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-
1.
World Health Organization , 2006. Weekly epidemiological record. 81: 13–20. Available at: http://www.who.int/vaccine_safety/committee/reports/wer8102.pdf?ua=1. Accessed November 9, 2022.
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2.
Garg RK , Mahadevan A , Malhotra HS , Rizvi I , Kumar N , Uniyal R , 2019. Subacute sclerosing panencephalitis. Rev Med Virol 29: e2058.
-
3.
Garg D , Kakkar V , Kumar A , Kapoor D , Abbey P , Pemde H , Mukherjee SB , Sharma S , 2022. Spectrum of movement disorders among children with subacute sclerosing panencephalitis: a cross-sectional study. J Child Neurol 37: 491–496.
-
4.
Pandey S , Tomar LR , Tater P , 2018. Pisa syndrome in a child with subacute sclerosing panencephalitis. JAMA Neurol 75: 255–256.
-
5.
Malhotra HS , Garg RK , 2015. Pearls & Oy-sters: Pisa syndrome: an unusual feature of adult-onset fulminant SSPE. Neurology 84: e12–e14.
-
6.
Pandey S , Shukla T , Mishra A , 2021. The spectrum of repetitive behaviors associated with subacute sclerosing panencephalitis. Mov Disord 36: 497–503.
-
7.
Arhan E , Akbaş Y , Serdaroğlu A , Direk MC , Okuyaz Ç , Havalı C , Aydın K , 2021. Magnetic resonance imaging patterns of subacute sclerosing panencephalitis may mimic metabolic disorders: clinical, electroencephalographic and imaging features of six cases. Acta Neurol Belg 121: 955–965.
-
8.
Prasad C , Netravathi M , Kulanthaivelu K , Bhat MD , Pendharkar H , 2022. Subacute sclerosing panencephalitis: restricted diffusion and clinical evolution. Neurol India 70: 275–280.
-
9.
Tandra HV , Roy PS , Sharma R , Bhatia V , Saini AG , 2019. Subacute sclerosing panencephalitis presenting as choreoathetosis and basal ganglia hyperintensities. Neurohospitalist 9: 26–29.
-
10.
Das B , Goyal MK , Modi M , Mehta S , Chakravarthi S , Lal V , Vyas S , 2016. Atypical magnetic resonance imaging features in subacute sclerosing panencephalitis. Ann Indian Acad Neurol 19: 275–276.
-
11.
Turaga SP , Kaul S , Haritha Chowdary HA , Praveen P , 2012. Isolated bilateral basal ganglionic hyper intensities in early stage of subacutesclerosing panencephalitis: a case report. Neurol India 60: 521–522.
-
12.
Lebon S , Maeder P , Maeder-Ingvar M , Poloni C , Mayor-Dubois C , Roulet-Perez E , Jeannet PY , 2011. An initial MRI picture of limbic encephalitis in subacute sclerosing panencephalitis. Eur J Paediatr Neurol 15: 544–546.
-
13.
Sener RN , 2004. Subacute sclerosing panencephalitis with pontine involvement. J Comput Assist Tomogr 28: 101–102.
-
14.
Kathait A , Garg D , Chatterjee A , Reddy N , 2022. Cavitating leukoencephalopathy in subacute sclerosing panencephalitis. Ann Neurol 92: 1102–1104.
-
15.
Yaramiş A , Taşkesen M , 2010. Brainstem involvement in subacute sclerosing panencephalitis. Turk J Pediatr 52: 542–545.
-
16.
Kandadai RM , Yada P , Uppin MS , Jabeen SA , Cherian A , Kanikannan MA , Borgohain R , Challa S , 2014. Fulminant subacute sclerosing panencephalitis presenting with acute ataxia and hemiparesis in a 15-year-old boy. J Clin Neurol 10: 354–357.
| Past two years | Past Year | Past 30 Days | |
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| Abstract Views | 2323 | 1104 | 72 |
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Case Report: An Unusual Case of Subacute Sclerosing Panencephalitis with Distinctive Clinical and Neuroimaging Features
Ravindra Kumar Garg
Ravindra Kumar Garg
Department of Neurology, King George’s Medical University Uttar Pradesh, Lucknow, India
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Shweta Pandey
Shweta Pandey
Department of Neurology, King George’s Medical University Uttar Pradesh, Lucknow, India
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Harish Nigam
Harish Nigam
Department of Neurology, King George’s Medical University Uttar Pradesh, Lucknow, India
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D. B. Keerthiraj
D. B. Keerthiraj
Department of Neurology, King George’s Medical University Uttar Pradesh, Lucknow, India
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Imran Rizvi
Imran Rizvi
Department of Neurology, King George’s Medical University Uttar Pradesh, Lucknow, India
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Neeraj Kumar
Neeraj Kumar
Department of Neurology, King George’s Medical University Uttar Pradesh, Lucknow, India
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Ravi Uniyal
Ravi Uniyal
Department of Neurology, King George’s Medical University Uttar Pradesh, Lucknow, India
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Hardeep Singh Malhotra
Hardeep Singh Malhotra
Department of Neurology, King George’s Medical University Uttar Pradesh, Lucknow, India
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Praveen Kumar Sharma
Praveen Kumar Sharma
Department of Neurology, King George’s Medical University Uttar Pradesh, Lucknow, India
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ABSTRACT.
Subacute sclerosing panencephalitis (SSPE) is a relentlessly progressive brain disorder with invariable mortality. Subacute sclerosing panencephalitis is common in measles-endemic areas. We report an unusual SSPE patient with distinctive clinical and neuroimaging features. A 9-year-old boy came with a 5-month history of spontaneously dropping objects from both hands. Subsequently, he developed mental decline, a loss of interest in his surroundings, decreased verbal output, and inappropriate crying and laughing along with generalized periodic myoclonus. On examination, the child was akinetic mute. The child demonstrated intermittent generalized axial dystonic storm with flexion of upper limbs, an extension of lower limbs, and opisthotonos. Dystonic posturing was more dominant on the right side. Electroencephalography revealed periodic discharges. Cerebrospinal fluid antimeasles IgG antibody titer was markedly elevated. Magnetic resonance imaging revealed marked diffuse cerebral atrophy, and periventricular T2/fluid-attenuated inversion recovery hyperintensity. T2/fluid-attenuated inversion recovery images also revealed multiple cystic lesions present in the region of periventricular white matter. The patient was given a monthly injection of intrathecal interferon-α. The patient is currently continuing in the akinetic-mute stage. In conclusion, in this report, we described an unusual case of acute fulminant SSPE in which neuroimaging demonstrated unusual multiple small discrete cystic lesions in the cortical white matter. The pathological nature of these cystic lesions currently is not clear and needs to be explored.
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Author Notes
Authors’ addresses: Ravindra Kumar Garg, Shweta Pandey, Harish Nigam, D. B. Keerthiraj, Imran Rizvi, Neeraj Kumar, Ravi Uniyal, Hardeep Singh Malhotra, and Praveen Kumar Sharma, Department of Neurology, King George’s Medical University Uttar Pradesh, Lucknow, India, E-mails: garg50@yahoo.com, shwetapandey@kgmcindia.edu, harish.nigam007@gmail.com, dbkeerthiraj@gmail.com, imranrizvi09@gmail.com, drneeraj2903@gmail.com, ravi.sun.uniyal@gmail.com, drhsmalhotra@gmail.com, and pspgimer@gmail.com.
ProCite
RefWorks
Reference Manager
BibTeX
Zotero
EndNote
-
1.
World Health Organization , 2006. Weekly epidemiological record. 81: 13–20. Available at: http://www.who.int/vaccine_safety/committee/reports/wer8102.pdf?ua=1. Accessed November 9, 2022.
-
2.
Garg RK , Mahadevan A , Malhotra HS , Rizvi I , Kumar N , Uniyal R , 2019. Subacute sclerosing panencephalitis. Rev Med Virol 29: e2058.
-
3.
Garg D , Kakkar V , Kumar A , Kapoor D , Abbey P , Pemde H , Mukherjee SB , Sharma S , 2022. Spectrum of movement disorders among children with subacute sclerosing panencephalitis: a cross-sectional study. J Child Neurol 37: 491–496.
-
4.
Pandey S , Tomar LR , Tater P , 2018. Pisa syndrome in a child with subacute sclerosing panencephalitis. JAMA Neurol 75: 255–256.
-
5.
Malhotra HS , Garg RK , 2015. Pearls & Oy-sters: Pisa syndrome: an unusual feature of adult-onset fulminant SSPE. Neurology 84: e12–e14.
-
6.
Pandey S , Shukla T , Mishra A , 2021. The spectrum of repetitive behaviors associated with subacute sclerosing panencephalitis. Mov Disord 36: 497–503.
-
7.
Arhan E , Akbaş Y , Serdaroğlu A , Direk MC , Okuyaz Ç , Havalı C , Aydın K , 2021. Magnetic resonance imaging patterns of subacute sclerosing panencephalitis may mimic metabolic disorders: clinical, electroencephalographic and imaging features of six cases. Acta Neurol Belg 121: 955–965.
-
8.
Prasad C , Netravathi M , Kulanthaivelu K , Bhat MD , Pendharkar H , 2022. Subacute sclerosing panencephalitis: restricted diffusion and clinical evolution. Neurol India 70: 275–280.
-
9.
Tandra HV , Roy PS , Sharma R , Bhatia V , Saini AG , 2019. Subacute sclerosing panencephalitis presenting as choreoathetosis and basal ganglia hyperintensities. Neurohospitalist 9: 26–29.
-
10.
Das B , Goyal MK , Modi M , Mehta S , Chakravarthi S , Lal V , Vyas S , 2016. Atypical magnetic resonance imaging features in subacute sclerosing panencephalitis. Ann Indian Acad Neurol 19: 275–276.
-
11.
Turaga SP , Kaul S , Haritha Chowdary HA , Praveen P , 2012. Isolated bilateral basal ganglionic hyper intensities in early stage of subacutesclerosing panencephalitis: a case report. Neurol India 60: 521–522.
-
12.
Lebon S , Maeder P , Maeder-Ingvar M , Poloni C , Mayor-Dubois C , Roulet-Perez E , Jeannet PY , 2011. An initial MRI picture of limbic encephalitis in subacute sclerosing panencephalitis. Eur J Paediatr Neurol 15: 544–546.
-
13.
Sener RN , 2004. Subacute sclerosing panencephalitis with pontine involvement. J Comput Assist Tomogr 28: 101–102.
-
14.
Kathait A , Garg D , Chatterjee A , Reddy N , 2022. Cavitating leukoencephalopathy in subacute sclerosing panencephalitis. Ann Neurol 92: 1102–1104.
-
15.
Yaramiş A , Taşkesen M , 2010. Brainstem involvement in subacute sclerosing panencephalitis. Turk J Pediatr 52: 542–545.
-
16.
Kandadai RM , Yada P , Uppin MS , Jabeen SA , Cherian A , Kanikannan MA , Borgohain R , Challa S , 2014. Fulminant subacute sclerosing panencephalitis presenting with acute ataxia and hemiparesis in a 15-year-old boy. J Clin Neurol 10: 354–357.
| Past two years | Past Year | Past 30 Days | |
|---|---|---|---|
| Abstract Views | 2323 | 1104 | 72 |
| Full Text Views | 2554 | 198 | 0 |
| PDF Downloads | 134 | 19 | 0 |