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Stephen Hawking has a motor neurone disease called amyotrophic lateral sclerosis (ALS)  (Source:
Ongoing BioMOx study reveals nerve damage pattern in the brains of MND patients

The Motor Neurone Disease (MND) Association and the Medical Research Council (MRC), along with researchers from the University of Oxford, have joined forces to conduct the Oxford Study for Biomarkers in MND/ALS (BioMOx). This resulted in the identification of a common signature of nerve damage in patients who have MND. 

Dr. Martin Turner and his team from the University of Oxford have discovered a "unique similarity" between nerve damage found in the region of the brain which connects the motor neurones to the brain, and nerve damage found in the corpus callosum, which is a region of the brain that acts as a connection between the right and left sides. 

Until now, patients with MND have had to wait too long for a diagnosis of MND because MND research was hindered by the lack of predictable markers, or biomarkers, of the progression of the disease. Researchers also lacked an early diagnostic test. With the use of an MND biomarker, MND patients can be diagnosed more quickly and accurately, which is the main goal of the BioMOx study. 

"The BioMOx study is one of the largest biomarker studies for MND in the world," said Dr. Brian Dickie, director of research development at the MND Association. "It's very encouraging to hear the first exciting results emerging from this four-year initiative."

In this particular study, researchers scanned healthy brains as well as brains that contained MND damage using an advanced magnetic resonance imaging (MRI) technique. Every six months, researchers for the BioMOx study followed changes in the brain, in blood and in spinal cord fluid of people with MND. By scanning and comparing specific areas of the brain, Turner and his team were able to find a pattern of nerve damage within MND patients, which has led to the discovery of a MND biomarker. 

"The finding of a common pattern of nerve pathway damage in a varied group of MND patients holds the promise of a much needed biomarker," said Turner. "This study confirms the ability of advanced MRI techniques to sensitively detect nerve damage in a wide range of people living with MND. It builds on a decade of international work, and shows that MRI is now a frontrunner in the quest to generate biomarkers of disease activity in MND." 

Researchers hope to use what they've discovered in the BioMOx study to develop improved treatments for patients with MND. 

"MRI scanning provides an exciting 'window on the brain,' allowing researchers to link the changes occurring in the central nervous system with the 'real world' symptoms of MND," said Dickie. "Understanding these changing events is going to be central to the development of future treatments."

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RE: Hawking and ALS
By mac2j on 11/5/2010 12:58:31 PM , Rating: 2
Actually thats not entirely true - about 1-5% of ALS patients follow a similar course to Stephen Hawkings.

The long survival in the paralyzed state is largely a function of the round the clock supportive care he gets and the lack of lung involvement.

BUT you're correct - there are 3 or 4 major patterns of ALS from very rapid (less than 1 yr survival from diagnosis) to the Hawking type at the other extreme. These likely represent different underlying genetic causes with the same outcome - motor neuron death ... determining and understanding what these are is essential to curing this horrible disease.

RE: Hawking and ALS
By Malhavoc on 11/5/2010 2:05:41 PM , Rating: 2
My father was diagnosed with ALS 10-12 years ago but it is thought that symptoms started coming about 20+ years ago but were not very severe and not identified at the time. Progress of the diesase has been slow overall but has sped up over the last few years where he needs 24hr care.

In the last year or two some of his doctor's believe it is not ALS and something yet undiagnosed and one quack told him he'd walk again last year but the quack went and died.

I know of at least 2 others within 50 km radius that have similar longevity after being diagnosed with ALS.

RE: Hawking and ALS
By ssjwes1980 on 11/5/2010 9:41:53 PM , Rating: 2
My Grandfather had it for 16 years when he was 12 years in he started to seem to get better for about a year the Doctors could explain it but in the end it progressed again and killed him. Glad they are finding new stuff out

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