Summary of the proceedings of the First International Workshop on CADASIL. Paris, May 19-21, 1993.
نویسندگان
چکیده
T he acronym CADASIL for "cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy" 1 was recently suggested for the disease formerly called "hereditary multi-infarct dementia" by Sourander in 1977. 2 This condition is characterized clinically by recurrent subcortical isch-emic strokes starting in mid-adulthood and sometimes leading to pseudobulbar palsy and dementia. Other symptoms include migrainelike attacks of headache and psychiatric symptoms. Magnetic resonance imaging (MRI) of the brain is always abnormal and shows relatively consistent signs of small, deep infarcts and leukoencephalopathy. Pathology likewise shows numerous small, deep infarcts with diffuse myelin loss and pallor of the hemispheric white matter. The underlying lesion is a nonatherosclerotic nonamyloid angiopathy affecting mainly the small penetrating arteries of the subcortical white matter and basal ganglia. 2 ^ Genetic linkage analysis performed in two unrelated French families recently assigned the disease locus to chromosome 19ql2.' Eight unrelated European families 1 " 9 have thus far been reported that share similar clinical, neuroimaging, and pathological features. All physicians involved in the study of these eight families were invited to take part in the First International Workshop on CADASIL, which was also attended by a number of neurologists, psychiatrists , neuroradiologists, and neuropathologists presently dealing with nonreported affected families. The aim of the workshop was twofold: first, to establish a kind of "state of the art" of CADASIL, and second, to form the basis of a European study group for this condition. summarized the clinical findings of one already-reported family.
منابع مشابه
How to Work Collaboratively Within the Health System: Workshop Summary and Facilitator Reflection
Effectiveness in health services research requires development of specific knowledge and skills for working in partnership with health system decision-makers. In an initial effort to frame capacity-building activities for researchers, we designed a workshop on working collaboratively within the health system. The workshop, based on recent research exploring health syste...
متن کاملMetastatic skin carcinoma, A Report on a Rare case
SUMMARY Development of metastatic deposits in skin indicates that the cancer is disseminated, and reveals a poor prognosis.but rarely, it is possible for cutaneous metastasis to be the first clinical manifestation of an occult internal malignancy. Unfortunately, a few of cutaneous metastatic deposits have characteristic clinical appearance, that may suggest the possibility of metastasis in dif...
متن کاملIEEE International Workshop On Robot And Human Communication
What do you do to start reading ieee international workshop on robot and human communication proceedings hosei university tokyo japan 1 3 september 1992 92th0469 7? Searching the book that you love to read first or find an interesting book that will make you want to read? Everybody has difference with their reason of reading a book. Actuary, reading habit must be from earlier. Many people may b...
متن کاملProceedings of the First MEFOMP International Conference of Medical Physics: November 2-4, 2011, Shiraz, Iran
متن کامل
Workshop on Trustworthy Software
s Collection Workshop Trustworthy Software 2006 INTERREG IIIC/e-Bird Serge Autexier, Stephan Merz, Leon van der Torre, Reinhard Wilhelm and Pierre Wolper Abstract. On 18-19 May 2006, the Saarland University organized a two-day workshop about "Trustworthy Software" in order to present and foster the research competence in the SaarLorLuxWallonie region in the area of developing safe, secure and r...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- Stroke
دوره 25 3 شماره
صفحات -
تاریخ انتشار 1994