Johann Conrad Brunner (1653-1727) and the first description of syringomyelia.

INTRODUCTION: Johann Conrad Brunner (1653-1727) was a European anatomist and physician whose research concerning the pancreas and duodenum has made him a prominent figure in medicine. However, Brunner should also be recognized for his descriptions of syringomyelia, which were originally published in 1688 and included in the second edition of Theophilus Boneti's compendium of postmortem examinations, the Sepulchretum, which was published in 1700. CONCLUSIONS: Our current understanding of syringomyelia is based on the early observations of researchers such as Johann Conrad Brunner.

Augustin Morvan (1819-1897), a little-known rural physician and neurologist.

Augustin Morvan (1819-1897) was a contemporary of Jean-Martin Charcot who practised medicine in rural Brittany. A perspicacious and astute clinician, he described three clinical pictures not previously isolated: in 1875 the semiology of myxoedema, in 1883 the neurological semiology of syringomyelia which he called "paretic analgesia of the upper extremities", and finally in 1890 the semiology of "fibrillary chorea", currently considered a model of synaptic pathology involving immunological damage to potassium channels and causing (as perfectly described by Morvan) myokymia, autonomic nervous system disturbances and agrypnia. "Fibrillary chorea" is today known as Morvan's syndrome and linked to limbic encephalitis.

History of the Emergence and Recognition of Syringomyelia in the 19th Century.

Charles-Prosper Ollivier d'Angers coined the term "syringomyelia" in 1827 to describe the presence of a cavity in the spinal cord, which he considered pathological in all cases. In 1882 in Germany, Otto von Kahler and Friedrich Schultze defined the clinical syndrome, which associated Duchenne-Aran muscular dystrophy of a limb with sensory dissociation. They explained the syndrome by the presence of an abnormal cavity, distinct from that found in the spinal cord of healthy adults. Although Guillaume Duchenne de Boulogne and Jean-Martin Charcot had observed cases in France, both failed to identify syringomyelia, whereas a family physician in Brittany, Augustin Morvan, described the clinical symptomatology using the term "analgesic whitlow" in 1883. Based on several dozen observations that they collated in their remarkable theses,Anna Bumler in Zurich in 1887 and Isidore Bruhl in Paris in 1890 established the complete clinical picture of syringomyelia, covering anatomic functional and pathological aspects. Whereas Charcot isolated pathologies by correlating clinical signs with anatomo-pathological lesions, the isolation of syringomyelia initially involved an anatomo-pathological concept before the semiology was defined. In addition, this work would later enhance physiological understanding of sensory spinal pathways.

Charles Prosper Ollivier d'Angers (1796-1845) and his contributions to defining syringomyelia.

INTRODUCTION: Distinguished as an anatomist, pathologist, and clinician, the Frenchman Charles Prosper Ollivier d'Angers dedicated his life to accelerating the forefront of neuroscience. At a young age, he explored the diseases and disorders of the spinal cord during a time when clinical neurological investigation scarcely existed. Ollivier d'Angers coined the term "syringomyelia." CONCLUSION: The coinage of the term syringomyelia by d'Angers shed light on the disorder causing more practitioners to investigate the spinal cord and its defects.

W. James Gardner: pioneer neurosurgeon and inventor.

W. James Gardner, a skillful neurosurgeon and inventor, is best remembered for his cervical tongs and hydrodynamic theory of syringomyelia. A pioneer of modern neurosurgery, Gardner trained under Charles Frazier in Philadelphia, and in 1929 he moved to Ohio where he became chief of neurosurgery at the Cleveland Clinic, a position he was to hold for the next 33 years. A large surgical practice made it imperative for Gardner to develop surgical methods that were quick, effective, and advantageous for patient and surgeon. He was an early proponent of the sitting position for patients undergoing cranial surgery, which led to the development of a neurosurgical chair with a head fixation device. To reduce the risks of hypotension and air embolism when the patient is in the sitting position, Gardner invented the clinical G suit. He was the first to advocate and use induced arterial hypotension for intracranial surgery and the first neurosurgeon in the US to publish his experiences performing lumbar discography. He converted an operating table so that he could induce hypothermia during aneurysm surgery and then applied pneumatic cuffs to occlude the major arterial supply to the brain. His pioneering work has been documented in many other areas such as hemifacial spasm and trigeminal neuralgia, for which he performed the first vascular decompression, in cervical sympathectomy for treatment of various ailments, and in the use of intrathecally delivered steroid drugs for sciatica. During his career, he authored 256 publications and one book on the dysraphic states. Many of his contributions to the discipline of neurosurgery are now taken for granted.

The pathophysiology of syringomyelia - historical overview and current concept.

Various ideas and hypotheses have been brought forward to explain the development of syringomyelia in the past two centuries. None of them offers a sufficient basis to serve as a concept for the treatment of all affected patients. Apart from a discussion of the different hypotheses this paper proposes a new pathophysiological concept based on clinical, experimental and literature studies. Syringomyelia is understood as a state of chronic interstitial edema of the spinal cord due to accumulation of extracellular fluid (ECF). This accumulation is caused by a cascade of events starting with obstruction of cerebrospinal fluid (CSF) flow and/or spinal cord tethering which ultimately alter ECF flow and increase ECF volume. Treatment should be targeted against the pathological process which causes CSF flow obstruction and cord tethering to inhibit this pathophysiological process at a decisive point.

History of post-traumatic syringomyelia: post traumatic syringomyelia prior to 1920.

STUDY DESIGN: Review of five cases of post-traumatic syringomyelia originally described between 1898-1920. OBJECTIVES: To describe the earliest presentation of post-traumatic syringomyelia and to compare to modern day views of this condition. SETTING: Historical review. RESULTS: Pathogenesis and interpretation of the findings are discussed in the light of current knowledge. CONCLUSION: Credit for the first description of post-traumatic syringomyelia (in 1898) should go to W Wagner and P Stolper.

[History, controversy and pathogenesis]. / Histoire, controverses et pathogénie.

In this paper, we discuss the historical and pathophysiological aspects of syringomyelia. Defined as fluid cavities extending beyond several segments within the spinal cord this pathological entity is a condition with many possible causes. Hindbrain herniation is the commonest foramen magnum abnormality associated with the so called "hindbrain related syringomyelia". A history of birth injury, a small posterior fossa, an arachnoid scarring of the basal cisterna, a segmentation abnormality of the superior cervical vertebrae, a hydrocephalus or arachnoid cyst of the posterior fossa are often present in this context. Previous theories of the origin and the mechanism of syringomyelia progression have been controversly proposed. Gardner and colleagues postulated that the primary event is the incomplete embryonic opening of the outlets of the fourth ventricle. The fluid arrived in the syrinx along the embryologically natural route down the central canal. Their hydrodynamic theory states that with each arterial pulse, the outflow of CSF is transmitted from the fourth ventricle down to the syrinx via the central canal. Most patients have patent fourth ventricle foramina and evidence of communication between the ventricle and the syrinx is rare. Williams then proposed his "cranio-spinal pressure dissociation theory". Significant pressure differential occur daily during activities that increase intrathoracic pressure such as sneezing, coughing and could be transmitted to the spinal fluid from the epidural spinal veins. The progression of the cavity is better understood and analyzed with dynamic MR imaging and quantitative analysis. The CSF flow from the cranial to the spinal subarachnoid space results from the expansile motion of the brain during the cardiac cycle. The progression of the cavity is based on pressure acting on the surface of the cord and does not require any communication of the fourth ventricle with the central canal and the syrinx. The origin of fluid cavity remain questionable. Aboulker but also Ball and Dayan pointed out the role of the perivascular spaces and the DREZ which are involved in the communication between the perimedullar CSF, the spinal cord extracellular spaces and the central canal. Other causes of syringomylia include acquired conditions which could be grouped under the heading of "non-hindbrain related syringomyelia". Arachnoid scarring is related in many case to spinal trauma or occurs after spinal meningitis, spinal intradural surgery, peridural anesthesia, subarachnoid hemorrhage. Rarely an extra medullary compression is discussed. The mechanism involved is here again an alteration of the CSF flow at the spinal level.

A short history of neuropathic arthropathy.

Neuropathic joint disease or Charcot joints are a chronic form of a degenerative arthropathy that is associated with decreased sensory innervation of the involved joints. Neuropathic arthropathies are considered to be an accelerated osteoarthritis that is precipitated by trauma to a joint not protected by its proprioceptive or nociceptive reflexes. This process continues until destruction of the joint occurs. The etiology of Charcot joints has varied throughout history as different diseases have become less or more prominent. Some of the offenders include diabetic neuropathy, tabes dorsalis, syringomyelia, and congenital indifference to pain. The morphology and pathogenesis of this condition has intrigued the minds of many, including Musgrave, Charcot, Virchow, Eloesser, Soto-Hall, and Key.

Impressäo basilar, Arnold-Chiari e siringomielia: análise de 20 casos operados / Basilar impression, Arnold-Chiari malformation and syringomyelia: an analysis of 20 operate cases

Säo analisados os resultados obtidos com o tratamento de 20 pacientes submetidos ao tratamento cirúrgico da siringomielia em associaçäo com impressäo e/ou malformaçäo de Arnld-Chiari. Säo enfatizados os estudos relacionados com a força muscular, atrofias e dissociaçäo siringomiélica