Perry disease is a hereditary neurodegenerative disease with autosomal dominant inheritance. It is characterized by parkinsonism, psychiatric symptoms, unexpected weight loss, central hypoventilation, and transactive-response DNA-binding protein of 43kD (TDP-43) aggregation in the brain. In 2009, Perry disease was found to be caused by dynactin I gene (DCTN1), which encodes dynactin subunit p150 on chromosome 2p, in patients with the disease. The dynactin complex is a motor protein that is associated with axonal transport. Presently, at least 8 mutations and 22 families have been reported; other than the “classic” syndrome, distinct phenotypes are recognized. The neuropathology of Perry disease reveals severe degeneration in the substantia nigra and TDP-43 inclusions in the basal ganglia and brain stem. How dysfunction of the dynactin molecule is related to TDP-43 pathology in Perry disease is important to elucidate the pathological mechanism and develop new treatment.

Objective: To clarify the reason why the target levels of low-density lipoprotein (LDL)-cholesterol are hardly attained.
Methods: The questionnaire was performed for 237 general practitioners on prescription of HMG-CoA reductase inhibitors (statins), just after the publication of “Japan Atherosclerosis Society (JAS) Guidelines for Prevention of Atherosclerotic Cardiovascular Diseases 2007”.
Results: The responses could be summarized as 1) almost all doctors recognized the significance of target levels of LDL-cholesterol; 2) however, general physicians have such a recognition to a lesser extent, compared with cardiologists; 3) when prescribing statins, 66.2% of them had concerns about drug-drug interactions; 4) as the adverse events with statins, they listed creatine kinase (CK) elevations (86.5%), hepatic dysfunction (74.3%) and myalgias/rhabdomyolysis (70.9%); 5) in contrast, less than 20% of them listed the fulminant hepatic failure, gastrointestinal symptoms and fluctuation of blood glucose levels; 6) the threshold value of CK to discontinue statins was 500 IU/L in 44.4% and 200-300 IU/L in 39.6%; and 7) 78.5% of them believed that statins have the pleiotropic ability such a anti-inflammatory effect clinically, in addition to lipid-lowering.
Conclusions: It is important to serve the drug information about the safety of statins to the general practitioners for the management of hyperlipidemia, based on original articles.