Tuesday, June 23, 2015

West on West syndrome

I am in possession of a photocopy of the original 1841 Lancet article (the adjacent article is Epistaxis-Plugging the Nares with Putty).  Try as I might, I could not get it imported into the blog.  [Addendum 9/13/17--see http://childnervoussystem.blogspot.com/2017/09/dr-wests-letter-to-lancet.html] Anyone desiring the photocopy, with copious underlining done by me, could contact me at gbreningstall@gillettechildrens.com.  I found that the text of the article was in Epileptic Disorders:

Duncan R. Infantile spasms: the original description of Dr West. 1841. Epileptic Disord. 2001 Jan-Mar;3(1):47-8.. 

On a peculiar form of infantile convulsions
West WJ

Lancet 1841 ; 1 : 724
Sir: I beg, through your valuable and extensively circulating Journal, to call the attention of the medical profession to a very rare and singular species of convulsion peculiar to young children. As the only case I have witnessed is in my own child, I shall be very grateful to any member of the profession who can give me any information on the subject, either privately or through your excellent Publication.

The child is now near a year old; was a remarkably fine, healthy child when born, and continued to thrive till he was four mounths old. It was at this time that I first observed slight bobbings of the head forward, which I then regarded as a trick, but were, in fact, the first indications of disease; for these bobbings increased in frequency, and at length became so frequent and powerful, as to cause a complete heaving of the head forward towards his knees, and then immediately relaxing into the upright position, something similar to the attacks of emprosthotonos: these bowings and relaxings would be repeated alternately at intervals of a few seconds, and repeated from ten to twenty or more times at each attack, which attack would not continue more than two or three minutes; he sometimes has two, three, or more attacks in the day; they come on whether sitting or lying; just before they come on he is all alive and in motion, making a strange noise, and then all of a sudden down goes his head and upwards his knees; he then appears frightened and screams out: at one time he lost flesh, looked pale and exhausted, but latterly he has regained his good looks, and, independent of this affection is a fine grown child, but he neither posseses the intellectual vivacity or the power of moving his limbs, of a child of his age; he never cries at the time of the attacks, or smiles or takes any notice, but looks placid and pitiful, yet his hearing, and vision are good; he has no power of holding himself upright or using his limbs, and his head falls without support.
Although I have had an extensive practice among women and children, and a large circle of medical friends, I have never heard or witnessed a similar complaint before. The view I took of it was that, most probably, it depended on some irritation of the nervous system from teething; and, as the child was strong and vigorous, I commenced an active treatment of leeches and cold applications to the head, repeated calomel purgatives, and the usual antiphlogistic treament; the gums were lanced, and the child frequently put into warm baths. Notwithstanding a steady perseverance in this plan for three or four weeks, he got worse, the attacks being more numerous, to the amount of fifty or sixty in the course of a day. I then had recourse to sedatives, syrup of poppies, conium, and opium, without any relief: at seven months old he cut four teeth nearly altogether without any abatement of the symptoms, and, up to this period, he was supported solely at the breast; but now, at the eighth month, I had him weaned, as he had lost flesh and appeared worse; I then only gave him alteratives, and occasionally castor-oil. Finding no benefit from all that had been done, I took the child to London, and had a consultation with Sir Charles Clarke and Dr. Locock, both of whom recognised the complaint; the former, in all his extensive pratice, had only seen four cases, and, from the peculiar bowing of the head, called it the "salaam convulsion"; the latter gentleman had only seen two cases; one was the child of a widow lady, it came on while she was in Italy, and, in her anxiety, she consulted the most eminent professional gentlemen of Naples, Rome, Florence, Genoa, and Paris, one of whom alone seemed to recognise the complaint. In another case, mercury, corrosive sublimate, opium, zinc, and the preparations of iron, were tried without the slightest advantage; and, about six months from the commencement of the symtoms, a new one was added; there began a loss of motion, in the whole of the right side, and the child could scarcely use either arm, hand, or leg. Sir Astley Cooper saw the child in this state; he had never seen or heard of such a case, and gave it as his opinion, that "it either arose from disease of the brain and the child will not recover, or it proceeds merely from teething, and, when the child cuts all its teeth, may probably get well"; some time after, this child was suddenly seized with acute fever; the head became hot, and there were two remaining teeth pressing on the gums; the child was treated accordingly; leeches to the head, purged, and lowered; the gums were freely lanced; in a few days the teeth came through, and the child recovered, and from that time the convulsive movements never returned. Sir C. Clarke knows the result of only two of his cases: one perfectly recovered; the other became paralytic and idiotic; lived several years in that state, and died at the age of 17 years. I have heard of two other cases, which lived one to the age of 17, the other 19 years, idiotic, and then died. I wrote to Drs. Evanson and Maunsell, of Dublin; the former gentleman being in Italy, the latter very kindly replied, he had seen convulsive motions in one finger, arm, or leg, but had never witnessed it to the extent of my poor child. As there has been no opportunity of a post-mortem examination the pathology of this singular disease is totally unknown.
Although this may be a very rare and singular affection, and only noticed by two of our most eminent physicians, I am, from all I have learnt, convinced that it is a disease (sui generis) which, from its infrequency, has escaped the attention of the profession. I therefore hope you will give it the fullest publicity, as this paper might rather be extended than curtailed. I am, Sir, one of your subscribers from the commencement, your faithful and obedient servant,
 W.J. West
Tunbridge, Jan. 26, 1841.
P.S. - In my own child's case, the bowing convulsions continued every day, without intermission, for seven months; he had then an interval of three days free; but, on the fourth day, the convulsions returned, with this difference, instead of bowing, he streched out his arms, looked wild, seem to lose all animation, and appeared quite exhausted.
Duncan continues:  When we read an old medical text, we have the comfortable perspective afforded by the advanced state of modern medical knowledge. We might look with amused condescention on the state of medical knowledge of a former time: the terminology is archaic, the treatments bizarre. We may be surprised when we see clinical descriptions of high quality, but of course we should not be. Doctors of past eras had no scans or tests, and relied entirely on their clinical acumen for diagnosis: one should expect their powers of observation and description to be better than ours, not worse. West's letter to the Lancet illustrates this dichotomy nicely. It contains a strikingly clear and accurate description of the clinical manifestations of the neurological disorder which we recognise a century and a half later. This description is nonetheless set in the midst of accounts of antiphlogistic treatments, leeches, gum lancings, corrosive sublimates, cold applications to the head and warm baths. In this regard, it is ironic to reflect that if Master West's illness had occurred only a few years later, this list of 'bizarre' treatments might have included at least one familiar therapeutic measure: bromide was described as a treatment for epilepsy in 1857. Modern treatments might of course have helped the spasms, but the cognitive result would likely have been the same.
West's letter describes the functional neurological catastrophe which is in the process of overtaking his own infant son. To modern eyes, the style of the letter seems formal, and the clinical description is written with the observational sang-froid one might expect of a serious clinician. How- ever, his account of the lengths to which he went to find a diagnosis and treatment is evocative more of parental desperation than of academic interest. At the end of the letter, he pleads that the editor of the Lancet will give the "fullest publicity" to the illness he described. One way or another, his plea has surely been answered, and West's name became attached to the illness he described. For West, the price was high. One would guess from his letter that he would gladly have given up his place in history for the health of his son.
R. Duncan
See also:  Cone TE Jr. On a peculiar form of infantile convulsions (hypsarrhythmia) as
described in his own infant son by Dr. W.J. West in 1841. Pediatrics. 1970

1 comment:

  1. Eling P, Renier WO, Pomper J, Baram TZ. The mystery of the Doctor's son, or
    the riddle of West syndrome. Neurology. 2002 Mar 26;58(6):953-5.

    Although the eponym "West syndrome" is used widely for infantile spasms, the originators of the term and the time frame of its initial use are not well known. This article provides historical details about Dr. West, about his son who had infantile spasms, and about the circumstances leading to the coining of the term West syndrome.

    Dr. West’s description of infantile spasms is still considered a classic. As described in the letter, the spasms often begin during the first year of life, occur in series, and may arise in infants who are otherwise seemingly healthy. However, these infantile spasms are associated with intellectual and motor deterioration. As noted in his letter, Dr. West assumed that the spasms were caused by an irritation of the nervous system, perhaps caused by teething. Attempting to treat this neuronal irritant, he initiated leeches and wet towel applications, and considered “calming tonics” as well as “calomel” (mercury-chloride). Dr. West then proceeded to treat the potential source of this nervous irritation, “phlogiston,” an ethereal substance assumed to be present and active in flammable matter. Phlogiston was generally regarded as the mechanism for the production of both fire and fever. Initially, the infant was put on a hot bath regimen. However, 4 weeks later, when the seizures were even worse, Dr. West turned to stronger calming agents (sedatives), including opium. When these “first-line” agents failed, West treated his then 8-month-old boy with drugs (alterantia), which, according to the Galenic system, alter the composition of body humors. However, neither these nor castor oil were effective. Notably, Dr. West attempted to treat his son’s malady by making assumptions about its etiology and pathophysiology. When this approach failed, he relied on “standard” potions in the medical armamentarium of his day...

    In Western literature, the 100-year period after West’s letter includes few papers on infantile spasms and the entity known today as West syndrome, and these papers were mainly from Germany and France. The first follow up of four cases, including the Doctor’s son, was by Newnham, in 1849.14 Newnham did not refer to West syndrome, but used the term “eclampsia nutans.” Féré15 described tic de Salaam or salutation and cited several previous authors including West. Féré” recognized that these “tics” might also result from CNS disorders such as birth trauma, meningitis, or stroke. He also noted the association of the spasms with mental retardation, and he was the first to distinguish between symptomatic and idiopathic forms of infantile spasms. This distinction was reintroduced by Zellweger in 1948...

    Throughout this 100-year epoch—between West’s letter in 1841 and until 1960—no mention of the term “West syndrome” is found in the literature. Thus, how and when did this term for the triad of infantile spasms arise?

    In 1960, Gastaut organized the 9th Colloque de Marseille (Marseille Colloquium), which focused on infantile spasms. The proceedings of this meeting have been published in the book L’Encephalopathie Myoclonique Infantile avec Hypsarythmie (infantile myoclonic encephalopathy with hypsarrhythmia).19 It was at this conference that Gastaut suggested West’s eponym for infantile spasms, which is widely used for the syndrome to date.