Introduction

Amyotropic Lateral Sclerosis (ALS), or Lou Gehrig's Disease, is predominantly a male disease that begins to affect individuals between the ages of forty and seventy years of age. The three forms of ALS are sporadic, familial, and Guamian. ALS progresses rapidly with the average life expectancy after diagnosis being two to five years. The disease is characterized by progressive weakness in the extremities that eventually begins to affect all motor tasks caused by degeneration of the motor neurons in the brain and spinal cord. Currently the mechanism for this illness has not been determined but a number of theories exist including glutamate toxicity, growth factor deficiency, oxidative stress, carbohydrate dysmetabolism, mitochondrial dysfunction, calcium and magnesium deficiency, autoimmune disease, heavy metal accumulation, and infectious diseases such as Bordetella pertussis. The link to Bordetella pertussis would explain the concentration of disease in third world coutries where vaccination is less common, while the male-predominance points to an X-linked genetic abnormality. The most likely hypothesis is a defective glutamate transmitter that allows glutamate to accumulate to toxic levels. ALS sufferers also have been shown to have elevated levels of serum IgG and the C4 component of complement, while 50% are found to have blood-brain barrier (BBB) damage.

Laboratories Investigating Amyotropic Lateral Sclerosis & the Role of the Blood Brain Barrier

• The University of Miami's ALS Clinical and Research Program
  
• The Neuroapoptosis Laboratory at Harvard Medical School