Craniospinal Hypotension

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Craniospinal Hypotension, also known as Intracranial Hypotension is not an actual symptom of Superficial Siderosis. It presents as a secondary result of the traumatic event (accident or surgery) which caused your Superficial Siderosis. Craniospinal Hypotension is a condition caused by a dural leak of your cerebral spinal fluid. Your body produces spinal fluid every day, but if there is a leak, then your system may not be able to replace enough volume to support and cushion your brain. This low volume creates a negative pressure inside your brain cavity.

“This was my first true symptom. I could not effectively describe what was happening to doctors. I had double sciatica at the same time, so it was dismissed. I kept it to myself and knew something was seriously wrong.

 

The pain came on in a split second, felt as if, it traveled from the bottom of my spine, shot to my brain, instantly had excruciating head pain and would collapse. I had no control of any part of my body, just limp. Lying horizontal seemed to stabilize and residual pain lasted for, at least 24 hours. I was told that my eyed rolled back as if, I was having a seizure with no body movement.

 

After diagnosis, the search was on for the cause of the iron deposits. It was a dural tear that leaked so fast the location could not be pinpointed exactly. Had two blood patches and glue patch that were unsuccessful.

 

After a year of searching, Drs at Mayo agreed to try surgery. Had a laminectomy T-3 thru T-8, dura had a 1″ x 1/8″ tear repaired. It has never happened since. The pain SIH caused brought me close to suicide. -Jennifer Soellner”.

 

The signs of craniospinal hypotension are a postural headache, nausea, vomiting, neck pain, visual and hearing disturbances, and vertigo. Diagnosis is dependent on the postural component of a headache; pain increases or eases with positional changes of your head and body.

When symptoms are significant, the first choice is an epidural blood patch. In this procedure, some blood is taken from the patient’s arm vein and is injected into the spinal canal in the space outside the dura. Epidural patching sometimes involves the use of fibrin sealant. These procedures may be repeated several times if the improvement is incomplete or does not last.

If a blood patch procedure is not effective you may have to have a neurosurgeon attempt to close your dural defect with surgery. Your dural leaks location will not be identified in some cases but if surgery is successful in finding and closing the leak the headaches will stop.

Updated: July 3, 2018

Sources:
Amrhein TJ, Befera NT, Gray L, Kranz PG. CT Fluoroscopy-Guided Blood Patching of Ventral CSF Leaks by Direct Needle Placement in the Ventral Epidural Space Using a Transforaminal Approach. AJNR Am J Neuroradiol. 2016 Jul 7;(37):1951-6.
Beck J, Ulrich CT, Fung C, Fichtner J, Seidel K, Fiechter M, et al. Diskogenic microspurs as a major cause of intractable spontaneous intracranial hypotension. Neurology. 2016 Aug 26;87(12):1220-6.
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
Living With SuperficialSiderosis Website PubMed Reference Library