“HNPP is painless, mild and rarely causes any signficant disability, with maybe only a few (2-10) Palsies in a lifetime”
There seemed to be a phase where all neurologists had this opinion of HNPP, and of course this conflicts with experience of many of us. But trying to explain to them the reality of our daily lives, that of many episodes of transient weakness and often longer periods of sensory disturbance including pain, usually as a result of pressure, or repetitive actions, seems to go totally over their heads.
Unfortunately, many Doctors are taught that neurological symptoms can’t come and go, and that this is usually a sign of a conversion reaction to stress, an anxiety state. This has in the past led to very inappropriate psychological intervention, which can never relieve the symptoms of pressure palsies, and thus leads to a deeper misunderstanding, and further belief on the part of the Medical profession that the symptoms are of a psychological nature.
I’ve sat in lectures given by senior registrars in psychiatry (as part of my training as a psychiatric nurse in the early 1980s), listening to these opinions of transient neurological symptoms. At the same time having episodes of ulnar nerve symptoms from sitting at a desk, long lasting numbness and pins and needles in the little finger and half of the ring finger. I realised that they were neurological but as they seemed to appear almost out of the blue, and remain for several days or weeks before gradually coming back to life, I felt unwilling to talk to a Doctor about them, thinking that I would be disbelieved.
At the same time I had been going deaf and suffering from permanent tinnitus, attempts to have this investigated resulted in GPs looking in my ear and pronouncing that I didn’t have an infection, therefore I must be either a) Anxious B) Malingering C) Depressed. After 6 years of this I was eventually sent to have further testing, at a clinic studying the ‘psychological’ causes of tinnitus (Thanks Doc, that was so thoughtful of you!!). After having hearing tests, speech discrimination tests and Balance tests, I was excluded from their study as I had physical problems not psychological ones. 13 years after the onset of permanent deafness I was diagnosed as having Meniere’s Disease.
Consequently I had the belief that my ‘palsies’ would also be misinterpreted as being of ‘psychological’ origin. Something that has come back to haunt me, despite having confirmed HNPP……….
However, HNPP has been well described in the medical literature, as causing many episodes of transient peripheral nerve dysfunction, which clear up over a period of minutes to days, and less often weeks and months. Eventually, permanent weakness can occur, for some if not all. This might take a lifetime, or for a suitably serious injury to occur. Every single person who has the HNPP gene deletion will have signs of Neuropathy on further testing, evidence of many tomacula (sausage shaped swellings of individual nerve fibres), conduction abnormalities – slowing, increased latency, and evidence of conduction block at common sites of nerve vulnerability, and in some cases axonal degeneration.
I find it quite incredible that some of us are just not believed, even when we have had a cast iron diagnosis of HNPP. Some neurologists will even go on to say, (I have some rather damning quotes from some ‘expert’ neurologists on this matter) that our diagnoses are ‘scientific’, and that all that really matters is their ‘clinical’ opinion, ie what they can illicit from their predominantly motor neurological tests.
My argument to them is, that all their tests prove is that we don’t suffer from ‘neurological test palsies’, when in reality, we may well be having many symptoms of palsies in ‘real’ life. Think of any occupational palsies, Bulb diggers palsy, pizza cutters palsy, screwdriver palsy, Kneeling down changing baby’s nappy palsy, sitting on the throne (toilet) too long palsy (sciatic symptoms, foot drop, and numbness), Bike riders palsy (pudendal nerve, a strange sensation to be sure…) the list is almost endless, and displays as a whole range of multifocal ‘entrapment-like’ symptoms. Further examples of entrapment-like neurological symptoms, Sciatica, piriformis syndrome, Meralgia paresthetica, carpal tunnel, tarsal tunnel, and many many more. Not everyone will have these all at the same time, and some people with HNPP will be lucky and not have many symptoms at all. Much will depend on the environmental circumstances, and possibly other genetic predispositions.
I was even told by one neurologist, after a brief neurological motor test, that I didn’t have a neuropathy. There was nothing he could send me to a surgeon for, ie an entrapment ( I suppose he was correct in that sense, but he had totally overlooked the frequent short term palsies caused by HNPP). In fact I had been previously diagnosed with HNPP by a surgeon who said he wouldn’t operate, because I had an inherent neuropathy, not an entrapment, and that surgery was rarely successful. One of these opinions is wrong, and it’s not the surgeon.
Would these neurologists deny that an epileptic has seizures because they haven’t seen the patient have one in their clinic. I doubt it somehow, they would rely on previously obtained scientific data, ie EEGs and the patients history from their GP. So why then do they doubt that we have short term transient palsies caused by minimal pressure, stretch and repetitive movements, in our everyday life. They have incontrovertible evidence of HNPP via DNA tests and often electrodiagnostic tests, and a history of such events as reported by our GPs. There is no reason to dismiss our symptoms other than that of ignorance and an inability to acknowledge that their ‘Clinical Neurological tests’ aren’t as infallible as they think.
”In the last analysis, we see only what we are ready to see, what we have been taught to see. We eliminate and ignore everything that is not a part of our prejudices”
Jean-Martin Charcot, 1825 – 1893