Hydrocephalus —The remarkable case of James Cardinal

Hydrocephalus —The remarkable case of James Cardinal

4 Surg Neurol 1987;27:4-8 Hydrocephalus The Remarkable Case of James Cardinal From Reports of Medical Cases, Volume II, 1831, by Richard Bright Edi...

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Surg Neurol 1987;27:4-8

Hydrocephalus The Remarkable Case of James Cardinal From Reports of Medical Cases, Volume II, 1831, by Richard Bright

Editor's Note In the Dictionary of Scientific Biography, Lester S. King writes: "The first third of the nineteenth century brought forth an extraordinary flowering of medical talent, among whom Bright was a truly outstanding figure." Although Richard Bright's name most often is associated with renal disease, "we must not ignore his broad interest in the entire field of internal medicine" [4]. Indeed, Bright's interests spanned an enormous range of disciplines, from geology to criminal justice. Even after turning to medical pursuits, he temporarily interrupted his studies at Edinburgh in 1809 to join an expedition to Iceland. During this and later journeys through Europe, Bright kept detailed notes and made meticulous drawings of the things he saw, drawings remarkable for their artistry and appeal. Bright's Travels attest to the man's gift of expression, both verbal and visual, and to his extraordinary character as physician and humanist. It is no surprise that his early interest in geology should have preceded his particular interest in anatomy. As his drawings and careful observations suggest, Bright was intrigued by form and found a special beauty in the structure of the world and of the human body. Burton Chance notes that when one recalls his later years, one must conclude that his genius as a clinician was of a piece with his priceless artistic

faculty. While many of his medical illustrations later on were by his own hand, those in his great work published in 1827 were executed under his direction and for which he held himself responsible as to the accuracy of their portrayal of the specimens. The beauty of these plates has not been surpassed although they werepublished 113 years ago; and by them was inaugurated an epoch of artistic excellencein pathological illustrations [2] [Figure 1]. The case ofJames Cardinal, to whom John Homans makes passing reference in his Textbook of Surgery as the boy "whose head is described by Bright as translucent with the sun shining behind him" [3], is valuable not only for its own merits but as illustrative of the talents of Richard Bright as well.

Case CCV. James Cardinal, aged 29, was admitted into Guy's Hospital, under the care of Sir Astley Cooper, D e c e m b e r 1st, 1824. H e was the subject of chronic hydrocephalus in a very marked degree, and had lately been in St. Thomas' Hospital for several months, under the care o f Mr. Green; his case, however, admitting of little medical and no surgical aid, he had been dismissed. His general appearance was very remarkable, from the extraordinary disproportion between his head and the other parts of his body; for he was well-formed, and nearly of the middle stature, but his head was at least twice the size which his spare body would lead us to expect. (Plate X X X I I I [Figure 2].) His countenance was not wanting in intelligence, though his forehead was very prominent, and there was a peculiar lengthened appearance in his eyes. On inquiring from his mother more particularly into his history, it was collected that he was born at Coggleshall in Essex, March 2nd, 1795, and that his head was then very little larger than natural, and had a pulpy feel, being apparently almost destitute o f bony matter. A fortnight after birth the head began to increase considerably, and by the end of the first month bone was obviously formed. The growth of the head proceeded with great rapidity till he was five years old, at which time the mother is of opinion that it was little less than at present. When he was about three years old, he bled at the nose in small quantities for several weeks at intervals of a few days. H e was unable to walk alone till he was nearly six years old, and then only on a level surface: if he attempted to run or stoop, he fell down, and upon one occasion about that period he lacerated his forehead by a fall; there was some little bleeding from the wound, and he was laid up for a week or two. At this time (when about six) he was sent to school, and soon learnt to read well, and write tolerably; the writing, however, was discontinued in consequence of the pain it produced in his head, which from being near-sighted, he was obliged to hold very low. When a child, his health was very good; and up to four or five years ago his appetite was ravenous; he was always very weak, and used to shake as he walked along. If a candle was held

Hydrocephalus

Surg Neurol 1987;27:4-8

REPORTS OF

MEDICAL

CASES, SELECTED

WITH A VIEW OF ILLUSTRATING

THE SYMPTOMS AND CURE OF DISEASES BY A REFERENCE TO

MORBID

ANATOMY.

BY RICHARD BRIGHT, M.D.F.R.S. &c. LECTURER ON THE PRACTICE OF MEDICINE, AXCnONZ or TH~ ~YSlCIANS To GUY'S HOSPITAL.

V O L U M E II. DISEASES

OF THE

BRAIN

AND NERVOUS

PART

SYSTEM;

I.

INCLUDING INFLAMMATION OF THE BRAIN AND ITS MEMBRANES;--ACUTE HYDROCEPHALUS; DELIRIUM TREMRNS ;--APOPLEXY;--PARAPLEGIA;--CONCUSSION ; CHRONIC HYDROCEPHALUS;--SPINA BIFIDA.

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disposition to sleep. Although he walked about quite erect, he was not altogether firm on his feet, but sometimes tottered a little and required support, and on stooping, giddiness was produced, so that he sometimes overbalanced himself. He was near-sighted, but heard with remarkable acuteness; his taste was perfect and his digestion good. His mental faculties were very fair: he read and wrote pretty well, but if he read long his eyes were apt to be weak and inflamed: his memory was tolerable, but it did not retain dates and periods of time; and it was stated of him that he was never known to dream. There was something childish and irritable in his manner, and he was easily provoked. He was stated not to have sexual desires, but he was fond of society, and affectionate to his mother. His voice was not manly, but feeble and somewhat hoarse: his bowels generally costive, his head ached occasionally; and if he moved it suddenly or violently, he used to complain of a sharp pain as if a penknife were entering his brain. He always lay on his left side, and was unable to lie on the right. He remained pretty well for a few weeks after his admission into Guy's Hospital, when he appeared to have renewed his cold; and febrile symptoms coming on, he lost his appetite entirely, had some diarrhoea, became exceedingly feeble, and died on the 24th of February 1825.

LONDON: PRINTED BY RICHARD TAYLOR* RED LION COURT, FLEET STREET. ~nLlsa~V BY LONGMAN, REES, ORME, BROWN, AND GREEN, PATERNOSTER-ROW;

^ND S. HIGHLEY, 174, FLEET-STREET. 1831. F i g u r e 1.

Title page o / R e p o r t s

of Medical Cases

by Bright.

behind his head, or the sun happened to be behind it, the cranium appeared semitransparent; and this was more or less evident till he attained his fourteenth year. Fits of an epileptic character came on at about the age of twenty-three, and since that his health has rather suffered. The bones have been closed about two years; the anterior fontanelle being last ossified. Since their closure he has had shooting pain in the vertex. In the summer of 1823 an abscess formed in his ear, which discharged for some time, and relieved his head greatly. At the time of his admission he appeared in tolerable health, except a slight cold, and the circumstance of his being liable to fits, which occurred at very irregular intervals, half a year sometimes intervening; while at other times, three or four would happen in a month; the last attacks had been in June, when they returned on two successive days. These fits came on without the slightest warning, and sometimes during sleep: they generally lasted about twenty minutes or half an hour, and were attended by foaming at the mouth, and followed by a

Sectio C a d a v e r i s On dissecting back the scalp and raising the calvaria, it was found that the whole of the upper part of the cranium had been filled with a clear transparent fluid contained only within the dura mater, and that the brain itself occupied the base of the cranium; the parts being so displaced that it was no easy matter to recognize all their bearings. It soon, however, became obvious, that what we saw looking like the flattened superior surfaces of the two hemispheres, was in fact the two lateral surfaces, which in health are opposed to each other through the centre of the brain; these had been separated and thrown back by the pressure of the fluid which, having first accumulated in the lateral ventricles, had forced a preternatural opening on one side of the corpus callosum, which, together with the whole fornix, had been nearly obliterated; and as the fluid increased, the hemispheres were gradually brought down to the base of the skull. The right hemisphere was quite flat, but the left was raised into a point at the side of the skull. About one pint of fluid was found in the ventricles, and probably, six or seven pints externally; for when the skull was completely freed from its contents, both solid and fluid, it would hold ten pints of water. The ventricles were so much disfigured by the fluid within, as well as by the peculiar position the brain had assumed, that it

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Surg Neurol 1987;27:4-8

Figure 2 (right). "His head was at least twice the size which his spare body wouM lead us to expect."

Figure 3 (below). "Appearance of the brain when the water was taken away."

Bright

Hydrocephalus

Figure 4. "The process which nature had adopted in closing up the unusual spaces between the regular bones of the cranium by the intervention of numerous ossa triquetra."

Surg Neurol 1987;27:4-8

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was with difficulty the various parts were discovered; to add to which difficulty--as it was thought advisable to procure drawings of the appearances,--two or three days elapsed before we could pursue the dissection more particularly, and then many o f the finer parts were rendered indistinct by exposure. The cerebellum was flattened and vascular. The accompanying engraving (Plate X X X I V [Figure 3].) gives a very faithful representation of the recent appearance of the brain when the water was taken away; and the skull itself being preserved in the Museum of Guy's Hospital, together with the whole skeleton, I have availed myself of the opportunity of having lithographic drawings executed from it, one o f which shows in a very remarkable manner the process which nature had adopted in closing up the unusual spaces between the regular bones o f the cranium by the intervention o f numerous ossa triquetra. (Plates X X X V . X X X V I . and X X X V I I . [Figure 4].) On carefully taking the different measurements of the skull, it was found that its circumference at the part through which it was divided, and which was as nearly as possible its largest diameter, was thirty-two inches and a quarter. The distance from the articulation o f the jaw on one side, to the corresponding point on the other, measured over the vertex, was twenty-one inches: from the insertion o f the ossa nasi into the frontal bone, to the posterior margin of the foramen magnum, twentythree inches and three-eighths. The long diameter from the projection of the os frontis to the projection o f the os occipitis, where the skull was divided, was ten inches and a half, including the thickness o f the skull; and one inch to the right of that line, was eleven inches: the transverse diameter, measured across the foramen magnum, was nine inches. The ossification of the skull might be considered complete; but at the anterior parts of what formed the anterior fontaneUe there was a small space where the bone was very thin, and even deficient, to an extent which would admit three or four pins' heads. The skull was very thin, except in a few places of small extent, as at the ridge of the os occipitis, and a ridge running along the inside of the left parietal bone. In the thicker parts more particularly, the bone on the inside had a spongy or worm-eaten appearance, and numerous small vessels seemed to have been imbedded in it. Between the chief bones there were numerous ossa triquetra of various sizes and forms, united together by well-formed sutures; of these there were not fewer than forty-five along the lambdoidal and sagittal sutures, and ten between the os frontis and the parietal bones; and there were some, badly defined, on the squamous suture of the temporal bones. The frontal suture was not perfectly obliterated.

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Bright

This case is very remarkable for the length of time to which life was protracted, and still more for the degree o f intelligence which was manifest in spite of the large accumulation of fluid; and also for the state of bodily health which was enjoyed. It is not improbable that much of this immunity from mental and corporal ailment depended on the situation which the fluid occupied; for after moderately distending the ventricles, it ceased to act in that way, having gradually opened for itself a passage by the side o f the corpus callosum, and thus the further effusion found its way externally to the brain. The mass of cerebral matter was scarcely diminished, and it appears probable that the effusion had not increased from an early period. The skull in this case illustrates very beautifully, the mode in which the unnaturally wide spaces between the bones become ossified, by the formation of numerous ossa triquetra. This process is often to be traced in its earlier stages, both on the living subject and in preparations . . . . From the Preface to

Reports of Medical

Cases [ 1] More than three years have now elapsed since the publication of my first volume of "Reports"; and if the kindness with which that work has been received by the

Profession has afforded constant encouragement in the progress of the present volume, it furnishes, now that the labour is complete, an additional source of anxiety, lest I should be about to forfeit any part of that good opinion which has been too liberally expressed. I am not however conscious that either my industry or my zeal has abated; and my opportunities have undoubtedly increased, not only by the ordinary circumstances which extend a professional man's sphere o f experience, but by the great augmentation which has taken place in the sumptuous Hospital to which I am attached; and in which I am proud to say that my Colleagues, whether medical or surgical, have by their unremitting kindness shown how little jealousy interferes between two professions, which as they are most properly separated in practice, should ever be united in study, in science, and in spirit.

References 1. Bright R. Reports of medical cases, selected with a view of illustrating the symptomsand cure of diseases by a reference to morbid anatomy. Vol 2. London: Richard Taylor, 1831:vii, 431-4. 2. Chance B. Richard Bright, traveller and artist. Bull Hist Med 1940;8:909-33. 3. HomansJ. Textbook of surgery.4th ed. Illinois:CharlesC Thomas, 1936:532. 4. King LS. Richard Bright. In: Gillispie CC, ed. Dictionary of scientific biography. Vol II. New York: Scribner's, 1970:463-5.