Functional neurological signs in hypermobile Ehlers–Danlos syndrome and hypermobile spectrum disorders with suspected neuropathic pain 2024 Fernandez+

Abstract

Background
The hypermobile Ehlers–Danlos syndrome (hEDS) and hypermobility spectrum disorders (HSD) are connective tissue disorders characterized by generalized joint hypermobility, associated with chronic pain and several symptoms, such as fatigue, dysautonomia, as well as psychiatric co-morbidities. Clinical observations of unusual manifestations during systematic sensory testing raised the question of a possible co-existence with a functional neurological disorder (FND). Hence, this study aimed to assess the presence of positive functional neurological signs (FNS) in a cohort of patients with hEDS/HSD.

Methods
The clinical data of hEDS/HSD patients (N = 24) were retrospectively analyzed and compared to a prospectively recruited age-/sex-matched healthy control group (N = 22). Four motor- and three sensory-positive FNS were assessed.

Results
Twenty-two patients (92%) presented at least one motor or sensory FNS. Five patients (21%) presented only a single FNS, 14 presented between 2 and 4 FNS (58%), and 3 patients presented 5 or more FNS (12%). None of the healthy controls presented motor FNS, and only two presented a sensory FNS.

Conclusions
The presence of FNS in hEDS/HSD deserves better clinical detection and formal diagnosis of FND to offer more adequate care in co-morbid situations. In fact, FND can severely interfere with rehabilitation efforts in hEDS/HSD, and FND-targeted physical therapy should perhaps be combined with EDS/HSD-specific approaches.

Open access, https://onlinelibrary.wiley.com/doi/10.1002/brb3.3441

 

Functional Neurological Signs = FNS

I searched for a definition of FNS and found this (it's only an Abstract) (Note - bolding is mine):

Functional symptoms and signs in neurology: assessment and diagnosis

Authors :

1. J Stone,
2. A Carson,
3. M Sharpe

It’s a Tuesday morning at 11.30 am. You are already 45 minutes behind. A 35 year old woman is referred to your neurology clinic with a nine month history of fatigue, dizziness, back pain, left sided weakness, and reduced mobility. Her general practitioner documents a hysterectomy at the age of 25, subsequent division of adhesions for abdominal pain, irritable bowel syndrome, and asthma. She is no longer able to work as a care assistant and rarely leaves the house. Her GP has found some asymmetrical weakness in her legs and wonders if she may have developed multiple sclerosis. She looks unhappy but becomes angry when you ask her whether she is depressed. On examination you note intermittency of effort and clear inconsistency between her ability to walk and examination on the bed. She has already had extensive normal investigations. The patient and her husband want you to “do something”. As you start explaining that there’s no evidence of anything serious and that you think it’s a psychological problem, the consultation goes from bad to worse….

In this article we summarise an approach to the assessment and diagnosis of functional symptoms in neurology, paying attention to those symptoms that are particularly “neurological”, such as paralysis and epileptic-like attacks. In the second of the two articles we describe our approach to the management of functional symptoms bearing in mind the time constraints experienced by a typical neurologist. We also address difficult questions such as: “What causes functional symptoms?”, “Are they real?”, and “Is there anything that can be done?”

We emphasise the need for a transparent and collaborative approach. As we will explain this depends on giving up a purely “psychological” view of functional symptoms in favour of a biopsychosocial view of causation in which dysfunction of the nervous system is the …

...

I get angry when I am asked if I'm depressed too. Because, in my experience, it is a sure-fire sign that the doctor I'm talking to is going to offer me an anti-depressant and ignore what I went to see him/her for. Perhaps I should call it the DIAA sign - Doctor is an ass sign. They will ignore all internal (hence invisible) signs, ignore pain, refuse to test anything, or perhaps just do a Full Blood Count and tell the patient everything is normal, and leave the patient to rot, and all that will be on offer is an anti-depressant. Women know this happens, so no wonder they get angry.

In this case a woman of 25 who has had a hysterectomy is likely to have poor health, poor hormone levels, low nutrients, poor energy, horrendous fatigue, ... and something that doctors won't acknowledge is that anti-depressants don't turn into those missing hormones after swallowing the tablets.

This is a list of the symptoms reported by women who've had hysterectomies :

https://hersfoundation.org/adverse-effects-data/

I wonder how a man who lost his testicles as a result of cancer or an accident is likely to feel. They might be depressed too. But anti-depressants are unlikely to help, and doctors are probably not going to prescribe them to men at the same rate they prescribe them to women with hysterectomies. Men in pain are treated for it, women are generally sedated for it.

 

Yeah, because she had that sinking realisation of "I see where this is going".

 

Lies, damn lies, and complete bullshit. This is the study they use to base the validity of those 'signs' being rule-in evidence.

 

Seems a bit like collecting non-fungible tokens.

The more non-existent biopsychosocial diagnoses and corresponding unproven therapies you collect from visiting doctors the more likely you are to collect even more from other doctors.

 

Absolutely frustrating; will this nonsense never end!

(Well of course not, as long as there are DIAAs - thanks to @Arnie Pye for the new acronym.)

How about a brain scan MRI for people who present with these symptoms?!!

Sometimes, this can reveal there are actually physical problems.

However, many physicians can't be bothered to stretch to an MRI.

 

I don't think that's a fair comparison. At some point, NFTs were worth a lot of money, they had a lot of value. Completely speculative unsustainable bubble value. But value nonetheless. Not just the promise and potential of value, people actually produced, sold and bought them with the intent of making profit on them, which some people did, if they sold quickly. Money in the bank, fungible currency, which allowed to buy other stuff. The BPS approach has yet to yield a single objectively quantifiable benefit, anything that isn't just vibes and feels.

And NFTs have never led to anyone being killed, injured, maimed or significantly degraded the quality of life of anyone, which can't be said of the biopsychosocial ideology. Directly anyway, there was that NFT party with UV lights that may have blinded some people, but even that is less harm overall.

This biopsychosocial model of illness is objectively less valuable and less harmful than NFTs, which are basically the modern textbook example of a worthless financial bubble, something literally created to be worthless speculation and named as such. Now that's just amazing. What's even more amazing, is that despite being so, the BPS bubble is still alive and growing. Unlike NFTs. So the people who got suckered into buying, selling and speculating on NFTs are more rational actors than the people who promote the BPS model. People who bought some worthless token about drawings of apes and other stuff. As opposed to people with medical degrees making serious decisions about the lives of real people.

Really it's hard to put into words just how incredible this all is. Financial bubbles are weird. Ideological bubbles that are clear net financial negatives, while boasting the opposite but never able to actually show any of this, are probably some of the weirdest shit humanity has ever produced. Far weirder than the NFT bubble.

 

Remember, it’s imperative that you transform this problem of yours into a problem for the patient. If you’re in a bad mood, why not internally blame them for it? After all, if there weren’t so many patients - with all of their interminable problems - would you be ‘behind’?

Either way, be sure to let it degrade the quality of your consultation.