The Cognitive Decline of Marshal Philippe Pétain.

In 1940, at the age of 84, Marshal Pétain was appointed the head of state and government of France. His health was excellent but he tired easily. He felt unable to learn and his memory was weak. During a crisis situation in 1942, he did not lead, plan and decide and he was replaced as head of government. From 1943 on, he was increasingly apathetic. In 1945/1946 he had difficulty finding words after a short conversation. A parliamentary committee concluded in 1947 that he was senile. His mental condition worsened in the years thereafter. In retrospect, it is clear that the final responsibility for the policies of the French government in the Second World War had rested on a man who was going through a predementia process of cognitive decline.

A short history of the notion of neurodegenerative disease.

The notion of heredity of degenerative constitutions of human beings contributed in the nineteenth century to the fear for deterioration of the human race and in the twentieth century to attempts by several Western Countries, particularly Germany, to improve the inborn qualities of their populations by eugenic measures. In the years following World War II, the term eugenics was eradicated from medicine. The qualification degenerative disappeared from genetics and psychiatry but remained in use to denote decay of tissues and cells. The adjective neurodegenerative came in vogue in neurology and was mostly applied to brain diseases. Definitions in the literature indicate that neurodegenerative diseases are considered as to be age related, incurable, and largely untreatable chronic progressive diseases of the central nervous system. Scrutiny of available data shows that the notion concerns an ill-defined group of genetic and idiopathic disorders. Genetic central nervous system diseases may become manifest at all ages and are accessible for symptomatic treatment. Investigations of animal models suggest that not all neurodegenerative diseases are inherently incurable. Alternatives for the terms neurodegenerative and degenerative are available.

Comparison of weakness progression in inclusion body myositis during treatment with methotrexate or placebo.

We investigated whether 5 to 20mg per week oral methotrexate could slow down disease progression in 44 patients with inclusion body myositis in a randomized double-blind placebo-controlled study over 48 weeks. Mean change of quantitative muscle strength testing sum scores was the primary study outcome measure. Quantitative muscle strength testing sum scores declined in both treatment groups, -0.2% for methotrexate and -3.4% for placebo (95% confidence interval = -2.5% to +9.1% for difference). There were also no differences in manual muscle testing sum scores, activity scale scores and patients' own assessments after 48 weeks of treatment. Serum creatine kinase activity decreased significantly in the methotrexate group. We conclude that oral methotrexate did not slow down progression of muscle weakness but decreased serum creatine kinase activity.