Three Cases of Superficial Siderosis

J Korean Neurol Assoc > Volume 14(3); 1996 > Article

Journal of the Korean Neurological Association 1996;14(3): 848-854.

표재성 철침착증 3례

김병곤, 노재규, 전범석, 한문희*

서울대학교 의과대학 신경과학교실, 방사선과학교실*

Three Cases of Superficial Siderosis

Byung-Gon Kim, M.D., Jae-Kyu Roh, M.D., Beom-Seok Jeon, M.D., Moon-Hee Han, M.D.

Department of neurology and Neuroradiology*, College of Medicine, Seoul National University

Abstract

Superficial siderosis is a rare condition characterized by hemosiderin deposition in leptomeninges, subpial tissue, brainstem, cerebellum, spinal cord, and cranial nerves. Slowly progressive hearing loss and gait ataxia are invariable clinical manifestations. We report three patients with their clinical and radiological features. All patients presented with hearing loss and cognitive dysfunction. Two showed gait ataxia and myelopathic symptoms and signs. Decreased visual acuity, hand tremor, limb ataxia, dysarthria, and nystagmus were also present. All patients showed typical MRI findings: marked linear hypointensities around the cerebellum, brainstem, and the surface of the cerebral cortex, especially in sylvian fissures. Two patients had brain tumors : pituitary adenoma and oligodendroglioma. Another patient had no definite bleeding source. Hemosiderin deposition is caused by chronic and recurrent subarachnoid hemorrhage derived from tumor, vascular malformation, aneurysm, posthemispherectomy, and unknown bleeding sources. Diagnosis is easily made by characteristic clinical manifestations and MRI findings. The selective vulnerability of the cerebellum and the 8th cranial nerve depends upon their own histological and biochemical characteristics. Benefits of the iron chelating agents are questionable. Removal of the possible bleeding sources is the most reliable strategy to prevent the disease progression.