Acute Idiopathic Syringomyelia: A Case Report

Abstract

There have been isolated reports in the literature of the natural history and less than complete knowledge of the epidemiology and pathophysiology of syringomyelia. This article describes a clinically acute-onset abnormal patient with localized dilatation of the central canal of the spinal cord who had no evidence of present or prior Chiari's malformation, trauma, infection, tumor, or other predisposing pathologic condition. It is suggested that this distinct condition be labeled "acute idiopathic syringomyelia".

Clinical course of incidental syringomyelia without predisposing pathologies

“…For patients with incidental syringomyelia with no predisposing pathology, conservative treatment with close observation is therefore warranted rather than surgery early in the clinical course. Chen et al 2 1 Chiari malformation, trauma, infection, neoplasm Nakamura et al 3 15 Chiari malformation, spinal cord tumor, trauma, adhesive arachnoiditis, other arachnoid pathology Iskandar et al 8 5 Hindbrain herniation Kastrup et al 10 1 Trauma, spinal tumor, craniocervical or intracerebral anomaly Ataizi et al 12 1 Trauma, spinal tumor, craniocervical or intracerebral pathology Struck et al 13 8 b Chiari malformation, tumor, trauma, other cause of syringomyelia Bogdanov et al 14 17 c Chiari 1 malformation, tumor, arachnoiditis, previous meningitis, subarachnoid hemorrhage, trauma a The number of patients who had idiopathic syringomyelia. b Of 14 patients, eight had idiopathic syringomyelia.…”

“…Although all syringomyelia in the current study were ''idiopathic'' as defined here, we have added ''which have no predisposing pathology'' because idiopathic syringomyelia has not been a generally accepted term, and this definition varies between sources (Table 2). 2,3,8,10,12,13,14 Some reports of ''incidental'' syringomyelia have been associated with Chiari malformation or tumor. 5,15 Although these reports share some characteristics with our series, they are markedly different in their predisposing pathology, which particularly affects treatment and prognosis.…”

“…2 The term ''hydromyelia'' refers to a dilatation of the central canal, and ''syringomyelia'' refers to a fluid-filled cavity outside of the spinal canal, typically with gliotic walls. 12,16 Although Roser et al 17 distinguished between syringomyelia and hydromyelia using electrophysiological methods, in this study, we used the term ''syringomyelia'' to describe broadly both types of fluid-filled cavities within the spinal cord because it is difficult to distinguish between these two abnormalities in practice, and many spinal cord cavities have features of both conditions.…”

“…[1][2][3][4] There has been an apparent increase in the incidence of syringomyelia because previously undiagnosed, small subclinical lesions are now being detected with MRI. 2 The management of these incidental lesions requires a consideration of the natural course of the lesion. 5 In general, the treatment for syringomyelia associated with Chiari malformation consists of either drainage of the syringomyelia or the correction of the bony abnormality.…”

“…The exact pathogenesis and development of syringomyelia are uncertain, and the natural course of syringomyelia is variable. 2,6,10,11 Moreover, as a result of the small number of studies on incidental syringomyelia, little is known about syringomyelia with no predisposing pathology, such as Chiari malformation or spinal cord tumor.…”