Superficial siderosis

Douglas J Lanska MD FAAN MS MSPH (Dr. Lanska of the Great Lakes VA Healthcare System and the University of Wisconsin School of Medicine and Public Health has no relevant financial relationships to disclose.)
Originally released February 8, 2000; last updated February 4, 2017; expires February 4, 2020

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 (Lanska 2014). Although superficial siderosis had been produced experimentally in dogs as early as 1960 (Iwanowski and Olszewski 1960), superficial siderosis had been considered a rare condition, usually diagnosed postmortem, until the advent of CT and MRI in the mid-1980s (Pinkston et al 1983; Pribitkin et al 1994; Fearnley et al 1995). It is now increasingly recognized.

The content you are trying to view is available only to logged in, current MedLink Neurology subscribers.

If you are a subscriber, please log in.

If you are a former subscriber or have registered before, please log in first and then click select a Service Plan or contact Subscriber Services. Site license users, click the Site License Acces link on the Homepage at an authorized computer.

If you have never registered before, click Learn More about MedLink Neurology  or view available Service Plans.