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  • Updated 10.21.2020
  • Released 02.08.2000
  • Expires For CME 10.21.2023

Superficial siderosis

Introduction

Overview

The author explains the clinical presentation, pathophysiology, prevention, diagnostic workup, and management of superficial siderosis of the central nervous system. Superficial siderosis is a potentially devastating syndrome usually caused by recurrent subarachnoid hemorrhage with accumulation of hemosiderin and other iron-containing pigments in the leptomeninges, brain surface, brainstem, cerebellum, cranial nerves, and spinal cord. Superficial siderosis can mimic many other central nervous system degenerative or multifocal disorders, including amyotrophic lateral sclerosis and multiple sclerosis.

Key points

• Superficial siderosis is a rare and potentially devastating syndrome caused by recurrent subarachnoid hemorrhage with accumulation of hemosiderin and other iron-containing pigments in the leptomeninges, brain surface, brainstem, cerebellum, cranial nerves, and spinal cord.

• Common features include progressive sensorineural hearing loss, cerebellar ataxia, pyramidal signs (eg, spastic paraparesis, quadriparesis, etc.), ataxia, and headache.

• Superficial siderosis is caused by recurrent subarachnoid hemorrhage with dissemination of heme by circulating cerebrospinal fluid and subsequent accumulation of hemosiderin and other iron-containing pigments in the leptomeninges, brain surface, brainstem, cerebellum, cranial nerves, and spinal cord.

• The condition may be progressively disabling or fatal. Even with successful surgical resection of the causative lesion (when that is identified), significant functional recovery cannot be anticipated. However, progression generally is lessened or averted, at least in the short periods of follow-up reported.

• A wide variety of conditions may cause superficial siderosis, including various cerebral or spinal vascular abnormalities, neoplasms or other mass lesions of the brain and spinal cord or surrounding structures, trauma, and rarely some neurosurgical procedures, congenital abnormalities, and other sources of vascular damage.

• Management is directed at resection of the source of bleeding.

Historical note and terminology

Superficial siderosis is a rare and potentially devastating syndrome caused by recurrent subarachnoid hemorrhage with accumulation of hemosiderin and other iron-containing pigments in the leptomeninges, brain surface, brainstem, cerebellum, cranial nerves, and spinal cord (51). Although superficial siderosis had been produced experimentally in dogs as early as 1960 (36), superficial siderosis had been considered a rare condition, usually diagnosed postmortem, until the advent of CT and MRI in the mid-1980s (73). It is now increasingly recognized.

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