Welcome,

Amyotrophic lateral sclerosis (also known as Lou Gehrig's disease) is a devastating neurodegenerative disease, those living with the disease become progressively paralyzed due to degeneration of the upper and lower motor neurons in the brain and spinal cord. Eighty per cent of people with ALS die within two to five years of diagnosis - unable to breathe or swallow. Along with ALS, neurodegenerative diseases include Alzheimer's disease, Huntington's disease and Parkinson's disease. According to the World Health Organization, neurodegenerative diseases are predicted to surpass cancer as the second leading cause of death in Canada by 2040.

ALS has no known cure or effective treatment. For every person diagnosed with ALS a person with ALS dies. Approximately 2,500 - 3,000 Canadians currently live with ALS.

The ALS Society is committed to:

Support research towards a cure for ALS.
Support provincial ALS societies in their provision of quality care for persons living with ALS.
Build public awareness of ALS and its impact.

A Randomized Clinical Trial of Lithium Carbonate with Riluzole versus Placebo with Riluzole in ALS Shows No Benefit

In February 2008, Dr. Francesco Fornai and colleagues at the University of Pisa, Italy, reported in a pilot study that lithium carbonate at dosages of 300-450 mg daily (titrated to a plasma level of 0.4-0.8 mEq/liter) combined with riluzole showed a large positive effect in people with ALS (Fornai, F., et al., Lithium delays progression of amyotrophic lateral sclerosis. PNAS, 2008.105(6): p. 2052-2057).

To further investigate lithium carbonate as a possible treatment for ALS, a randomized, blinded, multicenter trial of lithium carbonate with riluzole versus placebo with riluzole was conducted in people with ALS in the U.S. and Canada. The study used similar dosing to the Italian study. The study was conducted by the Northeast ALS (NEALS) and Canadian ALS (CALS) Consortia and was sponsored by the National Institute of Neurological Disorders and Stroke (NINDS) of the National Institutes of Health, the ALS Association and the ALS Society of Canada. This unique collaboration between investigators and funding organizations resulted in a novel study design and expeditious execution of the trial to efficiently answer a critically important clinical question. Study leaders included Drs. Swati Aggarwal, Lorne Zinman, Jeremy Shefner and Merit Cudkowicz.

An interim analysis was conducted after enrollment of the 84th subject and presented to the NINDS Data and Safety Monitoring Board in September 2009. Based on the interim analysis the trial was stopped for futility. This study did not show the same beneficial effect of lithium carbonate on the progression of ALS as the prior pilot study conducted in Italy.

Although the results are disappointing, it was very important for the ALS community to quickly and efficiently determine if the large benefit first observed for lithium could be replicated in a well controlled trial. With the ongoing assistance and commitment of patient volunteers, researchers can now focus on other promising therapeutics for patients with ALS.

Recent ALS Information, Research Updates, and Related Articles:

1.	Synapse formation can be triggered artificially

The formation of synapses – the junctions through which neurotransmitters relay chemical signals allowing neurons to communicate information – typically requires contact between presynaptic and postsy ◊

"Synapse formation can be triggered artificially"

2.	Phase 3 talampanel trial begins

Talampanel, a drug currently not approved in Canada or the United States, is undergoing an international Phase 3 trial involving 500 patients with ALS in centres across Europe, Canada and the US. In a ◊

"Phase 3 talampanel trial begins"

3.	Genetic contributors to motor neuron diseases

The recent discovery of a number of genes known to cause motor neuron diseases such as ALS has provided new insights into the mechanisms underlying motor neuron degeneration, according to a recent art ◊

"Genetic contributors to motor neuron diseases"

4.	Defining survival as an outcome measure for clinical trials in ALS

Clinical trials in ALS often use patient survival as an outcome measure to examine whether a treatment prolongs life. But variables affecting survival rates – such as emergency tracheostomy – are dif ◊

"Defining survival as an outcome measure for clinical trials in ALS"

5.	Encouraging trial results for KNS-760704

At the 20th Internal Symposium on ALS/MND, researchers presented the results of a Phase 2 clinical trial testing the safety and tolerability of a drug called KNS-760704 for use by ALS patients. KNS-7 ◊

"Encouraging trial results for KNS-760704 "

6.	Molecule discovered that might help ALS Patients

University of Texas researchers identified a molecule that reduces symptoms and prolongs life in mice with a type of ALS, according to a study in the Dec. 11, 2009, issue of Science. Lead author Eric ◊

"Molecule discovered that might help ALS Patients"

7.	Misfolded SOD1 not a feature of sporadic ALS

Abnormal folding of superoxide dismutase 1 (SOD1) – a protein known to gain harmful properties from heritable gene mutations in familial ALS – does not appear to cause cases of familial ALS without SO ◊

"Misfolded SOD1 not a feature of sporadic ALS"

8.	TDP-43 mutations cause motor deficits in zebrafish

Mutations in the TAR DNA-binding protein (TDP-43) gene cause motor neuron defects in zebrafish and lead to impaired swimming, researchers from Université de Montreal report. Interestingly, insufficie ◊

"TDP-43 mutations cause motor deficits in zebrafish"

9.	Evidence for ALS as a disorder of RNA metabolism

Defects in RNA binding might represent a disease mechanism common to all cases of ALS, a leading Canadian researcher reports. ◊

"Evidence for ALS as a disorder of RNA metabolism"

10.	Formaldehyde exposure linked to ALS risk

Formaldehyde exposure is associated with an increased risk for ALS, according to a longitudinal study conducted by Mark Weisskopf, PhD, ScD, and colleagues from the Harvard School of Public Health, ◊

"Formaldehyde exposure linked to ALS risk"

11.	Cognitive and behavioural dysfunction in ALS

Amyotrophic lateral sclerosis – a disease once thought to only rob patients of their ability to move – is now recognized as having cognitive and behavioural effects in one out of two patients. This o ◊

"Cognitive and behavioural dysfunction in ALS"

12.	ALS patients with SOD1 mutations are less likely to have cognitive deficits

Patients with familial ALS (FALS) caused by mutations in the SOD1 gene are less likely to exhibit cognitive changes than those without SOD1 mutations, according to a report published in The Journal of ◊

"ALS patients with SOD1 mutations are less likely to have cognitive deficits"

13.	Invitation to Submit a Letter Of Intent

Invitation to Submit a Letter Of Intent(LOI) - National Population Health Study of Neurological Conditions // Invitation a presenter une lettre d'intention -Étude nationale de la santé des population ◊

"Invitation to Submit a Letter Of Intent"

14.	NRP Priority Announcement

NRP Priority Announcement ◊

"NRP Priority Announcement"

15.	Gene variant implicates faulty cellular secretion in ALS

A variation in the chromogranin gene may confer susceptibility to ALS and accelerate disease onset, researchers from Laval University report. ◊

"Gene variant implicates faulty cellular secretion in ALS"