Adamkiewitz Syndrome in Marfan Syndrome and Rehabilitation Outcomes- 3 Cases and Review of the Literature

Yıl 2022, Cilt: 39 Sayı: 3, 910 - 912, 30.08.2022

Zeynep Kıraç Ünal Gulnur Celik Sadik Yigit Yildiz Damla Cankurtaran Ece Ünlü Akyüz İlker İnce

Öz

Acute Spinal Cord Ischemia Syndrome (ASCIS) is rare but it is clinically very significant. Aortic dissection (AD) is one of the reasons of ischemia of the spinal cord. The artery of Adamkiewicz provides the major blood supply to the anterior thoracolumbar spinal cord, and injury to this artery can cause consequential neurologic damage.
Three male patients applied to our rehabilitation clinic with paraplegia. All three had AD due to Marfan Syndrome and all had clinical findings due to Adamkiewitz artery ischemia. Although it was later learned that one of the patients later died from a new ruptured aortic aneurysm, we achieved good motor gains in these rare cases with rehabilitation.
Although paraplegia is rare as the initial symptom in AD, vascular etiology shouldn’t be forgotton in acute paraplegia. The prognosis of ASCIS is known to be poor, but a good and individualized rehabilitation program is required.

Anahtar Kelimeler

Acute Spinal Cord Ischemia Syndrome, Marfan Syndrome, Paraplegia, Rehabilitation

Kaynakça

• 1. Weidauer S, Nichtweiß M, Hattingen E, Berkefeld J. Spinal cord ischemia: aetiology, clinical syndromes and imaging features. Neuroradiology 2015; 57(3): 241-257.
• 2. Takagi H, Ota H, Natsuaki Y, Komori Y, Ito K, Saiki Y, et al. Identifying the Adamkiewicz artery using 3-T time-resolved magnetic resonance angiography: its role in addition to multidetector computed tomography angiography. Jpn J Radiol 2015; 33: 749–756.
• 3. Nedeltchev K, Loher TJ, Stepper F, Arnold M, Schroth G, Mattle HP, et al. Long-term outcome of acute spinal cord ischemia syndrome. Stroke 2004; 35(2): 560-565.
• 4. Khan IA. Clinical manifestations of aortic dissection. J Clin Basic Cardiol 2001; 4: 265.
• 5. Prakash, B, Pai RK., Chaitra V, Ramasamy P, Shuba N. A Case of Acute Paraplegia Due to Aortic Dissection in Marfan Syndrome. Journal of Neurosciences in Rural Practice 2017; 8(2): 316-319.
• 6. Pyeritz RE. Marfan syndrome: 30 years of research equals 30 years of additional life expectancy. Heart 2009; 95: 173–175.
• 7. Alblas CL, Bouvy WH, Lycklama À Nijeholt GJ, Boiten J. Acute spinal-cord ischemia: evolution of MRI findings. J Clin Neurol 2012; 8: 218-223.
• 8. Novy J, Carruzzo A, Maeder P, Bogousslavsky J. Spinal cord ischemia. Clinical and imaging patterns, pathogenesis and outcomes in 27 patients. Arch Neurol 2006; 63: 1113–1120.
• 9. Mull M. Acute spinal cord ischemia: diagnosis without therapeutic options? Clin Neuroradiol 2005; 15: 79–88.
• 10. Loeys BL. Revised Ghent criteria for the diagnosis of Marfan syndrome (MFS) and related conditions. J Med Genet 2010; 47: 476-485.
• 11. Elmacı T. Kardiovasküler cerrahi girişimlerde spinal iskemi. Journal of Istanbul Faculty of Medicine 2011; 63(4).
• 12. Robertson CE, Brown RD Jr, Wijdicks EF, Rabinstein AA. Recovery after spinal cord infarcts: long term outcome in 115 patients. Neurology 2012; 78:114.
• 13. Ge L, Arul K, Stoner M, Mesfin A. Etiology and Outcomes of Spinal Cord Infarct: A Case Series From a Level 1 Trauma Center. Global Spine J 2020; 10(6): 735-740.