Epileptiskā encefalopātija bērniem ar CSWS sindromu Latvijā
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Date
2021
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Rīgas Stradiņa universitāte
Rīga Stradiņš University
Rīga Stradiņš University
Abstract
Darba mērķis: veidot klīniskās, elektroencefalografiskās un radioloģiskās ainas raksturojumu bērniem ar eCSWS Bērnu klīniskajā universitātes slimnīcā (BKUS).
Materiāli un metodes: retrospektīvs pētījums, kurā tika iekļauti un ar IBM SPSS Statistics 26,0 analizēti dati par 4-12 gadīgiem eCSWS pacientiem Bērnu klīniskajā universitātes slimnīcā (BKUS) no 2017. līdz 2020. gadam.
Rezultāti: pētījumā tika izmantotas 78 pacientu klīniskās vēstures (35,9% sievietes). Vidējais vecums 7,9 gadi (4,0 - 12,0; SD 2,1).
Klīniski novērotās izpausmes bija krampji (88,5%), valodas attīstības aizture (55,1%) , uzvedības traucējumi (37,2%), mācīšanas iemaņu traucējumi (25,6%), intelektuālās attīstības aizture (23,1%), bērnības autisms (15,4%) un citi jaukti attīstības traucējumi (10,3%).
Magnētiskās rezonanses izmeklējums bija pieejams tikai 71,8%, no kā 32,1% gadījumos novērota pozitīva atrade.
Lielāka daļa no pacientiem saņēma specifisko medikamentozo terapiju (85,9%) – 61,2% lietojuši vienu medikamentu monoterapijā, 38,8% lietojuši divus un vairāk.
Kā indikators terapijas efektivitātei, pīķu viļņu indekss divās sekojošās elektroencefalogrammās bija pieejams 69,2% pacientu. Līdz ar terapiju pīķu viļņu indekss palielinājas 18,5%, nemainījas 72,2% un samazinājas 9,3% pacientu.
Starp klīniskām pazīmēm, izmantotām diagnostiskajām pieejām un terapijas iznākumiem statistiski nozīmīga atšķirība netika konstatēta.
Secinājumi: klīniski eCSWS ir heterogēns sindroms, kuram raksturīgi smagi deficīti. Diagnostiskai pieejai ir nepieciešama uzlabošana, balstoties uz nepietiekamu magnētiskās rezonanses izmeklējumu un atkārtotu elektroencefalogrammu skaitu. Mazs pozitīvas dinamikas procents pēc elektroencefalogrammas datiem liek aizdomāties arī par terapeitīskās pieejas papildinājumu un pielāgošanu.
Aim of the study: to analyze characteristics of eCSWS in Latvian patients. Materials and Methods: a retrospective study of medical records of CSWS patients in Children’s Clinical University Hospital from 2017 to 2020. Data was analysed using IBM SPSS Statistics 26,0. Results: final sample consisted of medical records of 78 patients (35,9% female). Average age was 7,9 years (4,0-12,0; SD 2,1). Clinical presentations were as follows: seizures (88,5%), delay in language development (55,1%), behavioral disorders (37,2%), learning disabilities (25,6%), intellectual disability (23,1%), childhood autism (15,4%), and mixed developmental disorder (10,3%). Magnetic resonance imaging was done only in 71,8% of cases with positive findings in 32.1% of investigations. Most patients received specific treatment (85,9%) – 61,2% had tried one, 38,8% had used two or more medications. As an indicator of therapeutical success, spike-wave index in two consequent electroencephalograms was available for 69,2% of patients. Spike-wave index increased in 18,5%, remained unchanged in 72,2% and decreased in 9,3% of cases. No statistically significant differences between clinical presentation, results of investigations and therapeutical outcome were found. Conclusions: clinical presentation of eCSWS is heterogeneous, including severe disability. Diagnostic approach is in need of improvement based on insufficient amount of performed investigations. The small proportion of patients with positive electrographic dynamics shows the necessity for improvements in therapeutic approach.
Aim of the study: to analyze characteristics of eCSWS in Latvian patients. Materials and Methods: a retrospective study of medical records of CSWS patients in Children’s Clinical University Hospital from 2017 to 2020. Data was analysed using IBM SPSS Statistics 26,0. Results: final sample consisted of medical records of 78 patients (35,9% female). Average age was 7,9 years (4,0-12,0; SD 2,1). Clinical presentations were as follows: seizures (88,5%), delay in language development (55,1%), behavioral disorders (37,2%), learning disabilities (25,6%), intellectual disability (23,1%), childhood autism (15,4%), and mixed developmental disorder (10,3%). Magnetic resonance imaging was done only in 71,8% of cases with positive findings in 32.1% of investigations. Most patients received specific treatment (85,9%) – 61,2% had tried one, 38,8% had used two or more medications. As an indicator of therapeutical success, spike-wave index in two consequent electroencephalograms was available for 69,2% of patients. Spike-wave index increased in 18,5%, remained unchanged in 72,2% and decreased in 9,3% of cases. No statistically significant differences between clinical presentation, results of investigations and therapeutical outcome were found. Conclusions: clinical presentation of eCSWS is heterogeneous, including severe disability. Diagnostic approach is in need of improvement based on insufficient amount of performed investigations. The small proportion of patients with positive electrographic dynamics shows the necessity for improvements in therapeutic approach.
Description
Pediatrija
Pediatrics
Veselības aprūpe
Health Care
Pediatrics
Veselības aprūpe
Health Care
Keywords
CSWS, epileptiskā encefalopātija, ESES, elektriskais status epilepticus miega laikā, SWI, pīķu viļņu indekss, EEG, MRI, CSWS, epileptic encephalopathy, ESES, electrical status epilepticus during sleep, SWI, slow wave index, EEG, MRI