It is challenging to pinpoint the exact cause of superficial siderosis associated headaches. Multiple factors play into the reason, but one thing all patients have in common is the headaches linked to Superficial Siderosis are chronic, bring excruciating pain, and often completely disabling. Over-the-counter pain medications will have little to no effect, and in many cases, prescription pain medications have limited success.

Orthostatic Headaches

Intracranial Hypotension is a known factor in orthostatic headaches. Also referred to as postural or low-pressure headaches, orthostatic headaches have a unique positional characteristic. The pain will appear almost immediately while in an upright position but ease or go away entirely upon lying down.

Thunderclap Headaches

Thunderclap headaches are documented in superficial siderosis when hemosiderin is found on the supratentorial area of the brain. As the name suggests, pain arrives instantaneously like a clap of thunder with excruciating intensity presenting within the first minute. Nausea, numbness, weakness, vision, and speech disturbances may also appear during a thunderclap headache.

Occipital Neuralgia

These headaches will cause pain that affects the back or one side of the head and neck. People often have sensations of shooting, zapping, stinging, or burning.

Active Bleeds

For patients who still experience active bleeding, Dr. Levy has hypothesized a connection between the onset of a headache during the times you are experiencing an active bleed. Fresh blood infiltrates your central nervous system, irritating surface linings causing inflammation. As a result, the pain and stiffness in your neck area is the body’s response to the swelling.


A reaction to hyperreflexia stimulus in your autonomic nervous system may result in a throbbing headache.

Sources: Superficial siderosis is a rare neurologic disease characterized by progressive sensorineural hearing loss, cerebellar ataxia, pyramidal signs, and neuroimaging findings revealing hemosiderin deposits in the spinal and cranial leptomeninges and subpial layer. The disease progresses slowly, and patients may present with mild cognitive impairment, nystagmus, dysmetria, spasticity, dysdiadochokinesia, dysarthria, hyperreflexia, and Babinski signs. Additional features reported include dementia, urinary incontinence, anosmia, ageusia, and anisocoria. Superficial siderosis MedGen UID: 831707 •Concept ID: CN226971 •Finding Orphanet: ORPHA247245
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