Images in Neurology
Tumefactive Virchow-Robin spaces: A rare cause of obstructive hydrocephalus Naseer A Choh, Feroze Shaheen, Irfan Robbani, Manjeet Singh, Tariq Gojwari Department of Radiodiagnosis, Sheri Kashmir Institute of Medical Sciences, Srinagar, Jammu and Kashmir, India For correspondence: Prof. Naseer A. Choh, Department of Radiodiagnosis, Sheri Kashmir Institute of Medical Sciences,
Srinagar - 190 018, Jammu and Kashmir, India. E-mail: [email protected] Ann Indian Acad Neurol 2014;17:345-6
A middle-aged male presented with complaints of mild headache and unsteadiness of gait for past 2 years. His physical examination and lab results, including the cerebrospinal fluid (CSF) examination were unremarkable. Magnetic resonance imaging (MRI) of brain was done which revealed a cluster of cysts in the midbrain isointense to CSF on all sequences [Figures 1 and 2]. No enhancement of the cyst walls was seen on post-gadolinium images [Figure 3]. MR spectroscopy did not show any choline, lipid, or amino acid peak in the lesions. These were seen to cause mass effect on aqueduct of Sylvius and mild obstructive hydrocephalus. Review of MRI done before 2 years revealed the same lesions in the mesencephalothalamic region; no change in size or signal intensity was observed in the present scan. The final diagnosis was tumefactive Virchow-Robin spaces causing mild hydrocephalus. The patient was referred to neurosurgery department for further management. A VP shunt was placed with dramatic resolution of symptoms. The Virchow-Robin or perivascular spaces are pial-lined, fluidfilled spaces accompanying perforating arteries and venules. Typical perivascular spaces occur along lenticulostriate arteries above the anterior perforating substance and anterior commissure. They may occur less commonly in subcortical location, subinsular region, and cerebellum.[1] Rarely they may become enlarged (called tumefactive Virchow-Robin spaces) and cause mass effect and hydrocephalus (especially those found in mesencephalothalamic region); these need to be distinguished from cystic neoplasms.[2] Some authors have proposed the term Virchoma for enlarged mesencephalic perivascular spaces.[3] Tumefactive or cavernous dilation of
Virchow-Robin spaces occurring in mesencephalothalamic region may be associated with hydrocephalus. The diagnostic features for tumefactive or cavernous dilation of Virchow-Robin spaces in this case are, an absence of signal from the lesion on the fluid a enuated inversion recovery (FLAIR) imaging, no abnormal signal in the adjacent neuroparenchyma, no contrast enhancement on post-gadolinium scans, and no change in size or appearance over 2 years. The differential diagnosis of tumefactive perivascular spaces includes cystic neoplasms, parasitic cysts, ventricular diverticulae, nonneoplastic neuroepithelial cysts, and mucopolysaccharidosis.[3-5] Extreme unilateral dilation of Virchow-Robin spaces may also be encountered.[6] Large Virchow-Robin spaces (VRS) have been associated with age, dementia, multiple sclerosis, trauma, hypertension, and incidental white ma er lesions.[1,7,8] Surgical shunting of the hydrocephalus associated with VR spaces has produced variable clinical results (with improvement in gait disturbances, bradykinesia, and urinary symptoms). We conclude that mesencephalic tumefactive Virchow Robin spaces can cause obstructive hydrocephalus, and rare manifestations of a common entity should always be considered.
Access this article online Quick Response Code:
Website: www.annalsofian.org
DOI: 10.4103/0972-2327.138524
Figure 1: Axial T1-weighted images show cluster of cysts in the mesencephalic region isointense to cerebrospinal fluid (CSF) Annals of Indian Academy of Neurology, July-September 2014, Vol 17, Issue 3
346
Choh, et al.: Tumefactive Virchow Robin spaces
Figure 2: Coronal fluid attenuated inversion recovery (FLAIR) image shows CSF isointense cysts in mesencephalothalamic region causing mild hydrocephalus. Periventricular hyperintense signal is due to transependymal seepage and/or microvascular ischemia
Figure 3: Coronal post-gadolinium T1-weighted images do not reveal any enhancement of cysts, consistent with the diagnosis of tumefactive perivascular spaces 6.
Shiratori K, Mrowka M, Toussaint A, Spalke G, Bien S. Extreme, unilateral unilateral widening of Virchow-Robin spaces: Case report. Neuroradiology 2002;44:990-2. Ogawa T, Okudera T, Fukasawa H, Hashimoto M, Inugami A, Fujita H, et al. Unusual widening of Virchow-Robin spaces: MR appearance. AJNR Am J Neuroradiol 1995;16:1238-42. Heier LA, Bauer CJ, Schwartz L, Zimmerman RD, Morgello S, Deck MD. Large Virchow-Robin spaces: MR-clinical correlation. AJNR Am J Neuroradiol 1989;10:929-36.
References 1. 2.
3.
4.
5.
Kwee RM, Kwee TC. Virchow-Robin spaces at MR imaging. Radiographics 2007;27:1071-86. Salzman KL, Osborn AG, House P, Jinkins JR, Ditchfield A, Cooper JA, et al. Gaint tumefactive perivascular spaces. AJNR Am J Neuroradiol 2005;26:298-305. Papayannis CE, Saidon P, Rugilo CA, Hess D, Rodriguez G, Sica RE, et al. Expanding Virchow- Robin spaces in the midbrain causing hydrocephalus. AJNR Am J Neuroradiol 2003;24:1399-403. Flors L, Leiva-Salinas C, Cabrera G, Mazón M, Poyatos C. Obstructive hydrocephalus due to cavernous dilation of VirchowRobin spaces. Neurology 2010;74:1746. Kanamalla US, Calabro F, Jinkius JR. Cavernous dilation of mesencephalic Virchow-Robin spaces with obstructive hydrocephalus. Neuroradiology 2000;42:881-4.
7.
8.
How to cite this article: Choh NA, Shaheen F, Robbani I, Singh M, Gojwari T. Tumefactive Virchow-Robin spaces: A rare cause of obstructive hydrocephalus. Ann Indian Acad Neurol 2014;17:345-6. Received: 07-12-13, Revised: 14-01-14, Accepted: 03-10-14 Source of Support: None, Conflict of Interest: None declared.
Staying in touch with the journal 1)
Table of Contents (TOC) email alert Receive an email alert containing the TOC when a new complete issue of the journal is made available online. To register for TOC alerts go to www.annalsofian.org/signup.asp.
2)
RSS feeds Really Simple Syndication (RSS) helps you to get alerts on new publication right on your desktop without going to the journal’s website. You need a software (e.g. RSSReader, Feed Demon, FeedReader, My Yahoo!, NewsGator and NewzCrawler) to get advantage of this tool. RSS feeds can also be read through FireFox or Microsoft Outlook 2007. Once any of these small (and mostly free) software is installed, add www. annalsofian.org/rssfeed.asp as one of the feeds.
Annals of Indian Academy of Neurology, July-September 2014, Vol 17, Issue 3
Copyright of Annals of Indian Academy of Neurology is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Tumefactive Virchow-Robin spaces: A rare cause of obstructive hydrocephalus Naseer A Choh, Feroze Shaheen, Irfan Robbani, Manjeet Singh, Tariq Gojwari Department of Radiodiagnosis, Sheri Kashmir Institute of Medical Sciences, Srinagar, Jammu and Kashmir, India For correspondence: Prof. Naseer A. Choh, Department of Radiodiagnosis, Sheri Kashmir Institute of Medical Sciences,
Srinagar - 190 018, Jammu and Kashmir, India. E-mail: [email protected] Ann Indian Acad Neurol 2014;17:345-6
A middle-aged male presented with complaints of mild headache and unsteadiness of gait for past 2 years. His physical examination and lab results, including the cerebrospinal fluid (CSF) examination were unremarkable. Magnetic resonance imaging (MRI) of brain was done which revealed a cluster of cysts in the midbrain isointense to CSF on all sequences [Figures 1 and 2]. No enhancement of the cyst walls was seen on post-gadolinium images [Figure 3]. MR spectroscopy did not show any choline, lipid, or amino acid peak in the lesions. These were seen to cause mass effect on aqueduct of Sylvius and mild obstructive hydrocephalus. Review of MRI done before 2 years revealed the same lesions in the mesencephalothalamic region; no change in size or signal intensity was observed in the present scan. The final diagnosis was tumefactive Virchow-Robin spaces causing mild hydrocephalus. The patient was referred to neurosurgery department for further management. A VP shunt was placed with dramatic resolution of symptoms. The Virchow-Robin or perivascular spaces are pial-lined, fluidfilled spaces accompanying perforating arteries and venules. Typical perivascular spaces occur along lenticulostriate arteries above the anterior perforating substance and anterior commissure. They may occur less commonly in subcortical location, subinsular region, and cerebellum.[1] Rarely they may become enlarged (called tumefactive Virchow-Robin spaces) and cause mass effect and hydrocephalus (especially those found in mesencephalothalamic region); these need to be distinguished from cystic neoplasms.[2] Some authors have proposed the term Virchoma for enlarged mesencephalic perivascular spaces.[3] Tumefactive or cavernous dilation of
Virchow-Robin spaces occurring in mesencephalothalamic region may be associated with hydrocephalus. The diagnostic features for tumefactive or cavernous dilation of Virchow-Robin spaces in this case are, an absence of signal from the lesion on the fluid a enuated inversion recovery (FLAIR) imaging, no abnormal signal in the adjacent neuroparenchyma, no contrast enhancement on post-gadolinium scans, and no change in size or appearance over 2 years. The differential diagnosis of tumefactive perivascular spaces includes cystic neoplasms, parasitic cysts, ventricular diverticulae, nonneoplastic neuroepithelial cysts, and mucopolysaccharidosis.[3-5] Extreme unilateral dilation of Virchow-Robin spaces may also be encountered.[6] Large Virchow-Robin spaces (VRS) have been associated with age, dementia, multiple sclerosis, trauma, hypertension, and incidental white ma er lesions.[1,7,8] Surgical shunting of the hydrocephalus associated with VR spaces has produced variable clinical results (with improvement in gait disturbances, bradykinesia, and urinary symptoms). We conclude that mesencephalic tumefactive Virchow Robin spaces can cause obstructive hydrocephalus, and rare manifestations of a common entity should always be considered.
Access this article online Quick Response Code:
Website: www.annalsofian.org
DOI: 10.4103/0972-2327.138524
Figure 1: Axial T1-weighted images show cluster of cysts in the mesencephalic region isointense to cerebrospinal fluid (CSF) Annals of Indian Academy of Neurology, July-September 2014, Vol 17, Issue 3
346
Choh, et al.: Tumefactive Virchow Robin spaces
Figure 2: Coronal fluid attenuated inversion recovery (FLAIR) image shows CSF isointense cysts in mesencephalothalamic region causing mild hydrocephalus. Periventricular hyperintense signal is due to transependymal seepage and/or microvascular ischemia
Figure 3: Coronal post-gadolinium T1-weighted images do not reveal any enhancement of cysts, consistent with the diagnosis of tumefactive perivascular spaces 6.
Shiratori K, Mrowka M, Toussaint A, Spalke G, Bien S. Extreme, unilateral unilateral widening of Virchow-Robin spaces: Case report. Neuroradiology 2002;44:990-2. Ogawa T, Okudera T, Fukasawa H, Hashimoto M, Inugami A, Fujita H, et al. Unusual widening of Virchow-Robin spaces: MR appearance. AJNR Am J Neuroradiol 1995;16:1238-42. Heier LA, Bauer CJ, Schwartz L, Zimmerman RD, Morgello S, Deck MD. Large Virchow-Robin spaces: MR-clinical correlation. AJNR Am J Neuroradiol 1989;10:929-36.
References 1. 2.
3.
4.
5.
Kwee RM, Kwee TC. Virchow-Robin spaces at MR imaging. Radiographics 2007;27:1071-86. Salzman KL, Osborn AG, House P, Jinkins JR, Ditchfield A, Cooper JA, et al. Gaint tumefactive perivascular spaces. AJNR Am J Neuroradiol 2005;26:298-305. Papayannis CE, Saidon P, Rugilo CA, Hess D, Rodriguez G, Sica RE, et al. Expanding Virchow- Robin spaces in the midbrain causing hydrocephalus. AJNR Am J Neuroradiol 2003;24:1399-403. Flors L, Leiva-Salinas C, Cabrera G, Mazón M, Poyatos C. Obstructive hydrocephalus due to cavernous dilation of VirchowRobin spaces. Neurology 2010;74:1746. Kanamalla US, Calabro F, Jinkius JR. Cavernous dilation of mesencephalic Virchow-Robin spaces with obstructive hydrocephalus. Neuroradiology 2000;42:881-4.
7.
8.
How to cite this article: Choh NA, Shaheen F, Robbani I, Singh M, Gojwari T. Tumefactive Virchow-Robin spaces: A rare cause of obstructive hydrocephalus. Ann Indian Acad Neurol 2014;17:345-6. Received: 07-12-13, Revised: 14-01-14, Accepted: 03-10-14 Source of Support: None, Conflict of Interest: None declared.
Staying in touch with the journal 1)
Table of Contents (TOC) email alert Receive an email alert containing the TOC when a new complete issue of the journal is made available online. To register for TOC alerts go to www.annalsofian.org/signup.asp.
2)
RSS feeds Really Simple Syndication (RSS) helps you to get alerts on new publication right on your desktop without going to the journal’s website. You need a software (e.g. RSSReader, Feed Demon, FeedReader, My Yahoo!, NewsGator and NewzCrawler) to get advantage of this tool. RSS feeds can also be read through FireFox or Microsoft Outlook 2007. Once any of these small (and mostly free) software is installed, add www. annalsofian.org/rssfeed.asp as one of the feeds.
Annals of Indian Academy of Neurology, July-September 2014, Vol 17, Issue 3
Copyright of Annals of Indian Academy of Neurology is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
