10 mg) and Dilantin loading to "break" her status.

Key fact: She retains perfect consciousness through all of this. There is no classic post-ictal of any kind. She remembers everything that is said.

Psychosocial: Her depression was attributed to her husband's repeated infidelity, after leaving his first wife to be with her. She had been on Prozac 60mg until two years ago, when it was d/c due to "getting well." All of her surgical activity and a grueling workout schedule with supplements too numerous to count initiated at the time of discovery of the infidelity, four years ago. She is persistently evasive when asked directly about her home life or psychiatric history. She denies anxiety but her affect, physical demeanor, and thought content reveal extensive preoccupation with her physical health and how she needs a definitive answer to her seizures but expresses apathy at pursuing any non-neurologic workup. I should mention at this moment that she was in healthcare previously.

Labs: Only significant for a CK of 1400. So she is convulsing, and having witnessed one event I can say with certainty that her extremities undergo asymmetric spasms (R more than L). But she responds to vocal commands (such as "open your eyes") while the contractions continue.

Meds prior: Keppra as I mentioned, Diazepam 2mg for flying, and an exhaustive list of vitamins.

Differential: Frontal lobe seizures, possible hyperkinetic, vs. PNES, either from conversion or factitious. My money is on the psychiatric diagnosis, even though it is a dx of exclusion.

Where do we go with her that would be helpful, ethical and appropriate?

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[nitemagi]

Hmm, very interesting. My area of interest is conversion disorders, though I haven't yet been able to carve out enough research time for my own projects.

And always keep in mind advice on sdn isn't to be considered medical advice.

So I have multiple answers to your question.

1. There's the standard, commonly used solution, which is to give "care as usual," essentially treat it as if it is neurological, with some suggestions peppered in about the improvement course. Suggestion and reassurance is typically effective in conversion. Less so in factitious. Some say this type of care is a waste of resources or deceptive in that we're pretending the condition is "organic." Take it as you like, this is traditionally how I've seen conversion and factitious cases dealt with, with varied success. If it's blindness, for example, you might say "well this should get better in the next week doing exercises, and you'll notice first some shadows, then colors, then shapes, then normal vision." Change it for weakness and you give physical therapy with a short hospital course.

2. Next is I think one of the more interesting. I read about it in the Oxford textbook of Psychiatry, and some refer to it as a double-bind intervention. Essentially you say to the patient "Your condition is either organic or due to a psychiatric condition. If it's organic, then this pill [placebo] should cure it. If it doesn't, then you need to see a psychiatrist and/or therapist on a regular basis." Often, at least as is theorized, they prefer to have it be organic rather than the stigma of it being a mental illness, and so get better. But if they don't you have a solution as well. Which leads to...

3. My current intervention, which requires some expertise or guidance. Essentially psychotherapy, hypnosis, or an amytal interview. Amytal interviews usually requires someone with expertise administering it, maybe even an anesthesiologist, but should induce quick resolution of symptoms. Be careful though as there's a case report of suicide following this. Even though DSM-V will be getting rid of the psychological reason for the symptom, it's probably still there. We just shouldn't use it as a diagnostic criteria (because you can't always figure it out). I'm a hypnosis guy myself and conversion patients are typically very hypnotizable. Factitious will likely be more resistant. Try finding the Hypnotic Induction Profile online. That can get you started.

 

[Divine Furor]

Thanks for your thoughts, nitemagi. It's worth reiterating that none of this is gospel for treatment, and I wouldn't construe internet advice (however well-intended) as a solution to what is proving a difficult case.

Elements of suggestions 1 and 2 had occured to our team: so far 1 has been the dominant paradigm and 2, while tempting (to essentially "call out" the psychopathology with an ultimatum), doesn't seem entirely ethical. Plus, the patient knows JUST ENOUGH of medicine to be dangerous and difficult to fool with a pill or absolute treatment approach. The "organic or not" approach was tried, to an extent, by the neurology team that prescribed the Keppra. It had an efficacy window of about 2 weeks before the symptoms recurred and were notably worse. I'm not so worried about coddling the condition by continuing to treat it as organic, it's just that the medical interventions pose some danger: high-dose benzos, high-dose antiepileptics....and that CK is coming from somewhere....

Item 3 is really interesting: I will look up the Hypnotic Induction Profile. I wish there was a way to make a psychiatric overture to a patient like this without the concomitant alienation that occurs ("You think I'm crazy" or "It's all in my head?") when you utter the words.

 

[OldPsychDoc]

Play the "Stress" card: "You know, I'm not sure what's actually causing this, but it sure seems to get worse when you're having to deal with a lot of emotional issues..." Throw out some mind-body mumbo-jumbo, dazzle them with big words like "neuroendocrine" and "HPA axis dysfunction", move toward starting your psychotropic/psychotherapeutic combo platter of choice with the goal of having this disrupt their life less often.

 

[loveoforganic]

Some relevant lit attached in case you don't have access.

What I find interesting about PNES is how it doesn't seem to be consciously controlled behavior, but those with PNES tend to conveniently have their seizures in locations where they won't injure themselves.

 

[nitemagi]

#2 has been criticized as being potentially unethical by some clinicians with whom I've brought up the idea. I think treating it as if it's real when you know it isn't is in some ways equally deceptive and unethical.

I think the way to present the idea of a psychological intervention if it's therapy is "I know you're having a lot of distress over this problem, and talking with someone may help you better manage that distress." For the hypnosis, it depends on if you believe that the unconscious [however you want to define that] has the ability to change any level of physiological symptoms, which I would argue it does. Then it's a matter of selling that to the patient.

 

[nitemagi]

Some relevant lit attached in case you don't have access.

What I find interesting about PNES is how it doesn't seem to be consciously controlled behavior, but those with PNES tend to conveniently have their seizures in locations where they won't injure themselves.

That's true for all of conversion disorders. Look up Astasia-Abasia, for example.

 

[whopper]

I've ranted about this before. The new "PC" term, "psychogenic non-epileptic seizures," is not appropriate.

1) It's not a seizure
2) It might not be psychogenic. The jury is still out. Some people believe that all, what I'm going to call it which is more accurate, seizure-like episodes are volitional and are a form of malingering or factitious disorder. Others believe it is psychogenic and a form of conversion disorder, others believe it could be either depending on the person, and it is also possible that in some people it is psychogenic, but they may also exaggerate or fabricate a seizure-like episode from time to time.

I'm not advocating one vs the other because there is no consensus backed by hard science. I am, however, saying that to say it is psychogenic when this is not proven is not good practice although it's starting to be be accepted as a term.

3) Non-epileptic? Several people with seizure-like episodes that are not seizures have an epileptic disorder. There is a strong correlation between both disorders and this merits the question that there may be some association between them.

This to me is like the Holy Roman Empire ruled by Charlemagne. It wasn't Holy, it wasn't Roman, and arguably it wasn't an Empire, yet it was called that.

But to get to what you asked...


1) Psychotherapy: whether or not this disorder is psychogenic or fabricated, the treatment is the same, psychotherapy. This is better left to outpatient since it could take several weeks to months before results are seen.
2) Psychological testing: MMPI among other tests
3) consider a serum prolactin level, one for a baseline level and the other could be taken if during the seizure or within 20 minutes of it.

 

[Doc Samson]

Before you offer any treatment for PNES, you need to diagnose it first - that means vEEG until an episode is captured (even if it's more than 24 hours). In my institution we routinely monitor for a week or more in order to capture an episode. Anecdotally - in my experience the patients with the most dramatic "obvious PNES" presentations end up having frontal lobe epilepsy (which you accurately list first in your DDx).

 

[Divine Furor]

I've ranted about this before. The new "PC" term, "psychogenic non-epileptic seizures," is not appropriate.

1) It's not a seizure

Great point, one I hadn't thought of. But if we had to give a term to people who either consciously or unconsciously (or both) present with seizure-like activity as a representation of their psychological distress or unresolved conflict, what would it be? And I'm curious what kind of hard science we could find that would lend veracity to one overarching term?

Oh, and can you spare one more moment for med student education: why the prolactin level?

 

[Divine Furor]

Before you offer any treatment for PNES, you need to diagnose it first - that means vEEG until an episode is captured (even if it's more than 24 hours). In my institution we routinely monitor for a week or more in order to capture an episode. Anecdotally - in my experience the patients with the most dramatic "obvious PNES" presentations end up having frontal lobe epilepsy (which you accurately list first in your DDx).

And to tie this discussion neatly up for now--she was transferred to an inpatient unit for at minimum 3 days of vEEG monitoring this afternoon, fortunately with their EMR linked to mine. So perhaps I'll be able to shed some light on what happened in a few days here. Thanks for indulging the discussion.

 

[IAmAUser]

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[nitemagi]

Great point, one I hadn't thought of. But if we had to give a term to people who either consciously or unconsciously (or both) present with seizure-like activity as a representation of their psychological distress or unresolved conflict, what would it be? And I'm curious what kind of hard science we could find that would lend veracity to one overarching term?

Oh, and can you spare one more moment for med student education: why the prolactin level?

Prolactin is elevated in the immediate period after a seizure. You literally have to have the phlebotomist waiting, because if you wait to order it until an episode, they never get there in time. As whopper said, within the first 20 minutes.

 

[whopper]

why the prolactin level?

Yes as mentioned above.

However, to bring it all together so it'll stick in your memory....
(A professor once told me a neat and novel idea. If you actually understand why it's happening, only then does it stick in your head! Wow, after so many professors cramming data without explaining, someone finally said something that made sense!)

Several hormones fluctuate based on the specific state of the person at the time. E.g. testosterone could rise or fall based on the results of football game, seeing a hot nekked female (or male) taking off her (his) clothes, or having a real-life Crying Game situation in front of your eyes where you all of a sudden notice a prominent Adam's apple on someone you thought was that hot nekked female.

Why prolactin? Here's why.

Prolactin in almost everybody stays within a very specific and defined amount. Unlike other hormones, it does not transiently go up or down in a dramatic manner. The only situations where prolactin goes above normal are 1) when the person is pregnant or breast feeding, 2) the person is on a dopamine blocking medication such as an antipsychotic, and even when this happens it does not go up by much, 3) there is a pituitary tumor, 4) specific and very rare neurological instances such as neurologic syncope, or 5) the person actually had a seizure but that seizure must have affected the pituitary to some degree.

The convenience is that all 5 situations are highly rare, and even if someone does have 1 or 2, they will not dramatically raise the prolactin levels and can still give results on a lab test that will be distinguishable between a seizure vs no seizure.

So okay, what happens? In a seizure, there should be an elevated prolcatin level because the electrical activity will cause the pituitary to produce prolactin at a dramatically raised level that pretty much never happens except in the four conditions.

Exactly how much should it be raised? The rule is 2-3x the baseline depending on the study. A baseline could be obtained after the event if there is not a previous prolactin level. If the person is on an antipsychotic, don't expect any elevated prolactin that fits the 2-3x above baseline rule.

The prolactin level must be obtained either during or within about 20 minutes of the seizure. Some sources have differing durations such as 30 minutes. During that period of time the body's enzymes will start to degrade the prolactin in the blood which will in effect render a serum prolactin level useless if the treatment team waits too long.

Problems with the serum prolactin test: 1) Video EEGs are a more accurate method of testing and should be pursued if available 2) it's difficult to get an serum test during or right after a seizure. 3) The pituitary must be affected and if the seizure is not a grand-mal seizure, the seizure may have not affected the pituitary. Serum prolactin levels are of little use where the person shows behavior indicative of a partial seizure. 4) Although extremely rare, syncope could look like a seizure and will not elevate prolactin.

So far in 4 years of residency, 1 year of fellowship and 1.5 years of attending practice I've had six patients where I was very confident the person did not have real seizures and it was merely seizure-like episodes. In one of them, it was verified by an EEG., In one of them, the person stopped having the episodes after psychotherapy started, and the person showed several signs that he was malingering and had factitious disorder (yes both). E.g. anytime he didn't get anything he wanted, he claimed to be suicidal. We did a SIRS which highly suggested he was malingering symptoms of mental illness and he had a history that just fit the profile of someone with factitious disorder (institutionalized since childhood, several special needs that were provided by services not by his family, etc). My psychologist gave him treatments designed to enhance delayed gratification and self sufficiency. His seizure-like episodes stopped.

In another case, the person admitted to me that she intentionally faked seizures. This person also had combined factitious and malingering disorders. She also had a rare genetic condition where she craved attention (William's Syndrome). She told me she had a seizure and noticed how much attention she got from it and from there she learned to fake seizures to be the center of attention. She only started to improve from her very serious Axis II problems after the psychologist and I ignored her when she made pathological attempts for attention and we focused our psychotherapy on having her work on self sufficiency.

That and a bunch of other factors....E.g. when she had her seizure-like episodes, I'd tell the staff members in a very loud manner, "okay ladies, we're going to have to get a serum prolactin level, that means she's going to have to get stuck by a needle!" She got up and told us she felt better and started begging us to not have her stuck for a blood draw. (An M-FAST and a SIRS showed she faked symptoms of mental illness.)

 

[Therapist4Chnge]

In another case, the person admitted to me that she intentionally faked seizures. This person also had combined factitious and malingering disorders. She also had a rare genetic condition where she craved attention (William's Syndrome). She told me she had a seizure and noticed how much attention she got from it and from there she learned to fake seizures to be the center of attention. She only started to improve from her very serious Axis II problems after the psychologist and I ignored her when she made pathological attempts for attention and we focused our psychotherapy on having her work on self sufficiency.

If you really want to confuse things, try and evaluate a patient that transitions between factitious, malingering, conversion, etc. Psych assessment can really help shed some light on a situation, and I definitely prefer it before attempting a double-bind dilemma/intervention.

 

[whopper]

psychotherapy on having her work on self sufficiency.

If you really want to confuse things, try and evaluate a patient that transitions between factitious, malingering, conversion, etc

One of the advantages of working in a long term facility is I got weeks if not months, even years to check out a patient. I can spend hours on a patient a day, and I can have a psychologist do a battery of psychological testing, I can order psychiatric consults from top people in the country (E.g. Henry Nasrallah, Guttmacher award winning forensic psychiatrists), I can present the case in front of a team of several psychiatrists for review, in addition to plenty of other interventions not typically available in other psychiatric settings (TOMM, HCR-20, STATIC-99, MMPI testing, etc).

In this type of setting, I feel very confident in finding people who are exaggerating/fabricating mental illness and yes in several of those cases, some of these people did have real issues. Just because someone has a headache doesn't mean they are having the worst headache of their life.

 

[nitemagi]

For factitious, the data isn't super-supportive of confrontation as being successful. I think confrontation leads to hostility, and the risk of switching providers. There's a good study that actually looked at it sitting around somewhere. A small percentage of chronic refractory pt's who were high utilizers became asymptomatic, though.

 

[whopper]

For factitious, the data isn't super-supportive of confrontation as being successful.

Agree though sometimes you really have no choice but to tell the patient. I try to do it in a nonjudgmental manner if it comes to that. For example, in a forensic setting, I have no choice but to tell the Court my opinion, and in that setting I believe it's better for me to tell the patient instead of hearing it in Court while I testify in a manner they may interpret would be against them.

I do not let the person "get away" with malingering/factitious behaviors. The Williams Syndrome patient I mentioned was placed on medications for ADHD, and she did have that disorder. The first day on the medication she claimed to not to be able to walk without being lightheaded. We measured her BP lying down and standing, both were about the same. She showed no behaviors that were indicators of her symptoms.

I told her if she was truly lightheaded, we could not let her out of the unit. She stopped complaining of being lightheaded because she could not attend one of her favorite groups that was off-unit. She claimed she could not raise her right arm without being in extreme pain and demanded a physical therapist. I got a ball played catch with her where she was showing no problems, then I told her I was going to write a note recommending she not get physical therapy. She claimed to be suicidal and swallowed some laundry detergent. The brand we had on the unit was non-toxic. I gave her a box of it and told her she could swallow as much of it as she wanted and to have a party with it.

With each of my responses, her specific problematic behavior immediately stopped. You can respond spade-to-spade without getting confrontational.

In regards to pseudoseizures, a neurologist told me that one time a patient actually lunged at him and strangled him after he told her the seizures were not real. Telling her the seizure-like activity a fake seizure in a brusque manner is not appropriate, in part because it could be psychogenic and a form of conversion disorder.

 

[Therapist4Chnge]

Detergent Party! It will be the new Four Loco.

 

[Fermata]

All three times I've seen a diagnosed or suspected PNES has been in an ER setting and wasn't the reason for triage. There was a spectrum of flavor from psychogenic to malingering. All three had varying levels of insight with one even stating that he thought his own problems were psychosomatic....

All three at presentation were already loaded up on heavy psychotropic cocktails.

All three did not enjoy the sternal rub.


I do think at this stage that pseudoseizures is a more honest label than PNES. It does make me wish I had a greater grasp of psychometric testing.

 

[whopper]

Although extremely rare, syncope could look like a seizure and will not elevate prolactin.

Error, that should be the syncope could elevate the prolactin. I was thinking the right thing but I did not type the right thing.

As for sternal rubs, one thing I also do is tell the treatment team to not exactly use the smallest needle when obtaining a serum prolacting level when the person is exhibiting seizure like behavior. We use the same needle anyway, but the person hearing "Nurse, use the largest needle possible, you know the one that is very painful" has caused some of the patients I mentioned above to stop their seizure-like activity and start begging to not be stuck. That intervention has stopped one of the patients from exhibiting any future episodes while I had him.

As for the term "pseudoseizure" and the movement to remove the name with a more PC term that is actually prone with error, some patients are most definitely faking seizures. How could one argue that faking a seizure is not impossible, especially when several people with this problem even admit they were intentionally faking it.

I have no problem with those wanting to point out that this phenomenon may be a form of conversion disorder, but I disagree with the movement to make it out that all of these episodes are that. When you have patients that stop their behavior when they know they will get stuck with a needle, patients critiquing and even giving advice to others on how to fake one (and yes I have witnessed that), and patients telling me they intentionally faked them when psychotherapy for malingering was getting somewhere.....