• Users Online: 176
  • Home
  • Print this page
  • Email this page
Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Login 


 
 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 8  |  Issue : 4  |  Page : 272-275

Cerebral hemiatrophy: Case series of three cases


Department of Radiodiagnosis and Imaging, GMC, Srinagar, Jammu and Kashmir, India

Date of Submission25-May-2020
Date of Acceptance23-Sep-2020
Date of Web Publication27-May-2022

Correspondence Address:
Obaid Ashraf
Department of Radiodiagnosis and Imaging, GMC, Srinagar, Jammu and Kashmir
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/cjhr.cjhr_57_20

Rights and Permissions
  Abstract 


Pediatric cerebral hemiatrophy is a rare entity with widespread etiology. Imaging in form of computed tomography and magnetic resonance imaging is helpful in differentiating between the etiologies of hemiatrophy. We describe imaging findings in three different cases of hemiatrophy due to Sturge-Weber syndrome, Dyke-Davidoff-Masson syndrome, and Rasmussen encephalitis.

Keywords: Dyke-Davidoff-Masson syndrome, hemiatrophy, magnetic resonance imaging, Rasmussen encephalitis, Sturge-Weber syndrome


How to cite this article:
Rafiq S, Dar MA, Ashraf O, Ellahi I. Cerebral hemiatrophy: Case series of three cases. CHRISMED J Health Res 2021;8:272-5

How to cite this URL:
Rafiq S, Dar MA, Ashraf O, Ellahi I. Cerebral hemiatrophy: Case series of three cases. CHRISMED J Health Res [serial online] 2021 [cited 2022 Jul 6];8:272-5. Available from: https://www.cjhr.org/text.asp?2021/8/4/272/346101




  Introduction Top


Atrophy of one cerebral hemisphere in the presence of normal contralateral hemisphere is known as hemiatrophy. It occurs due to variety of congenital like intrauterine vascular injury and acquired conditions such as postictal hemiatrophy, Rassmussens encephalitis, Sturge-Weber syndrome (SWS), hemimegalencephaly, Dyke-Davidoff-Mason syndrome, perinatal intracranial hemorrhage, and postischemic, traumatic or hypoxic changes. Shift of midline structures toward abnormal or diseased and paucity of sulcal prominence[1] help to differentiate in utero insults from secondary cerebral hemiatrophy. We describe hemiatrophy in three pediatric patients, one each of SWS, Dyke Davidoff Masson syndrome (DDMS), and Rasmussen Encephalitis. SWS is a vascular phakomatosis characterized by cortical anoxia due to stasis or occlusion of persistent fetal vasculature.[2] Classical features of SWS such as leptomeningeal pial angioma and choroidal angioma can be demonstrated by computed tomography (CT) and magnetic resonance imaging (MRI).[3] DDMS was first described by Dyke et al. in 1933 characterized by the clinical features of hemiparesis, seizures, facial asymmetry, and mental retardation.[4],[5] Rasmussen encephalitis is a chronic inflammatory disease characterized by progressive neurological deterioration, cognitive decline, and intractable seizures.[6]


  Case Reports Top


Case 1

A 3-year-old child presented to the causality department with a history of recurrent seizures, developmental delay weakness of the left side of the body. Clinical examination reveals port wine stain on the right side of the face and visual impairment. MRI was advised. MRI revealed evidence of right cerebral hemiatrophy, right enhancing pial angioma with enhancing and enlarged ipsilateral choroid plexus (choroidal angioma) [Figure 1] with gyriform areas of hypointensity in right temporooccipeto-parietal region on fluid attenuated inversion recovery [Figure 2], T2-weighted images with blooming on susceptibility weighted imaging (SWI) suggestive of calcification. There is evidence of smooth enhancing thickening around the posterior segment of the globe suggestive of choroidal angioma [Figure 3]. The diagnosis of SWS was made.
Figure 1: Coronal postcontrast magnetic resonance image revealing enhancing right choroidal angioma with overlying enhancing pial angioma and right hemiatrophy

Click here to view
Figure 2: Axial fluid attenuated inversion recovery magnetic resonance revealing hypointensity along pial angioma in right temporo-occipetal region

Click here to view
Figure 3: Axial postcontrast magnetic resonance image showing evidence of right smoothly enhancing posterior segment choroidal angioma along with enhancing right pial angioma

Click here to view


Case 2

A 15-year-old child presented to the pediatric outpatient department with right hemiplegia, cognitive impairment, difficulty in studying and walking, history of seizures, and mental retardation. Basic laboratory tests are normal. Her perinatal period was uneventful. MRI is advised, but the patient is unable to cooperate and still despite proper sedation. The patient underwent computerized tomography of the head which revealed hemiatrophy of left cerebral hemisphere, dilated left lateral ventricle [Figure 4] and [Figure 5], prominent frontal subarachnoid spaces, mildly thickened left calvarial vault [Figure 6], and mild hyperpneumatization of paranasal sinuses. There was no involvement of basal ganglia. Diagnosis of DDMS was made.
Figure 4: Axial computed tomography revealing evidence of left hemiatrophy with dilated left lateral ventricle and enlarged extraaxial spaces

Click here to view
Figure 5: Coronal computed tomography revealing evidence of left hemiatrophy with dilated left lateral ventricle and enlarged extraaxial spaces

Click here to view
Figure 6: Coronal computed tomography showing mildly thickened left calvarial bony vault

Click here to view


Case 3

A 6-year-old female child presents to the pediatric outpatient department with left-sided hemiplegia, speech disturbances, and intractable tonic–clonic seizures from 3 years. Seizures are resistant to pharmacotherapy. Her developmental milestones are normal. There is no history of birth trauma. She has exaggerated upper and lower limb tendon reflexes. MRI is advised. MRI revealed evidence of prominent right extraaxial spaces, especially around the insular region and dilated right ventricle [Figure 7]. There was atrophy of right caudate nucleus [Figure 8] and hippocampus [Figure 9] along with diffuse loss of right cerebral white matter. Left cerebral hemisphere is normal. There was no evidence of any calvarial thickening or hyperpneumatization of paranasal sinuses. In view of right hemiatrophy, the involvement of basal ganglia and loss of white matter diagnosis of Rasmussen's encephalitis is made.
Figure 7: Coronal fluid-attenuated inversion recovery magnetic resonance showing evidence of right lateral ventricle dilatation with prominent extra-axial spaces and white matter loss

Click here to view
Figure 8: Axial fluid attenuated inversion recovery magnetic resonance revealing evidence atrophied right caudate nucleus with prominent periinsular extra-axial spaces with white matter loss

Click here to view
Figure 9: Coronal fluid attenuated inversion recovery magnetic resonance showing evidence of right lateral ventricle dilatation, atrophied right hippocampus, and caudate nucleus with white matter loss

Click here to view



  Discussion Top


The prevalence of SWS is 1 in 50,000 live births.[7] It is a sporadic neurocutaneous syndrome with predominant vascular manifestations. Contrast-enhanced MRI is the investigation of choice in SWS for depicting the presence and extent of leptomeningeal angiomatosis which is the hallmark of SWS. Enhancement of the leptomeninges occurs due to leptomeningeal angiomatosis and homogeneous enhancement of an enlarged ipsilateral choroid plexus are due to choroid plexus angioma.[8] Ischemia and hypoxia due to abnormal cerebrovenous drainage lead to progressive neurologic deterioration. MR perfusion imaging and single-photon emission CT scanning have revealed decreased perfusion in the areas of leptomeningeal enhancement[9] which then progresses to hemiatrophy. Hemiatrophy in SWS is usually unilateral and confined to the parieto-occipital area, but can involve entire hemisphere or there can be bilateral involvement.[10],[11] Gyriform hypointensities on T1- and T2-weighted MRI images seen as the areas of blooming on SWI occur due to venous collateralization and cortical calcification. These present as tramline calcifications on CT with underlying leptomeningeal angiomatosis.[11]

DDMS is a childhood hemiatrophy with compensatory ipsilateral calvarial hypertrophy.[12],[13] DDMS occurs secondary to vascular, congenital or acquired ischaemic disease, traumatic or inflammatory insult occurring in utero or during the childhood period.[12] Calvarial changes occur only if brain damage occurs before 3 years of age.[13] Failure of brain growth results in compensatory changes like hyperpneumatized sinuses due to inward redirection of nearby calvarial growth. CT is preferred over conventional radiography in DDMS.[14]

Rasmussen encephalitis was discovered in 1958 by Theodore Rasmussen.[15],[16] Rasmussen encephalitis is usually unicerebral and generally occurs in children under the age of 15 years with median age of 6 years.[15],[16] It is a diagnosis of exclusion and typically insidious in onset.[15] It is characterized by unilateral hemispheric atrophy, abrupt appearance of focal, persistent motor seizures, followed by hemiplegia and progressive cognitive deterioration.[17] Atrophy of the head of the caudate, volume loss most accentuated in the insular and peri-insular regions, atrophy of hippocampus along with hemiatrophy as in our case have been seen in Rasmussen encephalitis.[15],[16]

Differential diagnosis of hemiatrophy include basal cell germinoma, Fishman syndrome, Silver-Russell Syndrome, Post-Ictal cerebral hemiatrophy, Hemiconvulsion-Hemiplegia-Epilepsy Syndrome, Linear Scleroderma, Linear Sebaccious Nervus Syndrome, Parry Romberg Syndrome, and Haberland Syndrome (Fishman Syndrome).[18]


  Conclusion Top


Cross-sectional imaging in form of MRI and CT plays a vital role in differentiating the etiology of cerebral hemiatrophy as well as correctly describing the spectrum of the disease.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
McMonagle P, Deering F, Berliner Y, Kertesz A. The cognitive profile of posterior cortical atrophy. Neurology 2006;66:331-8.  Back to cited text no. 1
    
2.
Rumboldt Z, Castillo M, Huang B, Andrea Rossi. Brain Imaging with MRI and CT: An Image Pattern Approach: Cambridge University Press Cambridge Medicine; 2013. p. 428.  Back to cited text no. 2
    
3.
Thomas-Sohl KA, Vaslow DF, Maria BL. Sturge-Weber syndrome: A review. Pediatr Neurol 2004;30:303-10.  Back to cited text no. 3
    
4.
Dyke CG, Davidoff LM, Masson CB. Cerebral hemiatrophy and homolateral hypertrophy of the skull and sinuses. Surg Gynecol Obstet 1933;57:588-600.  Back to cited text no. 4
    
5.
Yerdelen D, Zafer F. Dyke–Davidoff–Masson syndrome. Neurosurg Q 2009;19:59-61.  Back to cited text no. 5
    
6.
Rasmussen T, Olszewski J, Lloydsmith D. Focal seizures due to chronic localized encephalitis. Neurology 1958;8:435-45.  Back to cited text no. 6
    
7.
Boyer RS. Disorders of histogenesis: Neurocutaneous syndromes. In: Osborn AG, editor. Diagnostic Neuroradiology. 1st ed. St Louis, Mo: Mosby; 1994. p. 72-113.  Back to cited text no. 7
    
8.
Zhou J, Li N, Zhou X, Wang J, Ma H, Zhang R. Sturge Weber Syndrome, A case report and review of literature. Chin Med J 2010;123:117-21.  Back to cited text no. 8
    
9.
Lin DD, Barker PB, Kraut MA, Comi A. Early characteristics of Sturge-Weber syndrome shown by perfusion MR imaging and proton MR spectroscopic imaging. AJNR Am J Neuroradiol 2003;24:1912-5.  Back to cited text no. 9
    
10.
Kelley TM, Hatfield LA, Lin DD, Comi AM. Quantitative analysis of cerebral cortical atrophy and correlation with clinical severity in unilateral Sturge-Weber syndrome. J Child Neurol 2005;20:867-70.  Back to cited text no. 10
    
11.
Marisa KB, Glenn AT. Pictorial essay: MRI of cerebral microhaemorrhages. AJR 2007;189:720-5.  Back to cited text no. 11
    
12.
Goyal J, Shah V, Rao S, Jindal N. Dyke Davidoff Masson syndrome in Children. The Internet Journal of Pediatrics and Neonatology 2008;10.  Back to cited text no. 12
    
13.
Sharma S, Goyal D, Negi A, Sood RG, Jhobta A, Surya M. Dike Davidoff Mason Syndrome. Neuroradiol 2006;16:165-6.  Back to cited text no. 13
    
14.
Radswiki R, Gaillard F. Cerebral hemiatrophy; 2011. Available from: http://radiopaedia.org/articles/cerebral-hemiatrophy. [Last accessed on 2020 04].  Back to cited text no. 14
    
15.
Akinkunmi M, Salisu M, Awosanya G. Rasmussen Encephalitis In A Nigerian Child: A Case Report. The Internet Journal of Radiology. 2009;12.  Back to cited text no. 15
    
16.
Bien CG, Granata T, Antozzi C, Cross JH, Dulac O, Kurthen M, et al. Pathogenesis, diagnosis and treatment of Rasmussen encephalitis: A European consensus statement. Brain 2005;128:454-71.  Back to cited text no. 16
    
17.
Zhang YH, Pu LH, Liu XY, Xiong H, Li YL, Liu XZ, et al. Clinical characteristics and treatment of Rasmussen syndrome in 16 children. Zhonghua Er Ke Za Zhi 2007;45:697-702.  Back to cited text no. 17
    
18.
Uduma FU, Emejulu JK, Motah M, Okere PC, Ongolo PC, Muna W. Differential diagnoses of cerebral hemiatrophy in childhood: A review of literature with illustrative report of two cases. Glob J Health Sci 2013;5:195-207.  Back to cited text no. 18
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9]



 

Top
 
 
  Search
 
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

 
  In this article
Abstract
Introduction
Case Reports
Discussion
Conclusion
References
Article Figures

 Article Access Statistics
    Viewed134    
    Printed2    
    Emailed0    
    PDF Downloaded23    
    Comments [Add]    

Recommend this journal


[TAG2]
[TAG3]
[TAG4]