Functional neurological symptoms as initial presentation of Creutzfeldt-Jakob disease: case series 2022 Gómez-Mayordomo et al

Hutan said:
The videos won't play for me, but it's appalling that the caption of the first one points to the infantile speech of a patient, as if it was a confirmation of FND.
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It took a couple of clicks in the timeline bar and the play button in bottom left but they did eventually play for me, despite no login. You can also download the videos if you hover in the upper right of the video.

Having seen them I am none the wiser, particularly with regard to "infantile speech". I showed that one to my wife. She said the speech was very slow, as if the patient was struggling to speak and that the intonations were all in the wrong place and that it would sound very wrong to a native speaker.

The demonstrations of distractable tremors and movement disorders leave me non-plussed. It seems as if the whole concept is predicated on "real" / structural disease having to behave identically each time and everything else is "functional". Eg a spinal cord transection at mid thoracic level causes motor and sensory loss at that level and will examine this way each time.

I'm no expert but brain function relies on complex interneural interactions (with synapse assistance from supporting cell types). Some of this activity happens below conscious level and yet we can promote some things to conscious control. Eg we breathe without thinking, but we can take over rate, rhythm, depth at will (within limits). Other things like pulse/BP not so much, otherwise we'd all have been cured of POTS by the LP.

Why wouldn't these speech and motor capabilities alter variably under the influence of adjacent activity or structural pathology? This idea of rule-in signs being definitive for FND and nothing else seems so bizarre. Sure, let's have a diagnostic category of FND that genuinely isn't conversion / hysteria etc, but instead genuinely attempts to understand network interplay and dysfunction; or variable neurometabolic dysfunction. But how can you propose that any such diagnosis can not be wrong by virtue of a "test" (rule-in signs) and that that means all other investigations must stop? Particularly when you publish examples where this was wrong ("oh no, they just had FND overlay") — not to mention the vast numbers of long haulers being diagnosed with "FND".

Talk about "unhelpful illness beliefs", it's obtuse.
 
Another reference in this 2022 paper that I think is worthy of comment:
A Case of Sporadic Creutzfeldt-Jakob Disease Presenting as Conversion Disorder, 2017

The clinicians looked at this older woman, divorced, with few friends and yet denying depression, and assumed that she was a prime candidate for FND. (this literature is full of people either admitting they have depression, or being diagnosed with alexithymia (inability to know what they are feeling) as well as depression)
Ms. T was a 64-year-old Caucasian woman with no past psychiatric history and a past medical history of hypertension, hypothyroidism, and Lyme disease who presented with two months of new-onset worsening tremulous jerky movements of her right upper extremity, right-sided numbness, ataxia, headaches, and joint pain. The patient did not exhibit myoclonus or confusion. Her symptoms had occurred in the context of several psychosocial stressors. Ms. T was admitted to a general inpatient medical unit for further evaluation and treatment.
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Psychiatry was consulted within the first week of her hospitalization. Although she did not have a psychiatric history, the patient had been facing significant life stressors. She was divorced, lived alone, and had few confidants. One year prior, she had lost her mother and another close family member. In order to tend to her ailing mother, she had taken an early retirement from a job in healthcare and relocated to reside with her. She now wished to return to her employment. Nevertheless, apart from anxiety related to her health, the patient denied all psychiatric symptoms, including depression.
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During the first week of hospitalization, the neurology team observed that her tremors and other neurological symptoms were distractible, variable, and entrainable.
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Some tests were done, but not the ones that would have been most useful in ruling out CJD.
Based on the Centers for Disease Control and Prevention’s criteria for the diagnosis of sCJD, Ms. T exhibited cerebellar signs (ataxia) and extrapyramidal signs (tremors) at the onset, which, when interpreted in the context of impaired neurocognitive testing and an unremarkable diagnostic workup, arguably placed her in the “possible” sCJD category. The initial workup was extensive but only included CSF 14-3-3 levels. Other markers such as CSF Tau, S100b, and NSE could have aided. The sensitivities of these markers range from 73% to 86% [15]. Newer tests, such as real-time quaking-induced conversion (RT-QuIC) assays of CSF and nasal mucosa, have a diagnostic sensitivity and specificity of 96% and 100%, respectively [16]. Although there are no current disease-modifying therapies for sCJD, prompt diagnosis allows for appropriate palliative care to be initiated, provides some closure to the patient and the patient’s family, and reduces the risk of iatrogenic transmission.
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So, instead of that palliative care for her and her family, she was told that she had an FND and given therapy to fix her functional disabilities. They tried to put her on a drug for anxiety and depression, but she refused. It is awful to think about what this poor woman went through. The consultants left her to the therapists. She became worse.
By the end of the first week of her hospitalization, the patient was given a provisional diagnosis of a functional neurological (movement) disorder by the neurology service. Given her presentation, in conjunction with an unremarkable workup, this diagnosis was maintained by the psychiatry team. Though the patient denied symptoms of depression, it was posited that her significant psychosocial stressors could be contributing to her symptoms. Escitalopram was recommended as a low risk intervention for both anxiety and possible depression; the patient provided informed refusal. Her hypothyroidism and mildly elevated TSH were addressed by adjusting her levothyroxine dosage. Physical and occupational therapy were started, with the intention of addressing her functional disabilities.
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After two weeks of hospitalization, the patient remained ill. Since her presentation was thought to be mainly functional, many consultants became less involved in her care. Then, her condition further declined.
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So, a month after admission to hospital she was tested specifically for CJD, and she died a month later.
neuron specific enolase (NSE) levels, which had not been measured previously, were markedly elevated. Clinical suspicion for sCJD was high, despite only meeting the criteria for “possible” sCJD. Ultimately, a definitive diagnosis of sCJD was made via brain biopsy. The patient was eventually transferred to hospice care and passed away two months after her initial arrival to the hospital or four months after symptom onset.
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What I find particularly incredible is that the authors of this case study still think that an FND diagnosis might have been correct. For goodness sake, this woman had her brain disintegrating and they think she was manifesting her subconscious depression as physical symptoms? It's incredible to see the authors tying themselves in knots, still defending an FND diagnosis
It remains unclear whether the patient’s functional symptoms were an early manifestation of sCJD or a cooccurring phenomenon. Functional symptoms have been found in the initial stages of many neurological disorders
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Perhaps early prion mediated neuronal loss in particular brain regions led to our patient’s early symptoms, which were consistent with a functional neurological disorder. As the disease progressed, they could have resolved into clearer and more distinct neurological signs. If so, this could challenge our understanding of the mechanism behind functional illness and the binary distinction of mind versus body. Conceivably, subtle neurological changes may underlie pathological changes which eventually result in the phenotype of functional symptoms.
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This case challenges our perception of conversion disorder as an entirely psychological phenomenon and neurological disease as entirely biological [5]. If disease can exist in an immaterial, “functional” realm, how then can it influence a brain made of matter and mass and produce bodily symptoms? Perhaps the connections are subtler than thought.
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Or perhaps it's just that a sufficiently sensitive test was not done in the early weeks, making the disease appear to exist in the 'immaterial, functional realm"?
 
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Functional symptoms have been found in the initial stages of many neurological disorders
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It amazes me how they can just gloss over the fact that this stuff is common in disintegrating brains and jump to a psychosocial conclusion. Even a fatal disease will not save you from these vultures pushing SSRIs.
 
It remains unclear whether the patient’s functional symptoms were an early manifestation of sCJD or a cooccurring phenomenon. Functional symptoms have been found in the initial stages of many neurological disorders

And this isn't a stark warning about the irrelevance of FND?

What does it take for the rest of medicine to wake the eff up to what is really happening here? :grumpy:
 
Arnie Pye said:
I have an idea that they are already awake to what is happening, but the profession is so depressed and burnt out that they don't care.
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It matches their internal prejudices so they are just fine with it. Its just an extension of what they were taught and further what everyone in medicine has reinforced into them and the fact they have left a trail of destruction of wrong Munchausen and other somatic diagnoses isn't on their radar because there are no consequences for it. The entire system is rotten to the core, you would need to fire 95% of everyone to get rid of it.
 
Arnie Pye said:
I have an idea that they are already awake to what is happening, but the profession is so depressed and burnt out that they don't care.
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Here's one thing - if these individuals spending all their time creating a non-illness were in the workforce, or recruitment meant there weren't those who were trained and moved into this area in the first place - then that might be a help.

I just find it astounding you've got staff struggling to actually do real work due to a lack of staff and an increasingly large bunch siloing themselves into positions where they tart around with all this. And that is staff of all kinds - doctors are qualified and then move into their specialisms, no reason if theirs turns out to be a turd they shouldn't help on the shop floor using their skills in an area they can help.

To me it also seems clear that all this type of thing doesn't save any work, reduce costs or staff time and certainly causes huge complications that I bet led to ferreting on paperwork re: 'what happened' far more complex behind the scenes than the authors are making out.

Who/what is this area 'adding' anything to/for? Other than those in it?
 
Hutan said:
Another reference in this 2022 paper that I think is worthy of comment:
A Case of Sporadic Creutzfeldt-Jakob Disease Presenting as Conversion Disorder, 2017

The clinicians looked at this older woman, divorced, with few friends and yet denying depression, and assumed that she was a prime candidate for FND. (this literature is full of people either admitting they have depression, or being diagnosed with alexithymia (inability to know what they are feeling) as well as depression)





Some tests were done, but not the ones that would have been most useful in ruling out CJD.



So, instead of that palliative care for her and her family, she was told that she had an FND and given therapy to fix her functional disabilities. They tried to put her on a drug for anxiety and depression, but she refused. It is awful to think about what this poor woman went through. The consultants left her to the therapists. She became worse.



So, a month after admission to hospital she was tested specifically for CJD, and she died a month later.



What I find particularly incredible is that the authors of this case study still think that an FND diagnosis might have been correct. For goodness sake, this woman had her brain disintegrating and they think she was manifesting her subconscious depression as physical symptoms? It's incredible to see the authors tying themselves in knots, still defending an FND diagnosis



Or perhaps it's just that a sufficiently sensitive test was not done in the early weeks, making the disease appear to exist in the 'immaterial, functional realm"?
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"Perhaps early prion mediated neuronal loss in particular brain regions led to our patient’s early symptoms, which were consistent with a functional neurological disorder."
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"If so, this could challenge our understanding of the mechanism behind functional illness and the binary distinction of mind versus body."
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It should, but I'm not sure that they are really getting it. What they describe as FND could be caused by prion mediated neuronal loss - how is that 'mind' now?

Have these people lost the ability to follow logic, as well as science now?

"Conceivably, subtle neurological changes may underlie pathological changes which eventually result in the phenotype of functional symptoms."
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This is stupid. Take out the word 'functional' - which I'm unsure how even they will be able to define it so that it can be 'true' in all of their statements in this article - and it is basic 'derrh obvious'. Add in functional and what? pathological changes now = functional symptoms [that aren't caused by any pathological changes]?

Smells of covering-up/glossing over when you introduce the word subtle for someone who died of CJD months later, and had a possible CJD diagnosis and enough suspicion to have done half the tests.

I guess the word phenotype in this industry/dept is a hiding word to cover 'not actually functional at all'. Someone needs to pull this area up for misusing language to create ambiguity behind which to hide incompetence.

There is clearly no accountability in the type of people who choose this area - this is weasel words of someone called up in front of the headmaster trying to pretend something that went wrong somehow wasn't actually wrong at all?
 
Arnie Pye said:
If someone is suffering from CJD then none of their CJD symptoms can be "caused by" FND or conversion disorder or hysteria.
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And this the abstract doesn't propose (thanksfully).

The term FND in its genuin meaning doesn't say anything about causation (though the WHO classification may well).


The term FND in its proper meaning is not to critisize, only when it comes to the implication of the interpretation, that it would be caused by believes, habits asf, only then its not proven, and it might be even unlikely - as it is in the case of CJ indeed wrong.

So the abstract actually does a nice job, as it gives an example of FND not caused by any psychological themes.


I think this accuracy is crucial to keep in mind.
 
spinoza577 said:
And this the abstract doesn't propose (thanksfully).
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The paywall doesn't help us in assessing the text, but we've seen the lack of clarity around what the term FND means used against patients. It's like so many terms in this field - 'biopsychosocial' sounds completely sensible, but it typically is not used in a sensible way.

I think the information that we can get from the references, which include at least one paper by some of the authors of this paper and a lot of papers by leading 'FND as a synonym for conversion disorder' proponents, tells us a lot about where they are coming from. Perhaps you are right, and, after a series of FND patients turning out to be CJD patients, all of these people have suddenly seen the light. Perhaps they now think of FND as being completely unrelated to conversion disorder, that is, it's simply neurological symptoms with no identified pathology. I doubt it though.
 
Some of the latest papers from Jon Stone make it clear that he believes FND with its opt in symptoms and neuroimaging has put hysteria on 21st century scientific footing. He has written about how sad it was that the remarkable work done by the likes of Briquet was lost until it was recovered in the late 1990s (by him and Sharpe presumably)

This is similar to the way Wesseley tried to convince people that ME was just a modern version of neurasthenia.

The idea of hysteria and conversion disorder where thoughts and beliefs can induce physical disease is at the core of FND no matter how they talk about it. The missed diagnosis of CJD is justified because a though induced "overlay" was what they were treating. If you think of it this way it is easier to understand why they are so bent on teasing out all the FND aspects of every disease you have heard of.
 
"At the same time as they present the FND diagnosis as a kind of place-holder pending further developments, they also seem to indicate that it exists as an entity independently of the prion disease."
Functional neurological symptoms as initial presentation of Creutzfeldt‐Jakob disease: case series.​

"The explanation seems to be that early markers or indications of the prion disease led patients to engage in heightened monitoring of their symptoms—one of the prominent factors said to be involved in generating FND. And that these patients, therefore, actually suffered from FND as well as their prion disorder. So FND was the right diagnosis after all."​

FND is basically defined as physical symptoms in the absence of underlying organic disease, a mismatch between psycho-neurology and the rest of the body, a false reading.

But if there is underlying organic disease, especially progressive fatal disease, then the patient better be paying very close attention indeed to what is happening. In which case then the whole concept of FND and the assumption that the "heightened monitoring of their symptoms" is pathological and should not be indulged in by clinicians, are irrelevant, and very dangerous.

What they are claiming is that the FND diagnosis cannot be wrong (falsified), and cannot have adverse consequences. In a case where it was wrong and had adverse consequences.

This is a deliberate strategy, and has been since Wessely's book on clinical trials in psych gave the authoritative nod to the BPS club to indulge in the lowering of methodological standards.

All to avoid admitting: 'We don't know. We got nothing.'
 
Sean said:
But if there is underlying organic disease, especially progressive fatal disease, then the patient better be paying very close attention indeed to what is happening. In which case then the whole concept of FND and the assumption that the "heightened monitoring of their symptoms" is pathological and should not be indulged in by clinicians, are irrelevant, and very dangerous.
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If a patient isn't paying very close attention to what is happening, then how else can they report symptoms which lead to diagnosis? You can't even get a referral from primary care to secondary care, where progressive, fatal diseases are diagnosed without reporting symptoms first to a GP. I'm not sure how medics now want this to go. Should patients now somehow turn up, stay silent and let the doctor carry out objective symptom investigations? Out of the multiple symptoms, a patient could be suffering from, how will the doctor always know which exact subjective symptoms they should query to find out what the patient is suffering from?

Next year FND will be included in the new and first Acquired brain injury and neurological conditions guideline. I can't think of a good reason for it to be included if it amounts to the nonsense I've read on this thread. I sense there will be a rise in medicolegal claims going forward.
 
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