Mustafa KOÇ, Selami SERHATLIOĞLU, Hacı ORHAN

Psikiyatrik Semptomlarla Başvuran Nörowilson Olgusunda Reversibl Kranial MRG Bulguları

Wilson hastalığı; otozomal resessif geçiş gösteren, bakır metabolizmasındaki bozukluktan kaynaklanan metabolik bir depo hastalığıdır. Hastalığın semptomları; karaciğer, beyin, kornea, böbrek ve diğer dokularda toksik bakır birikimi ile gelişir. Wilson hastalığı tanısı, 5-40 yaş arasında nörolojik ve psikiyatrik semptomlar sergileyen hastalarda göz önünde bulundurulmalıdır. MRG de izlenen en sık anormallik, T2 ağırlıklı görüntülerde lentiküler, talamik ve kaudat nükleusta ayrıca beyaz cevherde artmış sinyal intensiteleridir. Tedavi sonrasında bazal ganglionlardaki sinyal intensiteleri kaybolabilmektedir. Hastalığın tanı, tedavi ve radyolojik görüntüleme özelliklerinin bilinmesi önemlidir. Çünkü erken tanı ve tedavi ile hastalardaki onarılamayan doku hasarı önlenebilmekte, buna bağlı olarak prognoz olumlu yönde etkilenmektedir. Biz bu olgumuzda kranial MRG de Wilson hastalığının tutulumu ile uyumlu sinyal değişiklikleri tesbit edilen hastanın, tanı anında ve tedavi sonrasında oluşan MRG görüntü özelliklerini tartıştık. ©2007, Fırat Üniversitesi, Tıp Fakültesi

Anahtar Kelimeler:

Wilson hastalığı, Manyetik rezonans görüntüleme, Bazal ganglion

Reversible Cranial MRI Findings of Neurowilson Case Hospitalized with Phsiciatric Sympthomps

Wilson's disease is a metabolic storage disease resulting from the malfunction in the copper metabolism showing autosomal recessive progress. The symptoms of the disease become apparent with the buildup of toxic copper residues in the liver, brain, cornea, kidney and other tissues. The diagnosis of the Wilson's disease should be taken into account in the patients showing neurological and physiological symptoms between the ages of 5 and 40. The most widely viewed abnormalities in T2 dominant images of MRI are the increased intensities in lenticular, thalamic, nucleus caudatus and the white matter. The signal intensities may disappear in the basal ganglia after the treatment. It is important to know the pre-diagnosis, treatment and radiological screenings of the disease. Because, if the disorder is detected early and treated correctly, the irreversible tissue damage can partly be prevented, thus the prognosis is positively affected. We report a case where cranial MRI shows signal intensities of Wilson disease, to discuss the characterization of MRI imaging at diagnosis and after treatment. ©2007, Firat University, Medical Faculty

Keywords:

-,

PDF

___

Kinnear Wilson SA. Progressive lenticular degeneration: a familial nervous disease associated with cirrhosis of the liver. Brain 1912; 34: 295-309.
Magalhaes AÇA, Caramelli P, Menezes JR, et al. Wilson’s disease: MRI with clinical correlation. Neuroradiology 1994; 36: 97-100.
Aisen AM, Martel W, Gabrielsen TO, et al. Wilson disease of the brain: MR imaging. Radiology 1985; 157: 137-141.
Dörnemann H, Petsch R, Braitinger S. MRI beiMorbus Wilson. Zur Gewebedifferenzierung vonintrazerebralen Scadigungen. ROFO Fortschr GebRontgenstr Nuclearmed 1987; 147: 570-571.
Şener RN. Wilson’s disease: MRI demonstration of cavitations in basal ganglia and thalami. Pediatr Radiol 1993; 23: 157.
Schoen RE, Sternlieb I. Clinical aspects of Wilson disease. Am J Gastroenterol 1990; 85: 1453-1457.
Brugieres P, Combes C, Ricolfi F, et al. A typical presentation of Wilson disease: a possible consequence of paramagnetic effect of copper? Neuroradiology 1992; 34: 222-224.
Prayer L, Wimberger D, Kramer J, et al. Cranial MRI in Wilson’s disease. Neuroradiology 1990; 32: 211-214.
Hester N, van Wassenaer-van Hall HN, van den Heuvel AĞ, et al. Cranial MR in Wilson Disease: Abnormal White Matter in Extrapyramidal and Pyramidal Tracts. Am J Neuroradiol 1995; 16: 2021–2027.
Saatçi I, Topçu M, Baltaoğlu FF, et al. Cranial MR findings in Wilson’s disease. Acta Radiol 1997; 38(2): 250-258.
van Wassenaer-van Hail HN, van den Heuvel AĞ, Algra A, Hoogenraad TU. Wilson disease: findings at MR imaging and CT of the brain with clinical correlation. Radiology 1996; 198: 531- 536.
Ma KC, Ye ZR, Wu JV. Glial fibrillar acidic protein immunohistochemical study of Alzheimer I & II astrogliosis in Wilson’s disease. Acta Neurol Scand 1988; 78: 290–296.
Engelbrecht V, Schlaug G, Hefter H, Kahn T, Modder U. MRI of the brain in Wilson disease: T2 signal loss under therapy. J Comput Assist Tomogr 1995; 19: 635-638.
Takahashi W, Yoshii F, Shinohara Y. Reversible magnetic resonance imaging lesions in Wilson’s disease: clinical- anatomical correlation. J Neuroimaging 1996; 6: 246-248.
Nawras K, Doris TH, Thomas MR. Wilson’s disease. South Med J 1997; 90: 535-538.
Kabul Tarihi: 04.09.2006