Jacob Lockhart Clarke's and John Hughlings Jackson's first description of syringomyelia

Jacob Augustus Lockhart Clarke (1817–1880) was an anatomist and neurologist. He was brought up by his mother in France, as his father had died when he was about 7 years of age. He returned to England in 1830, and was apprenticed to an elder brother, whom he followed into medicine. He studied in London at Guy's and St Thomas' Hospitals and was made a Licentiate of the Society of Apothecaries in 1841. He entered private practice in Pimlico and also performed microscopic research on the pathology and physiology of the brain and nervous system. His first paper, ‘Researches into the structure of the spinal cord’, was received by the Royal Society on 15 October 1850, of which he became a Fellow in 1854, and was published in the Medico-Churgical Transactions in 1851. He published much original work in several journals, including the Royal Society's Transactions and Proceedings, the Medico-Chirurgical Transactions, the Journal of the Microscopical Society, and Beale's Archives of Medicine. He discovered Clarke's column, also known as the nucleus dorsalis, and published on tetanus, diabetes, paraplegia, and muscular atrophy. He introduced the method of mounting cleared sections in balsam, a major advance in histological technique. He also collaborated with Duchenne in his studies on muscular dystrophy.¹

Clarke was awarded the gold medal of the Royal Society in 1864 and the royal medal of the Royal Society in 1864. He was made an Honorary Fellow of the King and Queen's College of Physicians in Ireland in 1867. He attended St George's Hospital and qualified as a surgeon late in life, obtaining his MRCS (Eng) in 1863. He later went on to obtain his medical degree at St Andrews in 1869 and became a Member of the Royal College of Physicians, London, in 1871, the same year in which he became Physician to the Hospital for Epilepsy and Paralysis in Regent's Park, which is now the National Hospital for Neurology and Neurosurgery in Queen's Square. He remained there until the end of his life, and died at his sister's house in Balham, London, on 25 January 1880. He was attended during his last illness by John Hughlings Jackson, his co-author on the paper we feature here.

John Hughlings Jackson (1835–1911) was born at Providence Green, Green Hammerton, Yorkshire, on 4 April 1835, the youngest son in a family of four sons and one daughter born to Samuel Jackson, a yeoman landowner and brewer, and Sarah, nee Hughlings. Apprenticed in York to William C Anderson MRCS, he read medicine at the York Medical and Surgical School, and continued his studies at St Bartholomew's Hospital, London, where James Paget was one of his teachers. After qualifying MRCS in 1856, he worked until 1859 as House Surgeon to the York Dispensary and returned to London in 1859. He considered giving up medicine to study philosophy, but was dissuaded by Jonathan Hutchinson. In 1863 he became Assistant Physician to the London Hospital and Lecturer in Physiology, and was appointed Physician in 1874, serving on the staff until 1894.

In 1862 Jackson became Assistant Physician to the National Hospital for the Paralysed and Epileptic in Queen Square, which had been established in 1859, and stayed on until 1906. He married his cousin, Elizabeth Dade Jackson, who was an author of children's stories, but they had no children. She died in May 1876 of an illness characterized by partial seizures complicating pregnancy. In 1868 Jackson, who had become MRCP (London) in 1860, was elected FRCP, delivered the Goulstonian, Croonian, and Lumleian lectures at the Royal College of Physicians, and was elected FRS in 1878.

Jackson is known for three specific areas of work: left cerebral hemispheric disease leading to speech defects, focal epileptic seizures (Jacksonian epilepsy), and the evolutionary hierarchy of the nervous system. His work relied on meticulous clinical observations of thousands of patients, whose symptoms and signs were recorded in great detail. He wrote more than 300 papers in various journals, including The London Hospital Reports, Brain, the Lancet, and the British Medical Journal. In 1892 he was one of the founding members of the National Society for the Employment of Epileptics, now the National Society for Epilepsy. He became known as the ‘father of English neurology’. He died of pneumonia at his home in Manchester Square, London, on 7 October 1911 and was buried in Highgate Cemetery.

Lockhart Clarke and Hughlings Jackson together presented this first account of syringomyelia to the Society in 1867. Unusually, the paper begins immediately with an account of a case, without any preamble, and the opening sentence is highly novelistic: ‘Mrs. H-, Aet. 38, was always delicate.’ After a description of the initial presentation, with pain and weakness in the right arm, shoulder, and neck, with progressive wasting of the muscle of the hand andforearm, Jackson gave a detailed account of the clinical progress and Clarke of the postmortem findings.

The term ‘syringomyelia’ first appeared a few years after the publication of Clarke and Jackson's paper. The first citation in the Oxford English Dictionary is from the fourth edition of Austin Flint's textbook A Treatise on the Principles and Practice of Medicine (1880): ‘Cavities of variable size and length and more or less centrally located, may be developed in the spinal cord in various ways. The name syringomyelus or syringomyelia is given to these pathological canals.’ But presumably it must have been coined before that.

Eponyms associated with Lockhart Clarke and Hughlings Jackson

Clarke's body: alveolar sarcomatous intranuclear bodies of the breast

Clarke's columns: the dorsal nucleus of the spinal cord

Jackson's cerebellar fits (also known as Stewart–Holmes syndrome): a specific movement disorder with posturing secondary to posterior cranial fossa pathology

Jacksonian epilepsy: focal seizures, often involving one limb, with generalized spread

Jackson's laws: 1. Those neural functions that are most lately developed are also the first to be lost. This is restated in a variation as Jackson's rule: after epileptic attacks, simple nervous processes are more quickly regained than complex ones. 2. ‘The study of the causes of things must be preceded by the study of things caused.’

Jackson–MacKenzie syndrome: brainstem syndrome, also known as alternating hypoglossal hemiplegia, whose main clinical features are ipsilateral ninth, 10th, and 12th cranial nerve palsies, producing anaesthesia of the pharynx and larynx, paresis of the palate, the sternomastoid and trapezius muscles, and the tongue, and contralateral hemiparesis, usually occurring as a result of a medullary tegmental stroke or tumour

Jacksonian march: the spread of tonic–clonic epileptic movements through contiguous body parts on one side of the body as the causative epileptic discharge successively excites adjacent cortical regions, characteristic of Jacksonian epilepsy

Jackson–Weiss syndrome: a dominantly inherited form of craniosynostosis, mapped to chromosome 10q, characterized by broad toes and fusion of the tarsal bones

Selected bibliography by Lockhart Clarke and Hughlings Jackson

Researches into the structure of the spinal cord. Philosophical Transactions of the Royal Society of London, 1851;141:607–21. Description of Clarke's columns and his method of mounting cleared sections in balsam.

Observations on defects of sight in brain disease. Ophthalmic Hospital Reports, London, 1863–1864;4:10–19, 389–446; Ophthalmic Hospital Reports, London, 1865–1866;5:51–78, 251–306.

Loss of speech: its association with valvular disease of the heart, and with hemiplegia on the right side. Defects of smell. Defects of speech in chorea. Arterial regions in epilepsy. Clinical lectures and reports by the medical and surgical staff of the London Hospital, 1864;1:388–471.

Selected Writings of John Hughlings Jackson, edited by James Taylor (2 volumes, 1931).

DECLARATIONS

Competing interests

None declared

Funding

None

Ethical approval

Not applicable

Guarantor

MR

Contributorship

Both authors contributed equally

Acknowledgements

This paper was originally published as Chapter 16 of Doctoring History by Manoj Ramachandran and Jeffrey K Aronson, published by the Royal Society of Medicine Press in 2010