[Vitamin signaling underlying degenerative diseases].

Our knowledge of the significance of nutrients in the treatment of degenerative diseases of tne nervous system and muscle is poor. In addition to scarcity of vitamins in the diet, a tissue-specific vitamin deficiency may underlie these diseases. Gene defects of folate or thiamine transport proteins cause severe pediatric encephalopathies that can partly or completely be prevented with early vitamin therapy.

[Investments of research and treatment of brain diseases will pay of time].

In 2010, a quarter of direct healthcare cost in Europe were spent on brain diseases. The importance of preventing and treating brain diseases and maintaining of functional capacity of the brain will increase in our society with ageing population and with increasing cognitive requirements of modern working life. Public funding of basic and clinical neuroscience has, however, frozen to levels achieved years ago, clinical research of brain diseases being at a particular risk.

[Mitochondrial recessive ataxia syndrome (MIRAS) and valproate toxicity].

The clinical phenotypes vary considerably and can be divided into three groups: 1) childhood-onset encephalopathy and hepatopathy, 2) juvenile onset refractory epilepsy and migraine-like headaches, and 3) adult-onset ataxia and neuropathy with additional symptoms such as psychiatric symptoms and cognitive impairment. The life-threatening MIRAS epilepsy should be actively treated, as it is associated with poor prognosis. The form of MIRAS, starting as acute, treatment resistant epilepsy, is important to diagnose, since valproate therapy almost always leads to acute liver failure requiring liver transplantation.