Browns Equa Rd and His Syndrome

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THE LANCET Vol 356 July 1, 2000 61

Brown-Squard syndromeThe physical signs of classic Brown-Squard syndrome areshown in figure 2. The points to note are total loss ofsensation and flaccid paralysis at the level of the lesion dueto destruction of nerve fibres at that segment of the spinalcord. The spinothalamic tracts enter the cord and travelipsilaterally for one to two levels before crossing over, and,therefore, the contralateral loss of spinothalamic sensationbegins a few spinal levels below the site of the lesion(figure 3).

Students are taught that this syndrome is caused byhemisection or lateral injury to the spinal cord, andconsists of ipsilateral motor weakness and hypoaesthesiawith contralateral loss of pain and temperature sensation.Most studies on Brown-Squard syndrome in the 20thcentury have been of sporadic cases generally traumatic orneoplastic in origin, but with a variety of causes.1 The fullclassic syndrome is normally caused by extramedullarylesions but intramedullary inflammatory lesions, such as inmultiple sclerosis can result in partial or complete Brown-Squard syndrome.

Lancet 2000; 356: 6163University Hospital, Nottingham NG7 2UH, UK (R Tattersall MD, B Turner MB)

Correspondence to: Prof R Tattersall, Curzon House, Gotham,Nottinghamshire NG11 0HQ, UK

Charles-Edouard Brown-SquardCharles-Edouard Brown-Squard (181794)2,3 was one ofthe most remarkable medical men of the Victorian age andis remembered particularly for his contributionsto neurology (admiringly) and to endocrinology(pejoratively). He was born in Mauritius to an Irish-American sea captain and a French mother. Mauritius hadbeen ceded to Britain in 1814, thus although he did notlearn English until 1852, Brown-Squard was for most ofhis life a British subject. He claimed that he used hismothers and fathers combined names to distinguishhimself from all other Browns. Brown-Squard had a flairfor self-publicity and was as well known in England andthe USA as in France. He was an extraordinarily energeticman who frequently worked 20 h a day and published 577papers. Michael Aminoff speculates that for much of hisadult life, Brown-Squard had periods of malaise anddepressionwhen things became too much for him, hewould move, usually to another country. He is said to havecrossed the Atlantic more than 60 times and set upresidence in the USA four times, France six, and Englandonce.

NeurologyBrown-Squard moved to Paris intending to become awriter but, after his manuscripts were repeatedly rejected,he became a medical student. He qualified in 1846 bydefending his thesis on physiology of the spinal cord. Theanatomy of motor and sensory nerves and spinal pathways

Brown-Squard and his syndrome

Robert Tattersall, Benjamin Turner

Eponyms

Case presentation

A woman aged 41 years presented with a 5-day history ofprogressive weakness of the right leg with a pronounced limpand difficulty climbing stairs. For 2 days she had also noticednumbness in her left leg, but no back pain or bladderdisturbance. She had previously been fit, did not smoke anddrank little alcohol. In the right leg there was sustained clonusat the ankle with pyramidal weakness and an extensor plantarresponse. The left leg had normal tone, power, and plantarresponse. Sensory examination showed absence of pain andtemperature sensation between the thoracic levels T710, andabsent vibration and proprioception caudal to T7 on the right.On the left, there was absence of pain and temperature withpreserved vibration and proprioception caudal to T7 on the right.On the left, there was absence of pain and temperature withpreserved vibration and proprioception sensation below the T10level. Magnetic resonance (MR) images showed anintramedullary lesion towards the right at the level of T7 and T8;the spinal cord was expanded and active inflammation wasseen after gadolinium was administered. Oligoclonal bands inotherwise normal cerebrospinal fluid and on MR images of thebrain showing three periventricular high-signal lesionssuggested a background of chronic inflammation of the centralnervous system (figure 1). The woman was treated fortransverse myelitis and given a course of intravenousmethylprednisolone after which she made a moderate recovery.

Figure 1: Magnetic resonance imageShowing demyelinating lesion in the right side of the spinal cord.

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of sensation were poorly understood at the time.Before 1825, Charles Bell (17741842) and FranoisMajendie (17831855) had shown that the anteriornerve roots were motor and the posterior sensory(priority for this discovery was contested but it is nowremembered as the Bell-Majendie law). Brown-Squards doctoral thesis was separated intotwo parts. In the first, he noted thatimmediately after spinal-cord section in thefrog, reflexes were lost, but weresubsequently recovered and exaggerated; inthe second part, he studied the effects oflesions in various parts of the cord, the mainconclusion being that the posteriorcolumns were not the main sensorypathways.

The finding for which Brown-Squardlater became famous, that lateralhemisection caused ipsilateral hyper-aesthesia and lost or diminishedsensation in the contralateral limb,was first published in 1849. In 1858,he made his first visit to England,here he gave six lectures at the Royal Collegeof Surgeons on physiology and pathology of thenervous system. These lectures and some inGlasgow in 1859 were such a success that hewas headhunted for the newly establishedNational Hospital for the Relief of the Paralysedand Epileptic (later the National Hospital forNervous Diseases, Queens Square, London). Here,Brown-Squard became a victim of his own success,and it is said that he decided to leave when he lookedout of the window of his consulting room and saw thesquare gridlocked by the carriages of his fashionablepatients. At the 1862 annual meeting of the BritishMedical Association,he described a typicalcase of his syndrome,a sea captain whohad been stabbed inthe neck. Immediatelyafter the injury, thepatient had had acomplete right andpartial left hemiplegia,but 8 years later,when Brown-Squardsaw him, the righthemiplegia had partlyand the leftcompletely recovered.What interestedB r o w n - S q u a r dwere the sensoryfindings; in theparalysed limbs tactilesensation to tickling and temperature was greatlyincreased, but was lost in the non-paralysed left limbs.When he returned to the subject in The Lancet in 1869 hestated that a lesion in one of the lateral halves of the spinalcord produces: 1st, paralysis of voluntary movements inthe same side; 2nd, anaesthesia to touch, tickling, painfulimpressions, and changes of temperature in the oppositeside; 3rd, paralysis of the muscular sense in the sameside.4

EndocrinologyBrown-Squards first work in endocrinology was in1856 when he reported to the Academy of Sciences inParis that removal of both adrenals in various animals

was fatal, mostly within 24 h. He concluded that thefunction of the adrenal glands is essential to life and

is one of the most important functions in theanimal physiology.5 These experiments wereprobably stimulated by Addisons monograph,which had been discussed at the Academy ofMedicine in Paris in August, 1855, whenTrousseau suggested the eponym, Addisonsdisease. Olmstead termed Brown-Squards

next contribution to endocrinology aspectacular finish to a spectacular life. Theoccasion was a meeting of the Society ofBiology in Paris on June 1, 1889, when

Brown-Squard reported that he hadrejuvenated himself with dailyinjections of a solution of testicularblood, seminal fluid, and testicular

extract from guinea pigs and dogs.The premise for his experiments was that celibateshad unusual mental and physical energy, which

suggested that the testes produced a dynamogenicsubstance. For Brown-Squard the conclusive factorwas that the injections had increased his strength sothat he could now run upstairs and lift weights67 kg heavier than before. Most impressively, theaverage length of his jet of urine had increasedby 25%.

These observations were greeted with incredulityin England where the British Medical Journal

compared them with the wild imaginings of medievalphilosophers in search of an elixir vitae.6

An anonymous doctor wrote: I consider theidea of injectingthe seminal fluidof dogs and rabbitsinto human beingsa disgusting one, andwhen the treatmentalso involves thepractice of masturba-tion, I think it is timefor the medical pro-fession in England torepudiate it. Vivisec-tion may be an openquestion, but self-abuse is not. Thepopular press had afield day at Brown-Squards expenseand most doctorsand physiologistsin England and

Germany were extremely sceptical about his claims.Nevertheless, by the end of 1889, more than 12 000 doctorsworldwide were using his fluid with various results. A UScorrespondent to The Lancet reported sarcastically that Inthe hands of one experimenter, the paralysed immediatelywalk, the lame throw aside canes and crutches, the deafhear, and the blind see. The same experiments failedaltogether in the hands of another. Brown-Squardsupplied his organic preparations free to doctors with the

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Figure 2: Features of classic Brown-Squard syndrome

Ipsilateral loss of all sensory modalities at the level of the lesion

Ipsilateral flacid paralysis at the level of the lesion

Ipsilateral spastic paraparesis below the lesion

Ipsilateral loss of vibration and position sense below the lesion

Contralateral loss of pain and temperature below the lesion



proviso that they relayed the results to him. When hepresented his last report to the Academy of Sciences in1893, he claimed that the best results with testicularextract had been obtained in tabes dorsalis (benefit in 314of 405 cases). Even such difficult conditions asParkinsonism and diabetes were helped, if not cured. Hewas careful not to claim that testicular extract was auniversal panacea, but suggested that it built up thenervous force and added to the bodys defences.

In an article in the British Medical Journal in 1893,Brown-Squard claimed that he had founded the scienceof endocrinology with his adrenalectomy experiments in

1856, and that ever since he had believed that all tissuesproduced an internal secretion.7 This belief was supportedby Minkowskis (1889) findings that pancreatectomycaused severe diabetes and Murrays (1891) study on thecure of myxoedema by thyroid extract. Immodestly,Brown-Squard concluded that the great movement intherapeutics as regards the organic liquid extracts hasorigin in the experiments I made on myself in 1889,experiments which were at first so completelymisunderstood. In an accompanying editorial theBritish Medical Journal commented that: Though manyjeered at him as the discoverer of the secret ofperpetual youth, the notion has steadily gained groundthat there is, after all, something in it. Since also, thesuccess that has followed the injection of thyroid extract inmyxoedema, we can hardly wonder that this belief hasincreased.

The drought that descended on endocrinology, and thedisrepute into which it fell, until the discovery of insulin in1921 was laid squarely at Brown-Squards door. At thetime of formation of the Endocrine Society in 1917, it wasthought that any young clinician who showed an interest inthe subject was straying from the path of seriousmedicine and heading for the endocrine goldfields.However, history judges Brown-Squard as a scientist, hecomes across as a lively character who is described in theLives of Fellows of the Royal College of Physicians as awholly likable and humorous man. It is fitting, at a timewhen eponyms are shunned, that Brown-Squard is, likeAddison, a name known to all medical students.

References1 Koehler PJ. The Brown-Squard syndrome: true or false? Arch Neurol

1986; 43: 92124.2 Olmsted JMD. Charles-Edouard Brown-Squard: a 19th century

neurologist and endocrinologist. Baltimore, USA: Johns Hopkins Press,1946.

3 Aminoff MJ. Brown-Squard: a visionary of science. New York: RavenPress, 1993.

4 Brown-Squard CE. Lectures on the physiology and pathology of thenervous system and on the treatment of organic nervous affections:lecture II, part 1. Lancet 1869; i: 13.

5 Brown-Squard CE. Recherches exprimentales sur la physiologie et lapathologie des capsules surrnales. C R Acad Sci 1856; 43: 42225.

6 The pentacle of rejuvenescence. BMJ 1889; 1: 1416.7 Brown-Squard CE. On a new therapeutic method consisting of the

use of organic liquids extracted from glands and other organs. BMJ1893; 1: 114547.

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Figure 3: Major spinal cord tracts and relation tohemisection of cordHemisection shown in green.


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Browns Equa Rd and His Syndrome