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Challenges in diagnosing SSPE

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Abstract

Purpose

The typical clinical presentation of subacute sclerosing panencephalitis (SSPE) includes behavioral and intellectual changes followed by myoclonia. However, there are a considerable number of SSPE cases which present with distinct clinical features that can lead to a diagnostic difficulty. In this report, we summarize the clinical features of patients with SSPE who have uncommon presentations or features of the disease or coexisting medical conditions which may lead to diagnostic difficulties.

Methods

We studied 173 patients, all under the age of 17. Patients were included in the study group according to following criteria: onset of the disease before age 2 years, seizures occurring before the onset of myoclonia and/or behavioral symptoms, extrapyramidal or cerebellar signs and ocular manifestations as initial presenting symptoms, fulminant course including coma or death within 6 months. Additionally, patients with onset of SSPE at the setting of a known neurological disorder are defined as another group in the study.

Results

Out of 173 patients with SSPE who were followed in two neurology centers, 31 (17.9%) met our criteria.

Conclusions

We found a relative high frequency of these clinical features. Our findings suggest that clinicians should be aware of this clinical characteristics and rule out the disease in cases were other common causes have been excluded, especially in countries with insufficient measles immunization

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Conflict of interest

The authors declare that they have no conflict of interest.

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Authors and Affiliations

  1. Department of Neurology, Cerrahpasa Faculty of Medicine, Istanbul University, 34098, Fatih, Istanbul, Turkey

    Ozdem Erturk, Veysi Demirbilek, Cengiz Yalcinkaya & Aysin Dervent

  2. Department of Neurology, Tam Med Hospital, Gaziantep, Turkey

    Beyhan Karslıgil

  3. Department of Neurology, Haseki Hospital, Istanbul, Turkey

    Ozlem Cokar

  4. Department of Neurology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey

    Zuhal Yapıcı, Candan Gurses & Aysen Gokyigit

  5. Department of Physiology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey

    Guher Saruhan Direskeneli & Sibel Yentur

  6. Department of Public Health, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey

    Emel Onal

  7. Department of Microbiology, Faculty of Medicine, Yeditepe University, Istanbul, Turkey

    Gulden Yılmaz

Authors
  1. Ozdem Erturk
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  2. Beyhan Karslıgil
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  3. Ozlem Cokar
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  4. Zuhal Yapıcı
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  5. Veysi Demirbilek
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  6. Candan Gurses
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  7. Cengiz Yalcinkaya
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  8. Aysen Gokyigit
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  9. Guher Saruhan Direskeneli
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  10. Sibel Yentur
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  11. Emel Onal
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  12. Gulden Yılmaz
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  13. Aysin Dervent
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Corresponding author

Correspondence to Veysi Demirbilek.

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Erturk, O., Karslıgil, B., Cokar, O. et al. Challenges in diagnosing SSPE. Childs Nerv Syst 27, 2041–2044 (2011). https://doi.org/10.1007/s00381-011-1603-x

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  • DOI: https://doi.org/10.1007/s00381-011-1603-x

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