Two brothers presented with a slowly progressive scapuloperoneal syndrome starting in early childhood. Initially there were myopathic EMG changes, but these changed to those of denervation. Neuromuscular biopsies at an interval of five years confirmed the neurogenic character of the muscle atrophy.

progressive hemifacial atrophy, also known as parry-romberg syndrome, is an uncommon degenerative and poorly understood condition. it is characterized by a slow and progressive but self-limited atrophy affecting one side of the face. the incidence and the cause of this alteration are unknown. a cerebral disturbance of fat metabolism has been proposed as a primary cause. possible factors that ar...

Let S = Sg,n be a connected, orientable surface of genus g ≥ 0 with n ≥ 0 punctures. Let Teich(S) denote the corresponding Teichmüller space, and let Mod(S) denote the mapping class group of S. Understanding the analogy of Teich(S) with symmetric spaces is a wellknown theme. Recall that a complete Riemannian manifold X is symmetric if it is symmetric at each point x ∈ X: the map γ(t) 7→ γ(−t) w...

We describe the geometry of geodesics on a Lorentz ellipsoid: give explicit formulas for the first integrals (pseudo-confocal coordinates), curvature, geodesically equivalent Riemannian metric, the invariant area-forms on the timeand space-like geodesics and invariant 1-form on the space of null geodesics. We prove a Poncelet-type theorem for null geodesics on the ellipsoid: if such a geodesic ...

We introduce a family of extremal polynomials associated with the prolongation of a stratified nilpotent Lie algebra. These polynomials are related to a new algebraic characterization of abnormal subriemannian geodesics in stratified nilpotent Lie groups. They satisfy a set of remarkable structure relations that are used to integrate the adjoint equations.

Inspiratory resistance induced by breathing through an impedance threshold device (ITD) reduces intrathoracic pressure and increases stroke volume (SV) in supine normovolemic humans. We hypothesized that breathing through an ITD would also be associated with a protection of SV and a subsequent increase in the tolerance to progressive central hypovolemia. Eight volunteers (5 men, 3 women) were i...

We shall be interested in the following Erdős-Ko-Rado-type question. Fix some set B ⊂ [n]. How large a family A ⊂ P [n] can we find such that the intersection of any two sets in A contains a cyclic translate (modulo n) of B? Chung, Graham, Frankl and Shearer have proved that, in the case where B is a block of length t, we can do no better than to take A to consist of all supersets of B. We give...

Horizontal gaze palsy with progressive scoliosis (HGPS) is a rare, autosomal recessive disorder characterized by a congenital absence of conjugate horizontal eye movement, with progressive scoliosis developing in childhood or adolescence. The authors identified two unrelated consanguineous families with HGPS. Genomewide homozygosity mapping and linkage analysis mapped the disease locus to a 30-...