Three Cases of Superficial Siderosis.
- Author:
Byung Gon KIM
1
;
Jae Kyu ROH
;
Beom Seok JEON
;
Moon Hee HAN
Author Information
1. Department of Neurology, College of Medicine, Seoul National University.
- Publication Type:Case Report
- MeSH:
Adenoma;
Aneurysm;
Ataxia;
Brain Neoplasms;
Brain Stem;
Cerebellum;
Cerebral Cortex;
Cranial Nerves;
Diagnosis;
Disease Progression;
Dysarthria;
Gait Ataxia;
Hand;
Hearing Loss;
Hemorrhage;
Hemosiderin;
Humans;
Iron Chelating Agents;
Magnetic Resonance Imaging;
Oligodendroglioma;
Pituitary Neoplasms;
Siderosis*;
Spinal Cord;
Subarachnoid Hemorrhage;
Tremor;
Vascular Malformations;
Visual Acuity
- From:Journal of the Korean Neurological Association
1996;14(3):848-854
- CountryRepublic of Korea
- Language:Korean
-
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.
