Hutchinson-Gilford-Progerie-Syndrom (HGPS)
Carotid
artery dissection in Hutchinson-Gilford Progeria: a case report. Occlusion of the internal carotid artery in the petrous segment to the cavernous segment (single arrow) preceded by a long and progressive stenosis in the sub-petrous segment (double arrow)
Carotid
artery dissection in Hutchinson-Gilford Progeria: a case report. Coiling in sub-petrous segment (single arrow).Supra bulbar stenosis and post-stenotic dilatation (double arrow)
Hutchinson-Gilford
progeria syndrome accompanied by severe skeletal abnormalities in two Chinese siblings: two case reports. Radiography and magnetic resonance imaging features in case 1. (A,B) The elder sibling showed generalized and marked osteoporosis with relative expansion of the metaphyseal areas of the bones, completely absent clavicles, partial osteolysis of the bilateral posterior segments of the first to fourth ribs, absence of the costal head of the right 12th rib; severescoliotic deformities, complete osteolysis and resorption of the superior fragment of both radii, a dislocation of the inferior segment of the right ulnoradial joint, and an old, healed fracture of the interior segment of the right ulna. (C) Fixed flexion deformities of the phalanges in the elder sibling with absence of the distal phalanges of the second to fifth fingers. (D) Magnetic resonance imaging features of the elder sibling showing a small pituitary gland with a concavity of the superior border (with a height of about 1mm).
Progeria is a term used for any syndrome in which a person is prematurely aged, however, it most commonly refers to Hutchinson-Gilford progeria syndrome (HGPS).
Radiographic features
Although the primary clinical manifestations of progeria include short stature and skin changes, the disease is associated with a variety of imaging manifestations :
- erosion of superior aspects of ribs
- osteolysis of distal clavicle
- acro-osteolysis
- Wormian bones
- osteoporosis
- rib notching
- periarticular soft tissue calcification
- premature atherosclerotic vascular calcification
Differential diagnosis
History and etymology
The term "progeria" comes from the combination of "pro" (πρό), the greek word for "before" or "premature", and "gēras" (γῆρας), a term for "old age".
HGPS was first described by Jonathan Hutchinson in 1886 and independently by Hastings Gilford in 1897 .
Siehe auch:
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Assoziationen und Differentialdiagnosen zu Progerie: 