Thursday, May 26, 2011

Restricted Blood Flow, Moyamoya Disease and MS


May 26, 2011


From my friend Mark--someone with MS who was treated at Stanford after my husband--a story in his local paper caught his eye this morning.

When they first met in 1991, Corona residents Douglas Wang and Lizabeth Tso didn't know of the love and heartache that would follow them over the next 20 years.

Eight years later, they met a second time and fell in love.

In December 2001, Wang was diagnosed with multiple sclerosis. Nine months after that, at age 28, he suffered a massive stroke that left him paralyzed on his right side. He was then diagnosed with Moyamoya disease, a rare disorder that constricts blood flow to the brain.

Mark wondered, what the heck is Moyamoya Disease, and how is it like MS?  
Turns out, this disease, which restricts blood flow in the brain, is often MISDIAGNOSED as MS.

Moyamoya is a disease of the intracranial arteries which deliver blood inside the brain.  CCSVI is a disease of the extracranial veins.  But the mechanism of injury is hypoxia, not the immune system.

And to add another interesting layer to this tale...here is research from Stanford University, where Dr. Steinberg looked at pwMS and those with Moyamoya.


Misdiagnosis of Moyamoya Disease as Multiple Sclerosis Variants in a Case Series of Seven Patients
Omar Choudhri MD; Teresa Bell-Stephens RN; Robert M. Lober MD PhD; Nancy Fishbein; Gary K. Steinberg MD, PhD

Introduction
Multiple sclerosis is a relatively common demyelinating condition of the brain and spinal cord that can present insidiously with multiple neurological symptoms.Some multiple sclerosis symptoms could be mimicked by transient ischemic attacks in the setting of a vasculopathy such as moyamoya disease.

Moyamoya disease is an idiopathic chronic cerebrovascular disorder, characterized by progressive occlusion of vessels comprising the circle of Willis and clinical presentation can vary depending on the ischemic territory. Here we present a series of patients managed as having multiple sclerosis for many years before being correctly diagnosed with moyamoya disease.

This study investigates the radiographic, laboratory and clinical overlap between the two clinical entities and outcomes after surgical revascularization.

Methods
Seven patients found in retrospective review of the 448 patients in the Stanford Moyamoya Database from 1991-2010 to have initial diagnosis of multiple sclerosis on presentation. MRI imaging, angiograms, CSF results and clinical follow-up data were reviewed.

Results
Male : Female ratio of our patients was 1:6 which correlates with female preponderence in both conditions. The average age of patientsmwas 35 years and duration of diagnosis for presumed multiple sclerosis varied between 6 months to 20 years. Extremity paresthesia was the most common neurological symptom in these patients. MRI periventricular FLAIR or T1 changes were the most common diagnostic clue to multiple sclerosis and a repeat MR angiogram or formal angiogram subsequently confirmed the diagnosis of moyamoya disease.

Greater than 60% of patients had improvement of their neurologic symptoms following cerebral
revascularization.

Conclusions
We believe that moyamoya disease should be considered in multiple sclerosis variants who present with paresthesias and do not respond to traditional immunosuppressive therapies.
Early diagnosis and revascularization procedures could help prevent further neurologic sequelae. Furthermore, subtle MRI findings should suggest obtaining a CTA/MRA or a formal angiogram to identify moyamoya vessels.

Given that Moyamoya is yet ANOTHER DIFFERENTIAL diagnosis for MS which involves the cerebrovasculature, I believe those with an MS diagnosis deserve to be tested with angiograms and MRVs, to learn if they have a vascular component to their disease.  The evidence continues to grow.

Joan

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