Arch Ital Biol
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Review Biography Historical Article
ALS, what new 144 years after Charcot?
The most important challenge of ALS remains finding biomarkers. Clinical features remain of key importance in the diagnosis and for follow up. Neurophysiology remains difficult to use in clinical trials. ⋯ Among proteins and chemical markers, one of the most promising marker is the level of Nogo in muscle biopsy. In CSF, many candidate proteins have been suggested but their sensitivity and specificity remains disappointing. Recently, -omics technologies have been applied to try to discover biomarkers in ALS, including genomic, proteomic and metabolomic methodologies.
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There is increasing clinical, imaging and neurophatological evidence that amyotrophic lateral sclerosis (ALS) represents a multisystem neurodegenerative disease. Neurodegeneration is not restricted to motor neurons, but also includes parts of the brain other than the motor cortex, especially the prefrontal and/or anterior temporal lobe, that contribute to the clinical syndrome. ⋯ The presence of a frontal dementia in ALS has also crucial practical consequences for management of the patients, whose disorder requires critical life decisions for enteral nutrition and respiratory complications. It is our intent to provide a brief overview of the relationships between ALS and FTD.