CASE REPORT Multi-cystic white matter enlarged Virchow Robin spaces in a 5-year-old boy Erhan Bayram & Gulcin Akinci & Yasemin Topcu & Handan Cakmakci & Ozlem Giray & Derya Ercal & Semra Hiz Received: 27 September 2011 /Accepted: 13 December 2011 /Published online: 29 December 2011 # Springer-Verlag 2011 Introduction Virchow–Robin spaces (VRS) are perivascular, pial-lined areas in the brain that surrounds small arteries and can be seen on brain magnetic resonance imaging (MRI) [6, 9]. On MRI sequences, VRS are fluid-filled spaces, and their signal intensities are identical to those of cerebrospinal fluid [4, 22]. VRS are typically less than 5 mm in diameter and accepted as normal anatomical structures. The VRS around the perforating arteries are in continuity with the perivascu- lar spaces around arterioles in the subpial region, and when they perforate the brain surface and extend into the brain tissue, they are usually called enlarged VRS [3, 7, 21, 24]. Dilatation of VRS may be present in physiological condi- tions and many different diseases such as mucopolysacchar- idosis, mannosidosis, Lowe syndrome, Coffin–Lowry syndrome, and ectodermal dysplasia [9–11, 15, 17]. In childhood, dilatation of VRS is rarely secondary to specific progressive disorders but usually appears in developmental delay [4]. VRS are usually asymptomatic, but if there is an expan- sion to the brain parenchyma, various clinical symptoms can be seen associated with the mass effect [21]. Extreme dila- tation of VRS has been reported with different clinical findings, including headache, tics, dementia, seizures, ex- trapyramidal symptoms, ataxia, developmental delay, mac- rocephaly, or no neurologic abnormalities [1, 2, 5]. The visibility of VRS is dependent on the MRI sequence that is used [9]. T2-weighted MRI sequences lead to better visualization of VRS [20]. On MRI T2-weighted imagings, VRS are isointense relative to CSF and show no enhance- ment after contrast agent administration [21]. T1-weighted images show hyposignal intensity [4]. By the radiological appearances, that can be differentiated from cystic neopla- sia, parasitic infections, cystic infarction, and some meta- bolic disorders [6, 10, 13]. VRS can occur in many different locations, and the most common location is around the lenticulostriate arteries. Oc- casionally, that occurs around the arteries which have per- forated the cerebral cortex and extend into the white matter [10, 13, 21]. In this report, we described the 2-years follow-up of a 5-year-old boy with enlarged VRS, developmental delay, macrocephaly, hair with a double crown, and clinodactyly. The patient was not consistent with known syndrome descriptions. The symptoms of this patient may belong to a new syndrome that was previously unidentified. Case report A 5-year-old boy presented with a complaint of develop- mental delay. He was born after an uneventful pregnancy with a birth weight of 3,000 g, and he was the second child of nonconsanguineous parents. In the patient history, he was able to sit unaided at 18 months, walk at 24 months, and E. Bayram (*) : G. Akinci : Y. Topcu : S. Hiz Division of Pediatric Neurology, Department of Pediatrics, Dokuz Eylul University, Izmir, Turkey e-mail: dr.erhanbayram@yahoo.com H. Cakmakci Department of Radiology, Dokuz Eylul University, Izmir, Turkey O. Giray : D. Ercal Department of Genetics, Dokuz Eylul University, Izmir, Turkey Childs Nerv Syst (2012) 28:743–746 DOI 10.1007/s00381-011-1663-y