Neurodegenrative diseases result in a progressive loss of function of neurons in the brain including the death of neurons.  Neurodegenerative diseases present differently with a range of symptoms that could lead to cognitive, physical, emotional and behavioural decline, depending on the disease.  As the population ages the incidence of neurodegeneration increases. An aging population may significantly add to the burden of treating and caring for those living with a degenerative brain disorder.

Some more prevalent neurodegenerative disorders in Canada include,  Alzheimer’s disease, Parkinson’s disease, multiple sclerosis,  amyotrophic lateral sclerosis (ALS) and Huntington disease.  For most diseases symptoms get worse over time and unfortunately few of the diseases are curable or have reliable treatments to stop or slow the disease progression.

Hunting for the cure   

Of the Neurodegenerative diseases Huntington disease (HD) is a particularly difficult disease that impacts generations of a family.  HD is a genetic disease, which means that at least one of the two copies of the huntingtin gene has a defect that can cause the disease. Parents with HD have a 50% chance of passing on the disease to each child, and with each generation, HD starts earlier and earlier in life. With a definitive genetic test, a person can know if they will get HD later in life, or not. 

"Huntington’s disease (HD) is a progressive brain disorder that affects 1 in 8000 people worldwide."

Symptoms usually appear in people between the ages of 30 and 45 but Juvenile HD can appear in young children and late onset HD can appear in senior adults.  HD causes neuron death in the brain and in time people with Huntington’s experience loss of motor control, cognitive decline and behavioural and emotional disturbances.  Where symptoms of Huntington disease may vary between individuals they do lead to incapacitation and death. Huntington’s disease (HD) is a progressive brain disorder that affects 1 in 8000 people worldwide.  

Unlike Parkinson’s or Alzheimer’s diseases, Huntington’s disease has only one definitive known cause, and is only caused by a defect in one gene. This is a huge advantage for researchers in understanding how an ageing brain degenerates in these types of diseases. Researchers have created accurate models of HD in mice that allow the animals to get disease in a manner similar to humans. This has not yet been possible with Parkinson’s or Alzheimer’s diseases.

Even with much lower incidence than other neurodegenerative diseases, HD research is far more advanced at understanding the exact mechanism of how brain cells are not working properly, leading to early loss.  Many of the pathways of cell loss seen in HD are now known to be seen in Alzheimer’s disease, suggesting that there may be some common pathways affected in brain cells for all of these neurodegenerative diseases, including Frontal-temporal dementia (FTD).

Finding the funding   

There are 5.5 million Canadians living with a brain disease, disorder or injury.   The dollar cost to government is staggering and the physical, emotional and economic costs to individuals and families, is frightening.  The time is right to identify opportunities and strategies to improve health care for all Canadians with neurological conditions.