Subscribe to RSS
Subacute Sclerosing Panencephalitis (SSPE): Experience from a Tertiary-Care Pediatric CenterFunding None.
Introduction Subacute sclerosing panencephalitis (SSPE) is a devastating neurodegenerative disease occurring as a complication of measles infection that is still prevalent in low-resource countries. Clinical and electrographical variability in SSPE can lead to diagnostic delays.
Methods Children diagnosed with SSPE in a tertiary care pediatric hospital in India in a period of 8 years were included in the study. The diagnosis was established on the basis of Dyken's criteria. The demographic data, clinical presentations, investigations, treatment approaches, and outcomes were reviewed and recorded.
Results Thirty-four patients were included in the analysis. Average age at symptom onset was 7 years, 5 months. Majority of the children were not vaccinated for measles. Most patients (80%) presented with stage 2 of illness. Nearly 25% presented with atypical clinical features. Myoclonus was the most predominant feature seen after diagnosis. Electroencephalography (EEG) was the most useful investigation for suspecting the diagnosis. All patients showed deterioration in neurological status with time and 20% died during follow-up.
Conclusion Atypical presentations of SSPE must be recognized in areas with high incidence to institute timely treatment and establish prognosis. EEG findings were found to be the most important indicator for diagnosis. Measles eradication will pave the way for elimination of this dreaded disease.
Written informed consent was obtained from the parent(s) of the patients for the publication of patient data and accompanying images.
Ethical approval for the publication was obtained from the ethics committee of the institute for this study.
M.G. conceptualized the study and wrote the initial draft. M.G. and A.A. were involved in data collection and literature search. S.D.K. revised the initial drafts. A.U.H. and K.N.S. revised and edited the manuscript. All authors were involved in patient care. The final manuscript has been read, revised, and approved by all the authors.
Article published online:
10 February 2022
© 2022. Association for Helping Neurosurgical Sick People. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)
Thieme Medical and Scientific Publishers Pvt. Ltd.
A-12, 2nd Floor, Sector 2, Noida-201301 UP, India
- 1 Garg RK. Subacute sclerosing panencephalitis. J Neurol 2008; 255 (12) 1861-1871
- 2 Bellini WJ, Rota JS, Lowe LE. et al. Subacute sclerosing panencephalitis: more cases of this fatal disease are prevented by measles immunization than was previously recognized. J Infect Dis 2005; 192 (10) 1686-1693
- 3 Campbell H, Andrews N, Brown KE, Miller E. Review of the effect of measles vaccination on the epidemiology of SSPE. Int J Epidemiol 2007; 36 (06) 1334-1348
- 4 Demir E, Aksoy A, Anlar B, Sönmez FM. Atypical presentations of SSPE: a clinical study in four cases. Turk J Pediatr 2007; 49 (03) 295-300
- 5 Dyken PR. Subacute sclerosing panencephalitis. Current status. Neurol Clin 1985; 3 (01) 179-196
- 6 Kornberg AJ, Harvey AS, Shield LK. Subacute sclerosing panencephalitis presenting as simple partial seizures. J Child Neurol 1991; 6 (02) 146-149
- 7 Bamford JM, Sandercock PAG, Warlow CP, Slattery J. Interobserver agreement for the assessment of handicap in stroke patients. (letter) Stroke 1989; 20 (06) 828
- 8 Khare S, Kumari S, Verghese T. Subacute sclerosing panencephalitis in Delhi. J Trop Pediatr 1994; 40 (06) 326-328
- 9 Jagtap SA, Nair MD, Kambale HJ. Subacute sclerosing panencephalitis: a clinical appraisal. Ann Indian Acad Neurol 2013; 16 (04) 631-633
- 10 Gutierrez J, Issacson RS, Koppel BS. Subacute sclerosing panencephalitis: an update. Dev Med Child Neurol 2010; 52 (10) 901-907
- 11 Erturk O, Karslıgil B, Cokar O. et al. Challenges in diagnosing SSPE. Childs Nerv Syst 2011; 27 (12) 2041-2044
- 12 Goraya J, Marks H, Khurana D, Legido A, Melvin J. Subacute sclerosing panencephalitis (SSPE) presenting as acute disseminated encephalomyelitis in a child. J Child Neurol 2009; 24 (07) 899-903
- 13 Misra A, Roy A, Das Kr S. Parkinsonian presentation of SSPE: report of two cases. Neurol Asia 2008; 13: 117-120
- 14 Prashanth LK, Taly AB, Sinha S, Ravi V. Subacute sclerosing panencephalitis (SSPE): an insight into the diagnostic errors from a tertiary care university hospital. J Child Neurol 2007; 22 (06) 683-688
- 15 Blume WT, Kibara M. Atlas of Pediatric Electroencephalography. 2nd edition.. Philadelphia, PA: Lippincott-Raven; 1999. 370.24
- 16 Ekmekci O, Karasoy H, Gökçay A, Ulkü A. Atypical EEG findings in subacute sclerosing panencephalitis. Clin Neurophysiol 2005; 116 (08) 1762-1767
- 17 Dyken PR, Swift A, DuRant RH. Long-term follow-up of patients with subacute sclerosing panencephalitis treated with inosiplex. Ann Neurol 1982; 11 (04) 359-364
- 18 Gascon GG. International Consortium on Subacute Sclerosing Panencephalitis. Randomized treatment study of inosiplex versus combined inosiplex and intraventricular interferon-α in subacute sclerosing panencephalitis (SSPE): international multicenter study. J Child Neurol 2003; 18 (12) 819-827
- 19 Becker D, Patel A, Abou-Khalil BW, Pina-Garza JE. Successful treatment of encephalopathy and myoclonus with levetiracetam in a case of subacute sclerosing panencephalitis. J Child Neurol 2009; 24 (06) 763-767
- 20 Krauss GL, Bergin A, Kramer RE, Cho YW, Reich SG. Suppression of post-hypoxic and post-encephalitic myoclonus with levetiracetam. Neurology 2001; 56 (03) 411-412