Sunday, June 03, 2012

Reflections On Dopamine Illness, Part I : Recasting Neuropsychiatry

(For MCK and EMK)

Disclaimers - 

1. I am not a doctor, I am a retired solicitor. If you feel that either you or someone you are responsible for should see a doctor, go to the doctor.

2. I am not undertaking this exercise in order to help you sue your doctor. As previously stated I have no medical training, the theories contained in these posts are mine and mine alone, I accept no liability for any course of action anyone, anywhere, undertakes in response to them, and I will not assist anyone anywhere who seeks to use them in order to advance their cause in any litigation against any doctor.

OK. Now we all know where we stand.

At first I thought it referred merely to a spitting tic of a type not uncommon in Tourettes; but it still didn’t all add up.

The Latin verb ‘to spit’ is the First Conjugation sputo, -tare, -tavi, - tatum, and the phrase I needed to translate was ‘risus indecens, ira turpior spumante rictu’. ‘Sputo’ and ‘spumante’ are clearly of the same root, but ‘spumante’ indicates the performance of some action other than spitting.

In his translation of Chapter 5 Verse 30 of Gaius Suetonius Tranquillus’s ‘The Twelve Caesars’ (Penguin Books 1957, republished 2000, p. 177), relating to the emperor Claudius, Robert Graves, the author of ‘I, Claudius’ and ‘Goodbye To All That’, translated this phrase as ‘ uncontrolled laugh, a horrible habit, under the stress of anger of slobbering at the mouth...’.  But this still didn’t match up with any Tourettes tic I’d heard of; until the answer came to me completely out of the blue about six weeks later, in a thoroughly Tourettic manner.

I had been looking at the wrong word. 

The phrase is not merely ‘spumante’, but ‘spumante rictu’. ‘Rictu’, with its obvious relationship to the English ‘rictus’, suggests that the real meaning of the phrase ‘spumante rictu’ is ‘liquid emanated from his mouth while his face was fixed’. Anger may certainly have been displayed during the course of the facial movements thus described, but if Suetonius was correctly describing the actions of his mouth it was possible that the action that Graves had translated as ‘slobbering’ was in fact salivating.

And if he salivated, then it would suggest that Claudius not only displayed symptoms of Tourettes, which he certainly did, but also that he showed symptoms of Parkinsonism; not the degenerative affliction known as ‘Parkinson’s Disease’ (‘paralysis agitans’), but  ‘secondary’ or ‘atypical Parkinson's’. The excitement I felt at realising that this was a possible answer to the questions posed by Claudius’s baffling symptoms was intense. 

And it that was the case, it was equally possible that during these episodes he might have been suffering a symptom of Parkinsonism known as an 'oculogyric crisis'; as Dr. Gilbert Onuaguluchi noted on pps 53 and 54 of 'Parkinsonism' (Butterworths, 1964, aforesaid), 

"Emotional disturbances occur in all severe cases. Despite rigidity and immobility during the crisis, the patients are rarely silent: grunting, mumbling, howling or moaning are commonplace: and some emit sudden squeaking sounds like the plaintive cry of a seagull...A few patients sometimes have outbursts of uncontrollable laughter...Increased salivation is seen in less than 50 percent of patients and in these it is inconstant". 

In his 'The Deification Of Claudius The Clod', Seneca remarked that Claudius had a voice like a sea monster; and according to the relevant Wikipedia entry, oculogyric crises can also occur as reactions to neuroleptics, the class of drugs used to treat Tourette Syndrome.

I am indebted to Dr. George M. Burden for his wonderful paper 'The Imperial Gene', to my mind a work of genius, for the idea of revisiting the Julio-Claudians in this context. We'll be seeing more of them.

This might seem like little more than groping in the dark for convenient answers in the nether reaches of neurological history, the type of speculation which might make the speculator feel terribly clever while making an ass of themselves in the process but one of little useful application to modern medicine, but I would respectfully beg to differ. On the other hand, I am an interested party.

I have Tourette Syndrome; or do I? While Peter Hitchens might consider the confession of dopamine imbalance such as Tourettes to be, what was it, ah, yes, "an assertion, miles outside the rules of objective, demonstrable, predictive, repeatable or experimental science," I can assure him that I was in fact diagnosed with that condition in 1992, at the Southern General Hospital in Glasgow, and have required regular treatment for it since; yet I'm not quite as sure of that diagnosis as I used to be. 

Given the apparent level of public interest in the illness, which is certainly far greater than that in halitosis or varicose veins, it might be thought that reminiscences of Tourette Syndrome would fill the shelves, but there are remarkably few of them; Meige and Feindel's 'Tics And Their Treatment', with the foreword 'Confessions of a Ticquer', is apparently not available in the University of Glasgow Library, pretty much leaving the researcher with James McConnel's 'Life, Interrupted', a very personal book but still of value. My original plan had been merely to share experiences of having lived with the illness for over thirty years. Contrary to what some seem to think, it is an illness; I am firmly of the belief that something that makes its sufferers feel as bad as Tourettes can does not deserve to be called a ‘syndrome’, or ‘condition’, or to bear some other codename, some arcane linguistic semaphore, which serves only to tell people that organised, professional medicine can’t cure it and so should be called anything other than what it is; an unpleasant, often deeply debilitating illness. This may spare some doctors’ blushes, but it often doesn’t help the sufferer feel any better.  

However, the background reading I have done on the illness, conducted from March to May of 2012, in preparation for writing what I thought would be my memoirs has led me to what some might consider to be a rather startling conclusion, and the scope and nature of the book have changed accordingly. The principal change is that no book will now be written. The subject matter is really too technical for a medically unqualified person to deal with, the process of recollecting those events which helped lead me to where I am has been too unpleasant and the discipline of writing a 100,000 word book is beyond me. I am a blogger, and the immediacy inherent in both the production and publication of blog posts makes the blog a form of literature which it is very easy for me to produce. I should never have taken myself offline for any reason, and I won't be making that mistake again: a textbook example of 'kinetic melody' in action.

However, the second conclusion I have reached is that there might in fact be no such phenomenon as Tourette Syndrome (hereinafter and in all subsequents posts 'T'), or indeed Attention Deficit Disorder (ADD), or Attention Deficit Hyperactivity Disorder (ADHD), or Oppositional Defiance Disorder (ODD), or Obsessive Compulsive Disorder (OCD), or any other picking from the biochemical grab-bag called the ‘neuropsychiatric’ family of disorders, that tombola of perverse pathologies, which anyone has so far cared to give a name to. They might all just be variations of Parkinsonism (hereinafter and in all subsequents posts 'P'), or even just something which can best be described as 'Dopamine Illness' (hereinafter and in all subsequents posts 'D')

The reason for this lies in what would seem to be a logical impossibility. P of the type that Claudius may have suffered from is caused by the failure of the brain to produce sufficient dopamine to enable ordinary function, while T is caused by a surfeit of dopamine which impairs ordinary function. How can symptoms suggesting both a surfeit of a substance and its lack appear in the same illness? For they certainly do. I am living proof. I suffer intermittent rigidity crises in all limbs, have a chorea (dancing movements) in my fingers and can swim, climb, and walk backward with ease but have difficulty in walking forward unassisted, all of which might suggest P. While researching what I thought would be my book, I discovered that I can achieve normal forward motion without any difficulty; it’s just that I have to keep my eyes closed to do it; not the sort of thing you expect to find out about yourself while walking up a hill in Lanarkshire.

It's with no sense of anger, really more a kind of mild irritation, that you learn that Jean-Martin Charcot first described the alleviation of rigidity upon suspension in water in the late 19th Century (Oliver Sacks, 'Awakenings', Picador, 4th edition,  1990, page 6, note 8), and that you might not therefore have had to wait until you went for a swim while on holiday on Tenerife in 2008 to find that out for yourself, or that Onuaguluchi had observed the lessening of rigidity affected by closing the eyes (Note 1., 04/06/12 07.00: after the original publication of this post in the early hours of 03/06/12, on the afternoon of that day I discovered that fluidity of movement is restored while engaged in another activity; of all things, it was trampolining, and I haven't seen that one mentioned anywhere else. Note 2, 04/06/12 20.43 I've been having another think about this, and there might be other activities which P sufferers can engage in that loosen rigidity and give them a chance to exercise. When swimming and trampolining, the feet are disconnected from the ground. It could perfectly well be the case that P motion difficulties require that the sufferer attempt to be moving forward in a normal horizontal manner in order to appear. This would explain why I, like some of Sacks's patients, can tackle stairs quite easily while suffering difficulty on the flat. Those of his patients who referred to 'maps' might be right on the money. I have come to conclude that my need for a mobility aid is not to enable my legs to support my weight, but to provide my brain with a reference point in relation to the ground; the eyes cannot do it (explaining why the ability to walk unimpaired is restored when the eyes are closed, as the brain does not then need to both interpret both visual images and provide a sense of direction at the same time), so both the sound and the vibration of the stick when it hits the gound serve the same function. Another example of this phenomenon might have been given by Sacks in Chapter 7 of 'The Man Who Mistook His Wife For A Hat', entitled 'On The Level', in which the proprioception of his patient, a sufferer of ordinary Parkinson's Disease, had his corrected with a pair of custom-made spectacles. Given that both swimming and trampolining require that the feet be elevated from the ground, it would be interesting to see whether rigidity was loosened in P sufferers in other activities in which this is required, such as cycling or possibly even horse-riding. After posting the first note this morning, I remembered that I had no difficulty using an exercise bike when attending physiotherapy in January 2009. On-road cycling would possibly be far too dangerous for the P sufferer, given the need to both start and stop, but cycling on a purpose-built cycle track might be very useful; remember Sacks's comments upon how the patients in the 'Highland Hospital' thrived in a unit that was designed with curved surfaces, whereas those in 'Mount Carmel' struggled with the building's angular layout; the same principle could apply in this). However, in the recent past I have also suffered from involuntary swearing (coprolalia), and continue to suffer self-repetition (palilalia), obsessive-compulsive behaviours and intrusive thoughts, what the late Arthur Shapiro described as ‘mental coprolalia’, all of which suggest T. How can this be the case?

There can be only one logical answer to that question, which is that all these phenomena are expressions of the same thing; and if the extremes of T and P are the same thing, everything which is between them or classed as being related to them must be the same thing as well. T has been defined as a ‘spectrum disorder’. As the ‘waxing and waning’ of tics over periods of years is well-known, this would certainly seem to be true. Some sufferers will only ever have mild tics, others will have periods of respite from more aggravating happenings while the worst affected will be debilitated to the point of disability. However, what the apparent existence of both T and P in the same patient would seem to suggest is that all mere two-dimensional straight line models of a severity spectrum in D as a whole must be considered inadequate. While a spectrum certainly does exist, it must instead be turned on its axis and expanded outwards into three dimensions, and to a scale which Oliver Sacks has described, with blinding insight, as ‘infinite’  (‘Awakenings’, p.97). 

Sacks used the word in relation to P to express the idea that the existence of one part of P implies the possibility of all parts. When I read this, I shouted 'Yes! Yes! Yes!', not quite in the manner of Meg Ryan but with similar brio, for what he was suggesting was precisely in line with my own experience. I had gone from obsessive counting (arithromania) in 1978 (T) to involuntary movements in the head and arms in 1991(T?)  to a diagnosis of T in 1992 to requiring to use mobility aids in 2007 (P?) to being able to swim without difficulty in 2008 (P) to being able to walk with my eyes closed in 2012 (P) while also shouting 'Toilet Duck!' (T). Sacks believes that the presence of one aspect of P implies the possibility of all parts; my belief, based on mere experience, is that the existence of one part of either T or P implies the existence of all parts of T and P; and if that is the case then the current classifications of T and P are inadequate, and should be replaced with one term that covers both, indeed all, dopamine dysfunction phenomena; D.

I am, therefore, wholly at one with Mr. Peter Hitchens in our mutual belief that ADHD does not exist (I did tell him that some of my conclusions might surprise him). What we also share is our mutual lack of medical training. Where we differ, I think, is in our reasons for believing this state of affairs to be the case; and  my belief is that the difficulties involved in diagnosing any kind of D illness present doctors with enormous problems of perception, and solving this problem of perception may be the key to the recasting of neuropsychiatry as it is currently understood . Mr. Hitchens seems to believe that ADHD does not exist at all; I believe that its continued diagnosis may not be capable of being justified as it is presently defined. 

For it is clear with anyone with eyes to see that T, and ADD, and ADHD, and ODD, and all the other neuropsychiatric illnesses do exist. They tic, and they swear, and they fidget, so therefore they are. My view is that they just don’t exist in the way formal medicine seems to think they do, as separate illnesses. My wholly untrained belief is that while do they exist, they do not exist as illnesses but as symptoms, perhaps something that might best be called, as I’ve said, a D illness of the type that already makes Tourettes the illness in the books with more symptoms than any other (see, for example, ‘Gilles de la Tourette Syndrome’, by Arthur K. Shapiro and others, Raven Press, 2nd edn,. 1988, p. 452; "26 different simple and 54 complex motor tics, 77 sounds, 118 coprophilic symptoms, and nine echophilic symptoms. Seventy-seven percent of patients report from 11 to 60 different symptoms during the course of their illness")

If you take the time and trouble to read the aforementioned work ‘Gilles de la Tourette Syndrome’, for years the leading textbook on T, you will see that the phrase ‘the problem of heterogeneity’ recurs time and time again. The late Arthur Shapiro, may he rest in peace, was a giant in the field of T research. A most distinguished psychiatrist who held no illusions about his discipline’s limits, in 1965 he became the first doctor in the United States to prescribe haloperidol to a T patient, and was later instrumental in helping to establish the Tourette Syndrome Association. I come not to bury Shapiro; the rigour and devotion, indeed single-mindedness, he applied to his research and practice have helped improve the lives of tens, if not hundreds, of thousands of people.

However to this reader it is very clear that the enormous variety of symptoms that he was presented with gave Shapiro a severe problem. A good Linnaen intent in adhering to the highest clinical and academic standards, and always conscious of that most American impediment to the advance of scientific knowledge – the prospect of being sued - to the lay reader he seems at times to be lost in a taxonomical fog, unable to see the wood of illness for the trees of symptoms in a grail quest for verifiable conclusions. Branches were hacked away and raised as trophies while the trunk was ignored, his findings becoming more and more specialised and over-evolved, with the prospect of diagnostic clarity eventually diminishing into a downwardly spiralling haze of Transient Tic Disorders (TTD), Paroxysmal Myoclonic Dystonia, With Vocalisation (PMD), until the final descent into a catch-all ‘Tic Disorder not otherwise specified’. Learning of the existence of PMD was an epiphany for me; I seemed to possess all the symptoms, and, most tellingly, they had appeared at precisely the same age, 21, as the patients reported by Shapiro; but that couldn’t be right, because they all had ADD and I didn’t. What I had was like PMD, but Shapiro indicates it couldn’t be PMD. Back to the drawing board.

While academically admirable, and exercised with the very best of clinical and personal intentions, and at enormous and unacknowledged personal expense in terms of both time and money – their dogged and most scrupulously scientific endeavours to track down Tourettes must have cost the Shapiro family hundreds of thousands of dollars - Shapiro’s extreme rigour in the pursuit of verifiable certainty might in no small measure have been a consequence of having himself been obsessive, a status he freely admitted to in ‘Gilles de la Tourette Syndrome’ (p. 229), as well as admitting to having spent four years in analysis trying to get to the bottom of it.

Having read Shapiro’s book it is very easy to imagine how an obsessive nature in a great intellect driven to adhere to high standards could prevent a scholar seeing what to a layman, albeit a sophisticated one, seems absolutely clear, that the illness may be a great deal broader than researchers have thus far imagined. Arthur Shapiro perceived that the range of symptoms he was presented with was a problem, whereas the real problem might not have been that he saw too many symptoms but that he didn’t see enough; and as the yoke of Linnaeus is not easy nor his burden light, research into T seemed to descend into a frenzy of exhaustive analysis of what might be mere symptoms in the hope of discovering new illnesses; a process which if incorrect is as futile as examining your fingers while forgetting, or not realising, or, worst of all, completely ignoring, that they’re part of your hands. Even in 1988, Shapiro was complaining about the number of papers that were being published on T. The extraordinary volume of data on T may have acted as much of an impediment to its understanding as an aid. 

That D presents a vast variety and range of symptoms in all its forms has been well-known for years; indeed the presence of a phenomenon with an enormous range of symptoms might even be suggestive that the condition is affective of dopamine, or that the sufferers should be considered at risk of later dopamine illness. In 'Awakenings' (page 14 note 19), Oliver Sacks records that the phenomenon as exhibited amongst post-encephalitic Parkinsonians 'fascinated physiologists as well as physicians, and led, in the 1920's and 1930's to the founding of behavioural neurology as a science'. In his later book 'The Man Who Mistook His Wife For A Hat' (Picador, 1985, p. 87), Sacks writes of Tourettes that "(i)n the years that immediately followed the publication of Tourette's original papers (in the 1880's) many hundreds of cases of this syndrome were described - no two cases being quite the same". It may be the case that volume and variety of symptoms, either as a reaction to an event or in an unspecified illness, could itself be considered as indicative of some sort of dopamine dysfunction, and that investigating that connection should be a high priority. 

If I am right, every study of T which does not come to the same conclusions as I have must be wrong, either in whole or in part. Their findings may have been correctly observed, but by mistaking symptoms for illnesses they have proceeded from the wrong starting point. For someone with no medical training to think in this way seems appallingly arrogant, even to the thinker. 
Yet in 'Awakenings', Sacks famously recorded how a number of his post-encephalitic Parkinsonian patients developed either T or Tourettic symptoms when administered L-DOPA. Similarly, Shapiro records how a small number of his T patients developed what he called an 'Extrapyramidal Parkinsonian Hand-Finger Movement' when administered neuroleptics ('Gilles de la Tourette Syndrome', page 432), and Sacks has recorded how 'Witty Ticcy Ray', the first T sufferer to whom he administered the neuroleptic haloperidol, initially 'presented a picture, even on (a) minute dose, of marked Parkinsonism' ('The Man Who Mistook His Wife For A Hat', page 93: by the time of publication the issue had been resolved). When medication is administered for P, patients can exhibit something that looks like T, and vice versa. Logically, that suggests to me that the function which is being treated in both cases is the same; and if the same function is being treated in both cases, to my mind it makes little sense that the symptoms being displayed should be considered to be suggestive of different illnesses. 
These very eminent doctors, whose regard for their patients shines through everything they write, clearly believe that they are dealing with different conditions, and it's embarrassing to suggest that you disagree with them. Here I must take a leap of faith, and say why I think they're wrong; and I am indebted to a fellow Glaswegian for helping me find the answer. 
On pages 20 and 21 of his still in print pop-psychiatric classic from 1960 ‘The Divided Self’ (Tavistock Publications (1959) Ltd. 1960, Penguin 1990), the late R. D. Laing showed the now well-known image of a picture which can be viewed in one, other or both of two ways, either as two faces in black facing each other or as a vase (his word) between the two. He describes the image as follows –

“In this figure, there is one thing on the paper which can be seen as a vase or as two faces turned towards each other. There are not two things in the paper; there is one thing there, (MK’s italics) but, depending on how it strikes us, we see two different objects”.

And in a nutshell, that is precisely the problem which I believe both psychiatrists and neurologists have encountered when faced with dopamine illness and which, in my view, they have not resolved; not one of function or symptoms but of perception. Bob sweats and festinates, so he must be classically P, while Jim has eye tics and palilates, so he must have T. What this mentality cannot resolve is the situation of those people who do both; who are both palilalic and obsessive (T) yet who also suffer rigidity crises, and who can walk backwards but not forwards (P); people like me. The doctors cannot be blamed for this. These people are dedicated professionals who have trained for many years and who have made their diagnoses while exercising the highest degrees of care and skill, and who have their patients' best interests at the forefront of their minds in everything they do. However, they are trained to heal through science. 

Lest The Cult Of Richard Dawkins feel the urge to swarm down upon me like a flock of bungee-jumping harpies, I am not advocating any return to mysticism in medicine, nor to faith healing, nor to leeching patients, nor to examining the stool to determine the presence of dropsy or the bloody flux (although as anyone who’s actually read the leaflet contained in any packet of anti-inflammatory medication should agree, the examination of the stool certainly does still play a role in modern medicine; it’s just that instead of the doctor doing it, the patient now does it for them). However, we must recognise that modern science and therefore modern scientific thinking and thought processes have their limits. We map the stars without often having a blind clue about the processes taking place inside our own heads, five inches behind our eyebrows. This lack of knowledge concerning how our brains work presents a gross challenge to the scientific mindset, as the scrupulous scientist Arthur Shapiro found out again and again and again. In our routinely scientific, symptom–chopping world established according to principles determined by hyper-rational, post-Enlightenment thought processes, people like me should not exist, but we do. If anyone were ever to ask me to guess, I would hazard the opinion that a probable majority of dopamine illness sufferers exhibit both T and P symptoms in varying degrees at the same time; the difficulty they encounter in seeking the answer to their questions – What is the name of my illness? What do I have ?– is that the doctors they consult are only able to see the symptoms present at the time of consultation.  The symptoms of both T and P are notorious for waxing and waning. A diagnosis of T assumes that those symptoms which wax and wane within the patient will always be those of T. I would respectfully suggest that this urge towards therapeutic classification, unintentionally but effectively stamping the patient with a label like a tin of peaches in a cannery, impedes rather than enhances the understanding of dopamine illness, and that a more fruitful approach would be for patients exhibiting the qualifying symptoms of anything currently classified as a neuropsychiatric illness to be diagnosed with generic 'Dopamine Illness' instead.

To use a highly simplistic and probably unscientific analogy, the wheels of a car perform different functions depending upon whether the car is moving forward or in reverse; yet at all times they remain wheels. T is forward, P is reverse, but D is the wheel. That's what I think. 

This view of T and P may be controversial. I couldn’t really care less about that, but I am very concerned about the impact it might have on some people who have been diagnosed as suffering from T. I would never wish to push any T sufferer into an existential crisis. Too many people have spent too many years merely coming to terms with the facts that they have been told that they have that illness and that they then have to deal with it for them to be put off balance by someone without any formal training merely expressing an opinion. They will have heard enough of them already – ‘You’re a hypochondriac!’ ‘You don’t swear, how can you have Tourettes?’ While ADHD sufferers must endure the thundering public wrath of critics of Ritalin, T sufferers tend to be seasoned recipients of others’ private opinions on the validity of their illness, an indignity which is nothing less than an attack on their identity, and one which never seems to be visited upon cancer patients and heart attack survivors. Many might need my opinions like they need a new tic. This must be acknowledged; there is no reason why any member of a group which can already face great difficulty integrating into what is sometimes called ‘mainstream society’ should be pushed further away from it for any reason. 

Nor is it my intention to see anyone who receives a wage for supporting T sufferers, or the sufferers of any other declared illness which might be the same thing as T or P, lose their job. That they have been supporting people whose illness I happen to think has been wrongly defined does not mean that those people should not still be supported.

One of the consequences of the approach to D I am suggesting might be a revisiting of the processes by which some medications are prescribed for some D patients. If a patient has exhibited ADHD symptoms at some point but has developed into T, it might be the case that Ritalin, for example, might no longer be an appropriate medication for that patient; and if another medication is substituted in consequence that would be only be a good and wholesome thing, for it would deny the shrill and persistent critics of Ritalin the chance to undermine the probably already shaky confidence that worried parents have in the medication which a doctor has prescribed for their children in the utmost good faith; a medication which in many cases proves to be very successful, enhancing both well-being and quality of life. 

The broader issue of treatments will be addressed in several later pieces.



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