JASPAC: Japan Spastic Paraplegia Research Consortium

Hereditary spastic paraplegia.

Hereditary spastic paraplegia (HSP) or Strümpell-Lorrain syndrome is a heterogeneous group of inherited disorders, with prevalence ranged from 4.3 to 9.6 cases per 100,000 population. A common feature of these disorders is the slowly progressive and often severe spasticity, noticeably especially in the low limbs. Conventionally, HSP is divided into two clinical groups, uncomplicated (pure spast...

Hereditary Spastic Paraplegia.

111 families, and Paskind and Stone (1933) also summarized the literature with accounts of another 36 reports from 40 families. Since then about 50 further reports have appeared, covering some 60 families, bringing the total so far to 176 reports of 215 families. Of these the majority have originated in Germany or elsewhere in Europe, but with a substantial number from the Americas. In Britain,...

Subdural Hematoma and Spastic Paraplegia

This is a case of progressive lower limb weakness in a 72-year-old man secondary to chronic bilateral subdural hematoma. This patient presented with a 3 week history of progressive difficulty in walking, with upper motor neuron signs but no sensory deficit. He had no significant risk factors for chronic subdural hematoma. Literature search revealed only one similar documented case of painless b...

Spastic Paraplegia in Middle Age.

[Japan Multiple System Atrophy Research Consortium (JAMSAC)].

Multiple system atrophy (MSA) is a sporadic neurodegenerative disorder characterized by various combinations of autonomic dysfunction, cerbellar symptoms, parkinsonism and pyramidal signs. Although molecular mechanisms of MSA remain to be elucidated, genome-wide association studies (GWAS) may provide clues as to the susceptibility genes for MSA. Establishment of the natural history of MSA is es...