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Psikoz Atağında Gelen Bir Down Sendromu ve Nadir Bir Komplikasyon: Bazal Ganglion Kalsifikasyonu-Retrospektif Bir Tarama

Year 2018, Volume: 6 Issue: 1, 6 - 9, 30.04.2018

Abstract

Down sendromu (DS), en sık karşılaşılan kromozom anomalisidir. Fenotipik özelliklerinin yanı sıra birçok organ sistemlerini etkilemektedir. Mental motor gerilik, hipotiroidi, konjenital kalp hastalıkları, işitme ve görme sorunları, epilepsi ve kas-iskelet sistemi ile ilgili problemler nedeniyle ile de takibi gerekebilir. Mental retardasyon DS’a sıklıkla eşlik ettiği için çoğunlukla nöroloji polikliniklerinde takip edilir. Kimi zaman gelişen ek nörolojik problemler nedeniyle kranial görüntüleme gerekmektedir. Bazal ganglion kalsifikasyonu %0.3-0.6 oranında görülen nadir bir komplikasyondur. Down sendromuna daha sık eşlik ettiği bilinmektedir. Bu konuda erişkinlerde bildirilen vakaların aksine çocukluk yaş grubunda az sayıda DS ve bazal ganglion kalsifikasyon (BGK) birlikteliği raporlanmıştır. Bir komplikasyon olarak karşılaştığımız bu kalsifikasyonun DS’li çocuklarda erken yaşlanma ile ilgili olduğu düşünülmektedir.

 Down sendromu ve davranış problemleri nedeni ile takip edilmekte olan hastanın kranial MR’ında BGK tespit edildi. Çocukluk çağında nadir görülmesi nedeni ile bahsedilmeye değer bulundu. Ayrıca kliniğimizde takip edilen DS’lu hastalarda BGK sıklığının tespit edilmesi amaçlandı. Bu amaçla, Pediatrik Nöroloji Polikliniği’nde takip edilmekte olan tüm DS’lu hastalar geriye dönük olarak tarandı.


References

  • Reference1. Radhakrishnan R, Towbin AJ. Imaging findings in Down syndrome. Pediatr Radiol. 2014;44(5):506-21.
  • Reference2. Mann DMA. Calcification of the basal ganglia in Down's syndrome and Alzheimer's disease Acta Neuropathol 1988 76:595-8.
  • Reference3. Ieshima A, Kisa T, Yoshino K, Takashima S, Takeshita K. A morphometric CT study of Down's syndrome showing small posterior fossa and calcification of basal ganglia. Neuroradiology. 1984;26(6):493-8.
  • Reference4. Wegiel J, Kuchna I, Wisniewski T, de Leon MJ, Reisberg B, Pirttila T, Kivimaki T, Lehtimaki T. Vascular fibrosis and calcification in the hippocampus in aging, Alzheimer disease, and Down syndrome. Acta Neuropathol. 2002;103(4):333-43.
  • Reference5. Okano S, Takeuchi Y, Kohmura E, Yoshioka H, Sawada T. Globus pallidus calcification in Down syndrome with progressive neurological deficits. Pediatr Neurol 1992 8:72-4.
  • Reference6. Thase ME. Basal ganglia calcification and psychosis in Down's syndrome. Postgrad Med J. 1984;60(700):137-9.
  • Reference7. Bourgeois JA. Fahr's disease - a model of neuropsychiatric illness with cognitive and psychotic symptoms. Acta Psychiatr Scand. 2010;121(1):78.
  • Reference8. Uslu Fİ, Hanağası HA. Hipoparatroidizm ve Bilateral Striopallidodentat Kalsinozis. Nöropsikiyatri Arşivi. 2006; 43: 31-36
  • Reference9. Takashima S, Becker LE. Basal ganglia calcification in Down's syndrome. J Neurol Neurosurg Psychiatry. 1985;48(1):61-4.
  • Reference10. Kłosowska A, Ćwiklińska A, Kuchta A, Berlińska A, Jankowski M, Wierzba J. Down syndrome, increased risk of dementia and lipid disturbances. Dev Period Med. 2017;21(1):69-72
  • Reference11. Kendirli, T, Bingöler, B.E, Tutar, E, İnce, E, Deda, G. Down sendromlu bir olguda bazal gangliyon kalsifikasyonu. Adnan Menderes Üniversitesi Tıp Fakültesi Dergisi,2004;5(3):41-42
  • Reference12. Sedat Işıkay. A rare cause of basal ganglia calcification: Down syndrome Gaziantep Med J 2014;20(2):190-192
  • Reference13. Arya R, Kabra M, Gulati S. Epilepsy in children with Down syndrome. Epileptic Disord. 2011;13(1):1-7.

Down syndrome in the psychosis and a rare complication: basal ganglyon calsification-a retrospective screen

Year 2018, Volume: 6 Issue: 1, 6 - 9, 30.04.2018

Abstract

Down syndrome (DS) is the most common chromosomal anomaly. It affects many organ systems as well as its phenotypic properties. Mental motor retardation, hypothyroidism, congenital heart diseases, hearing and vision problems, epilepsy and musculoskeletal problems may also be required. Mental retardation is often followed up in neurology outpatient clinics because it is often accompanied by DS. Cranial imaging is necessary because of the occasional neurological problems. Basal ganglion calcification is a rare complication seen in the rate of 0.3-0.6%. It is known to accompany Down syndrome more frequently. In this respect, in contrast to the cases reported in adults, a small number of DS and basal ganglion calcification (BGC) associations have been reported in childhood age group. This calcification, which we encounter as a complication, is thought to be related to premature aging in DS children. 

BCC was detected in the cranial MR of the patient being followed for the cause of Down's syndrome and behavior problems. It was worth mentioning that it was rarely seen during childhood. It was also aimed to determine the frequency of BGC in DS patients followed up in our clinic. For this purpose, all DS patients under follow-up at the Pediatric Neurology Policlinic were retrospectively screened.


References

  • Reference1. Radhakrishnan R, Towbin AJ. Imaging findings in Down syndrome. Pediatr Radiol. 2014;44(5):506-21.
  • Reference2. Mann DMA. Calcification of the basal ganglia in Down's syndrome and Alzheimer's disease Acta Neuropathol 1988 76:595-8.
  • Reference3. Ieshima A, Kisa T, Yoshino K, Takashima S, Takeshita K. A morphometric CT study of Down's syndrome showing small posterior fossa and calcification of basal ganglia. Neuroradiology. 1984;26(6):493-8.
  • Reference4. Wegiel J, Kuchna I, Wisniewski T, de Leon MJ, Reisberg B, Pirttila T, Kivimaki T, Lehtimaki T. Vascular fibrosis and calcification in the hippocampus in aging, Alzheimer disease, and Down syndrome. Acta Neuropathol. 2002;103(4):333-43.
  • Reference5. Okano S, Takeuchi Y, Kohmura E, Yoshioka H, Sawada T. Globus pallidus calcification in Down syndrome with progressive neurological deficits. Pediatr Neurol 1992 8:72-4.
  • Reference6. Thase ME. Basal ganglia calcification and psychosis in Down's syndrome. Postgrad Med J. 1984;60(700):137-9.
  • Reference7. Bourgeois JA. Fahr's disease - a model of neuropsychiatric illness with cognitive and psychotic symptoms. Acta Psychiatr Scand. 2010;121(1):78.
  • Reference8. Uslu Fİ, Hanağası HA. Hipoparatroidizm ve Bilateral Striopallidodentat Kalsinozis. Nöropsikiyatri Arşivi. 2006; 43: 31-36
  • Reference9. Takashima S, Becker LE. Basal ganglia calcification in Down's syndrome. J Neurol Neurosurg Psychiatry. 1985;48(1):61-4.
  • Reference10. Kłosowska A, Ćwiklińska A, Kuchta A, Berlińska A, Jankowski M, Wierzba J. Down syndrome, increased risk of dementia and lipid disturbances. Dev Period Med. 2017;21(1):69-72
  • Reference11. Kendirli, T, Bingöler, B.E, Tutar, E, İnce, E, Deda, G. Down sendromlu bir olguda bazal gangliyon kalsifikasyonu. Adnan Menderes Üniversitesi Tıp Fakültesi Dergisi,2004;5(3):41-42
  • Reference12. Sedat Işıkay. A rare cause of basal ganglia calcification: Down syndrome Gaziantep Med J 2014;20(2):190-192
  • Reference13. Arya R, Kabra M, Gulati S. Epilepsy in children with Down syndrome. Epileptic Disord. 2011;13(1):1-7.

Details

Primary Language Turkish
Subjects Health Care Administration
Journal Section Original Articles
Authors

Beril DİLBER 0000-0002-7633-0060

Tülay KAMAŞAK 0000-0002-5212-0149

Betül DİLER DURGUT This is me 0000-0002-0322-2843

Elif ACAR ARSLAN 0000-0002-3284-107X

Sevim ŞAHİN 0000-0001-5415-5874

Ali CANSU 0000-0002-1930-6312

Publication Date April 30, 2018
Acceptance Date December 20, 2018
Published in Issue Year 2018 Volume: 6 Issue: 1

Cite

Vancouver DİLBER B, KAMAŞAK T, DİLER DURGUT B, ACAR ARSLAN E, ŞAHİN S, CANSU A. Psikoz Atağında Gelen Bir Down Sendromu ve Nadir Bir Komplikasyon: Bazal Ganglion Kalsifikasyonu-Retrospektif Bir Tarama. pediatr pract res. 2018;6(1):6-9.