The first descriptions of syringomyelia
Authors: Andreas K Demetriades
Journal: Journal of the Royal Society of Medicine (2012)

The historical significance of the paper by Jacob Lockhart Clarke and John Hughlings Jackson was eloquently presented.1 Claims of ‘first’ descriptions, however, are almost always subject to interpretation.

The first description of the clinical syndrome of syringomyelia is generally attributed to Sir William Gull (1816–1890), who presented in 1862 a case of progressive atrophy of the hands.2 He had a lasting interest in spinal cord pathology, his description of paraplegia forming a large part of the Goulstonian lectures he delivered in 1848. It is conceivable that Gull and Clarke interacted at Guy's Hospital, where the former spent his career and the latter studied towards becoming a licentiate of the Society of Apothecaries.

The first description of the term ‘syringomyelia’ came in 1827 by Charles Prosper Ollivier d'Angers (1796–1845), who published a case of spinal cord cavity in continuity with the fourth ventricle. Changing career from the military, having lost in the battle of Waterloo, he studied medicine in Paris. He was a contemporary of Francois Magendie (1783–1855) reflecting the growing interest in Parisian medicine in the structure and function of the central nervous system. Interested in comparative embryology and anatomical pathology of the spinal cord, he published his treatise on the spinal cord and its diseases.3 The term appears in the second edition of three (1827); it was applied in a general manner to the existence of any canal or cavity within the cord, and d'Angers regarded a central canal as pathological. In his chapter dealing with the defects in the formation of the cord he writes: ‘Many anatomists have described a central canal in the spinal cord. Ch. Etienne, in his description of the cord, says that such a canal is always present, and that it is continued into the brain. This opinion is also held by Colombo, who compares the canal to that of a quill; it is likewise accepted by Piccolhomini, Bauhin, and Malpighi. I have, however, clearly demonstrated from the internal structure of the spinal cord that in its normal state it does not contain a central canal’.

Through to the modern day there has been confusion as to the pathophysiology of syringomyelia. Amongst neurosurgeons the term hydromyelia is generally used for a dilated central canal, whereas syringomyelia refers to cystic cavities within the cord. Cystic cavities per se in the spinal cord had actually been described much earlier; by Johann Conrad Brunner (1653–1727) in ‘Misc. Nat. curios.’ in 1688 and Giovanni Battista Morgagni (1682–1771) in 1761 in his ‘Adversaria Anatomica VI’. Nevertheless, Charles Estienne (1503–1564), also known as Carolus Stephanus, contemporary of Andreas Vesalius, provided the first description of cavities in the spinal cord, but also of the entity of the central spinal canal, in his treatise ‘La dissection du corps Human’ (1543).

In summary, Estienne's ‘first’ was an anatomical description and distinction between the spinal canal and cystic cord cavities. D'Angers’ ‘first’ was the coinage and use of the pathoanatomical term syringomyelia. Gull's ‘first’ was the correlation of anatomy to function and the description of the clinical syndrome of syringomyelia. Clarke and Jackson helped popularise the term in an era of evolution for British neurology.