Background: Subacute sclersing panencephalitis (SSPE) is a very rare progressive, fatal neurodegenerative disease of the control nervous system of childhood and early adolescence. It is a slow virus disease caused by persistent defective measles virus infection of the brain
Objective: To see the clinical andneuro-imaging findings in children with Subacute sclerosing panencephalitis.
Methods: This retrospective study was conducted at Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, a tertiary care premier Postgraduate Medical Institution in Bangladesh. Thirty (30) Subacute sclerosing panencephalitis (SSPE) children were evaluated at paediatric neurology ward during the period January 2010 to December 2017. Diagnosis was based on typical clinical characteristic features, the presence of periodic discharges on EEG, demonstration of raised antibody titer against measles in the plasma and cerebrospinal fluid Detected by ELISA in all patients.
Results: Total number of studied children were 30. Mean age was 10.2±3.1 year and Male female ratio was 5:1. Most of the patient arrived from poor socio-economic (83.33%) background of rural area (66.67%) of Bangladesh. Among them 46.67% had history of measles infection during early childhood. Progressive deterioration of school performance (50%), gait disturbance (70%), myoclonus (83%) dysarthria (43%) and Ocular manifestations like optic atrophy & papilledema (83.33%) were the main presenting feature of our studied children. All of the patients (100%) showed positive measles specific antibody IgG in CSF and On electroencephalographic findings showed periodic burst suppression in 90.90% cases. Most of the children (56.6%) were in stage II category and other 3.3%, 33.3%,6.6%, were stage I, stage III, stage IV category respectively. Neuroimaging study showed abnormalities in 45.83% cases included periventricular white matter hyper intense signal changes, cortical atrophy and ischaemic change.
Conclusion: In our study most of the SSPE patient were in stage II.About half of the patient had history of measles infection during early childhood. Neuroimaging abnormalities found in about half of the cases and majority cases were in stage II. Common neuroimaging abnormalities were periventricular white matter hyper intense signal changes and cortical atrophy.
Agnarsdottir G. Subacute sclerosing panencephalitis. In: Waterson AP, editor. Recent Advances in Clinical Virology. New York: Churchill Livingstone; 1977. pp. 21–49.
Van Bogaert L. Uneleuco-encephalite sclerosantesubaigue. J Neurol Neurosurg Psychiatry 1945; 8: 101–20.
Campbell H, Andrews N, Brown KE, Miller E (2007) Review of the effect of measles vaccination on the epidemiology of SSPE. Int J Epidemiol 36: 1334–1348.
Saha V, John TJ, Mukundan P, Gnanamuthu C, Prabhakar S, Arjundas G, et al. High incidence of subacute sclerosing panencephalitis in south India. Epidemiol Infect. 1990; 104: 151–156.
Sidra K Jafri, Raman Kumar, Shahnaz H Ibrahim. Subacute sclerosing panencephalitis- current perspectives. Pediatric Health, Medicine and Therapeutics. 2018;9: 67-71.
Swaiman KF, Ashwal S, Ferriero DM, Schor NF, Finkel RS et al. Swaiman’s Pediatric Neurology Principles and Practice.6thed, Elsevier Edinburgh London New York Oxford Philadelphia St Louis Sydney Toronto 2018; 902-904.
Garg RK. Subacute sclerosing panencephalitis. Postgrad Med J 2002; 78: 63–70.
Halsey NA, Modlin JF, Jabbour JT, et al. Risk factors in subacute sclerosing panencephalitis: a case-control study. Am J Epidemiol 1980; 111: 415–24.
Miller C, Farrington CP, Harbert K. The epidemiology of subacute sclerosing panencephalitis in England and Wales 1970–1989.Int J Epidemiol 1992; 21: 998–1006.
Bhat AR, Nair MD, Sarada C, Radha krishnan K. Subacute sclerosing panencephalitis: Experience of a tertiary referral centre in Thiruvanthapuram, Kerala. Neurol India.1996; 44: 6–9.
Modlin JR, Halsey NA, Eddins DL, et al. Epidemiology of subacute sclerosing panencephalitis. J Pediatr 1979; 94: 231–6.
Prashanth LK, Taly AB, Ravi V, Sinha S, Arunodaya GR. Adult onset subacute sclerosing panencephalitis: Clinical profile of 39 patients from a tertiary care centre. J NeurolNeurosurg Psychiatry, 2006; 77: 630–3.
Green field JG. Encephalitis and encephalomyelitis in England and Wales during last decade. Brain 1950; 73: 141–66.
Bellini WJ, Rota JS, Lowe LE, Katz RS, Dyken PR, et al. (2005) Subacute sclerosing panencephalitis: more cases of this fatal disease are prevented by measles immunization than was previously recognized. J Infect Dis 192: 1686–1693.
Weissbrich B, Schneider-Schaulies J, terMeulen V (2003) Measles and its neurological complications. In: Nath A, Berger JR, editors. Clinical Neurovirology. New York: Dekker. 401–430.
Campbell C, Levin S, Humphreys P, Walop W, Brannan R (2005) Subacute sclerosing panencephalitis: results of the Canadian Paediatric Surveillance Program and review of the literature. BMC Pediatr 5: 47.
Rima BK, Duprex WP (2005) Molecular mechanisms of measles virus persistence. Virus Res 111: 132–147.
Garg RK (2008) Subacute sclerosing panencephalitis. J Neurol 255: 1861–1871.
Gutierrez J, Issacson RS, Koppel BS (2010) Subacute sclerosing panencephalitis:an update. Dev Med Child Neurol 52: 901–907.)
Sonia M, Lalit D, Shobha B, Sheffali G, Amandeep S, Veena K, et al. Subacute sclerosing panencephalitis in a tertiary care centre in post measles vaccination era. J Commun Dis. 2009; 41: 161–7.
Vaidya SR, Wairagkar NS, Raja D, Khedekar DD, Gunasekaran P, Shankar S, et al. First detection of measles genotype D7 from India.Virus Genes 2008; 36: 31-4.
Cece H, Tokay L, Yildiz S, Karakas O, Karakas E, Iscan A. Epidemiological findings and clinical and magnetic resonance presentations in subacute sclerosing panencephalitis. J Int Med Res 2011; 39: 594-602.
Rafique A, Amjad N, Chand P, Zaidi SHZ, Rana MS, et al.Clinical and Demographic Characteristics Journal Subacute Sclerosing Panencephalitis: Journal of the College of Physicians and Surgeons Pakistan 2014, Vol. 24 (8): 557-560.
Brismar J, Gascon GG, Steyern KVV, Bohlega S. Subacute Sclerosing Panencephalitis: Evaluation with CT and MRAJNR Am J NeuroradiolApril 1996; 17:761–772.