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Case Reports
. 2017:2017:5483543.
doi: 10.1155/2017/5483543. Epub 2017 Jan 21.

Central Nervous System Involvement in Henoch-Schonlein Purpura in Children and Adolescents

Iliyana H Pacheva  1 , Ivan S Ivanov  1 , Krastina Stefanova  1 , Elena Chepisheva  2 , Lyubov Chochkova  1 , Dafina Grozeva  1 , Angelina Stoyanova  1 , Stojan Milenkov  1 , Penka Stefanova  2 , Anna Petrova  3
Affiliations
Case Reports

Central Nervous System Involvement in Henoch-Schonlein Purpura in Children and Adolescents

Iliyana H Pacheva et al. Case Rep Pediatr. 2017.

Abstract

Central nervous system (CNS) involvement in Henoch-Schonlein purpura (HSP) is rare but poses diagnostic difficulties. The aim of the study was to establish the frequency of CNS involvement in HSP, to analyze its clinical characteristics and do a literature review. Medical files of patients with HSP admitted at the Department of Pediatrics, Plovdiv, were studied retrospectively for a five-year period (2009-2013). Diagnosis was based on the American College of Rheumatology criteria. Out of 112 children with HSP 1 case (0.9%) had CNS involvement presenting as Posterior Reversible Encephalopathy Syndrome (PRES), which may be a result of CNS vasculitis or arterial hypertension. It was an 8-year-old girl with atypical HSP which started with abdominal pain requiring surgery. On the third day after the operation a transient macular rash and arterial hypertension appeared, followed by visual disturbances, hemiconvulsive epileptic seizures, postictal hemiparesis, and confusion. Head CT showed occipital hypodense lesions and MRT-T2 hyperintense lesion in the left occipital lobe. The patient experienced a second similar episode after 2 weeks when palpable purpura had also appeared. Neurological symptoms and MRI resolved completely. HSP can be an etiological factor for PRES in childhood. Although PRES is a rare complication of HSP, clinicians must be aware of it and avoid diagnostic and therapeutic delays.

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Conflict of interest statement

The authors declare no conflict of interests.

Figures

Figure 1
Figure 1
Distribution of patients by age and sex.
Figure 2
Figure 2
Head CT after the first episode with neurological symptoms: subcortical parietooccipital, parasagittal hypodense lesions in the white matter bilaterally but more on the left.
Figure 3
Figure 3
Cerebral МRI: hyperintense in Т2 and FLAIR lesion in the left occipital area.
See this image and copyright information in PMC

References

    1. Gedalia A. Henoch-Schönlein purpura. Current Rheumatology Reports. 2004;6(3):195–202. doi: 10.1007/s11926-004-0068-2. - DOI - PubMed
    1. Nielsen H. E. Epidemiology of Schönlein-Henoch purpura. Acta Paediatrica. 1988;77(1):125–131. doi: 10.1111/j.1651-2227.1988.tb10610.x. - DOI - PubMed
    1. Saulsbury F. T. Henoch-Schonlein purpura in children: report of 100 patients and review of the literature. Medicine. 1999;78(6):395–409. doi: 10.1097/00005792-199911000-00005. - DOI - PubMed
    1. Calviño M. C., Llorca J., García-Porrúa C., Fernández-Iglesias J. L., Rodriguez-Ledo P., González-Gay M. A. Henoch-Schönlein purpura in children from northwestern Spain: a 20-year epidemiologic and clinical study. Medicine. 2001;80(5):279–290. doi: 10.1097/00005792-200109000-00001. - DOI - PubMed
    1. Bérubé M. D., Blais N., Lanthier S. Neurologic manifestations of Henoch-Schönlein purpura. Handbook of Clinical Neurology. 2014;120:1101–1111. doi: 10.1016/B978-0-7020-4087-0.00074-7. - DOI - PubMed

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