Decoding The Superficial Siderosis MRI

MRI Superficial Siderosis5

Tracking Your Hemosiderin   There has been a definite increase in people diagnosed with superficial siderosis. The improvements in neuroimaging have resulted in advanced iron sensitive 2-D and 3-D MRI techniques. Thankfully you can now be diagnosed in vivo and, if you’re fortunate, early enough to do something. Researchers have now identified three branches of superficial siderosis, each with a unique clinical presentation and pathology. Infratentorial Superficial Siderosis (iSS) Type1 Classical is the superficial siderosis which affects our group. Clinically it presents with hearing loss, ataxia, myelopathy and slow progressing neurodegeneration. Infratentorial Superficial Siderosis (iSS) Type 2 Secondary will show classic hemosiderin staining on the MRI but will not present with any clinical symptoms or degeneration. The newcomer is Cortical Superficial Siderosis (cSS). Hemosiderin deposition is limited to cortical sulci of the cerebral hemispheres. The cerebellum, brain stem, and spine escape deposits. Cortical Superficial Siderosis (cSS) has different clinical symptoms and causes. It seems to be age related with a connection to cerebral small vessel disorders. Annual MRI   Gary is in his fourth year now of chelation therapy. His first dose of Ferripriox was in July, 2014 and his original hematologist always made sure he ordered annual MRI scans of his head and spine.…

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Understanding Ferriprox

Ferriprox-deferiprone

There is some confusion when it comes to understanding Ferriprox and superficial siderosis. Ferripriox (Deferiprone) is a prescription drug whose primary purpose is treating people who have transfusional iron overload due to Thalassemia syndromes. Deferiprone was designed to bind to this iron and remove it in a process called Chelation therapy. It’s my understanding if a person suffers from iron overload they feel better when chelation lowers iron levels. Superficial siderosis patients do not have too much iron in their bloodstream. They have iron deposits (hemosiderin) stuck to areas of their brain (most often in the cerebellum or spinal cord) that are the result of blood infiltrating into their central nervous system from a trauma. An accident, surgery, stroke, etc., but somehow blood was introduced into their spinal fluid. The average person will naturally absorb and remove this blood. A rare few people will not be able to remove it. This iron is toxic to nerve function and ultimately can end in nerve death. There are many other prescription iron chelation medications on the market. Studies have shown Ferriprox as the only prescription drug, at this time, able to cross the blood-brain barrier so it might have a chance at removing hemosiderin…

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Neurodegenerative Vision Problems

neurodegenerative vision problems

When Your Eyes Have A Mind Of Their Own You move your eyes more than your heart beats. Vision problems often are one of the mysterious, unexplained symptoms people are plagued with long before their doctor knows what is wrong. While not as frequent as sensorineural hearing loss or balance issues a large percentage will suffer from some vision disturbance. Anisocoria, diplopia, nystagmus, dry eyes, or phantom images in your peripheral vision should all sound an alarm. An experienced ophthalmologist will recognize you may be exhibiting an early symptom of a neurodegenerative disorder and suggest neurology consults. Superficial Siderosis patients may have at least one of these symptoms or they can be an overachiever like Gary. At one time he was experiencing every vision problem we just listed. Years earlier Gary’s optometrist had diagnosed early dry macular degeneration in one eye so he always has regular screenings. I see an ophthalmologist, so I mentioned his vision problems during my appointment. In the beginning First were his complaints of double vision and problems with depth perception. He would see things out of the side of his eye, moving or stationary, that wasn’t there. Driving our backroad after an errand Gary was…

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Tips For Developing Great Patient-Provider Communication Skills

Patient Provider Communication Skills

Communication must be a two-way street Rare disease patients often battle a variety of complex clinical symptoms. A visit to your specialist often results in as much confusion by the end of your appointment as at the beginning. Healthcare providers are often under pressure to keep patient face time to a minimum, so clear communication is a vital step in understanding your plan of care. Your doctor needs accurate information to help you. It’s essential patients with short-term memory problems or mild cognitive impairment pre-plan for what they need or expect to learn during their appointment. Gary keeps a small daily notebook where he records his vitals, notes about medication or symptom changes, fluctuations in blood pressure, pain levels and new questions. Without this health diary, he is unable to remember significant details or forgets essential questions whether an appointment is a few months down the road or tomorrow. He brings his notebook to every regular office or hospital visit. Physicians find details with dates and times very helpful. It also helps if you can tell the story of events leading up to a specific incident. I was doing my usual ____ late in the afternoon, and then I was doing…

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The Power Of Community

power of community

We’re proud to publish the first of many collaborations with blogger and Superficial Siderosis activist Rori Daniel. Find her powerful, insightful and informative blog site here. –  thesilentbleed.co.uk   Fear is a powerful emotion. Anxiety shows no sympathy. Fear destroys if you give it power. A rare disease diagnosis, especially one as unknown as Superficial Siderosis, forces you to face your worst fears. It’s hard. We are not built to face fear alone. Thankfully, early patients and caregivers sparked the creation of our most powerful resource. Community. Your future has changed so how should you plan? How is your family going to be affected? Where should you look for care?. Chronic illness brings countless unanswered questions. The very nature of superficial siderosis or any rare disease should make finding your community a priority. One person may struggle to search for answers, but there is an astounding well of knowledge waiting for your questions. The wisdom of community is powerful. You’ll find your community happy to offer support no matter if you’re facing a new medical procedure, fighting through pain,  dealing with a new symptom or need a …….read more here

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Our Superficial Siderosis Community: Meet Robert Cameron

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  Robert Cameron has seen the world. Graduating with a degree in Marine Transportation from the United States Merchant Marine Academy he rose through the ranks and took command of his first vessel in 1980. A voyage that would continue for more than 25 years, Captain Robert traveled the world; Australia, New Zealand, South Africa, Japan and the United States making memories for a lifetime. A meningocele was found wrapped around the nerves at the T-4 area of Roberts’ spine. The discovery led to a 12-hour surgery and resulted in an uncontained slow bleed. In 2005, a diagnosis of superficial siderosis changed his course. Retirement finds him splitting his time between Florida, where he is he under the care of Dr. James Meschia, Head of Neurology Jacksonville Mayo Clinic and his summer home on Nantucket island. Opting to give chelation therapy a chance, he feels there has been a slowing of the progression and even seen some improvement. Not one to let an opportunity slip past he decided to battle back against memory loss issues and exercise his mind by tackling his lifelong dream of writing. During his time as a sea captain, he was witness to the beautiful marine…

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The Common Thread Searching For The Unexplained

common thread

Diagnosis of superficial siderosis is rare. It’s so unique in fact if you searched for information on one of the national or international rare disease databases until the last few years it wasn’t listed.  We should be glad so few will face a diagnosis of superficial siderosis, but you can’t help but wonder. Is there a common thread to be found? I find myself thinking about this subject a lot. Dr. Levy has said they don’t know why some people are overwhelmed by the blood infiltration. An unlucky spin of the wheel. We’re not medical professionals but what if we brainstorm a little and toss around a few ideas. First, let’s talk about what we do know. Superficial Siderosis is not hereditary; you aren’t born with it. Superficial Siderosis is not contagious; you can’t spread it or catch it.  Age or gender do not play a significant role in the diagnosis; patients were diagnosed, in the past, at an older age due to the slow nature of the progression, but newer MRI technology is making earlier detection possible. All confirmed superficial siderosis patients had experienced unnatural blood infiltration into their central nervous system; trauma, surgery, aneurysm or stroke that caused long-term bleeding.…

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Exploring Ayurveda For Cerebellar Ataxia And Pain Control

Western medicine is innovative, but if you search through PubMed, you find very few drug trials for degenerative cerebellar ataxia. There is no known cure for ataxia, so our best hope still lies in finding a therapy to ease the symptoms. With all the modern innovations available what if one possible answer lies in an ancient system of holistic healing? Ayurvedic medicine deserves a look. “Ayurveda” comes from the Sanskrit words Ayur (life) and Veda ( knowledge) and is the oldest (5,000-year-old) recorded system of medicine in history. It combines the concept of whole health; lifestyle, exercise, diet along with medicinal herb compounds, some which predate written history. Many traditional therapies are now being studied in a clinical setting using western methods of research, double-blind trials, and research review to prove or disprove their efficacy. The most promising trial we’ve previously highlighted is Degenerative Cerebellar Ataxia After Ayurvedic Therapy. In 2009 a joint study in India was conducted by the Department of Neurology, National Institute of Mental Health and Neurosciences, Advanced Ayurvedic Research Unit, National Institute of Mental Health, and the National Department of Neurosciences, Psychiatric and Neurological Rehabilitation.  In this study, they sought to discover if traditional Ayurvedic medicine could have a positive effect on balance issues caused by…

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20 Strength Exercises When You’re Fighting Mobility Problems

bands exercise

  Finding a user-friendly exercise program for patients diagnosed with related ataxia symptoms isn’t easy. For example, Gary is limited to lifting 10lbs so he doesn’t cause fresh bleeding into his CNS. Standing or sitting with free weights is a no-no; even without the lifting limit, you wouldn’t want to be the spotter with his balance problem. Before his nerve block, getting down on the floor to do planks, sit-ups or leg lifts would bring an instant migraine. Exercising was a minefield. Gary’s appointment for his balance and gait evaluation gave us the opportunity to ask the kinesiology department these questions. He needed a low-impact strength program designed so it: Could be done at home Wouldn’t trigger headaches Balance problems wouldn’t interfere Muscle tone could be strengthened or maintained   The physical therapist built a program with ten strength exercises, each set targeting the upper or lower body using resistance bands. If you’ve never used a resistance band during exercise, I think you will be surprised with their efficiency. The therapist first demonstrated each activity and then had Gary repeat it. Most are done sitting on a chair. A few of the more advanced exercises require holding on to a…

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What’s Your Fall Risk?

ataxia gait balance falls

If you’re living with a gait and balance impairment, this is a familiar scenario. You’re innocently walking, minding your own business, and out of nowhere an angry rogue floor knocks you down. No reason, just walking, then BOOM,  down you go. Laughing about a rogue surface helps lighten an embarrassing situation, but mobility problems can impair your ability to exercise, put you at risk for sprains, bruises or worst case, breaking a bone. Gary’s gait and balance have slowly been declining. His balance is not as bad as his gait on flat surfaces, but if he’s on uneven ground or attacked by an angry floor its impossible to stop a fall. His neurologist felt it was important he start a physical therapy program to maintain muscle strength. His PCP thought it might be time to transition from a cane to a walker. Cerebellar degeneration or atrophy is often the underlying cause of cerebellar ataxia. The cerebellum plays a significant role in coordinating your fine motor function, gait, balance, vision, and swallowing. Cerebellar ataxia will also cause fatigue, affect your executive function, personality, mood, and increase your risk of depression. Yes, There Will Be A Test We see many rotating triage nurses monthly, and they’re required…

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