Autosomal dominant spinocerebellar ataxias (SCAs) are a group of neurodegenerative disorders clinically characterized by late-onset ataxia and variable other manifestations. Genetically and clinically, SCA is highly heterogeneous. Recently, CAG repeat expansions in the gene encoding TATA-binding protein (TBP) have been found in a new form of SCA, which has been designated SCA17. To estimate the frequency of SCA17 among white SCA patients and to define the phenotypic variability, we determined the frequency of SCA17 in a large sample of 1,318 SCA patients. In total, 15 patients in four autosomal dominant SCA families had CAG/CAA repeat expansions in the TBP gene ranging from 45 to 54 repeats. The clinical features of our SCA17 patients differ from other SCA types by manifesting with psychiatric abnormalities and dementia. The neuropathology of SCA17 can be classified as a "pure cerebellar" or "cerebello-olivary" form of ataxia. However, intranuclear neuronal inclusion bodies with immunoreactivity to anti-TBP and antipolyglutamine were much more widely distributed throughout the brain gray matter than in other SCAs. Based on clinical and genetic data, we conclude that SCA17 is rare among white SCA patients. SCA17 should be considered in sporadic and familial cases of ataxia with accompanying psychiatric symptoms and dementia.

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Abstract. We start discussing the group of automorphisms of the field of complex numbers, and describe, in the special case of polynomials with only two critical values, Grothendieck's program of 'Dessins d' enfants', aiming at giving representations of the absolute Galois group. We describe Chebycheff and Belyi polynomials, and other explicit examples. As an illustration, we briefly treat difference and Schur polynomials. Then we concentrate on a higher dimensional analogue of the triangle curves, namely, Beauville surfaces and varieties isogenous to a product. We describe their moduli spaces, and show how the study of these varieties leads to new interesting questions in the theory of finite (simple) groups.

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Abstract. We construct many new surfaces of general type with q = p g = 0 whose canonical model is the quotient of the product of two curves by the action of a finite group G, constructing in this way many new interesting fundamental groups which distinguish connected components of the moduli space of surfaces of general type.We indeed classify all such surfaces whose canonical model is singular (the smooth case was classified in an earlier work).As an important tool we prove a structure theorem giving a precise description of the fundamental group of quotients of products of curves by the action of a finite group G.