Child's Nervous System

, Volume 34, Issue 12, pp 2377–2390 | Cite as

Clinico-radiological approach to cerebral hemiatrophy

  • Ai Peng TanEmail author
  • Yen Ling Jocelyn Wong
  • Bingyuan Jeremy Lin
  • Hsiang Rong Clement Yong
  • Kshitij Mankad
Review Paper



Cerebral hemiatrophy is an uncommon neuroimaging finding of diverse etiologies, conventionally classified into two broad categories: congenital and acquired. The authors propose an alternative pragmatic clinical approach to cerebral hemiatrophy, classifying its diverse etiologies into a single event insult such as an in utero stroke, or a progressive disorder from an inflammatory or neoplastic process, the latter of which needs urgent intervention and will be the focus of our review paper. Illustrative cases will also be presented to facilitate the understanding of the discussed disorders.


A systematic approach, linking both clinical and neuroimaging features, is important to facilitate the diagnostic workup of cerebral hemiatrophy. This may potentially help avoid large-scale investigations. Determining the underlying aetiology of cerebral hemiatrophy may impact treatment and prognostication as some conditions such as Rasmussen encephalitis and Parry-Romberg syndrome may benefit from timely implementation of immunomodulatory therapy.


Cerebral hemiatrophy Sturge-Weber syndrome Parry-Romberg syndrome Rasmussen’s encephalitis Dyke-Davidoff-Masson syndrome 


Compliance with ethical standards

Conflict of interest



  1. 1.
    Jacoby CG, Go RT, Hahn FJ (1977) Computed tomography in cerebral hemiatrophy. AJR Am J Roentgenol 129:5–9. CrossRefPubMedGoogle Scholar
  2. 2.
    Sirén A-L, Radyushkin K, Boretius S, Kämmer D, Riechers C-C et al (2006) Global brain atrophy after unilateral parietal lesion and its prevention by erythropoietin. Brain 129:480–489. CrossRefPubMedGoogle Scholar
  3. 3.
    McMonagle P, Deering F, Berliner Y, Kertesz A (2006) The cognitive profile of posterior cortical atrophy. Neurology 66:331–338. CrossRefPubMedGoogle Scholar
  4. 4.
    Uduma FU, Emejulu J-KC, Motah M, Okere PCN, Ongolo PC et al (2013) Differential diagnoses of cerebral hemiatrophy in childhood: a review of literature with illustrative report of two cases. Glob J Health Sci 5:195–207. CrossRefPubMedGoogle Scholar
  5. 5.
    Sharma S, Goyal D, Negi A, Sood RG, Jhobta A et al (2006) Dyke-davidoff masson syndrome. Indian J Radiol Imaging 16:165. CrossRefGoogle Scholar
  6. 6.
    Paprocka J, Jamroz E, Adamek D, Marszal E, Mandera M (2006) Difficulties in differentiation of Parry-Romberg syndrome, unilateral facial sclerodermia, and Rasmussen syndrome. Childs Nerv Syst 22:409–415. CrossRefPubMedGoogle Scholar
  7. 7.
    Kister I, Inglese M, Laxer RM, Herbert J (2008) Neurologic manifestations of localized scleroderma: a case report and literature review. Neurology 71:1538–1545. CrossRefPubMedGoogle Scholar
  8. 8.
    Zhang Y, Pu L, Liu X, Xiong H, Li Y et al (2007) Clinical characteristics and treatment of Rasmussen syndrome in 16 children. Zhonghua Er Ke Za Zhi 45:697–702PubMedGoogle Scholar
  9. 9.
    Nagahama Y, Joshi C, Dlouhy B, Wu AY, Abel TJ et al (2017) Functional hemispherotomy in Rasmussen syndrome in the absence of classic MRI findings. Epilepsy Behav Case Rep 7:24–27. CrossRefPubMedGoogle Scholar
  10. 10.
    Aggarwal A, Aggarwal AK, Kapoor A, Kapoor R (2001) Bansal A (2017) Hemiatrophy of brain: antenatal ultrasonography and MRI/postnatal MRI diagnosis with the introduction of “shifted falx sign”. J Med Ultrason 44:147–151. CrossRefGoogle Scholar
  11. 11.
    Comi AM (2006) Advances in Sturge-Weber syndrome. Curr Opin Neurol 19:124–128. CrossRefPubMedGoogle Scholar
  12. 12.
    Lo W, Marchuk DA, Ball KL, Juhász C, Jordan LC et al (2012) Updates and future horizons on the understanding, diagnosis, and treatment of Sturge-Weber syndrome brain involvement. Dev Med Child Neurol 54:214–223. CrossRefPubMedGoogle Scholar
  13. 13.
    Ch’ng S, Tan ST (2008) Facial port-wine stains—clinical stratification and risks of neuro-ocular involvement. J Plast Reconstr Aesthet Surg 61:889–893. CrossRefPubMedGoogle Scholar
  14. 14.
    Mehta M, Salas AH, Fay A (2009) Trigeminal dermatome distribution in patients with glaucoma and facial port wine stain. Dermatology (Basel) 219:219–224. CrossRefGoogle Scholar
  15. 15.
    Inan C, Marcus J (1999) Sturge-Weber syndrome: report of an unusual cutaneous distribution. Brain Dev 21:68–70CrossRefGoogle Scholar
  16. 16.
    Dutkiewicz A-S, Ezzedine K, Mazereeuw-Hautier J, Lacour J-P, Barbarot S et al (2015) A prospective study of risk for Sturge-Weber syndrome in children with upper facial port-wine stain. J Am Acad Dermatol 72:473–480. CrossRefPubMedGoogle Scholar
  17. 17.
    Waelchli R, Aylett SE, Robinson K, Chong WK, Martinez AE et al (2014) New vascular classification of port-wine stains: improving prediction of Sturge-Weber risk. Br J Dermatol 171:861–867. CrossRefPubMedPubMedCentralGoogle Scholar
  18. 18.
    Shirley MD, Tang H, Gallione CJ, Baugher JD, Frelin LP et al (2013) Sturge-Weber syndrome and port-wine stains caused by somatic mutation in GNAQ. N Engl J Med 368:1971–1979. CrossRefPubMedPubMedCentralGoogle Scholar
  19. 19.
    Griffiths PD (1996) Sturge-Weber syndrome revisited: the role of neuroradiology. Neuropediatrics 27:284–294. CrossRefPubMedGoogle Scholar
  20. 20.
    Terdjman P, Aicardi J, Sainte-Rose C, Brunelle F (1991) Neuroradiological findings in Sturge-Weber syndrome (SWS) and isolated pial angiomatosis. Neuropediatrics 22:115–120. CrossRefPubMedGoogle Scholar
  21. 21.
    Benedikt RA, Brown DC, Walker R, Ghaed VN, Mitchell M et al (1993) Sturge-Weber syndrome: cranial MR imaging with Gd-DTPA. AJNR Am J Neuroradiol 14:409–415PubMedGoogle Scholar
  22. 22.
    Comi AM (2003) Pathophysiology of Sturge-Weber syndrome. J Child Neurol 18:509–516. CrossRefPubMedGoogle Scholar
  23. 23.
    Evans AL, Widjaja E, Connolly DJA, Griffiths PD (2006) Cerebral perfusion abnormalities in children with Sturge-Weber syndrome shown by dynamic contrast bolus magnetic resonance perfusion imaging. Pediatrics 117:2119–2125. CrossRefPubMedGoogle Scholar
  24. 24.
    Lin DDM, Barker PB, Hatfield LA, Comi AM (2006) Dynamic MR perfusion and proton MR spectroscopic imaging in Sturge-Weber syndrome: correlation with neurological symptoms. J Magn Reson Imaging 24:274–281. CrossRefPubMedGoogle Scholar
  25. 25.
    Adams ME, Aylett SE, Squier W, Chong W (2009) A spectrum of unusual neuroimaging findings in patients with suspected Sturge-Weber syndrome. AJNR Am J Neuroradiol 30:276–281. CrossRefPubMedGoogle Scholar
  26. 26.
    Portilla P, Husson B, Lasjaunias P, Landrieu P (2002) Sturge-Weber disease with repercussion on the prenatal development of the cerebral hemisphere. AJNR Am J Neuroradiol 23:490–492PubMedGoogle Scholar
  27. 27.
    Avez-Couturier J, Auvin S, Cuisset JM, Soto-Ares G, Lamblin MD et al (2006) Status epilepticus in a 2-month-old infant. Arch Pediatr 13:1540–1542. CrossRefPubMedGoogle Scholar
  28. 28.
    Whitehead MT, Vezina G (2015) Osseous intramedullary signal alteration and enhancement in Sturge-Weber syndrome: an early diagnostic clue. Neuroradiology 57:395–400. CrossRefPubMedGoogle Scholar
  29. 29.
    Curatolo P (2009) MRI appearance of Sturge-Weber syndrome in tuberous sclerosis complex: is the neural crest the culprit? J Child Neurol 24:263–266. CrossRefPubMedGoogle Scholar
  30. 30.
    Tan AP, Chong WK (2018) A child with Apert syndrome and Sturge-Weber syndrome: could fibronectin or the RAS/MAPK signaling pathway be the connection? Childs Nerv Syst. CrossRefGoogle Scholar
  31. 31.
    El-Kehdy J, Abbas O, Rubeiz N (2012) A review of Parry-Romberg syndrome. J Am Acad Dermatol 67:769–784. CrossRefPubMedGoogle Scholar
  32. 32.
    Sommer A, Gambichler T, Bacharach-Buhles M, von Rothenburg T, Altmeyer P et al (2006) Clinical and serological characteristics of progressive facial hemiatrophy: a case series of 12 patients. J Am Acad Dermatol 54:227–233. CrossRefPubMedGoogle Scholar
  33. 33.
    Khan M, Khan M, Negi R, Gupta N (2014) Parry Romberg syndrome with localized scleroderma: a case report. J Clin Exp Dent 6:e313–e316. CrossRefPubMedPubMedCentralGoogle Scholar
  34. 34.
    Longo D, Paonessa A, Specchio N, Delfino LN, Claps D et al (2011) Parry-Romberg syndrome and Rasmussen encephalitis: possible association Clinical and neuroimaging features. J Neuroimaging 21:188–193. CrossRefPubMedGoogle Scholar
  35. 35.
    Aynaci FM, Sen Y, Erdöl H, Ahmetoğlu A, Elmas R (2001) Parry-Romberg syndrome associated with Adie’s pupil and radiologic findings. Pediatr Neurol 25:416–418CrossRefGoogle Scholar
  36. 36.
    Okumura A, Ikuta T, Tsuji T, Kato T, Fukatsu H et al (2006) Parry-Romberg syndrome with a clinically silent white matter lesion. AJNR Am J Neuroradiol 27:1729–1731PubMedGoogle Scholar
  37. 37.
    Tollefson MM, Witman PM (2007) En coup de sabre morphea and Parry-Romberg syndrome: a retrospective review of 54 patients. J Am Acad Dermatol 56:257–263. CrossRefPubMedGoogle Scholar
  38. 38.
    Chiu YE, Vora S, Kwon E-KM, Maheshwari M (2012) A significant proportion of children with morphea en coup de sabre and Parry-Romberg syndrome have neuroimaging findings. Pediatr Dermatol 29:738–748. CrossRefPubMedPubMedCentralGoogle Scholar
  39. 39.
    Madasamy R, Jayanandan M, Adhavan UR, Gopalakrishnan S, Mahendra L (2012) Parry Romberg syndrome: a case report and discussion. J Oral Maxillofac Pathol 16:406–410. CrossRefPubMedPubMedCentralGoogle Scholar
  40. 40.
    Cory RC, Clayman DA, Faillace WJ, McKee SW, Gama CH (1997) Clinical and radiologic findings in progressive facial hemiatrophy (Parry-Romberg syndrome). AJNR Am J Neuroradiol 18:751–757PubMedGoogle Scholar
  41. 41.
    Wong M, Phillips CD, Hagiwara M, Shatzkes DR (2015) Parry romberg syndrome: 7 cases and literature review. AJNR Am J Neuroradiol 36:1355–1361. CrossRefPubMedGoogle Scholar
  42. 42.
    Rigamonti P, Squarza S, Politi M, Sangermani R, Cariati M et al (2017) Parry-Romberg syndrome: conventional and advanced MRI follow-up in a boy. Neuroradiol J 30:445–447. CrossRefPubMedPubMedCentralGoogle Scholar
  43. 43.
    Uhrhan K, Rabenstein M, Kobe C, Drzezga A, Fink GR et al (2017) Brain glucose metabolism in Parry-Romberg Syndrome. Clin Nucl Med 42:e251–e252. CrossRefPubMedGoogle Scholar
  44. 44.
    Asai M, Sago T, Utani A (2015) Parry-Romberg syndrome associated with contralateral cerebral atrophy. Eur J Dermatol 25:624–625. CrossRefPubMedGoogle Scholar
  45. 45.
    Granata T, Gobbi G, Spreafico R, Vigevano F, Capovilla G et al (2003) Rasmussen’s encephalitis: early characteristics allow diagnosis. Neurology 60:422–425CrossRefGoogle Scholar
  46. 46.
    Hart YM, Andermann F, Robitaille Y, Laxer KD, Rasmussen T et al (1998) Double pathology in Rasmussen’s syndrome: a window on the etiology? Neurology 50:731–735CrossRefGoogle Scholar
  47. 47.
    Morse RP (2004) Rasmussen encephalitis. Arch Neurol 61:592–594. CrossRefPubMedGoogle Scholar
  48. 48.
    Alvarez-Barón E, Bien CG, Schramm J, Elger CE, Becker AJ et al (2008) Autoantibodies to Munc18, cerebral plasma cells and B-lymphocytes in Rasmussen encephalitis. Epilepsy Res 80:93–97. CrossRefPubMedGoogle Scholar
  49. 49.
    Deprez L, Weckhuysen S, Holmgren P, Suls A, Van Dyck T et al (2010) Clinical spectrum of early-onset epileptic encephalopathies associated with STXBP1 mutations. Neurology 75:1159–1165. CrossRefPubMedGoogle Scholar
  50. 50.
    Weckhuysen S, Holmgren P, Hendrickx R, Jansen AC, Hasaerts D et al (2013) Reduction of seizure frequency after epilepsy surgery in a patient with STXBP1 encephalopathy and clinical description of six novel mutation carriers. Epilepsia 54:e74–e80. CrossRefPubMedGoogle Scholar
  51. 51.
    Bien CG, Granata T, Antozzi C, Cross JH, Dulac O et al (2005) Pathogenesis, diagnosis and treatment of Rasmussen encephalitis: a European consensus statement. Brain 128:454–471. CrossRefPubMedGoogle Scholar
  52. 52.
    Pradeep K, Sinha S, Saini J, Mahadevan A, Arivazhagan A et al (2014) Evolution of MRI changes in Rasmussen’s encephalitis. Acta Neurol Scand 130:253–259. CrossRefPubMedGoogle Scholar
  53. 53.
    Geller E, Faerber EN, Legido A, Melvin JJ, Hunter JV et al (1998) Rasmussen encephalitis: complementary role of multitechnique neuroimaging. AJNR Am J Neuroradiol 19:445–449PubMedGoogle Scholar
  54. 54.
    Olson HE, Lechpammer M, Prabhu SP, Ciarlini PDSC, Poduri A et al (2013) Clinical application and evaluation of the Bien diagnostic criteria for Rasmussen encephalitis. Epilepsia 54:1753–1760. CrossRefPubMedGoogle Scholar
  55. 55.
    Lee J, Lee BL, Yoo KH, Sung KW, Koo HH et al (2009) Atypical basal ganglia germinoma presenting as cerebral hemiatrophy: diagnosis and follow-up with 11C-methionine positron emission tomography. Childs Nerv Syst 25:29–37. CrossRefPubMedGoogle Scholar
  56. 56.
    Villani A, Bouffet E, Blaser S, Millar B-A, Hawkins C et al (2008) Inherent diagnostic and treatment challenges in germinoma of the basal ganglia: a case report and review of the literature. J Neurooncol 88:309–314. CrossRefPubMedGoogle Scholar
  57. 57.
    Ozelame RV, Shroff M, Wood B, Bouffet E, Bartels U et al (2006) Basal ganglia germinoma in children with associated ipsilateral cerebral and brain stem hemiatrophy. Pediatr Radiol 36:325–330. CrossRefPubMedGoogle Scholar
  58. 58.
    Wei X-H, Shen H-C, Tang S-X, Gao C-H, Ren J-L et al (2016) Radiologic features of primary intracranial ectopic germinomas: case reports and literature review. Medicine (Baltimore) 95:e5543. CrossRefGoogle Scholar
  59. 59.
    Lee SM, Kim I-O, Choi YH, Cheon J-E, Kim WS et al (2016) Early imaging findings in germ cell tumors arising from the basal ganglia. Pediatr Radiol 46:719–726. CrossRefPubMedGoogle Scholar
  60. 60.
    Sadamura Y, Sugiyama K, Uchida H, Hanaya R, Eguchi K et al (2011) Intracranial germinoma presenting with hemiatrophy—follow-up results and literature review—two case reports. Neurol Med Chir (Tokyo) 51:148–152CrossRefGoogle Scholar
  61. 61.
    Higano S, Takahashi S, Ishii K, Matsumoto K, Ikeda H et al (1994) Germinoma originating in the basal ganglia and thalamus: MR and CT evaluation. AJNR Am J Neuroradiol 15:1435–1441PubMedGoogle Scholar
  62. 62.
    Wong S-T, Yuen S-C, Fong D (2009) Pathophysiological mechanism of ipsilateral cerebral and brainstem hemiatrophy in basal ganglia germ cell tumors: case report. Childs Nerv Syst 25:693–699. CrossRefPubMedGoogle Scholar
  63. 63.
    McKinney AM, Thompson LR, Truwit CL, Velders S, Karagulle A et al (2008) Unilateral hypoxic-ischemic injury in young children from abusive head trauma, lacking craniocervical vascular dissection or cord injury. Pediatr Radiol 38:164–174. CrossRefPubMedGoogle Scholar
  64. 64.
    Oderich GS, Sullivan TM, Bower TC, Gloviczki P, Miller DV et al (2007) Vascular abnormalities in patients with neurofibromatosis syndrome type I: clinical spectrum, management, and results. J Vasc Surg 46:475–484. CrossRefGoogle Scholar
  65. 65.
    Pauline L, Kannan B, Saravannan V, Eswaradass VP (2014) Linear nevus sebaceous syndrome. Ann Indian Acad Neurol 17:468–469. CrossRefPubMedPubMedCentralGoogle Scholar
  66. 66.
    Pascual-Castroviejo I (1978) Vascular and nonvascular intracranial malformation associated with external capillary hemangiomas. Neuroradiology 16:82–84CrossRefGoogle Scholar
  67. 67.
    Oe H, Yuasa R, Tsuchiyama M, Kitamura K (1999) Neuroimaging findings of hemiconvulsions, hemiplegia, epilepsy (HHE) syndrome. Rinsho Shinkeigaku 39:485–488PubMedGoogle Scholar
  68. 68.
    Bhat RY, Kakkar S (2014) Prakashini K (2014) Hemiconvulsion-hemiplegia-epilepsy syndrome: clinical course and neuroradiological features in a 20-month-old girl. BMJ Case Rep. Google Scholar
  69. 69.
    Toldo I, Calderone M, Boniver C, Dravet C, Guerrini R et al (2007) Hemiconvulsion-hemiplegia-epilepsy syndrome: early magnetic resonance imaging findings and neuroradiological follow-up. Brain Dev 29:109–111. CrossRefPubMedGoogle Scholar
  70. 70.
    Cole AJ (2004) Status epilepticus and periictal imaging. Epilepsia 45(Suppl 4):72–77. CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Diagnostic ImagingNational University Health SystemSingaporeRepublic of Singapore
  2. 2.Department of PaediatricsNational University Health SystemSingaporeRepublic of Singapore
  3. 3.Department of NeuroradiologyGreat Ormond Street Hospital NHS Foundation TrustLondonUnited Kingdom

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