Living standard is related to microregional differences in stroke characteristics in Central Europe: the Budapest Districts 8-12 Project.

OBJECTIVES: To test whether str oke features relate to living standard within one city by comparing 2 districts. METHODS: District-8 (D-8) ranks the last, whereas District-12 (D-12) is the second regarding personal monthly income of the 23 districts of Budapest, Hungary. Stroke cases hospitalized in 2007 were identified by the database of the National Health Insurance Fund and postal codes for living address. Case certification was performed by personal visits to the general practitioners. Demographic data, risk factors and survival status in 2010 were analyzed using the anonymized database. RESULTS: Three-year case fatality was 36.6 % in D-8 and 31.5 % in D-12 (p = 0.24). Of the fatal cases, men were more than 12 years younger in D-8 than in D-12 (69.2 ± 13.3 vs. 82.4 ± 9.2 years, p < 0.001). Men died younger than women in D-8 (69.2 ± 13.3 vs. 75.2 ± 12.4; p = 0.036), but not in D-12 (82.4 ± 9.2 vs. 81.9 ± 7.3, p = 0.8). Non-treated hypertension, alcohol dependence, and smoking were significantly more prevalent in the poor district (p < 0.01 for all). CONCLUSION: In national stroke programs of former Eastern Block countries, primary prevention should focus especially on male populations of less wealthy regions.

[Neurological complications of Fabry-disease]. / A Fabry-kór neurológiai szövodményei.

BACKGROUND: Fabry-disease (FD) is a rare X-linked lysosomal storage disease. Deficiency of alpha-galactosidase A activity leads to the accumulation of neutral glycosphingolipids, primarily globotriaosylceramide (GL-3) in various tissues, particularly blood vessels, kidneys, myocardium and in ganglions of the peripheral and autonomic nervous system and causes diverse symptoms. The classical phenotype is seen in most males and rarely in females. In women, symptoms start later and the severity is milder. Both peripheral and central nervous system can be both affected. OBJECTIVES: Fabry-patiens and gene-carriers in the central region of Hungary are treated in the 2nd Pediatric Department, Semmelweis University. These patients are consulted by an interdisciplinary team. At present, four hemizygous male, four heterozygous female Fabry-patients and three asymptomatic heterozygous gene carriers are followed. RESULTS: After reviewing the neurological complications of FD, we present clinical and neuroimaging data of our patients. CONCLUSION: We emphasize that neurologists should suspect the rare monogenic FD in the case of acroparaesthesia and heat-cold intolerance in childhood or adolescence; clinical signs of TIA/stroke or unexplained MRI alterations suggesting small vessel disease in young adults. Early diagnosis and introduction of enzyme replacement therapy (ERT) can halt or reverse progression.