CEM PAKETÇI, Pinar Edem, DIDEM SOYDEMIR, Gamze UZAN, Çağatay Günay, AYŞE SEMRA HIZ, uluç yiş

Pediatrik Guillain Barre Sendromu: Klinik, Elektrofizyolojik ve Prognostik Özellikler

Amaç: Bu çalışmada Guillain Barre Sendromu tanısı ile izlenen hastaların klinik, elektrofizyolojik, laboratuvar ve radyolojik bulgularının ve izlem sonuçlarının araştırılması amaçlanmıştır. Gereç ve Yöntem: 2011-2019 yılları arasında kliniğimizde Guillain Barre Sendromu tanısı almış ve en az bir yıl süre ile izlenmiş 29 hasta değerlendirildi. Hastaların demografik, klinik, laboratuvar, radyolojik ve elektrofizyolojik bulguları, tedavi ve izlem sonuçları retrospektif olarak incelendi. Hastaların fonksiyonel değerlendirmesinde Hughes skalası kullanıldı. Bulgular: Hastaların 18’i erkekti (erkek/kadın oranı: 1,64). Yaş aralığı 1,4-16,4 (ortanca 9,1) yıldı. Hastaların 21’inde (%72,4) enfeksiyon öyküsü vardı. Başvuru sırasında 26 hastanın (%89,7) derin tendon refleksleri alınamıyor veya azalmıştı. Elektrofizyolojik özelliklerine göre sekiz hasta demiyelinizan, on bir hasta akut motor aksonal, dört hasta akut sensorimotor aksonal, altı hasta Miller Fisher sendromu idi. Tüm hastalar intravenöz immünglobulin tedavisi almış, bir hastada ek olarak plazmaferez uygulanmıştı. İzlemde sekiz hastada sekelli iyileşme görülmüş, iki hastada ise hastalık tekrarlamıştı. Sonuç: Gelişmekte olan ülkelerde daha sık olarak bildirilen aksonal formlar bizim hasta grubumuzda da daha fazlaydı. Derin tendon reflekslerinin normal ya da artmış olarak bulunmasının akut flask güçsüzlük ile gelen hastalarda Guillain Barre Sendromu tanısının dışlanması için yeterli olmadığı, özellikle aksonal formların düşünülebileceği kanısına varılmıştır. Çocuklarda fonksiyonel iyileşme sık olsa da uzun dönemde kronik şikâyetlerin devam edebileceği ve rekürrensler olabileceği bilinmeli, hastalar dikkatle izlenmeye devam edilmelidir.

Anahtar Kelimeler:

anormal derin tendon refleksi, çocuk, Guillain-Barré Sendromu

Pediatric Guillain Barre syndrome: clinical, electrophysiological and prognostic features

Objective: In this study, it was aimed to investigate the clinical, electrophysiological, laboratory and radiological findings and outcomes of patients who were followed up with the diagnosis of Guillain Barre Syndrome. Materials and Methods: Twenty-nine patients diagnosed with Guillain Barre Syndrome and followed for at least one year, between 2011 and 2019 were enrolled. The clinical, laboratory and electrophysiological findings, treatment and follow-up results were analyzed retrospectively. Hughes scale was used for the functional evaluation of the patients. Results: Eighteen of the patients were male (male/female ratio: 1.64). The age range was 1.4-16.4 (median 9.1) years. Preceding infection was found in 21 patients (72.4%). At the time of admission, 26 patients’ (89.7%) deep tendon reflexes were not elicited or decreased. According to their electrophysiological characteristics, eight patients were demyelinating, eleven patients were acute motor axonal, four patients were acute sensorimotor axonal and six patients were Miller Fisher syndrome. All patients received intravenous immunoglobulin therapy and one patient needed plasmapheresis as well. Eight patients recovered with sequelae during the follow-up, and two patients had recurrence. Conclusion: Axonal forms, which are reported more frequently in developing countries, were also higher in our patient group. It was concluded that the presence of deep tendon reflexes as normal or increased is not sufficient to exclude the diagnosis of Guillain Barre Syndrome, and especially axonal forms can be considered in patients with acute flask weakness. Although functional improvement is common in children, it should be known that chronic complaints and recurrences may occur in the long term.

Keywords:

abnormal deep tendon reflex, child, Guillain-Barré Syndrome, prognosis,

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