Bracchi M, Savoiardo M, Triulzi F, Daniele D, Grisoli M, Bradac G B, Agostinis C, Pelucchetti D, Scotti G
Department of Neuroradiology, University of Torino, Italy.
AJNR Am J Neuroradiol. 1993 Jan-Feb;14(1):227-36.
To report the clinical and neuroradiologic findings of superficial siderosis of the CNS, due to chronic subarachnoid bleeding of unknown origin.
We observed seven cases. The main clinical manifestations were progressive deafness and ataxia. Four patients had had previous cranial or cervical trauma, with root avulsion in two, many years before onset of deafness and ataxia. Neuroradiologic studies included MR (0.5 T in four and 1.5 T in three) and angiography of the brain in all cases, CT in six cases, MR of the spine in six, and myelography in four.
MR demonstrated a rim of marked hypointensity in T2-weighted images, consistent with hemosiderin deposits, on the surface of cerebellum, brain stem, inferior part of cerebral hemispheres, and spinal cord. CT showed cerebellar atrophy in five cases, and a rim of mild hyperdensity around the brain stem in two. Angiographic studies were negative. Myelography showed cervical nerve root avulsion in two cases and a cervicodorsal extradural cyst in one. Cerebrospinal fluid contained RBCs in all the six examined cases.
Although CT may occasionally suggest the diagnosis of superficial siderosis, MR demonstrates this abnormality to better advantage.
报告因不明原因慢性蛛网膜下腔出血导致的中枢神经系统表面铁沉积症的临床及神经放射学表现。
我们观察了7例患者。主要临床表现为进行性耳聋和共济失调。4例患者既往有颅脑或颈部外伤史,其中2例有神经根撕脱,发生在耳聋和共济失调出现前多年。神经放射学检查包括MR(4例为0.5T,3例为1.5T),所有病例均行脑血管造影,6例行CT检查,6例行脊柱MR检查,4例行脊髓造影。
MR在T2加权像上显示小脑、脑干、大脑半球下部及脊髓表面有一圈明显的低信号,符合含铁血黄素沉积。CT显示5例有小脑萎缩,2例脑干周围有一圈轻度高密度影。血管造影检查均为阴性。脊髓造影显示2例有颈神经根撕脱,1例有颈胸段硬膜外囊肿。6例脑脊液检查均发现有红细胞。
虽然CT偶尔可提示表面铁沉积症的诊断,但MR能更清楚地显示这种异常。