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Yıl:2016; Cilt: 3; Sayı: 3>> Özet
TAM METİN
The Journal of Neurobehavioral Sciences; 2016;3(3):116-118
72 Yaşındaki Isaac Sendrom’lu Adam: Farklı Sonuçları Olan Nadir Bir Olay
AA Saeed, R Saeed, Z Mohammed, S Hamid
Shar Teaching Hospital of Sulaimania, Kurdistan
Nöromiyotoni, sürekli kas lifi hareketinden kaynaklı kas gerginliğiyle kendini belli eden nöromüsküler bir hipereksitilabite bozukluğudur. Bu rahatsızlık, voltaja duyarlı, presinaptik potasyum kanallarına karşı gerek izolasyon gerekse paraneoplastik bir süreç görevi üstlenen yüksek antikor seviyesine sahip immün aracılı bir bozukluktur. Belirtileri arasında kasların rahatlama hareketi esnasında kas çekilmesi (miyokimi), kramplar, psödomiyotoni (kas gevşemesinde gecikme), aşırı terleme ve bazen de kas güçsüzlüğü sayılabilir. Bu olgu sunumnda, bir ay boyunca her iki uyluğunda ağrı tespit edilen yetmiş iki yaşında bir erkek hastayı incelemekteyiz. Buna göre hastalık, ayaklara ve göğüs bölgesini de içine alacak şekilde git gide kötüleşmiş durumdaydı. Çekilen beyin tomografisinde, beyin atrofisi olduğu ortaya çıkmıştı. EMG sonuçları, tipik Isaacs Sendromu belirtisi olan karakteristik dalga sıklığı ve genişliği tespitiyle bağlantılı olarak nöromiyotonik boşalımlar boyunca fasikülasyon (kas seğirmesi) bulgusuna işaret etmekteydi. Ayrıca, potasyum kanalı antikor miktarı oldukça yüksek seviyedeydi. Sonuç olarak hastaya Isaacs Sendromu teşhisi kondu. Hasta iki ay boyunca üç günlük süreyle önce metil prednizolon, sonrasında da oral steroid ve metotreksat tedavisi uygulanarak takip altına alında ve bu süre zarfında hastanın durumunda önemli ölçüde iyileşme gözlemlendi. Bu klinik olay, Kürdistan’da kaydedilen ilk Isaacs Sendromu vakasıdır.
A 72 Year Old Man with Isaacs' Syndrome: A Rare Entity with Different Outcomes
Neuromyotonia is a neuromuscular hyperexitability disorder characterized by muscle stiffness caused by continuous muscle fiber activity. It is an immune mediated disorder with elevated antibody level against presynaptic, voltage gated potassium channels, either as isolation or as a paraneoplastic process. Symptoms usually include muscle twitching during rest (myokymia), cramps, peudomyotonia (delayed relaxation), increased sweating, and sometimes motor weakness. In this case report, we present a seventy two year old man who presented with pain in both thighs for one month. It gradually became worse to involve feet and chest. His brain CT scan showed features of brain atrophy. EMG showed fasciculation along neuromyotonic discharges with characteristic wave in frequency and amplitude typical of Isaacs syndrome. Potassium channel antibodies were very high. Diagnosis of Isaacs syndrome was made. He was followed up for two months with treatment by three day course of methyl prednisolone followed by oral steroid and methotrexate with much improvement. This is the first case of Isaacs syndrome in Kurdistan.

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