Although they are common symptoms of ALS , there is little information on the prevalence spasticity and spasticity-related pain. Consecutive patients were prospectively recruited from an referral center. Clinical assessment, functional scores, features pains has been recorded. In a cohort 150 patients, 36% presented with spasticity. Spastic younger, longer duration disease. Spasticity accelerat...

BACKGROUND Mutations in the SPG3A gene (atlastin protein) cause approximately 10% of autosomal-dominant hereditary spastic paraplegia. For many subjects with an SPG3A mutation, spastic gait begins in early childhood and does not significantly worsen even over many years. Such subjects resemble those with spastic diplegic cerebral palsy. To date, only 9 SPG3A mutations have been reported. OBJE...

An unusual form of hereditary spastic paraplegia is described. Affected females have a late-onset slowly progressive spastic paraparesis. Affected males show oligophrenia with a rapidly progressive spastic quadriplegia. The mode of inheritance is consistent with sex-linkage, with partial manifestation in female carriers.

This study investigated the acoustic phonetic characteristics of Korean diphthongs in 16 healthy controls, in 8 speakers with spastic dysarthria, and in 8 speakers with flaccid dysarthria. The subjects read /ja/, /wi/ and mean formant values were analyzed based on the spectrograms. The formant transition duration for the vowel /ja/ was longer for speakers with flaccid dysarthria than the contro...

X linked hereditary spastic paraplegia is a rare condition that has been divided into two forms (the pure spastic form and the complicated form) as a function of clinical course and severity. A gene for pure hereditary spastic paraplegia (SPG2) has been mapped to the proximal long arm of the X chromosome (Xq21) by linkage to the DXS17 locus, while a gene for a complicated form of the disease ha...

The passive mechanical properties of small muscle fiber bundles obtained from surgical patients with spasticity (n = 9) and patients without neuromuscular disorders (n = 21) were measured in order to determine the relative influence of intracellular and extracellular components. For both types of patient, tangent modulus was significantly greater in bundles compared to identical tests performed...

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...

INTRODUCTION: Spasticity and contracture commonly occur in neurological disorders. Biomechanical changes of spastic muscle fibers are still not clear and mainly sarcomere length was investigated in spastic fibers in vivo. On the one hand, it was reported that single fibers from spastic muscle of children with cerebral palsy had shorter in vitro length of about 1.84 μm. On the other hand, in viv...