Bir Kıl Keçisinde Nöronal Lipofusinozis

Öz

Bu olgu raporunda, üç yaşlı, dişi bir kıl keçisinde görülen nöronal lipofusinozisin tanımlanması amaçlandı. Keçinin sistemik nekropsisi yapıldıktan sonra alınan doku örneklerinin kesitleri; lipofusin pigmenti için hematoksilen-eozin (HE), periodic acid-schiff (PAS), Schmorl’s ve long Ziehl-Neelsen (ZN) boyama yöntemleri ile; hemosiderin için Turnbull blue ve melanin için melanin removal method II yöntemleri ile boyandıktan sonra ışık mikroskobunda değer-lendirildi. Herhangi bir histokimyasal uygulama görmemiş preparatlar direkt olarak floresan mikroskopta; ayrıca pons ve medulla oblongata’dan alınan doku örnekleri elektron mikroskopta incelendi. Makroskobik incelemede, meninksler matlaşmış ve serebral hemisferler hafif sert kıvamlı idi. Mikroskobik incelemede, pons ve medulla oblongata’da bulunan nöronların sitoplazmalarında değişen yoğunluklarda kırmızı ya da sarı-kahve renkli pigment granülleri görüldü. Bu granüller sıklıkla perinüklear yerleşimli olup, bazı nöronlarda sitoplazmanın tamamını doldurmuştu. Pigment birikimi ile birlikte meningeal ve perinöronal ödem, perivasküler kanama ve nöron dejenerasyonu belirlendi. PAS, Schmorl’s ve Long ZN boyama yöntemleri ile pozitif; Turnbull blue ve melanin removal method II boyama yöntemleri ile negatif bo-yanma özelliği gösteren pigment granüllerinin tamamı, floresan mikroskoptaki direkt incelemede floresan pozitif yan-sımalar verdi. Elektron mikroskobik incelemede de bu pigment granüllerinin lipofusin pigmenti olduğu doğrulandı.

Anahtar Kelimeler

• Nöronal lipofusinozis
• patoloji
• elektron mikroskopi
• keçi

Neuronal Lipofuscinosis in a Hairy Goat

Öz

In the present case, neuronal lipofuscinosis was determined in a three-year-old female hair goat. The sys-temic necropsy was performed. Tissue sections were stained with hematoxylin and eosin (HE), periodic acid schiff stain (PAS), Schmorl’s and long Ziehl-Neelsen (ZN) methods for lipofuscin; Turnbull’s blue method for hemosiderin and melanin removal method II for melanin. Unstained deparaffinized sections were examined microscopically for fluores-cence in transmitted ultraviolet light. In addition, samples of pons and medulla oblongata were collected and processed for transmission electron microscopy investigations. Macroscopically, the meninges were opaque and the cerebral hemispheres had firm consistency. Microscopically, pigment granules in varying intensity were observed in the cytop-lasm of the neurons of the pons and medulla oblongata. Red or yellowish-brown grannules were usually seen in peri-nuclear localization, and filled all cytoplasm of some neurons. Moreover, meningeal and perineuronal edema, perivas-cular hemorrhage and neuronal degeneration were prominent histopatologic findings. The granules were positively stained with PAS, Schmorl’s and long ZN methods, but negatively stained with Turnbull’s blue method and melanin removal method II. Pigments found in all affected neurons were brightly autofluorescent. Electron microscopy also confirmed that the pigment granules were lipofuscin.

Anahtar Kelimeler

• Neuronal lipofuscinosis
• pathology
• electron microscopy
• goat

Kaynakça

1. Baghban F, Gharagozlou MJ, Bazargani TT, Samani R. Neuronal lipofuscinosis in an adult horse. In-dian J Vet Pathol 2013; 37: 89-90.
2. Birincioğlu SS, Çalış İ, Avcı H, Erdağ B. Pathological and phytochemical investigation of neuronal lipo-fuscinosis caused by Asphodelus aestivus in sheep: I. pathological findings. Turkish J Vet Anim Sci 2005; 29: 1351-6.
3. Birincioğlu SS, Schmahl W, Avcı H. Neuronal and extraneuronal lipofuscinosis in merino sheep grazing Asphodelus aestivus seeds in western Turkey. Turkish J Vet Anim Sci 2012; 36: 476-82.
4. Carson FL, Hladik C. Histotechnology, A Self-Instructional Text. Third Edition. Hong Kong: Academic Society for Clinical Pathology Press, 2009.
5. Cheville NF. Cell Pathology. Second Edition. Iowa: The Iowa State University Press, 1983; pp. 153-8.
6. Culling CFA, Allison RT, Barr WT. Cellular Pathology Technique. Fourth Edition. London: Butterworth & Co Publishers Ltd, 1985; pp. 278-88.
7. Glees P, Hasan M. Lipofuscin in neuronal aging and diseases. Bargmann W, Doerr W. eds. In: Normal and Pathological Anatomy. Stuttgart: Georg Thime Publishers 1976; pp. 1-58.
8. Huxtable CR, Chapman HM, Main DC, Vass D, Pear-se BHG, Hilbert BJ. Neurological disease and lipo-fuscinosis in horses and sheep grazing Trachyand-ra divaricata (branched onion weed) in South Western Australia. Aust Vet J 1987; 64: 105-8.

9. Jolly RD, Douglas BV, Davey PM, Roiri JE. Lipofus-cin in bovine muscle and brain: A model for stud-ying age pigment. Gerontol 1995; 41 (2): 283-95.
10. Jolly RD, Arthur DG, Kay GW, Palmer DN. Neuronal ceroid-lipofuscinosis in Borderdale sheep. NZ Vet J 2002; 50 (5): 199-202.
11. Jung T, Bader N, Grune T. Lipofuscin: Formation, distribution, and metabolic consequnces. Ann NY Acad Sci 2007; 1119: 97-111.
12. Luna LG. Manual of Histologic Staining Methods of the Armed Forces Institute of Pathology. Third Edition. New York: Mc Graw-Hill Book Company, 1968.
13. Nardocci N, Cardona F. Neuronal ceroid lipofuscino-sis: A review. Ital J Neurol Sci 1998; 19: 271-6.
14. Tammen I, Cook RW, Nicholas FW, Raadsma HW. Neuronal ceroid lipofuscinosis in Australian Merino sheep: A new animal model. Euro J Pediatr Neurol 2001; 5 (Suppl A): 37-41.
15. Wohlsein P, Deschl U, Baumgartner W. Non lesions, unusual cell types and postmortem artifacts in the central nervous system of domestic animals. Vet Pathol 2013; 50 (1): 122-43.