DOI: http://dx.doi.org/10.18203/2320-6012.ijrms20203480

Subacute sclerosing panencephalitis: case based review

Raman Singh, Zubin A. Mahajan, Sameera R. Mehta, Jayesh Ranchhod, Soham J. Nadkarni

Abstract


Subacute sclerosing panencephalitis (SSPE) is a chronic debilitating condition that occurs in children affected with measles. SSPE is broadly distinguished as typical SSPE, the more rampant form, occurring over a period of years following primary measles infection, while the atypical has a more rapidly progressive course over weeks to months. SSPE can present with cognitive, epileptic, autonomic, pyramidal and ophthalmologic manifestations with scholastic decline being the primary feature. The management of SSPE focuses on improvement of quality of life and prolongation of survival which can be achieved with the use of supportive care modalities and immunomodulators respectively. This is a comprehensive review which discusses several parameters of SSPE such as epidemiology, pathophysiology, clinical presentations, and detailed management protocol for this condition. As part of this review, we also discuss a case of rapidly progressive, fulminant and atypical SSPE in a five-year-old male presenting clinically with myoclonic jerks of lower extremities.


Keywords


Isoprinosine, Measles virus, Myoclonic jerks, Subacute sclerosing panencephalitis

Full Text:

PDF

References


Bellini WJ, Rota JS, Lowe LE, Katz RS, Dyken PR, Zaki SR, et al. Subacute sclerosing panencephalitis: more cases of this fatal disease are prevented by measles immunization than was previously recognized. J Infectious Disea. 2005 Nov 15;192(10):1686-93.

Garg RK. Subacute sclerosing panencephalitis. Postgraduate Med J. 2002 Feb 1; 78(916):63-70.

Griffin DE, Ward BJ, Esolen LM. Pathogenesis of measles virus infection: an hypothesis for altered immune responses. J Infectious Disea. 1994 Nov 1;170(Supplement_1):S24-31.

Jain RS, Sannegowda RB, Srivastava T, Jain R, Mathur T, Gandhi P. A rare presentation of subacute sclerosing panencephalitis with acute fulminant course and atypical radiological features. Ann Indian Acad Neurol. 2013 Oct 1;16(4):732.

Gökçil Z, Odabaşi Z, Aksu A, Vural O, Yardim M. Acute fulminant SSPE: clinical and EEG features. Clinical Electroencephalography. 1998 Jan;29(1):43-8.

World Health Organization. Weekly epidemiological record. No. 2, 2006, 81,13-20. Geneva, Switzerland. Available at: http://www.who.int/vaccine_safety/committee/reports/wer8102.pdf?ua=1 Accessed on 1 May 2020.

Alkan A, Korkmaz L, Sigirci A, Kutlu R, Yakinci C, Erdem G, et al. Subacute sclerosing panencephalitis: Relationship between clinical stage and diffusion‐weighted imaging findings. J Magn Resonance Imaging: J Intern Society for Magnetic Resonance Med. 2006 Mar;23(3):267-72.

Ko SB, Ortega-Gutierrez S, Choi HA, Claassen J, Presciutti M, Schmidt JM, et al. Status epilepticus–induced hyperemia and brain tissue hypoxia after cardiac arrest. Archives of neurology. 2011 Oct 1;68(10):1323-6.

Beersma MF, Galama JM, Van Druten HA, Renier WO, Lucas CJ, Kapsenberg JG. Subacute Sclerosing Panencephalitis In The Netherlands—1976–1990. Intern J Epidemiol. 1992 Jun 1;21(3):583-8.

Jabbour JT, Duenas DA, Sever JL, Krebs HM, Horta-Barbosa L. Epidemiology of subacute sclerosing panencephalitis (SSPE): a report of the SSPE registry. JAMA. 1972 May 15;220(7):959-62.

Chiu MH, Meatherall B, Nikolic A, Cannon K, Fonseca K, Joseph JT, et al. Subacute sclerosing panencephalitis in pregnancy. The Lancet Infectious Dis. 2016 Mar 1;16(3):366-75.

Oyanagi S, Rorke LB, Katz M, Koprowski H. Histopathology and electron microscopy of three cases of subacute sclerosing panencephalitis (SSPE). Acta neuropathologica. 1971 Mar 1;18(1):58-73.

Ohya T, Martinez AJ, Jabbour JT, Lemmi H, Duenas DA. Subacute sclerosing panencephalitis: correlation of clinical, neurophysiologic and neuropathologic findings. Neurology. 1974 Mar 1;24(3):211-18.

Iked K, Akiyama H, Kondo H, Arai T, Arai N, Yagishita S. Numerous glial fibrillary tangles in oligodendroglia in cases of subacute sclerosing panencephalitis with neurofibrillary tangles. Neuroscience letters. 1995 Jul 14;194(1-2):133-35.

Lewandowska E, Szpak GM, Lechowicz W, Pasennik E, Sobczyk W. Ultrastructural changes in neuronal and glial cells in subacute sclerosing panencephalitis: correlation with disease duration. Folia neuropathologica. 2001;39(3):193-202.

Khadilkar SV, Patil SG, Kulkarni KS. A study of SSPE: early clinical features. J Pediatr Neurol. 2004 Jan 1;2(2):73-7.

Gadoth N. Subacute sclerosing panencephalitis (SSPE) the story of a vanishing disease. Brain and Development. 2012 Oct 1;34(9):705-11.

Eroglu E, Gokcil Z, Bek S, Ulas UH, Ozdag MF, Odabasi Z. Long-term follow-up of patients with adult-onset subacute sclerosing panencephalitis. J Neurological Sciences. 2008 Dec 15;275(1-2):113-16.

Cole AJ, Henson JW, Roehrl MH, Frosch MP. Case 24-2007: A 20-Year-Old Pregnant Woman with Altered Mental Status. New England J Med. 2007 Aug 9;357(6):589-600.

Dimova PS, Bojinova VS. Case of subacute sclerosing panencephalitis with atypical absences and myoclonic-atonic seizures as a first symptom. J Child Neurol. 2004 Jul;19(7):548-52.

Babu RB, Biswas J. Bilateral macular retinitis as the presenting feature of subacute sclerosing panencephalitis. J Neuro-Ophthalmol. 2007 Dec 1;27(4):288-91.

Agarwal A, Singh R, Kumar A, Dogra MR, Gupta A. Bilateral subretinal fluid and retinal vasculopathy associated with subacute sclerosing panencephalitis. Retinal Cases and Brief Reports. 2017 Apr 1;11(2):145-7.

Baillif S, Tieulie N, Queyrel V, Cornut PL, Gastaud P. New aspects of viral necrotizing retinitis in subacute sclerosing panencephalitis with spectral-domain optical coherence tomography. Retinal Cases and Brief Reports. 2012 Jul 1;6(3):235-41.

Risk WS, Haddad FS. The variable natural history of subacute sclerosing panencephalitis: a study of 118 cases from the Middle East. Arch Neurol. 1979 Oct 1;36(10):610-4.

Gutierrez J, Issacson RS, Koppel BS. Subacute sclerosing panencephalitis: an update. Developmental Med Child Neurol. 2010 Oct;52(10):901-7.

Ekmekci Ö, Karasoy H, Gökçay A, Ülkü A. Atypical EEG findings in subacute sclerosing panencephalitis. Clinical Neurophysiol. 2005 Aug 1;116(8):1762-7.

Dyken PR. Subacute sclerosing panencephalitis: current status. Neurologic Clinics. 1985 Feb 1;3(1):179-96.

Anlar B, Yalaz K, Ustacelebi S. Symptoms and clinical signs, laboratory data in 80 cases of subacute sclerosing panencephalitis. Revue Neurologique. 1988;144(12):829-32.

Owens GP, Gilden D, Burgoon MP, Yu X, Bennett JL. Viruses and multiple sclerosis. The Neuroscientist. 2011 Dec;17(6):659-76.

Jayakumar PN, Taly AB, Arya BY, Nagaraj D. Computed tomography in subacute sclerosing panencephalitis: report of 15 cases. Acta neurologicascandinavica. 1988 Apr;77(4):328-30.

Praveen-Kumar S, Sinha S, Taly AB, Jayasree S, Ravi V, Vijayan J, et al. Electroencephalographic and imaging profile in a subacute sclerosing panencephalitis (SSPE) cohort: a correlative study. Clinical neurophysiology. 2007 Sep 1;118(9):1947-54.

Jones C, Huttenlocher P, Dyken P, Jabbour JT, Maxwell K. Inosiplex therapy in subacute sclerosing panencephalitis: a multicentre, non-randomised study in 98 patients. Lancet. 1982 May 8;319(8280):1034-7.

Dyken PR, Swift A, Durant RH. Long‐term follow‐up of patients with subacute sclerosing panencephalitis treated with inosiplex. Ann Neurol: Official J Am Neurolog Association Child Neurol Soc. 1982 Apr;11(4):359-64.

Aydin ÖF, Şenbil N, Kuyucu N, Gürer YY. Combined treatment with subcutaneous interferon-α, oral isoprinosine, and lamivudine for subacute sclerosing panencephalitis. J Child Neurol. 2003 Feb;18(2):104-8.

Jovic NJ. Epilepsy in children with subacute sclerosing panencephalitis. SrpArhCelok Lek 2013;141:434-40.

Watanabe M, Hashimoto K, Abe Y, Kodama EN, Nabika R, Oishi S, et al. A novel peptide derived from the fusion protein heptad repeat inhibits replication of subacute sclerosing panencephalitis virus in vitro and in vivo. PloS one. 2016;11(9).

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 Jun;24(6):763-7.

Maalouf M, Rho JM, Mattson MP. The neuroprotective properties of calorie restriction, the ketogenic diet, and ketone bodies. Brain Research Reviews. 2009 Mar 1;59(2):293-315.

Yaqub BA. Subacute sclerosing panencephalitis (SSPE): early diagnosis, prognostic factors and natural history. J Neurological Sci. 1996 Aug 1;139(2):227-34.

Risk WS. Subacute sclerosing panencephalitis with remission in a Bosnian refugee child. Pediatric Infectious Dis J. 2003 Aug;22(8):757-8.

Risk WS, Haddad FS. The variable natural history of subacute sclerosing panencephalitis: a study of 118 cases from the Middle East. Arch Neurol. 1979 Oct 1;36(10):610-4.

Prashanth LK, Taly AB, Ravi V, Sinha S, Rao S. Long term survival in subacute sclerosing panencephalitis: an enigma. Brain and Development. 2006 Aug 1;28(7):447-52.

Douglas-Escobar M, Weiss MD. Hypoxic-ischemic encephalopathy: a review for the clinician. JAMA Pediatr. 2015 Apr;169(4):397-403.

Van Laerhoven H, de Haan TR, Offringa M, Post B, van der Lee JH. Prognostic tests in term neonates with hypoxic-ischemic encephalopathy: a systematic review. Pediatrics. 2013 Jan 1;131(1):88-98.

Thoresen M, Hellström-Westas L, Liu X, de Vries LS. Effect of hypothermia on amplitude-integrated electroencephalogram in infants with asphyxia. Pediatrics. 2010 Jul 1;126(1):e131-9.

Morita K, Nabeshima T, Buerano CC. Japanese encephalitis. Rev Sci Tech. 2015 Aug 1;34(2):441-52.

Dung NM, Turtle L, Chong WK, Mai NT, Thao TT, Thuy TT, et al. An evaluation of the usefulness of neuroimaging for the diagnosis of Japanese encephalitis. J Neurol. 2009 Dec 1;256(12):2052.

Newman MP, Blum S, Wong RC, Scott JG, Prain K, Wilson RJ, et al. Autoimmune encephalitis. Internal Medicine Journal. 2016 Feb;46(2):148-57.

Lakshmi V, Malathy Y, Rao RR. Serodiagnosis of subacute sclerosing panencephalitis by enzyme linked immunosorbent assay. Indian J Pediatr. 1993 Jan 1;60(1):37-41.