J Korean Neurol Assoc.  1996 Sep;14(3):848-854.

Three Cases of Superficial Siderosis

• Kim BG
• Roh JK
• Jeon BS
• Han MH

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.

MeSH Terms

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
Hemosiderin
Iron Chelating Agents