Hereditary spastic paraplegia (HSP) is a group of genetically heterogeneous neurodegenerative disorders characterized by slowly progressive spasticity and weakness of the lower limbs. HSP is caused by failure of development or selective degeneration of the corticospinal tracts, which contain the longest axons in humans. The most common form of HSP is caused by mutations of the spastin gene (SPA...

Statement of the Problem The purpose of this study was to ascertain whether spastic and athetoid cerebral palsied children differ in performance on tasks which employ Jensen's Two-Level Theory of Mental Abilities. Previous research established that spastic and athetoid children perform differently on tests which measure psycholinguistic abilities. Since language and intelligence are closely rel...

PURPOSE Spastic mutant mice have abnormal gait and righting behavior, and the responses of their retinal ganglion cells have recently been shown to be abnormal. The former defects have been linked to a reduction of glycine-receptor density in the spinal cord of spastic mutants, but the cause of the retinal defects has not yet been determined. The authors thus tested for reduced glycine-receptor...

Increasing evidence suggests that structural changes within muscle and surrounding tissues are associated with creating and/or increasing muscle stiffness and resistance to stretch in spastic cerebral palsy (CP). The goal of this preliminary study was to determine whether Myofascial Structural Integration, a specific, complementary, deep tissue manipulation technique designed to reorganize musc...

Leg muscle EMG responses evoked by short treadmill acceleration impulses applied during stance were analysed in patients with spastic hemiparesis. The compensatory reactions on the unaffected side consisted of a diphasic pattern of leg muscle activation. The first response could best be described as a polysynaptic spinal stretch reflex response. This response was absent on the spastic side, exc...

Though epilepsy occurs in 15-90% of children with cerebral palsy (CP) its clinical course is not well defined. We therefore conducted studies of 198 children with CP seen in Pediatric Neurology Department of the Medical Academy in Białystok between 1994 and, 2001. The aim was to evaluate the risk factors, incidence and prognosis of epilepsy in CP. The overall epilepsy incidence was 41.4%. Epile...

The aims of the present study were to investigate the effects of spasticity (1) on muscle tendon unit (MTU) length-force characteristics of rat m. gastrocnemius (GAS), m. soleus (SOL) and m. plantaris (PL), and (2) on epimuscular myofascial force transmission between synergistic GAS, SOL and PL, as well as between the calf muscles and antagonistic m. tibialis anterior (TA). The effects of spast...

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...

Spastic thumb deformity is the result of imbalance between intrinsic and extrinsic forces acting across unstable joints. This paper presents a classification of spastic thumb deformity based on the accurate assessment of the deforming forces, outlines methods for their correction and reviews the results of our surgery. Thumb reconstruction procedures were performed in 32 patients with 33 spasti...