Subacute Sclerosing Panencephalltis: Clinical and Laboratory Manifestations

  • Hardiono D. Pusponegoro Department of Child Health, Universitas Indonesia Medical School/Dr. Cipto Mangunkusumo Hospital, Jakarta
  • Jimmy Passat Department of Child Health, Universitas Indonesia Medical School/Dr. Cipto Mangunkusumo Hospital, Jakarta
  • M. Hardjono Abdoerachman Department of Child Health, Universitas Indonesia Medical School/Dr. Cipto Mangunkusumo Hospital, Jakarta
Keywords: subacute sclerosing panencephalitis; SSPE; mental changes; myoclonus; frequent falls

Abstract

We reviewed clinical and laboratory findings of 12 cases of Subacute sclerosing panencephalitis (SSPE) hospitalized at our department from 1985 to 1991. All cases were diagnosed and hospitalized at the 2nd stage. The principal clinical manifestations were mental changes, myoclonus, and frequent falls. Other clinical manifestations were ocular changes, involuntary movements, loss of social contact, and spasticity. Diagnosis was based on suppression-burst pattern in EEG and positive antibody titer to measles in serum and cerebrospinal fluid. CT scan was not diagnotic, since it was either normal or showed only non-specific cortical atrophy. Eleven patients (91, 7%) recalled a history of measles in the past. Age of onset of SSPE varied among cases and was difficult to specify precisely due to its subtle nature. None of the cases had been vaccinated against measles.

SSPE is a rare disease, but is almost always fatal with prolonged suffering of the patient. Based on our experience with SSPE patients, we recommend to broaden the immunization programme against measles.

References

1. Connolly JH, Allen IV, Hurtwith LJ et al. Measles virus antibody and antigen in SSP E. Lancet 1967;i:542-4.
2. Jabbour JT, Duenas DA, Sever JL. Epidemiology of sub acute sclerosing panencephalitis (SSPE). J Am med Ass 1972; 220: 959-62.
3. Dyken PR. Subacute Sclerosing Panencephalitis. Neurol. Clin North Amer 1985; 3: 179-96.
4. Bouteille M, Fontaine C, Vendenne C, et al. Sur un cas d'encephalite subarque a inclusions Etude anatomoclinique et Ultrastrucale. Rev. neurol. 1965; 113: 454. Cited from: Bell WE, Me Cormick WEeds. Neurologic infections in children. 2nd ed. Philadelphia: Saunders, 19… : 417-91.
5. Chen TT, Watanabe I, Zeman W, et al. Subacute sclerosing panencephalitis; propagation of measles virus from brain nucleocapsids in cells cultured from brain. Nature (London) 1969; 222:888-90
6. Payne FE, Baublis JV, ltabashi HH. Isolation of measles virus from cell cultures of brain from a patient with subacute sclerosing panencephalitis ..... 1969: 585-89.
7. Abdoerrachman Mh. Measles in pediatric Ward, RSCM, Jakarta Presented at KONIKA V, Medan, 1981.
8. Passat J, Pusponegoro HD, Soetomenggolo TS, et al. Subacute sclerosing panencephalitis. Manifestasi klinis dan laboratoris. Presented at KONIKA Vlll, Ujung Pandang, 1990.
9. Modlin JF, Halsey NA, Eddins DL, et al. Epidemiology of subacute sclerosing panencephalitis. J Pediat 1979; 94: 231-6.
10. Modlin JF, Jabbour JT, Witte JJ, et al. Epidemiologic studies of measles, measles vaccine, and sub acute sclerosing panencephalitis. Pediatric 1977; 59: 505-12.
11. Ohya Y, Martinez Aj ,Jabbour JT, et al. Subacute sclerosing panencephalitis. Correlation of clinical, Neurophysiologic and neuropathologic findings. neurologiy, Minneap. 1974; 24:211-8.
12. Markand ON, Panszi JG, The electroencephalogram in subacute sclerosing panencephalitis. Arch Neurol. 1975; 32: 719-26. Dikutip dari Bell WE. Me Cormick WE. Neurologic infections in children. 2 nd ed. Philadelphia:Saunders 1983:417.
13. Jabbour jT_. Slow virus diseases; in Swaiman and Wright, The practice of pediatric neurologiy. vol 2, 2nd ed. London: Mosby 1982:717
14. Adels BR, Gajdusek DC, Gibs CJ, et al. Attempts to transmit subacute sclerosing panencephalitis and isolate a measles related agent, with a study of the immune response in patients and experimental animals. Neurology 1968; 18:30-51
15. Jabbour JT, Garcia JI-1, Lemmi H, et al. Subacute Sclerosing Panencephalitis. A Multidisciplinary Study of Eight Cases. J Am Med Assoc. 1969; 24:2248-54.
16. Horta-Barbosa I, Krebs J-1, Ley A, e l al. Progressive increase in cerebrospinal fluid measles antibody levels in subacute sclerosing panencephalitis Pediatrics 1971; 47:782-3.
17. Bove KE, Shelburne SA. Subacute inclusion body encephalitis associated with myopathy. Arch Neurol 1972; 27: 42-4.
Published
2019-01-29
How to Cite
1.
Pusponegoro H, Passat J, Abdoerachman M. Subacute Sclerosing Panencephalltis: Clinical and Laboratory Manifestations. PI [Internet]. 29Jan.2019 [cited 26Apr.2024];32(5-6):107-7. Available from: https://paediatricaindonesiana.org/index.php/paediatrica-indonesiana/article/view/2106
Section
Pediatric Neurology
Received 2019-01-29
Published 2019-01-29