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Case report Hemiatrophy as a presentation of a glioependymal cyst Teresa Ferreira a , Dheeraj Khurana a, *, Sandeep Mohindra b , Kirti Gupta c a Department of Neurology, Postgraduate Institute of Medical Education and Research, Sector 12, Chandigarh 160012, India b Department of Neurosurgery, Postgraduate Institute of Medical Education and Research, Sector 12, Chandigarh 160012, India c Department of Histopathology, Postgraduate Institute of Medical Education and Research, Sector 12, Chandigarh 160012, India A R T I C L E I N F O Article history: Received 21 February 2014 Received in revised form 27 May 2014 Accepted 8 June 2014 Available online 19 June 2014 Keywords: Hemiatrophy Glioependymal cyst Cerebral amyotrophy Intracranial cyst 1. Introduction Glioependymal (neuroglial) cysts are benign developmental epithelial lined lesions that occur anywhere along the neuraxis. Intraparenchymal Glioependymal cysts present with a varying symptomatology. 2. Case report A 20 year old male presented with progressive left sided weakness since 2 years. The weakness started from the left lower limb progressing on to involve the left upper limb since 2 months prior to presentation. He also complained of occasional holocranial headaches for the last 2 years which had increased in frequency and severity over the past 2 months. There was history of transient visual obscurations lasting a few seconds. Examination revealed bilateral papilloedema, left facial hemiatrophy, UMN facial paresis and left spastic hemiparesis with wasting of the left arm and thigh (Fig. 1). The wasting was most apparent in the left lower thigh (5 cm lesser circumference than the corresponding area of the right thigh) and left calf. He had a hemiparetic gait. Cortical sensations were normal. Cranial MRI revealed a 8.4 cm 6.8 cm 6.5 cm sized nonenhancing lesion in the right frontoparietal area which was isointense to CSF on T1 W, T2 W and FLAIR images (Fig. 2A and B). The cystic lesion was seen to compress the ipsilateral ventricle and surrounding brain tissue and was associated with a midline shift. A right parietal craniotomy with marsupialization of the cyst was carried out. Histopathology of the lesion showed a collapsed glioependymal cyst lined by a single layer of ependymal cells without basement membrane (Fig. 2C). Following surgery the patient has shown steady recovery with subsidence of headaches and improvement in power on the left side. Follow up CT scan after one year shows sustained remission with decompression of the lateral ventricle (Fig. 2D). 3. Discussion Glioependymal cyst also referred to as neuroglial cyst, ependymal cyst, epithelial cyst or choroidal epithelial cyst account for less than 1% of all intracranial cysts [1]. They are congenital lesions, arising from rests of embryonic neural tube elements that become sequestered within the developing white matter. These may be intraparenchymal or extraparenchymal, of which the former type is more common. Intraparenchymal cysts occur most frequently in the frontal lobe although may occur anywhere along the neuraxis. These are rounded, smooth, and unilocular containing clear fluid that resembles CSF. They are lined by ependymal (columnar epithelium) or choroid plexus cells (low cuboidal epithelium). Most patients remain asymptomatic but some become symptomatic and symptoms depend on the site and size of the cyst. Common clinical presentation is with seizures [2], hemiparesis, hemianopia, hemianaesthesia, move- ment disorders, raised intracranial pressure (ICP) and altered sensorium [3] . * Corresponding author at: Department of Neurology, Level 1, Nehru Hospital, Postgraduate Institute of Medical Education and Research, Sector 12, Chandigarh 160012, India. Tel.: +91 172 2756690. E-mail address: [email protected] (D. Khurana). http://dx.doi.org/10.1016/j.clineuro.2014.06.005 0303-8467/ ã 2014 Published by Elsevier B.V. Clinical Neurology and Neurosurgery 124 (2014) 6–7 Contents lists available at ScienceDirect Clinical Neurology and Neurosurgery journa l homepage: www.e lsevier.com/loca te/clineuro
