objective hereditary spastic paraplegia (hsp) is a degenerative disease of genetic origin affecting the corticospinal tracts in the spinal cord. there are three forms of inheritance: autosomal dominant hsp, autosomal rececive hsp and x-linked hsp. this disease is characterized by progressive spasticity of leg muscles with varying degrees of stiffness and weakness of other muscle groups. in this...

spastic paraplegia 18 is an autosomal recessive disorder characterized by motor dysfunction, joint contracture and mental retardation. we describe two families as three cases. case 1 is a 35-year-old woman with and spasticity and mild weakness in lower limbs. case 2 and 3 are a sister and brother aged six and two respectively. the older sister suffered from lower limb spasticity, equinovarus an...

Background: In the Czech Republic a standardized measuring method has not been applied yet in order to monitor amount and intensity of physical activity persons with spinal cord injury. Objective: The purpose this study was translate, adapt validate administration scoring manual semi-structured interview Physical Activity Recall Assessment for People Spinal Cord Injury (PARA-SCI), focused on su...

Coarctation and occlusion of the aorta is a rare condition that typically presents with hypertension or cardiac failure. However, neuropathy or myelopathy may be the presenting features of the condition when an intraspinal subarachnoid hemorrhage has compressed the spinal cord causing ischemia. We report two cases of middle-aged males who developed acute non-traumatic paraplegia. Undiagnosed co...

WE REPORT THE SUCCESSFUL TREATMENT OF THORACOABDOMINAL DISSECTION, WHICH EXTENDED INTO THE LEFT ILIAC ARTERY, DESPITE TWO INDEPENDENT COMPLICATIONS: graft infection and a relatively rare, delayed postoperative paraplegia. The paraplegia suddenly occurred on postoperative day 10, and after an intravenous infusion of heparin and methylprednisolone, it gradually subsided. Moreover, graft infection...

Paraplegia is a rare complication of surgery for acute type A aortic dissection. We report a case of delayed postoperative paraplegia associated with necrosis of the thoracic vertebral bodies and soft tissue. The pathogenesis of delayed postoperative paraplegia is unknown, but our case report would strongly suggest ischemia of the descending thoracic intercostal arteries as the causative mechan...

Alterations in proteins that regulate endoplasmic reticulum morphology are common causes of hereditary spastic paraplegia (SPG1-78, plus others). Mutations in the REEP1 gene that encodes an endoplasmic reticulum-shaping protein are well-known causes of SPG31, a common autosomal dominant spastic paraplegia. A closely-related gene, REEP2, is mutated in SPG72, with both autosomal and recessive inh...