A familial form of paraplegia characterized by spasticity – hypertonicity and weakness - limited mainly to the lower extremities. This is followed by involvement of upper limbs, dysarthria, and dysphagia. Degeneration of the pyramidal tract of the spinal cord and of the columns of Goll is the principal histological feature. Ocular complications may include strabismus, pupillary defects, macular degeneration, and optic atrophy. Occurs in either sex, but is more common in young males. This is a very rare disease, with a frequency of about 1 in 30.000 persons. It is most common in Nordic countries. Inheritance is autosomal recessive (usually), or sex-linked.
- A. G. G. von Strümpell:
Beiträge zur Pathologie des Rückenmarks.
Archiv für Psychiatrie und Nervenkrankheiten, Berlin, 1880, 10: 676-717.
- M. Lorrain:
Contribution à l’étude de la paraplégie spasmodique familiale.
Thesis de Paris, 1898.