Neurodegeneration is a slow and progressive loss of neuronal cells in specified regions of the brain. This is associated with aging and leads to cognitive or motor disfunctions, depending on which neurons are affected. The causes are unknown, but there are specific genetic loci associated with specific diseases. Examples of neurodegeneration include – Alzheimer’s disease, Parkinson’s disease, Amyotrophic lateral sclerosis (ALS), and Huntington’s disease.
Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s disease, is a progressive neurodegenerative disease of the brain and spinal cord that results in loss of muscle control. ALS affects the ability to speak, move and breathe. There are almost 6,000 new cases per year in the United States. About 10% of the cases are inherited (familial ALS) and 90% of cases are spontaneous. Typical onset occurs from the 40’s through mid-60’s. A number of genes have been identified as associated with ALS, notably mutations in SOD-1.
There are more than 5 million people living with Alzheimer’s disease (AD) in the United States. Amyloid plaques and tau tangles are a common feature in the brain of patients with advanced AD. The cortex and hippocampus are primarily affected resulting in memory loss and a decline in reasoning and decision making abilities. As the disease progresses, patients lose the ability to care for themselves and perform everyday tasks.