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Year : 2018  |  Volume : 33  |  Issue : 4  |  Page : 366-367  

Tc-99m ECD brain perfusion single-photon emission computed tomography in Parry–Romberg syndrome


1 Department of Nuclear Medicine and PET, All India Institute of Medical Sciences, New Delhi, India
2 Department of Neurology, CN Centre, All India Institute of Medical Sciences, New Delhi, India

Date of Web Publication9-Oct-2018

Correspondence Address:
Madhavi Tripathi
Department of Nuclear Medicine and PET, All India Institute of Medical Sciences, New Delhi - 110 029
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijnm.IJNM_104_18

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   Abstract 


We report a 15-year-old girl referred to our department for a brain perfusion study with a diagnosis of Parry–Romberg syndrome with left hemifacial atrophy and left enophthalmos. In the presence of a normal magnetic resonance imaging, Tc-99m ECD brain perfusion revealed left temporal lobe hypoperfusion with preserved perfusion in rest of the cortical and subcortical regions and both cerebellar hemispheres.

Keywords: Parry–Romberg syndrome, single-photon emission computed tomography/computed tomography, Tc-99m ECD


How to cite this article:
Angamuthu M, Tripathi M, Parida G, Goyal V, Damle N, Bal C. Tc-99m ECD brain perfusion single-photon emission computed tomography in Parry–Romberg syndrome. Indian J Nucl Med 2018;33:366-7

How to cite this URL:
Angamuthu M, Tripathi M, Parida G, Goyal V, Damle N, Bal C. Tc-99m ECD brain perfusion single-photon emission computed tomography in Parry–Romberg syndrome. Indian J Nucl Med [serial online] 2018 [cited 2019 Oct 22];33:366-7. Available from: http://www.ijnm.in/text.asp?2018/33/4/366/242929



A 15-year-old girl with progressive left hemifacial atrophy [Figure 1]a and [Figure 1]b was diagnosed as Parry–Romberg syndrome (PRS) and referred to our department for a brain perfusion study. She had no neurological complaints and was being evaluated for right enophthalmos and any associated intracranial abnormality. The Tc-99m ECD brain perfusion single-photon emission computed tomography (SPECT) revealed left temporal hypoperfusion [Figure 1]c and [Figure 1]d-arrow and [Figure 1]g and [Figure 2]. Her magnetic resonance imaging (MRI), on the other hand, did not reveal any significant abnormality [Figure 1]e and [Figure 1]f.
Figure 1: (a and b) a 15-year-old girl with progressive left hemifacial atrophy, (c and d) transaxial and coronal Tc-99m ECD brain perfusion images reveal left temporal hypoperfusion, while (e and f) transaxial fluid-attenuated inversion recovery magnetic resonance imaging reveals no abnormality

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Figure 2: Transaxial Tc-99m ECD perfusion single-photon emission computed tomography images showing left temporal hypoperfusion

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Progressive hemifacial atrophy known as PRS is a rare self-limiting degenerative disorder affecting hemifacial skin, muscles, and bone.[1],[2],[3] The involvement of nervous system and ophthalmic system is common while pathogenesis is yet unexplained. Neurological symptoms include headache, trigeminal neuralgia, and focal epilepsy, and MRI is an excellent method to characterize intracranial involvement.[4],[5] Intracranial calcification, brain atrophy, and leptomeningeal enhancement are some of the MRI findings that have been reported. Though PRS is characterised by hemifacial atrophy, the presence of cerebral hemiatrophy on imaging may require it to be differentiated from Rasmussems and Sturge Weber.

Brain perfusion changes have been described in PRS on SPECT. Hyperperfusion involving ipsilateral cerebral cortex with hypoperfusion in white matter of the same hemisphere[6] has been reported in a clinically asymptomatic patient with extensive white matter abnormalities on MRI. Hypoperfusion involving the right parietooccipital region has been described in a case of PRS with epilepsy.[7] Abnormal SPECT with unrevealing MRI has also been reported in progressive hemifacial atrophy.[8] Thus, addition of Tc-99m ECD brain perfusion SPECT may help explain the functional significance of structural abnormalities and details of central nervous system involvement in PRS, hereby prognosticating these patients.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest



 
   References Top

1.
Wong M, Phillips CD, Hagiwara M, Shatzkes DR. Parry Romberg syndrome: 7 Cases and literature review. AJNR Am J Neuroradiol 2015;36:1355-61.  Back to cited text no. 1
    
2.
Doolittle DA, Lehman VT, Schwartz KM, Wong-Kisiel LC, Lehman JS, Tollefson MM. CNS imaging findings associated with Parry-Romberg syndrome and en coup de sabre: Correlation to dermatologic and neurologic abnormalities. Neuroradiology 2015;57:21-34.  Back to cited text no. 2
    
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Patel H, Thakkar C, Patel K. Parry-Romberg syndrome: A rare entity. J Maxillofac Oral Surg 2010;9:247-50.  Back to cited text no. 3
    
4.
Tolkachjov SN, Patel NG, Tollefson MM. Progressive hemifacial atrophy: A review. Orphanet J Rare Dis 2015;10:39.  Back to cited text no. 4
    
5.
de Paula RA, Ribeiro BN, Bahia PR, Ribeiro RN, de Carvalho LB. Parry-Romberg syndrome: Findings in advanced magnetic resonance imaging sequences – Case report. Radiol Bras 2014;47:186-8.  Back to cited text no. 5
    
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Okumura A, Ikuta T, Tsuji T, Kato T, Fukatsu H, Naganawa S, et al. Parry-Romberg syndrome with a clinically silent white matter lesion. AJNR Am J Neuroradiol 2006;27:1729-31.  Back to cited text no. 6
    
7.
DeFelipe J, Segura T, Arellano JI, Merchán A, DeFelipe-Oroquieta J, Martín P, et al. Neuropathological findings in a patient with epilepsy and the Parry-Romberg syndrome. Epilepsia 2001;42:1198-203.  Back to cited text no. 7
    
8.
Blaszczyk M, Królicki L, Krasu M, Glinska O, Jablonska S. Progressive facial hemiatrophy: Central nervous system involvement and relationship with scleroderma en coup de sabre. J Rheumatol 2003;30:1997-2004.  Back to cited text no. 8
    


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  [Figure 1], [Figure 2]



 

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