İstemsiz hareketlerle başvuran hastaların etyolojik ve klinik özelliklerinin değerlendirilmesi
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Date
2011
Authors
Uzun, Meltem
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Publisher
Uludağ Üniversitesi
Abstract
Hareket bozuklukları (HB) istemsiz ve amaçsız yapılan hareketler olup, genellikle ekstrapiramidal sistem ve/veya serebellumun etkilenmesi sonucu oluşur. Kore, ballismus, distoni, atetoz, miyoklonus, tik, tremor, stereotipik hareketler gibi yaygın görülenler ve diskinezi, shuddering, haz fenomeni, jitteriness, spasm nutans, istemsiz göz hareketleri gibi daha nadir görülen tipleri vardır. Çocuklarda hareket bozuklukları genellikle altta yatan başka bir hastalığın (perinatal dönemde hipoksi, kafa travması, İntrakranial kanama, inme, kitle, ensefalit, Wilson hastalığı, romatizmal hastalıklar, metabolik hastalıklar, antiepileptik ilaçlar, tirotoksikoz..vb) belirtisi olarak sekonder ortaya çıkar. Çocuklarda daha az sıklıkla da primer hareket bozuklukları şeklinde de görülmektedirler.Bu çalışmada Mayıs 2010- Mayıs 2011 tarihleri arasında, Uludağ Üniversitesi Tıp Fakültesi Çocuk Sağlığı ve Hastalıkları Ana Bilim Dalı Çocuk Nöroloji Bilim Dalı polikliniğinde hareket bozukluğu tanısı konan, yaşları 5 ay-18 yaş arasındaki değişen 52 kız, 77 erkek çocuktan oluşan 129 hasta incelenmiş ve ailelerinden izin alınarak hastalıkları ile ilintili video kamera çekimleri yapılmıştır.Tüm hastalara bakıldığı zaman kız/erkek oranı 0.67 (52 / 77) ve yaş ortalaması 9.10 ± 5.47 yıl olarak bulundu. Başvuru yakınmalarına bakıldığında en sık tremor 31 (%24), ikinci sırada distoni 26 (%20.1) ve üçüncü sırada kore 20 (%15.5) vakada görülmüştür. Tremor saptanan hastaların %58'inde tremor posturaldir. Altta yatan en sık sebep ilaca bağlı olup (%32.2), bunu esansiyel tremor (%22.8) izlemiştir. Etiyolojideki diğer tanılar, menenjit-ensefalit, HİE, intrakranial kitle, vasküler patolojiler (hipertansiyon, anjiom), psikojenik hastalık (depresyon, anksiyete), nöropati ve travmadır. Bu hastalarda yapılan kranial MRG vakaların 21'inde (%67.7) normalken, 10'unda (%32.3) anormal bulunmuştu. Tremorla başvuran vakalarda tedavi olarak en sık propranolol kullanılmıştır. Distoni hastalarımızda en sık fokaldir (%34.6). Altta yatan sebepler sıklık sırasıyla; HİE, sarılık, menenjit-ensefalitdir. Ayrıca bu grupta yer alan 21 hastada hafiften ağıra kadar değişen derecede serebral paralizi (SP) bulguları vardı. Bu gruptan bir erkek hastamıza nadir görülen ve ilaca bağlı gelişen fokal distonilerden biri olan oramandibulolingual distoni, bir kız hastaya Wilson hastalığı, bir erkek hastaya da sporadik distoni tanısı konmuştur. MRG görüntüleme vakaların hastaların 10'unda (%38.4) normalken 13'ünde (% 50) anormal bulgular izlenmiştir. Anormal bulgu saptanan 3 vakada (%11.5) bazal ganglion tutulumu saptanmıştır. En çok kullanılan ilaçlar baklofen, L-dopa, pimozid, klonezepamdır. Korea yakınması ile başvuran 20 vakanın altta yatan sebepler sıklık sırası ile 6 hastada akut romatizmal ateş, 4 hastada hipoksik-iskemik ensefalopati (HİE), birinde kernikterus, 3 hastada psikojenik kore hareketleri, 2 hastada geçirilmiş menenjit/ ensefalit, birinde anti-fosfolipid antikor sendromu, bir hasta idiopatik olarak düşünülmüştür. Bir hastada neden bulunamamıştır. 17 yaşındaki bir kız hastamızın 13 yaşında kore başlamış ve syndenham kore tanısı almış, 2 yıl sonra tekrar kore sebebiyle araştırıldığında daha önce negatif olan anti kardiyolipin antikorları pozitif saptanmış. Sydenham koresi tüm hareket bozukluklarının % 4.6'sını, kore bozukluğu olanların ise % 30'unu oluşturmuştur. Kardiyak tutulum sadece 1 hastada mitral yetmezlik olarak saptandı. Tedavide bütün hastalar VA kullandı, 2 hastaya ayrıca haloperidol eklendi.Çalışmamızda saptanan diğer hareket bozuklukları, atetoz, koreatetoz-distoni, stereotipik hareketler, tik, diskinezi, shuddering, haz fenomeni, jitteriness, spasm nutans, istemsiz göz hareketleridir. Bunların birçoğu nöbet veya paroksismal hastalıklarla karışabilmektedir. Bu nedenle hastaları iyi gözlemek ve gerekirse video kamera çekimi yapmak gerekir.Hareket bozuklukları çocuklarda aktiviteyi kısıtlayan, ciddi özürlere yol açabilen bir hastalık grubudur. Etiyolojiye bakıldığında HİE, sarılık, SK, ilaçlar, psikojenik gibi önlenebilir sebepler olması nedeniyle koruyucu önlemler alınması durumunda prevelansı azalacaktır. Bu bozuklukların tedavisi zor ve bazen tedaviye dirençlidir. Tanınması bazen zor olan ve pek çok nörolojik hastalıklarla karışabilen bu hastalıklar daha çok vurgulanması gereken ve çözüm bekleyen geniş bir konudur.
Movement Disorders (MD) are unintentional movements, which are generally resulted from a possible harm to the extrapyramidal system and/or cerebellum. There are such common types as chorea, ballismus, dystonia, athetozis, myoclonus, tic, tremor, stereotypical movements as well as such rare types as dyskinesia, shuddering, masturbation, jitteriness, spasmus nutans, and unintentional eye movements. Movement disorders appear as a secondary indication of an underlying disease In this study, 52 female and 77 male, a total of 129, patients aged between 5 months and 18 years old who were diagnosed for movement disorder have been investigated and video-recorded through their parents’ consent between May 2010 and May 2011 in Child Neurology Department of Uludag University. Female/male ratio of the group was 0.67 (52 / 77) and average age was found to be 9.10 ± 5.46. The most common disorders were tremor 31 (24%), dystonia 26 (20.1%) and chorea 20 (15.5%) respectively. The average age among the patients with tremor was 11.75 (0,5- 18 year old). The most frequent underlying reason was medicine induced tremor (32.2%) and essential tremor follows that with 22.8%. Cranial MRG was found to be abnormal in 10 (32.2%) cases. 9 cases were treated with propranolol and 2 with primidone. The average age among the cases with dystonia was 8.2 (1-17). The most frequent reasons were hypoxic-ischemic ensefalophaty, jaundice, menengitis-ensephalitis. Cranial MRG was abnormal in 61.6% of the patients. 14 patients were given medicine. The most frequently used medicine were baclofen, L-dopa, primozid, klonezepam, and 2 patients were applied botulinum toxoid. Six out of 20 patients with chorea disorder were thought to be Sydenham chorea, 4 hypoxic-ischemic encephalophaty, 1 kernicterus, 3 psychogenic chorea movements, 2 menengitis/encephalitis, 1 antiphospolipid antibody syndrome and 1 idiopatic.1 patient remained undiagnosed. One 17 year old female patient developed chorea at 13 and diagnosed for syndenham and started valproic acid treatment. 9 months later, the medicine treatment was stopped. 2 years later, when searched for chorea, previously negative anti-cardiolipin antibodies were tested positive. Chorea has relapsed in 2 patients (one syndenham, and the other AFS) 2 years later and in one patient (syndenham) 1 year later. Sydenham chorea has been detected in 4 female (66.6%) and 2 male (33.6%), a total of 6 patients. Cardiac retention was detected in only one patient as mitral failure. All the patients were treated with valporic acid, and 2 patients were provided with haloperidol. Besides all the above, the disorders detected in our study are as follow: stereotypical movements, koreatetoz-distonia mixed type, twitching, dyskinesia, shuddering, masturbation, jitteriness, spasm nutans, unintentional eye movements. Most of them might be mistaken with seizure and paroxysmal diseases. A meticulous observation, and if necessary, video recording is required. Movement disorders limit children’s activity and have the potential of causing serious defects. When looked at ethiology, as such reasons as HIE, infections, Syndenham chorea, medicine and psychogenic is avoidable, its prevalence would decrease. The treatment for these disorders is hard and sometimes resistant to treatment. These diseases are sometimes very hard to diagnose and might be mistaken for many other neurological diseases. Thus they need to be emphasized and beg for solutions.
Movement Disorders (MD) are unintentional movements, which are generally resulted from a possible harm to the extrapyramidal system and/or cerebellum. There are such common types as chorea, ballismus, dystonia, athetozis, myoclonus, tic, tremor, stereotypical movements as well as such rare types as dyskinesia, shuddering, masturbation, jitteriness, spasmus nutans, and unintentional eye movements. Movement disorders appear as a secondary indication of an underlying disease In this study, 52 female and 77 male, a total of 129, patients aged between 5 months and 18 years old who were diagnosed for movement disorder have been investigated and video-recorded through their parents’ consent between May 2010 and May 2011 in Child Neurology Department of Uludag University. Female/male ratio of the group was 0.67 (52 / 77) and average age was found to be 9.10 ± 5.46. The most common disorders were tremor 31 (24%), dystonia 26 (20.1%) and chorea 20 (15.5%) respectively. The average age among the patients with tremor was 11.75 (0,5- 18 year old). The most frequent underlying reason was medicine induced tremor (32.2%) and essential tremor follows that with 22.8%. Cranial MRG was found to be abnormal in 10 (32.2%) cases. 9 cases were treated with propranolol and 2 with primidone. The average age among the cases with dystonia was 8.2 (1-17). The most frequent reasons were hypoxic-ischemic ensefalophaty, jaundice, menengitis-ensephalitis. Cranial MRG was abnormal in 61.6% of the patients. 14 patients were given medicine. The most frequently used medicine were baclofen, L-dopa, primozid, klonezepam, and 2 patients were applied botulinum toxoid. Six out of 20 patients with chorea disorder were thought to be Sydenham chorea, 4 hypoxic-ischemic encephalophaty, 1 kernicterus, 3 psychogenic chorea movements, 2 menengitis/encephalitis, 1 antiphospolipid antibody syndrome and 1 idiopatic.1 patient remained undiagnosed. One 17 year old female patient developed chorea at 13 and diagnosed for syndenham and started valproic acid treatment. 9 months later, the medicine treatment was stopped. 2 years later, when searched for chorea, previously negative anti-cardiolipin antibodies were tested positive. Chorea has relapsed in 2 patients (one syndenham, and the other AFS) 2 years later and in one patient (syndenham) 1 year later. Sydenham chorea has been detected in 4 female (66.6%) and 2 male (33.6%), a total of 6 patients. Cardiac retention was detected in only one patient as mitral failure. All the patients were treated with valporic acid, and 2 patients were provided with haloperidol. Besides all the above, the disorders detected in our study are as follow: stereotypical movements, koreatetoz-distonia mixed type, twitching, dyskinesia, shuddering, masturbation, jitteriness, spasm nutans, unintentional eye movements. Most of them might be mistaken with seizure and paroxysmal diseases. A meticulous observation, and if necessary, video recording is required. Movement disorders limit children’s activity and have the potential of causing serious defects. When looked at ethiology, as such reasons as HIE, infections, Syndenham chorea, medicine and psychogenic is avoidable, its prevalence would decrease. The treatment for these disorders is hard and sometimes resistant to treatment. These diseases are sometimes very hard to diagnose and might be mistaken for many other neurological diseases. Thus they need to be emphasized and beg for solutions.
Description
Keywords
Hareket bozuklukları, Hiperkinetik bozukluklar, Kore, Distoni, Atetoz, Tremor, Stereotipik hareket, Tik, Miyoklonus, Pediatric movement disorders, Hyperkinetic movement disorders, Chorea, Dystonia, Atethosis, Stereotypic movements, Tics, Myoclonus
Citation
Uzun, M. (2011). İstemsiz hareketlerle başvuran hastaların etyolojik ve klinik özelliklerinin değerlendirilmesi. Yayınlanmamış uzmanlık tezi. Uludağ Üniversitesi Tıp Fakültesi.