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 many times completely disabling. Over-the-counter pain medications will have little to no effect, and in many cases, prescription pain medications have limited success.

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 are documented in superficial siderosis. 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.

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. Your pain and stiffness in your neck area is the body’s response to the swelling.

“….The stimulation of the trigeminal nerve causes blood vessels in the coverings of the brain to dilate, and this allows white blood cells to leak into the surrounding space. If you obtain some spinal fluid and look at it under the microscope, you may see white blood cells, which means that you see a little pus in the spinal fluid, just as if you had an infected wound. So, migraine produces inflammation in the brain’s coverings, just like poison ivy produces a rash on the skin or a sinus infection produces inflammatory changes in the nose. This event is called “sterile inflammation”; the sterile part refers to the fact that the meninges are inflamed merely from the continued stimulation of the nerve…”
-Dr. Charles Matthews, Neurologist,  Director of the North Carolina Comprehensive Headache Clinic



Updated: November 7, 2020

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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