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Subacute Sclerosing Panencephalitis Presenting with Unilateral Periodic Myoclonic Jerks

Published online by Cambridge University Press:  02 December 2014

Zaitoon M. Shivji ,
Ibrahim S. Al-Zahrani ,
Yousef A. Al-Said  and
Mohammed M.S. Jan
Zaitoon M. Shivji
Affiliation:
Department of Neurosciences, King Faisal Specialist Hospital and Research Centre, Jeddah, Kingdom of Saudi Arabia
Ibrahim S. Al-Zahrani
Affiliation:
Department of Neurosciences, King Faisal Specialist Hospital and Research Centre, Jeddah, Kingdom of Saudi Arabia
Yousef A. Al-Said
Affiliation:
Department of Neurosciences, King Faisal Specialist Hospital and Research Centre, Jeddah, Kingdom of Saudi Arabia
Mohammed M.S. Jan
Affiliation:
Department of Neurosciences, King Faisal Specialist Hospital and Research Centre, Jeddah, Kingdom of Saudi Arabia
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Abstract

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Background:

Subacute sclerosing panencephalitis (SSPE) is a rare complication of measles virus infection. The disease is characterized by behavioural abnormalities, intellectual deterioration, motor weakness, and generalized myoclonic jerks progressing to coma and death in one to two years in 80% of the cases. The myoclonic jerks are associated with characteristic generalized slow periodic complexes on electroencephalography (EEG). The symptoms and signs of SSPE are frequently quite variable. The clinical course is equally variable and difficult to predict. The characteristic periodic myoclonus can rarely occur unilaterally particularly in the early stages of the disease. As well, the periodic EEG complexes have been reported unilaterally in up to 3% of cases.

Case Report:

A 12-year-old boy, who was seen at a later stage with atypical manifestation of myoclonic body jerks confined entirely unilaterally, combined with contralateral periodic EEG complexes. One could assume clinically that the more diseased hemisphere was responsible for generating the jerks. However, brain magnetic resonance imaging revealed asymmetric hemispheric changes suggesting that the less neurologically damaged hemisphere is responsible for generating the unilateral myoclonic jerks. This has led to the interpretation that the more severely damaged hemisphere has lost the neuronal connectivity required to generate these periodic myoclonic jerks.

Conclusion:

Subacute sclerosing panencephalitis may have asymmetric hemispheric involvement, not only early, but also in the advanced stages of the disease, which can result in unilateral periodic myoclonic jerks.

Résumé:

RÉSUMÉ: Introduction:

La panencéphalite sclérosante subaiguë (PESS) est une complication rare de l'infection par le virus de la rougeole. La maladie se caractérise par des anomalies du comportement, une détérioration intellectuelle, de la faiblesse musculaire et des secousses myocloniques généralisées progressant vers le coma et la mort en un ou deux ans chez 80% des cas. Les secousses myocloniques sont associées à des complexes périodiques lents généralisés à l'électroencéphalographie (ÉEG). Les symptômes et les signes de la PESS sont souvent variables. L'évolution clinique est également variable et difficile à prédire. Le myoclonus périodique caractéristique peut être unilatérale dans de rares cas, particulièrement dans les phases précoces de la maladie. Des complexes périodiques unilatéraux à l'ÉEG ont également été rapportés chez environ 3% des cas.

Observation:

Il s'agit d'un garçon de 12 ans chez qui on a observé des manifestations atypiques à un stade avancé de la maladie, soit des secousses myocloniques exclusivement unilatérales associées à des complexes périodiques contralatéraux à l'ÉEG. On pourrait présumer selon la clinique que l'hémisphère le plus touché était responsable des secousses. Cependant, l'IRM du cerveau a montré des changements hémisphériques asymétriques suggérant que l'hémisphère moins atteint était responsable des secousses myocloniques unilatérales, ce qui a donné lieu à l'interprétation suivante: l'hémisphère le plus sévèrement atteint a perdu la connectivité neuronale nécessaire pour générer ces secousses myocloniques périodiques.

Conclusions:

Il peut exister une atteinte hémisphérique asymétrique dans la panencéphalite sclérosante subaiguë en phase précoce de la maladie, mais aussi dans les phases avancées, ce qui peut donner lieu à des secousses myocloniques périodiques unilatérales.

Type
Case Report
Information
Canadian Journal of Neurological Sciences , Volume 30 , Issue 4 , November 2003 , pp. 384 - 387
Copyright
Copyright © The Canadian Journal of Neurological 2003

References

REFERENCES

1. Johnson, RT, Griffin, DE, Hirsch, RL, et al. Measles encephalo-myelitis – clinical and immunologic studies. New Eng J Med 1984;310: 137–141.Google Scholar
2. Dawson, JR. Cellular inclusions in cerebral lesions in lethargic encephalitis. Am J Pathol 1933;9:7–15.Google Scholar
3. Greenfield, JG. Encephalitis and encephalomyelitis in England and Wales during the last decade. Brain 1950;73:141–166.Google Scholar
4. Zilber, N, Kahana, E. Environmental risk factors for subacute sclerosing panencephalitis (SSPE). Acta Neurol Scand 1998; 98(1):49–54.Google Scholar
5. Cobb, W. The periodic events of subacute sclerosing leuko-encephalitis. Electroencephalogr Clin Neurophysiol 1966;21: 278–294.Google Scholar
6. Risk, WS, Haddad, FS. The variable natural history of subacute sclerosing panencephalitis: a study of 118 cases from the Middle East. Arch Neurol 1979; 36(10):610–614.Google Scholar
7. Sobczyk, W, Kulczycki, J. Sequence of clinical changes and evolution of MR images in a case of chronic SSPE. Neurol Neurochir Pol 1999;33(5):1165–1172.Google Scholar
8. Dimova, P, Bojinova, V. Subacute sclerosing panencephalitis with atypical onset: clinical, computed tomographic, and magnetic resonance imaging correlation. J Child Neurol 2000;15(4):258–260.Google Scholar
9. Markland, CN, Panszi, JG. The electroencephalogram in subacute sclerosing panencephalitis. Arch Neurol 1975;32:719–726.Google Scholar
10. Bohlega, S, Al-Kawi, MX. Subacute sclerosing panencephalitis: imaging and clinical correlation. J Neuroimaging 1994;4:71–76.Google Scholar
11. Bonifas-Galup, P, Hamano, K, Sebrosa, CJ, Plouin, P. Different clinical and electroencephalographic aspects of subacute sclerosing panencephalitis (SSPE). Apropos of 51 cases. Rev Electroencephalogr Neurophysiol Clin 1983;13(3):224–231.Google Scholar
12. Silva, DF, Lima, MM, Anghinah, R, Zanoteli, E, Lima, JG. Atypical clinical and electroencephalographic pattern in a patient with subacute sclerosing panencephalitis. Arq Neuropsiquiatr 1995;53(2):278–280.Google Scholar
13. Brimani, DJ, Sabeti, AA, Ghaffar-Pour, M, et al. Atypical aspects of the electroencephalogram in subacute sclerosing panencephalitis. Neurophysiol Clin 1990;20(2):95–103.Google Scholar
14. Gurses, C, Ozturk, A, Baykan, B, et al. Correlation between clinical stages and EEG findings of subacute sclerosing panencephalitis. Clin Electroencephalogr 2000;31(4):201–206.Google Scholar
15. Dogulu, CF, Ciger, A, Saygi, S, Renda, Y, Yalaz, K. Atypical EEG findings in subacute sclerosing panencephalitis. Clin Electroencephalogr 1995;26(4):193–199.Google Scholar
16. Ghanem, Q. Unilateral subacute sclerosing panencephalitis complexes with contralateral myoclonus. Acta Neurol Scand 1981;63(1):76–80.Google Scholar