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Subacute Sclerosing Panencephalitis Presenting with Unilateral Periodic Myoclonic Jerks

  • Zaitoon M. Shivji (a1), Ibrahim S. Al-Zahrani (a1), Yousef A. Al-Said (a1) and Mohammed M.S. Jan (a1)

Abstract

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: 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.

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References

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Subacute Sclerosing Panencephalitis Presenting with Unilateral Periodic Myoclonic Jerks

  • Zaitoon M. Shivji (a1), Ibrahim S. Al-Zahrani (a1), Yousef A. Al-Said (a1) and Mohammed M.S. Jan (a1)

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