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Fluid in the flute: Reversible hydromyelia
Journal of the Neurological Sciences 236 (2005) 85 – 86 www.elsevier.com/locate/jns
Short communication
Fluid in the flute: Reversible hydromyelia Tim Wehner a, Jeffrey S. Ross b, Richard M. Ransohoff c,* a
The Cleveland Clinic Foundation, Department of Neurology-S 90, 9500 Euclid Avenue, Cleveland, OH 44195, United States b The Cleveland Clinic Foundation, Department of Radiology, 9500 Euclid Avenue, Cleveland, OH 44195, United States c The Cleveland Clinic Foundation, Department of Neurology and Neurosciences-NC 30, 9500 Euclid Avenue, Cleveland, OH 44195, United States Received 3 March 2005; accepted 6 April 2005 Available online 21 June 2005
Abstract Non-communicating syrinx formation in association with demyelinating disease has been reported in approximately 50 patients. It has been unknown whether this is a permanent or potentially reversible phenomenon. We report a patient with acute transverse myelitis who developed a syrinx that resolved within 6 months. Mechanisms of syrinx formation in demyelinating disease are discussed. D 2005 Elsevier B.V. All rights reserved. Keywords: Syrinx; Hydromyelia; Demyelinating disease; Transverse myelitis
1. Introduction Syrinx cavities in the brainstem or spinal cord can be divided into communicating and non-communicating forms. Non-communicating syrinx has been described in association with tumor, trauma, infection and inflammation of the spinal cord [1]. It has been uncertain whether cavities that form during intense intrathecal inflammation result from expansion of the central canal or tissue necrosis. With the advent of magnetic resonance imaging (MRI), numerous patients with syrinx or hydromyelia and inflammatory central nervous system (CNS) disease have been described (reviewed in [2]). Here we report a patient who, in the setting of acute transverse myelitis, developed hydromyelia that resolved almost completely within 6 months.
2. Case report One week after a febrile upper respiratory illness, a 45year-old Caucasian male presented with band-like chest pressure and acute urinary retention. * Corresponding author. Fax: +1 216 444 7927. E-mail address: [email protected] (R.M. Ransohoff). 0022-510X/$ - see front matter D 2005 Elsevier B.V. All rights reserved. doi:10.1016/j.jns.2005.04.013
For years, the patient had experienced episodes of polyarthralgias with swelling of hand and finger joints, photosensitivity, as well as chest heaviness and dyspnea on exertion. A CT of the chest had demonstrated multifocal, predominantly peripheral opacities in both lungs, suggesting a chronic inflammatory process. Serologic testing for rheumatologic disease had been negative. His symptoms had been attributed to a ‘‘Lupus-like syndrome’’ and he had improved on prednisone and hydroxychloroquine. One day prior to presentation, the patient had noted sudden onset of band-like chest pressure around his nipples, radiating to the back. On the day of presentation, he had been unable to void his bladder. While being evaluated in the Emergency Department, he developed paraplegia and lost sensation below T6. MRI demonstrated inflammatory expansion of the spinal cord from T2 to T6 – T7 and a fluid-filled central cavity from T6 – T7 to T10 (Fig. 1A). No enhancement was demonstrated. MRI of the brain was normal. Cerebrospinal fluid (CSF) showed two white blood cells, glucose of 79 mg/dl and protein of 41 mg/dl. No oligoclonal bands or malignant cells were detected. The patient was diagnosed with acute transverse myelitis, and high-dose intravenous corticosteroids were started immediately. Motor improvement began within days and
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T. Wehner et al. / Journal of the Neurological Sciences 236 (2005) 85 – 86
b) As a consequence of edema, localized circulatory insufficiency may lead to ischemia which than results in tissue necrosis, ultimately leading to cavity formation. c) Demyelination by itself may lead to tissue necrosis and subsequent cavity formation. d) Arachnoiditis may lead to meningeal fibrosis, which may disturb CSF hydrodynamics in a way that permits cavity formation.
Fig. 1. T2-weighted sagittal MRI study of the spine on the day of onset of myelitis demonstrates an ill-defined T2 hyperintensity within the upper thoracic cord from T2 to T6/7 (arrowheads) and a well-defined cavity (arrows) extending from T6/7 to T10 (A). Six months later, the inflammatory T2 hyperintensity has resolved and only minimal residual hydromyelia (arrows) is detected (B). Axial images at T9 – 10 level are shown in insets.
continued gradually. Six months after onset, he had a full functional recovery. Spinal MRI at this point demonstrated only minimal residual hydromyelia (Fig. 1B).
Two postmortem examinations of patients with syrinx in inflammatory CNS disease have been described [3,4]. One patient with clinically and pathologically proven multiple sclerosis [3] whose midthoracic (T6 –T8) hydromyelia had been previously demonstrated with MRI was found to have a slit-like cavity extending over two lower cervical segments, closing in the upper thoracic cord and then reopening in the midthoracic cord microscopically. The luminal surface of this cavity was not lined with ependymal cells and it was not connected to the patent central canal, which was of normal size. These findings suggest syrinx formation due to tissue necrosis. The other patient who also had multiple sclerosis [4] was found to have enlargement of the central canal without any other cavity formation at autopsy, suggesting syrinx formation due to edematous expansion of the central spinal canal. The latter mechanism offers the most likely explanation for the findings in our patient. His complete functional recovery excludes significant tissue necrosis. Brain MRI excluded an abnormality of the craniocervial junction. In addition, there was no evidence radiographically or in the CSF for arachnoiditis. Although no pre-morbid imaging study is available, the reversibility of the hydromyelia strongly suggests that there was no significant cavity prior to the current illness. Thus, edema formation leading to altered CSF flow and enlargement of the central spinal canal with subsequent disappearance of the fluid during resolution of the illness offers the most plausible explanation for the imaging findings in our patient.
3. Discussion This report represents the first detailed account of reversible syrinx cavity formation, demonstrated with imaging studies, during an episode of acute myelitis. Fluctuating hydromyelia has been reported in Japanese patients with multiple sclerosis [2]. The relationship between inflammatory CNS disease and syringomyelia seems to be non-coincidental, and several mechanisms have been suggested to explain the formation of a syrinx in inflammatory CNS disease [2]: a) Edema associated with inflammatory cord lesions may disturb CSF hydrodynamics, with impaired circulation of CSF leading to enlargement of the central canal or an eccentric cavity.
References [1] Ransohoff RM, Whitman GJ, Weinstein MA. Noncommunicating syringomyelia in multiple sclerosis: detection by magnetic resonance imaging. Neurology 1990;40:718 – 21. [2] Larner AJ, Muqit MM, Glickman S. Concurrent syrinx and inflammatory central nervous system disease detected by magnetic resonance imaging: an illustrative case and review of the literature. Medicine 2002;81:41 – 50. [3] Matsuda M, Tabata K, Miki J, Ishigame H, Asano M, Ikeda S. Multiple sclerosis with secondary syringomyelia: an autopsy report. J Neurol Sci 2001;184:189 – 96. [4] Kleinschmidt-DeMasters BK, Newell J. Multiple sclerosis with hydromyelia demonstrated at autopsy. J Neurol Sci 1996;136:185 – 8.
Short communication
Fluid in the flute: Reversible hydromyelia Tim Wehner a, Jeffrey S. Ross b, Richard M. Ransohoff c,* a
The Cleveland Clinic Foundation, Department of Neurology-S 90, 9500 Euclid Avenue, Cleveland, OH 44195, United States b The Cleveland Clinic Foundation, Department of Radiology, 9500 Euclid Avenue, Cleveland, OH 44195, United States c The Cleveland Clinic Foundation, Department of Neurology and Neurosciences-NC 30, 9500 Euclid Avenue, Cleveland, OH 44195, United States Received 3 March 2005; accepted 6 April 2005 Available online 21 June 2005
Abstract Non-communicating syrinx formation in association with demyelinating disease has been reported in approximately 50 patients. It has been unknown whether this is a permanent or potentially reversible phenomenon. We report a patient with acute transverse myelitis who developed a syrinx that resolved within 6 months. Mechanisms of syrinx formation in demyelinating disease are discussed. D 2005 Elsevier B.V. All rights reserved. Keywords: Syrinx; Hydromyelia; Demyelinating disease; Transverse myelitis
1. Introduction Syrinx cavities in the brainstem or spinal cord can be divided into communicating and non-communicating forms. Non-communicating syrinx has been described in association with tumor, trauma, infection and inflammation of the spinal cord [1]. It has been uncertain whether cavities that form during intense intrathecal inflammation result from expansion of the central canal or tissue necrosis. With the advent of magnetic resonance imaging (MRI), numerous patients with syrinx or hydromyelia and inflammatory central nervous system (CNS) disease have been described (reviewed in [2]). Here we report a patient who, in the setting of acute transverse myelitis, developed hydromyelia that resolved almost completely within 6 months.
2. Case report One week after a febrile upper respiratory illness, a 45year-old Caucasian male presented with band-like chest pressure and acute urinary retention. * Corresponding author. Fax: +1 216 444 7927. E-mail address: [email protected] (R.M. Ransohoff). 0022-510X/$ - see front matter D 2005 Elsevier B.V. All rights reserved. doi:10.1016/j.jns.2005.04.013
For years, the patient had experienced episodes of polyarthralgias with swelling of hand and finger joints, photosensitivity, as well as chest heaviness and dyspnea on exertion. A CT of the chest had demonstrated multifocal, predominantly peripheral opacities in both lungs, suggesting a chronic inflammatory process. Serologic testing for rheumatologic disease had been negative. His symptoms had been attributed to a ‘‘Lupus-like syndrome’’ and he had improved on prednisone and hydroxychloroquine. One day prior to presentation, the patient had noted sudden onset of band-like chest pressure around his nipples, radiating to the back. On the day of presentation, he had been unable to void his bladder. While being evaluated in the Emergency Department, he developed paraplegia and lost sensation below T6. MRI demonstrated inflammatory expansion of the spinal cord from T2 to T6 – T7 and a fluid-filled central cavity from T6 – T7 to T10 (Fig. 1A). No enhancement was demonstrated. MRI of the brain was normal. Cerebrospinal fluid (CSF) showed two white blood cells, glucose of 79 mg/dl and protein of 41 mg/dl. No oligoclonal bands or malignant cells were detected. The patient was diagnosed with acute transverse myelitis, and high-dose intravenous corticosteroids were started immediately. Motor improvement began within days and
86
T. Wehner et al. / Journal of the Neurological Sciences 236 (2005) 85 – 86
b) As a consequence of edema, localized circulatory insufficiency may lead to ischemia which than results in tissue necrosis, ultimately leading to cavity formation. c) Demyelination by itself may lead to tissue necrosis and subsequent cavity formation. d) Arachnoiditis may lead to meningeal fibrosis, which may disturb CSF hydrodynamics in a way that permits cavity formation.
Fig. 1. T2-weighted sagittal MRI study of the spine on the day of onset of myelitis demonstrates an ill-defined T2 hyperintensity within the upper thoracic cord from T2 to T6/7 (arrowheads) and a well-defined cavity (arrows) extending from T6/7 to T10 (A). Six months later, the inflammatory T2 hyperintensity has resolved and only minimal residual hydromyelia (arrows) is detected (B). Axial images at T9 – 10 level are shown in insets.
continued gradually. Six months after onset, he had a full functional recovery. Spinal MRI at this point demonstrated only minimal residual hydromyelia (Fig. 1B).
Two postmortem examinations of patients with syrinx in inflammatory CNS disease have been described [3,4]. One patient with clinically and pathologically proven multiple sclerosis [3] whose midthoracic (T6 –T8) hydromyelia had been previously demonstrated with MRI was found to have a slit-like cavity extending over two lower cervical segments, closing in the upper thoracic cord and then reopening in the midthoracic cord microscopically. The luminal surface of this cavity was not lined with ependymal cells and it was not connected to the patent central canal, which was of normal size. These findings suggest syrinx formation due to tissue necrosis. The other patient who also had multiple sclerosis [4] was found to have enlargement of the central canal without any other cavity formation at autopsy, suggesting syrinx formation due to edematous expansion of the central spinal canal. The latter mechanism offers the most likely explanation for the findings in our patient. His complete functional recovery excludes significant tissue necrosis. Brain MRI excluded an abnormality of the craniocervial junction. In addition, there was no evidence radiographically or in the CSF for arachnoiditis. Although no pre-morbid imaging study is available, the reversibility of the hydromyelia strongly suggests that there was no significant cavity prior to the current illness. Thus, edema formation leading to altered CSF flow and enlargement of the central spinal canal with subsequent disappearance of the fluid during resolution of the illness offers the most plausible explanation for the imaging findings in our patient.
3. Discussion This report represents the first detailed account of reversible syrinx cavity formation, demonstrated with imaging studies, during an episode of acute myelitis. Fluctuating hydromyelia has been reported in Japanese patients with multiple sclerosis [2]. The relationship between inflammatory CNS disease and syringomyelia seems to be non-coincidental, and several mechanisms have been suggested to explain the formation of a syrinx in inflammatory CNS disease [2]: a) Edema associated with inflammatory cord lesions may disturb CSF hydrodynamics, with impaired circulation of CSF leading to enlargement of the central canal or an eccentric cavity.
References [1] Ransohoff RM, Whitman GJ, Weinstein MA. Noncommunicating syringomyelia in multiple sclerosis: detection by magnetic resonance imaging. Neurology 1990;40:718 – 21. [2] Larner AJ, Muqit MM, Glickman S. Concurrent syrinx and inflammatory central nervous system disease detected by magnetic resonance imaging: an illustrative case and review of the literature. Medicine 2002;81:41 – 50. [3] Matsuda M, Tabata K, Miki J, Ishigame H, Asano M, Ikeda S. Multiple sclerosis with secondary syringomyelia: an autopsy report. J Neurol Sci 2001;184:189 – 96. [4] Kleinschmidt-DeMasters BK, Newell J. Multiple sclerosis with hydromyelia demonstrated at autopsy. J Neurol Sci 1996;136:185 – 8.