Clinical Neurology and Neurosurgery 124 (2014) 6–7

Case report

Hemiatrophy as a presentation of a glioependymal cyst

Teresa Ferreira a, Dheeraj Khurana a,*, Sandeep Mohindra b, Kirti Gupta c

aDepartment of Neurology, Postgraduate Institute of Medical Education and Research, Sector 12, Chandigarh 160012, IndiabDepartment of Neurosurgery, Postgraduate Institute of Medical Education and Research, Sector 12, Chandigarh 160012, IndiacDepartment 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 2014Received in revised form 27 May 2014Accepted 8 June 2014Available online 19 June 2014

Keywords:HemiatrophyGlioependymal cystCerebral amyotrophyIntracranial cyst

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Clinical Neurology and Neurosurgery

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1. Introduction

Glioependymal (neuroglial) cysts are benign developmentalepithelial lined lesions that occur anywhere along the neuraxis.Intraparenchymal Glioependymal cysts present with a varyingsymptomatology.

2. Case report

A 20 year old male presented with progressive left sidedweakness since 2 years. The weakness started from the left lowerlimb progressing on to involve the left upper limb since 2 monthsprior to presentation. He also complained of occasional holocranialheadaches for the last 2 years which had increased in frequencyand severity over the past 2 months. There was history of transientvisual obscurations lasting a few seconds. Examination revealedbilateral papilloedema, left facial hemiatrophy, UMN facial paresisand 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 rightthigh) and left calf. He had a hemiparetic gait. Cortical sensationswere normal. Cranial MRI revealed a 8.4 cm � 6.8 cm � 6.5 cm sizednonenhancing lesion in the right frontoparietal area which wasisointense to CSF on T1 W, T2 W and FLAIR images (Fig. 2A and B).

* Corresponding author at: Department of Neurology, Level 1, Nehru Hospital,Postgraduate Institute of Medical Education and Research, Sector 12, Chandigarh160012, India. Tel.: +91 172 2756690.

E-mail address: dherajk@yahoo.com (D. Khurana).

http://dx.doi.org/10.1016/j.clineuro.2014.06.0050303-8467/ã 2014 Published by Elsevier B.V.

The cystic lesion was seen to compress the ipsilateral ventricle andsurrounding brain tissue and was associated with a midline shift. Aright parietal craniotomy with marsupialization of the cyst wascarried out. Histopathology of the lesion showed a collapsedglioependymal cyst lined by a single layer of ependymal cellswithout basement membrane (Fig. 2C). Following surgery thepatient has shown steady recovery with subsidence of headachesand improvement in power on the left side. Follow up CT scan afterone year shows sustained remission with decompression of thelateral ventricle (Fig. 2D).

3. Discussion

Glioependymal cyst also referred to as neuroglial cyst,ependymal cyst, epithelial cyst or choroidal epithelial cystaccount for less than 1% of all intracranial cysts [1]. They arecongenital lesions, arising from rests of embryonic neural tubeelements that become sequestered within the developing whitematter. These may be intraparenchymal or extraparenchymal, ofwhich the former type is more common. Intraparenchymal cystsoccur most frequently in the frontal lobe although may occuranywhere along the neuraxis. These are rounded, smooth, andunilocular containing clear fluid that resembles CSF. They arelined by ependymal (columnar epithelium) or choroid plexus cells(low cuboidal epithelium). Most patients remain asymptomaticbut some become symptomatic and symptoms depend on the siteand size of the cyst. Common clinical presentation is withseizures [2], hemiparesis, hemianopia, hemianaesthesia, move-ment disorders, raised intracranial pressure (ICP) and alteredsensorium [3].

Fig. 2. Cranial MRI (T1 W axial) showing a cystic lesion (8.4 cm � 6.8 cm � 6.5 cm)in the right frontoparietal area isointense to CSF and compressing the ipsilateralventricle and surrounding brain tissue with a midline shift (A) and Coronal (T2 WFLAIR) showing the cyst causing maximum atrophy of the overlying parietalcortex representing the leg, arm and upper face (homunculus superimposed) (B).Collapsed cyst lined by single layer of ependymal cell (arrow) without basementmembrane (H and E) (C), 1 year post-operative follow up cranial noncontrast CTshowing decompressed right lateral ventricle (D).

Fig. 1. Atrophy of left lower limb.

T. Ferreira et al. / Clinical Neurology and Neurosurgery 124 (2014) 6–7 7

The patient described above presented with progressivehemiparesis and raised ICP which are known presentations ofthis cystic lesion. The hemiatrophy on the side contralateral to thecyst was unusual. The wasting was most pronounced in the leftthigh and calf. This area corresponded to the area of maximumthinning of the sensory and motor cortex caused by the cyst asrepresented on the motor and sensory homunculii (Fig. 2B).Hemiatrophy has been described in lesions affecting the contra-lateral parietal lobe and thalamus and especially when they are oflong duration and occurring early during life [4,5]. The prominenceof this cyst in the right parietal lobe causing a mass effect andatrophy of the surrounding parietal lobe and overlying cortex waslikely responsible for the hemiatrophy. The predominant thinningof the right parietal lobe as well as mass effect on the thalamuscould lead to decrease sensory inputs being projected to thepremotor cortex thus leading to hemiatrophy [4,5].

Surgical treatment is warranted in symptomatic cysts. Surgicaloptions available include cysto-peritoneal shunt, cysto-arachnoidshunt, partial excision, marsupialization, fenestration or totalexcision.

4. Conclusion

Glioependymal cysts may present with contralateral hemia-trophy. These cysts arising from embryonic cell rests enlargeslowly and lead to hemiatrophy due to pressure and subsequentatrophy of the surrounding parietal lobe and thalamus.

References

[1] Osborn AG. Neuroglial cyst. Diagnostic imaging: brain. Salt Lake City: Utah:Amirsys; 2004. p. I-7–I-20.

[2] Boochvar JA, Shafa R, Forman MS, O’Rourka DM. Symptomatic lateral ventricularependymal cysts: criteria for distinguishing these rare cysts from othersymptomatic cysts of the ventricles: casereport. Neurosurgery2000;46:1229–32.

[3] Zheng S-P, Ju Y, You C. Glioependymal cyst in children: a case report. Clin NeurolNeurosurg 2013;115:2288–90.

[4] Campbell WW. DeJong’s: the neurologic examination. 6th ed. Philadelphia:LWW; 2005. p. 403.

[5] Guthrie L. Muscular atrophy and other changes in nutrition associated withlesions of the sensory cortex of the brain with especial reference to the possibleexistence of trophic representation in the postcentral area. Proc R Soc Med1918;11:21–6.