R. NICKBRYANMDS. JAMESZINREICHMD
Contrast-enhanced MRI scans of the brain revealed low-signal hemosiderin deposition on the surface of the brain that resembled a thin, black pencil line. The regions involved included the brainstem and the cerebellum.
Superficial siderosis, which was first described by Hamill1 in 1908, is a rare entity characterized by a combination of sensorineural hearing loss, cerebellar ataxia, dementia, and pyramidal signs. It is caused by chronic, sometimes intermittent, subarachnoid hemorrhage, often resulting in progressive mental deterioration.2 Pathologically, there is deposition of hemosiderin in those parts of the central nervous system that are in close proximity to cerebrospinal fluid (CSF), including meninges, subpial tissue, spinal cord, and cranial nerves. This deposition of hemosiderin is associated with gliosis, neuronal loss, and demyelination. It is thought that the cochlear nerve and the cerebellar cortex are particularly vulnerable owing to their accelerated ferritin synthesis.3
Diagnosis Quiz Case 1. Arch Otolaryngol Head Neck Surg. 2001;127(6):716. doi:
Customize your JAMA Network experience by selecting one or more topics from the list below.
Create a personal account or sign in to: