Imaging and neurochemical markers for diagnosis and disease progression in ALS

Based on the development of a transgenic animal model, an increasing number of experimental strategies have revealed the potential to modify the selective degeneration of motor neurons, a feature unique to motor neuron diseases such as amyotrophic lateral sclerosis (ALS). The translation of this success into therapeutic effects in human diseases is a challenge of the future. For this purpose, tools must be developed which serve as diagnostic and surrogate markers for diagnosis and disease progression. Currently, to understand the pathogenesis of the spinal cord disease, the focus remains on more traditional electrodiagnostic techniques. For the characterization of the involvement of brain structures, imaging techniques are increasingly explored. This review focuses on the use of imaging techniques as surrogate markers for the involvement of the brain in motor neuron disorders, but also tries to point out that the final goal will be the development of cheap biochemical markers for the screening of populations at risk. V 2001 Elsevier Science B.V. Ail rights reserved.