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Ash Singhal, Tim Bowen-Roberts, Paul Steinbok, Doug Cochrane, Angela T. Byrne and John M. Kerr

T he natural history of syringomyelia in nonoperatively treated patients remains unclear. With its pathogenesis not well defined, 13 ambiguity exists with regard to managing this condition, especially when syringomyelia is identified incidentally on MR imaging. Most neurosurgeons would choose to operate when a syrinx is progressing in size or deemed symptomatic. 7 There is no accepted strategy, however, for the management of syringes found on MR images when symptoms are subtle or not thought to be related to the syrinx. Although some other studies have

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Barry J. Leaney, Peter W. Rowe and Geoffrey L. Klug

T he term “phakomatosis” has been applied to a varied and uncommon group of congenital disorders affecting primarily the skin and central nervous system (CNS). Of these disorders, neurocutaneous melanosis, in which there is an excessive proliferation of melanin-producing cells in both the skin and leptomeninges, is the least common. We present the clinical progress, radiological findings, and therapeutic intervention in a unique case of neurocutaneous melanosis with progressive syringomyelia. Case Report This 6-year-old boy initially presented at the age

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Matthew M. Peterson, Liviu Craciun and John D. Heiss

S yringomyelia is a progressive disease, which if left untreated often leads to debilitating central myelopathy with permanent sensory and motor deficits. Syringomyelia was identified in the late 19th century 1 and its resistance to permanent treatment spurred neurosurgeons in the early and mid-20th century to develop innovative therapeutic approaches. 3 , 5 , 11 , 18 , 19 , 23 , 24 , 26 , 27 , 33 , 34 One technique that was devised to treat syringomyelia but is no longer practiced was reported by Dr. Dean H. Echols ( Fig. 1 ). His technique to treat

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Spiros Sgouros and Bernard Williams

S yringomyelia is the condition involving longitudinal cavities within the spinal cord that extend over several segments. 13 The most common cause of syringomyelia is a structural lesion at the foramen magnum, usually a hindbrain hernia, a term used to include both Chiari Type I and Type II deformities. 42, 43 Other lesions at the foramen magnum include tumor and meningeal fibrosis or arachnoiditis. The second most common cause of syringomyelia is localized meningeal fibrosis due to spinal cord trauma. Drainage into the subarachnoid space by myelotomy or

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John D. Heiss, Giancarlo Suffredini, René Smith, Hetty L. DeVroom, Nicholas J. Patronas, John A. Butman, Francine Thomas and Edward H. Oldfield

S yringomyelia is more often associated with CM-I than any other etiology. 17 , 18 , 37 The objective of surgical treatment of syringomyelia associated with the CM-I is to decompress the cerebellar tonsils, eliminate the syrinx, and prevent the progression of myelopathy. A reduced syrinx size, which is associated with disease stabilization, occurs after craniocervical decompression or syrinx fluid shunting. Unlike craniocervical decompression, syrinx shunting does not address the underlying cause of the syringomyelia, requires invasion of the spinal cord

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Sarah J. Hemley, B. Biotech, Jian Tu and Marcus A. Stoodley

S pinal cord injury remains a significant clinical problem, with an estimated 13,000 new cases of SCI occurring in the US each year. 21 , 32 Up to one-third of such patients develop posttraumatic syringomyelia, causing pain and neurological deficits in addition to those incurred with the original injury. Current treatments for syringomyelia are often not effective, with only 50% of patients showing improvement. It is unlikely that more effective therapies will be developed without a greater understanding of posttraumatic syrinx pathophysiology. Existing

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Arnold H. Menezes

H indbrain herniation syndrome, often referred to as CM-I, is a disorder that had traditionally been defined as downward herniation of the cerebellar tonsils through the foramen magnum of more than 4–5 mm on sagittal MR imaging. 3 , 10 , 23 , 25 This anomaly is associated with syringomyelia in 45%–68% of patients. 22 It occurs in conjunction with osseous abnormalities of the CVJ. Changes in CSF dynamics contribute to the symptoms and the clinical syndrome characterized by occipital Valsalva-type headaches, lower cranial nerve abnormalities, and spinal

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Nicholas D. Coppa, H. Jeffrey Kim and Kevin M. McGrail

S yringomyelia occurs in approximately 40 to 65% of patients in whom a CM-I has been diagnosed. 13 Hydrocephalus is associated with approximately 7% of patients with a CM-I. 12 There have been a number of reports of the spontaneous resolution of syringomyelia in the setting of an existing CM-I. 1 , 2 , 5–11 , 15–20 , 22 We present the first case of a patient with a cervicothoracic syrinx and CM-I that spontaneously resolved after CSF otorrhea developed in the patient. This case is presented with the aim of relating this patient’s pathophysiology to our

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Philippe Paquis, Michel Lonjon, Madeleine Brunet, Jean Claude Lambert and Patrick Grellier

S yringomyelia is considered to be a noninherited developmental disorder of the spinal cord, most frequently found in association with a Chiari Type I malformation. 2 However, rare familial cases have been reported in which autosomal dominant or recessive inheritance was present. 4, 5, 23 Chatel, et al., 6 suggested that the incidence of inherited syringomyelia is approximately 2%. An association between syringomyelia and inherited diseases such as Noonan's syndrome or phacomatoses has also been mentioned. 3, 11, 18 Blepharophimosis

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Luigi Peserico, Paolo Angiari, Gian Antonio Merli, Vittorio Sonnino, Pietro Faglioni and Gian Carlo Canossi

S everal diagnostic procedures have been adopted to provide an accurate diagnosis of communicating syringomyelia in order to consider the Gardner operation at the craniovertebral junction. These procedures include oil myelography in the supine position, 1 gas myelography, 2 isotope myelocisternography, 3, 4 and Pantopaque ventriculography. Combined isotope ventriculography and lumbar myelocisternography, however, has never been proposed nor has it been carried out to the best of our knowledge. The purpose of this report is to briefly present and discuss the