New treatment for ALS 5 years away, doctor predicts

Doctor Predicts ALS Treatment in 5 Years

Ottawa Citizen

By Rebecca Wigod
Monday November 15, 1999
Copyright 1999 Ottawa Citizen

Canadian medical researchers have made a breakthrough they believe will lead to new treatments for Lou Gehrig's disease within five years.

Dr. Michael Strong has replicated in human tissue a finding about nerve cells affected by the disease that had previously been made only in mice.

''We've got a mechanism (by which the disease progresses). We've proven it in a mouse model. We've seen it in humans,'' Strong, an Ontario researcher and physician, said Friday in Vancouver.

''I don't think it's unreasonable to think this will have an impact (on therapy) in the next five years.''

Lou Gehrig's disease -- also called motor neuron disease and amyotrophic lateral sclerosis (ALS) -- attacks nerve cells, particularly those in the spinal cord. It doesn't affect the mind, but it weakens the arms, legs and throat, leading inevitably to paralysis and death.

In 1993, Victoria's Sue Rodriguez felt so strongly that ALS had made her life not worth living she took her fight for the right to a doctor-assisted suicide to the Supreme Court. The court ruled against her. Rodriguez eventually killed herself, with a physician's help.

Strong, who does research on the disease at the University of Western Ontario's John P. Robarts Research Institute, has been studying the ''rocks'' of neurofilament, or protein, that are found in nerve cells affected by ALS.

Recently, after more than 10 years of work, he discovered that human nerve cells affected by ALS are deficient in low-molecular-weight neurofilament -- one of the three main kinds.

He will present his research to 600 colleagues from around the world at the 1999 Motor Neuron Disease Symposium, which opens today in Vancouver.

His finding mirrors one made in mice by Dr. Jean-Pierre Julien, who works at the McGill University Health Centre in Montreal.

Julien's work, published this week in the Journal of Cell Biology, describes how he and fellow scientists manipulated genes in the mice, postponing the onset of ALS and extending the average mouse life span by six months.

''Six months in the life span of a mouse is equivalent to 20 years for a human being,'' Julien, who will present his findings here this week, said in Dialogue, the journal of the ALS Society of Canada.

Strong believes Julien's research team and his own have found out enough about what happens in ALS for scientists soon to be able to slow, or even reverse, the progress of the disease.

''To say we will stop the disease dead in its tracks, I don't know. But if we can change the tempo of the illness, I think we can allow recovery,'' said Strong, who not only does laboratory research but also runs a large clinic in which he looks after ALS patients.

North Vancouver's Harry Paddon hopes his wife, Helen, will be able to benefit from the therapeutic advances bound to flow from Strong's and Julien's discoveries.

Helen Paddon, 47, used to be a confidential secretary at the Labour Relations Board, but ALS robbed her of mobility and independence. She had to stop working in October 1996.

''There's got to be a way to fix this, to make this problem better,'' said her 47-year-old husband and caregiver.

Helen uses a power wheelchair. She can't even turn over in bed without Harry's help.

She couldn't say much about her hopes. ''I started losing my voice in July 1997,'' she explained in a cracked whisper.

She feels more hopeful about her prognosis than Rodriguez did, partly because Harry -- who has worked in laboratories and the biotechnology industry -- is optimistic.

Neurologist Dr. Andrew Eisen, president of the ALS Society of B.C., looks after Helen. He said there are 60 to 75 genetic mutations that cause the disease.

Helen, who has Finnish ancestry, has an unusual mutation called D90A, seen mostly in Finland and Sweden. She has lived with ALS for 12 years, whereas many patients die within three years.

Eisen said ALS is an extremely complicated condition that may actually be present in affected people from birth. Symptoms come on slowly and then there is an event in the body, called ''the final cascade'' that accelerates it terribly.

''I don't think a single medicine is ever going to be the answer,'' Eisen said. ''It's going to have to be some kind of cocktail.''

More than 3,000 people in Canada live with ALS.

Copyright 1999 Deutsche Presse-Agentur
September 20, 1999, Monday, BC Cycle
02:08 Central European Time
LENGTH: 682 words
BYLINE: By Carsten Wieland
DATELINE: Tubingen

The important cable is attached to the crown and sends the variation in Hans-Peter Salzmann's brain waves to a computer. Seven further electrodes send signals from the paralysed 44-year-old to the processor.

His eyes fix on a ball on the monitor. If Salzmann thinks of peace, the ball travels down the screen; if he thinks of hectic it bounces upwards. For one year, he has been choosing letters in this way, becoming possibly the first man in the world who can write without expending any muscular energy. Researchers at Tubingen University have made this possible with their unique Thought-Transfer Machine.

Professor Niels Birbaumer and his team at the Institute for Medical Psychology and Behavioural Neurology work with patients suffering from amyotrophic lateral sclerosis (ALS), a degenerative nervous-system disorder. The British physicist Stephen Hawking suffers from ALS, and first brought the disease to public attention.

In ALS the nerve cells that transmit impulses between the brain and the muscles - the motor neurons - are gradually killed off. In advances stages only the heart is left beating and metabolism intact.

For the past six years Salzmann has been unable to breathe unaided, air being fed into his through an incision in his windpipe. In his case, the so-called Locked-in Syndrome is complete. And yet Salzmann can still recognise colours, pitch and feelings even if he cannot pass on his impressions on his own.

Very few ALS patients are in fact despondent. "Only in the initial phases of artificial respiration can depression arise," says Birbaumer. The ability to communicate can do wonders for a person's morale. "When they can no longer do that, they usually give up," says the psychologist.

This is where his Thought-Transfer Machine comes in. It raises the quality of life for these prisoners of their own bodies. Birbaumer explains the slight tendency to depression thus: "The brain can compensate for many deficiencies. The things which remain for the patient come to the fore. For example, in blind people, hearing becomes far more important."

"Salzmann always comes across as being very cheerful," says psychologist Nicola Neumann, who looks after him. "It is important for him to learn to write with the new system, which has provided with a challenge."

Salzmann, who was a lawyer in Stuttgart before the terrible disease cut short his career, could only communicate to people through blinking his eyelids before he started using Dr Birbaumer's machine on his own. After just one week, a person can learn to control up to 80 per cent of their low-range brain frequencies, says Birbaumer.

"The patients are all dying to write on it," says fellow researcher Thilo Hinterberger. They all find simple tasks, such as turning a light on and off by brain signals, boring after a short while. At present, three patients in Stuttgart, Tubingen and Mannheim have managed to write letters using the software.

In Munich and Berlin ALS patients are also trying to control their brain signals using the invention from Tubingen. Several have travelled there especially for trials. And further afield, a Swiss sufferer recently showed interest as well.

The price of a machine is still around 10,600 dollars. Hinterberger hopes that they will soon become smaller and cheaper. The special writing software has already been introduced to the international scientific community by Nature magazine (Vol. 398, p. 297). Currently, the team are working on ways to help ALS sufferers surf the Internet.

Through their experience with Salzmann, the researchers have been able to improve the program. "The most important challenge is now that the patients be able to write quicker," says Hinterberger. At the moment, one word can take up to an hour to produce on the screen.

Salzmann once told Neumann that he had discovered slowness through the disease. His carer remembers how his first letter took several days. "It was a thank-you-letter to Professor Birbaumer and an invitation to us all to celebrate at his house."