Chiselborough 1850, Goitre and Endemic Cretinism—'Chiselborough Neck'

by Simon Spurway


 

Around the mid-1800’s there were scientific reports of a ‘malady effecting the Somersetshire village of Chiselborough’, being the medical condition of goitre. Goitre results from a malfunctioning of the thyroid gland and is most commonly associated with a deficiency of dietary iodine and/or hereditary issues. This however was unknown at these times. If evident from birth it is termed as cretinism, where both mental and physical disabilities might be evident.

As you will shortly see, its impact is dramatic. The condition’s local name of ‘Chiselborough neck’ reflected the gross physical distortion of the thyroid gland evident in individuals unfortunate enough to suffer.

The original report (*1), which caused notoriety for the village throughout the English and Scottish newspapers, was prepared by Dr. Hugh Norris, Senior House Surgeon to the Royal Maternity Hospital, Edinburgh. He was also, at time, a practitioner in South Petherton. The report was initially presented to the Royal Medical Society of Edinburgh in 1847. Later it was published by The Medical Journal of London and also copied, together with comment, in many nationwide newspapers.

For copyright reasons the version below is abridged.

Note that an original medical description of cretinism was defined as ‘a condition characterized by physical deformity and learning difficulties that is caused by a congenital (evident from birth) thyroid deficiency’.


NOTICE OF A REMARKABLE DISEASE ANALOGOUS TO CRETINISM EXISTING IN THE SMALL VILLAGE OF CHISELBOROUGH, WEST OF ENGLAND

Dr Hugh Norris MA, MD, Cantab, Senior Surgeon Edinburgh Hospital

Royal Medical Society of Edinburgh, 26th November 1847

Chiselborough Village is hemmed in an extremely small valley, its hills of densely wooded orchards; lowest part damp and dirty, temperature mild. Village open only towards the west, where flows the sluggish Parrett. Population, 540 at the last census.

In Chiselborough I have ascertained to exist four groups of people, all suffering from an extremis of goitre with grossly enlarged thyroid and thus medically termed as cretins, or cretinous.

I. Four perfect idiots : 2 male, 2 female.

II. Fourteen partial idiots or imbeciles : 4 male, 10 female. All unable to articulate so as to be understood, and with memories so deficient as to prevent their being trusted in the simplest affairs of life. Though not prone to mischief, all require constant watching, walking with an uncertain step and a waddling gait.

III. Five deaf and dumb villagers, all female. Two educated and of quick parts, two not so. One but little removed from perfect idiocy.

All members of the above groups have enlarged thyroid glands.

IV. To the above I add a final class, a majority of the whole population, having deficiency of intellect, usually dull perception, and marked impediment of speech and largely affected by bronchocele. It is noted that the most formidable instances of goitre are in this last class, and those with the largest tumours of the neck are subject to epileptic fits.

The appearance of these unfortunate beings is not that of the ordinary accidental idiot. Their appearance is exceedingly unprepossessing; stature is short, corpulent and bloated, limbs bony and rickety and malformed. Their lips are turgid, noses flattened, eyes are small and sunken. Heads are misshapen and covered with a profusion of coarse, ragged hair. Facial contour is wide and flat, forehead very low. Their expressions differ remarkably. Some always laughing; these are the least ill looking. Many gaze upon you with utter vacancy whilst others express constant suffering and patient endurance. Others gaze at you with utter vacancy. Amongst the females, I fancied I could detect an habitual peculiar lustful expression of the eye.

The walk of these cretins is more the waddle of the duck than the shuffle of the ordinary idiot. Generally they are very weakly, but some are capable of considerable bodily exertion. Their character is mild and inoffensive, unless provoked when they become highly dangerous. Temper is very uncertain, with outbursts of passion. They have no tendency to commit mischief for mischief’s sake, but their total ignorance of moral responsibility often causes acts of a mischievous nature which renders constant surveillance.

The very remarkable pre-ponderance of females over males must strike every one, their having monthly fits of wildness locally attributed to that much maligned planet, the moon. Surprisingly, men who would be deemed imbecile elsewhere still find women of no comparable problem willing to become their wives and bear families, but of a very mixed race. In general however ‘intermarriage’ seems more obviously prevalent than is the norm.

The disease is most prevalent in the lowest and poorest parts of the village, and yet well-marked cases are not wanting in the highest and most airy situations. I do not find instances of a whole family being affected, indeed some of the worst cases have siblings of modest intellect, performing domestic and agricultural labour.

It is not my purpose to enter into investigation of the cause of this malady, with the only known comparisons being the cretins of Switzerland who drink only melted snow. This obviously is not the case in Chiselborough. The extremely high practice of intermarriage has been reasoned as a cause for this malady, for circumstances render the parish so uninviting to strangers that the poor inhabitants are under the necessity of “putting up” with each other, and thus the family is often ‘kept-within’. My own impression is a combination of circumstance. The water is of inferior quality, as the offspring of strangers acquire bronchocele. The air is very impure, hemmed in by the hills. Great quantities of rotting vegetation exist in the orchards and several green stagnated pools also exist.

I may conclude by observing that, in one adjoining but impoverished parish, the inhabitants are remarkable for their healthfulness.

Dr Hugh Norris MA, MD, Cantab


By 1871 the problem seems to have disappeared. Dr. Charles Fagge of Guy’s Hospital (*2) contacted Dr. Norris at his residence at South Petherton, who reported back that ‘improved sanitation, better food, better education, fewer inter-marriages and greater contact with the outer world combined to eradicate the disease. Only scattered examples now remain’. It is also possible that the shorter life span associated with cretinism caused sufferers to die out in the interim. Norris’s reply however gives no indication of any understanding at this time of the significance of dietary iodine.

However, Norris’s original paper raises interesting comments and questions :

• How/why did Chiselborough’s situation come to the attention of Dr. Norris? How widely was it known about and reported before his paper?

• How was the standing of the village impacted in the wider community?

• Is there supporting evidence of this dramatic problem? Many people referenced Norris’s work, but as for stand-alone descriptions there are only a couple. One is by Dr. Read (*3) who earlier in 1836 in his History of the cause of Bronchocele reported that ‘Chiselborough in Somerset contained 24 well defined cretins in its population of 360’. Unfortunately the original document can’t be found in order to obtain further details. Then later a German expert, Dr. Guggenbuhl (*4) was reported in The London Medical Gazette of I85I to have made a tour of England and to have found cretinism in several of the counties, notably in Chiselborough, Somerset.

• Does other, earlier evidence of the Chiselborough problem exist that we as yet are unaware of?

• There were other reported cases of cretinism reported in England, e.g. Leek in Derbyshire. The Chiselborough situation appears however to have been a ‘worst case’ and is reported elsewhere as being ‘England’s only endemic case of cretinism’.

• If a lack of dietary iodine was to blame, what was its cause? The food diet of Chiselborough would have been largely the same as for surrounding villages (without goitre). So, was the local water to blame? If it was, then logic suggests the problem would be the more deeply sourced natural springs (e.g. at Roundwell, North Street, and Springfields) that most likely pass through hard ground rock rather than water from locally dug wells which obtained their water from the natural water table. The latter would be expected to have a comparable mineral/element content to that of neighboring villages (again without goitre).

• What part did the significant level of inbreeding play? Thyroid problems can be hereditary, so it must be a candidate cause along with a lack of dietary iodine. Multi-generational inbreeding could have exacerbated things further.

• For how long had this problem existed in the village? Was it something that had endured since early settlement or something that had come about later on?

• Such an extensive malady could have been expected to cause people to move away from the village and yet the village population was at a historic high (1841 census number of 540). Today’s census states 275.

• Are there any medical/municipal/parish/church/school records after this report that detail efforts to curb the goitre problem of Chiselborough?

• Is there exaggeration in the report of Norris? It may be assumed that a medical report that could easily be cross examined and challenged should be accurate in statement. That said, much of the support information, e.g. ‘the air is very impure, hemmed in by the hills’, is subjective, possibly intended to ‘embellish the story’.

• What was the impact upon village life, finances and resources of so many people with such limited capacity/capability and high care needs? They all had to be housed, fed and clothed, but offered little in return, at best restricted manual labour.

• Why were the cases most prevalent lower down in the village? Presumably this relates to the high concentration of buildings.

• What picture of Chiselborough village life in the 1840’s does this report and other sources give us? The description is not of a pretty village with wisteria and rose adorned old cottages as of today, but of a dirtier place with stagnant water, a perceived lack of airflow and rotting vegetation. In other words, workaday for its time. The number of individuals living in each cottage would have been significantly higher than as today and the number of dwellings smaller as confirmed by the Tithe map (1842). Most cottages would have been functional, not glamorous.

Many of the now older cottages would have been relatively young, whilst others have since disappeared. The Tithe and later maps also confirm that orchards were plentiful, particularly on the south (but north facing) valley flank. The earliest photographic evidence (late 1890’s?) reveals a valley with far fewer hedgerows and large trees than as today. As a result the strip lynchets higher up the hills are very evident, presumably with sheep grazing them. Today it is very difficult to make out the lynchets due to overgrowth. Likewise, Chiselborough Common was hard grazed by animals and chickens.

At the same time the areas’ farming and aligned trades were sufficiently productive so as to support a great many people. Sheep farming was flourishing as evident by the existence of the annual livestock fair on Fairplace. This would have been unusual for a small village.

There is however no getting away from the evidence that Chiselborough’s goitre/cretinism problem was probably the worst in England. Inbreeding, particularly amongst the goitreous inhabitants, was commonplace and above typical levels, and its effects very obvious. This became a self compounding problem as people from outside of the village were, in general, unlikely to want to ‘marry in’.

So there you have it, a very different Chiselborough to today. One final note (in jest) - if you are Chiselborough born and bred, you might want to keep quiet about it!

1. NOTICE OF A REMARKABLE DISEASE, ANALOGOUS TO CRETINISM, EXISTING IN A SMALL VILLAGE IN THE WEST OF ENGLAND. Dr H. Norris, Royal Medical Society of Edinburgh, 26th Nov 1847

2. ON SPORADIC CRETINISM OCCURRING IN ENGLAND, Dr C. Fagge, Guy’s Hospital 28th Feb 1871

3. SPORADIC CRETINISM Being a Thesis for the Degree of M.D. of Edinburgh University by W. G. T H0MPS0N, M.B., Ch.B., Edin., 1899

4. PREVALENCE AND GEOGRAPHICAL DISTRIBUTION OF ENDEMIC GOITRE F. C. KELLY, B.Sc., Ph.D., F.R.I.C. W. W. SNEDDEN, M.A., B.Sc., Ph.D. Chilean Iodine Educational Bureau, London. 1958

Notes:

• Correct thyroid function is now known to be dependent upon the presence of iodine-containing compounds from food or drink. Nowadays these are often added to table salt.

• Other causes of incorrect thyroid function are sex (females predominate), age (worsens over 40) and hereditary issues. In-breeding in Chiselborough could have exacerbated the latter.

• Correct thyroid operation is critical for the brain and bodily development of the unborn foetus and subsequent infant. Treatment in infancy will reverse the physical changes, but not the neurological damage.

• Someone with untreated cretinism would likely live into adulthood, but have a shorter than normal life expectancy and significant disabilities.